Umphred's Neurological Rehabilitation, 6E (2013) (PDF) (UnitedVRG) - 1

UMPHRED’S
NEUROLOGICAL
REHABILITATION
UMPHRED’S
NEUROLOGICAL
REHABILITATION SIXTH EDITION
Darcy A. Umphred, PT, PhD, FAPTA

Emeritus Professor and Past Chair
Department of Physical Therapy
School of Pharmacy and Health Sciences
University of the Pacific
Stockton, California
Gordon U. Burton, PhD, OTR/L

Professor Emeritus and Past Chair
Department of Occupational Therapy
San Jose State University
San Jose, California
Rolando T. Lazaro, PT, PhD, DPT, GCS

Associate Professor
Samuel Merritt University
Oakland, California
Margaret L. Roller, PT, MS, DPT

Professor and Graduate Coordinator
California State University, Northridge
Northridge, California
Clinical Instructor
NeuroCom International, a division of Natus
Clackamas, Oregon
3251 Riverport Lane
St. Louis, Missouri 63043
UMPHRED’S NEUROLOGICAL REHABILITATION ISBN: 978-0-323-07586-2
Copyright © 2013 by Mosby, an imprint of Elsevier Inc.

Copyright © 2007, 2001, 1995, 1990, 1985 by Mosby, Inc., an affiliate of Elsevier Inc.
Chapter 18: Beyond the Central Nervous System: Neurovascular Entrapment Syndromes: Laura J. Kenny
retains copyright to her original figure 18-6.
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Notices
Knowledge and best practice in this field are constantly changing. As new research and experience broaden
our understanding, changes in research methods, professional practices, or medical treatment may become
necessary.
Practitioners and researchers must always rely on their own experience and knowledge in evaluating and
using any information, methods, compounds, or experiments described herein. In using such information
or methods they should be mindful of their own safety and the safety of others, including parties for whom
they have a professional responsibility.
With respect to any drug or pharmaceutical products identified, readers are advised to check the most
current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be
administered, to verify the recommended dose or formula, the method and duration of administration, and
contraindications. It is the responsibility of practitioners, relying on their own experience and knowledge
of their patients, to make diagnoses, to determine dosages and the best treatment for each individual
patient, and to take all appropriate safety precautions.
To the fullest extent of the law, neither the Publisher nor the uthors, contributors, or editors, assume any
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Library of Congress Cataloging-in-Publication Data

Umphred’s neurological rehabilitation / [edited by] Darcy Umphred ... [et al.].—6th ed.
p. ; cm.
Neurological rehabilitation
Rev. ed. of: Neurological rehabilitation / [edited by] Darcy A. Umphred ; with section editors, Gordon U.
Burton, Rolando T. Lazaro, Margaret L. Roller. 5th ed. c2007.
Includes bibliographical references and index.
ISBN 978-0-323-07586-2 (hardcover : alk. paper)
I. Umphred, Darcy Ann. II. Neurological rehabilitation. III. Title: Neurological rehabilitation.
[DNLM: 1. Nervous System Diseases—rehabilitation. WL 140]
616.8’0462—dc23
2012019193
Executive Content Strategist: Kathy Falk

Content Manager: Jolynn Gower
Senior Content Development Specialist: Christie M. Hart
Publishing Services Manager: Catherine Jackson
Senior Project Manager: Carol O’Connell
Design Direction: Kim Denando
Printed in the United States of America
Last digit is the print number: 9 8 7 6 5 4 3 2 1

Dedicated to: A sequential process of learning from the first to the sixth edition.
To
Gordon, Jeb, Benjamin, my mother, Janet, my daughters-in-law Julianne and Tassie, and
our four grandchildren, Jackson, Jelena, Alexander, and Cameron, whose love, patience,
and understanding constantly gave and have continued to give me strength and joy.
To
All those special people whose insights, wisdom, guidance, and patience have helped to
give the authors of these chapters their unique gifts and talents, as well as their willingness
to share their thoughts with all of you.
To
Very dear friends, colleagues, and past chapter authors who gave so much energy, dedication,
and service both scholarly as well as practicing clinicians. Over the last 30 years, the book’s
family has changed, as has the evolution of the text. With great regret we have had to say
good-bye to five authors, Mary Jane Bouska, Jane Schneider, Laura Smith, Donna El-Din,
and Christine Nelson. The professions will miss all of you, but your gifts continue to make
a difference.
To
Life, to each person’s journey and to all those who give opportunities for others’ growth
along that journey. Special thanks to my immediate family and all my friends and colleagues
I hold so close to my heart. Because of all of you, my journey has been constantly renewed
with love, warmth, and guidance. No one could feel wealthier than I.
To
Each day we are allowed to walk on this earth. Life is very precious, and how long we
will be allowed to stay is an unknown. I have learned to appreciate this time and hope
none of us become so busy that we forget its finiteness. Enjoy the journey and always
find time to stop and appreciate who you are, what you are, and those gifts you have
been given to share with the rest of us. We walk alone in physical form but surrounded
by all the energies of others as well as our own. Hopefully, as we continue this adventure
we share and give positive thoughts and energy to life and leave having given much
more than we have taken. Each person we meet, whether as a student, a teacher, a family
member, a friend, a colleague or a patient, has something to teach us and, hopefully, we
in turn allow those we meet to grow and learn from us. This is a wondrous journey, and
I hope all of you enjoy your path as much as I have mine.
To
My fellow editors, Dr. Gordon Burton, Dr. Rolando Lazaro, and Dr. Margaret Roller, for
all the time and total commitment they have made to the sixth edition of this text. Few
individuals reading and using this book and the complimentary online video site will
ever appreciate the hundreds if not thousands of hours these individuals made editing
chapters and videos and the totality of their commitment to the future of our professions.
Darcy A. Umphred
Contributors
Paula M. Ackerman, MS, OTR/L Sandra G. Bellamy, PT, MS, DPT, PCS
SCI Post Acute Rehab Manager Associate Professor
Shepherd Center Department of Physical Therapy
Atlanta, Georgia University of the Pacific
Janet Marie Adams, PT, MS, DPT
Professor Janet R. Bezner, PT, PhD
Department of Physical Therapy Vice President, Education and Governance and
California State University, Northridge Administration
Northridge, California Deputy Executive Director
American Physical Therapy Association
Diane D. Allen, PT, PhD Alexandria, Virginia
Associate Professor
University of California San Francisco/San Francisco State William G. Boissonnault, PT, DHSc, FAAOMPT, FAPTA
University Professor
Graduate Program in Physical Therapy Physical Therapy Program
San Francisco, California University of Wisconsin-Madison
Madison, Wisconsin
Leslie K. Allison, PT, PhD
Assistant Professor Jennifer M. Bottomley, PT, MS, PhD
East Carolina University Academic ad Clinical Educator in Geriatric Physical
College of Allied Health Sciences Therapy International
Department of Physical Therapy Adjunct Professor at the MGH Institute of Health Care
Greenville, North Carolina Professionals
Coordinates Rehabilitation Services for the Committee to
Brent D. Anderson, PT, PhD, OCS End Elder Homelessness/HEARTH in Boston
President Consultant for Amedisys Home Health Care and Hospice
Polestar Education Vice President for International PhysioTherapists Working
Adjunct Professor with Older People (IPTOP)
University of Miami Independent Educator and Geriatric Rehabilitation
Department in Physical Therapy Consultant
Miami, Florida Boston, Massachusetts
Ellen Zambo Anderson, PT, MA, GCS Annie Burke-Doe, PT, MPT, PhD
Associate Professor Associate Professor
University of Medicine and Dentistry of New Jersey University of St. Augustine for Health Science
Newark, New Jersey San Marcos, California
Joyce Ann, OTR/L, GCFP Gordon U. Burton, PhD, OTR/L
Occupational Therapist, Guild Certified Feldenkrais Practitioner Professor Emeritus and Past Chair
Highland Park, Illinois Department of Occupational Therapy
Myrtice B. Atrice, PT, BS San Jose State University
SCI Clinical Manager San Jose, California
Shepherd Center Katie Byl, PhD
Atlanta, Georgia Assistant Professor
Amy J. Bastian, PT, PhD University of California, Santa Barbara
Professor Department of Electrical and Computer Engineering
Neuroscience Santa Barbara, California
Johns Hopkins School of Medicine Marten Byl, PhD
Director Principal Scientist
Motion Analysis Laboratory Physical Sciences Inc.
Kennedy Krieger Institute Handover, Massachusetts
Baltimore, Maryland Visiting Scientist
Joanna C. Beachy, MD, PhD University of California, Santa Barbara
Associate Professor Santa Barbara, California
Division of Neonatology
Associate Director NBICU
University of Utah
Salt Lake City, Utah
vii
viii Contributors
Nancy N. Byl, PT, MPH, PhD, FAPTA Clayton D. Gable, PT, PhD
Professor and Chair Emeritus US Army, Headquarters MEDCOM
School of Medicine, Department of Physical Therapy and Fort Sam Houston, Texas
Rehabilitation Science Private Practice PT
University of California, San Francisco Adult and Pediatric Neuology
San Francisco, California San Antonio, Texas
Home Ex Pro
Beate Carrière, PT, CAPP, CIFK Chief Executive Officer
Physical Therapist, Author, Teacher Odessa, Texas
Evergreen Physical Therapy Specialist
Pasadena, California Mary Lou Galantino, PT, PhD, MSCE
Professor of Physical Therapy
Laurie Ruth Chaikin, MS, OTR/L, OD, FCOVD Holistic Health Minor Coordinator
Clinical Field Supervisor School of Health Sciences
VisionCare, Inc. The Richard Stockton College of New Jersey
Saratoga, California Galloway, New Jersey
Alain Claudel, PT, DPT, ECS Adjunct Researcher, CCEB
Board Certified Specialist in Clinical Electrophysiology Adjunct Associate Professor of Family Medicine and
Director, Rehabilitation Services Community Health
Community Hospital of the Monterey Peninsula University of Pennsylvania
Monterey, California Philadelphia, Pennsylvania
Carol M. Davis, DPT, EdD, FAPTA Teresa Gutierrez, PT, MS, PCS, C/NDT
Professor Emerita Pediatric Rehab Northwest, LLC
Department of Physical Therapy Gig Harbor, Washington
University of Miami Miller School of Medicine Ann Hallum, PT, PhD
Myofascial Release Physical Therapist Dean of Graduate Studies
Polestar Pilates Rehabilitation San Francisco State University
Coral Gables, Florida Professor
Judith A. Dewane, PT, DSc, NCS Graduate Program in Physical Therapy
Assistant Professor (CHS) University of California/San Francisco State University
Doctor of Physical Therapy Program San Francisco, California
Department of Orthopedics and Rehabilitation Jeffrey Kauffman, MD
University of Wisconsin Holistic Health Associate
Madison,Wisconsin Sacramento, California
Peter I. Edgelow, PT, MA, DPT Laura J. Kenny, PT, OCS, FAAOMPT
Assistant Clinical Professor Clinical Specialist
Graduate Program in Physical Therapy Occupational Health Department
University of California, San Francisco Oakland, California
San Francisco, California
David M. Kietrys, PT, PhD, OCS
Barbara Edmison, PT Associate Professor
Center Coordinator of Clinical Education Rehabilitation and Movement Sciences
Therapy Services Department University of Medicine and Dentistry of New Jersey
Santa Barbara Cottage Hospital Newark, New Jersey
Santa Barbara, California
Kristin J. Krosschell, PT, DPT, MA, PCS
Teresa A. Foy, OT, BS Assistant Professor
OT Therapy Manager Department of Physical Therapy and Human Movement
SCI Program Sciences
Shepherd Center Feinberg School of Medicine
Atlanta, Georgia Northwestern University
Kenda Fuller, PT, NCS Chicago, Illinois
Owner Rolando T. Lazaro, PT, PhD, DPT, GCS
South Valley Physical Therapy Associate Professor
Specialist in Neurologic Physical Therapy Department of Physical Therapy
Denver, Colorado Samuel Merritt University
Oakland, California
Contributors ix
Rachel M. Lopez, PT, MPT, NCS Myla U. Quiben, PT, PhD, DPT, GCS, NCS, CEEAA
Physical Therapist Assistant Professor
Barrow Neurological Institute Department of Physical Therapy
St. Joseph’s Hospital University of Texas Health Science Center San Antonio
Phoenix, Arizona San Antonio, Texas
Marilyn MacKay-Lyons, PT, PhD Walter Racette, CPO
Associate Professor Associate Clinical Professor
School of Physiotherapy University of California San Francisco
Dalhousie University Department of Orthopaedics
Affiliated Clinical Scientist San Francisco, California
Physical Medicine and Rehabilitation
Clinton Robinson, Jr.
QEII Health Sciences Centre
Grand Master
Halifax, Nova Scotia, Canada
9th Dan Taekwondo Black Belt
Shari L. McDowell, PT, BS Department of Physical Education
Inpatient Spinal Cord Injury Program Manager American River College
Shepherd Center Sacramento, California
Atlanta, Georgia
Margaret L. Roller, PT, MS, DPT
Rochelle McLaughlin, MS, OTR/L, MBSR Professor and Graduate Coordinator
Adjunct Faculty Department of Physical Therapy
San Jose State University California State University, Northridge
San Jose, California Northridge, California
Department of Occupational Therapy Clinical Instructor
Stanford Hospital Farewell to Falls NeuroCom International, a division of Natus
Stanford, California Clackamas, Oregon
Bay Area Pain and Wellness Center
Susan D. Ryerson, PT, DSc
Los Gatos, California
Owner, Making Progress
Marsha E. Melnick, PT, PhD Neurological Rehabilitation
Professor Emerita Alexandria, Virginia
San Francisco State University Research Scientist
Clinical Professor Center for Biomechanics and Rehabilitation Research
University of California, San Francisco National Rehabilitation Hospital
UCSF/SFSU Graduate Program in Physical Therapy Washington, DC
San Francisco, California
Dale Scalise-Smith, PT, PhD
Sarah A. Morrison, PT, BS Dean, School of Health Professions and Education
Director Spinal Cord Injury Services Professor of Physical Therapy
Shepherd Center, Inc. Utica College
Atlanta, Georgia Utica, New York
Susanne M. Morton, PT, PhD Osa Jackson Schulte, PT, PhD, GCFP/AT
Assistant Professor Executive Director and Continuity Assistant Trainer
Department of Physical Therapy and Rehabilitation Feldenkrais Professional Training Program
Science Movement and Healing Center
University of Iowa Clarkson, Michigan
Iowa City, Iowa Contingent Physical Therapist
Community Care Services
Mari Jo Pesavento, PT, PCS Henry Ford Health System
Pediatric Physical Therapist Detroit, Michigan
Pediatric Clinical Specialist
Rehabilitation and Development Department Claudia R. Senesac, PT, PhD, PCS
Hope Children’s Hospital Clinical Assistant Professor
Oak Lawn, Illinois Department of Physical Therapy
University of Florida
Darbi Breath Philibert, MHS, OTR/L Gainesville, Florida
Pediatric Occupational Therapist
Private Practice Eunice Yu Chiu Shen, PT, PhD, DPT, PCS
New Orleans, Louisiana Physical Therapy Education Coordinator
Department of Public Health
Robert Prue, PhD County of Los Angeles
Associate Professor California Children’s Services
School of Social Work El Monte, California
University of Missouri, Kansas City
Kansas City, Missouri
x Contributors
Timothy J. Smith, RPh, PhD Marcia Hall Thompson, PT, DPT, DSc
Professor and Chair Assistant Professor
Physiology and Pharmacology Department of Physical Therapy
Thomas J. Long School of Pharmacy and Health Sciences California State University, Fresno
University of the Pacific Fresno, California
Heidi Truman, CPO
Sebastian Sovero, MS Clinical Orthotist/Prosthetist
Doctoral Student University of California, San Francisco
Department of Electrical and Computer Engineering Department of Orthopaedic Surgery
University of California, Santa Barbara San Francisco, California
Santa Barbara, California
Karla M. Tuzzolino, PT, NCS
Kerri Sowers, PT, DPT Staff Physical Therapist
United States Equestrian Federation Paraequestrian Barrow Neurological Institute
National Classifier St. Joseph’s Hospital
Staff Physical Therapist Phoenix, Arizona
Atlanticare Regional Medical Center
Darcy A. Umphred, PT, PhD, FAPTA
Atlantic City, New Jersey
Emeritus Professor and Past Chair
Corrie J. Stayner, PT, MS Department of Physical Therapy
Adjunct Faculty School of Pharmacy and Health Sciences
Physical Therapy Program University of the Pacific
Arizona School of Health Sciences Stockton, California
A.T. Still University
John Upledger, DO
Physical Therapist
Developer
Barrow Neurological Institute
Craniosacral Therapy
Phoenix, Arizona
The Upledger Institute
James Stephens, PT, PhD, CFP Palm Beach Gardens, Florida
Living Independently for Elders, LIFE
Richard W. Voss, DPC, MSW, MTS
School of Nursing
Professor
University of Pennsylvania
West Chester University of Pennsylvania
Adjunct Assistant Professor
Department of Undergraduate Social Work
Temple University
West Chester, Pennsylvania
Physical Therapy Department
Philadelphia, Pennsylvania John G. Wallace, Jr., PT, MS, OCS
Movement Learning and Rehab Chief Executive Officer
Havertown, Pennsylvania BMS Practice Solutions
Upland, California
Bradley W. Stockert, PT, PhD
Professor Therese Marie West, PhD, MT-BC, FAMI
Department of Physical Therapy Board-Certified Music Therapist and Fellow of the
California State University, Sacramento Association for Music and Imagery
Sacramento, California Retired
Estacade, Oregon
Jane K. Sweeney, PT, PhD, PCS, C/NDT, FAPTA
Professor and Graduate Program Director Gail L. Widener, PT, PhD
Doctoral Programs in Pediatric Science Associate Professor
Rocky Mountain University of Health Professions Department of Physical Therapy
Provo, Utah Samuel Merritt University
Practitioner/Owner Oakland, California
Pediatric Rehab Northwest, LLC
Gig Harbor, Washington Patricia A. Winkler, PT, DSc, NCS
Assistant Professor (retired)
Stacey E. Szklut, MS, OTR/L Regis University
Executive Director and Owner School of Physical Therapy
South Shore Therapies Denver, Colorado
Weymouth and Pembroke, Massachusetts
George Wolfe, PT, PhD
Candy Tefertiller, DPT, ATP, NCS Professor Emeritus
Director of Physical Therapy Department of Physical Therapy
Craig Hospital California State University
Englewood, Colorado Northridge, California
Preface to the Sixth Edition
Each edition of this book brings new insights, new visions, of disease/pathology, a high level of analysis and skill devel-
and new avenues for therapists to advance their respective opment in objective measurements of functional behavior,
analytical and clinical skills when assisting individuals with and intervention strategies based on best practice and
neurological impairments to improve their quality of life. evidence. During the last three and a half decades, the thera-
The explosion of new information within neuroscience and peutic management of clients has undergone many stages of
its impact on the evidence base of both evaluation and inter- evolution. Evidence-based practice that encompasses both
vention strategies has and will continue to modify and effectiveness and efficacy through clinical studies and basic
improve services to the many individuals seeking our exper- science research should be guiding the choices of interven-
tise. With this new knowledge, many individuals within the tion procedures today. This shift in paradigm from specific
professions of physical and occupational therapy and other treatment approaches to a problem-solving model that looks
related health care disciplines will assist patients throughout at the functional ability, activity limitations, life participation,
the world to attain a level of life participation that they, as and quality of life of the client has lead to a transformation of
patients, define as quality of life. As the complex interac- services throughout the world. As these problem-solving
tions of all systems slowly unravel their mysteries in front of approaches become operational, more effective, reliable, and
the eyes and within the hands of practicing clinician and valid therapeutic examinations and management strategies are
researchers, the possibilities of new variables that affect being presented in the literature. Yet, our understanding
outcomes will continue to arise and challenge the mind of of how humans learn, relearn, or adapt is far from reaching
the learner. Having a tether to basic neuroscience allows closure. Neuroplasticity, once thought impossible, has be-
therapists of today and those of the future to stretch to limits come widely accepted as fact within the area of neurological
and levels of understanding that boggle the rigid linear rehabilitation. Given the many unknowns and the fact that
thinker of yesterday. With the explosion of new research what is “known” often changes daily, all learners are chal-
over the last five years, this sixth edition has stretched our lenged to keep a mind open to change and to new learning
professions to the unknowns we might have considered the while holding on to a flexible paradigm that allows for effec-
distant future a few years ago. These doors have led to tive examination, evaluation, and treatment of clients within a
integration of systems and help us discover what seems like dynamic, ever-changing environment. Client-centered care
unanswerable questions and continue to ground us to the has shown that willing participation by the consumer of our
evidence base of today’s practice. This book mirrors a fam- services leads to the greatest potential outcomes and satisfac-
ily dedicated to the advancement and quality of life of oth- tion of the client. No longer will therapy be done to the patient
ers. This book does not belong to the publisher, the editor, but instead will encompass and be enhanced by family’s
or even the chapter authors. We are just participants on life’s and client’s goals and expectations. Master clinicians of
journey and have come together to share what we have the past have always taken these patient goals into consider-
learned and to help future colleagues evolve farther than we ation whether formally or informally. Thus their outcomes
had at the same age. The book belongs to the learners, those always exceeded others and they never had problems with
students who are willing to question today’s practice and compliance.
look toward new and innovative ways to provide better and Cost of services, managed care environments, limitations
more effective patient care, to prevent loss of life participa- in visits, and practice patterns all create challenges to today’s
tion, and to enhance the quality of life of all individuals who professional. Young therapists are expected to graduate from
cross their paths. school and immediately practice as experienced clinical
Thirty-two years and five previous editions have passed problem solvers. Young colleagues feel they are expected to
since this book was conceived. In the evolution of a person, know the answers, not to discover them. Yet, within the
the attainment of 32 years usually signifies adulthood ap- clinical arena, problem-solving success is always dependent
proaching middle age. Thirty-two years of evolution of this on one variable, and that variable is the patient. As long as
book has encompassed new visions, greater evidence base to the unique qualities of the patient are considered, a therapist
practice within health care delivery, huge advancements in will be able to select examination procedures and appropriate
neuroscience and intervention strategies, and without a interventions using clinical reasoning. Graduates of today
doubt many more questions. Mastery can never be obtained, and tomorrow have the knowledge and skill and have prac-
because new visions constantly suggest a new beginning ticed clinical problem-solving through their education. The
while mastery suggests knowledge and wisdom of the only variables they will always need to add will be those
whole. The journey has led the reader from a book whose unique characteristics of each patient.
initial problem-solving focus was understanding medical This book is designed to provide the practitioner and
diagnosis and science as it related to neurological problems advanced therapy student with a variety of problem-solving
to a book whose focus is placed on movement diagnosis and strategies that can be used to tailor treatment approaches to
the ways to empower individuals in need of our services to individual client needs and cognitive style. The treatment of
the highest quality of life attainable through functional persons with neurological disabilities requires an integrated
movement. The evolution of the professions encompass approach involving therapies and treatment procedures used
in-depth integration of movement science, a comprehension by physical, occupational, speech and language, music and
xi
xii Preface to the Sixth Edition
recreational therapists; nurses; pharmacists; orthotists; phy- The book continues to evolve in order to meet the chang-
sicians; and a variety of other health care providers as well ing demands placed upon us as clinicians and educators. As
as the family’s expectation, values, and social beliefs. Con- we have finally assumed the role of movement specialists
tributors to this book were selected for their expertise and both in wellness and in rehabilitation, our clinical observa-
integrated knowledge of various subject areas. The result is, tional skill and the ability to compare normal to abnormal
we hope, a blend of state-of-the-art information about movement patterns when formulating a diagnosis, progno-
the therapeutic management of persons with neurological sis, and treatment plan has brought both professions to the
disabilities. obvious evolution of clinical doctorates. Our place in health
This book is organized to provide the student with a com- care and the responsibility we assume should positively
prehensive discussion of all aspects of neurological reha- impact the quality of life of all those individuals for whom
bilitation and to facilitate quick reference in a clinical situa- we provide service. We hope this book and the online video
tion. Section I, “Foundations for Clinical Practice in site will continue to aid all of you as tools to use when con-
Neurological Rehabilitation,” constitutes an overview of fronted with questions regarding movement problems seen
foundational theories. This includes the entire diagnostic in individuals with neurological problems.
process used by movement specialists. The basis for this During the conceptualization and preparation of all
process ranges across many cognitive areas and theories, six editions, many individuals gave time, guidance, and
and thus concepts and integration are presented in a variety emotional support. To all those individuals I extend my sin-
of chapters. Additional emphasis has been placed both on cere appreciation. There are many people to thank in the
health and wellness along with the visual analysis of func- preparation of this sixth edition: the authors, the researchers,
tional movement development and change across the life the illustrators, each person assisting during the process
span. Theoretical constructs of motor control, motor learn- of publication, and the patients. No person could have
ing, and neuroplasticity, as well as the limbic components accomplished the end product alone. Yet, during the editing
role in movement science and psychosocial variables are process of this edition, some specific individuals came to
again updated and discussed. The complexity of examina- deserve special recognition and thanks:
tion tools and treatment categories and techniques have The staff at Elsevier who worked on the publication of
again been edited to help the learner see and analyze the vast this edition: Christie M. Hart and Carol O’Connell. All the
opinions available as part of today’s practice. To complete teachers and healers who have crossed our paths in the last
this foundational section, discussion ends with the need 40 years and helped us to continually realize that before
for reliable and valid documentation, which should lead to we can find answers, design research projects, and establish
reimbursement for services within various clinical environ- efficacy, we must identify and acknowledge unknowns and
ments. Section II, “Rehabilitation Management of Clients formulate questions.
with Neurologic System Pathology,” offers an in-depth dis- Each family member or significant other who encouraged
cussion and analysis of the therapeutic management of and supported all of us from the moment we began the edito-
the most common neurological disabilities encountered by rial process to the day the book reached the learner, we are
physical and occupational therapists. As professions are be- forever appreciative.
coming autonomous and entry into practice at a doctoral My entire family, all of whom helped me make the time
level of study, the importance of the learner comprehending to complete this manuscript.
and analyzing each clinical problem confronted when treat- My two sons, Jeb and Ben, whose support I have had since
ing individuals with movement dysfunction caused by neu- the beginning of this book. Both are creative and brilliant
rological problems cannot be overemphasized. Hopefully young professionals in their own right, yet have tirelessly
these chapters will clarify how to examine and treat both helped me take very complex concepts and ideas and trans-
general and specific movement problems seen in individuals form them into illustrations that can be comprehended. As
with injury to specific areas of the central and peripheral small children during the book’s conception, their tolerance far
nervous system. Section III, “Neurological Disorders and exceeded their age. As children, they allowed me to take pic-
Applications Issues,” is devoted to recent advances in gen- tures, many of which have been used to actualize the chapter
eral approaches to intervention and rehabilitation that might on movement development across the life span. As young
affect any of the diagnostic categories discussed in Section adults, their support and guidance always gives me strength.
II. The importance of other system problems, such as car- As successful professionals, they have continued to teach me.
diopulmonary and chronic movement problems, has been Today, they are also husbands and parents. Their wives have
emphasized to help the learner integrate the critical nature of given to me two daughters who also help me learn and grow.
an integrated systems model. Two new chapters, one on But, of course the new life found in our grandchildren forever
robotics and one on imaging, have been added in order to allows me the opportunity to watch development of the mind,
enlarge the reader’s comprehension of tools and expecta- body, and spirit of each of them as they have begun their
tions that will become commonplace in years to come. adventure of life.
Special features within all three parts are examinations, As a critical aspect of this edition, the three section edi-
evaluations, prognosis, and intervention strategies using tors from the fifth edition stepped into the book editor role.
sound clinical reasoning. Case studies are presented within Thus, I cannot extend more gratitude, respect, and love to
each clinical-based chapter to help the reader with the Gordon Burton, Rolando Lazaro, and Margaret Roller.
problem-solving process. Online clinical movement exam- Three leaders, visionaries, and genuinely caring and loving
ples have been provided to help the learner visually recog- individuals, they have certainly made a significant impact on
nize movement problems commonly seen in individuals the direction this book has taken in this edition and will be
with specific neurological diagnoses. in the future.
Preface to the Sixth Edition xiii
Last, my husband, Gordon, who is the only one who quality of those lives is extremely important to us and those
truly knows what demands this book places on me and around us. It is hoped that this book will guide colleagues to
everyone around me. His support has never dwindled, nor help consumers in attainment of that quality. It is hoped,
his acceptance of my choices. The demands of this edition with the eyes and minds of so many outstanding colleagues
as well as life in general has certainly tried both of us as sharing their experiences and their desire to ground what
we have entered the later part of our life adventure. But, as they do into evidence-based practice, that the learner will
always, he is my best friend and present to support me embrace the adventure with the same vigor and enthusiasm
when needed. that so many have from the past.
This book was conceived 32 years ago. It was presented For each of us the journey is today and the adventure
in print to the world 27 years ago. Both dates signify young tomorrow, no matter how many tomorrows we may have.
adulthood and the evolution from conception to a responsi- May all of you have the joy, the challenge, the excitement,
bility as an adult. We are all interconnected in a tapestry that and the learning adventure I have had throughout my entire
has allowed this book to evolve into what it is today. For professional career.
that, I give thanks as an author, as the editor, as a consumer
but most importantly as a learner. Our lives are finite but the Darcy A. Umphred
Enhance Your Learning and Practice Experience
The images below are QR (Quick Response) codes. The codes How it works:
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xvii
SECTION I
Foundations for Clinical Practice

in Neurological Rehabilitation
CHAPTER 1 Foundations for Clinical Practice*

DARCY A. UMPHRED, PT, PhD, FAPTA, ROLANDO T. LAZARO, PT, PhD, DPT, GCS, and
MARGARET L. ROLLER, PT, MS, DPT
KEY TERMS OBJECTIVES

clinical problem solving After reading this chapter the student or therapist will be able to:
diagnostic model: examination, evaluation, diagnosis, 1. Analyze the interlocking concepts of a systems model and discuss how cognitive,
prognosis, intervention affective, sensory, and motor subsystems influence normal and abnormal function of
disablement and enablement models the nervous system.
empowerment 2. Use an efficacious diagnostic process that considers the whole patient/client and
holistic model for health care delivery includes evaluation, examination, diagnosis, prognosis, intervention, and related
International Classification of Functioning, Disability documentation, leading to meaningful, functional outcomes.
and Health (ICF) 3. Apply the International Classification of Functioning, Disability and Health (ICF) to the
learning environment clinical management of patients/clients with neuromuscular dysfunction.
systems model 4. Discuss the evolution of disablement, enablement, and health classification models,
neurological therapeutic approaches, and health care environments in the United States
and worldwide.
5. Discuss the interactions and importance of the patient, therapist, and environment in
the clinical triad.
6. Consider how varying aspects of the clinical therapeutic environment can affect
learning, motivation, practice, and ultimate outcomes for patients/clients.
7. Define, discuss, and give examples of a holistic model of health care.
P hysical therapists (PTs), occupational therapists (OTs),

and other health care individuals involved in improving
the function and quality of life of individuals with neuro-
roles that multiple systems within and outside the human
body play in the causation, progression, and recovery pro-
cess of a variety of common neurological problems. A sec-
muscular dysfunction must have a thorough understanding ondary objective is to orient the clinician to a theoretical
of the client as a total human being. This foundational con- framework that uses techniques for enhancing functional
cept is critical for high-level professional performance. With movement, enlarges the client’s repertoire for movement
the use of a clinical problem-solving, diagnosis-prognosis alternatives, and creates an environment that empowers the
approach, this book orients the student and clinician to the client to achieve the highest levels of activity, participation,
and quality of life.
Methods of examination, evaluation, prognosis, and inter-
*
This chapter and the concepts of this book are dedicated to Dr. Donna vention must incorporate all aspects of the client’s nervous
El-Din, PT, PhD, FAPTA, who has participated in the evolution and vision system and the influences of the external environment on
of where PT practice is today and will be over the next decade. Dr. El-Din
had hoped to participate in the writing of this chapter but was taken from
those individuals. In the clinical management of patients
us in May 2010. We will forever be grateful for her dedication, teaching, with neurological disabilities, the overlap of basic knowledge
and friendship to thousands of physical therapists across the world. and practical application of examination and intervention
1
2 SECTION I n Foundations for Clinical Practice in Neurological Rehabilitation
techniques among all disciplines involved in the care of the authors of this book use various cognitive strategies and
client is great. Delineation of individual professional roles in methods of addressing specific neurological deficits. A range
the treatment of these clients is often based on administrative of strategies for examining clinical problems is presented to
decisions and current billing practices for services provided, facilitate the reader’s ability to identify variations in prob-
rather than distinct boundaries defined by title. This book lem-solving methods. Many of the strategies used by one
emphasizes the selection of examination and intervention author may apply to situations presented by other authors.
strategies that have been demonstrated as evidence based. Just as clinicians tend to adapt learning methods to solve
Clinicians must also be open to generating new hypotheses specific problems for their clients, readers are encouraged to
as clinical problems present themselves without clear evi- use flexibility in selecting treatments with which they feel
dence to guide practice. comfortable and to be creative when implementing any
A clinical problem-solving approach is used because it is therapeutic plan.16 Although the framework of this text has
logical and adaptable, and it has been recommended by always focused on evidence-based practice and improvement
many professionals during the past 40 years.1-7 The concept of quality of life of the patient, the terminology used by pro-
of clinical decision making based in problem-solving theory fessionals has shifted from focusing on impairments and
has been stressed throughout the literature over the past de- disabilities of an individual after a neurological insult (the
cades and has guided the therapist toward an evidence-based International Classification of Impairments, Disability and
approach to patient management. This approach clearly Health [ICIDH]) to a classification system that considers
identifies the therapist’s responsibility to examine, evaluate, functioning and health at the forefront: the International
analyze, draw conclusions, and make decisions regarding Classification of Functioning, Disability and Health (ICF).28
prognosis and treatment alternatives.8-24 The ICF considers all health conditions, both pathological
This book is divided into three sections. and non–disease related; provides a framework for examin-
Section I lays the foundation of knowledge necessary to ing the status of body structures and functions for the pur-
understand and implement a problem-solving approach to pose of identifying impairments; includes activities and limi-
clinical care across the span of human life. The basic knowl- tations in the functional performance of mobility skills; and
edge of the function of the human body in disease and repair considers participation in societal and family roles that con-
is constantly expanding and often changing in content, the- tribute to quality of life of an individual. The personal char-
ory, and clinical focus. This section reflects that change in acteristics of the individual and the environmental factors to
both philosophy and scientific research. which he or she is exposed and in which he or she must func-
Roles that therapists are currently playing and will be tion are included as contextural factors that influence health,
asked to play in the future are changing.25-27 Therapists pathology, and recovery of function.29 The ICF provides a
are experts in normal human movement across the life span common language for worldwide discussion and classifica-
(see Chapter 3) and how that movement is changed after life tion of health-related patterns in human populations. The
events, and with disease or pathological conditions. Thera- language of the ICF has been adopted by the American
pists realize that health and wellness play a critical role Physical Therapy Association (APTA), and the revised ver-
in movement function as a client enters the health care sys- sion of the Guide to Physical Therapist Practice reflects this
tem with a neurological disease or condition (see Chapter 2). change.30 Each chapter in this book strives to present and use
In many U.S. states, clients are now able to use direct access the ICF model, use the language of the ICF, and present a
for therapy services. In this environment, therapists must comprehensive, patient-oriented structure for the process of
medically screen for disease and pathology to determine examination, evaluation, diagnosis, prognosis, and interven-
conditions that are outside of the defined scope of practice, and tion for common neurological conditions and resultant func-
make appropriate referrals to other medical professionals tional problems. Consideration of the patient/client as a
(see Chapter 7). They must also make a differential diagno- whole and his or her interactions with the therapist and the
sis regarding movement dysfunctions within that therapist’s learning environment is paramount to this process.
respective scope of practice (see Chapter 8). Section I has Chapters in Section II also include methods of examina-
been designed to weave together the issues of evaluation tion and evaluation for various neurological clinical problems
and intervention with components of central nervous sys- using reliable and valid outcome measures. The psychometric
tem (CNS) function to consider a holistic approach to each properties of standard outcome measures are continually be-
client’s needs (see Chapters 4, 5, 6, and 9). This section ing established through research methodology. The choice of
delineates the conceptual areas that permit the reader to objective measurement tools that focus on identifying impair-
synthesize all aspects of the problem-solving process in the ments in body structures and functions, activity-based func-
care of a client. Basic to the outcomes of care is accurate tional limitations, and factors that create restrictions in par-
documentation of the patient management process, as well ticipation and affect health quality of life and patient
as the administration and reimbursement for that process empowerment is a critical aspect of each clinical chapter’s
(see Chapter 10). diagnostic process. Change is inevitable, and the problem-
Section II is composed of chapters that deal with specific solving philosophy used by each author reflects those changes.
clinical problems, beginning with pediatric conditions, pro- Section III of the text focuses on clinical topics that can
gressing through neurological problems common in adults, be applied to any one of the clinical problems discussed in
and ending with aging with dignity and chronic impairments. Section II. Chapters have been added to reflect changes in
In Section II each author follows the same problem-solving the focus of therapy as it continues to evolve as an emerging
format to enable the reader either to focus more easily on one flexible paradigm within a multiple systems approach. A
specific neurological problem or to address the problem from specific body system such as the cardiopulmonary system
a broader perspective that includes life impact. The multiple (see Chapter 30) or complementary approaches used with
CHAPTER 1 n Foundations for Clinical Practice 3
interactive systems (see Chapter 39) are also presented as are becoming clearer, so has the role of the consumer. In
part of Section III. These incorporate not only changes in the today’s health care environment, the responsibility of both
interactions of professional disciplines within the Western the therapist and the patient begins with health and wellness
medical allopathic model of health care delivery, but also and proceeds to regaining optimal health, wellness, and
present additional delivery approaches that emphasize the functioning after neurological insult.
importance of cultural and ethnic belief systems, family Levin32 points out that there is a lot that consumers can
structure, and quality-of-life issues. Two additional chapters do for themselves. Most people can assume responsibility to
have been added to Section III. Chapter 37 on imaging em- care for minor health problems. The use of nonpharmaceuti-
phasizes the role of doctoring professions’ need to analyze cal methods (e.g., hypnosis, biofeedback, meditation, and
how medical imaging matches and mismatches movement acupuncture) to control pain is becoming common practice.
function of patients. Chapter 38 reflects changes in the role The recognition and value of a holistic approach to illness
of PTs and OTs as they integrate more complex technologies are receiving increasing attention in society. Treatment
into clinical practice. designed to improve both the emotional and physical needs
Examination tools presented throughout the text should of clients during illness has been recognized and advocated
help the reader identify many objective measures. The reader as a way to help individuals regain some control over their
is reminded that although a tool may be discussed in one lives (see Chapters 5 and 6).
chapter, its use may have application to many other clinical A holistic model (holos, from the Greek, meaning
problems. Chapter 8 summarizes the majority of neurologi- “whole”) of health care seeks to involve the patient in the
cal tools available to therapists today, and the authors of each process and take the mystery out of health care for the con-
clinical chapter may discuss specific tools used to evaluate sumer. It acknowledges that multiple factors are operating in
specific clinical problems and diagnostic groups. The same disease, trauma, and aging and that there are many interac-
concept is true with regard to general treatment suggestions tions among those factors. Social, emotional, environmental,
and problem-solving strategies used to analyze motor control political, economic, psychological, and cultural factors are
impairments as presented in Chapter 9; authors of clinical all acknowledged as influences on the individual’s potential
chapters will focus on evidence-based treatments identified to maintain health, to regain health after insult, or to maintain
for specific patient populations. a quality of health in spite of existing disease or illness. Mea-
sures of success in health care delivery have shifted from the
THE CHANGING WORLD OF HEALTH CARE traditional standard of whether the person lives or dies to the
To understand how and why disablement, enablement, and assessment of the extent of the person’s quality of life and
health classification models have become the accepted mod- ability to participate in life after some neurological insult.
els used by PTs and OTs when evaluating, diagnosing, prog- Moreover, “quality of life” or living implies more than
nosing, and treating clients with body system impairments, physical health. It implies that the individual is mentally and
activity limitations, and participation restrictions resulting emotionally healthy as well. It takes all dimensions of a
from neurological problems, it is important for the reader to person’s being into consideration regarding health. From
review the evolution of health care within our culture. This the beginning, even Hippocrates emphasized treatment of the
review begins with the allopathic medical model because this person as a whole, and the influence of society and of the
model has been the dominant model of health care in Western environment on health.
society and forms the conceptual basis for health care in An approach that takes this holistic perspective centers
industrialized countries.31 The allopathic model assumes its philosophy on the patient as an individual.33 The indi-
that illness has an organic base that can be traced to discrete vidual with this orientation is less likely to have the physi-
molecular elements. The origin of disease is found at the cian look only for the chemical basis of his or her difficulty
molecular level of the individual’s tissue. The first step to- and ignore the psychological factors that may be present.
ward alleviating the disease is to identify the pathogen that Similarly, the importance of focusing on an individual’s
has invaded the tissue and, after proper identification, apply strengths while helping to eliminate body system impair-
appropriate treatment techniques including surgery, drugs ments and functional limitations in spite of existing disease
(see Chapter 36), and other proven methods. or pathological conditions plays a critical role in this model.
It is implicit in the model that specialists who are profes- This influences the roles PT and OT will play in the future
sionally competent have the sole responsibility for the iden- of health care delivery and will continue to inspire expanded
tification of the cause of the illness and for the judgment as practice in these professions.
to what constitutes appropriate treatment. The medical The health care delivery system in Western society is
knowledge required for these judgments is thought to be the designed to serve all of its citizens. Given the variety of
domain of the professional medical specialists and therefore economic, political, cultural, and religious forces at work
inaccessible to the public. PTs and OTs have never been in American society, education of the people with regard to
responsible for the diagnosis or treatment of diseases or their health care is probably the only method that can work
pathological conditions of a specific client. Instead, we have in the long run. With limitations placed on delivery of
always focused on the body system impairments resulting in medical care, the client’s responsibility for health and heal-
activity limitations and inability to participate in life that ing is constantly increasing. The task of PTs and OTs to-
have been caused by the specific disease or pathological day is to cultivate people’s sense of responsibility toward
condition. Therapists are also responsible for analyzing the their own health and the health and well-being of the com-
interactions of all other systems and how they compensate munity. The consumer has to accept and play a critical role
for or are affected by the original medical problem. As our in the decision-making process within the entire health care
roles within Western health care delivery have expanded and delivery model to more thoroughly guarantee compliance
with prescribed treatments and optimal outcomes.34-40 PTs the end result should lead to a higher quality of life for the
and many OTs today are entering their professional careers consumer.
at a doctoral level and beginning to assume the role of Providers are more willing to include the client by
primary care providers. A requisite of this new responsibil- designing individualized plans of care, educating, address-
ity is the performance of a more diligent examination and ing issues of minority groups, and becoming proactive
evaluation process that includes a comprehensive medical team caregivers.37-40 The influence of these methods ex-
screening of each patient/client.41,42 Patient education will tends to the community and leads to greater patient/client
continue to be an effective and vital approach to client satisfaction. The research as of 2011 demonstrates the im-
management and has the greatest potential to move health portance of patient participation, and this body of work is
care delivery toward a concept of preventive care. The high expected to grow.
cost of health care is a factor that will continue to drive The potential for OTs and PTs to become primary provid-
patients and their families to increase their participation ers of health care in the twenty-first century is becoming a
in and take responsibility for their own care.43 Reducing greater reality within the military system as well as in some
the cost of health care will require providers to empower large health maintenance organizations (HMOs).48-53 The
patients to become active participants in preventing and role a therapist in the future will play as that primary pro-
reducing impairments and practicing methods to regain vider will depend on that clinician’s ability to screen for
safe, functional, pain-free control of movement patterns disease and pathological conditions, examine and evaluate
for optimal quality of life. clinical signs that will lead to diagnoses and prognoses that
fall inside and outside of the scope of practice, and select
In-Depth Analysis of the Holistic Model appropriate interventions that will lead to the most effica-
Carlson44 thinks that pressure to change to holistic thinking cious, cost-effective treatment.
in medicine continues as a result of a societal change in its The role of therapists in the area of neurological reha-
perspective of the rights of individuals. A concern to keep bilitation will first be in the area of health and wellness.
the individual central in the care process will continue to Medical screening and early detection of neurological prob-
grow in response to continued technological growth that lems should facilitate early referral of the consumer to a
threatens to dehumanize care even more. The holistic model medical practitioner. This may occur in a wellness center or
takes into account each person’s unique psychosocial, politi- in physical and occupational therapy clinics where the pa-
cal, economic, environmental, and spiritual needs as they tient is being seen for some other problem such as back pain.
affect the individual’s health. Similarly, patients may reenter physical or occupational
The nation faces significant social change in the area of therapy after a neurological insult as someone who has a
health care. The coming years will change access to health chronic movement dysfunction or degenerative condition
care for our citizens, the benefits, the reimbursement pro- that may be getting worse and who needs some instruction
cess for providers, and the delivery system. Health care to regain motor function.
providers have a major role in the success of the final prod- Neurological rehabilitation is taking place and will
uct. The Pew Health Professions Commission45 identified continue to take place in a changing health care environ-
issues that must be addressed as any new system is devel- ment and ever-evolving delivery system. The balance be-
oped and implemented. Most, if not all, of the issues involve tween visionary and pragmatist must be maintained by the
close interactions between the provider and client. These practitioner. By the end of the twenty-first century, neuro-
issues include (1) the need of the provider to stay in step logical rehabilitation will have evolved into a new shape
with client needs; (2) the need for flexible educational and form, will take place within a very different health
structures to address a system that reassigns certain respon- system, and will involve the client as the center of the
sibilities to other personnel; (3) the need to redirect national dynamic exchange among wellness, disease, function, and
funding priorities away from narrow, pure research access empowerment.
to include broader concepts of health care; (4) the licens-
ing of health care providers; (5) the need to address the THERAPEUTIC MODEL OF NEUROLOGICAL
issues of minority groups; (6) the need to emphasize gen- REHABILITATION WITHIN THE HEALTH
eral care and at the same time educate specialists; (7) the CARE SYSTEM
issue of promoting teamwork; and (8) the need to empha-
size the community as the focus of health care. There are Traditional Therapeutic Models
other important issues, but the last to be included here is Keen observation of human movement and how impairments
mentioned in more detail because of its relevance to the in the neuromusculoskeletal system alter motor behavior and
consumer. Without the consumer’s understanding during functional mobility led several remarkable therapists to
development of a new system, the system could omit sev- develop unique models of therapeutic interventions. These
eral opportunities for enrichment of design. Without the models include those of Ayers (sensory integration), Bobath
understanding of the consumer during implementation of a (NeuroDevelopmental Treatment [NDT]), Brunnstrom (move-
new system, the consumer might block delivery systems ment therapy approach), Feldenkrais (Functional Integration
because of lack of knowledge. Thus, the delivery of service and Awareness Through Movement), Klein-Vogelbach (Func-
must be client centered and client and family driven, and tional Kinetics), Knott and Voss (Proprioceptive Neuromus-
the focus of intervention needs to be in alignment with cular Facilitation [PNF]), and Rood (Rood approach to
client objectives and desired outcomes.33-36,46,47 Today, as neuromuscular dysfunction). These were the first behavior-
stated earlier,43 this need may be driven more by financial ally based models introduced within the health care delivery
necessity than by ethical and best practice philosophy, but system, and they have been used by practitioners within the
professions of physical and occupational therapy since the were often demonstrated by the client, and thus the success
middle of the twentieth century. These individuals, as master of the treatments and the skill of these master clinicians
clinicians, tried to explain what they were doing and why cannot be denied.
their respective approaches worked using the science of the
time. From their teachings, various philosophical models Physical and Occupational Therapy Practice
evolved. These models were isolated models of therapeutic Models
intervention that were based on successful treatment proce- Disablement models have been used by clinicians since the
dures as identified through observation and described and 1960s. These models are the foundation for clinical out-
demonstrated by the teachers of those approaches. The gen- comes assessment and create a common language for health
eral model of health care under which these approaches care professionals worldwide. The first disablement model
were used was the allopathic model of Western medicine, was presented in 1965 by Saad Nagi, a sociologist.54,55 The
which begins with disease and pathology. Today, our models Nagi model was accepted by APTA and applied in the first
must begin with health and wellness, with an understanding Guide to Physical Therapist Practice, which was introduced
of variables that lead a client into the health care delivery in 2001.30 In 1980 the ICIDH was published by the World
system, and an understanding of how the nervous system Health Organization (WHO).56 This model helped expand
works and repairs itself. on the International Classification of Diseases (ICD), which
During the past decades, both short-term and full- has a narrow focus based on categorizing diseases. The
semester courses, as well as literature related to treatment ICIDH was developed to help measure the consequences of
of clients with CNS dysfunction, have been divided into health conditions on the individual. The focus of both the
units labeled according to these techniques. Often, inter- Nagi and the ICIDH models was on disablement related to
relation and integration among techniques were not explored. impaired body structures and functions, functional activi-
Clinicians bound to one specific treatment approach with- ties, and handicaps in society (Figure 1-1). The WHO ICF
out considering the theoretical understanding of its step- model28 evolved from a linear disablement model (Nagi,
by-step process may have lacked the basis for a change ICIDH) to a nonlinear, progressive model (ICF) that en-
of direction of intervention when a treatment was ineffec- compasses more than disease, impairments, and disable-
tive. It was difficult, therefore, to adapt alternative treatment. It includes personal and environmental factors that
ment techniques to meet the individual needs of clients. contribute to the health condition and well-being of indi-
As a result, clinical problem solving was impeded, if not viduals. The ICF model is considered an enablement model
stopped, when one approach failed, because little integra- as it not only considers dysfunctions, but helps practitioners
tion of theories and methods of other approaches was and researchers understand and use an individual’s strengths
never stressed in the learning process. Similarly, because in the clinical presentation. Each of these models provides
a specific treatment has a potential effect on multiple body an international standard to measure health and disability,
systems and interactions with the unique characteristics of with the ICF emphasizing the social aspects of disability.
each client’s clinical problem, establishing efficacy for The ICF recognizes disability not only as a medical or bio-
interventions using a Western research reductionist model logical dysfunction, but as a result of multiple overlapping
became extremely difficult. This does not negate the po- factors including the impact of the environment on the
tential usefulness of any treatment intervention, but it functioning of individuals and populations. The ICF model
does create a dilemma regarding efficacy of practice. is presented in Table 1-1 and discussed in greater depth in
Similarly, the rationale often used to explain these thera- Chapters 4, 5, and 8.
peutic models was based on an understanding of the ner- It is easy to integrate the ICF model into behavioral
vous system as described in the 1940s, 1950s, and 1960s. models for the examination, evaluation, diagnosis, progno-
That understanding has dramatically changed. With the sis, and intervention of individuals with neurological sys-
basic neurophysiological rationale for explaining these tem pathologies (see Figure 1-1). Whether an individual’s
approaches under fire for validity and the inability to dem- activity limitations, impairments, and strengths lead to a
onstrate efficacy of these approaches using traditional restriction in the ability to participate in life activities, the
research methods, many of these treatment approaches are perception of poor health, or restriction in the ability to
no longer introduced to the student during academic train- adapt and adjust to the new health condition will determine
ing. However, if these master clinicians were much more the eventual quality of life of the person and the amount of
effective than their clinical counterparts, then the hands- empowerment or control he or she will have over daily life.
on therapeutic nature of their interventions may still be The importance of the unique qualities of each person and
valid in certain clinical situations, but the neurophysiolog- the influence of the inherent environment helped to drive
ical explanation for the intervention may be very different. changes to world health models. The ICF is widely ac-
To make statements today saying that these masters did cepted and used by therapists throughout the world and
not use theories of motor learning or motor control is ob- is now the model for health in professional organizations
vious because those theories and the studies supporting such as APTA in the United States.30
them had not yet been formulated. Yet patients treated by As world health care continues to evolve, so will the
these master clinicians demonstrated improvements for WHO models. The sequential evolution of the three models
which, it would seem according to our present-day theo- is illustrated in Table 1-1. This evolution has created an
ries, that concepts of motor learning must have been rein- alignment with what many therapists and master clinicians
forced and repetitive practice encouraged. Although the have long believed and practiced—focus on the patient, not
verbal understanding of behavior sequences used to pro- the disease. The shift from disablement to enablement mod-
mote motor learning did not exist, these behavior sequences els of health care is a reflection of this change in perspective.
Direct access
Body structures Quality of life and

Pathology and Activity/ Participation/
and functions/ empowerment
Perceived environment Activity Participation
Impairment Or
wellness Internal and limitations restrictions
(systems disempowerment
external factors
interaction)
Phase 1 Risk factors

Risk factors
Diagnosis 1. Inherent risk factors
Psychosocial or CNS 2. Environmental and
factors lesion psychosocial parameters
Nutritional Environmental
constraint
Environmental
Pain
factors
Other
Risk factors State of the
Motor pool
Peripheral
Cardiovascular/ properties
pulmonary (ROM, strength,
Volitional/ non- elasticity, etc.)
volitional
patterns of
muscle activity
Risk factors
Figure 1-1 n Behavioral model for evaluation and treatment based on the International Classification
of Functioning, Disability and Health (ICF) enablement schema. ROM, Range of motion.
n ENABLEMENT AND DISABLEMENT MODELS WIDELY ACCEPTED THROUGHOUT THE

TABLE 1-1
WORLD OVER THE LAST 50 YEARS*
ICF model, Health condition Body function and Activity Participation

WHO 200130 structure (impairments) (limitations) (restrictions)
ICIDH model, Disease or pathology Organ systems Disabilities Handicaps
WHO 198056 (impairments)
Nagi model, Disease or pathology Organ systems Functional Disabilities
196555 (impairments) limitations
ICF, International Classification of Functioning, Disability and Health; ICIDH, International Classification of Impairments, Disabilities, and Handicaps; WHO,
World Health Organization.
*Placed in table form to show similarities in concepts across the models. Please note that the ICF is a nonlinear, progressive model of enablement and includes
contextual factors (personal and environmental) that contribute to the well-being of the individual. Also refer to Chapter 8 for a more detailed discussion of
the ICF model.
Conceptual Frameworks for Client/Provider Statistical Model

Interactions The statistical model framework considers some predeter-
Three conceptual frameworks for client-provider interactions mined set of mathematical values as the main driver for
are commonly used in the current health care delivery sys- patient/client management decisions. For example, in today’s
tem. Each framework serves a different purpose and is used health care arena there is often a disconnect between the
according to the goals of the desired outcome and the group extent of a patient’s clinical problems and the number of
interpreting the results (Figure 1-2). The four primary con- approved, predetermined treatment visits needed to remedi-
ceptual frameworks include (1) the statistical model, (2) the ate these problems. In this situation the clinician must make
medical diagnostic model, (3) the behavioral or enablement certain clinical decisions before deciding service and must
model, and (4) the philosophical or belief model. determine how best to meet the needs of the patient given
Philosophical/Belief Medical/Diagnostic
Behavioral/Enablement
Philosophical/Belief Behavioral/Enablement
Medical/Diagnostic
Figure 1-2 n Types of clinical models. A, Isolated paradigms. B, Complex interactive paradigms.
C, Systems approach or paradigm. D, Systems interaction on traditional paradigms.
this limitation. Another illustration of this model involves emission tomography (PET scan), evoked potentials, and
the use of “numbers” or “grades” obtained from some out- laboratory studies (see Chapter 37). When abnormal test
come measure to make a determination on either the extent results are correlated with gross clinical signs and patient
of functional limitation or the efficacy of a particular inter- history such as high blood pressure, diabetes, or head
vention. For example, if a patient scores 14 out of 24 points trauma, a medical diagnosis is made along with an antici-
one week and 17 out of 24 points the next, and the payer pated course of recovery or disease progression. This medi-
knows that a score of 19 means the individual’s risk of fall- cal diagnostic model is based on an anatomical and physio-
ing is reduced, then the payer often permits additional ther- logical belief of how the brain functions and may or may not
apy visits. Those payers generally have little interest in the correlate with the behavioral and enablement models used
reasons why the client moved from a score of 14 to 17, only by therapists.
that the person is improving. If clinicians do not provide
these types of quantitative measurements, payment for ser- Behavioral or Enablement Model
vices often is denied. To be able to optimize care under this The behavioral or enablement model evaluates motor perfor-
model, today’s therapists need to be flexible critical thinkers mance on the basis of two types of measurement scales. One
who are able to skillfully document and communicate prog- type of scale measures functional activities, which range from
ress to individuals who need numbers, as well to provide this simple movement patterns such as rolling to complex patterns
information to patients and their families, who are in emo- such as dressing, playing tennis, or using a word processor.
tional crisis because of problems associated with the neuro- These tools identify functional activities or aspects of life
logical dysfunction, in a manner that they can understand. performance that the person has been or is able to do and
Because efficacy of any intervention may be questioned serve as the “strengths” when remediating from activity limi-
by anyone, including the client, family, physician, third- tations or participation restrictions. The second scale looks at
party payer, or a lawyer, outcome tools that clearly measure bodily systems and subsystems and whether they are affecting
problems in all domains must be carefully selected. Before functional movement. These measurement tools must look at
an evaluation tool is chosen, the specific purpose for the specific components of various systems and measure impair-
request for examination and the model by which to interpret ments within those respective areas or bodily systems. For
the meaning of the data must be identified. example, if the system to be assessed is biomechanical, a
simple tool such as a goniometer that measures joint range of
Medical Diagnostic Model motion might be used, whereas a complex motion analysis
Physicians are educated to use a medical disease or patho- tool might be used to look at interactions of all joints during
logical condition diagnostic model for setting expectations a specific movement. These types of measurements specifi-
of improvement or lack thereof. In patients with neurologically look at movement and can be analyzed from both an
cal dysfunction, physicians generally formulate their medi- impairment and an ability perspective. Chapter 8 has been
cal diagnosis on the basis of results from complex, highly designed to help the reader clearly differentiate these two
technical examinations such as magnetic resonance imaging types of measurement tools and how they might be used in the
(MRI), functional magnetic resonance imaging (fMRI), diagnosis, prognosis, and selection of intervention strategies
computerized axial tomography (CAT or CT scan), positron when analyzing movement.
Philosophical or Belief Model and incorporated into patient care by rehabilitation profes-
A fourth model or framework for client-provider interaction sionals.37-40 This integration and acceptance will guide
that may still be found in clinical use is a philosophical or health care practice well into the next decade.
belief model such as those described by master clinicians The need for students to develop problem-solving strate-
from the past, including Rood, Knott, Bobath, and Ayers, or gies is accepted by faculty across the country and by the
homeopathic models such as acupuncture or Chinese medi- respective accrediting agencies of health professionals. Un-
cine. These philosophical models, when applied to func- fortunately, we may not be educating students to the level of
tional outcomes, would be included with today’s behavioral critical thinking that we hope.59,60 The need for this cogni-
models and encompass a systems approach. The gap be- tive skill development in clinicians may be emergent as both
tween philosophy and practice is narrowing as evidence is physical and occupational therapy professions have moved
slowly showing that many of these approaches positively or plan to move to a doctoring professions.49 All health-
affect patient outcomes. Research has also identified ap- related professions must evolve as patient care demands
proaches that have no efficacy. The link is outcome mea- increase, delineation of professional boundaries become
sures and whether a patient changes in participating in and less clear, and collaboration becomes a more integral factor
has a quality of life. Thus the change is seen in the patient. in providing high-quality health care.
Research today has created an alignment with what many All previously presented models (statistical, medical,
therapists and master clinicians have long believed and prac- behavioral, or belief) can stand alone as acceptable models
ticed—focus on the patient, not the disease. for health care delivery (see Figure 1-2, A) or can interact or
Therapists appreciate a statistical model through research interconnect (Figure 1-2, B). These interconnections should
and acceptance of evidence-based practice. A third-party validate the accuracy of the data derived from each model.
payer also uses numbers to justify payment for services or to The concept of an integrated problem-solving model for
set limits on what will be paid and for specific number of neurological rehabilitation must also identify the functional
visits that will be covered. Therapists also appreciate physi- components within the CNS (Figure 1-2, C).
cians’ knowledge and perspective of disease and pathology A model that identifies the three general neurological
because of the effect of disease and pathology on functional systems (cognitive, emotional, motor) found within the
behavior and the ability to engage and participate in life. On human nervous system can be incorporated into each of the
the other hand, third-party payers and physicians may not be other models separately or when they are interconnected
aware of the models used by OTs and PTs. It is therefore (Figure 1-2, D). A systems or behavioral model that focuses
critical that therapists make the bridge to physicians and on the neurological systems is much more than just the mo-
third-party payers because research has shown that interdis- tor systems and their components, or cognition with its
ciplinary interactions help reduce conflict between profes- multiple cortical facets, or the affective or emotion limbic
sionals and provide better consistency for the patient.57,58 system with all its aspects. The complexity of a neurological
It is a medical shift in practice to recognize that patient systems model (Figure 1-3), whether used for statistics, for
participation plays a critical role in the delivery of health medical diagnosis, for behavioral or functional diagnosis, or
care. The importance of the patient and what each individual for documentation or billing, cannot be oversimplified. As
brings to the therapeutic environment has been recognized the knowledge bank regarding central and peripheral system
Figure 1-3 n Systems model. Dynamic interactive subcomponents: whole to part to whole.
F2ARV, Fear and frustration, anger, rage, violence; GAS, general adaptation syndrome; L.T., long term;
S.T., short term.
function increases, as well as knowledge about their interac- methods have been developed to show evidence of efficacy,
tions with other functions within and outside the body, the or they may be discarded for the same reason. Until these
complexity of a systems model also enlarges.61 The reader approaches have gone beyond belief in their effects, therapists
must remember that each component within the nervous will always need to expend additional focus measuring quan-
system has many interlocking subcomponents and that each titative outcomes and analyzing accurately functional re-
of those components may or may not affect movement. sponses. Because the research is not available does not mean
Therapists use these movement problems as guidelines to the approach has no efficacy (see Chapter 39). Thus the clini-
establish problem lists and intervention sequences. These cian needs to learn to be totally honest with outcomes, and
components, considered impairments or reasons why some- quality of care and quality of life remain the primary objective
one has difficulty moving, are critical to therapists but are for patient management. Today, models that incorporate
of little concern within a general statistics model and may health and wellness have been added to these disablement
have little bearing on the medical diagnosis made by the and enablement models to delineate the complexity of the
physician. problem-solving process used by therapists62,63 (see Chapter 2).
In addition to the Western health care delivery paradigms This delineation should reflect accurate behavioral diagnoses
are the interlocking roles identified within an evolving trans- based on functional limitations and strengths, preexisting
disciplinary model (Figure 1-4). Within this model, the envi- system strengths and accommodations, and environmental-
ronments experienced by the client both within the Western social-ethnic variables unique to the client. Similarly, it in-
health care delivery system and those environments external cludes the family, caregiver, financial security, or health care
to that system are interlocking and forming additional system delivery support systems. All these variables guide the direc-
components; they influence one another and affect the ulti- tion of intervention64 (Figure 1-5). These variables will affect
mate outcome demonstrated by the client. Because all these behavioral outcomes and need to be identified through the
once-separate worlds encroach on or overlay one another and examination and evaluation process. Many of these variables
ultimately affect the client, practitioners are now operating in may not relate to the CNS disease or pathological condition
a holistic environment and must become open to alternative medical diagnosis to which the patient has been assigned.
ways of practice. Some of those alternatives will fit neatly and The client brings to this environment life experiences.
comfortably with Western medical philosophy and be seen as Many of these life events may have just been a life experi-
complementary. Evidence-based practice, which used linear ence; others may have caused slight adjustments to behavior
research to establish its reliability and validity, has provided (e.g., running into a tree while skiing out of patrolled down-
therapists with many effective tools both for assessment and hill ski areas and then never doing it again), some may have
treatment, but we still are unable to do similar analyses while caused limitations (e.g., after running into the tree, the left
simultaneously measuring multiple subsystem components. knee needed a brace to support the instability of that knee
We can measure tools and interventions across multiple sites during any strenuous exercising), or caused adjustments in
but are a long way from truly understanding the future of best motor behavior and emotional safety before that individual
practice. Other evaluation and treatment tools may sharply entered the heath care delivery system after CNS problems
contrast with Western research practice, having too many occurred. The accommodations or adjustments can dramati-
variables or variables that cannot be measured; therefore ar- cally affect both positively and negatively the course of in-
riving at evidence-based conclusions seems an insurmount- tervention. To quickly accumulate this type of information
able problem. In time many of these other assessment tools regarding a client, the therapist must become open to the
and intervention strategies may be accepted, once research needs of the client and family. This openness is not just
EVOLVING TRANSDISCIPLINARY
Look at PT/OT/others
ENVIRONMENT
other than health care
Subcultural Religion
characteristics
ENVIRONMENT
Life within health care delivery Learning styles
experiences
Patient
Other
PT OT
Family Nursing
Figure 1-4 n Transdisciplinary model for delivery paradigms.

Quality of Life
A.I.M.
An Integrated Model
Pre-Disease Disease Activities Participation in Life

Health/Wellness Pathology Functional Resources Enablement
Ev L EvL-A LI-A L-M Impairment Activity Disabilities/

Limitations participation
LIFE
Hypothesis Formation
Driven by suspected impairments
Examination Eval/Diagnosis Prognosis Intervention

Tests/Measures Identified/Prioritized Strategies
Confirm or Refute System and Sub-system Client PT Techniques
Hypothesis Impairments
External Factors:
Funding
Culture
Support System
Di e
sc
ov enc
ery vid
E
Figure 1-5 n Clinical problem-solving process incorporating life events, pathological condition,
and postdisease state into a functional diagnosis. Ev, Event, disease; I, identifiable impairment; L,
life; L-A, life with adaptation; L-M, life with modifications.
sensory, using eyes and ears, but holistic and includes a or its process, but they are in a position of responsibility to
bond that needs to and should develop during therapy (see examine body systems for existing impairments and to
Chapters 5 and 6). analyze normal movement to determine appropriate inter-
ventions for activity-based functional problems. Some dif-
EFFICACY ferences in this responsibility may exist between practice
Efficacy has been defined as the “ability of an intervention settings. Therapists in private practice act independently to
to produce the desired beneficial effect in expert hands and select both examination tools and intervention approaches
under ideal circumstances.”65 When any model of health that are efficacious and prove beneficial to the patient.
care delivery is considered, the question the therapist must Within a hospital-based system, therapists may be ex-
ask is “Which model will provide the most efficacious pected to use specific tools that are considered a standard
care?” Therapists may not diagnose a pathological disease of care for that facility, regardless of the therapy diagnosis
and treatment rendered. In some hospitals and rehabilita- of intervention you are teaching?” The answer was, “Yes, all
tion settings a clinical pathway may be employed that de- I need are two dynamic PET units that can be worn on both
fines the roles and responsibilities of each person on a the client’s and the therapist’s heads while performing thera-
multidisciplinary team of medical professionals. Regard- peutic interventions. I also need a computer that will simul-
less of which clinical setting or role the therapist plays, it taneously correlate all synaptic interactions between the
is always the responsibility of the therapist to be sure that therapist and the client to prove the therapeutic effect.” The
the plan of care is appropriate, is consistent with the medi- physician said, “We don’t have those tools!” The response
cal and therapy diagnoses, meets the needs of the patient, was, “You did not ask me if the research tools were avail-
and renders successful outcomes. If the needs of the par- able, only if I know how to obtain an efficacious result.”
ticular client do not match the progression of the pathway, Thus, the creativity of the therapist will always bring the
it is the therapist’s responsibility to recommend a change professions to new visions of reality. That reality, when
in the client’s plan of care. Efficacy does not come because proven to be efficacious, assists in validating the accepted
one is taught that an examination tool or intervention pro- interventions used by the professional. The therapist today
cedure is efficacious, it comes from the judicious use of has a responsibility to provide evidence-based practice to
tools to establish impairments, activity limitations, and the scientific community…but more important, also to the
participation restrictions, identify movement diagnoses, client. Therapeutic discovery usually precedes validation
create functional improvements, and improve quality of through scientific research. This discovery leads the way to,
life in those individuals who have come to us for therapy. first, effective interventions, followed by efficacious care. If
Today’s health care climate demands that the therapeutic research and efficacious care always have to come before
care model be efficient, be cost-effective, and result in mea- the application of therapeutic procedures, nothing new will
surable outcomes.66 The message being given today might evolve because discovery of care is most often, if not al-
be considered to reflect the idea that “the end justifies the ways, found in the clinic during interaction with a client.
means.” This premise has come to fruition through the linear Thus the range of therapeutic applications will become se-
thought process of established scientific research. Yet when verely limited and the evolution of neurological care stopped
a holistic model is accepted into practice it becomes appar- if that discovery is ignored because there is no efficacy as
ent that outcome tools are not yet available to simultane- defined by today’s research models. However, performing
ously measure the interactions of all body systems that make interventions because the approaches “have been typically
up the patient, making it difficult to apply models that pur- done in the past” could be wasteful and irresponsible.
port to balance quality and cost of care. Thus we must guard
against the reductionist research of today, which has the DIAGNOSIS: A PROCESS USED BY ALL
potential to restrain our evolution and choice of therapeutic PROFESSIONALS WHEN DRAWING
interventions. Sometimes the individuals making decisions CONCLUSIONS
about what to include and what to eliminate with regard to Diagnosis is a conclusion drawn regarding specific diseases
patient services are not health care providers. They are indi- and pathological processes within the human body; when
viduals who are trained to use evidence gained from num- made by a physician it is considered a medical diagnosis.
bers or statistics to make their decision and do not have Diagnosis made by a PT or an OT is a conclusion drawn
knowledge of the patient, his or her situation, or the effect of regarding the status of body systems, activity and participa-
the neurological condition on function. Therapists should tion, and their interactions considering the patient’s personal
always be able to defend their choice and use of intervention factors and the environment. Specific activity-based func-
approaches. This becomes even more relevant as the cost of tional limitations and the impairments within the body sys-
health care rises. tems that affect the client’s ability to control quiet postures
Evidence-based practice is basic to the care process.30,67,68 or dynamic movement in any activity become a focus in the
Clinicians need to identify which of their therapeutic inter- diagnostic process. The functional loss itself may or may not
ventions have demonstrated positive outcomes for particular reflect specific diseases or pathological conditions within
clinical problems or patient populations and which have the CNS but does reflect specific impairments within that
not.69 Those that remain in question may still be judged as client’s body. PTs and OTs, by use of functional behavioral
useful. The basis for that judgment may be a client satisfac- models, are becoming comfortable with the diagnosis of
tion variable that has become a critical variable for many body system problems (impairments), activity restrictions
areas in health care delivery.70,71 But there is still limited (functional limitations), and participation and the concep-
information on patient satisfaction with PT and OT services, tual understanding that the diagnosis made by a PT or an OT
although within the last few years more information has is very different from that made by a physician.
become available.72-76 Although patient satisfaction is a Once the interpretation has been made, a therapist must
critical variable within the ICF model, there are always draw conclusions regarding those results and their interac-
problems with satisfaction and outcomes versus identifica- tions. That interpretation leads to a therapy diagnosis. The
tion of specific measurable variables within the CNS that are interpretation of the evaluation results and their interaction
affecting outcomes. One reason for the problem of integrat- with therapist’s and client’s desired outcomes, available re-
ing patient satisfaction with PT and OT services within a sources, and client’s potential lead to the prognosis. Selection
neurorehabilitation environment is the large discrepancy of the best and most efficient resources to achieve the desired
between the variables we can measure and the variables outcome will lead to establishment of the treatment interven-
within the environment that are affecting performance. For tion plan or “road map.”
example, when a neurosurgeon once asked the question to The diagnostic process used by therapists is complex
one of the editors, “Do you know how to prove the theories and is clearly divided into two specific phases of differential
diagnosis (Figure 1-6). This is further explained in Chapters differential diagnosis. This form of system screening is part
7 and 8. of history taking and may be redone periodically throughout
treatment if the therapist has questions regarding changes in
Phase 1: Differential Diagnosis: System body systems. In the Guide to Physical Therapist Practice30
Screening for Possible Disease or Pathology this step is called systems review and review of systems.
With the increasing use of direct access and the length of There may be times when the therapist has received a refer-
time therapists spend with clients, clinicians have become ral for a chronic problem and during the medical screen the
acutely aware of the need to screen systems for signs of patient demonstrates signs of a potential medical condition
disease and pathological conditions.51 Accreditation stan- that has nothing to do with the referral. In that situation the
dards for both PTs and OTs require the new learner to de- therapist may continue with treatment but also should refer
velop these skills before graduation. This screening process the patient back to a physician for a more thorough evalua-
is used to determine whether the client should be referred to tion of the new problem.
another practitioner, such as a physician, or can progress to For example, a patient was referred to a PT for treatment
diagnosis, prognosis, and intervention within the specific of chronic back pain caused by degenerative disc disease.
discipline. Thus Phase 1 of this differential diagnosis sepa- During performance of a system screening the therapist de-
rates a client’s clinical problems into those that fall within a termined that the patient had generalized weakness on the
therapist’s scope of practice and those that do not. If the left side. On a return visit 2 days later the patient continued
Phase 1 differential diagnosis shows signs and symptoms to demonstrate mild weakness on the left side. The therapist
totally outside a therapist’s scope of practice, then a referral referred the patient back to the doctor, and the subsequent
to an appropriate practitioner must be made. If the signs and MRI showed that the woman had a large tumor in the right
symptoms both fall within the clinician’s scope of practice lower frontal-temporal lobe area. Over subsequent treat-
and overlap with that of other disciplines, the therapist must ments the therapist was able to eliminate most of the chronic
refer and decide (1) to treat to prevent problems until the pain in her back. The patient to this day feels the therapist
other practitioner’s treatment can be performed, (2) to man- saved her life.77
age the limitations in activity and participation in spite of the Once a clinician determines that the client’s need for
pathological process, or (3) to manage functional loss and service falls within his or her respective scope of practice,
impairments and therefore correct the pathological cause. In then Phase 2 differential diagnosis begins.
some cases the overlapping with other disciplines may not
necessitate an immediate referral, but interactions must be Phase 2: Differential Diagnosis within a
made when needed to ensure the best outcome from inter- Therapist’s Scope of Practice
vention. However, when the information obtained by the Once the client’s signs and symptoms have been determined
therapist from this phase of differential diagnosis indicates a to fall clearly within the scope of PT and OT practice, a de-
possible immediate and life-threatening condition, the thera- finitive therapy diagnosis, prognosis, and plan of care can be
pist must act accordingly by calling 911 and referring the established. The use of an enablement model such as the ICF
patient to a medical physician. Chapter 7 has been designed will help the therapist best capture the patient’s strengths,
to help the reader grasp a better understanding of Phase 1 of impairments, activity limitations, and participation restric-
tions, which can then be used to determine the patients goals,
address the individual’s needs, and optimize function and
quality of life (see Figure 1-1). The client’s functional goals
Patient self refers or is sent by a referral source and expectations may include activities of daily living, job
skills, recreation and leisure activities, or the skills required
for performance of typical societal roles. Each of these goals
must have a realistic, objective, measurable outcome that is
Phase One: Medical screening for disease and pathology based on the results of carefully chosen examination tools. An
• System level screening only in-depth conceptual framework for selection of appropriate
• For referral to practitioner who diagnoses disease
and pathology if red flags present
examination procedures needed to evaluate and draw appro-
• Determine whether to refer only or to refer and priate diagnostic conclusions can be found in Chapter 8.
intervene Two important clinical components affect the accuracy of
• (Chapter 2) the diagnostic conclusion. First, the clinician must establish
accurate, nonbiased results. This fact seems obvious, but
with the pressures of third-party payers, family members,
other care providers, and the desire to have the client im-
prove, it is easy to submit to drawing a conclusion based on
Phase Two: Client remains within scope of practice and
desired outcomes rather than facing what is truly present
proceeds with diagnostic process
• Examinations and evaluations to determine
and realistic. The second factor deals with the honesty of the
impairments and disabilities. Foundation for interaction between the therapist and the client. This “bond-
establishment of a diagnosis and prognosis ing” is critical for obtaining accurate examination results.
(Chapter 3) and Safety, trust, and acceptance of the client as a human being
• Selection of available interventions to determine play key roles in therapeutic outcomes and thus in efficacy
best practice (Chapter 4)
of practice.78-81 The reader is referred to Chapters 5 and 6 to
Figure 1-6 n The diagnostic process used for best practice by develop a greater understanding of the impact this bonding
physical and occupational therapists. has on clinical outcomes.
The specific cognitive process used by therapists before (point B). The outcomes will state whether the intervention
formulation of a therapy diagnosis might be conceptualized will (1) eliminate functional limitations through changes,
as a nine-step process. As the therapist enters into the clini- adaptations, and learning within the client as an organism or
cal environment of the client, he or she starts collecting data (2) improve function through compensation and modification
that might be relevant to the analysis of the clinical problem of the external environment. Once the therapy diagnosis has
(step 1). This includes information obtained through obser- been established, a clinician must consider many factors
vation, history taking, chart review, and interviews. The when making a prognosis. Some factors are related to the
therapist must take that array of divergent information and internal environment of the client, such as number and extent
determine what data are relevant to the case while disregard- of impairments, level of physical conditioning or decon-
ing what may be irrelevant information (step 2). This body ditioning of the client, the ability and motivation to learn,
of knowledge is then differentiated into various body sys- participate, and change, and the neurological disease or con-
tems that might be affected by the identified problems. If a dition that led to the existing problems. The client’s support
specific system does not seem to be affected, then it can be systems have a dramatic impact on prognosis. Cultural and
eliminated, at least temporarily, from the diagnostic process ethnic pressures, financial support to promote independence,
(step 3). Generally, a clinician performs activity-based test- availability of appropriate skilled professional services, pre-
ing at this time to obtain a general understanding of the scribed medications, and the interaction of all of these factors
strengths and limitations of the individual in terms of func- need to be considered. Specific environmental factors such as
tion (step 4). After performing examination procedures and belief in health care and agreement about who has the re-
observing patterns of movement and specific normal and sponsibility for healing can create tremendous conflict among
abnormal responses, the therapist once again diverges his or current health care delivery systems; the client; the family;
her thought processes back to separate large body systems to and you, the clinician.79,82,83 All of these variables affect
classify problems in the appropriate system (step 5). The prognosis. The last aspect of determining prognosis relates to
therapist further subdivides these large systems into their empowerment of the client. Who sets the goals? Who deter-
components to assess specific subsystem deficits and mines function? Who identifies when a therapeutic modality
strengths (step 6). This will allow the therapist to categorize should be used versus a meaningful life activity? If consen-
objective measurements of impairments that are recognized sus to these questions cannot be found by the therapist and
as deficits within subsystems. Clusters of specific signs and the client, then conflict between anticipated and actual out-
symptoms will emerge that will help direct the clinicians to come will result and a definitive prognosis will not be
a therapy diagnosis. Once the therapist has obtained these achieved.
clusters of symptoms within specific subsystems, two addi- Once a prognosis has been established, the therapist’s
tional convergent steps need to be completed. First, the pres- next step is to identify the intervention strategies that will
ence and lack of impairments and how those impairments guide the client to the desired outcome within the time
interact to cause dysfunction in a major body system are frame identified. (Refer to Chapter 9 and all chapters in
determined (step 7). Second, how those impairments affect Section II.)
the interaction of the major system with other major body
systems is determined (step 8). These eight steps tell the DOCUMENTATION
therapist exactly why the client has difficulty performing Documentation of the examination, evaluation, goals, plan,
specific functional activities. The problem list that incorpo- and daily interventions has always been integral to the
rates the severity of impairments that have interacted to cause therapeutic process. However, there is added emphasis in
loss of function gives the clinician the therapy diagnosis. The today’s health care environment, as well as a renewed re-
number and extent of impairments along with an understand- spect for the importance of the issue. Documentation must
ing of the cause of loss of function will lead the therapist produce a clear framework from which to record and follow
to establishment of various prognoses and identification of client progress. Documentation communicates the process
optimal intervention strategies. The last step (step 9) requires of care and the product or outcome of that process. The
the therapist to diverge his or her thought processes back outcome is the realistic reflection of the effectiveness of
to the client’s total environment to determine the accuracy care. The goals should be stated in measurable functional
of the diagnosis, prognosis, and selected treatment interven- terms and prioritized in order of importance to the client.
tions as they interact with the client as a whole. Although The number of goals developed by the therapist takes into
some completion of this diagnostic process may occur within account the realistic probability of effectiveness of interven-
minutes after a client and therapist begin their interactions, tions, the environment in which the interventions will likely
the process is continual, and at any time a therapist may need occur, and support systems available to the client. As the
to go back to previous steps to obtain and analyze new and process goes forward, the therapist may add, delete, or
relevant information. change a functional goal, and so states that on the client’s
record. Refer to Chapter 10 for further information about
PROGNOSIS: HOW LONG WILL IT TAKE TO this process.
GET FROM POINT A TO POINT B?
If a client has a variety of impairments, activity limitations, INTERVENTION
and participation restrictions, then a variety of appropriate Clients with neurological diseases or conditions can interact
prognoses may be formulated. These prognoses could be with the medical community for either short or long periods.
used to speculate the amount of time or number of treatments They possess neurological problems of all types that range
it will take to get from the existing activity limitations and from sudden to insidious in onset and presentation. All
participation restrictions (point A) to the desired outcomes aspects of human function are represented in the variety of
problems. If individual beliefs and values energize and mo- client will determine the extent to which the intervention
tivate physical behavior, think of the possibilities for stimu- would be considered contrived. Contrived interventions can,
lating wellness. Return to wellness might be considered rein time, lead to functional independence of the client, but as
turn to previous function, maintenance of function, slowing long as the therapist needs to control the environment, func-
progression of functional loss, habilitation of function never tional independence has not been achieved. There are many
achieved, and striving for excellence in performance. Refer ways to get to a desired outcome. Involving the client in the
to Chapter 9 for additional discussion. goal setting and intervention planning process will lead to the
The established plan of care determines the interventions best result These interactions require trust of the therapist as
and the method, or road map, toward the achievement of the a guide and teacher. Refer to Chapter 9 and all chapters in
agreed-on outcome goals. The therapist, in collaboration Section II for a more thorough discussion of intervention
with the client, can choose from restrictive and nonrestric- strategies.
tive treatment environments and interventions to best achieve Most treatment interventions used for clients with CNS
identified goals. The available choices of interventions will pathology incorporate principles of neuroplasticity, adap-
depend on the therapist’s skill, the level of function and abil- tation, motor control, and motor learning in various envi-
ity of the client to control his or her own neuromuscular ronmental contexts. Thus, the consideration of the basic
system, and treatment tools and strategies that are available science of central and peripheral nervous system function
in the clinic. Yet freedom within that established environ- (see Chapter 4) and a behavioral analysis of movement
ment must exist if learning by the client is to occur. Another (see Chapter 3) must be included in any conceptual model
way to consider intervention is to refer to it as a clinical road used as a foundation for the entire diagnostic process.
map (Figure 1-7). Within the map, a therapist, through pro- Also of considerable significance is the client-therapist
fessional education, efficacy of preexisting clinical path- interaction, which is labeled the learning environment. This
ways, and clinical experience can generally identify the may be the critical factor in the success or failure of thera-
most expedient way to guide a client toward the desired peutic interventions. The concept of human movement as a
outcome. When the specific client enters into this interac- range of observable behaviors, the complexity of the CNS as
tion, slight variations off the existing pathway may lead to a control center, and the interactions between the client and
quicker outcomes. If the client diverges away from the de- therapist within closed and open learning environments
sired end product, it is the therapist’s responsibility to guide form an abstract conceptual triad. Each part of this triad has
that individual back into the clinical map. For example, if a unique characteristics that have the ability to influence per-
therapist and client are working on coming to standing pat- formance and progress in the clinical setting. Together they
terns and the client begins to fall, the therapist would need allow for the client to be viewed as a total human being, al-
to guide the client back into the desired movement patterns lowing the therapist to consider multiple constructs at once
and not allow the fall. In that way the client is working on so that a client’s responses and movement patterns may all
the identified outcome. Falling as a functional activity be considered and developed simultaneously. In this way,
should be taught as a different intervention and would be key signs such as movement in body parts distant to the area
considered part of a different clinical map. The degree to being treated, pain, or a response of the autonomic nervous
which the therapist needs to control the response of the system will not be missed. Attention to these responses
Figure 1-7 n Concept of clinical mapping.

may be the answer to attaining goals and successful client- motor development requires new motor plans that lead to
therapist rapport. the infant’s ability to achieve functional movement through
motor learning. These new motor programs will be modi-
Concept of Human Movement as a Range of fied and reintegrated along with other programs to develop
Observable Behaviors normal motor control in more complex patterns and envi-
As researchers continue to unravel the mysteries of brain ronments because of neuroplasticity. Each pattern, and the
function and learning, their understanding of how children advancement from one pattern to another, requires time
and adults initially learn or relearn after neurological insult and repetition for mastery.
is often explained with new and possibly conflicting theo- Two important aspects of the clinical problem-solving
ries. Yet behavioral responses observed as functional pat- process emerge when observing motor behavior. First, the
terns of movement, whether performed by a child, adoles- evaluation of motor function is based on the interaction of
cent, young adult, or older person, are still visually identified all components of the motor system and the cognitive and
by a therapist, family member, or innocent observer as either affective influences over this motor system, as stated pre-
normal or abnormal. viously. Second, the therapist needs to recognize which
Human beings exhibit certain movement patterns that aspects of the movement are deficient, absent, distorted,
may vary in tonal characteristics, amplitude, aspects of the or inappropriate when cross-referenced with the desired
specific movement sequences, and even the sequential nature outcome (part of the diagnosis-prognosis process). These
of development. Yet the range of acceptable behavior does behaviors, although dependent on many factors, are con-
have limitations, and variations beyond those boundaries are sistent regardless of age of the client. Some clients may
recognizable by most people. A 5-year-old child may ask not have had the opportunity to experience the desired
why a little girl walks on her toes with her legs stuck to- skill, whereas others may have lost the skill as a result of
gether. If questioned, that same 5-year-old child may have changes within the CNS or disuse. In either case, the nor-
the ability to break down the specific aspects of the move- mal accepted patterns and range of behaviors remain the
ment that seem unacceptable even to that 5-year-old child. same. Refer to Chapter 3 for an additional discussion of
From birth a sighted individual observes normal human movement analysis across the life span.
movement. Because the range of behaviors identified as nor-
mal within any functional activity does not vary from indi- The Complexity of the Central Nervous System
vidual to individual, human movement patterns are predict- as a Control Center
able. This concept does provide flexibility in analysis of The concept of the CNS as a control center is based on a
normal movement and its development. Some children therapist’s observations and understanding of the sensory-
choose creeping as a primary mode of horizontal movement, motor performance patterns reflective of that system. This
whereas others may scoot. Both forms of movement are nor- understanding requires an in-depth background in neuro-
mal for a young child. In both cases each child would have anatomy, neurophysiology, motor control, motor learning,
had to develop normal postural function in the head and and neuroplasticity and gives the therapist the basis for
trunk to carry out the activity in a normal fashion. Thus for clinical application and treatment. Understanding the intri-
the child to develop the specific functional motor behavior, cacies and complex relationships of these neuromechanisms
the various components or systems involved in the integrated provides therapists with direction as to when, why, and in
execution of the act would require modulation in a plan of what order to use clinical treatment techniques. Motor be-
action. Because the action must be carried out in a variety of haviors emerge based on maturation, potential, and degen-
environmental contexts, the child would need the opportunity eration of the CNS. Each behavior observed, sequenced, and
to practice in those contexts, identify errors, self-correct to integrated as a treatment protocol should be interpreted ac-
regulate existing plans, and refine for skill development. cording to neurophysiological and neuroanatomical princi-
Thus each movement has a variety of complex systems inter- ples as well as the principles of learning and neuroplasticity.
actions, which when summated are expressed by means of As science moves toward a greater understanding of the
the motor neuron pool to striated muscle tissue function. The neuromechanisms by which behaviors occur, therapists will
specifics of that function, whether fine or gross motor con- be in a better position to establish efficacy of intervention.
trol, or full-body or limb-specific movement, still reflect the Unfortunately, our knowledge of behavior is ahead of our
totality of the interaction of those systems. No matter the understanding of the intricate mechanisms of the CNS that
age of the individual, the motor response still reflects that create it. Thus the future will continue to expand the reli-
interaction, and the behavior can be identified as normal and ability and validity of therapeutic interventions designed to
functional, functional but limited in adaptability, or dysfunc- modify functional movement patterns. First, therapists need
tional and abnormal. Because of the simplicity or complexity to determine what interventions are effective within a clini-
of various movements and the components necessary to cal environment. Then the efficacy of specific treatment
modulate control over various movements, therapists can variables can be studied and more clearly identified. The
(1) look at any movement pattern, (2) evaluate its compo- rationale for the use of certain treatment techniques will
nents, (3) identify what is missing, and (4) incorporate treat- likely change over time. As our knowledge of the CNS con-
ment strategies that help the client reduce impairments and tinues to evolve, so will the validation of techniques and
achieve the desired functional outcome. approaches used in therapeutic environments. At that point,
One can be confident that no infant will be born, jump evidence-based practice will truly be a reality. Chapters 4
out of the womb, walk over to the physician, and shake and 5 have additional information and references on CNS
hands or say “hi” to mom and dad before learning to roll function. Chapter 9 has an in-depth discussion of interven-
or control posture of the trunk and head. Instead, normal tion options.
Concept of the Learning Environment instituted.84 The use of preferential sensory input modes
The concept of the learning environment is the most abstract such as visual compared with verbal or kinesthetic does not
and complex of the three concepts in the clinical triad mean that other modes are ineffective, nor do all modes
model. For that reason it is by far the most difficult to pres- function optimally in any given situation.
ent in concrete terms. Components of the therapist, the pa- One way to determine preferential learning styles is by
tient, and the clinical environment formulate and maintain taking a thorough history. Leisure activities and job choices
this environment. often give clues to learning styles. For example, a client who
To comprehend the dynamics of the learning environ- loved to take car engines apart or build model ships demon-
ment and function with optimal success, the clinician must strates a preference for the visual-kinesthetic learning style,
do the following: whereas a client whose preference for pure enjoyment was
n Understand the learning process and provide an envi- sitting in a chair with a novel demonstrates a probable pref-
ronment that promotes learning. erence toward verbal learning. Again, this does not mean
n Investigate the use of sensory input and motor output that the clients in the examples mentioned could not selec-
systems, feed-forward and feedback mechanisms, and tively use all methods, but it does illustrate the issue of pref-
cognitive processing as means for higher-order learn- erence. Both the position of the lesion and the preferential
ing. learning style can play key roles in matching the learner with
n Use the principles and theories of motor control, mo- a particular treatment environment and identifying potential
tor learning, and neuroplasticity to facilitate learning for recovery of function. For example, if a client has had a
and carryover of treatment into real-life environments. massive insult to the left temporal lobe and before the trauma
n Obtain knowledge of both the client’s and the pro- showed poor ability in using the right parieto-occipital lobe,
vider’s learning styles. If these learning styles are not then spatial or verbal strategies may be ineffective in the
compatible, then the clinician is obligated to teach us- relearning process. However, a client with the same lesion
ing the client’s preferred style. who had high-level right parieto-occipital function before
n Attend to the sensory-motor, cognitive, and affective the insult will probably learn at a much faster rate if visual-
aspects of each client, regardless of the clinical em- kinesthetic strategies are used to promote learning.
phasis at any given time. The client’s external environment is the second critical
At all times there are four distinct components of the component.85 All external stimuli, including noise, lighting,
learning environment in operation: the internal and external temperature, touch, humidity, and smell, modulate the cli-
environments of the client, and the internal and external ent’s responses. External inputs can invoke either negative
environments of the clinician (Figure 1-8). All four repre- or positive influences on internal mechanisms and alter the
sent interactive components of the learning environment. client’s ability to manipulate the world. A therapist should
make every effort to be aware of what externally is influenc-
The Client ing the client.86-89 It is important to know what is happening
A critical component of the ability to learn is the client’s to the client both within and outside of the hospital or clinic
internal environment. When a lesion occurs within a body experience. Any behavioral change displayed by the client,
system it affects the entire internal environment of the client such as a change in mood or attitude, or a change in muscle
both directly and indirectly. If the lesion occurs before initial tone could serve as an indicator to the therapist that an envi-
learning, then habilitation must take place. These clients ronmental effect may have occurred. A follow-up determi-
may possess a genetic predisposition for a specific learning nation of what may have happened can help the therapist
style, even though one has not yet been established. The understand the situation, help the client deal with the envi-
therapist should test the inexperienced CNS by creating ex- ronmental influences, and allow the therapist to obtain ad-
periences in various contexts that require a variety of types ditional professional assistance if needed.
of higher-order processing to discover optimal methods of The third critical component is the internal environment
learning that best suit the CNS of the client. Then the thera- of the clinician.90 The clinician should be aware of personal
pist can employ the most effective strategies in treatment. If internal factors that can influence patient responses. Every-
previous learning has occurred and preferential modes of one has preferential styles of teaching and learning; yet
operating have been established, then the therapist needs to many of us may be unaware of what they are and how they
know what those are and whether they have been affected by affect our outlook on life and interactions with other people.
the neurological insult so that proper rehabilitation can be A common example of a mismatch of styles is what happens
when two people are arguing opposing sides of a political
issue. Although both individuals may process the same data,
they may have different learning strategies and come up
with very different conclusions.
The interplay of learning styles occurs continually in an
academic setting. A student who is asked the question
“What do you want out of this course?” would probably say,
“A good grade.” Getting a good grade requires doing well
on course requirements, including tests. High-grade test
performance usually depends on not only a knowledgeable
demonstration of a subject but also the way in which the
teacher formulates the question and the teacher’s expecta-
Figure 1-8 n Clinical learning environment. tion for a response. In a clinical setting, it is important that
the clinician be aware of the client’s response to the practi- 3. An individual will revert to safer or more familiar mo-
tioner’s request. tor programs or ways to solve problems and succeed
This external-internal environmental interaction concept at functional tasks when task demands are new, diffi-
brings up another important clinical consideration.91 As stu- cult, or unfamiliar.
dents, most of us probably “clashed” with one or two teach- 4. The learning effect occurs in multiple areas of the
ers with whose learning styles we could never identify. As CNS simultaneously when teaching and learning are
learners we cannot or will not adapt to all learning styles. For focused within one area of the CNS.
that reason there may be some clients who do not respond to 5. Motivation is necessary to drive the individual to try
our teaching. When that seems evident, a shift of therapists is to experience what would be considered unknown.
appropriate for the rehabilitation process to succeed. Simultaneously, success at the activity is critical to
The fourth component of the learning environment is the keep the individual motivated to continue to practice.
clinician’s external environment. It is generally expected 6. Clinicians need to be able to analyze an activity as a
that personal life should never affect professional work. To whole, determine its component parts, and use problem-
accept this assumption, however, may be to deny that emo- solving strategies to design effective individualized
tions affect behavioral patterns (see Chapters 5 and 6 ). Re- treatment programs. At the same time, if independence
sponse patterns can vary without cognitive awareness when in living skills is an objective, the therapist needs to
an individual is emotionally upset or under stress. For ex- teach the client those problem-solving strategies rather
ample, suppose that Mr. Smith, who has a hypertonic condi- than teaching the solution to the problem.
tion because of a stroke, comes down early for therapy each Although all six learning principles seem simple, their
morning, has a cup of coffee, and chats while you write application within the clinical setting is not always as obvi-
notes. If one day you are under extreme stress and do not ous. Principles 1 and 2 are intricately linked with the ap-
feel like interacting as Mr. Smith rolls his wheelchair into propriateness and difficulty of tasks presented to clients. If a
your office, you might say, “Mr. Smith, I’ll be with you in a client is asked to perform a task such as standing, rolling,
few minutes. Go over to the mat, lock your brakes, pick up relaxing, dressing, or maneuvering a wheelchair, a problem
the pedals, and we’ll transfer when I get there.” Mr. Smith has been presented that requires a sequence of acts leading
will quickly sense a change in your behavior. Society has to a solution. To succeed, the client must be able to plan the
taught him that you are a professional and that your personal entire task and modulate all motor control during the se-
life does not affect your job. Thus he may draw a logical quence of the entire activity. If steps are not mastered, if
conclusion that he must have done something to change sequencing is inappropriate or absent, or if motor control
your behavior. When you go to transfer him you notice he is systems are not modulated accurately, dependence on the
more hypertonic than usual and ask, “Is something bother- clinician to solve the problem is reinforced. If the clinician
ing you? You’re tighter than usual,” and so goes the interac- can differentiate missing components (impairments) from
tion. Your external environment altered your internal state functioning systems, creating an environment that encour-
and, thus, normal response patterns. In turn, you altered ages and allows the CNS to adapt and learn ways to regain
Mr. Smith’s external environment, changing his internal bal- that control, it will lead to optimal self-empowerment of the
ance, and created a change of emotional tone that resulted in client and will help eliminate disabilities. Error in the ability
increased hypertonicity.92,93 If instead of interacting with to intrinsically self-correct during practice is critical for mo-
Mr. Smith as if nothing were wrong, you had informed him tor learning. Error that always leads to failure does not help
you were upset over something unrelated to him, you might the client learn avenues of adaptation. Linked intricately
have avoided creating a negative environment. Mr. Smith’s with success is the challenge of the task. The greater the task
responses may have been different if you had shared with difficulty or complexity, the greater the challenge and con-
him the fact that there are days that you are upset and have sequently the greater the satisfaction of success.
mood changes. As he accepts your changes as normal, you There is a subtle interplay among degree of difficulty,
may have created an opportunity for him to also exhibit challenge, and success. Selecting tasks that are age appro-
a range of behavioral moods. You have also given him an priate, clinically relevant, and goal related is a challenge to
opportunity to offer his assistance to comfort or help you if the therapist. For the patient to be successful, the therapist
he so desires. Such behavior encourages interdependence must be a creative problem solver and knowledgeable about
and social interaction and facilitates long-term goals for all the client’s needs, abilities, and goals. If the tasks are too
rehabilitation clients. simple or if the client considers them unimportant, boredom
Each client is unique. Therefore it is difficult to analyze will ensue and progress may diminish. If the tasks are too
the specifics related to each individual’s learning environ- difficult, the client may feel defeated and may turn away
ment. However, six basic learning principles have been es- from them. In such cases a child tends to withdraw physi-
tablished that are relevant to both the client and the clinician cally, whereas an adult usually avoids the problem. Being
in any learning environment.94-96 These six principles of the late to therapy, having to leave early, needing to go to the
learning experience are as follows: bathroom, and scheduling conflicting sessions are all avoid-
1. Individuals need to be able to solve problems and prac- ance behaviors that may be linked to inappropriate tasks.
tice those solutions as motor programs if independence The third learning principle describes a behavior inher-
in daily living is desired. This requires the use of intrin- ent in all people: reversal. When confronted by a problem,
sic feedback systems to modulate feed-forward motor individuals revert to patterns that produce feelings of
plans as well as correct existing plans. comfort and competence when solving the problem. In
2. The possibility of success must exist in all functional Figure 1-9, a 2-year-old child is confronted with just such
tasks, regardless of the level of challenge to the client. a conflict. The bridge he wants to cross is unstable. The
Figure 1-9 n Reverting to more comfortable behavior patterns

when confronted with a problem. A, Scooting. B, Bunny hopping.
C, Creeping. D, Cruising. E, Walking.
task goal is to cross the bridge; how that is accomplished and finally to reciprocal walking. The child’s reversal
is not as relevant as the task specificity. Therefore the lasted approximately 2 minutes. Although reverting to
child chooses a 6-month-old behavior and thus scoots. On more familiar or comfortable ways of solving problems
gaining confidence, the child sequences from scooting to is normal, it creates constant frustration in the clinic if
four-point bunny hopping, then creeping, on to cruising, it is prolonged. For example, if a client with residual
hemiplegia has spent a week modifying and controlling a example, Mr. Brown, a 63-year-old bank president with a
hypertonic upper-extremity pattern during a simple task wife, four children, and 10 grandchildren, survives an oper-
and is now confronted with a more difficult problem, the able brain tumor with residual right hemiparesis and mini-
hypertonia within the limb will most likely return with the mal cognitive-affective deficits. The client’s work history
added complexity of the task. If another client has suc- indicates that he was highly success oriented. Unknown to
cessfully worked to obtain the standing position and then most persons is that for 63 years Mr. Brown desired to be a
is asked to walk, the strong synergistic patterns that had passive-dependent person, but circumstances never allowed
been controlled may return. The pattern or plan for stand- him to manifest those behaviors. With the neurological in-
ing is different from that for walking, and the emotional sult, he is in a position to actualize his needs. Until the client
implications of walking are very high. The clinician desires to improve, therapy will probably be ineffective;
should anticipate the possibility of the patient returning to thus, motivating the client becomes critical. This might be
a more stereotypical pattern. This possibility must also be accomplished in a variety of ways. Knowing that Mr. Brown
explained to the patient. Anticipating that less efficient values privacy, especially with respect to hygiene, that he
patterns will usually return as the tasks demanded in- thoroughly enjoys dancing and birdwatching in the forest,
crease in complexity, the clinician can attempt to modify and that he ascribes importance to being accepted in social
the unwanted responses and let the patient know that the situations, such as cocktail parties, helps the therapist create
response is actually normal given the CNS dysfunction, a learning environment that motivates this client toward in-
but that movement can be changed and normalized with dependence. Being independent in hygiene requires certain
practice. The key to comprehension of this concept is not combinations of motor actions, including sitting, balance,
the behavior itself; instead, it is the attitude of a therapist and transfer skills. Being able to birdwatch deep in an un-
toward a new task presented to the client. If the clinician populated forest requires ambulation skills, tolerance of the
expects the client to be successful, the client will also upright position for extended periods of time, and endur-
expect success. If failure occurs, both parties will be dis- ance. Being socially accepted depends to a large extent not
appointed and a potentially negative clinical situation will only on grooming but on normal movement patterns, espe-
be created; however, if the client succeeds, both will have cially in the upper extremity and trunk. Creating a therapeu-
expected the result and their attitude will be neither excited tic environment that stresses independence in the three goals
nor depressed. On the other hand, a clinician who expects identified by the client will simultaneously create further
the client to revert to an old behavior can prepare the cli- independence in other areas. Whether the client decides to
ent. If the client reverts, neither party will be disappointed; return to banking and other activities in conflict with his per-
but if no reversion occurs, both will be excited, pleased, sonality will need to be addressed later. Another way to moti-
and encouraged by the higher functional skill. By under- vate Mr. Brown is to place him in an environment in which he
standing the concept, the clinician can maintain a very is not satisfied, such as a nursing home or his own home with
positive clinical environment without the constant negative an assistant rather than his wife to help him with his needs.
interference of perceived failure when a client does revert. Dissatisfaction with the current external environment will
The fourth learning principle deals with the totality of generally motivate an individual to change. Obviously, cre-
the client. Whether the area of emphasis is motor perfor- ating a positive environment for change versus a negative
mance, emotional balance, or perceptual integration, all ar- one would be the method of choice.
eas are affected. Therefore understanding and respect for all The sixth learning principle has been discussed in earlier
areas are important if optimal client function is a primary sections. To be a successful teacher of motor skills and to
objective. This does not suggest that therapists should ad- assist the client in recovery of function, the clinician should be
dress each aspect of personality; however, integration of the able to break more complex motor plans and functional tasks
client’s physical, mental, and spiritual areas should be a re- into smaller component parts. These parts can then be taught
sponsibility of the staff. Awareness of possible adverse ef- successfully and then integrated back into the whole activity
fects of one learned behavior on other CNS functions can for optimal learning to occur. The therapist should always
help avoid potential problems. For example, if working on strive to allow each client to solve movement problems and
lower extremity patterns creates extreme upper extremity develop strategies for reaching the outcome goals, rather than
hypertonicity through associated patterns, the clinician is producing the desired outcome for or with the patient.
not dealing with the client as a whole. Many additional learning principles from the fields of
The unknown creates fear and curiosity for most indi- education, development, and psychology can be used to ex-
viduals, and the fifth learning principle points out that for plain the behavioral responses seen in our clients. It is not
most clients the unknown is all encompassing, whatever the expected that all therapists will intuitively or automatically
degree of prior learning. For a client whose only difficulty is know how to create an environment conducive to helping
a flaccid upper extremity, functional activities such as toilet- the patient achieve optimal potential. Yet all can become
ing, dressing, or eating will be troublesome and unfamiliar. better at creating a beneficial learning environment by un-
Motivation is a critical factor for success. Maintaining moti- derstanding how people learn. The critical importance of
vation to try while ensuring a high degree of success is an being honest and accurate with prognosis and how that will
important teaching strategy that tends to encourage present ultimately affect function outcomes, participation in life,
and future learning. and quality of life cannot be overemphasized.97
An additional comment regarding clients who lack moti- The principles presented in this chapter deliver a strong
vation should be made. If a client chooses to be totally de- message: individuals need to solve problems and most want
pendent and has no need to become independent, then a to solve the problem given a chance that the solution will
therapist will probably fail at whatever task is presented. For be successful. Unless the task fits the individual’s current
capability, adaptation using whatever is available will be- health care delivery. As the gap grows between technology
come the consensus that drives the motor performance and the users of that technology, client health education
through the CNS. Learning is taking place in all aspects of becomes more important than ever.103 McNerney31 notes
life, and the client must ultimately take responsibility for the that although health care providers are now making efforts
means to solve the problem.98 to educate their clients, they are doing so with little consis-
tency, enthusiasm, theoretical base, or imagination and
often with little coordination with other services. The
THE CLIENT AND PROVIDER RELATIONSHIP
health care professional continues to receive training and
The Client’s Role in the Relationship embrace professional organizational membership that
Active participation in life and in relationships promotes places a premium on control of information and control of
learning. Rogers99 defines significant learning as learning the decision making. There is and should be a special effort
that makes a difference and affects all parts of a person. We to introduce health education concepts into the basic edu-
have spoken of a relationship centered on an individual’s cational programs of health care professionals. McNerney
health. One of the individuals involved in the relationship identified many of the problems three decades ago, and
(the therapist) has knowledge that is to be imparted to or many still exist today.
skills to be practiced by the other. The relationship “works” When patients are given more information about their
if the learning environment facilitates exchange between the illnesses and retain the information, they express more
participants. The concept of equal partners is crucial. The satisfaction with their caregivers. A study by Bertakis104
issue and practice of informed consent is not just political or tested the hypothesis that patients with greater understand-
ethical; it is central to client care. Voluntarism has to be ing and retention of the information given by the physician
practiced by both practitioner and client. Each has a moral would be more satisfied with the physician-patient rela-
obligation to facilitate the process of health care within the tionship. The experimental group received feedback and
moment. Although the Western world of medicine has retained 83.5% of the information given to them by the
steadily climbed a path toward excellence in medical tech- physician. The control group received no feedback and
nology and clarification of medical diagnosis as seen in retained 60.5% of the information. Not surprisingly, the
WHO’s ICD-10,100 it has not as easily recognized the cli- experimental group was more satisfied with the physician-
ent’s need to assume an equal role in the decision making or patient relationship.
for the practitioner to seek the client’s help. Consumers are If the client is to be informed and included in the treat-
now seeking to play a more active role in their health care. ment process, client health education will have to go beyond
This role has developed out of our scientific understanding the current styles of information giving. If the client is to
of motor learning (see Chapter 4) and the fiscal necessity of assume some of the responsibility for his or her therapy, the
decreasing the number of therapeutic visits. therapist will have to facilitate that involvement. The atti-
Consumers of health care are becoming aware of the affect tude of the therapist toward educating clients about their
of medicine’s control over their lives. This awareness has health could affect his or her ability to facilitate client in-
been fueled by the price they are paying for that health care. volvement in the care process.
A recent Surgeon General’s report confirms that expendi- The more the professional sees himself or herself as the
tures for health are increasing. In addition, preventive care expert, the less likely he or she will be to see the client as
assumes major importance in view of the fact that seven out capable of responsibility or expertise in the care process. If
of 10 deaths in the United States today are the result of communication skills and health education were an integral
degenerative diseases, such as heart disease, stroke, and part of medical school and health care professional school
cancer.101 Like other major causes of death, trauma (cited as curricula, perhaps the health care professionals would temper
the most frequent cause of death in persons younger than their assumption of the “expert” professional role. Payton105
age 40 years102) is increasingly linked to lifestyles. points out that it is the client alone who can ultimately decide
During their training, individuals in the health care pro- whether a goal is worth working for. Careful planning can be
fessions internalize values that reinforce the traditional influential in helping all providers include the client in the
professional attitude alluded to earlier. Many of these val- process.
ues do not support a partnership relationship with the cli- The health care delivery system in the United States has
ent. Society is beginning to question the traditional role of to serve all citizens.106 That is no easy task. The United
the health care professional as the knowledge expert; how- States is a society of great pluralism. It is a free society. It is
ever, professional educational institutions and organiza- a society that is used to being governed by persuasion, not
tions resist the pressure to change the image. The profes- coercion. Given the variety of economic, political, cultural,
sions still hold the image of great authority given to them and religious forces at work in American society, education
by the public and fostered through increased political ac- of the people with regard to their health care is probably the
tivity. This is true for both those professionals dealing with only method that can work in the long run. The future task
disease and pathology (physicians, nurses, pharmacists) of health education will be to “cultivate people’s sense of
and those dealing with functional movement problems responsibility toward their own health and that of the com-
limiting participation in life (OTs, PTs, Speech Language munity.” Health education is an effective approach with
Pathologists). perhaps the most potential to move us toward a concept of
The major purpose of the patient’s relationship with the preventive care.
health care professional is to exchange information useful Becker and Maiman107 discussed Rosenstock’s Health
to both regarding the health care of the client. McNerney31 Belief model as a framework to account for the individual’s
calls health education of the client the missing link in decision to use preventive services or engage in preventive
health behavior. Action taken by the individual, according to assume responsibility for their own care; however, better
the model, depends on the individual’s perceived suscepti- outcomes can be achieved if this is permitted and accepted.
bility to the illness, his or her perception of the severity of Therapists are caught up in the same problems of the
the illness, the benefits to be gained from taking action, and health care system as other health care professionals. Infla-
a “cue” of some sort that triggers action. The cue could be tion has often caused profit to become a more important
advice from a friend, reading an article about the illness, a motive than human care considerations for setting priorities
television commercial, and so on. In some way, the person in our clinics. Research is heavily focused on technical pro-
is motivated to do something. cedures, yet the relationship with patients in the care process
Mass media has promoted individuals’ education, which is vital. Singleton131 labeled this phenomenon a paradox in
may correctly guide or misguide consumers’ decision mak- therapy. Despite the commitment to humanistic service on
ing.108,109 This concept was put forward as early as 1976.110 which the profession was founded, the service rendered is
Today the consumer thus has a heightened expectation of the often mechanistic.
quality of care he or she will receive. Similarly, consumers The educational programs should emphasize whole-
come to receive medical care because of media education, patient treatment, increased communication skills, inter-
whereas in the past that level of education was not avail- disciplinary awareness, and patient-centered care.44-46,132
able.111,112 As of today, the media are just beginning to be The change in roles described previously requires a pro-
used by OT and PT professions, in the hope that this media fessional who demonstrates a potential for the assumption
use will educate the public regarding when, where, and how of many roles, responsibilities, and choices along the care
to decide on whether to seek our services. It will be a few pathway. The therapist working with the client with neu-
years before research can be done to determine if this use of rological problems must always be ready to respond to
media will assist in educating the public. triggers anywhere along the pathway from early interven-
Many aspects of today’s lifestyles do not reinforce well- tion, midway during a crisis, or later during long-term
ness. The obesity seen throughout the industrialized world care, because these triggers signal a need for change. Of
proves that point.113-115 Yet whether the client takes some equal importance, the path must be well documented to
responsibility for his or her functional problems and recov- empower the therapist to reflect on current and prognosti-
ery depends a great deal on whether the health care pro- cated treatment intervention.
vider gives some to him or her. Today’s literature certainly
reinforces the need for patient responsibility and active The Provider’s Role in the Relationship
participation in one’s own functional recovery.116-124 This Gifted therapists are often thought to have intuition. Yet in-
change in responsibility may be caused by third-party pay- tuitive behavior is based on experience, a thorough knowl-
ers and the lack of funding versus promotion of a new edge of the area, sensitivity to the total environment, and
health care model in which the patient is an active partici- ability to ask pertinent questions as the therapist evaluates,
pant, but as long as the change occurs, the world will be conceptualizes about, and treats clients (Chapter 5). How
better served. these questions are formulated and the answers documented
Fink125 in 1980, over three decades ago, recognized the vary among therapists, but the result is the formulation of a
importance of the provider-patient relationship. The state of unique profile for each client.
what is referred to as health varies for each client and in- Cognitive-perceptual processing by the client will often
cludes both the simple and complex variables of that client’s determine the learning environment to be used, the sequences
life, from the last nutritional meal to the totality of the cli- for treatment, and the estimated time needed for therapeu-
ent’s life event history (see Figure 1-5). The relationship of tic intervention. Thus the client is in a position to play an
the therapist and the client can lead to better use of the important role with the therapist within the clinical problem-
health care system by giving responsibility to the con- solving process. In that interactive environment, the thera-
sumer.126,127 The therapist has an advantage over most other pist can ask questions regarding cognitive, affective, and
health care practitioners, who see the patient at infrequent motor domains that will help clarify, document, and guide
intervals and who seldom touch the patient as a PT or an OT future decisions regarding empowerment of the client. (See
does. the client profile questions regarding cognitive, affective,
The illness or trauma of the client that represents a disin- and sensorimotor areas in Boxes 1-1, 1-2, and 1-3.) The mo-
tegrating force in his or her life may represent an opportu- tor output area is the main system the client uses to express
nity for the therapist to grow professionally. The client and thoughts and feelings and demonstrate independence to
the therapist may have different psychological backgrounds, family, therapists, and community. This motor area cannot be
and, although the client’s presence is usually related to a evaluated effectively by itself while the cognitive-perceptual
medical crisis, the therapist’s presence may support the pur- and the affective-emotional areas are negated. If that rigidity
poses of personal growth, financial gain, prestige, and un- becomes a standard of care, accurate prognosis and selection
conscious gratification in influencing the lives of others of appropriate interventions will continue to be inconsistent
through professional skill.128 and lack effectiveness within the clinical environment. No
As the consumer becomes more involved, so should the one will question that therapists today need to use reliable
family.129 Patients in therapy have always been happier with and valid examination tools in order to measure efficacy of
the family involved.130 The therapist must be willing to fa- our interventions. Finding the link between structured ex-
cilitate the involvement of the family members and help amination and intuitive knowledge is a characteristic of
them learn to take responsibility for some of the care and master clinicians. Even therapists who intuitively know a
decision making. Most health care professionals are not patient’s problem need to use objective measures today to
conditioned to allowing patients and family members to verify that intuition. Therefore third-party payers can justify
BOX 1-1 n COGNITIVE AREA QUESTIONS FOR CLIENT PROFILE
A. Sensory input: awareness level

1. What sensory systems are intact, and which have impairments?
2. Are any sensory systems in conflict with others?
3. If conflict between systems is present, to which system does the client pay attention?
B. Perceptual awareness and development
1. What specific perceptual processing deficits does the client have, and how would that affect motor performance?
2. Do the perceptual problems relate to input distortion, processing deficits, or both? If input distortion is alleviated, is
information processed appropriately?
C. Preferential higher-order cognitive system
1. Was or is the individual’s primary preferential system verbal, spatial, or kinesthetic?
2. Is the client’s preferential system different from yours? If so, can you work through the client’s system?
3. Is the client’s preferential system affected by the clinical problems?
4. Can the client adequately use nonpreferred systems?
D. Level of cognition
1. Is the client functioning on a concrete, abstract, or fragmented level?
2. Does the client’s level of cognition change? If so, when and why?
3. Is the client realistic? Does the client exercise judgment? If so, when? If not, when and why?
4. Which inherent systems or outside influences are interfering with or distorting the client’s potential? (Systems within the
individual and the environment around the client, such as the staff, the family, and the other patients, must be considered.)
BOX 1-2 n AFFECTIVE AREA QUESTIONS FOR CLIENT PROFILE
A. Level of adjustment or stage of adjustment to the health condition

1. At what level or stage of adjustment is the client with respect to the system problem and ability to participate in functional
activities?
2. At what level of adjustment is the family?
3. Will the level of adjustment of the client or family affect treatment?
4. If emotions are affecting treatment, what can be done to eliminate this problem?
B. Level of emotional control
1. Can the client exercise impulse control?
2. When does the degree of emotional or impulse control vary?
3. How did and does the client respond to stress?
4. How did and does the client respond to perceived success and failure?
5. What types of stresses outside of the specific physical system problems are being placed on the client?
C. Attitude (attitude toward bodily system problems and the functional ability is covered to some degree under level of
acceptance, although additional information needs to be gathered)
1. Before the onset of the bodily system problem, what was the client’s attitude toward individuals with cognitive
and/or motor system problems, and specifically, those related to his or her primary system problem?
2. What is the client’s attitude toward your professional domain?
3. What is the family’s attitude toward individuals with bodily system limitations and inability to participate in normal life
activities, especially those related to its family member?
4. What is the family’s attitude toward your professional domain?
D. Social adjustment
1. At what social developmental stage is the client’s performance?
2. Is the social interaction in alignment with cognitive and sensorimotor stages of development?
3. Are the family’s social interactions and expectations at the level of the client’s performance?
4. Is the client’s level of social adjustment the same as the rehabilitation team’s level of expectation?
5. Is the client aware of his, her, or others’ socially appropriate or inappropriate behavior?
BOX 1-3 n SENSORIMOTOR AREA QUESTIONS FOR CLIENT PROFILE
A. Level of motor performance with respect to performance

1. Is the client’s level of motor performance or sensory and motor integration congruous with the staff’s expected
performance level?
2. Is the client’s level of motor control integration congruous with the family’s expected performance level?
3. Is the client’s level of motor function congruous with his or her expected level of performance and participation?
B. Functional skills
1. What functional skills does the client perform in a normal fashion?
2. What functional skills does the client perform given systems limitations?
3. What functional skills has the client learned to perform that are reinforcing stereotypical patterns or hindering normal
movement and the ability to participate?
4. What functional skills do the client and family consider of primary importance? Will breaking these down to smaller
component skills hinder normal learning?
C. Abnormal patterns
1. What patterns are present?
2. When are these normal and abnormal patterns observed? Do they vary according to spatial positions?
3. Is there ever a shifting or altering in degree of these abnormal patterns? If so, under what circumstances does this variance
occur?
D. Degree of cortical override
1. Does the client need to inhibit abnormal output by intentional thought, or does he or she use procedural adjustment
through normal feed-forward mechanisms?
2. What amount of energy is being used to override abnormal output?
3. Can the client use cognitive systems to control motor output?
4. What amount of energy are you demanding the client to use when attending to the task? Are you asking the client to fully
attend to the specific motoric task, or are you overloading the system to take away some cortical attention?
payment for services while the patient benefits from the and philosophical reasons they came into existence. The
intuitive guidance in the clinical decision making by the only concept that is guaranteed in the future is change.
therapist. Both physical and occupational therapy are dynamic pro-
Once the therapist has a clear understanding of the cli- fessions with the ability to adapt and evolve to provide
ent’s strengths and weaknesses, specific clinical problems the health care service expected and deserved by the
can be identified and treatment procedures selected that consumer. The future is up to every practitioner. The con-
allow flexibility in treatment sessions. Many treatment sug- sumer of our services is dependent on our willingness
gestions for various problems can be found in Chapter 9 to learn, adapt, and provide a high quality of care at an
and Chapters 11 to 27. appropriate cost for the best outcomes. We have done that
in the past, are doing it in the present, and will continue
CONCLUSION doing it in the future.
In this sixth edition of the textbook, we hope to bring the
reader into the clinical practice of the twenty-first century. References
As the professions continue to evolve in depth and breadth, To enhance this text and add value for the reader, all refer-
the future will encapsulate the knowledge, skill, and les- ences are included on the companion Evolve site that
sons of the past and the needs and problems of current accompanies this textbook. This online service will, when
and immediate health delivery systems while maintaining available, provide a link for the reader to a Medline abstract
unique scopes and parameters of practice in an ever- for the article cited. There are 132 cited references and other
changing environment. The professions must adapt and general references for this chapter, with the majority of
grow as they embrace change without losing the integrity those articles being evidence-based citations.
CHAPTER 2 Health and Wellness:
The Beginning of the Paradigm
JANET R. BEZNER, PT, PhD

paradigm After reading this chapter the student or therapist will be able to:
perceptions 1. Define and differentiate the terms health and wellness.
well-being 2. Describe the characteristics of wellness.
wellness 3. Compare and contrast illness, prevention, and wellness paradigms.
whole person 4. Identify and analyze a variety of wellness measures.
5. Synthesize a wellness approach into neurorehabilitation.
I n learning to cope with the often chronic nature of their

conditions, individuals with neurological disease, not un-
like individuals with health conditions of other systems,
disease. Considering the catastrophic nature of many neuro-
logical diseases that compromise physical health, it is even
more important to distinguish between health and wellness
learn to rely on their abilities to adapt and compensate for to recognize and pursue avenues to enhance overall quality
their activity limitations and participation restrictions to of life and well-being.
regain the ability to participate in life. Although not an H. L. Dunn first conceptualized the term wellness in 1961
uncommon approach to life for any human being, the and offered the first definition of the term: “an integrated
achievement of health or wellness takes on an increased method of functioning which is oriented toward maximizing
focus for individuals with chronic health conditions, and it the potential of which the individual is capable.”2 Since
is strongly correlated to the quality of life they achieve. Dunn’s introduction of the term, numerous researchers and
A casual consideration of the terms health and wellness educators have attempted to explain wellness by proposing
indicates that they are similar, if not the same, in meaning, various models and approaches.3-11 Although the literature is
a commonly held belief among those without health condi- full of references to and information about wellness, includ-
tions. This interpretation of the terms becomes problematic, ing numerous definitions of the term, a universally accepted
however, in the presence of health conditions. Can an indi- definition has failed to emerge. Several conclusions can be
vidual with a health condition be well? Can a person without drawn, however, from the abundance of literature regarding
a health condition be ill? The concepts of health and well- wellness.
ness and their associated meanings and measures will be For many people, including the public, health and well-
explored in this chapter to provide a perspective for move- ness are synonymous with physical health or physical well-
ment specialists that will enhance their ability to promote being, which commonly consists of physical activity, efforts
health and well-being in clients with neurological disease. to eat nutritiously, and adequate sleep. Research has indi-
cated that when the public is asked to rate their general
DEFINITIONS AND RELATIONSHIPS health, they narrowly focus on their physical health status,
AMONG TERMS choosing not to consider their emotional, social, or spiritual
The classic understanding of the term health from a bio- health.12 Referring to the definition of wellness from Dunn,
medical perspective is “absence of disease.” The antonym of and consistent with numerous other theorists, it is obvious
health, therefore, is disease. The World Health Organization that wellness, as it is defined, includes more than just physi-
contributed to the confusion between the terms health and cal parameters.
wellness when in 1948 it defined health as “a state of com- The common themes that emerge from the various
plete physical, mental and social well-being, and not merely models and definitions of wellness suggest that wellness
the absence of disease or infirmity.”1 Indeed, there are nu- is multidimensional,2,4-13 salutogenic or health causing,*
merous illustrations of the influence of the mind and spirit and consistent with a systems view of persons and their
on the body and thus the importance, from a public health environments.2,15-17 Each of these characteristics will be
perspective, of considering more than the physical state of explored.
the body when formulating solutions to health problems. First, as a multidimensional construct, wellness is more
However, there is also value in differentiating health from than simply physical health, as the more common under-
more global concepts such as wellness and quality of life, if standing of the term might suggest. Among the dimensions
for no other reason than to explain the phenomenon that an
individual can be diseased and well or can experience a high
quality of life while simultaneously living with a chronic *
References 1, 2, 4, 7, 8, 10, 14.
25
included in various wellness models are physical, spiritual, Wellness

intellectual, psychological, social, emotional, occupational,
and community or environmental.18 Adams and colleagues18 Physical Social
in 1997, toward the aim of devising a wellness measurement
tool, proposed six dimensions of wellness on the basis of the
Spiritual Psychological
strength and quality of the theoretical support in the litera-
ture. The six dimensions and their corresponding definitions
are shown in Table 2-1.
Emotional
The second characteristic of wellness is that it has a salu- Intellectual
togenic or health-causing focus,14 in contrast to a pathogenic
focus in an illness model. Emphasizing the factors that cause
health (e.g., salutogenic) supports Dunn’s2 original defini-
tion, which implied that wellness involves “maximizing the
potential of which the individual is capable.” In other words,
wellness is not just preventing illness or injury or maintain-
ing the status quo; rather, it involves choices and behaviors
that emphasize optimal health and well-being beyond the
status quo. Thus an individual may or may not be well
before pathological conditions and health conditions involve
the body and similarly may be well during an acute episode
or chronic pathology or health condition whether that
chronic problem results in static activity limitations or even
progressive participation restrictions.
Third, wellness is consistent with a systems perspective.
In systems theory each element of a system is independent Illness
and contains its own subelements, in addition to being a
subelement of a larger system.12,15,16 Furthermore, the ele- Figure 2-1 n The wellness model.
ments in a system are reciprocally interrelated, indicating
that a disruption of homeostasis at any level of the system
affects the entire system and all its subelements.15,16 There- other dimensions.18 As an example, an individual who has a
fore overall wellness is a reflection of the state of being knee injury and undergoes surgery to repair the anterior
within each dimension and a result of the interaction among cruciate ligament will probably have at least a short-term
and between the dimensions of wellness. Figure 2-1 illus- decrease in physical wellness. Applying systems theory and
trates a model of wellness reflecting this concept. Vertical according to the model, this individual may also have a
movement in the model occurs between the wellness and decrease in other dimensions such as emotional or social
illness poles as the magnitude of wellness in each dimension wellness in the postoperative period. The overall effect of
changes. The top of the model represents wellness because these changes in these dimensions will be a decrease in
it is expanded maximally, whereas the bottom of the model overall wellness, which anecdotally we know occurs when
represents illness. Bidirectional horizontal movement oc- patients have an illness or injury.
curs within each dimension along the lines extending from A term related to wellness, quality of life, is also used
the inner circle. As per systems theory, movement in every to indicate the subjective experience of an individual in a
dimension influences and is influenced by movement in the larger context beyond just physical health. Quality of life
has been defined as “an individual’s perception of their posi-
tion in life in the context of the culture and value systems in
n DEFINITIONS OF THE DIMENSIONS
TABLE 2-1 which they live and in relation to their goals, expectations,
OF WELLNESS18 standards, and concerns. It is a broad ranging concept
affected in a complex way by the person’s physical health,
psychological state, level of independence, social relation-
Emotional The possession of a secure sense of self-identity
ships, and their relationship to salient features of their envi-
and a positive sense of self-regard
ronment.”19 Parallel to the issues related to the concept of
Intellectual The perception that one is internally energized
wellness, there is lack of agreement in the literature on the
by the appropriate amount of intellectually
definition of quality of life and its theoretical compo-
stimulating activity
nents,20-23 as well as variation in the use of subjective or
Physical Positive perceptions and expectancies of physical
objective quality-of-life indicators.21 Implied by the World
health
Health Organization definition, and supported by several
Psychological A general perception that one will experience
other authors, quality of life is best conceptualized as a sub-
positive outcomes to the events and circum-
jective construct that is measured through an examination
stances of life
of a client’s perceptions. In other words, quality of life, like
Social The perception that family or friends are avail-
wellness, is the subjective experience of health, illness,
able in times of need, and the perception that
activity and participation, the environment, social support,
one is a valued support provider
and so forth, and it is best measured through an assessment
Spiritual A positive sense of meaning and purpose in life
of client perceptions.
CHAPTER 2 n Health and Wellness: The Beginning of the Paradigm 27
A WELLNESS PARADIGM prevention approach typically results in a change in an indi-

The ultimate importance of gaining an understanding of vidual’s behavior. In contrast, the variables measured in
health and wellness is to be able to apply it when interacting a wellness paradigm are perceptual, indicating what the
with patients/clients. In this sense, the goal would be to im- patient/client thinks and feels about herself or himself.
prove the health and well-being of the client, in addition to Although clinical, physiological, and behavioral variables
improving movement and participation. A comparison of the are useful indicators of bodily wellness and are commonly
traditional “illness” paradigm with both “prevention” and used to plan individual and community interventions, their
“wellness” paradigms will identify ways in which a physical utility as wellness measures falls short.24 Clinical and phys-
or occupational therapist can incorporate a wellness para- iological measures assess the status of a single system, most
digm into the treatment of a patient with a neurological commonly the systems within the physical domain of well-
condition in the context of rehabilitation. The three ness. It can be argued that behavioral measures are a better
approaches or paradigms are contrasted in Table 2-2 on six reflection of multiple systems because of the importance and
parameters, including the view of human systems, program influence of motivation and self-efficacy on the adoption of
orientation, dependent variables, client status, intervention behaviors, but they do not describe the wellness of the mind.
focus, and intervention method. On the other hand, perceptual measures, capable of assess-
As stated previously, in a wellness paradigm each dimen- ing all systems and having been shown to predict effectively
sion or part of the system affects and is affected by every other a variety of health outcomes,18,25-29 can complement the in-
part, resulting in an integrative view of the human system. In formation provided by body-centered measures insofar as
contrast, in a traditional illness or medical model, the systems they are valid, congruent with wellness conceptualizations,
are independent. There are specialties in medicine by body and empirically supportable.24
system (e.g., neurology, orthopedics, gynecology), and in many The influence of perceptions on health and wellness has
physical and occupational therapy education programs, courses been demonstrated repeatedly in the literature with a multi-
are arranged by body system (e.g., neurology, orthopedics, plicity of patient/client populations and in a variety of
cardiopulmonary physical dysfunction, psychosocial), as indi- settings. Mossey and Shapiro25 demonstrated more than
cators of the independence of the systems. In a prevention ap- 25 years ago that self-rated health was the second strongest
proach, there is recognition that the systems interact, or influ- predictor of mortality in the elderly, after age. Numerous
ence one another, but not in the reciprocal fashion characteristic other researchers have replicated these findings in other
of wellness. populations, lending support to the value of perceptions in
The program orientation of an illness paradigm is the understanding health and wellness and indicating that how
pathology or disease-causing issue, whereas the orientation well you think you are may be more important than how
of a prevention paradigm is normogenic, meaning efforts are well you are as measured by clinical tests and measures or
aimed at maintaining a normal state or condition (e.g., nor- the judgment of a health professional. Wilson and Cleary24
mal muscle length, tone). Shifting to a wellness paradigm argued for the use of perceptions in understanding and ex-
requires a salutogenic or health-causing approach,14 with a plaining quality of life, proposing that health perceptions
focus on how to achieve greater well-being, health, or qual- provide an important link between the biomedical model or
ity of life. This shift emphasizes the capabilities and abilities clinical/illness paradigm, with its focus on “etiological
of the individual rather than the limitations and deficits. agents, pathological processes, and biological, physiologi-
The variables of interest in an illness
. paradigm are clinical, and clinical outcomes,” and the quality-of-life model
cal variables, such as blood tests, Vo2max (maximum vol- or social science paradigm, with its focus on “dimensions
ume of oxygen use), and tests of muscle strength. Changes of functioning and overall well-being”24 (Figure 2-2). Citing
in these variables result in labeling the patient more or less studies that have used perceptual measures, including the
ill. In a prevention paradigm, the variables measured are Mossey and Shapiro25 study, Wilson and Cleary24 state that
behavioral—for example, whether the individual smokes, health perceptions “are among the best predictors of [out-
exercises, or wears a helmet. Positive improvement in a comes from] general medical and mental health services as
well as strong predictors of mortality, even after controlling
for clinical factors.”24
Shifting to client status in each of the three paradigms,
TABLE 2-2 n THE WELLNESS MATRIX
the subject receiving treatment in an illness paradigm is
called the patient, whereas in a prevention paradigm the
ILLNESS PREVENTION WELLNESS subject is a person-at-risk because of the focus on risk
factors and the maintenance of a state of normalcy. In a
View of human Independent Interactive Integrative
wellness paradigm, the client is considered a whole per-
systems
son, to emphasize the multiple systems interacting to
Program orien- Pathogenic Normogenic Salutogenic
produce a state of well-being, and, more important, that a
tation
high-functioning or intact physical dimension, although
Dependent Clinical Behavioral Perceptual
important, is not necessary to achieve a state of well-being
variables
or a high quality of life.
Client status Patient Person at risk Whole person
Consistent with the client status elements, the focus of
Intervention Symptoms Risk factors Dispositions
intervention in an illness paradigm is on symptoms and in
focus
a prevention approach on risk factors. Consistent with a
Intervention Prescription Lifestyle Values clari-
whole-person focus in a wellness approach, the intervention
method modification fication
focuses on dispositions. Defined as a prevailing tendency,
Characteristics of the
Individual
Symptom Values
Amplification Personality Preferences
Motivation
Biological & General

Symptom Functional Overall
Physiological Health
Status Status Quality of
Variables Perceptions Life
Psychological Social and

Social and
Supports Economic
Psychological
Supports
Supports
Characteristics of Nonmedical
the Environment Factors
Figure 2-2 n Health-related quality-of-life conceptual model. (Modified from Wilson IB, Cleary
PD: Linking clinical variables with health-related quality of life. JAMA 273:59–65, 1995.)
mood, or inclination or the tendency to act in a certain man- an illness paradigm is prescribed to correct or improve the
ner under given circumstances, dispositions produce percep- illness. Given that risk factors are the focus in a prevention
tions, which can be measured to indicate a global or psycho- paradigm and the aim is to maintain or return the person-
social assessment of the whole person, given input from at-risk to a normal state, the intervention method that is most
all of the systems. Combined with symptom and risk factor appropriate is lifestyle modification in an attempt to change
assessment, perceptions of the individual provide valuable the behavior that is producing the identified risk. The inter-
additional information about a client that can enhance the vention method in a wellness approach is called values
therapists’ ability to intervene and the success of the inter- clarification, and it is consistent with the focus on disposi-
ventions selected. Table 2-3 lists a few measurement tools tions and measurement of perceptions. The aim of values
that assess client perceptions. clarification is to enhance self-understanding by surfacing
The intervention method used in an illness paradigm is the person’s perceptions of the situation and its impact on
prescriptive. The prescriptive meaning is based on the sys- his or her life. When values clarification can precede inter-
tem affected and symptoms reported. An intervention in vention prescription and lifestyle modification, wellness
TABLE 2-3 n SAMPLE ITEMS FROM PERCEPTUAL MEASUREMENT TOOLS
INSTRUMENT PERCEPTUAL CONSTRUCT SAMPLE ITEMS (RESPONSES)

Short Form 3632 General health perceptions “In general, would you say your health is _______?” (excellent,
very good, good, fair, or poor)
“Compared with 1 year ago, how would you rate your health in
general now?” (much better than 1 year ago, somewhat better,
about the same, somewhat worse, much worse)
Satisfaction with Life Life satisfaction “In most ways my life is close to my ideal”
Scale45 “I am satisfied with my life” (7-point Likert scale from strongly
disagree [1] to strongly agree [7])
Perceived Wellness Perceived wellness “I am always optimistic about my future”
Survey28 “I avoid activities that require me to concentrate” (6-point Likert
scale from very strongly disagree [1] to very strongly agree [6])
NCHS General General well-being “How have you been feeling in general?” (in excellent spirits; in
Well-Being very good spirits; in good spirits mostly; up and down in spirits a
Schedule40 lot; in low spirits mostly; in very low spirits)
“Has your daily life been full of things that were interesting to
you?” (all the time, most of the time, a good bit of the time, some
of the time, a little of the time, none of the time)
Philadelphia Geriatric Morale “Things keep getting worse as I get older”
Center Morale Scale41 “I am as happy now as when I was younger” (yes, no)
Memorial University of Happiness “In the past months have you been feeling on top of the world?”
Newfoundland Scale “As I look back on my life, I am fairly well satisfied” (yes, no,
of Happiness42 don’t know)
will be enhanced because the intervention will be more and physical activity frequency, and perceptual measures
targeted and considerate of the person rather than the health include patient/client self-assessment tools such as global
condition. indicators of health status (“Compared with other people
your age, would you say your health is excellent, good, fair,
MEASUREMENT OF WELLNESS or poor?”)30 and the Short Form 36 (SF-36) Health Status
As a result of the varied way that wellness has been defined Questionnaire31 (see Table 2-3).
and understood, a variety of wellness measures exist. Con- Although some perceptual measures assess only single
sistent with the characteristics of wellness described, a well- system status (e.g., psychological well-being, mental well-
ness measure should reflect the multidimensionality and being), numerous multidimensional perceptual measures
systems orientation of the concept and have a salutogenic exist that can serve as wellness measures. Perceptual con-
focus. In the literature, as well as in daily practice, clinical, structs that have been used as wellness measures include
physiological, behavioral, and perceptual indicators are all general health status,31 subjective well-being,31,32 general
touted as wellness measures. Clinical measures include well-being,33,34 morale,35,36 happiness,37,38 life satisfac-
serum cholesterol level and blood pressure, physiological tion,39-41 hardiness,42,43 and perceived wellness18,44,45 (see
indicators include skinfold measurements and maximum Table 2-3). Refer to Figure 2-3 for the “Perceived Wellness
oxygen uptake, behavioral measures include smoking status Survey” used by many professionals to help conceptualize
Perceived Wellness Survey

The following statements are designed to provide information about your wellness perceptions. Please
carefully and thoughtfully consider each statement, then select the one response option with which you
most agree.
Very Very
Strongly Strongly
Disagree Agree
1. I am always optimistic about my future. 1 2 3 4 5 6

2. There have been times when I felt inferior to most of the people I knew. 1 2 3 4 5 6
3. Members of my family come to me for support. 1 2 3 4 5 6
4. My physical health has restricted me in the past. 1 2 3 4 5 6
5. I believe there is a real purpose for my life. 1 2 3 4 5 6
6. I will always seek out activities that challenge me to think and reason. 1 2 3 4 5 6
7. I rarely count on good things happening to me. 1 2 3 4 5 6
8. In general, I feel confident about my abilities. 1 2 3 4 5 6
9. Sometimes I wonder if my family will really be there for me when 1 2 3 4 5 6
I am in need.
10. My body seems to resist physical illness very well. 1 2 3 4 5 6
11. Life does not hold much future promise for me. 1 2 3 4 5 6
12. I avoid activities which require me to concentrate. 1 2 3 4 5 6
13. I always look on the bright side of things. 1 2 3 4 5 6
14. I sometimes think I am a worthless individual. 1 2 3 4 5 6
15. My friends know they can always confide in me and ask me for advice. 1 2 3 4 5 6
16. My physical health is excellent. 1 2 3 4 5 6
17. Sometimes I don’t understand what life is all about. 1 2 3 4 5 6
18. Generally, I feel pleased with the amount of intellectual stimulation 1 2 3 4 5 6
I receive in my daily life.
19. In the past, I have expected the best. 1 2 3 4 5 6
20. I am uncertain about my ability to do things well in the future. 1 2 3 4 5 6
21. My family has been available to support me in the past. 1 2 3 4 5 6
22. Compared to people I know, my past physical health has been excellent. 1 2 3 4 5 6
23. I feel a sense of mission about my future. 1 2 3 4 5 6
24. The amount of information that I process in a typical day is just 1 2 3 4 5 6
about right for me (i.e., not too much and not too little). 1 2 3 4 5 6
25. In the past, I hardly ever expected things to go my way. 1 2 3 4 5 6
26. I will always be secure with who I am. 1 2 3 4 5 6
27. In the past, I have not always had friends with whom I could share my 1 2 3 4 5 6
joys and sorrows.
28. I expect to always be physically healthy. 1 2 3 4 5 6
29. I have felt in the past that my life was meaningless. 1 2 3 4 5 6
30. In the past, I have generally found intellectual challenges to be 1 2 3 4 5 6
vital to my overall well-being.
31. Things will not work out the way I want them to in the future. 1 2 3 4 5 6
32. In the past, I have felt sure of myself among strangers. 1 2 3 4 5 6
33. My friends will be there for me when I need help. 1 2 3 4 5 6
34. I expect my physical health to get worse. 1 2 3 4 5 6
35. It seems that my life has always had purpose. 1 2 3 4 5 6
36. My life has often seemed void of positive mental stimulation. 1 2 3 4 5 6
Figure 2-3 n The Perceived Wellness Survey.18
the client’s perception of her or his wellness. This survey n Providers who have a high level of confidence in their
was first published in the American Journal of Health knowledge and skill to guide clients to optimize their
Promotion in 1997.18 potential (e.g., achieve greater wellness)49,50
Physical therapists assess perceptions as a part of the n Providers who are role models and who assume the
patient/client history, as recommended in the “Guide to role of facilitator, which will establish a relationship
Physical Therapist Practice.”46 Occupational therapists and an environment in which clients can attain greater
assess perceptions as part of their focus on human perfor- wellness
mance and occupation. Some of the kinds of perceptions It may be most instructive to consider first how a well-
that can be assessed include perceptions of general health ness approach could be adopted with clients who are seem-
status, social support systems, role and social function- ingly healthy or without pathology. As experts in movement
ing, self-efficacy, and functional status in self-care and problems associated with the causes and consequences of
home management activities and work, community, and pathological conditions, physical and occupational thera-
leisure activities. Although a few of these categories are pists should play a significant role in primary and second-
included in overall wellness, such as general health status ary prevention. Indeed, intervention programs designed
and social and role functioning, measuring wellness per- by therapists for patients/clients with pathology generally
ceptions specifically can provide additional and more include instruction in preventive behaviors and activities
complete information about the patient that both the (secondary prevention). Although appropriate and worth-
physical and occupational therapist can use to formulate while, these efforts do not produce the significant outcomes
a plan that can be insightful to the patient/client. There- that primary prevention programs might because they are
fore perceptual tools should be used when measuring applied after the onset of risk, illness, or injury. Contempo-
wellness. rary practice includes a role for the physical and occupa-
tional therapist in primary prevention—that is, interacting
MERGING WELLNESS INTO REHABILITATION with clients to promote health and improve wellness before
Incorporating wellness into rehabilitation requires that the they become patients.
therapist or provider modify the traditional approach used Because individuals without overt disease are typically
to treat patients, which involves changing the focus from unmotivated to seek professional assistance, consideration
illness to wellness, being a role model of wellness, incorpo- must be given to how a provider recruits those without dis-
rating wellness measures into the examination, considering ease. A focus on wellness and health-causing activities is a
the client within his or her system, and offering services powerful solution to this dilemma. In a sports or athletic
beyond the traditional patient-provider relationship. Estab- context, this approach would be considered “performance
lishing a wellness approach also requires that the provider enhancing” and would be marketed to individuals who have
assume the role of a facilitator or partner rather than that of goals and ambitions related to improving athletic perfor-
an authority figure.47 mance in a specific context (e.g., improving 10K time,
When a patient is ill, it is often appropriate for the health increasing cycling distance or speed). In a general wellness
care provider to act as the expert because the patient has context, an appropriate marketing message might be to
limited ability to provide self-care and is relying on the pro- improve quality of life or productivity, or any subjective
vider for information and skills to recover and improve. In a measure that a client deems important. The same knowledge
wellness paradigm the best approach is to believe that the and skills therapists use when intervening to prevent injury,
client knows best in terms of maximizing her or his poten- delay or prevent the progression of disease, or enhance qual-
tial; therefore assuming a partner or facilitator role is more ity of movement are useful in a primary prevention context
appropriate and will create a relationship in which the client in which the goal is to improve quality of life, well-being,
feels empowered to take control. Rather than “making” the and productivity. The difference is the context in which the
client well, the provider can view the client as a whole per- knowledge and skills are applied. Adopting a wellness para-
son within a biopsychosocial context and partner with the digm and a client-centered perspective or focus creates an
client to discover the most appropriate path to achieve well- environment surrounding the client-provider relationship
ness. This approach is consistent with a client-centered that both empowers the client to make meaningful changes
perspective, in comparison to a biomedical approach in and establishes a partnership that is most conducive to
which the emphasis is on impairment and activity limita- change and improvement. The improvement of quality of
tions.48-50 Recent discussions in the literature by a variety of life or wellness requires a consideration of the client as a
health care providers suggest there is an important role for a whole person, by definition, as discussed previously in this
client-centered approach within traditional medical set- chapter. Using a client-centered, whole-person approach to
tings.48-52 Client-centered care requires the following: the design of an intervention program requires a consider-
n Assessment of and consideration for client thoughts, ably different approach than the traditional, biomedical
feelings, and expectations approach of measuring clinical and behavioral variables to
n Education about the client’s condition to enhance the identify impairments and functional loss and creating an
client’s ability to take responsibility for her or his own intervention aimed at ameliorating the impairment.49,50 Sud-
well-being denly clients are more than their diseases, which sends a
n A shift in professional identity from expert advisor to much different message and creates a much different rela-
partner and facilitator tionship between the provider and the client.
n Excellent communication skills, including the use Applying this same approach to individuals with chronic
of language the client can understand and effective health conditions implies that the therapist must attend to
listening skills more than just impairments and activity limitations and their
causes when designing intervention programs and determin- opportunity to keep the client at the focus of the intervention
ing the best approach to adopt with an individual client. It and design interventions in partnership with the client that
requires consideration of issues such as social support given will stand a greater chance of producing positive, meaning-
and received, intellectual curiosity, physical self-esteem, ful outcomes.49 The following examples attempt to illustrate
general self-esteem, optimism, and so forth. Recognition the adoption and application of a wellness paradigm within
of these dimensions of the individual provides a unique neurorehabilitation.
CASE STUDY 2-1 n WELLNESS INTERVENTION CONCEPTS IN NEUROREHABILITATION

The client is a 56-year-old poststroke (cerebrovascular client for the purpose of helping the client achieve his goal.
accident [CVA]) man who expresses a desire to return to The partnership relationship established in this case requires
a pre-CVA hobby, fly fishing. In addition to the physical the therapist to be confident that she or he can identify the
requirements necessary to fly fish, which the therapist would motor control, motor programming, and perceptual and
typically assess and then incorporate into the intervention cognitive aspects of the movement program such as the
plan for the goal of independence in fly fishing, a wellness strength, motion, postural requirements, axial-distal motor
approach requires additional considerations. Recognizing the relationships for fly fishing, and the client’s self-efficacy
client’s desire to fly fish requires the therapist to explore the post-CVA, even though the therapist had never participated
client’s goals and expectations and incorporate them into in or observed the activity. Throughout the process, excellent
the intervention plan, as well as to appreciate the role of fly communication is required between the therapist and client to
fishing in the achievement of well-being for this client. After ensure that expectations are clear and that understanding is
the therapist provides education about living after a CVA, the achieved to establish and meet goals. The task of learning
client is aware that fly fishing is a realistic expectation. To how to fly fish may have also required the therapist to go
understand the biomechanics and physical requirements of beyond the typical boundaries of traditional care, including
fly fishing, the therapist partners with the client to learn the challenging reimbursement limitations, to assist the client in
process of fly fishing, thus gaining from the “expertise” of the his quest for enhanced well-being.
CASE STUDY 2-2

The client is a 25-year-old man with a posttraumatic brain realistic goal and expresses a willingness to assist the client
injury who would like to be able to spend more time with his in achieving his goal. To provide the interventions necessary,
friends. During the discussion about his desires, the therapist, the therapist must understand the physical requirements of
learning that the group plays basketball one or two times per basketball, obtain access to a basket and ball, interact with
week, inquires about the client’s interest in joining the group. the client’s friends, be open to learning about basketball from
The client indicates that he used to play basketball but is con- the “expertise” of the client, and support and motivate the
cerned about his ability to run and produce the movements client. The therapist recognizes that playing basketball with his
necessary to play now. The therapist, in the role of facilitator friends will contribute greatly to the client’s overall well-being
and partner, assures the client that playing basketball is a and is thus a worthwhile goal.
In both cases the therapist functions as the movement spe- References

cialist, also recognizing, however, the role of movement in the To enhance this text and add value for the reader, all refer-
enhancement of emotional and social well-being within a ences are included on the companion Evolve site that ac-
paradigm of overall wellness. The potential contributions this companies this textbook. This online service will, when
approach can make to the overall quality of life of individuals available, provide a link for the reader to a Medline abstract
living with neurological disease are immense and within the for the article cited. There are 52 cited references and other
scope of practice and abilities of the physical and occupational general references for this chapter, with the majority of
therapist. Viewing the client within a larger context than the those articles being evidence-based citations.
narrowly focused physical dimension provides increased
opportunity to have an impact on well-being and quality of life.
CHAPTER 3 Movement Analysis across the Life Span
DALE SCALISE-SMITH, PT, PhD, and DARCY A. UMPHRED, PT, PhD, FAPTA

abnormal movement strategies After reading this chapter, the student or therapist will be able to:
developmental theory 1. Comprehend the complexity and interlocking nature of human development over a
neuroconstructivism lifetime.
nonlinear dynamics 2. Differentiate traditional theories of development from contemporary theories.
normal movement strategies 3. Analyze the differences among various subsystems within the human organism.
stages of motor development 4. Identify elements of physiological changes over a lifetime.
systems theory 5. Analyze normal movement strategies and identify subsystems responsibility for success
of a motor task.
6. Identify normal changes in motor strategies over a lifetime and synthesize differences
between normal movement patterns and pathological movement problems across the
life cycle.
A s physical and occupational therapists assume greater

roles in primary care of patients/clients, they recognize
the importance of a multifactorial approach. To competently
behaviors is critical to examining variables that alter or
impair these same behaviors in other individuals. Kandel
and colleagues5 suggested that “the task of neural science is
evaluate functional movement across the life span, clinicians to understand the mental processes by which we perceive,
must possess knowledge and skills in the development of act, learn, and remember” (p. 3). This view supports the
skilled, refined movement across domains. Only then will interactive and collaborative nature of intrinsic and extrin-
therapists be prepared to perform the necessary evaluative sic systems to accomplish motor learning tasks.
and diagnostic testing and effectively develop and imple- Given the interactive nature of different subsystems, it
ment plans of care aimed at minimizing impairments, main- would seem most effective for clinicians to recognize the
taining or regaining functional skills, and improving quality need for implementing a multidimensional approach when
of life. devising intervention programs. To discuss the interactive
Throughout much of the twentieth century, developmen- nature of these issues, this chapter will (1) briefly provide
tal researchers were heavily focused on skill acquisition a historical perspective of theories of motor development,
from infancy through early childhood.1-3 In the 1970s, as (2) discuss domains (cognition, memory, perception, and
research paradigms directed at motor development and motor so on) associated with life span motor development from
learning evolved, it became evident that changes in motor prenatal development through older adulthood, (3) discuss
skills were not limited to childhood but occurred throughout the impact of various body systems on motor skill acquisi-
the life span. Consequently, the concept of life span devel- tion, and (4) describe behavioral changes that may posi-
opment came to incorporate the prenatal period through tively or negatively influence motor performance across
older adulthood. the life span.
During infancy (birth to 1 year) and childhood (1 year to With the focus of this book on neurological rehabilita-
10 years) acquisition of motor skills coupled with cognitive tion, it is imperative to incorporate the complex and interac-
and perceptual development are the primary foci of devel- tive nature of the physiological, cognitive, and perceptual
opmental researchers and clinicians. As the young child systems. Although readers can read about movement devel-
transitions to adolescence (11 to 19 years) and has the op- opment across the life span, it will not integrate into clinical
portunity to experience motor behaviors across different practice until the therapist understands movements of the
environmental contexts, more complex behaviors emerge. client and how those movements reflect the summation of
Adulthood (20 to 59 years) signals a period when skills are systems interacting to allow that individual to express move-
refined and motor behaviors mature. Only through practice ment, whether that be as a functional task, a written script,
and repetition are skills attained and retained. Individuals or the use of verbal language. Without that link between
who continue to use motor learning strategies into late identified movement and motor control expressing that
adulthood (60 years through death) often report more suc- movement, often the therapist misses critical clues to the
cessful aging than those who do not engage in such motor analysis of the central nervous system (CNS) of the client and
skills.4 how best to provide an environment that provides the oppor-
Identifying mechanisms that enable individuals to be tunity for that individual to improve functional skills and
successful in acquisition and retention of functional motor quality of life. For that reason, figures have been inserted to
33
help the reader understand the differences among movement time.13 Perhaps his greatest contribution to motor develop-
patterns across the life span. ment was the conceptualization of milestones as markers to
evaluate infant behavior.
THEORIES OF DEVELOPMENT Although McGraw was a proponent of ontogenic devel-
Development is often portrayed as a series of stages through opment as one variable influencing motor development, she
which an infant progresses, with a fixed order to the did not believe, as Gesell did, that it was the sole determi-
sequence.6 A developmental theory may be characterized as nant.15 Rather, McGraw attempted to explain the emergence
a systematic statement of principles and generalizations of motor behaviors through environmental influences as
that provides a coherent framework for studying develop- well as CNS maturation.2 She examined the temporal and
ment. Historically, development was thought to be linear, qualitative aspects of motor skill acquisition through her
occurring in an invariant sequence and resulting in behav- study of Jimmy and Johnny,16 a study of twin brothers in
ioral changes that are direct reflections of the maturation of which one twin was provided an exercise program, whereas
anatomical and physiological systems.7,8 Development is no intervention was afforded the other twin. She found tem-
generally examined in terms of quantitative and qualitative poral and qualitative differences in the boys’ acquisition of
change. Although it is universally accepted that acquisition motor skills and attributed differences in acquisition of these
of developmental skills is not reversible, the underlying behaviors to disparities in practice opportunities.
principles surrounding the emergence of these behaviors McGraw believed that the acquisition of the movement
has evolved over the past 50 to 75 years. (process) is as important as when (chronological time
Early developmental theorists used neuromaturational frame) the behavior is acquired (the outcome). She further
models of CNS organization as the framework for concep- elaborated that, within the constraints imposed by the devel-
tualizing development.1,2 These researchers provided elab- oping CNS, a rich and challenging environment can and
orate descriptions of posture acquisition and a blueprint does facilitate temporal efficiency in acquisition of motor
delineating skill development. Research focused on the behaviors. And finally, she proposed that practicing motor
emergence of cognitive and affective behaviors and ignored skills influences emergence of the same behavior.
the processes and mechanisms involved in acquiring motor Sufficient evidence exists to support the premise that
skills.9 Several investigators attributed developmental although some predetermined processes occur at relatively
changes to intrinsic variables such as maturation of the similar points in development, not all motor behaviors emerge
CNS, whereas others associated changes with extrinsic at the same biological, chronological, or psychological age in
variables involving the environment.1,2,10,11 every individual. Although motor milestones provide infor-
During the 1930s and 1940s, Arnold Gesell and Myrtle mation regarding outcome, no information can be derived
McGraw led a cadre of avant-garde researchers exploring about the process of attaining motor skills from those specific
the field of infant motor development. Gesell1,12 described milestones. Perhaps a more realistic explanation may be that
the normative time frame for when behaviors emerge, and emergence of new skills occurs out of a need to solve specific
McGraw2 examined the underlying mechanisms responsi- problems within the environment. Working within this con-
ble for the emergence of these behaviors. The underlying text, it is evident that traditional theories of development and
premise, the foundation for their elaborate descriptions of maturation fail to adequately encapsulate the innate variabil-
motor development, was based on maturational processes ity in human development.9,10
in the CNS. Within the last few decades, researchers have used more
Gesell, a pioneer in developmental research, was a pro- current theories of development when designing studies
ponent of the theory that nature drives development.13 He involving infants and young children.17-19 These investiga-
proposed that growth is a process so complicated and so tors examined the process of skill acquisition rather than
sensitive that intrinsic factors are solely responsible for using traditional methods that assess outcome as a measure
influencing development. He used the evolutional thinking of motor development.20
of Darwin and Coghill to explain changes in motor behav- Although early pioneers in developmental research
iors. Coghill,14 in his work with salamander embryos, described development as linear, uniform, and sequential,
reported that motor behaviors, like swimming, emerge in an Thelan and Smith21 depict development as “messy,” “fluid,”
orderly sequence as connections of specific neural struc- context-sensitive, and nonlinear. Linear and nonlinear dy-
tures appear. Coghill concluded from his observations of namics are derived from mathematics. Linear dynamics is
emergence of behaviors in the salamanders that human described by the proportional relationship of the initial condi-
infant motor behaviors appear in a predictable sequence tion to the outcome, whereas no such proportional relation-
and at predictable chronological ages. ship exists in nonlinear dynamics. Nonlinearity is used to
With Coghill’s research as the foundation for his think- describe complex systems, in this case biological or more
ing, Gesell embraced the concept of a hierarchical organiza- specifically human systems.22 Within these complex systems
tion of the CNS. He believed that the emergence of motor exists a level of unpredictability, given the interactive and in-
behaviors was contingent on maturation in the CNS and terdependent nature of biological systems.
concluded that only after the emergence of higher-level Thelan and Smith21 suggested that, although traditional
neural structures would complex motor behaviors appear. theories of development support the premise that behaviors
Within this constrained theoretical perspective, extrinsic or emerge in accordance with a relatively fixed temporal
environmental stimuli, human or otherwise, were thought to sequence, an organism may exhibit “precocial” abilities
have little impact on the appearance of motor behaviors. when the context is altered and the behavior emerges earlier
Gesell concluded that infant development is preprogrammed than expected. These authors stated that immature systems
and linear, emerging at predetermined stages or periods in exhibit behaviors that are variable and easily disrupted.
CHAPTER 3 n Movement Analysis across the Life Span 35
Although development of some organisms in a controlled are the result of the dynamic interplay among elements of
laboratory environment may reflect more traditional per- the system.26
ceptions of development, outside, in a more naturalistic Embedded in the general systems theory is nonlinear
environment, development is more likely to be flexible, dynamics, a concept in which behaviors are not described as
fluid, and tentative. Thelan and Smith also found that fac- the sum of their parts. Thus within a nonlinear model, a
tors most likely to have an impact on performance are the mathematical model is derived.24,27 Characterized within
“immediacy of the situation” and the “task at hand” rather this model is the notion that systems may change in a sud-
than “rules” of the performance. Given this perspective, den, discontinuous fashion. During development a small
Thelan and Smith21 identified six goals as essential to increase or decrease in one parameter leads to changes in
developmental theory. These goals are as follows: the behavior. This abrupt change, identified as a bifurcation,
1. To understand the origins of novelty. causes the system to move out of its previous state and
2. To reconcile global regularities with local variability, toward a new state of being.
complexity, and context-specificity. Throughout development, periods of rapid differentiation
3. To integrate developmental data at many levels of or change occur when an organism is most easily altered or
explanation. modified. These periods were identified by Scott28 as “criti-
4. To improve a biologically plausible yet non-reductionist cal periods.” Physiological systems are most vulnerable
account of the development of behavior. during these periods and may be seriously affected by both
5. To understand how local processes lead to global intrinsic and extrinsic factors acting on the system. These
outcomes. periods occur at different times for different body systems.
6. To establish a theoretical basis for generating and Understanding systems theory and the concept of critical
interpreting empirical research.21 (p. xviii) periods is crucial to all aspects of motor development.
Thelan and Smith urged developmental researchers to As scientists began to revisit theories of motor develop-
devise paradigms that attempt to explain development in ment, they discarded some of the traditional theories and
terms of diversity, flexibility, asynchrony, and “the ability of embraced contemporary concepts of nonlinear dynam-
even young organisms to reorganize their behavior around ics.24,25,27 Proponents of nonlinear dynamics contend that
context and task” (p. 18).21 modifications in motor behaviors are the result of dynamic
Contemporary theorists inferred that developmental interactions among the musculoskeletal, peripheral and
changes are nonlinear and emergent and may be the result of central neuromuscular, cardiovascular and pulmonary, and
the interactive effects of intrinsic and extrinsic variables. cognitive and emotional systems.26 These interactive, mul-
This divergence from traditional thinking compelled avant- tidimensional elements are vulnerable to changes in orga-
garde scholars to propose new theories.21,23,24 Investigators nizational and behavioral abilities (system) over time.28,29
described behaviors as complex, interactive, cooperative, Some theorists propose that as skills are acquired and
and reflects an ability to organize and regroup around task organizational or behavioral changes occur, the system is
and context, rather than conforming to a rigid structure and driven to identify the most efficient and effective strategy
rule-driven hierarchy, as many earlier cognitive researchers to produce motor behavior(s).28,29 Yet others purport that
believed.21,24 Contemporary theorists exploit technology to variability implies that typical healthy individuals may
derive a model to explain variability and flexibility in devel- use a variety of strategies to produce the same behavioral
oping, mature, and aging populations.24 outcome and that variability is an indication of the indi-
viduals’ flexibility in responding to unpredictable pertur-
GENERAL SYSTEMS THEORY bations.24,30 Implicit in nonlinear dynamics is the concept
Systems theory, first described by von Bertalanffy in 1936, of critical periods in development.28,29,31 Investigators sug-
was not discussed in great detail until 1948. In 1954 von gested that interventions imposed during a critical period
Bertalanffy and colleagues from three other professions met may more easily positively or negatively modify the
to discuss systems movement.25 Theorists then applied sys- behavior. Recognizing the crucial role systems theory
tems theory to a variety of human and nonhuman systems. and critical periods play in development is vital to com-
As theorists became acquainted with systems theory, they prehending how developmental skills emerge. The multi-
became more receptive to alternate theoretical proposals of factorial nature of nonlinear dynamics illustrates the
growth and development in living organisms. complexity of development and the difficulty in identify-
Systems theory may be applied as a transdisciplinary ing the appropriate variables that influence motor skill
model examining relationships of structures as a whole.26 development.
“The notion of a system may be seen as simply a more self- With use of concepts previously described, it is reason-
conscious and generic term for the dynamic interrelatedness able to expect that a small change in any subsystem may
of components”26; von Bertalanffy proposed this theory to result in a large change in a motor behavior. This is evident
more adequately describe biological systems, investigate in work by Thelan and colleagues examining stepping in
principles common to all complex organisms, and develop infants 8 weeks of age.29,31-34 They reported that introduc-
models that can be used to describe them.26 ing a small change in one element of the system, identified
Principles that embody general systems theory include as a small weight applied to an infant’s leg, resulted in the
nonsummative wholeness, self-regulation, equifinality, and infant being unable to step. The authors deduced that small
self-organization.26 Contrary to systems theory in disci- changes in one subsystem, in this case the musculoskeletal
plines such as traditional physics, in which systems are said system, may result in a change in the outcome. This lends
to be closed, von Bertalanffy suggested that biological sys- support to the hypothesis that modifying one aspect of
tems are open and modifiable and that changes in the system a multicomponent system, especially during a critical
period, may cause the system to evolve into an entirely system include early closure of the epiphyseal plates, result-
new behavior. ing in significantly smaller stature. Conversely, these young
Periods of rapid differentiation, although often observed women’s reproductive cycles are significantly skewed. Women
during early life, have also been observed across the life would also have menopause and aging issues associated with
span. Changes in anthropometric measures, such as weight hormonal changes earlier than other women of the same
gain during pregnancy, influence coordination between chronological age. Although no consistent method has been
limbs and cause emergence of a different gait pattern. established for measuring biological age, there is general
Changes in one system, in this case the endocrine system, agreement that a wide variability of biological aging exists
result in increased ligamentous laxity at the pelvis and also and that a number of factors contribute to accelerated or
contribute to gait alterations. decelerated biological aging.
Menopause may be another critical period. During meno-
pause, decreases in hormone production are thought to lead Aging
to osteoporosis and cardiac disease.35 Examples described “Aging refers to the time-sequential deterioration that occurs
previously provide evidence across the life span that the in most animals including weakness, increased susceptibility
dynamic interplay within a system and among systems may to disease and adverse environmental conditions, loss of
significantly influence emergence and disappearance of mobility and agility, and age-related physiological changes”
behaviors. (p. 9).41 Although Goldsmith’s description of aging is typi-
Although research in the beginning of the twentieth cen- cally viewed as an inevitable fact of life, there is scientific
tury was heavily focused on development of the very young, evidence and theoretical support for the idea that age-related
studies during the latter part of the century were directed changes will eventually be more medically treatable than
toward research on aging. Technological advances in medi- previously thought.38,41,42
cine have dramatically increased life expectancies. During Scientists are hesitant to attribute a decline in functional
the twentieth century, the number of individuals in the movement in older adults to a decline in physiological sys-
United States older than 65 years old grew from 3 million tems or to diminished opportunities for practice or condi-
to 35 million.36 Perhaps the most significant statistic is that tioning.4 Rowe and Kahn reported that “with advancing age
the oldest old grew from 100,000 in 1900 to 4.2 million in the relative contribution of genetic factors decreases and [of]
2000.36 By 2011 the Baby Boomer generation will begin the nongenetic factors increases” (p. 446).4 Age-related fac-
turning 65 years old, and the number of older individuals tors that are modifiable may be used to identify individuals
will increase sharply between 2010 and 2030.36 By 2030, who may or may not age successfully. For instance, lifestyle
Americans over the age of 65 years will represent nearly choices, including diet, physical activity, and other health
20% of the population, and by 2050 the number of individu- habits, and behavioral and social factors have a potent effect
als over the age of 85 years could grow to 21 million.36 and accelerate or decelerate aging. Evidence to support this
Given this incredible demographic transformation and that was initially derived from a 10-year study conducted by
current policymakers are, in large part, the generation di- Rowe and Kahn.43 The authors identified three critical fac-
rectly affected by these statistics, a significant paradigm tors that contribute to aging successfully: avoidance and
shift in funded research has evolved over the past quarter absence of disease, maintaining cognitive and physical func-
century. “As such, aging and death are inseparable partners tioning, and “sustained engagement in life.”43 Recently
to growth and development”37 (p. 32). Recognizing that a researchers suggested that Rowe and Kahn’s classification
critical mass of Americans are entering older adulthood, of successful aging is too restrictive and may lead to classi-
terminology that operationally defines and is then applied fying individuals with relatively minor health problems as
consistently when referring to the aging population or an unhealthy. McLaughlin and colleagues44 suggested that a
individual is imperative. critical variable in defining successful aging is first identify-
ing what the goal is for measuring successful aging. Only
Biological and Chronological Age then can researchers determine how best to define and mea-
Age can be described in terms of chronological age and bio- sure successful aging. Although controversy exists regard-
logical age.38 Chronological age is the period of time that a ing defining successful aging, factors that contribute to
person has been alive, beginning at birth. In infants it is successful aging hinge on higher levels of physical activity,
measured in days, weeks, or months, whereas in adults it is increased social interactions, and positive perception of
expressed in terms of years and at times decades. health, as well as no smoking, chronic diseases (arthritis,
Although chronological age is measured in terms of tem- diabetes), or impaired cognition.45,46 Consequently, develop-
poral sequencing, biological age is related more to functioning healthy behaviors early in life may be critical to maintain-
ing and physiological aging of organ systems.39 For example, ing good health and may play a significant role in successful
a triathlete may have biologically younger cardiovascular aging. Factors associated with aging are generally identified
and pulmonary systems than same-age peers who do not as either age related or age dependent. Age-dependent changes
perform high-level aerobic activities. Another example might within organ systems are observed in individuals at a similar
be a child who underwent precocious puberty. Precocious age, whereas age-related changes may be accelerated or
puberty, identified as puberty earlier than 8 years of age decelerated in same-age individuals on the basis of intrinsic
in girls and 9.5 years in boys, results in acceleration in a or extrinsic factors related to lifestyle. Just as variables
biological system before same-chronological-age peers.40 associated with lifestyle (extrinsic factors) influence aging,
Physiological changes include elevated hormonal levels, genetics (intrinsic factors) also play a significant role. From
which would then stimulate development of breast tissue and a genetic perspective, structural and functional changes are
early menstruation in girls. Changes in the musculoskeletal generally thought to be a consequence of aging and are
therefore predictable and consistent across physiological Human exposure to intrinsic and extrinsic free radicals
systems. Variables thought to influence the genetic potential causes large numbers of reactive oxygen molecules to inter-
for longevity include environmental factors such as toxins, act with DNA, leading to mutations thought to be the cause
radiation, and oxygen free radicals. Free radicals are highly of a variety of diseases, including cancer, atherosclerosis,
reactive molecules produced as cells turn food and oxygen amyloidosis, age-related immune deficiency, senile demen-
into energy.47 In summary, the use of biological age rather tia, and hypertension. Although some scientists suggest that
than chronological age may be a more accurate reflection of aging has many factors that can accelerate or decelerate the
an individual’s true age. process, other scientists suggest a much simpler, prepro-
grammed theory, known as the Hayflick limit.38,51-53
Theories of Aging Hayflick and Moorhead53,54 proposed that there is a finite
Throughout the twentieth century, the average life expec- number of times that a normal cell is capable of dividing.
tancy of individuals living in the United States increased. Current thinking is that cells are capable of dividing up to
The second half of the twentieth century signaled a shift in 50 times. Cell division is recognized as one way in which
the focus of human development research, from infant and cells age and, after attaining the maximum number of divi-
child development to older adult development. sions, finally die.
Scientists view aging as a progressive accumulation of The factor thought to limit a cell’s ability to divide infi-
changes over time that increases the probability of disease nitely is the presence of telomeres. Telomeres are minute
and death.48 Given that portrayal of aging, researchers have units at the end of the DNA chain.53 Each time a cell
proposed myriad hypotheses regarding aging. Aging theo- divides a small amount of the telomere is used in the pro-
ries evolved because there is no single factor or mechanism cess. Eventually, when cells have exhausted the supply of
responsible for physiological aging.38 Biological aging telomeres available, the cell is unable to divide and cell
theories, similar to developmental theories, are attributed death ensues.
to complex, underlying mechanisms.38,41,42,49 Although Telomerase, a substance that can lengthen telomeres, is
theorists attempt to classify aging theories, these theories available in human cells. Typically, telomerase is switched
are rarely mutually exclusive. Some theories were formu- off in all cells except the reproductive cells. The availability
lated around control of physiological functioning, others of telomerase in reproductive cells allows for many more
around cellular changes, and still others around genetic divisions than previously observed in the Hayflick limit. In
causes. addition to the presence of telomerase in reproductive cells,
Neuroendocrine theory is based on the premise that hor- scientists have also discovered that telomerase remains
mones play a significant role in aging.42,49 Hormones are active in cancer cells. Both reproductive and cancer cells
vital to repairing and regulating bodily functions. Hormone divide well beyond the 50-division limit. Consequently,
production decreases significantly during aging and limits scientists are now working toward activating telomerase in
the body’s ability to repair and regulate itself as effectively. all cells to slow or stop aging. If scientists are successful
Although hormonal decline is one plausible explanation for in activating telomerase in other cells, it may stimulate skin
age-related changes, it does not account for all changes. cell regrowth for burn patients and cure diseases that result
Harman50 proposed the free radical theory on the basis of his from failure of aging cells to divide, as in macular degen-
investigations that examined the effects of radioactive mate- eration or Hutchinson-Guilford progeria syndrome.55,56 The
rials on human tissue.50 downside of this is that scientists may have a difficult time
Harman reported that when human tissue is exposed controlling the telomerase and in fact may see more uncon-
to radiation, a byproduct is formed. He identified the trolled cell growth—cancer—one of the greatest threats to
byproduct, an unstable compound, as a free radical. Over prolonged existence.
time, human tissue with free radicals showed evidence Although many aging theories are directed at mecha-
of biological defects consistent with accelerated aging. nisms that negatively influence aging, other theories are
Harman postulated that accumulation of free radicals in focused on factors that have a positive impact on aging
human tissue may also occur as a part of the normal aging processes. One such process is the caloric restriction the-
process. This became known as the free radical theory of ory.50,52,53,57 Liang and colleagues,57 with use of several
aging.50-52 genetic mouse models, investigated the impact of dietary
Free radicals are highly reactive molecules that damage control on the life span. The authors reported that the mice
proteins, lipids, and deoxyribonucleic acid (DNA). In some did, in fact, have their life spans extended when their di-
instances the free radicals combine with enzymes and turn etary intake was controlled. Although these findings are
into water and a harmless form of oxygen that moves harm- potentially significant, given the small sample size and
lessly through the cells.51 In other instances the oxygen model examined, these data were not generalizable to all
binds with intrinsic or extrinsic sources that influence the species. The researchers suggested that these preliminary
aging process. data provide a foundation for scientists to examine whether
Scientists have suggested several different ways that free dietary control will extend the life span in humans as it did
radicals influence aging through intrinsic and extrinsic in the mouse models.
mechanisms.51,52 An example of an intrinsic mechanism Although a large body of literature exists examining the
would be chronic infections that extend phagocytic activity underlying mechanisms associated with aging, it seems
and expose tissues to oxidants, creating cumulative oxida- inconceivable that any one mechanism is responsible for age-
tive changes in collagen and elastin. Extrinsic sources of related changes. More likely is that aging may be attributed
free radicals include environmental toxins—for example, to multiple factors, including lifestyle choices, in combina-
industrial waste and cigarette smoke. tion with the physiological and environmental factors.58
Garilov and Gavrilova58 conducted an exhaustive review of Structural and functional differences in the musculo
aging theories and concluded that additional research is skeletal system of a child versus an adult are attributed to
necessary to further elaborate and validate existing aging the presence and predominance of muscle fiber types. For
theories and dispel unlikely theories. example, infant muscles are composed predominantly of
In summary, scientists are unsure how much of the type I (slow-twitch) fibers, whereas adult muscles contain
decline in motor behaviors in older adults is attributable to types I and II (fast-twitch) fibers. Behaviorally, infant
true decline in physiological systems, how much is attribut- movements are characterized predominantly by postural
able to expected decline, how much to a decreased ability movements. The capacity to produce a greater repertoire
to perform skilled behaviors under variable conditions, and of movements, including rapid or ballistic movements,
how much to decreased practice or conditioning.59-62 This emerges later in development.
suggests that physical or occupational therapy intervention Distinct differences also exist in temporal differentiation
may provide older adults with strategies to positively influ- of the muscular systems of males and females of the same
ence successful aging rather than being applied only after chronological age. Through adolescence, boys show evidence
a negative outcome of aging is realized or a neurological of a significantly greater increase in fiber size compared with
insult has occurred. girls.67 In addition, differences exist in the age at which the
The exogenous and endogenous variables of aging are number of muscle fibers dramatically increases. Girls report-
thought to be interrelated and provide an expansive description edly have a steady increase in the muscle fibers from 3.5 to
of the deleterious changes at the cellular, organ, and system 10 years of age. In contrast, boys have two periods of rapid
level that accompany both aging and many age-associated differentiation in the number of muscle fibers. The first period
diseases. The accumulation of damage is in DNA, proteins, occurs from birth until 2 years of age and the second from
membranes, and organelles, as well as the formation of insol- ages 10 to 16 years.67 Although the pace slows considerably,
uble protein aggregates. Many organ systems, such as the muscle fiber development continues in men and women well
cardiovascular system, the brain, and the eye, are not pro- into middle adulthood.
grammed for indefinite survival. Consequently, the inability to Age-related changes evident in the musculoskeletal
maintain the integrity of tissues and organs is the end result of system include decreased fiber size, loss of muscle mass,
the multidimensional aspect of aging. denervation of muscle fibers, decline of total muscle fiber
number, and decreased quantity of fast-twitch fibers.68-70
PHYSIOLOGICAL CHANGES IN BODY Muscle mass decreases beginning at around age 50 years,
SYSTEMS ACROSS THE LIFE SPAN and by age 80 years up to 40% of muscle mass has been
Organ systems undergo critical physiological changes across lost.71 Muscle force production likewise decreases at a rate
the life span. These alterations are observed most often during of about 30% between 60 and 90 years of age. Additional
periods of rapid differentiation. Applying concepts of dynamic musculoskeletal changes documented in older adults include
systems theory to life span development may help to explain decreased tensile strength in bone, reduced joint flexibility,
how small changes in biologic systems have a significant and limited speed of movement. Decreased muscle mass in
impact on the individual as a whole. a person older than 60 years may be attributed to decreased
Examining interactions among variables within different size, fewer type II muscle fibers, and an increase in fat infil-
body systems may provide insight into when one system tration into the muscle tissue.72,73 Clinically these factors
may play a greater or lesser role in acquisition, retention, or manifest as reduced muscle force production during high-
deterioration of functional motor behaviors. The next sec- velocity movements.
tion will examine how different systems develop and their Currently, scientists are examining the premise that, as an
contribution to functional movement. individual ages, muscular changes are more likely attribut-
able to decreased motor activity levels and are age related
Musculoskeletal System rather than being solely age dependent.69,72 Acknowledging
Structural and functional adaptations in the musculoskeletal that investigators had previously found that muscle power
system are evident across the life span. The musculoskeletal deteriorates more quickly with age, scientists set out to mea-
system provides a structural framework for the body to sure training effects in older adults.61,74 These investigators
move and serves as protection for the internal organs. concluded that with training older adults were capable of
Skeletal muscle tissue first appears during the fifth week improving strength, power, and endurance.
of embryonic development and continues to develop into The skeletal system, similar to the muscular system, expe-
adulthood.53,63 During this early period of embryonic devel- riences periods of growth, stability, and degeneration. The
opment, the differentiation of musculoskeletal system is immature skeletal system is composed primarily of preosse-
rapid: during the fifth week of embryonic life the limb buds ous cartilage and physes (growth plates).75 More simply,
appear, by the seventh week muscle tissue is present in the bone in infants and young children is flexible, porous (lower
limbs, and limb movements emerge as early as the eighth mineral count), and strong with a thick periosteum.75,76 Given
week of prenatal life.40,64,65 these properties of immature bone, a child is less likely to
Whereas many of the structural aspects of the musculo- have a fracture because the periosteum is strong and conse-
skeletal system are formed prenatally, muscle and bone quently the bones absorb more energy before the break point
continue to grow into adulthood. Motor skill acquisition in- is reached. In addition, if a fracture does occur, healing is
volves considerable variability among young children from usually quicker because callus is formed faster and in greater
age 5 months through 3 years. During this time, the rate of amounts in children than in adults.
growth of muscle tissue is reportedly two times faster than A primary difference between the child’s and the adult’s
that of bone.66 skeletal system is the presence of the growth plate complex
in children. Whereas primary ossification occurs prena- increasing until the individual approaches adolescence, and
tally, secondary ossification is not complete until the child then gradually decreasing. Exceptions may be seen in ath-
reaches skeletal maturity, generally at age 14 years in girls letes, dancers, and other individuals involved in activities
and 16 years in boys.76,77 that incorporate flexibility training. Loss of flexibility as a
Even after bones have attained their full length, they con- consequence of age may have a negative impact on func-
tinue to grow on the surface. This is termed appositional tional independence in older adults. Flexibility is thought to
growth and continues throughout most of life. During child- be directly proportional to the amount, frequency, and vari-
hood and adolescence, new bone growth exceeds bone resorp- ability of motor activities performed. As activity increases,
tion and bone density increases. Until age 30 years, bone so does flexibility. Conversely, as individuals exhibit de-
density increases in most individuals, and bone growth and creased levels of motor activity, often associated with age,
reabsorption remain stable through middle adulthood. Later flexibility decreases.78
in adulthood, resorption exceeds new bone growth and bone By age 70 years, flexibility is thought to have de-
density declines.78 creased by 25% to 30%.70 Although this was purported
Women exhibit more loss of bone mass than men do.79 to be age dependent, it may be more likely that it is age
Decreased bone density in women is generally attributed related.69 Regularly performing exercise directed toward
to differences in the types and levels of hormones present. improving strength and flexibility can reverse the effects
Although the difference is most significant during meno- of inactivity for most individuals, even those older than
pause, premenopausal women still lose bone density at a 90 years of age.69,81
higher rate than their male peers do. Although it may take longer for older individuals to
Osteopenia is the presence of a less-than-normal amount regain strength or flexibility than a young adult or child, mus-
of bone and, if not treated, may result in osteoporosis. Pro- culoskeletal tissue is modifiable throughout life. Modifying
gressive loss of bone density, observed into older adulthood, the strength and flexibility of an older adult requires that other
is commonly identified as osteoporosis. Osteoporosis is bodily systems be capable of modifying performance levels to
more common in women than in men and is a major cause meet the increased needs of the musculoskeletal system.
of fractures and postural changes in both sexes.80 As scientists continue to examine functional changes
Overall, much of the growth in the musculoskeletal sys- across different systems as a consequence of age, physical
tem is related to demands placed on the system. Intrinsic and occupational therapists must educate individuals regard-
and extrinsic forces imposed on the musculoskeletal sys- ing the importance of embracing a physically active lifestyle
tems of typically and atypically developing children may and methods to enhance quality of life at each stage in an
lead to structural and functional differences in their respec- individual’s life (see Chapter 2). Although all systems con-
tive skeletal structures. Consequently, temporal sequenc- tribute to an individual’s health and wellness across the life
ing, acquisition, and characteristics of motor behaviors span, the cardiovascular and pulmonary systems play a key
emerge differently in typically and atypically developing role (see Chapter 30).
children. Similarly, age-related changes in older adulthood
may be accelerated in direct proportion to decreased levels Cardiovascular and Pulmonary Systems
of activity.81 Older adults who maintain more active life- The cardiovascular system is composed of the heart, lungs,
styles and place greater physical demands on their muscu- and associated vascular complex. It is responsible for pump-
loskeletal systems are more likely to have an improved ing blood through the coronary, pulmonary, cerebral, and
bone density and muscle mass than their peers who are not systemic circulations, with the goal of perfusing all bodily
as active.82 tissues for the delivery of oxygen and vital nutrients and
Sarcopenia, the age-related loss of muscle mass, affects picking up waste products for elimination. The pulmonary
strength, power, and functional independence in older system is responsible for oxygen transport, gas exchange,
adults.72,82 Although these changes are observed in many and removal of airborne pollutants that may enter during
older adults, the degree of the muscular changes varies.70 respiration (see Chapter 30).
Researchers examining sarcopenia in older adults reported The interdependent nature of the cardiovascular and pul-
that men are affected more by sarcopenia than women monary systems is evident in the fact that, each minute, all
are.72,83 In fact, men with sarcopenia manifest four times the of the body’s blood travels through the lungs before being
rate of activity limitations than do men with a normal mus- returned to the left side of the heart for ejection into the
cle mass. Changes in the cross-sectional area of muscles systemic circulation.84 Because of this relationship, changes
directly affect the force production of a given muscle; con- in heart function can dramatically affect lung function, and
sequently, as the cross-section of a muscle diminishes, its vice versa. In addition, these two systems are connected as
ability to produce force decreases. As an individual ages the part of a larger closed pressure-volume loop through the
number and size of the muscle fibers decrease, resulting in a peripheral circulatory structures. Likewise, any alteration in
reduction in strength.80 Although this is true in all muscles, the function of the peripheral vessels will affect both the
the impact is greater on muscles of the lower extremities heart and lungs, and vice versa.
than in those of the upper extremities.72 The function and homeostasis of the cardiovascular,
Although strength is critical to musculoskeletal function, pulmonary, and peripheral vascular systems are influenced
flexibility is equally as important. Flexibility incorporates by both internal and external forces.84 Internal mechanisms
joint motion and the extensibility of the tissues that cross of control are based on the autonomic nervous system, the
the joint. The degree of flexibility changes across the life relative health of the anatomic structures involved, the
span as a direct result of aging and activity level.10 Changes growth and development of the structures, and the behav-
in flexibility are evident throughout life: limited at birth, ioral and emotional adaptations of a particular individual.
All those internal mechanisms are subject to changes with Structurally, the heart doubles in size by year 1, and its
growth and development, aging, and the unique life experi- size increases fourfold by year 5. Many of the changes as-
ences of an individual. Growth and development primarily sociated with size are complete by the time the child has
affect the physics of the system by altering volumes, reached maturity. Recall that cardiac output (CO) is equal
lengths, smooth and myocardial muscular tension, and to stroke volume times heart rate. As the size of the heart
physiological capacitance within the system to support the increases (increasing the volume capacity for each stroke),
growing body. Numerous effects of aging have an impact the heartbeat decreases and the blood pressure increases.89
on the adaptability of the system. Behavioral and emotional Heart rate in a newborn infant is generally 120 to 200 beats
responses influence both autonomic and volitional cardio- per minute (bpm), 80 bpm by 6 years of age, and 70 bpm
vascular and pulmonary reactions to stress. External forces by 10 years of age.87,90 Systolic blood pressure (defined as
include movement environment and activity level, which maximal pressure on the artery during left ventricular con-
alter the gravitational forces on the closed pressure-volume traction or systole) is 40 to 75 mm Hg at birth and in-
system. An increased activity level causes exercise stress, creases to 95 mm Hg by 5 years of age.87 Blood pressure
which requires an altered demand for oxygen and nutrients continues to rise into adolescence. The capacity to main-
to the structures providing the work. Finally, emotional tain exercise for longer periods and greater intensities
stress needs to be considered as an external factor. Behav- increases through early childhood. Although cardiovascu-
ioral responses to stress can affect functional movement lar disease is generally associated with adults, children as
and cause maladaptive coping mechanisms on any or all young as 5 years of age may show signs of or be at risk for
systems. As with anything, the age, cognitive status, and cardiovascular disease if they do not engage in regular
relative health of an individual will dictate the potential aerobic activity.87,91
success of these endeavors. Development of the pulmonary system occurs late in
Because oxygen transport and exchange are the primary prenatal and early postnatal life.90 As the lungs increase
requirements for sustaining life, efforts toward maximizing in size, tripling in weight during year 1, the capacity and
the efficiency of the cardiovascular and pulmonary systems efficiency increase while the respiratory rate decreases.90
represent a fundamental component of therapeutic practice. Although the vital capacity of a 5-year-old child is 20% of an
It is critical that no matter where a patient falls in the life adult’s, this is not usually a limiting factor during exercise.89
span, strategies for screening, prevention, and rehabilitation Overall, aerobic capacity increases during childhood and is
of the cardiovascular and pulmonary systems be incorpo- slightly higher in boys than in girls. The overall work capac-
rated into a comprehensive plan to promote optimal mobility ity of children increases most dramatically from 6 through
and independence.85 It is essential for therapists to keep in 12 years of age.89 Peak oxygen consumption is achieved
mind that all interventions have a direct or indirect impact early in adulthood and changes in direct relation to activity
on these systems and that it is their responsibility to monitor levels. Lungs of an average adult at rest take in about 250 mL
and manage those responses to maintain safety. of oxygen every minute and excrete about 200 mL of carbon
A detailed understanding of the anatomy of the heart, dioxide.92
lungs, and vessels, as well as the physiology and interrela- As activity decreases in older adulthood, so do the
tionship of the organs involved, is essential to the practice of structural and functional capacities of the cardiovascular
both physical and occupational therapy. Refer to Chapter 30 and pulmonary systems. Many of these changes are a result
for additional information. For pediatric therapists, the of decreased elasticity of the tissues, decreased efficiency
added knowledge of normal growth and development of of the structures, and decreased ability to increase work-
these structures is critical. load. CO decreases approximately 0.7% per year after
From weeks 3 to 8 of fetal life, the cardiac structures age 20 years so that by age 75 years the CO is 3.5 L/min,
are formed.63,64,86 All other structures of the cardiovascu- down from 5 L/min at age 20 years.92 Functional changes
lar system are fully developed and functional shortly after include a decrease in the overall maximum heart rate from
birth. Although the left and right ventricles are of similar 2001 bpm through young adulthood to 170 bpm by age
size at birth, by 2 months of age the muscle wall of the left 65 years.87 Older adults have less elastic vessels, and resis-
ventricle is thicker than that of the right ventricle.87 This tance to the blood volume increases. Consequently, older
is attributable to the fact that the left ventricle is respon- adults reach peak CO at lower levels than do younger
sible for pumping blood to the whole body, requiring a individuals. These cardiovascular changes may be com-
higher internal pressure and contractile force, whereas the pounded by inactivity, resulting in decreased capacity to
right ventricle is responsible for pumping blood only to perform activities that raise metabolic demands and increase
the lungs, a relatively low-pressure function in a healthy the requirement for oxygen transport.93 The impact of these
individual. normal aging responses, however, can be reduced through
It bears mentioning that the heart’s function begets structured aerobic and anaerobic activities. Conversely,
structure. Therefore if function becomes impaired, the physiological performance of the cardiovascular and pul-
structure is likely to adaptively change. For example, if the monary systems improves in response to growth and
resistance in the vascular system from the right ventricle to development.
the lungs becomes increased, the right ventricle must pump Throughout life, performance of motor activities and
harder, with a greater volume of blood, to overcome the activities of daily living (ADLs) is highly dependent on the
resistance.88 Over time, this will increase the size of the integrity of an individual’s cardiopulmonary and cardiovas-
ventricular walls because the myocardium is muscular tis- cular systems. Introduction of aerobic activities during early
sue that is as equally capable of hypertrophy as skeletal childhood has implications for improved health and well-
muscle tissue. ness across the life span. Although aging has a negative
impact on performance and efficiency of the cardiovascu- adults, even older adults, can form new neural connections
lar and pulmonary systems, aerobic exercise has a positive and grow new neurons as an outgrowth of learning and
impact on these systems. Changes in the cardiovascular and training.94,97 Before the work of Eriksson and colleagues,
pulmonary systems have a significant impact on other sys- researchers and clinicians believed that structural changes in
tems and consequently on overall body function. Information older adults, such as decreased numbers of corticospinal fi-
from these systems, including blood pressure and oxygen bers, intracortical inhibition, and neuronal degradation in
saturation rates, is communicated through the nervous sys- centers in the CNS, particularly the cerebellum and basal
tem. The nervous system, in turn, regulates responses of the ganglia, were inevitable.98 In contrast, findings from a study
cardiovascular and pulmonary systems through the autonomic conducted by Draganski and colleagues99 challenged tradi-
nervous system. tional constructs that the only possible changes in the adult
human brain were the result of negative changes caused by
Neurological System aging or pathology. Instead these researchers suggested that
The nervous system encompasses the CNS and the periph- a direct relationship existed between learning a novel task,
eral nervous system (PNS). The CNS includes the brain and juggling, and structural changes in the gray matter. The au-
spinal cord, and it is responsible for all bodily functions. thors caution that these structural changes were task specific
The PNS includes both the autonomic and the somatic and limited to the training period. Reexamination of mag-
nerves and is responsible for transporting impulses to and netic resonance imaging (MRI) scans, after 3 months of no
from the CNS.5 The capacity for humans to produce behav- training, demonstrated that subjects no longer displayed the
iors far beyond those of other animals is directly related same structural changes as during juggler training.
to the complex abilities of the CNS and interneuronal Loss of neurons in the centers controlling sensory infor-
communications. mation, long-term memory, abstract reasoning, and coordina-
Over the past two decades, technological advances have tion of sensorimotor information negatively affects function.
enabled neuroscientists to dramatically improve their under- For some individuals this may not have significant implica-
standing of the molecular changes in the nervous system tions. For others, CNS changes create serious functional
over time.94 Development of the CNS is coordinated through losses. Alterations in the CNS, including altered neural con-
intrinsic influences involving the temporal and spatial coor- trol and decreased efficiency in temporal sequencing of
dination of synaptic connections with genetic processes, muscle synergies, may play a role in postural instability and
along with extrinsic or environmental factors. Initially, devel- impaired sensation. Together these changes can result in
opment of the CNS is dependent on precise connections falls.68
formed between specific types of nerve cells and begins with Although the CNS, similar to other bodily systems, may
the recruitment of cells that form the neural plate, which have the capacity to compensate for some age-related
gives rise to the neural tube, and then differentiation of re- changes, the degree of compensation may be modulated by
gions of the brain begins.5,95 Changes in the nervous system the complexity of the task and continuation of “practice”
are predicated on critical periods, or times when different over time. Although some investigators have reported that
regions of the brain are sensitive to change, and occur across neuromuscular systems in older adults may not be as flexi-
the life span.5,28 ble as systems in younger adults, new studies examining
Each region of the brain is thought to undergo critical or changes in mature and aging systems are still in the early
sensitive periods at different ages. One of the most critical stages. Neuromuscular systems in older adults may not be
periods in development of the CNS occurs from birth as capable of rapidly reorganizing muscle synergies to pro-
through 1 year of age. During this period, when the system duce variable functional responses.98 The researchers did
is most vulnerable to change, intrinsic and extrinsic vari- say that this may be related not solely to the aging neuro-
ables may influence the nervous system structurally and logical system but to other factors including experience,
functionally. cardiovascular and musculoskeletal fitness, and current
Differentiation of cells in the nervous system begins dur- level of functional independence. Other scientists suggested
ing the embryonic period and continues throughout adult- an alternative view that repetition of motor activities may
hood.5,94 Development of the nervous system during embry- stimulate new growth in dendrites located proximal to neu-
onic life involves the overproduction of glial cells and rons previously lost.68 The authors were quick to add that,
neurons that, after they are no longer useful, die. Additional although the pathways or connections may be activated,
developmental changes noted in the nervous system include this may or may not result in improved functional ability.
increased myelination within the brain and an increase in Implicit in performance of many functional activities is
neuronal size.63 Much of the growth may be attributed to cognition. If changes in cognition coexist with changes in
these changes in the nervous system and may account for the other systems, it may be difficult to accurately interpret the
development of the infant’s brain, which increases to one underlying causes.
half the size of the adult brain during the first year of life.
Neural development, particularly in the cerebral cortex, Cognitive System
documented early in development may emerge out of envi- Cognition may be defined as awareness, perception, reason-
ronmental demands and the need to solve problems (tasks). ing, and judgment.100 Cognitive development involves pro-
Consequently, experiences can alter neural networks, and cesses of perception, action, attention, problem solving,
more complex experiences lead to increasing complexity of memory, and mental imagery. Action, from the perspective
the neural structures.96 Whereas researchers long supported of physical or occupational therapy, may be referred to as
the premise that decline of the nervous system begins gener- functional movement(s) and incorporates all the processes
ally after age 30 years, more recent studies indicate that described previously to successfully perform a specific task.
Jean Piaget, one of the most recognized scientists in Contemporary researchers approach developmental the-
developmental psychology of the twentieth century, was par- ory from a dynamic and nonlinear model.21,95,96,105 Over
ticularly intrigued with how biological systems affect what the past 10 years, advances in technology (e.g., diagnostic
individuals “know.”100,101 He observed interactions among imaging, functional magnetic resonance imaging [fMRI],
children of different ages and hypothesized that younger chil- magnetoencephalography [MEG], event-related potentials
dren’s thought processes were different from those of older [ERPs]) have dramatically improved the ability to document
children as evidenced through the differences in responses change within the developing brain.95 These technological
between them to the same questions. advances coupled with developmental paradigm shifts and
Piaget proposed that cognitive development moved in a computer modeling have led developmentalists to propose
linear, stagelike progression, each stage of which involves new theoretical frameworks to explain cognitive develop-
radically different schemes.101 He suggested four stages of ment. One model, called neuroconstructivism, incorporates
cognitive development, identified as sensorimotor state (in- intrinsic constraints and abilities of the CNS at the most
fancy), preoperational (toddler and early childhood), concrete basic cellular level with extrinsic influences involving envi-
operational (childhood and early adolescence), and formal ronmental experiences and interactions.95,96 Fundamental to
operational (adolescence and adulthood).100 He proposed that the neuroconstructivist theory is the principle of context
(1) sensorimotor behaviors stimulate cognitive development dependence, in which representations emerge in direct re-
and (2) problem solving as a measure of cognition enables sponse to the structural changes in the cognitive system.
infants and young children to identify and modify motor Embedded within neuroconstructivism is the concept of the
behaviors. infant as interactive, in contrast to more traditional develop-
Piaget’s theory of cognitive development focused around mentalists’ perception of the infant as passive. Experiences
how humans adapt within the environment and how these that individual infants engage in vary through processes
adaptations or behaviors are controlled.101 He postulated that involving competition and cooperation. The processes em-
behavioral control is mediated through schemas or plans, ployed during development may result in differing pathways
generated centrally. These schemas provide a representation or trajectories of development through which the outcome or
of the world in an effort to formulate an action plan. At birth, behavior is realized. Despite the variability in the individual
infants’ earliest schemas are organized around reflexive be- developmental trajectories, the behavioral outcome is often
haviors that are modified as the infant adapts to the affor- similar.
dances and constraints of the environment. This model is purportedly applicable to typical and
Piaget suggested that adaptations occur through two atypical development as well as mature and aging systems.
processes: assimilation and accommodation.101 He defines In contrast, whereas the processes and interactions among
assimilation as a process of altering the environment around multiple interactive constraints (biological and environmen-
cognitive structures. An example of assimilation is when an tal) may be similar in typical and atypically developing
infant, initially breast-fed, is transitioned to bottle feeding. systems, the constraints may differ. Hence, the outcome or
Accommodation refers to changes of the cognitive structures emergent behavior may be different.
to meet changing demands of the environment. Accommo- Current theories lend support to the concept that the cogni-
dation may be involved when an infant transitions from nu- tive system integrates multimodal input to process, interpret,
tritive sucking (breast or bottle) to nonnutritive sucking store, and retrieve information as a mechanism for informa-
(pacifier). tion processing and problem solving.100 Changes in cognition,
Much of Piaget’s work was based on descriptive case defined as relatively permanent changes in behavior, cannot
studies. Although some aspects of his theory were sup- be measured directly but rather must be inferred from changes
ported by subsequent studies, other aspects of his work observed across multiple systems.
have not been shown to have empirical evidence. The in- As the ability of infants to act on the environment devel-
consistencies of research findings examining Piaget’s ops, their ability to accurately detect and process relevant
stages of development may be indicative of the dynamic information becomes more efficient, lending support to the
and nonlinear nature of development and, more specifi- interdependence of the motor, cognitive, and perceptual
cally, cognition. systems.
Rather than postulating that infants are reflexive beings Information processing, defined as the ability to under-
with little or no volitional movements early on, it may be stand human thinking, is a critical factor that must be exam-
more appropriate to view infants as competent beings ined within the cognitive system. Initially, infants and young
with volitional and complex behaviors present at birth.102 children cannot recognize relevant cues or chunk informa-
Brazelton reported that a newborn infant turns toward the tion for storage. As children’s developing systems become
mother’s voice rather than toward an unfamiliar voice. In more adept at integrating information from multiple systems
addition, research conducted by Meltzoff and Moore103 and more efficient at processing information, they begin to
provides evidence supporting the complex nature of infant process relevant information more effectively. Consequently,
behavior. They found that infants as young as 2 to 3 weeks infants and young children may not use or interpret informa-
of age can imitate facial gestures performed by adults. tion as efficiently as older children.
Their work was supported by subsequent studies performed The integrative nature of movement, cognition, and per-
by independent investigators using different procedures and ception is evident in developmental psychology litera-
in different environments.104 These findings, contrary to ture.106 Given that these domains are interrelated, one area
Piaget’s proposal that infants were not capable of imitative cannot be examined in isolation of other interrelated sys-
behaviors until 1 year of age, provided scientists with a new tems. Acquisition of motor skills is the primary mechanism
perspective on infant behavior. for evaluating cognition and perception in prelinguistic
children. In addition, as individuals grow older, changes in for the driver, passengers, or others in the immediate vicinity
any system may influence functional movement. Finally, of the vehicle. Delays in processing and task execution
when examining functional movements, therapists must pose risks to the older adult or individuals with CNS defi-
always consider the individual’s cognitive and perceptual cits and may affect the individual’s level of independence
abilities. and quality of life.110
As higher-level cognitive processing skills become Although older adults experience deterioration in the
apparent, the child can accurately identify relevant cues, processing and retrieval of information, the extent of the
filter irrelevant cues, and process information more effi- decline is unpredictable. Cognitive deficits most frequently
ciently. One such higher-level cognitive processing skill is observed in older adults include word retrieval, recall, dual-
executive functioning. Adolescence signals a period during task execution, and activities involving rapid processing or
which executive functioning begins to mature.107 This pe- working memory.
riod may be characterized as critical in CNS development.
During this critical period, production of mature, adult-like Memory
decisions requires selective attention and increased inte- Memory can be broken down to three types: working,
gration of information via the prefrontal cortex. During the declarative, and procedural. Working memory, short-term
maturation process, adolescents may exhibit inconsistent memory, is the equivalent of the RAM of a computer.100
decision making, resulting in less-than-optimal outcomes. This is the mechanism that enables a child who does not
By young adulthood, as the individual approaches matu- appear to be attending to what the parent is saying to repeat
rity, optimal executive decision making becomes more what the parent has just said. Given the temporary nature of
consistent. this memory, no space in the hippocampus or amygdala is
Human systems are continuously pelted with sensory required. Working memory may in fact be more of a corti-
information through some or all of the sensory modalities. cal phenomenon. Declarative memory is what is typically
At any one time much more sensory information is available envisioned when we think of intermediate or long-term
than can possibly be processed. Consequently, the individ- memory. Declarative memory is the area where long-term
ual must learn to select information relevant to the task and information about everything an individual has ever learned
chunk the information for processing. or information acquired is stored, including facts, figures,
Another example of the multidimensional processes and names.100 An example of declarative memory is a
involved in higher-order tasks such as functional movement second-grade teacher recalling the name of a student she
is found in a study conducted by Hazlett and Woldorff.108 had in her class 15 years previously. Declarative memory is
They proposed that implicit in motor tasks are concepts of analogous to the hard drive in the computer. The third type
cognition including attention, perception, and information of memory is procedural memory. Procedural memory
processes. This multimethodological approach examined involves all motor activities, actions, habits, or skills that are
(1) the influence of attention on sensory and perceptual pro- learned through repetition in motor practice.100 Examples of
cessing, (2) the executive control of attention by higher procedural memory include walking, playing an instrument,
centers of the brain, and (3) the processes underlying multi- and driving a car.
sensory integration and the mechanisms by which attention Rovee-Collier and colleagues111-113 have conducted nu-
interacts with such integration processes.108 merous studies related to memory retention in prelinguistic
Throughout the life span, physical growth and develop- children. Evidence exists to support the premise that infants
ment of many systems have an impact on the acquisition and as young as 2 to 3 months of age are capable of identifying
performance of motor skills. Changes in one system and the relevant cues and chunking this information for later retrieval.
interactive effects on all other systems can lead to deleteri- One caveat is that retrieval of such information is possible
ous changes in motor performance as a whole. only when the specifics of the behavior are retained. Infant
A new paradigm that embraces the concept that memory memories are tightly linked to the specific information related
and cognition do not deteriorate as part of normal aging is a to the task, environment, and stimulus. Consequently, a slight
topic of discussion in scientific literature.109 This perspective change in any of these three components may result in an in-
was proposed after Gould and colleagues109 conducted a ability to retrieve information from infant memory. Retention
study that found that adult primates continue to develop new of information is directly proportional to the infant’s age. As
brain cells throughout life. The addition of new neocortical an infant grows older, the period for which information is
neurons throughout adulthood provides a continuum of neu- retained increases.
rons of different ages that may form a basis for marking As children grow older, they develop more effective and
the temporal dimension of memory. These late-generated efficient strategies to retain information. During adoles-
neurons play an important role in learning and memory of cence the brain enters a plastic period, particularly in the
older adults. frontal lobes. Neuronal connections that control sleeping
Changes in cognitive function are often revealed during and eating habits, regulate motor behavior, and modulate
tasks that require processing and retrieval of cognitive or impulses, decision making, memory, and other high-level
motor memory. Consequences of aging include slowed cognitive functions change significantly during adolescence.
information processing and increased time necessary to Given the plasticity of the adolescent’s brain, it is highly
perform motor skills. Even though learning may take more probable that environmental stimuli influence intrinsic
time in older adults, once a behavior is learned, retention changes in the adolescent’s CNS.
is similar to that of younger individuals. Of significance for Across the life span, some aspects of cognition seem to
older adults is delayed performance of long-standing tasks be impaired or changed before others. One area most sus-
such as driving a car, which may have serious consequences ceptible to age-associated changes is the prefrontal cortex.
This particular area of the brain is where information critical Pre-emotions are characterized as innate behaviors, present
to executive function, attention, and working memory is at birth, nonspecific, nonintentional, and used for communica-
stored.114 Although memory is one component of cognition tion to others about the state of the infant.126 Pre-emotions are
that is generally acknowledged to deteriorate as an individ- identified as comfort and distress. These responses may be
ual moves toward older adulthood, not all aspects of mem- positive or negative responses depending on the situations.
ory are affected at the same time or in the same way. Pre-emotions are the most fundamental sensations followed by
Episodic memory is reportedly the first to be impaired, basic emotions.
then working memory (short-term memory).114 Implicit Basic emotions and developmental timing of basic emo-
memory and semantic memory remain intact for a much tions include joy at 2 to 3 months, anger at 3 to 4 months,
longer period of time. Little information is available about sadness at 3 to 7 months, and fear at 7 to 9 months. These
procedural memory, memory of how to perform tasks. emotions are said to emerge in the absence of conscious
Researchers have suggested that an older adult’s declarative processing of stimuli. These responses are shared by other
memory is also affected by normal and neuropathological mammals, and do not involve complex cognitive process-
aging.42,114 These investigators suggested that although older ing. These behaviors lead to faster and more stereotypic
adults with deterioration in declarative memory are able to responses.126
perform tasks, the individual is unable to retain information Primary cognitive emotions are characterized as basic
and consequently unable to learn tasks.42 Many factors emotions with more specificity, in addition to a cognitive
negatively or positively influence memory, including the component. Primary cognitive emotions are highly depen-
nature of encoding or processing that information, such dent on the individual’s cognitive development as well as
as the source of the material or time of day material is cultural variations and socialization.
presented.114 Secondary cognitive emotions are depicted as complex
Although evidence exists that many aspects of memory constructs that involve social relations, with consideration
decline with age, recent evidence supports the premise that for expectations of the future. Consequently, secondary cog-
variables other than encoding and retrieving information nitive emotions are highly dependent on personal experi-
may have a significant impact on memory and remembering ences and culture. The authors provide an example of how
in older adults.115,116 Researchers at the University of Kuo- one situation—high performance in academics—would be
pio examined memory in older persons and focused on perceived by different cultures. Whereas a child from one
neuropsychological processes as a method for evaluating culture might receive praise for and exhibit pride in high
memory and other functions of the frontal lobe.116 The in- academic performance, a child from another culture could
vestigators reported that elderly subjects with subsequent have such performance deemphasized and display shame. In
degradation of the frontal lobe had memory loss. These re- addition to cognition, secondary cognitive emotions incor-
searchers suggested that some aspects of memory loss could porate social constructs of family, culture, previous experi-
be staved off through memory-sharpening activities and ences, and environment in formulating complex responses.
games, limitation of alcohol consumption, and participation These responses are cumulative, using previous experiences
in activities designed to retain details of skills and tasks. to render new responses.
Similar to these findings are the conclusions of May and Typically, emotional “development” emerges in child-
colleagues,115 who examined the role of emotion in memory hood. Examination of emotion in adults often involves a
tasks for older adults. They reported that older adults seem retrospective analysis of the behavior over a specified period
to be motivated to remember information that is emotion- of time. Although the focus of the research may be directed
ally relevant and meaningful. These findings lend support toward emotion, memory cannot be disentangled from the
to yet another system, the emotive system, which could emotion. With regard to aging in older adults, researchers
add vital information to an older adult’s memory and task generally have reported that emotions—in particular, nega-
performance. tive emotions—diminish later in life. Some researchers have
suggested that diminished negative emotions may be attrib-
Emotional System uted to decreased functioning in the amygdala.127 Still other
Although current literature does examine the emotional investigators have suggested that, rather, a decline in the
development of children,117-125 the normal emotional devel- functioning of the amygdala and decreased ability to recall
opment of adults over a life span remains a mystery. Zinck negative experiences may be attributed to the socioemo-
and Newen126 proposed a classification of emotion that tional selectivity theory (SST).128 The SST involves prioriti-
might provide a clearer understanding of responses within zation of memories and which temporal boundaries play a
and between individuals and factors that affect emotion. The role in prioritization. Specifically, older adults do not per-
authors characterized emotion into four categories based on ceive negative emotions as a priority; hence older adults are
when the emotions appear developmentally. Emotion is char- more likely to process positive emotions than negative.
acterized as a means of communicating state, expectations, A literature search of “normal emotional development
and reactions of an individual.126 Emotions are “interpersonal/ across the life span” was limited in scope and volume.129
interactive” behaviors that enable the individual to commu- Problems in normal emotional development can be identified
nicate to the world. The four categories are pre-emotions, throughout medical literature, but again the emphasis is on
basic emotions, primary cognitive emotions, and secondary children and adulthood emotional problems stemming from
cognitive emotions. Pre-emotions and basic emotions func- either pathological conditions or environmental conditions
tion as basic mental representations, whereas primary cog- during childhood.130-135 Within the literature, the reader can
nitive and secondary cognitive emotions are categorized as find discussions of emotional intelligence in adults and how
cognitive attitudes. emotional skills such as empathy or cultural sensitivity might
be taught.136-142 Specific aspects of an emotion or mood compared with when the five-word sentence was spoken in
change and how that might assist or hinder an individual isolation. Young adults (21 to 22 years old) reportedly did
within a psychosocial environment can be located,143-146 but not exhibit this same variability. In addition, investigators
the integration of the entire emotional system and its nor- reported that the duration of the simple and complex utter-
mal changes throughout life still eludes researchers. Future ances differed between children and adults. Unlike the
research directed toward aging and emotion has potential to youngest children (5 and 7 years), duration of the utterances
broaden the theoretical perspective by examining emotional decreased in adults. This finding provided evidence for the
experiences in an ecological context.147 In addition, identify- investigators’ theory that adults altered or shortened the
ing, measuring, and analyzing variables that appear to make complex utterances, given the shorter duration taken to utter
a difference in social and professional success will be future the complex sentence. Whereas earlier researchers reported
scholars’ dissertation studies.148 (See Chapter 5 on the limbic that both adults and children decrease their rate of speech
system and its influence on motor control and Chapter 9 during complex sentences,160 more recently investigators
on psychosocial adaptation and adjustment for additional suggested that adults may increase their rate of speech pro-
information.) duction. Sadagopan and Smith159 reported that although
both children and adults slow the rate of speech production,
Language children exhibited a much slower rate than adults in produc-
Consistent with all areas of development, acquisition of lan- ing complex sentences. Hence investigators concluded that
guage, receptive and expressive, is measured quantitatively whereas adults’ rate of speech production did explain some
and qualitatively. Critical to the acquisition of receptive and of the difference in utterance duration for the simple and
expressive language is sensory, cognitive, perceptual, and complex sentences, it did not fully explain the differences.
motor development in the infant and child. Researchers have This led investigators to suggest that differences in utterance
found evidence that language development emerges through duration may be attributed to both faster rate of speech and
nonverbal gestures or “signs”149-153 and is evident as early as altered or shortened complex sentences containing the sim-
6 months of age.152,153 As the number of nonverbal gestures ple five-word phrase.
increases, verbal communication reportedly emerges earlier The dynamic nature of language is grounded in the con-
than in infants who do not use nonverbal gestures.150,152,153 structs of dynamical systems theory involving intrinsic and
“Gesture thus serves as a signal that a child will soon be extrinsic mechanisms. These mechanisms evolve over time
ready to begin producing multi-word sentences.”154 Having and are highly sensitive to changes within and between
a large number of gestures at 18 months of age positively systems.
affects later language development and is the foundation for
later linguistic abilities.151 Perceptual System
Imitation, such as “mama” or “dada,” is often the first As researchers continue to examine the interactive and inter-
form of verbal communication, progressing to spontaneous dependent roles of body systems, the perceptual system must
single-word utterances. Infants produce their first spoken not be omitted. Perception, yet another process important to
single-word utterances as early as 12 to 15 months of age.155 performance of functional movements, involves acquisition,
During this time the child’s brain is undergoing rapid dif- interpretation, selection, and organization of sensory informa-
ferentiation in Broca’s area; at the same time, motoric ability tion. Perception is the very essence of the interaction between
to communicate verbally is emerging. Consequently it is organism and environment. Every movement gives rise to
evident that intrinsic (neurodevelopmental) constraints and perceptual information and in turn guides the organism to
extrinsic (environmental) factors affect the emergence of adapt movements accordingly.10,161
receptive and expressive language. Investigators examining Initially perception revolves around the infant’s visual
utterances in children and adults reported that utterances exploration of people, objects, and environmental activi-
produced by children do not approximate those of adults ties. Infants are capable, at birth, of visually exploring their
until 14 years of age.156,157 Recently researchers reported environment, people, and objects.101 Investigators have
that, consistent with findings of earlier investigators, articu- suggested that infants use information acquired through
latory movement speed increases from birth to adulthood.157 visual exploration to develop new methods of exploring
Throughout childhood, as language acquisition emerges, and discovering cues about the environment such as
children become more sophisticated in communicating and depth, distance, surface definition, and dimensionality of
more fully integrate information from intrinsic and extrinsic objects.162
sources to produce more complex utterances.155 A second phase of perceptual exploration emerges as an
Maner and colleagues156 reported that children exhibited infant’s exploratory behaviors transition to functional move-
increased variability when a five-word sentence was embed- ments such as reaching and kicking. Through these explor-
ded in longer sentences than when the child spoke only the atory behaviors emerge additional mechanisms for acquir-
five-word sentence. These findings led the investigators to ing information about the environment.6,161 Throughout
infer that a relationship existed between language process- development, active exploration enhances perceptual infor-
ing and movement in young children.158 In addition, adults mation through each new encounter and enables the infant
reportedly modified the five-word sentence when it was to recognize distinctive features and similar characteristics
embedded in the longer sentence, but 5-year-olds did not that allow the infant to differentiate between objects. The
modify the utterance. Sadagopan and Smith159 replicated the information generated from exploration provides new input
work of Maner and colleagues156 and found that children to many subsystems, in particular the sensory, motor, and
aged 5 to 16 years exhibited more variability when the five- cognitive systems that enable the individual to gain new
word sentence was embedded in a more complex sentence knowledge about the environment and the action.
Development of the infant’s perceptual system is depen- stimuli. Emergence of these reflexive motor behaviors was
dent on acquiring new information about the affordances of based on traditional models of CNS organization and mo-
the task that may influence performance of the action. As the tor development theories. Traditional theories of human
infant develops the capacity for independent mobility, the development emerged from animal models and studies in-
expanse of the environment increases, as do the opportuni- volving spontaneously aborted fetuses.14,166 Traditionally,
ties to integrate prior knowledge with newly acquired infor- sucking and stepping behaviors were examples of develop-
mation to discover unchartered surroundings. This again mental reflexes. By definition, a reflex is a consistent
supports the interactive and interdependent nature of sys- response to a consistent stimulus. By use of traditional
tems throughout development. As maturation progresses, models of CNS organization, developmental reflexes, pres-
infants develop the ability to evaluate information acquired ent at birth, become integrated as higher centers assume
from various systems and to make decisions about the opti- control over lower centers and then volitional movements
mal strategy for successfully navigating over or around a begin to emerge.
surface. With maturation, successful navigation of new envi- Over the past two to three decades, advances in tech-
ronments depends on opportunities for exploration that may nology have enabled scientists to gain more insight into
involve other processes in addition to motor processes. An fetal and infant motor abilities. More recently, research
example of this may be seen in a person trying to locate a has generated evidence that behaviors emerge out of a
building in an unfamiliar city. Adults typically use maps as need to solve a problem in the environment rather than
a visual representation of the surroundings that allow them to solely as a result of maturation in the CNS.167 Given this
find the location. Infants and young children are most accu- evidence supporting the premise that newborn infants are
rate in locating desired targets through active exploration. capable of producing complex volitional movements, pre-
This allows the child to acquire spatial information critical vious views of the infant as passive and “reflexive” are no
to locating the destination at a future time. longer accurate. In addition, continuing to refer to early
If perception is the process of integrating and organizing infant motor behaviors as “reflexes” may also not accu-
intrinsic and extrinsic input, then changes in sensory systems rately reflect the behavior. A reflex is defined as a consis-
as a consequence of aging are certain to affect perception.163 tent response given in response to a consistent stimulus.
The visual perceptual processing system is most often identi- Perhaps use of the term innate motor behaviors to reflect
fied as altered in older adults. Specifically, researchers have behaviors that are present at birth may be more appropri-
reported that although older adults are capable of discrimi- ate. See Figure 3-1, A to C, for a visual explanation of how
nating between variation in depth perception in a manner the complexity of the stepping reaction of a newborn
similar to that of younger adults, they are less able to dis- infant and the learned programming for upright posture
criminate between three-dimensional shapes of objects.164 and balance, including biomechanical range and force
Clearly the perceptual system is closely associated with production, will lead to the integration of stepping in
many other body systems, and therefore age-related or age- standing. Similarly, as an individual ages, loss of some of
associated structural and functional changes in associated the postural power, effectiveness of balance reactions, and
systems will affect the perceptual system. fear can create a potentially dangerous environment for
In addition, May and colleagues115 found that older adults an elderly person (Figure 3-1, D to E). Likewise, an indi-
placed less emphasis on perceptual aspects of an event than vidual with an abnormal or inefficient stepping pattern
they did on the emotional components when encoding infor- (Figure 3-1, F) should automatically stand out to a thera-
mation. They suggested that older adults may find emotional pist analyzing movement dysfunction. If a clinician does
information to be more meaningful than perceptual informa- not have a clear picture in his or her mind of the move-
tion and may retain more elaborate, detailed processing of ment pattern desired, then easily or quickly identifying the
emotional data than perceptual information. system or subsystem motor impairments seen in a client’s
Although there is no conclusive evidence regarding age- movement dysfunction may be outside a therapist’s ana-
related changes in perception, evidence may be emerging lytical repertoire.
that supports age-associated or individual differences.165 Contemporary research refutes the assertion that infants
Nonetheless, the role of perception in aging should continue are reactive organisms.95,96 In contrast, contemporary studies
to be investigated and should not be underestimated or purport that infants are competent and capable of producing
minimized until such time as adequate evidence exists. complex interactive behaviors at birth.101,168 Additional sup-
port for the complex nature of a newborn infant is evident in
MOTOR DEVELOPMENT the infant’s ability to discriminate and turn toward his or her
Movement is the primary mechanism by which prelinguistic mother’s voice rather than toward the voice of an adult with
children communicate with their environment. That said, it whom the infant is unfamiliar.
is no wonder that development of motor skills is greatest Evidence from studies examining motor development
during the first 2 years of life. Motor development may be indicate that motor behaviors do not always emerge in a
defined as the acquisition, refinement, and integration of linear and predictable sequence, nor do all individuals
biomechanical principles of movement in an effort to achieve achieve the same skills at the same chronological age.8,168
a motor behavior that is proficient.11 Rather, emergence of motor behaviors in an alternate se-
Early developmental researchers referred to infants as quence may be attributed to the intrinsic and extrinsic con-
reactive, inferring that, early on, infants are responsive to straints that contribute to motor development in a nonlinear
stimuli rather than capable of initiating functional move- fashion and is not necessarily indicative of atypical devel-
ments. Young infants were characterized as “reflexive” beings opment. Figure 3-2, A is an example of a child who had a
producing stereotypic primitive and postural responses to very large head at birth. His head circumference was in the
A B C
D E F
Figure 3-1 n Development and integration of stepping, upright vertical posture, and vertical balance
reaction: A, automatic stepping in a newborn infant; B, early cruising or side-stepping using multiple
points of support; C, early bipedal independent stepping; D, 90-year-old client stepping; E, 78-year-old
client with falling problems; F, abnormal stepping after traumatic brain injury.
99.9th percentile and remained so for the first 3 years of his child, he has taken full advantage of his environment to
life. His Apgar score at birth was 10, or normal. He was play and learn.
slow in rolling over and coming to sit and spent much of his Motor performance, measured both qualitatively and
time playing with his hands and feet and visually exploring quantitatively, is highly dependent on the task, the environ-
the environment. Figure 3-2, B and C illustrate that his ment, and the individual. Changes in motor performance
focus was on fine motor development throughout his first emerge in accordance with age-dependent changes, within
year, especially when he was placed in a vertical position. different systems, and with respect to environmental affor-
He loved to play catch when placed in a sitting position and dances and constraints. As skills emerge in speech, lan-
accurately trajected the ball toward a partner when playing guage, and cognition, other previously achieved skills may
by age 1 year. His early gross motor development was “regress.” In reality, acquisition of a new skill requires more
within the normal range but below the mean. He started attention than the previously attained skills; consequently,
independently walking at age 14 months and began running the infant or child’s attention is divided between the tasks.
on the same day. Figure 3-2, D through F illustrate that Lindenberger and colleagues169-171 conducted studies inves-
once he gained control over the heavy weight of his head, tigating life span changes in resource allocation during
he quickly caught up in gross motor skill. And, like any multitask activities. The researchers found that for young
A B C
D E F
Figure 3-2 n Head size can affect when a child initiates independent movement activities and
whether he develops gross or fine motor skills. A, A 2-month-old being fed (note large head). B, Child
at 5 months old placed in sitting has overcome delayed head control in vertical. C, By 9 months old,
the child has gained normal head control as well as fine motor skills. D, Head control in horizontal
crawling. E, Child running at 2 years of age. F, Normal head control on slide. By 1 year of age, head
size is no longer a variable.
children certain tasks require more attention, and attempt- of motor behaviors is never the same for any two individu-
ing to perform such a task in conjunction with a task requir- als, nor does decline in functional motor behaviors follow
ing less attention causes deterioration in performance of the same time line. Figure 3-3 illustrates how standing pat-
both tasks. Hence, deterioration of a previously attained terns will change with practice, be maintained as long as
skill is more likely a result of attentional demands of young practice continues, become extremely efficient within a
children performing high-attention tasks rather than a specific environmental context, or become deficient after
true “regression” of the skill (see Chapter 1, Figure 1-9, CNS injury.
p. 18). This progression is illustrated by a child confronted
with a new environment, who will seem as if he or she has Prenatal (0 to 40 Weeks’ Gestation)
regressed in motor performance while confronting and Development
solving a task-specific challenge—for example, crossing Motor behaviors emerge early in embryonic life. By the tenth
over a suspension bridge that moves from side to side, is week of fetal life the variety of observed movements in-
compliant to body weight, and creates a perceptual chal- creases, as does the frequency of the movements. Complex
lenge from the visual surround. Once the child understands movements are present by gestational age (GA) 12 weeks,
the task, his attention is directed toward developing effec- and goal-directed movements may be seen as early as GA
tive strategies to solve a problem and successfully perform 13 weeks. Facial movements, including sucking, swallowing,
the motor behavior of crossing the bridge. Stability or and yawning, are evident in the second and third trimesters.
consistency in performing a skilled movement is achieved The fetal activity level increases so that by week 14 GA,
by self-organization through practice and repetition.21 periods of quiet (no activity) are only 5 to 6 minutes in dura-
Performance of skilled movements, such as those observed tion. Investigators have documented 15 fetal movements
in athletes, is measured not only on the consistency in per- visible by 15 weeks of age.172 After initial observation of a
forming the task but also on the skilled performance of the motor behavior, it remains part of the fetal repertoire. Pooh
task under variable conditions.21 Conversely, decreased and Ogura’s172 research lends support to the premise that
frequency in performing a motor skill as an effect of age before delivery fetuses are in fact capable of producing com-
may result in a less rich repertoire of normal variability and plex motor behaviors.
may be a contributing factor to a decline in motor skills.30 The dynamic nature of birth and the associated change
Just as motor skills emerge from a multifactorial interweav- from the intrauterine to the extrauterine environment alter
ing of maturation and experience, deterioration in motor the production of movements previously observed in fetal
performance may be attributable to alterations in various life.65 As the newborn infant adapts to the forces in the
systems that occur as part of the aging process. Emergence extrauterine environment, motor behaviors emerge. These
A B C
D E F
G H I
Figure 3-3 n Standing as a functional activity will become procedural with practice and be main-
tained over a lifetime as long as impairments do not preclude practice or injury to the central nervous
system (CNS): A, early standing; B, relaxed standing as adults; C, standing on uneven surfaces; D,
procedural standing during a functional activity; E, advanced skill in standing as ballet dancer; F,
maintained functional standing in healthy 83-year-old elderly couple; G, elderly man developing
verticality impairment; H, subtle abnormal standing after head injury; and I, multiple subsystem
problems in standing after CNS injury.
complex behaviors lend additional evidence to the premise lower extremities to modulate stepping. This is just one
that at birth infants are competent beings. example of the significance of one system on another and
The extrauterine environment poses many challenges for the interdependent nature of body systems. Recognizing
the newborn infant. Consequently, fetal behaviors observed the interdependence of systems may provide one explana-
by ultrasonography may not be evident postnatally until the tion for the presence or absence of motor behaviors at any
infant learns to adapt to the new environment by modifying given time. These concepts have been transferred into the
movements to accommodate to the new forces imposed by therapeutic practice environment when working with indi-
gravity. Newborn infants must learn to use new strategies viduals who cannot generate enough force to produce the
to generate functional motor behaviors, given the different movement given the body size or cannot produce the pos-
environmental constraints. tural stability to reinforce the stepping pattern or one of
a variety of other motor components that support normal
Infancy (Birth to 12 Months) upright stepping or walking. (See Chapter 4 for theories
As alluded to earlier, newborn infants possess a rich array of of motor control and learning, Chapter 8 for therapeutic
motor behaviors. During the first year of life, motor behav- approaches to assist a client with learning or relearning
iors are the primary mechanism for learning. Every move- motor control, and Chapter 37, which introduces emerging
ment is a new and unique opportunity to gain knowledge practices to bridge the gap between normal human move-
about the environment. During each movement, new infor- ment development and technologies.)
mation is gathered in an effort to solve environmental prob- At birth, an infant is capable of turning toward the sound
lems, and as a result this motor planning fosters cognitive of her or his mother’s voice and visually focusing on objects
development. Similarly, each movement provides feedback 8 to 12 inches from the face.101 These behaviors are apparent
that intrinsically enables the infant to modify movements in when the infant’s head is supported, given that at birth the
accordance with changes in the environment, the skill, or newborn infant does not have the neck strength to maintain
growth parameters.173 This interdependence between per- head control against gravity. Similarly, auditory and visual
ception and motor behaviors allows one domain to facili- stimuli continue to bombard the infant and challenge the
tate acquisition of skills in the other domain in a reciprocal motor system, fostering the need to attain head control.
fashion. Infant motor behaviors during the first 3 months of life
Given the capabilities of a newborn infant, many behav- are focused on acquisition of head control in all planes of
iors previously identified as reflexes are in fact functional movement. Once the infant has achieved head control in the
motor behaviors that the infant is capable of modifying. supine and prone positions, the complexity of the tasks in-
Evidence that one such behavior, sucking, is not a reflex was creases exponentially on the basis of the new challenges and
supported by studies examining sucking rates when stimuli stimuli presented to the infant. For example, while in the
were varied.174,175 Researchers reported that the sucking prone position an infant may reach for an object out of reach
response varied depending on the level of hunger or environ- and then roll to attain the desired object. Improvements in
mental stimuli. In addition, when the stimulus is introduced visual acuity enable an infant to visually track people and
after feeding, after the infant is satiated, the stimulus may objects at greater distances while challenging the infant to
produce no response or a diminished response, thus refuting seek out the stimuli.
the idea that sucking is reflexive. By age 3 to 4 months, as the infant is able to maintain
Consequently, rather than refer to these behaviors as devel- head control in the upright position for longer periods, coor-
opmental reflexes, it seems more accurate to refer to such dinated eye-hand activities begin to emerge. Acquisition of
motor behaviors, evident at birth, as innate motor behaviors. manipulative skills involves perception and lends support
Innate motor behaviors are, in essence, functional behaviors for the coupling of developing cognitive, sensory, and motor
present at birth that are modifiable given alterations in feed- systems.23,161 Bushnell and Boudreau6 added that if the
back from intrinsic or extrinsic mechanisms. infant is unable to achieve a motor skill and this skill is
Additional evidence exists to refute the concept that other coupled with a sensory or cognitive task, that task may not
motor behaviors are reflexes. Stepping is one such behavior. be attained. Bushnell and Boudreau’s6 research focused on
Thelan and Fisher18,32,33 conducted a series of experiments the role of motor development in achieving skills in other
examining the stepping reflex in young infants. Early devel- domains.
opmentalists hypothesized that stepping reflexes, present at Reaching is one such task that the researchers suggest may
birth, became integrated and then later emerged as a voli- serve to promote skills in cognitive and sensory domains.
tionally controlled movement. Thelan and Fisher18,33 found Initial reaching activities enable the infant to gain information
that when one variable, weight, was altered, infants mim- relevant to depth perception, and coupling this information
icked “integration” or emergence of the behavior. Young then allows the infant to modulate parameters associated with
infants who were stepping had weights added to their lower reaching. For example, the infant must learn to vary the dis-
extremities, to simulate weight gain over the first few tance moved and force necessary to attain an object given
months. These infants stopped stepping. Similarly, infants a series of opportunities. Infant grasping and reaching may
who did not step were submersed in chest deep water, simu- initially seem inefficient, but with practice under varying
lating less weight in the lower extremities, and stepping situations efficiency and accuracy improve across multiple
appeared. Obviously, the presence and absence of this be- domains with varying rates. Figure 3-4 illustrates both the
havior was mediated by weight gain in the lower extremities error that provides feedback and the success during complex
and not by CNS control as early developmental researchers movement patterns after practice.
had postulated. Consequently, upright mobility emerges As infants develop an upright sitting posture, they use their
when the infant is able to garner the force production in the upper extremities for support. Sitting, a functional motor
all fours, and then upright (cruising and ambulation). Adolph

and Berger23 reported that as coordination of upper- and
lower-extremity movements with trunk control emerges and
infants begin upright mobility, they spend up to 50% of their
day performing balance- and mobility-based activities, vary-
ing the surface, distance, and other parameters each time the
task is performed (Figure 3-6). On the basis of research
conducted with infants acquiring independent mobility,
Adolph and Berger23 estimate that an infant walks up to 29
football fields each day.
Acquisition of independent mobility is complicated by new
manipulative skills, as discussed by Corbetta and Thelan.176
They found that while infants are achieving independent mo-
bility, their manipulative skills are highly variable and vacillate
between bimanual and unimanual tasks depending on the
nature of other tasks with which the infant is involved. Infants
may revert to performing bimanual activities, with upper
extremities coupled and movements synchronized, during
early acquisition of independent mobility, signaling the pres-
ence of multiple tasks requiring attention. Unimanual control
A signals uncoupling of the upper extremities and asynchronous
manipulation in young children.
Analyzing the early development of a child is often done
at specific times over the first year, such as at birth, 2 months,
6 months, and 9 months. As important as understanding and
analyzing a complex phase of development such as 3 months
is analyzing the changes in motor control and learning of a
specific motor function over a period of time. The develop-
ment of head control is a good example of how the ner-
vous system adapts and learns given different environmental
restraints over a period of time. The complexity and integra-
tion of movement patterns involved in a child gaining func-
tional control over the head in all spatial environments can
be delineated into many components. Each component can
then become a variable in determining how and if a child has
or will develop a specific movement pattern such as head
control (see Figure 3-2).
B Early Childhood (1 Year to 5 Years)
Figure 3-4 n Reaching activity. A, Error in reaching leads to Whereas the first year of life is characterized by periods of
learning, and B, reaching becomes accurate during a complex mo- rapid physical growth and acquisition of motor skills, the sec-
tor task. ond year signals a slower rate of growth, refinement of current
skills, and acquisition of new motor skills.177 Concurrently the
toddler experiences rapid differentiation in other domains in-
behavior, is tightly linked to the performance of most ADLs cluding cognition, speech, and social-emotional domains.
and occupational and leisure-time activities. Figure 3-5 At the onset of the second year of life, independent
depicts development of functional sitting over a lifetime. A ambulation becomes refined as other forms of mobility
delay in attaining independent sitting may directly affect up- wane. Dynamic balance in an upright bipedal posture
per extremity control, alter attainment of skills in other do- evolves as the infant develops more mature gait character-
mains, and ultimately affect an individual’s level of functional istics. Modification of parameters indicative of a more
independence. mature gait include narrowing of the base of support,
As upright trunk posture is attained and independent sit- decreased co-contraction in the lower extremities, and
ting emerges, usually by 6 months of age, and infants then improved intralimb coordination, as well as learning to
begin to explore using their manipulative skills. Manipulative modulate displacement and velocity.23 As gait matures and
skills are composed of reaching and grasping behaviors. toddlers have more opportunity to explore their environ-
Upper-extremity interlimb coordination bimanual and uni- ment, more challenges appear. Attempts to solve these
manual tasks include retrieving objects (placed within reach); problems and challenges result in the appearance of more
holding two objects, one in each hand; using two hands to complex motor behaviors that include running, climbing,
hold an object (bottle); and holding a toy in one hand while and jumping. Toddlers find particular pleasure in throwing,
retrieving another object with the free hand.176 kicking, and catching balls. In addition, toddlers assert
During the second half of year 1, the infant is focused on their independence through such activities as propelling
mobility, initially prone (rolling, crawling), then creeping on themselves with riding toys.
A B C
D E F
Figure 3-5 n Development and maintenance of functional sitting. A, Early support sitting during first
year. B, Independent sitting during play. C, Functional sitting in adolescents while studying. D, Adults sit-
ting without support while eating. E, Sitting as part of a social interaction of an adult group. F, Functional
G
changes in sitting in the elderly. G, Loss of adequate sitting programs after closed head injury.
Toddlers continue to explore and assert independence small objects [cereal, raisins]). Achieving these tasks enables
through activities involving bimanual and unimanual tasks. the toddler to perform rudimentary aspects of ADLs such as
Challenges to fine motor skills of toddlers involve manipulating eating and dressing and adds another degree of independence.
functional objects (large buttons, eating utensils, crayons, Figure 3-7 illustrates development of ambulatory skill over a
door knobs, and blocks; opening and closing jars to retrieve lifetime.
A B C
Figure 3-6 n Emergence of upright mobility: A, quadruped in preparation for quadrupedal creep-
ing, B, moving from quadruped into standing, and C, moving in vertical.
Preschool-age children pedal a tricycle and use a narrow reciprocal arm swing and a heel-to-toe gait pattern. Early in
base of support to walk along a balance beam. By age 3 years the preschool period, children mimic a “true” run and have
most children ascend stairs using alternating feet and by difficulty efficiently controlling all aspects of the behavior.
4 years most descend stairs alternately. Figure 3-8 shows a Finally, receipt and propulsion of balls of all shapes and sizes
preschooler descending stairs. The gait pattern matures with improve qualitatively.
A B
C D E
Figure 3-7 n Functional ambulation over the life span. A, Early independent walking. B, Two young adults walking on sand. C, Three
adults of different body sizes, each walking independently. D, Hiking with backpacks requires motor adaptations. E, Elderly man walking
with visual guidance instead of visual anticipation, creating potential functional impairments.
Motor skills that children display during this period

include galloping, hopping on one foot for up to 10 hops
(hopscotch), jumping rope, kicking a ball with improved
control (soccer), and bouncing a large ball (basketball).
Often these skills emerge while playing with peers during
directed (physical education or community-based team
sports) and nondirected (recess) periods. Mobility, balance,
and fine motor skills improve dramatically. Girls and boys
exhibit similar abilities in speed up to age 7 years but by age
8 years boys begin to outperform girls.177
During childhood, manipulative skills increase exponen-
tially. Figure 3-9, A through E, shows the amazing skill
developed between birth and age 4 years. Manipulative
skills assume a predominant role as part of the academic
experience, requiring high levels of practice and opportuni-
ties for refinement of the skills. Hand preference is con-
firmed by this age. As components of independence, many
of the manipulative skills achieved are directly related to
A self-care activities. Skills that improve dramatically include
dressing, including fastening and unfastening clothing;
tying shoes; using an implement for writing (coloring and
handwriting); and successfully manipulating utensils not
only to eat, but to socially interact while eating. As children
approach preadolescence (9 to 12 years of age), manipula-
tive skills improve dramatically. Children produce cursive
handwriting and complex drawings.
Perceptual development often improves significantly,
often in direct relationship to the demands of the tasks
along with practice, feedback, and motivation.177 Visual-
perceptual systems are nearing maturity and allow children
to participate in sophisticated activities such as archery,
baseball, dance, and swimming. Figure 3-10 helps bring to
light complex skill development during a lifetime. That skill
development may begin with a fun team sport activity and
lead to a lifetime of professional accomplishment.
The musculoskeletal system enters a period when muscle
growth is rapidly increased, accounting for a large percent-
B age of the weight gained during this period.177 Constraints
and affordances of the musculoskeletal system along with
Figure 3-8 n Child descending stairs successfully: A, attention demands of the tasks and environment are highly interactive
on stepping down and B, success at the task. and influential in skilled activities. Children are generally
flexible because muscle and ligamentous structures are not
Fine motor skills expand significantly during the preschool firmly attached to bones. Although this allows for flexibility,
period. The environmental demands of preschool and day it also poses risks for musculoskeletal injury. Care should be
care preparation for entering primary grades are the driving taken when participation in high-level athletic activities is a
force behind acquisition of many manipulative behaviors. consideration.
Most children begin to cut with scissors, copy circles or Qualitative changes in coordination, balance, speed, and
crosses, use a crayon to trace a circle, match colors, and often strength improve while existing motor skills become more
demonstrate hand preference. Although maturity certainly refined and controlled, more efficient, and more complex.177
plays a role in skill performance, the efficiency with which Qualitative improvements of motor skills may be attributed
skills are performed is also influenced by genetics, affor- to an asynchronous growth in children’s limbs in relation to
dances and constraints of the environment, practice, and the trunk. Consequently, better leverage is attained. Motor
intrinsic motivation. skills strongly influence social domains as boys and girls
begin to perform in organized sports teams in school and
Childhood (5 to 10 Years) the community. Competition within sports becomes a pow-
Childhood is characterized as a period when children begin erful force in motivating children to practice motor skills or
their formalized education, usually in a structured environ- directing children away from organized sports. Children
ment separate from their families. Consequently, a new set with poorly developed motor skills, as a result of either
of dynamics comes into play. Children take on new roles genetics or opportunity, may be excluded from team activi-
with peers and adults outside of the family. During this ties and experience social isolation.177 Similarly, a child may
period of social-emotional change, other systems also have the genetic potential to become a master in an area that
undergo changes. uses a specific motor task, but if he or she is never introduced
A B C
D E F
Figure 3-9 n Functional development of prehension. A, Early prehension while

body is supported. B, Gross grasp with visual system released. C, Development
and practice of eye-hand coordination during play. D, Early independent feeding
with error. E, Functional use of individual digit. F, Eating with few errors while
using utensils. G, Fine motor prehension while simultaneously using force and
G
direction of the upper extremity with little error during play.
to the specific environment, such as playing a piano, and the During later adolescence, when periods of physical
motor skill is never actualized. growth have stabilized, motor skills acquired previously con-
tinue to develop in speed, distance, accuracy, and power.
Adolescence (11 to 19 Years) Many adolescents are involved in competitive sports. How-
Early adolescence signifies a period characterized by im- ever, few exhibit performance levels identified with elite
proved quantitative performance and qualitative changes in athletes. Those athletes that do reach this high level of skill
skills along with physical growth (size and strength).11 By often have a genetic predisposition, environmental affor-
age 12 years, reaction times closely resemble those of the dances, adequate opportunities for high-level practice and
mature adult. Although skills involving balance, coordina- performance, and strong motivation. More often, adolescents
tion, and eye-hand coordination also continue to improve performing in competitive sports will find this is their avoca-
with respect to perceptual development and information tion rather than their vocation (see Figure 3-10).
processing, the rate is not as dramatic. Elite athletes, in con- Manipulative skills of adolescents resemble those of
trast, often continue to show steady improvement in qualita- adults. Greater dexterity of the fingers for more complex
tive and quantitative skill performance well into adulthood. tasks including art, sewing, crafts, knitting, wood carving,
erally focused on leisure activities or elite athletic competi-

tion. Leisure activities of many adults involve exercise of
some form. Maintaining a healthy lifestyle through exercise
and fitness is one method for staving off effects of aging and
degenerative diseases. Although many adults participate in
exercise for health and wellness (refer to Chapter 2 for ad-
ditional information), others who do not routinely exercise
are at risk for obesity and associated health problems. Often
some of the physical activity expressed through the motor
system is an example of parent-child bonds and creates a fun
environment for play. When a task-specific activity is se-
lected and challenged by family members, the motivation to
perform becomes high. In Figure 3-11, A, the observer
might think that all three individuals are performing simi-
larly with similar strategies. In reality, single-leg stance in-
creases in difficulty as the base of support decreases and the
body size changes. Note that the smallest individual has the
smallest foot and the largest base of support in proportion to
his body size. The adolescent is not as tall as his father, but
his foot size is significantly larger, which (1) increases his
A base of support, (2) gives him less input proportional to his
foot size or representation on the somatosensory cortex, and
(3) gives him higher degrees of freedom when shifting his
weight, which can either increase or decrease the task diffi-
culty depending on practice. Figure 3-11, B shows the way
both taller individuals use strategies to initially assume the
upright stance position while the smaller child attained the
single leg stance by stepping from a table. Body height,
weight, and amount of practice all are variables that will
help determine outcome. All three individuals achieve suc-
cess, although the specific motor patterns and strategies used
to succeed may be different. In Figure 3-11, C, a child with
severe sensory organization problems would not be able to
solve the challenge presented in Figure 3-11, A, because he
cannot even begin to stand independently on a large, stable
surface. Again, therapists need to be able to match the motor
program impairments causing the child with learning dis-
abilities to fail at the task and identify what programs are
needed or expressed in the success of all three individuals in
Figure 3-11, A.
The peak of muscular strength occurs at 25 to 30 years
of age in both men and women. After that period, muscle
strength decreases as result of a reduction in the number
and size of the muscle fibers.178 Loss is related to genetic
factors, nutritional intake, exercise regimen, and daily
activities.
B Middle Adulthood (40 to 59 Years)

Changes associated with aging have been identified in
Figure 3-10 n Complex skill development. A, Child participating the neuromuscular, musculoskeletal, and cardiovascular and
in a team sport. B, Adult demonstrating advanced skill development pulmonary systems. These age-associated changes can
as a professional baseball player. greatly affect motor performance, although the degree is
highly variable. Between 30 and 70 years of age, strength
loss is moderate, with about 10% to 20% of total strength
and musical performance enables adolescents to perform lost, for most activities—insignificant and undetectable by
these motor tasks with greater precision and proficiency. the individual.68,179 Participating in regularly scheduled ex-
ercise regimens that emphasize aerobic and strengthening
Adulthood (20 to 39 Years) activities may reduce effects associated with aging. Further-
Motor performance is relatively consistent in adulthood. more, developing muscle mass and competence in motor
Skillful performance of movements may be characterized skills early in life may have long-term positive effects on
not only through consistency, but also through the infinite adult skills and reduce the risk of disability and frailty later
ways in which the skill is performed. Hence, change is gen- in life.180
B C
Figure 3-11 n A complex task performed by individuals with different foot size and body compo-
sition. A, Three individuals of differing age, body composition, and experience successfully per-
forming the same task. B, The strategy used by the adolescent and adult to find verticality. C, A child
with learning difficulties failing at independent one-legged stance.
Older Adulthood (601 Years) be as much as 40% to 50% by the time an individual reaches
Age-related changes may be attributed to alteration in per- 80 years of age.65 The percentage decline is inversely related
ception, compensations in the neural mechanisms, and to the demand by the individual for repetition of the move-
changes between and within the different systems involved ments. For example, repetitive movements, such as playing
in motor skill performance.181 Integrated effects may in- tennis daily, running, playing golf, or downhill skiing, may
clude slowing in movement production and increased activa- significantly decrease the percentage of loss of strength
tion of agonist-antagonist muscle groups. An example of compared with individuals who do not participate in such
agonist-antagonist activation is during dynamic balance ac- activities.
tivities.182 After age 70 years, most individuals incur losses Although some effects are age associated and may be
in muscle strength of up to 30% over the next 10 years. reduced with regular exercise and increased motor activity,
Overall, the loss of muscle strength through adulthood may not all are modifiable. Willardson82 reported that older
adults who maintain more active lifestyles are more likely

to have a more favorable outcome than are peers who
were not as active. Findings by Voelcker-Rehage and
Willimczik97 supported the findings of Willardson82 in
regard to older adults and lifestyle. In addition, Voelcker-
Rehage and Willimczik97 found that older adults aged 60 to
69 years were able to acquire and refine a novel task (jug-
gling) at a level comparable to children 10 to 14 years old
and adults 30 to 59 years old. Only young adults aged 15 to
29 years performed at a higher skill level. Furthermore,
adults older than age 70 years were found to have limitations
in their ability to learn the novel motor skill. Moreover, as
individuals age, they generally have a decreased ability to
produce force, and they tend to coactivate agonist-antagonist
muscles.183 The researchers suggested that older adults
may coactivate agonist-antagonist muscles as a strategy to
(1) modulate movement variability and (2) maintain accu-
racy in movement. The investigators also reported that older
adults’ coactivation strategy compromised the subjects’
ability to rapidly accelerate their limbs in exchange for
improved accuracy of control.
In addition, information processing appears to be slowed
in older adults.181 Motor times have also been found to be
delayed in older adults, particularly when a higher-level
force is required.
Temporal coupling also appears to be altered in older
adults.181 Perhaps as individuals age, they are less able to
modulate timing of muscles during contraction and relax-
ation phases and are more likely to coactivate agonist- A
antagonist muscles. The outcome behaviors are typified
by poorly coordinated motor activities and increased time
to produce adequate muscle force to elicit the behavior.
In addition to reduced efficiency in movement production,
variability in performance of motor skills also increases with
age. Although small changes in individual systems may
not have a significant effect on functional movements, the
compounding effects of changes in several systems may
have serious implications for older adults and place them
at increased risk for falls and injuries.58 Figure 3-12, A and
B are examples of movement dysfunctions seen within
an elderly population. These alterations are limiting the
individual’s ability to respond to a given motor task. As
individuals age, there can be a large number of potential
alterations in the body systems that limit CNS and muscu-
loskeletal options when the individual tries to accomplish
a motor activity. These limitations can place individuals at
high risk of failure of any one motor task. The greatest
fear within this group is not death; it is falling, and as
a result losing independence. Prevention, as discussed
in Chapter 2, will be more and more important as the
world’s population of elderly individuals enlarges on a
yearly basis. B
Thirty percent of all community-dwelling elderly per- Figure 3-12 n As individuals age, more sedentary lifestyles, pre-
sons fall at least once each year.179,184 Factors contributing to existing long-term health issues such as chronic obstructive pulmo-
falls include intrinsic and extrinsic variables.185-187 Intrinsic nary disease or chronic back pain, and a decrease in environmental
alterations in the older adult have implications for perfor- challenges can lead to a higher risk of falls. A, Woman with chronic
mance of motor skills and potential for falls. Risks associ- back problems leading to a fixed trunk. B, Man with chronic obstruc-
ated with falls increase with age and when functions of tive pulmonary disease and a fixed flexed posture resulting from
the neuromuscular, musculoskeletal, cardiovascular and pul- inactivity.
monary, and sensory systems deteriorate. This deterioration
of covariant factors and not age itself is more closely related
to health and wellness and end-of-life age.188 Another criti- the cognitive, affective, motor, perceptual, sensory, and phys-
cal variable that is closely associated with motor decline in iological systems through client-selected activities. Activities
the aging is the individual’s social interactions and partici- directed toward the age and needs of the individual, such as
pation in life.189 Research has yet to determine whether a interactive dance mats for adolescent clients or ballroom
decrease in motor performance results from a decrease in dancing for the older adult, may provide the motivation nec-
participation in life. essary to practice the task a sufficient number of times to
Researchers have examined manipulative skills in older achieve the desired outcome. Figure 3-14, A to H show age-
adults and reported changes in muscle performance and appropriate challenges to individuals. The activity used with
flexibility.56,190,191 These changes resulted in decreased hand a child may be inappropriate for use with an adult, although
function associated with impaired performance of ADLs. a similar motor behavior may be the desired outcome. If the
individual identifies the activity, he will be more motivated
STRATEGIES FOR FOSTERING ACQUISITION and more likely to practice a desired skill. Carryover from a
AND RETENTION OF MOTOR BEHAVIORS clinical setting to a home or environmental setting is critical
ACROSS THE LIFE SPAN when looking at movement function over a life span.
Movements occur out of a need to solve problems in the Strategies used to achieve desired motor outcomes may
environment. Solving these problems is not dependent include a variety of feedback mechanisms to correct errors
on any one system but rather is a collaborative effort of and identify more efficient strategies to attain the motor
multiple systems. The clinician is responsible for examining skill. Embracing the concept of enablement rather than
the patient’s performance by evaluating the underlying con- disablement may also serve to motivate the client because
ditions and the strategies that the individual may use to individual abilities are acknowledged and promoted while
modify a behavior. Figure 3-13, A to F, presents an example strategies are used for acquisition or relearning of motor
of individuals standing up from a chair. The first panels skills. The needs of adolescent and adult clients are unique
(Figure 3-13, A and B) show a child whose feet are not and differ significantly from those of the young infant or
on the surface because the child’s legs are not long enough. child.
No matter the variance of the task, the child was motivated Opportunities for exploration that engage the infant
to succeed. The second individual (Figure 3-13, C and D), or young child are the primary motivation for movement.
an elderly man, has lost the ability to shift his weight for- Although motor activities serve as the primary focus, engag-
ward over his feet and thus is rising posterior on his heels, ing the infant or child provides stimulation that promotes
which will require anterior flexor power to prevent him from development across multiple domains (e.g., cognition, social,
falling backward. The third individual (Figure 3-13, E and communication). Environmentally challenging activities place
F) has residual motor problems after a stroke. She has been demands on the child that maintain a level of curiosity or
taught to come to stand over her less-involved leg versus motivation and encourage persistence in attaining a motor
centering her base of support between her two feet. The spe- skill that is successful and efficient. As the child matures,
cific way an individual learns, maintains, and relearns a play-based activities shift the focus, depending on the ex-
specific motor task as a functional activity will vary, but the pected outcomes. Overall, play is the primary mechanism
important principle will be to empower the individual to suc- that children use to mimic adult-like behaviors. Finally,
ceed with fluid, dynamic motor pattern options. Therapists children and adults use play or leisure activities as a means
need to visualize movement and place the movement pattern of promoting skill acquisition and proficiency across all
of the individual on top of that image. The specific motor developmental domains.
impairments will then become obvious and treatment op-
tions will be generated. Examination is vital to this process, Development of Head Control as an Example
although it often occurs in an environment far removed from of Movement Development across the Life
the client’s natural surroundings. Span and Its Impact on Quality of Life
Through acquisition of motor skills, individuals of all When analyzing the development of head control by view-
ages are afforded the opportunity to meet the environmental ing movement of a young child over the first few years, it
demands imposed by work, play, family, or personal activi- becomes clear that the motor control of the head in all spa-
ties. Refer to Figure 3-13 as an example of common motor tial positions is very complex, requiring the integration of a
activities used at work, play, and home. Motor skill acquisi- variety of movement patterns. The infant needs to develop
tion, retention, and decline are influenced by constraints both the flexors that bend the head forward as well as the
or affordances that affect opportunities for practice in an flexors that tuck the chin. These flexor patterns will be inte-
environment that challenges and drives the individual to grated into diagonal movements in order to roll over from
perform optimally. The client’s investment in achieving a supine to prone. A neck-righting program orients the body
successful outcome can help foster persistence in reaching to the head when the head is initially moving. Although the
the desired outcome. neck-righting program is present at birth, it will take the
Practice, the primary method for acquisition and retention child a couple of months to gain the power necessary to
of motor tasks, is exciting for very young children because independently flex and rotate the head against gravity with
each attempt is a new opportunity to achieve the outcome and the body following the head in order to roll over. As the
reach a new level of independence. In contrast, practice in flexor power improves, it will be integrated into patterns of
adolescent and adult populations may not be seen in the same coactivation with postural extensors. The extensor move-
light but rather as tedious and boring. Instead, physical and ments of the head include (1) extension from a flexion posi-
occupational therapists have the responsibility to challenge tion through hyperextension and/or rotation of the head in
A B
C D
Figure 3-13 n Three individuals coming to stand using differ-

ent motor patterns. A and B, A child rising to stand from a chair
by shifting his weight over his base of support and rising verti-
cally. C and D, An elderly man rising to stand without adequate
weight shifting forward over his base of support, requiring ad-
ditional flexor power to prevent falling backward into the chair.
E and F, A woman after a stroke rising over her less involved leg,
thus decreasing her symmetrical weight distribution and ability
to step in any direction with either foot as a response to center
E F
of gravity shifting outside her base of support.
all spatial planes, and (2) postural extension, holding and/or The righting programs need the underlying power to pro-
stabilizing each vertebra within the spinal column. These duce the force necessary to right the head. The heavier the
postural programs help coactivate the flexors and postural head, whether in weight of the cranium or its positioning
extensors simultaneously to stabilize the head in space. against gravity, the harder it is to right the head to vertical.
There are a variety of motor programs that assist in gaining Thus it could be hypothesized that if the head were in a
this control of the head. Head-righting reactions, using the vertical position or vertical in reference to gravity, it would
semicircular canals, are programmed to right the head, or be easier to control the head in space. The force production
bring it to face vertical, no matter where the head is in space. would be nominal compared with the force production
A B C
D E F
Figure 3-14 n Age-appropriate self-select func-

tional activities. A, Child walking across a creek.
B, Child balancing on a moving surface for fun.
C, Child riding a bike. D, Adult riding a bike.
E, Adult walking up a hill. F, Adult using snow-
G shoes. G, Adult fishing. H, Adults folding towels
H
into figures for fun.
needed when bringing the head up from horizontal or just motor control system to generate flexor tone in supine.
holding it against gravity when horizontal. There are motor When the child is placed prone, the skin sensitivity de-
programs triggering extensor tone due to the position of the creases extensor tone.
otoliths in the inner ear. The degree of tone will depend upon
whether the head is horizontal in supine, vertical, or in be- Flexor Control
tween. This response has been labeled the tonic labyrinthine Figure 3-15, A through F, illustrates the patterns of a healthy
reaction (TLR). The TLR is strongest in the supine position 4-week-old when being pulled to sit and returned to the floor.
because of the optimal pull of the otoliths by gravity. When Initially the child has difficulty flexing his head when trying
an individual is supine, the tactile input from pressure to to pull the head into flexion from the supine position because
the surface of the skin increases extensor tone. Thus, in the of both the extensor tactile system and the labyrinthine
supine position the tactile input and the information from mechanism, which inhibit the flexors while facilitating the
the hair cells of the otoliths are activating the motor pool extensor muscles. This can be seen in Figure 3-15, A through
of the extensors and simultaneously decrease the motor pool C. This lack of adequate flexor control would be consid-
of the flexors. These systems decrease the ability of the ered normal for the child’s age but also defined as a head lag
A B C
D E F
Figure 3-15 n A 1-month-old child being pulled to sit from supine and lowered back down.
A, Starting position in supine. B, Pulled toward vertical, maximal resistance from gravity, inadequate
response to stretch by neck flexors. C, Position of ears and otoliths inhibits flexors; thus inadequate
head righting is still seen. D, Child in vertical, weight through hips, quick stretch to head in all direc-
tions facilitates head control. E, Child lowered back toward horizontal continues to have adequate
head control. F, Maximal stretch from gravity; child still retains adequate neck flexion.
(Figure 3-15, A through C). As the child approaches vertical, when pulled from supine (Figure 3-16, A). This flexion con-
his weight is shifted down through his buttocks and over tinues as the child goes through midrange (Figure 3-16, B) and
his base of support. At that point the head control kicks in continues as the child approaches vertical (Figure 3-16, C).
(Figure 3-15, D and E). This motor control of the head in Unfortunately, the child does not have the integrated motor
vertical incorporates both postural extensors and flexors control over flexion and extension or the balance reactions
while optimizing the use of optic and labyrinthine righting in sitting to extend the legs as she approaches vertical. This
and stretch to both flexors and extensors muscle groups movement is a prerequisite for gaining control of long sitting
(Figure 3-15, D). The child is able to maintain better control in vertical. These new motor movements will become the
of head and neck flexors when lowered back down to supine foundation for balance reactions in vertical sitting.
(Figure 3-15, E and F). This ability to control the head A month later the child’s nervous system integrates the
while transitioning from sitting to supine illustrates the fan flexor patterns into smooth movement through 90 degrees of
swing principle: once the flexor program is elicited in the motion from supine to sitting (Figure 3-17, A through C).
vertical position, it can maintain head control for a longer The child also demonstrates a more integrated response to
period of time and through more degrees of motion as the being lowered backward from vertical (Figure 3-17, D). Yet
head movement progresses from vertical to horizontal. the child’s motor system has not developed the rotatory
Over the next month the child will develop flexor control aspects or control of the diagonal flexor patterns as shown in
in space by using head righting, muscle strength, and facilita- Figure 3-17, E. These rotatory patterns will develop as the
tion of the nervous system to keep the head and eyes oriented child practices rotation in rolling and then incorporates that
toward an object as the head travels through space. Motor rotation when coming to sit in a partial rotation pattern.
control over flexor patterns becomes more flexible, and the The child will not be able to independently initiate motor
power needed to perform tasks increases. Figure 3-16, A control over the adult pattern of coming to a sitting position
through C, demonstrates the pull to sit pattern in a healthy for at least 3 to 5 years but certainly should gain that control
3-month-old infant. She not only has learned to dampen the by age 6 (Figure 3-18). As the child’s age increases, the
influence of the TLR in the supine position and the skin’s movement patterns become more complex, and additional
influence on the extensor motor pool, but also has learned motor programs are learned and integrated. This aspect of
that by flexing most of her body parts (flexion facilitates head control will be maintained and integrated in movement
flexion) she will gain additional flexor control of the head patterns as the individual explores the environment.
A B C
Figure 3-16 n A 3-month-old healthy child pulled to sit. A, Initial stretch in horizontal pulls in
neck flexion along with flexion of the hips and knee. B, Adequate neck flexion persists as child looks
at therapist while being pulled to sit: midrange. C, Transitioning to vertical; less stress on neck flex-
ors, yet flexion persists in lower extremities.
A B C
D E
Figure 3-17 n Pull to sit in 4 month-old-healthy child. A, Child looks as therapist places finger to
the child’s head—recognized tactile stimulus, relaxed supine. B, Child pulled to sit, neck tucked and
hips flexed. C, In vertical, child relaxes hip flexors in order to have sitting balance. D, Child lowered
toward supine maintains neck and trunk control. E, Rotation added into lowering to supine pattern;
neck response is inadequate.
Extensor Control individual in a standing position were looking up at the

As the child develops flexion, the child also needs to develop stars). This pattern has a large range of motion, and the
the extensor control of the head. There are two extensor power needed will change depending on the head’s position
component patterns used by the CNS to control extension. in space, in relation to gravity, as well as the weight of the
The first pattern is controlling extension of the head from a head itself. The second pattern is considered “postural” and
totally flexed position (as if an individual were looking requires the small muscles of the neck and the shoulder and
down at his shoes) to a hyperextension position (as if an trunk to hold each vertebra in relation to the vertebra above
Figure 3-21, A through C, shows the same child at 1 week of

age while supported in a vertical position. Figure 3-21, C is
a beautiful example of the postural pattern one expects to
see consistently later in the child’s development. Obviously,
he is practicing these movements every time he is vertical
and given these limited degrees of freedom. Looking at the
same child at 1 month of age (Figure 3-22), the integration
of these postural movements is continuing down his entire
spine. This does not mean he can hold this pattern for an
extended period of time but does illustrate his ability to
move with control into a total postural extension pattern and
hold it at least briefly. On the same day and about 15 minutes
later, the child (Figure 3-23) was placed prone on the floor,
clearly demonstrating that he has not developed the neces-
sary power to control his head or upper trunk for postural
Figure 3-18 n Normal 6-year-old using adult independent coming- extension in the prone position. Although he is more relaxed
to-sit pattern. prone at 1 month than at birth (see Figure 3-20) and can lift
and turn his head in both directions, it will still be 1 or
2 additional months before he can extend his head and trunk
and the vertebrae below. This is the boney support encom- enough to prop on his elbows. It will take more time to roll
passing the spinal cord. Similarly, in the upper cervical re- from prone to supine.
gion as the flexor and extensor patterns work together in While the cervical and upper thoracic muscles are devel-
a coactivation pattern, chin tucking occurs. This pattern oping postural power, they need to learn to hold the head in
allows the head to remain stable in space, keeping the eyes space against gravity for longer periods of time. Postural
and the ears horizontal to the surroundings, a key compo- function requires coordination and coactivation of the
nent for perceptual learning. This movement pattern is often short extensors of the neck and the neck flexors—especially
called optic and labyrinthine righting of the head because the sternocleidomastoid, hyoid, and scalene muscles—in
both the eyes and the ears provide stimuli to trigger this order to tuck the chin and balance the head in space. These
motor response. movement patterns play a key role in stabilization of the
head in order to move the head in and out of vertical space.
Assessment of Head Control The motor control system uses both righting and balance
Most individuals analyze the development of postural con- reactions in order to gain full head and neck control of
trol by evaluating the extensor aspect of head control in the vertical space. The movements become more and more
prone position. But the prone position is not the first position complex, modifying and integrating various additional
in which a child begins to achieve independent head control. motor programs in order to have greater flexibility to con-
Figure 3-19, A through C, illustrates a newborn (2 days old) trol and move the head. These patterns give the eyes and
extending both the long extensors (Figure 3-19, A and B) ears the visual and auditory orientation needed to process
as well as moving into the postural extensor pattern compo- consistent external environmental information. Figure 3-24
nent when tucking the chin (Figure 3-19, C). While prone illustrates an individual after head injury who has lost his
the newborn child has very little independent control over automatic righting of the head in vertical. He has the ability
extension and can just clear the airways (Figure 3-20). This to bring his head to vertical by hyperextension of the neck
same limitation is not present when the child is in a sup- but does not do so automatically. This hyperextension pat-
ported vertical position. After a week the extensor patterns tern dramatically changes the motor patterns of head, neck,
in the vertical position have already become more functional and trunk control which will affect his upright behaviors
and demonstrate better motor control and greater power. including balance, gait, feeding, and social interactions.
A B C
Figure 3-19 n Newborn neck extension in supported vertical position. A, Newborn lacks pos-
tural extension of trunk and head. B, Newborn initiates neck extension, showing that patterns
exist. C, Newborn moves into postural extension of upper cervical region.
flexor head control by starting at supine. After the child has

been pulled to sit, flexor power production is beyond the
control of the child’s motor system. Once the child is verti-
cal, the influence of gravity on the weight of the head has
decreased. The vertical position also inhibits any influence
of the otoliths when supine. The upright position helps
to facilitate the labyrinthine and optic righting of the head.
Figure 3-25, D and E demonstrates how the tone within the
neck and shoulder girdle changes once the head attains a
vertical position. A therapist should not only see a change to
more normal tonal patterns but also observe relaxation of the
facial muscles—closure of the mouth and a more functional
position of the eyes in space for visual processing.
Movement demonstrates the ease or difficulty the motor
system is experiencing while trying to complete an activ-
Figure 3-20 n When placed prone, the newborn has excessive ity. The activity analyzed may be a basic pattern such as
flexion. head control or a complex one as seen in Figure 3-3, E.
Differentiating between the movements in Figure 3-15, A
and Figure 3-25, A should guide the clinician in establish-
Application of the Development of Head Control ing realistic prognoses. The first child, with practice and CNS
to Rehabilitation maturity, should automatically develop head control, whereas
The reason the patterns of head extension in vertical have the second child has gone beyond his age-appropriate move-
been presented is to illustrate that motor control progresses ment delays and has abnormal responses to the stretch stimuli.
from vertical to horizontal and then from horizontal to verti- Similar analysis can be made when looking at the exten-
cal. Therapists think of the prone position as the first posi- sor component of head control. The vertical position that
tion in which the child develops extensor control because it optimizes extension is kneeling or standing because com-
is the first position in which an observer sees independent pression down through the joints and spine facilitates
selection of extensor movement. However, extensor control extension. Using kneeling or standing may pull in too much
begins when an individual passively brings a young baby extension, especially if it results in hyperextension. In that
to sitting or standing. The baby learns to control the head case sitting may be appropriate even though it may facili-
with small movements that displace the center of gravity. tate flexion. When kneeling or standing, if inadequate
Understanding this stage of head control may be a critical extension still exists, then adding additional compression
component of working with a neurologically impaired (see Chapter 9, p. 207) down though the head or shoulder
youngster or an adult. girdle may help. Using an apparatus that takes away some
of the weight of the head or trunk reduces the demand on
Analysis between Normal and Impaired Motor the nervous system and may help the individual regain pos-
Function tural neck, shoulder girdle, and upper trunk control (see
Differentiating normal age-related reactions of any move- Chapter 9, p. 232). As the individual begins to demonstrate
ment impairment is the responsibility of a movement special- that control, the therapist can slowly take away the assis-
ist. For example, differentiating the movement of a normal tance with the expectations that the individual will gain
child who has a lag in head control (see Figure 3-15, B and independence in that activity.
C) from the movement of a child who does not demonstrate As the individual ages, changes in the control of the head
any head control (Figure 3-25, A through E) should guide the can lead to new problems. Figure 3-12, B, is an example of a
therapist in a decision about where to begin treatment. It is man who has a fixed flexed posture. If the flexed posture is
an unrealistic expectation for the child in Figure 3-25 to gain permanent, treatment alternatives must take into consideration
A B C
Figure 3-21 n Child 7 days old in vertical extension. A, Child eliciting active neck and truck exten-
sion in vertical at 7 days. B, Child does not have adequate righting of the head in vertical at 7 days
but is responding. C, Child pulls into postural extension of the neck and trunk, allowing for binocular
vision at 7 days.
Figure 3-22 n A 1-month-old child, when supported in a vertical

position, shows postural extension of the neck and trunk while
looking at his mother. Figure 3-24 n An individual who sustained a closed head injury
with residual lack of postural neck extension has poor head control
in vertical.
to be on restoring and progressing head control from vertical

to supine—that is, trying to help the neck muscles to retrain
a stable condition in the vertical position by using a collar
for support. Compression down through the cervical and
thoracic spine can facilitate flexors, extensors, and rotator
muscles. Then, as in the infant, with arms fixed on a stable
support surface, slowly let the patient move out of vertical
toward horizontal, moving only as far as head control is
maintained, which may be as limited as 10 to 20 degrees.
Repeat this activity many times in order for this activity to
become procedural. Patients can hold onto some stable sup-
port object at home in order to practice. The challenge for
both the therapist and the patient is to retrain head control in
order for the individual to be able to participate in life.
Figure 3-23 n Same child when placed prone, 15 minutes after
Figure 3-22 was taken. This child demonstrates that when prone he Summary
still has a flexor bias and has little adequate postural extension in Normal development of head control has been highlighted
this position. because so many clinicians become frustrated with clients
who do not have adequate head control. The examples of the
development of head control serve as a foundation for every
the impact this posturing will have on all ADLs. As stated movement pattern that people use, whether stationary or in
earlier, any patient can exhibit a range of problems in head motion. The process used to analyze the development of
control. How those movement impairments present them- head control can be used to analyze all movement patterns,
selves and which treatment alternatives a therapist might select whether the movement problem is caused by a bodily sys-
will depend on the clinician’s ability to analyze normal move- tem (biomechanical, cardiopulmonary, CNS or another sys-
ment and create an optimal environment for patients to engage tem problem) or by some environmental factor.
and practice those patterns.
SUMMARY
Clinical Example Clinicians must focus best practice toward successful client
Cervical torticollis in a child is an imbalance in rotation of management geared toward promotion of function and pre-
the neck and muscle groups secondary to position of the vention of chronic illness or disability for the youngest of the
fetus in utero. Cervical torticollis in adults is a focal dysto- young to the oldest of the old. As practitioners, we must
nia or an imbalance of excitation and inhibition of the neck embrace tenets central to the Healthy People 2020 project.
muscles. These patients have impaired integrative balance Physical and occupational therapists serve as role models for
responses. Standard medical intervention is botulinum toxin individuals of all ages and educate diverse groups of indi-
injections followed by physical therapy focusing on range viduals about the multifaceted, interactive systems involved
of motion and strengthening exercise. The emphasis needs in the acquisition, retention, and deterioration of motor
A B C
D E
Figure 3-25 n Child 21⁄2 years old with cerebral palsy being pulled to sit, and muscle action fa-
cilitated once vertical. A, Initial pull toward sit; no response to stretch of neck flexors. B, Continued
pull toward vertical, head at end range of neck extension. C, Trunk in vertical but neck remains in
horizontal; no flexor response. D, Child pulled beyond vertical as trunk extensors begin to activate
from stretch and mouth closure is beginning. E, Therapist facilitates head into vertical as mouth
continues to relax and close and eyes are looking at a target.
behaviors. Recognizing internal and external constraints or Human behavior is by nature complex. As such, no one
affordances that influence motor behaviors enables the clini- system or skill develops in isolation but rather emerges from
cian to devise a plan of care and the scientist to design a a complex interaction among multiple systems. Complex
study targeting the needs of the whole person. Analyzing, behaviors are evident beginning in utero and continuing
understanding, and visually recognizing movement patterns throughout life. No one theory explains the development of
that are efficient, fluid, and goal oriented and that vary across complex motor behaviors, and none encompass the essence
the life span are the first steps or prerequisites to evaluating of interindividual and intraindividual variability in aging.
abnormal movement patterns that do not fall within a normal Aspects of various theories provide evidence that an integra-
parameter. Figure 3-26, A to G, shows an example of rolling, tive perspective is a more accurate reflection of aging. As
a basic movement strategy controlled by the child midway theorized earlier, lifestyle choices and other modifiable be-
through the first year of life that can become an extremely haviors have potent effects on aging. Interventions designed
challenging activity after a CNS insult. Differentiating to provide older adults with strategies to positively influence
between components of a normal movement and deviations successful aging, rather than being sought after a negative
that prohibit normal movement falls into the clinical exper- outcome of aging is realized, may improve the quality of
tise of occupational and physical therapists (see Chapter 9, life. Optimal quality of life is what all individuals hope to
Figure 9-1, p. 199) Without the knowledge of normal move- obtain, whether learning to reach a cracker, climbing the
ment, analysis of the causation of abnormal movement highest mountain, or playing a game of bridge. Maintaining
would be difficult if not impossible. This chapter has been that quality before the end of life, no matter the age, is often
written to help the reader understand normal movement based on movement function. The client, whenever possible,
across the life span. It is the first step, and in sighted indi- should determine identification of the specific function.
viduals the analysis begins as soon as visual images are re- Identification of the necessary steps to get from existing
corded in the visual cortex. skill to desired skill is the role of a movement specialist,
Scientists acknowledge that development is characterized whether that therapist is dealing with preventive care or
as nonlinear, emergent, and dynamic, rather than sequential, postinsult care.
predictable, and stagelike. Dynamic systems theory, although
it does have certain limitations, provides a better explanation References
for development than do neuromaturational theories. The To enhance this text and add value for the reader, all refer-
emphasis or responsibility does not lie with any one system ences are included on the companion Evolve site that ac-
but varies across different systems as a consequence of age, companies this textbook. This online service will, when
genetics, or experience.104 That said, future studies directed available, provide a link for the reader to a Medline abstract
at examining human movement and optimal variability for the article cited. There are 191 cited references and other
through nonlinear dynamics may provide new perspectives general references for this chapter, with the majority of
in motor development and control. those articles being evidence-based citations.
A B
C D
E F G
Figure 3-26 n Rolling—an activity achieved within the first year. A, Child beginning rolling in
supine position. B, Child semiprone with trunk rotation. C, Child brings arm through to become
symmetrical while proceeding toward prone position. D, Child prone with postural extension. E,
Adult with traumatic brain injury; first try at rolling toward prone position from supine. F, Adult’s
second try at rolling, changing programming. G, Once prone, he is stuck, unable to extend.
CHAPTER 4 Contemporary Issues and Theories of Motor
Control, Motor Learning, and Neuroplasticity
MARGARET L. ROLLER, PT, MS, DPT, ROLANDO T. LAZARO, PT, PhD, DPT, GCS,
NANCY N. BYL, PT, MPH, PhD, FAPTA, and DARCY A. UMPHRED, PT, PhD, FAPTA

motor control theory After reading this chapter the student or therapist will be able to:
motor learning 1. Identify the evolution of motor control theories and discuss the utility of current theory
neuroplasticity in clinical practice.
2. Identify body structures and functions that contribute to the control of human posture
and movement.
3. Relate the cognitive, associative, and autonomous stages of motor learning to behavior
and skill performance.
4. Describe the variety of practice conditions that may be used to enhance motor learning
within a practice session.
5. Apply motor learning variables related to person, task, and environment within the
therapeutic setting.
6. Discuss neuroplasticity theories that explain how the nervous system adapts to
demands placed on learning and performance.
7. Discuss the relationship among motor control, motor learning, and neuroplasticity in the
production of functional movement behaviors.
T he production and control of human movement is a pro-

cess that varies from a simple reflex loop to a complex
network of neural patterns that communicate throughout the
goal-directed practice of meaningful, functional motor
skills.
This chapter introduces the reader to basic concepts of
central nervous system (CNS) and peripheral nervous sys- motor control, motor learning, and neuroplasticity. Figures
tem (PNS). Neural networks and motor pattern generators and tables are provided within each section to emphasize and
develop as the fetus develops in utero and are active before summarize concepts. A patient case example is used to illus-
birth. These simple patterns become building blocks for trate concepts in this chapter as they apply to the evaluation
more skillful, complex, goal-directed motor patterns as a and management of people with neurological conditions.
person develops throughout life. New motor patterns are This chapter provides a foundation for chapters in Section II:
learned through movement, interactions with rich sensory Rehabilitation Management of Clients with Neurological
environments, and challenging experiences that drive a per- System Pathology, and acts as a foundation for interacting with
son to solve problems. Personal desires and goals of the in- and treating patients in any clinical setting.
dividual shape the process of learning new motor skills at all
stages of life. If a condition exists or develops, or if an event MOTOR CONTROL
occurs that damages the nervous system and prevents nor- Motor control is defined as “the systematic transmission of
mal transmission, processing, and perception of information nerve impulses from the motor cortex to motor units, result-
in the PNS and CNS, movement control becomes abnormal, ing in coordinated contractions of muscles.”1
slow, labored, uncoordinated, or weak, or movement may This definition describes motor control in the simplest
not be produced at all. The damaged nervous system is able terms—as a top-down direction of action through the ner-
to repair itself, change, and adapt to some extent by means vous system. In reality, the process of controlling movement
of nerve regeneration and neuroplasticity. However, when begins before the plan is executed, and ends after the mus-
nerve cells die and neural connections are not viable, alter- cles have contracted. The essential details of a movement
native pathways within the nervous system exist to take the plan must be determined by the individual before the actual
place of the normal process and provide some means of execution of the plan. The nervous system actively adjusts
meeting the movement goal—whether it is to walk, use an muscle force, timing, and tone before the muscles begin to
arm to eat, or make a facial expression. This process of contract, continues to make adjustments throughout the mo-
change, healing, or motor learning depends on many factors tor action, and compares movement performance with the
including inherent elements of the individual such as goal and neural code (directions) of the initial motor plan.
age, the extent of tissue damage, and other physiological This extension of the definition takes into account that the
and cognitive processes, as well as external factors such as body accesses sensory information from the environment,
interactions with sensory and motor system challenges, and perceives the situation and chooses a movement plan that it
69
believes to be the appropriate plan to meet the outcome goal of predicting motor responses during patient examination and
the task that the person is attempting to complete, coordinates treatment. They help explain motor skill performance,
this plan within the CNS, and finally executes the plan through potential, constraints, limitations, and deficits. They allow
motor neurons in the brain stem and spinal cord to communi- the clinician to (1) identify problems in motor performance,
cate with muscles in postural and limb synergies, plus muscles (2) develop treatment strategies to help clients remediate
in the head and neck that are timed to fire in a specific manner. performance problems, and (3) evaluate the effectiveness of
The movement that is produced supplies sensory feedback to intervention strategies employed in the clinic. Selecting and
the CNS to allow the person to (1) modify the plan during per- using an appropriate model of motor control is important for
formance, (2) know whether the goal of the task has been the analysis and treatment of clients with dysfunctions of
achieved, and (3) store the information for future performance posture and movement. As long as the environment and task
of the same task-goal combination. Repeated performance demands affect changes in the CNS and the individual
of the same movement plan tends to create a preferred pattern has the desire to learn, the adaptable nervous system
that becomes more automatic in nature and less variable will continue to learn, modify, and adapt motor plans
in performance. If this movement pattern is designed and throughout life.
executed well, then it is determined that the person has
developed a skill. If this pattern is incorrect and does not Motor Programs and Central Pattern Generators
efficiently accomplish the movement goal, then it is considered A motor program (MP) is a learned behavioral pattern defined
abnormal. as a neural network that can produce rhythmic output patterns
with or without sensory input or central control.2 MPs are sets
Theories and Models of Motor Control of movement commands, or “rules,” that define the details of
We begin this section with a summary and historical per- skilled motor actions. An MP defines the specific muscles that
spective of motor control theories (Table 4-1). The control are needed, the order of muscle activation, and the force, tim-
of human movement has been described in many different ing, sequence, and duration of muscle contractions. MPs help
ways. The production of reflexive, automatic, adaptive, and control the degrees of freedom of interacting body structures,
voluntary movements and the performance of efficient, co- and the number of ways each individual component acts. A
ordinated, goal-directed movement patterns involve multiple generalized motor program (GMP) defines a pattern of move-
body systems (input, output, and central processing) and ment, rather than every individual aspect of a movement.
multiple levels within the nervous system. Each model of GMPs allow for the adjustment, flexibility, and adaptation of
motor control that is discussed in this section has both merit movement features according to environmental demands. The
and disadvantage in its ability to supply a comprehensive existence of MPs and GMPs is a generally accepted concept;
picture of motor behavior. These theories serve as a basis for however, hard evidence that an MP or a GMP exists has yet to
TABLE 4-1 n THEORIES OF MOTOR CONTROL
MOTOR CONTROL THEORY AUTHOR AND DATE PREMISE

Reflex Theory Sherrington 1906244 Movement is controlled by stimulus-response. Reflexes are combined into
actions that create behavior.
Hierarchical Theories Adams 1971245 Cortical centers control movement in a top-down manner throughout the
nervous system.
Closed-loop mode: sensory feedback is needed and used to control the
movement.
Open-loop mode: movements are preprogrammed and no feedback is used.
Dynamical Systems Theory Bernstein 196710 Movement emerges to control degrees of freedom.
Turvey 1977246 Patterns of movements self-organize within the characteristics of
Kelso and Tuller 1984247 environmental conditions and the existing body systems of the individual.
Thelen 1987248 Functional synergies are developed naturally through practice and
experience and help solve the problem of coordinating multiple muscles
and joint movements at once.
Motor Program Theory Schmidt 1976249 Adaptive, flexible motor programs (MPs) and generalized motor programs
(GMPs) exist to control actions that have common characteristics.
Ecological Theories Gibson and Pick 2000250 The person, the task, and the environment interact to influence motor
behavior and learning. The interaction of the person with any given
environment provides perceptual information used to control movement.
The motivation to solve problems to accomplish a desired movement task
goal facilitates learning.
Systems Model Shumway-Cook 200735 Multiple body systems overlap to activate synergies for the production
of movements that are organized around functional goals. Considers
interaction of the person with the environment.
CHAPTER 4 n Contemporary Issues and Theories of Motor Control, Motor Learning, and Neuroplasticity 71
be found. Advancements in brain imaging techniques may vestibular, hearing, and taste to temporal lobe). Sensory in-
substantiate this theory in the future.2,3 formation is first received and perceived, then associated
In contrast to MPs, a central pattern generator (CPG) is a with other sensory modalities and memory in the association
genetically predetermined movement pattern.4 CPGs exist cortex. Once multiple sensory inputs are associated with one
as neural networks within the CNS and have the capability another, the person is then able to perceive the body, its
of producing rhythmic, patterned outputs resembling normal posture and movement, the environment and its challenges,
movement. These movements have the capability of occur- and the interaction and position of the body with objects
ring without sensory feedback inputs or descending motor within the environment. The person uses this perceptual in-
inputs. Two characteristic signs of CPGs are that they result formation to create an internal representation of the body
in the repetition of movements in a rhythmic manner and (internal model) and to choose a movement program, driven
that the system returns to its starting condition when the by motivation and desire, to meet a final outcome goal.
process ceases.5 Both MPs and CPGs contribute to the Although the sensory input and motor output systems oper-
development, refinement, production, and recovery of motor ate differently, they are inseparable in function within the
control throughout life. healthy nervous system. Agility, dexterity, and the ability to
produce movement plans that are adaptable to environmen-
The Person, the Task, and the Environment: An tal demands reflect the accuracy, flexibility, and plasticity of
Ecological Model for Motor Control the sensory-motor system.
Motor control evolves so that people can cope with the The CNS uses sensory information in a variety of ways to
environment around them. A person must focus on detecting regulate posture and movement. Before movement is initiated,
information in the immediate environment (perception) that information about the position of the body in space, body parts
is determined to be necessary for performance of the task in relation to one another, and environmental conditions is
and achievement of the desired outcome goal. The individ- obtained from multiple sensory systems. Special senses of
ual is an active observer and explorer of the environment, vision, vestibular inputs that respond to gravity and movement,
which allows the development of multiple ways in which to and visual-vestibular interactions supply additional information
accomplish (choose and execute) any given task. The indi- necessary for static and dynamic balance and postural control
vidual analyzes a particular sensory environment and as well as visual tracking. Auditory information is integrated
chooses the most suitable and efficient way to complete the with other sensory inputs and plays an important role in the
task. The person consists of all functional and dysfunctional timing of motor responses with environmental signals, reaction
body structures and functions that exist and interact with time, response latency, and comprehension of spoken word.
one another. The task is the goal-directed behavior, chal- This information is integrated and used in the selection and
lenge, or problem to be solved. The environment consists of execution of the movement strategy. During movement perfor-
everything outside of the body that exists, or is perceived to mance, the cerebellum and other neural centers use feedback to
exist, in the external world. All three of these motor control compare the actual motor behavior with the intended motor
constructs (person, task, environment) are dynamic and vari- plan. If the actual and intended motor behaviors do not match,
able, and they interact with one another during learning and an error signal is produced and alterations in the motor behavior
production of a goal-directed, effective motor plan. are triggered. In some instances, the control system anticipates
and makes corrective changes before the detection of the error
Body Structures and Functions that Contribute signal. This anticipatory correction is termed feed-forward
to the Control of Human Posture and control. Changing one’s gait path while walking in a busy shop-
Movement ping mall to avoid a collision is an example of how visual in-
Keen observation of motor output quality during the perfor- formation about the location of people and objects can be used
mance of functional movement patterns helps the therapist in a feed-forward manner.
determine activity limitations and begin to hypothesize Another role of sensory information is to revise the refer-
impairments within sensory, motor, musculoskeletal, cardio- ence of correctness (central representation) of the MP before
pulmonary, and other body systems. The following section it is executed again. For example, a young child standing on
presents and defines some of these key factors, including a balance beam with the feet close together falls off of the
sensory input systems, motor output systems, and structures beam. An error signal occurs because of the mismatch
and functions involved in the integration of information in between the intended motor behavior and the actual motor
the CNS. result. If the child knows that the feet were too close
together when the fall occurred, then the child will space the
Role of Sensory Information in Motor Control feet farther apart on the next trial. The information about
Sensory receptors from somatosensory (exteroceptors and what happened, falling or not falling, is used in planning
proprioceptors), visual, and vestibular systems and taste, movement strategies for balancing on any narrow object
smell, and hearing fire in response to interaction with the such as a balance beam, log, or wall in the future.
external environment and to movement created by the body. Sensory information is necessary during the acquisition
Information about these various modalities is transmitted phase of learning a new motor skill and is useful for control-
along afferent peripheral nerves to cells in the spinal cord ling movements during the execution of the motor plan.6-8
and brain stem of the CNS. All sensory tracts, with the ex- However, sensory information is not always necessary when
ception of smell, then synapse in respective sensory nuclei performing well-learned motor behaviors in a stable and
of the thalamus, which acts as a filter and relays this infor- familiar context.6,7 Rothwell and colleagues7 studied a man
mation to the appropriate lobe of the cerebral cortex (e.g., with severe sensory neuropathy in the upper extremity. He
somatosensory to parietal lobe, visual to occipital lobe, could write sentences with his eyes closed and drive a car
with a manual transmission without watching the gear shift. motor neurons in the spinal cord. The ventral corticospinal
He did, however, have difficulty with fine motor tasks such tract system communicates primarily with proximal muscle
as buttoning his shirt and using a knife and fork to eat when groups to provide the appropriate amount of activation to
denied visual information. The importance of sensory infor- stabilize the trunk and limb girdles, thus allowing for dexter-
mation must be weighed by the individual, unconsciously ous distal limb movements. The lateral corticospinal tract
filtering and choosing appropriate and accurate sensory system communicates primarily with muscles of the arms
inputs to use to meet the movement goal. and legs—firing alpha motor neurons in coordinated syn-
Sensory experiences and learning alter sensory represen- ergy patterns with appropriate activity in agonist and an-
tations, or cortical “maps,” in the primary somatosensory, tagonist muscles so that movements are smooth and precise.
visual, and auditory areas of the brain. Training, as well as Other motor nuclei in the brain stem are programmed to fire
use and disuse of sensory information, has the potential to just before corticospinal tract activity in order to supply
drive long-term structural changes in the CNS, including the postural tone. These include lateral and medial vestibular
formation, removal, and remodeling of synapses and den- spinal tracts, reticulospinal tract, and rubrospinal tract sys-
dritic connections in the cortex. This process of cortical tems. Adequate and balanced muscle tone of flexors and
plasticity is complex and involves multiple cellular and extensors in the trunk and limbs occurs automatically, with-
synaptic mechanisms.9 Plasticity in the nervous system is out the need for conscious control. These brain stem nuclei
discussed further in the third section of this chapter. have tonic firing rates that are modulated up or down to ef-
fectively provide more or less muscle tone in body areas
Choice of Motor Pattern and the Control depending on stimulation from gravity, limbic system activ-
of Voluntary Movement ity, external perturbations, or other neuronal activity.
A choice of body movement is made based on the person’s
perception of the environment, his or her relationship to Adaptation
objects within it, and a goal to be met. The person chooses Adaptation is the process of using sensory inputs from mul-
from a collection of plans that have been developed and re- tiple systems to adapt motor plans, decrease performance
fined over his or her lifetime. If a movement plan does not errors, and predict or estimate consequences of movement
exist, a similar plan is chosen and modified to meet the choices. The goal of adaptation is the production of consis-
needs of the task. Once the plan has been chosen it is cus- tently effective and efficient skilled motor actions. When all
tomized by the CNS with what are determined to be the possible body systems and environmental conditions are
correct actions to execute given the perceived situation and considered in the motor control process, it is easy to under-
goal of the individual. stand why there is often a mismatch between the movement
plan that is chosen and how it is actually executed. Errors in
Coordination movements occur and cause problems that the nervous sys-
The movement plan is customized by communications tem must solve in order to deliver effective, efficient, accu-
among the frontal lobes, basal ganglia, and cerebellum, with rate plans that meet the task goal. To solve this problem the
functional connections through the brain stem and thalamus. CNS creates an internal representation of the body and the
During this process specific details of the plan are deter- surrounding world. This acts as a model that can be adapted
mined. Postural tone, coactivation, and timing of trunk and changed in the presence of varying environmental de-
muscle firing are set for proximal stability, balance, and mands. It allows for the ability to predict and estimate the
postural control. Force, timing, and tone of limb synergies differences between similar situations. This ability is learned
are set to allow for smooth, coordinated movements that are by practicing various task configurations in real-life envi-
accurate in direction of trajectory, order, and sequence. The ronments. Without experience, accurate movement patterns
balance between agonist and antagonist muscle activity is that consistently meet desired task goals are difficult to
determined so that fine distal movements are precise and achieve.11
skilled. This process is complicated by the number of pos-
sible combinations of musculoskeletal elements. The CNS Anticipatory Control
must solve this “degrees of freedom” problem so that rapid Anticipatory control of posture and postural adjustments
execution of the goal-directed movement can proceed and stabilizes the body by minimizing displacement of the cen-
reliably meet the desired outcome.10 Once these movement ter of gravity. Anticipatory control involves motor plans that
details are complete the motor plan is executed by the pri- are programmed to act in advance of movement. A com-
mary motor area in the precentral gyrus of the frontal lobe. parison between incoming sensory information and knowl-
edge of prior movement successes and failures enables the
Execution system to choose the appropriate course of action.3
Pyramidal cells in the corticospinal and corticobulbar tracts
execute the voluntary motor plan. Neural impulses travel Flexibility
down these central efferent systems and communicate with A person should have enough flexibility in performance to
motor neurons in the brain stem and spinal cord. The corti- vary the details of a simple or complex motor plan to meet
cobulbar tract communicates with brain stem motor nuclei the challenge presented by any given environmental context.
to control muscles of facial expression, mouth and tongue This is a beneficial characteristic of motor control. When
for speaking and eating, larynx and pharynx for voice and considering postural control, for example, a person will
swallow, voluntary eye movements for visual tracking and typically display a random sway pattern during standing
saccades, and muscles of the upper trapezius for shoulder that may ensure continuous, dynamic sensory inputs to mul-
girdle elevation. The corticospinal tract communicates with tiple sensory systems.12 The person is constantly adjusting
posture and position to meet the demand of standing upright the cerebellum, its tract systems, or its structure creates
(earth vertical), as well as to seek information from the problems of movement coordination, not execution or choice
environment. Rhythmic, oscillating, or stereotypical sway of which program to run. The cerebellum also plays a role
patterns that are unidirectional in nature are not considered in language, attention, and mental imagery functions that are
flexible and are not as readily adaptable to changes in the not considered to take place in motor areas of the cerebral
environment. Lack of flexibility or randomness in postural cortex (see Table 4-2).
sway may actually render the person at greater risk for loss The cerebellum plays four important roles in motor
of balance and falls. control13:
1. Feed-forward processing: The cerebellum receives
Control of Voluntary Movement neural signals, processes them in a sequential order,
Table 4-2 shows the body system processes involved in and sends information out, providing a rapid response
motor control, their actions, and the body structures in- to any incoming information. It is not designed to act
cluded. The following section explains these processes in like the cerebral cortex and does not have the capabil-
more detail. ity of generating self-sustaining neural patterns.
2. Divergence and convergence: The cerebellum receives
Role of the Cerebellum a great number of inputs from multiple body struc-
The primary roles of the cerebellum are to maintain posture tures, processes this information extensively through a
and balance during static and dynamic tasks and to coordi- structured internal network, and sends the results out
nate movements before execution and during performance. through a limited number of output cells.
The cerebellum processes multiple neural signals from 3. Modularity: The cerebellum is functionally divided
(1) motor areas of the cerebral cortex for motor planning, into independent modules—hundreds to thousands—
(2) sensory tract systems (dorsal spinal cerebellar tract, ven- all with different inputs and outputs. Each module
tral spinal cerebellar tract) from muscle and joint receptors appears to function independently, although they each
for proprioceptive and kinesthetic sense information result- share neurons with the inferior olives, Purkinje cells,
ing from movement performance, and (3) vestibular system mossy and parallel fibers, and deep cerebellar nuclei.
information for the regulation of upright control and balance 4. Plasticity: Synapses within the cerebellar system
at rest and during movements. It compares motor plan sig- (between parallel fibers and Purkinje cells, and syn-
nals driven by the cortex with what is received from muscles apses between mossy fibers and deep nuclear cells)
and joints in the periphery and makes necessary adjustments are susceptible to modification of their output strength.
and adaptations to achieve the intended coordinated move- The influence of input on nuclear cells is adjustable,
ment sequence. Movements that are frequently repeated which gives great flexibility to adjust and fine-tune the
“instructions” are stored in the cerebellum as procedural relationship between cerebellar inputs and outputs.
memory traces. This increases the efficiency of its role in
coordinating movement. The cerebellum also plays a role in Role of the Basal Ganglia
function of the reticular activating system (RAS). The RAS The basal ganglia are a collection of nuclei located in the
network exists in the brain stem tegmentum and consists forebrain and midbrain and consisting of the globus palli-
of a network of nerve cells that maintain consciousness dus, putamen, caudate nucleus, substantia nigra, and subtha-
in humans and help people focus attention and block out lamic nuclei. It has primary functions in motor control and
distractions that may affect motor performance. Damage to motor learning. It plays a role in deciding which motor plan
n COMPONENTS OF MOTOR CONTROL: BODY SYSTEM PROCESSES INVOLVED IN MOTOR

TABLE 4-2
CONTROL, THEIR ACTIONS, AND THE BODY STRUCTURES INCLUDED
PROCESS ACTION BODY STRUCTURES INVOLVED

Sensation Sensory information, feedback from exteroceptors Peripheral afferent neurons, brain stem, cerebellum,
and proprioceptors thalamus, sensory receiving areas in the parietal,
occipital, and temporal lobes
Perception Combining, comparing, and filtering sensory inputs Brain stem, thalamus, sensory association areas in the
parietal, occipital, visual, and temporal lobes
Choice of movement Use of the perceptual map to access the appropriate Association areas, frontal lobe, basal ganglia
plan motor plan
Coordination Determining the details of the plan including force, Frontal lobe, basal ganglia, cerebellum, thalamus
timing, tone, direction, and extent of the move-
ment of postural and limb synergies and actions
Execution Execution of the motor plan Corticospinal and corticobulbar tract systems, brain
stem motor nuclei, and alpha and gamma motor
neurons
Adaptation Compare movement with the motor plan and adjust Spinal neural networks, cerebellum
the plan during performance
or behavior to execute at any given time. It has connections Movement Patterns Arising from Self-Organizing
to the limbic system and is therefore believed to be involved Subsystems
in “reward learning.” It plays a key role in eye movements Coordinated movement patterns are developed and refined
through midbrain connections with the superior colliculus via dynamic interaction among body systems and subsys-
and helps to regulate postural tone as a basis for the control tems in response to internal and external constraints. Move-
of body positions, preparedness, and central set. Refer ment patterns used to accomplish a goal are contextually
to Chapter 20 for additional information on the basal appropriate and arise as an emergent property of subsystem
ganglia. interaction. Several principles relate to self-organizing sys-
tems: reciprocity, distributed function, consensus, and emer-
Information Processing gent properties.15
The processing of information through the sensory input, Reciprocity implies information flow between two or
motor output, and central integrative structures occurs by more neural networks. These networks can represent spe-
various methods to produce movement behaviors. These cific brain centers, for example, the cerebellum and basal
methods allow us to deal with the temporal and spatial com- ganglia (Figure 4-2). Alternatively, the neural networks can
ponents necessary for coordinated motor output and allow be interacting neuronal clusters located within a single cen-
us to anticipate so that a response pattern may be prepared ter, for example, the basal ganglia. One model to demon-
in advance. Serial processing is a specific, sequential order strate reciprocity is the basal ganglia regulation of motor
of processing of information (Figure 4-1) through various behavior through direct and indirect pathways to cortical
centers. Information proceeds lockstep through each center. areas. The more direct pathway from the putamen to the
Parallel processing is processing of information that can globus pallidus internal segment provides net inhibitory ef-
be used for more than one activity by more than one center fects. The more indirect pathway from the putamen through
simultaneously or nearly simultaneously. A third and the globus pallidus external segment and subthalamic nu-
more flexible type of processing of information is parallel- cleus provides a net excitatory effect on the globus pallidus
distributed processing.14 This type of processing combines internal segment. Alteration of the balance between these
the best attributes of serial and parallel processing. When the pathways is postulated to produce motor dysfunction.16,17
situation demands serial processing, this type of activity An abnormally decreased outflow from the basal ganglia
occurs. At other times parallel processing is the mode of is postulated to produce involuntary motor patterns, which
choice. For optimal processing of intrinsic and extrinsic produce excessive motion such as chorea, hemiballism,
sensory information by various regions of the brain, a com- or nonintentional tremor. Alternatively, an abnormally in-
bination of both serial and parallel processing is the most creased outflow from the basal ganglia is postulated to pro-
efficient mode. The type of processing depends on the con- duce a paucity of motions, as seen in the rigidity observed
straints of the situation. For example, maintaining balance in individuals with Parkinson disease (see Chapter 20).
after an unexpected external perturbation requires rapid pro- Distributed function presupposes that a single center or
cessing, whereas learning to voluntarily shift the center of neural network has more than one function. The concept
gravity to the limits of stability requires a different combina- also implies that several centers share the same function. For
tion of processing modes. example, a center may serve as the coordinating unit of an
In summary, information processing reinforces and activity in one task and may serve as a pattern generator or
refines motor patterns. It allows the organism to initiate oscillator to maintain the activity in another task. An advan-
compensatory strategies if an ineffective motor pattern tage of distributing function among groups of neurons or
is selected or if an unexpected perturbation occurs. centers is to provide centers with overlapping or redundant
And, most important, information processing facilitates functions. Neuroscientists believe such redundancy is a
motor learning. safety feature. If a neuronal lesion occurs, other centers can
Figure 4-1 n Methods of information processing. Figure 4-2 n Systems model of motor control.
assume critical functional roles, thereby producing recovery Controlling the Degrees of Freedom
from CNS dysfunction.18-22 Combinations of muscle and joint action permit a large
Consensus implies that motor behavior occurs when a number of degrees of freedom that contribute to movement.
majority of brain centers or regions reach a critical threshold A system with a large number of degrees of freedom is
to produce activation. Also, through consensus extraneous called a high-dimensional system. For a contextually appro-
information or information that does require immediate priate movement to occur, the number of degrees of freedom
attention is filtered. If, however, a novel stimulus enters the needs to be constrained. Bernstein10 suggested that the num-
system, it carries more weight and receives immediate atten- ber of degrees of freedom could be reduced by muscles
tion. A novel stimulus may be new to the system, may reflect working in synergies, that is, coupling muscles and joints of
a potentially harmful situation, or may result from the con- a limb to produce functional patterns of movement. The
flict of multiple inputs. functional unit of motor behavior is then a synergy. Syner-
Emergent properties may be understood by the adage gies help to reduce the degrees of freedom, transforming a
“the whole is greater than the sum of its parts.” This concept high-dimensional system into a low-dimensional system.
implies that brain centers, not a single brain center, work For example, a step is considered to be a functional synergy
together to produce movement. An example of the emergent pattern for the lower extremity. Linking together stepping
properties concept is continuous repetitive activity (oscilla- synergies with the functional synergies of other limbs cre-
tion). In Figure 4-3, A, a hierarchy is represented by three ates locomotion (interlimb coordination).
neurons arranged in tandem. The last neuron ends on a re- Functional synergy implies that muscles are activated in
sponder. If a single stimulus activates this network, a single an appropriate sequence and with appropriate force, timing,
response occurs. What is the response if the neurons are ar- and directional components. These components can be rep-
ranged so that the third neuron sends a collateral branch to resented as fixed or “relative” ratios, and the control comes
the first neuron in addition to the ending on the responder? from input given to the cerebellum from higher centers in
In this case (Figure 4-3, B), a single stimulus activates neu- the brain and the peripheral or spinal system and from prior
ron No. 1, which in turn activates neurons No. 2 and No. 3, learning (see Chapter 21).20,22,23 The relative parameters are
causing a response as well as reactivating neuron No. 1. This also termed control parameters. Scaling control parameters
neuronal arrangement produces a series of responses rather leads to a change in motor behavior to accomplish the task.
than a single response. This process is also termed endoge- For example, writing your name on the blackboard exempli-
nous activity. fies scaling force, timing, and amplitude. Scaling is the
Another example of an emergent property is the produc- proportional increase or decrease of the parameter to pro-
tion of motor behavior. Rather than having every MP stored duce the intended motor activity.
in the brain, an abstract representation of the intended goal Coordinated movement is defined as an orderly sequence
is stored. At the time of motor performance, various brain of muscle activity in a single functional synergy or the orderly
centers use the present sensory information, combined with sequence of functional synergies with appropriate scaling of
past memory of the task, to develop the appropriate motor activation parameters necessary to produce the intended mo-
strategy. This concept negates a hardwired MP concept. If tor behavior. Uncoordinated movement can occur at the level
MPs were hardwired and if an MP existed for every move- of the scaling of control parameters in one functional synergy
ment ever performed, the brain would need a huge storage or inappropriate coupling of functional synergies. The control
capacity and would lack the adaptability necessary for com- parameter of duration will be used to illustrate scaling. If
plex function. muscle A is active for 10% of the duration of the motor activ-
ity and muscle B is active 50% of the time, the fixed ratio of
A/B is 1:5. If the movement is performed slowly, the relative
time for the entire movement increases. Fixed ratios also in-
crease proportionally. Writing your name on a blackboard
very small or very large yields the same results—your name.
Timing of muscle on/off activation for antagonistic mus-
cles such as biceps and triceps, or hamstrings and quadri-
ceps, needs to be accurate for coordination and control of
movement patterns. If one muscle group demonstrates a
delayed onset or maintains a longer duration of activity,
overlapping with triceps “on” time, the movement will ap-
pear uncoordinated. Patients with neurological dysfunction
often demonstrate alterations in the timing of muscle activ-
ity within functional synergies and in coupling functional
synergies to produce movement.24,25 These functional move-
ment synergies are not hardwired but represent emergent
properties. They are flexible and adaptable to meet the chal-
lenges of the task and the environmental constraints.
Finite Number of Movement Strategies

The concept of emergent properties could conceivably im-
ply an unlimited number of movement strategies available to
Figure 4-3 n Emergent property. perform a particular task. However, limiting the degrees of
freedom decreases the number of strategies available for functional contracture may be limited in the ability to bend
selection. In addition, constraints imposed by the internal a joint only into a desired range, thereby decreasing the
environment (e.g., musculoskeletal system, cardiovascular movement repertoire available to the individual. Such a con-
system, metabolic activity, cognition) and external environ- straint produces adaptive motor behavior. Dorsiflexion of
ment (e.g., support surface, obstacles, lighting) limit the the foot needs to meet a critical degree of toe clearance dur-
number of movement strategies. Horak and Nashner26 ob- ing gait. If there is a range of motion limitation in dorsiflex-
served that a finite number of balance strategies were used ion, then biomechanical constraints imposed on the nervous
by individuals in response to externally applied linear per- system will produce adaptive motor behaviors (e.g., toe
turbations on a force plate system. With use of a life span clearance during gait). Changes in motor patterns during the
approach, VanSant27 identified a limited number of move- task of rising from supine to standing are observed when
ment patterns for the upper limb, head-trunk, and lower limb healthy individuals wear an orthosis to limit dorsiflexion.28
for the task of rising from supine to standing. The inability to easily open and close the hand with rotation
The combination of these strategies produces the neces- may lead to adaptations that require the shoulder muscula-
sary variability in motor behavior. Although an individual ture to place the hand in a more functional position. This
has a preferred or modal profile, the healthy person with adaptation uses axial and trunk muscles and will limit the
an intact neuromuscular system can combine strategies use of that limb in both fine and gross motor performance.
in various body regions to produce different movement Refer to Chapter 23.
patterns that also accomplish the task. Persons with neuro- Preferred, nonobligatory movement patterns that are sta-
logical deficits may be unable to produce a successful, ble yet flexible enough to meet ever-changing environmental
efficient movement pattern because of their inability to conditions are considered attractor states. Individuals can
combine strategies or adapt a strategy for a given environ- choose from a variety of movement patterns to accomplish
mental change (e.g., differing chair height for sit-to-stand a given task. For example, older adults may choose from a
transitions). variety of fall-prevention movement patterns when faced
with the risk of falling. The choice of motor plan may be
Variability of Movements Implies Normalcy negatively influenced by age-related declines in the sensory
A key to the assessment and treatment of individuals with input systems or a fear of falling. For example, when per-
neurological dysfunction lies in variability of movement and forming the Multi-Directional Reach Test,29 an older adult
in the notion that variability is a sign of normalcy, and ste- may choose to reach forward, backward (lean), or laterally
reotypical behavior is a sign of dysfunction. without shifting the center of gravity toward the limits of
Age, activity level, the environment, constraints of a stability. This person has the capability of performing a dif-
goal, and neuropathological conditions affect the selection ferent reaching pattern if asked, but prefers a more stable
of patterns available for use during movement tasks. When pattern.
change occurs in one or more of the neural subsystems, a Obligatory and stereotypical movement patterns suggest
new movement pattern emerges. The element that causes that the individual does not have the capability of adapting
change is called a control parameter. For example, an in- to new situations or cannot use different movement patterns
crease in the speed of walking occurs until a critical speed to accomplish a given task. This inability may be a result of
and degree of hip extension are reached, thereby switching internal constraints that are functional or pathophysiologi-
the movement pattern to a run. When the speed of the run is cal. The patient who has had a stroke has CNS constraints
decreased, there is a shift back to the preferred movement that limit the number of different movement patterns that
pattern of walking. A control parameter shifts the individual can emerge from the self-organizing system. With recovery,
into a different pattern of motor behavior. the patient may be able to select and use additional move-
This concept underlies theories of development and ment strategies. Cognition and the capability to learn may
learning. Development and learning can be viewed as mov- also limit the number of movement patterns available to the
ing the system from a stable state to a more unstable state. individual and the ability of the person to select and use new
When the control variable is removed, the system moves or different movement patterns.
back to the early, more stable state. As the control variable Obligatory and stereotypical movement patterns also
continues to push the system, the individual spends more arise from external constraints imposed on the organism.
time in the new state and less time in the earlier state until Consider the external constraints placed on a concert violin
the individual spends most of the time in the new state. player. These external constraints include, for example, the
When this occurs, the new state becomes the preferred state. length of the bow and the position of the violin. Repetitive
Moving or shifting to the new, preferred state does not obvi- movement patterns leading to cumulative trauma disorder in
ate the ability of the individual to use the earlier state of healthy individuals can lead to muscular and neurological
motor behavior. Therefore new movement patterns take changes.30-33 Over time, changes in dystonic posturing and
place when critical changes occur in the system because of changes in the somatosensory cortex have been observed.
a control parameter but do not eliminate older, less-preferred Although one hypothesis considers that the focal dystonia
patterns of movement. results from sensory integrative problems, the observable
Motivation to accomplish a task in spite of functional result is a stereotypical motor problem.
limitations and neuropathological conditions can also shift To review, the nervous system responds to a variety of
the individual’s CNS to select different patterns of motor internal and external constraints to develop and execute mo-
behavior. The musculoskeletal system, by nature of the ar- tor behavior that is efficient to accomplish a specific task.
chitecture of the joints and muscle attachments, can be a Efficiency can be examined in terms of metabolic cost to the
constraint on the movement pattern. An individual with a individual, type of movement pattern used, preferred or
habitual movement (habit) used by the individual, and time efficient motor behavior is one key to recovery and an impor-
to complete the task. The term attractor state is used in dy- tant consideration when intervention strategies are devel-
namical systems theory to describe the preferred pattern or oped. This will be discussed further in the next section of this
habitual movement. chapter.
Individuals with neurological deficits may have limited
repertoires of movement strategies available. Patients ex- Motor Control Section Summary
periment with various motor patterns in order to learn the Motor control theories have been developed and have
most efficient, energy-conscious motor strategy to accom- evolved over many years as our understanding of nervous
plish the task. Therapists can plan interventions that help to system structure and function has become more advanced.
facilitate refinement of the task to match the patient’s capa- The control of posture and movement is a complex process
bility, allowing the task to be completed using a variety of that involves many structures and levels within the human
movement strategies rather than limited stereotypical strate- body. It requires accurate sensory inputs, coordinated motor
gies, leading to an increase in function. outputs, and central integrative processes to produce skillful,
goal-directed patterns of movement that achieve desired
Errors in Motor Control movement goals. We must integrate and filter multiple sen-
When the actual motor behavior does not match the intended sory inputs from both the internal environment of the body
motor plan, an error in motor control is detected by the and the external world around us to determine position in
CNS. Common examples of errors in motor control are loss space and choose the appropriate motor plan to accomplish
of balance; inappropriate scaling of force, timing, or direc- a given task. We combine individual biomechanical and
tional control; and inability to ignore unreliable sensory in- muscle segments of the body into complex movement syner-
formation, resulting in sensory conflict. Any one or combi- gies to deal with the infinite “degrees of freedom” available
nation of these errors may be the cause of a fall or error in during the production of voluntary movement. Well learned
performance accuracy. motor plans are stored and retrieved and modified to allow
Errors also occur when unexpected factors disrupt the for flexibility and variety of movement patterns and pos-
execution of the program. For example, when the surface is tures. When the PNS or CNS is damaged and the control of
unreliable (sand, unstable, moving), this will force the indi- movement is impaired, new, modified, or substitute motor
vidual to adapt motor responses to meet the demand of the plans can be generated to accomplish goal-directed behav-
environment. Switching between closed environments (more iors, remain adaptable to changing environments, and pro-
stable) and open environments (more unpredictable) will duce variable movement patterns. The process of learning
challenge the individual to adapt motor responses. When an new motor plans and refining existing behaviors by driving
individual steps off of a moving sidewalk, a disruption in neuroplastic changes in the nervous system is discussed in
walking occurs. The first few steps are not smooth because the next sections of this chapter. The control of posture and
the person needs to switch movement strategies from one balance is also discussed in Chapter 22.
incorporating a moving support surface to one incorporating
a stationary support surface. MOTOR LEARNING
Errors occur in the perception of sensory information, Therapeutic interventions that are focused on restoring func-
in selection of the appropriate MP, in selection of the ap- tional skills to individuals with various forms of neurologi-
propriate variable parameters, or in the response execution. cal problems have been part of the scope of practice of
Patients with neurological deficits may demonstrate a com- physical therapists (PTs) and occupational therapists (OTs)
bination of these errors. Therefore an assessment of motor since the beginning of both professions. These two profes-
deficits in clients includes analysis of these types of errors. sions have emerged with a complementary background to
If a therapist observes a motor control problem, there is no examine, evaluate, determine a prognosis, and implement
guarantee that the central problem arises from within the interventions that empower clients to regain functional con-
motor system. Somatosensory problems can drive motor trol of activities of daily living (ADLs) (e.g., getting out of
dysfunction; cognitive and emotional problems express bed, bathing, walking, and eating, as well as working, play-
themselves through motor output. Thus it is up to the move- ing, and socially interacting) and resume active participation
ment specialist to differentiate the cause of the problem in life after neurological insult. These two professions spe-
through valid and reliable examination tools (see Chapter 8). cialize in the analysis of movement and possess knowledge
Once the cause of the motor problem has been identified, of the scientific background to understand why the move-
selection of interventions should lead to more outcomes. ment is occurring, what strengths and limitations exist
All individuals, both healthy and those with CNS dys- within body systems to produce that movement, and how
function, make errors in motor programming. These errors different therapeutic interventions can facilitate or enhance
are assessed by the CNS and are stored in past memory of the functional movement strategies that remediate dysfunction
experience. Errors in motor programming are extremely use- and ultimately carry over into improved performance of
ful in learning. Learning can be viewed as decreasing the daily activities and participation in life of an individual. PTs
mismatch between the intended and actual motor behavior. and OTs are also knowledgeable about diseases of body
This mismatch is a measure of the error; therefore a decrease systems (neurological, musculoskeletal, integumentary, car-
in the degree of the error is indicative of learning. Errors, diopulmonary, and integumentary systems) and how the
then, are an important part of the rehabilitation process. existence or progression of these pathological states affects
However, this does not mean that the therapist allows the cli- motor performance and quality of life. Consideration and
ent to practice errors over and over. The ability of the patient training of individuals who give assistance and support
to detect an error and correct it to produce appropriate and needed to help clients maintain functional skills during
transitional disease states is also a component of practice same conditions in which the task was practiced. This type
and of treating the client in a holistic manner. of test evaluates the patient’s ability to learn the task. This is
It is therefore important for clinicians to understand how in contrast to transfer tests, in which the patient performs
individuals learn or relearn motor tasks and how learning of the activity under different conditions from those in which
motor skills can best be achieved to optimize outcomes. the skill was practiced. This evaluates the ability of the
Motor learning results in a permanent change in the per- patient to use a previously learned motor skill to solve a dif-
formance of a skill because of experience or practice.34 The ferent motor problem.
end result of motor learning is the acquisition of a new Motor skills can be categorized as discrete, continuous,
movement, or the reacquisition and/or modification of or serial. Discrete motor skills pertain to tasks that have a
movement.35 The patient must be able to prepare and carry specific start and finish. Tasks that are repetitive are classi-
out a particular learned movement36 in a manner that is fied as continuous motor skills. Serial skills involve several
efficient (optimal movement with the least amount of time, discrete tasks connected in a particular sequence that rapidly
energy, and effort),37 consistent (same movement over re- progress from one part to the next.37 The category of a par-
peated trials),38 and transferrable (ability to perform move- ticular motor skill is a major factor in making clinical deci-
ment under different environments and conditions) to be sions regarding the person-, task-, and environment-related
considered to have learned a skill. variables that affect motor learning. This is discussed later
Long-term learning of a particular motor task allows the in the chapter.
patient to use this particular skill to optimize function. This
type of learning is expressed in declarative and procedural An Illustration of Motor Learning Principles
memory. Declarative or explicit memory is expressed by Motor learning is the product of an intricate balance be-
conscious recall of facts or knowledge. An example of this tween the feed-forward and feedback sensorimotor systems
could be the patient verbally stating the steps needed when and the complex central processor—the brain—for the end
going up the stairs with the use of crutches. This is opposed result of acquiring and refining motor skills. People go
to procedural (or nondeclarative) learning, in which move- through distinct phases when they learn new motor skills.
ment is performed without conscious thought (e.g., riding a Observe the sequential activities of the child walking off
bike or rollerblading). The interplay of conscious (cognitive the park bench in Figure 4-4, A through C. A clear under-
and emotional) and unconscious memory affects ultimate standing of this relationship of walking and falling is estab-
learning and may decrease the time needed to learn or re- lished. In frame A, the child is running a feed-forward pro-
learn a functional movement and its use in everyday activity. gram for walking. The cerebellum is procedurally responsible
The ability of an individual to have learned a motor skill for modulating appropriate motor control over the activity
is measured indirectly by testing the ability of a patient to and will correct or modify the program of walking when
perform a particular task or activity both over time and in necessary to attain the directed goal. Unfortunately, a simple
different environmental contexts (performance). The testing correction of walking is not adequate for the environment
must be done over a period of time to determine long-term presented in frame B. The cerebellum has no prior knowl-
learning and minimize the temporary effects of practice. edge of the feedback presented in this second frame and thus
In retention tests, the patient performs the task under the is still running a feed-forward program for stance on the
Figure 4-4 n A, Experiencing the unknown. B, Identifying the problem. C, Solving the problem.
left leg and swing on the right leg. The cerebellum and that the process of learning a motor task occurs in stages.
somatosensory cortices are processing a massive amount of During the initial stages of learning a motor skill, the intent
mismatched information from the proprioceptive, vestibular, of the learner is to understand the task. To be able to develop
and visual receptors. In addition, the dopamine receptors are this understanding requires a high level of concentration and
activated during the goal-driven behaviors, creating a bal- cognitive processing. In the middle and later stages, the in-
ance of inhibition and excitation. Once the executive or dividual learns to refine the movement, improve efficiency
higher cognitive system recognizes that the body is falling and coordination, and perform the skill within different en-
(which has been experienced from falling off a chair or bed), vironmental contexts. The later stages are characterized by
a shift in motor control focus from walking to falling must automaticity and a decreased level of attention needed for
take place. To prepare for falling, the somatosensory system successful completion of the task. It is important to empha-
must generate a sensory plan and then relay that plan to the size early that because the activities performed by a learner
motor system through the sensorimotor feedback loops. The during each stage of learning will be different, the role of the
frontal lobe will tell the basal ganglia and the cerebellum to clinician, the types of learning activities, and the clinical
brace and prepare for impact. The basal ganglia are respon- environment must also be different.
sible for initiating the new program, and the cerebellum The learning model described by Fitts and Posner39
carries out the procedure, as observed in Figure 4-4, C. The consists of a continuous progression through three stages:
child succeeds at the task and receives positive peripheral cognitive, associative, and autonomous.
and central feedback in the process. It is possible that this A learner functions in the cognitive stage at the begin-
experience has created a new procedural program that in ning of the learning process. The person is highly focused
time will be verbally labeled “jumping.” The entire process on the task, is attentive to all that it demands, and develops
of the initial motor learning takes 1 to 2 seconds. Because of an understanding of what is expected and involved in perfor-
the child’s motivation and interest (see Chapter 5), the pro- mance of the skill. Many errors are made in performance;
gram is practiced for the next 30 to 45 minutes. This is the questions are asked; cues, instructions, and guidance are
initial acquisition phase and helps the nervous system store given by the clinician; and demonstrations are found to be
the MP to be used for the rest of the child’s life. If this pro- helpful in this phase of learning. Performance outcomes are
gram is to become a procedural skill, practice must continue variable and inconsistent, but the improvements achieved
within similar environments and conditions. Ultimately the can be profound.
errors will be reduced and the skill will be refined. Finally, During the associative stage the learner refines move-
with practice, the program will enter the retention phase as a ment strategies, detects errors and problem solves indepen-
high-level skill. The skill can be modified in terms of force, dent of therapist feedback, and is becoming more efficient
timing, sequencing, and speed and is transferrable to differ- and reliable at achieving the task goal. The length of time
ent settings. This ongoing modification and improvement are spent in this phase tends to be dependent on the complexity
the hallmarks of true procedural learning. Modifications of the task. The ability to associate existing environmental
within the program will be a function of the plasticity that inputs with motor plans for improved timing, accuracy, and
occurs within the CNS throughout life as the child ages and coordination of activities to accomplish a task goal is im-
changes body size and distribution. Similar plasticity and the proved. Although variability in performance decreases, the
ability to change, modify, and reprogram motor plans will be client continues to explore solutions to best solve a move-
demanded by individuals who age with chronic sensorimotor ment problem.
limitations. Unfortunately, in many of these individuals, the Focused practice with repetition over time leads to the
CNS is not capable of producing and accommodating change, automatic performance of motor skills in the autonomous
which creates new challenges as they age with long-term stage of learning. The individual is in control of the learned
movement dysfunctions (see Chapters 27, 32, and 35). movement plan and is able to use it with little cognitive atten-
tion while involved in other activities. Skills are performed
Stages of Motor Learning with preferred, appropriate, and flexible speed, amplitude,
Several authors have developed models to describe the direction, timing, and force. Consistency of performance is a
stages of motor learning. These models are presented in hallmark of this phase, as is the ability to detect and self-
Table 4-3. Regardless of the model, it is widely accepted correct performance errors. Individuals who do not have the
cognitive skill to remember the learning can go through a
much longer repetitive practice schedule to learn the motor
skill, but there will be very little carryover into other func-
n STAGES OF MOTOR LEARNING—
TABLE 4-3 tional movements or activities.40-42
THREE MODELS In summary, the overall process of the stages of motor
learning as introduced by Fitts and Posner39 suggests that
MOTOR first a basic understanding of a task be established, along
LEARNING STAGE with a motor pattern. Practice of the task then leads to prob-
MODEL ONE STAGE TWO STAGE THREE lem solving and a decrease in the degrees of freedom during
performance, resulting in improved coordination and accu-
Fitts and Posner Cognitive Associative Autonomous
racy. As the learner continues to practice and solves the
(1967)39
motor task problem in different ways and with different
Bernstein (1967)10 Novice Advanced Expert
physical and environmental constraints, the movement plan
Gentile (1998)46 Acquire Develop consistency and
becomes more flexible and adaptable to a wide range of task
the plan adaptability
demands.
Bernstein10 presented a more biomechanical perspective cognitive and physical effort is expended to reach the task
as he addressed the problem of degrees of freedom during goal. Practice in appropriately challenging conditions leads
motor learning. He also broke the motor learning process to consistent, efficient, correct execution while maintaining
down into three stages: novice, advanced, and expert. He adaptive flexibility within the motor plan, allowing the client
proposed that these three stages are necessary to allow a to react quickly to changing conditions of the task.
learner to reduce the large number of degrees of freedom The three motor learning theories just presented simplify
that are inherent in the musculoskeletal system, including a complex process into simple stages to give a broad picture
structure and function of muscles, tendons, joints. He proof the development of skilled movement performance. Each
posed that as a person learns a new motor skill, he or she theory can be used to assist the therapist in the process of
gains coordination and control over the multiple interacting teaching and facilitating long-term learning or relearning of
variables that exist in the human body to master the target motor skills before and after insult to the nervous system.
skill. The ultimate goal of motor learning is the permanent acqui-
The novice stage is defined by the coupling of movement sition of adaptable movement plans that are efficient, require
parameters—degrees of freedom—into synergies. During little cognitive effort, and produce consistent and accurate
this stage some joints and movements may be “frozen” or movement outcomes.
restrained to allow successful completion of the task. An
example of this is posturally holding the head, neck, and Variables that Affect Motor Learning
trunk rigid while learning to walk on a narrow surface. The ecological model (constraints theory) of motor control
The advanced stage is achieved by combining body parts and learning states that motor learning involves the person,
to act as a functional unit, further reducing the degrees of the task, and the environment.47 For a purposeful and func-
freedom while allowing better interaction and consideration tional movement to occur, the individual must generate
for environmental factors. He considers that motor plans movement to successfully meet the task at hand, as well as
must be adapted to the dynamic environmental conditions in the demands of the environment where the task must be
which the task must be performed. In this stage the learner performed. For motor learning to be successful, several vari-
explores many movement solutions, reduces some degrees ables related to each of these three constructs must be taken
of freedom, develops more variable movement patterns, and into account.
learns to select appropriate strategies to accomplish a given
task. This stage of motor learning is accomplished through Variables Related to the Individual
practice and experience in performing a task in various en- The clinician must first differentiate general motor perfor-
vironments. To achieve this stage the learner progressively mance factors that are under the control of the individual’s
releases some couplings, allowing more degrees of freedom, cognitive and emotional systems and those that are con-
greater speed and amplitude of movement, and less controlled by the motor system itself. These concepts are pre-
straints on the action. Performance of the task becomes sented in Figure 4-5. There are many cognitive factors such
more efficient, is less taxing on the individual, and is exe- as arousal, attention, and memory, as well as cortical path-
cuted with decreased cognitive effort. Variability of perfor- ways related to declarative or executive learning, that have
mance becomes an indicator that a level of independence in specific influences over behaviors that are observed after
the activation of component body parts during a given task neurological insult.48,49 Other factors such as limbic connec-
has indeed been achieved. tions to cortical pathways affected by motivation, fear and
In Bernstein’s expert stage, degrees of freedom are now belief, and emotional stability and instability also dramati-
released and reorganized to allow the body to react to all of cally affect motor performance and declarative learning.
the internal and external mechanisms that may act on it at Some of these factors may also limit activity and participa-
any given time. At the same time, enhanced coactivation of tion. Therapists need to learn how to discriminate among
proximal structures is learned and used to allow for greater motor output, somatosensory input, cortical processing, and
force, speed, and dexterity of limb movements.43 limbic emotional state problems and identify how the latter
Gentile presented a two-stage model of motor learn- two systems affect motor output. With that differentiation,
ing.44,45 She considered motor learning from the goal of the
learner and strongly considered how environmental condi-
tions influence performance and learning.
Stage one requires the client to problem solve strategies GENERAL FACTORS AFFECTING MOTOR LEARNING
to get the idea of a movement and establish a motor pattern
that will successfully meet the demands of the task. As with • Arousal
the models presented previously, this process demands con- • Attention
scious attention to the components of the task and environ- • Motivation
• Memory: declarative vs. procedural
mental variables to formulate a “map” or framework of the - Verbal
movement pattern. Once this framework is established, the - Visual
client has a mechanism for performing the task; however, - Kinesthetic • Type of movement required
errors and inconsistency in performance accuracy are often • Practice schedule used
present.46 • Type of practice
• Type of reinforcement
During stage two the client attains improved consistency • Environmental context
of performance and the ability to adapt the movement pat-
tern to demands of specific physical and environmental situ-
ations. Greater economy of movement is achieved, and less Figure 4-5 n Concepts affecting motor learning.
clinicians should also be able to separate specific motor

TYPE OF MOVEMENT (TASK)/PRACTICE ENVIRONMENT
system deficits from motor control problems arising from
dysfunction within other areas of the CNS. Last, the
patient’s fitness level; current limitations in strength, endur- Whole learning Pure-part learning
ance, power, and range of motion; or pain level may also Practice entire task Parts learned separately
influence learning.35,50-55 at one time. and then put together
Variables Related to the Task Progressive-part Whole-part learning

learning
The two major variables related to the task itself that must Practice between whole
Components of skill and parts
be considered when facilitating motor learning include prac- combined in sequence. May go through whole
tice of the task and feedback related to task performance. practice parts whole, etc.
Practice. Practice is defined as “repeated performance in
order to acquire a “skill.”56 As the definition implies, several
repetitions of the task are usually required to be able to Basic principles
achieve skillful performance of a task. With other variables • Simple and discrete task: Whole learning
being constant, more practice results in more learning.35 To • Complex skills: Whole Part Whole
be effective, these repetitions must involve a process of Demands on memory and
problem solving rather than just repetition of the activity.57 What is complexity of task? information processing
The therapist can manipulate several variables related to Task organization:
practice to optimize motor learning of an individual with a number of separate components
movement dysfunction secondary to a neurological insult.
Practice Conditions. The term practice conditions refers • Intermediate skill and serial tasks: Progressive-part
to the manner in which the task or exercise is repeated with
respect to rest periods, the amount of exercise, and the Figure 4-6 n Type of movement (task) and practice environment.
sequence in which these tasks or exercises are performed.
According to apportionment of practice in relation to rest
periods, massed tasks or exercises can be classified as Whole learning suggests that the learner practice the en-
massed practice or distributed practice. Massed practice is tire movement as one activity. Asking a person to stand up
when the rest period is much shorter in relation to the incorporates the entire activity of coming to stand from sit-
amount of time the task or exercise is practiced.58 This is ting. Simple movements such as rolling, coming to sit, com-
contrasted against distributed practice, in which the time ing to stand, and walking might best be taught as a whole
between practice sets is equal to or greater than the amount activity as long as the individual has all the component parts
of time devoted to practicing a particular task or activity, to practice the whole.
such that the rest period is spread out throughout the prac- In pure-part learning the therapist introduces one part
tice.59 In terms of neurological physical therapy practice, it first, then this part is practiced by the learner before another
is important to consider the effect of physical and mental new part is introduced and practiced. Each part is critical to
fatigue when training. For example, physical fatigue sets in the whole movement, but which one is learned first does not
during massed practice of a particular balance exercise ac- matter. Learning a tennis serve is an excellent example of an
tivity in standing and may cause a patient to fall. Moreover, activity that can be taught as a pure-part. Learning to toss
individuals who are cognitively impaired may not respond the ball vertically to a specific spot in space is a very differ-
positively to sustained activity that requires considerable ent and a separate part from swinging the tennis racket as
concentration and therefore might fail in the performance of part of the serve. Learning to squeeze the toothpaste onto the
the skill. On the other hand, to be functional and useful in brush is a very different movement strategy from brushing
daily life, certain activities have to be performed without the teeth.
significant amounts of rest periods. For example, taking Progressive-part learning is used when the sequence
significant rest breaks when ambulating for even a short of the learning and the component parts need to be pro-
distance limits an individual’s ability to use walking in a grammed in a specific order. Line dancing is an activity
functional manner. Sometimes a patient needs more rest taught using progressive parts. Individuals with sequencing
periods in the initial stages of learning a skill to compensate deficits often need to be taught using progressive parts or the
for impairments in muscular endurance or cardiopulmonary individual will mix up the ordering of parts during an activ-
function, with the intent of decreasing these rest periods to ity. Therapists see this in the clinical arena when an indi-
achieve skill performance that reflect how that activity is vidual stands up from a wheelchair and then tries to pick up
used in real-life situations. Therefore therapists should con- the foot pedals and lock the brakes. Given that problem, that
sider the skill demands and the desired results when choos- patient needs to practice progressive part learning by first
ing one practice type versus another.59 locking the brake, then picking up the foot pedals and finally
Complete tasks or activities can usually be divided into standing. If the activity is not practiced using progressive-
smaller subcomponents. The way those subcomponents are part learning, the patient will have little consistency in how
practiced relative to the entire task or activity can also be the parts are put together, thereby placing that individual at
manipulated to optimize motor learning. To practice the high risk of failing at the functional task.
entire task or parts of the task, whole learning, pure-part Whole-part learning can be used when the skill or activ-
learning, progressive-part learning, or whole-part learning ity can be practiced between the whole and the parts. In the
may be used. Figure 4-6 summarizes these concepts. clinical environment, a common application of this concept
is whole to part to whole learning.60 First the therapist has task. Internal feedback pertains to sensory information that
the client try the whole activity, such as coming to stand or the patient receives that can be used to improve performance
reaching out to turn the door handle. Next, the therapist has of that particular task or activity in the future. The therapist
the client practice a component part. Finally the whole activ- provides extrinsic or augmented feedback with the intent
ity is practiced as a functional pattern. In this way therapists of improving learning of the task. In people with neurologi-
work on the functional activity, then work on correcting the cal dysfunctions, extrinsic feedback is important because
impairment or limitation, such as power production, range, the patient’s intrinsic feedback system may be impaired or
or balance, and then go back to the functional activity in absent.
order to incorporate the part learning into the whole. An Extrinsic feedback can further be classified as knowledge
example might be asking a patient to first stand up from a of performance (KP) or knowledge of results (KR). KP is
chair. As he tries to stand he generates too much power, given concurrently while the task is being performed and
holds his breath, and cannot repeat the activity more than can therefore also be called concurrent feedback. Feedback
once. The therapist decides to practice a component part by given concurrently, especially during the critical portions
first assisting the patient to a relaxed standing posture, then of the task, allows the patient to successfully perform the
having him eccentrically begin to sit into a partial squat, and activity.
then having him return to standing. As the patient practices, KR pertains to feedback given at the conclusion of the
he will increase the range of lengthening and eventually will task (therefore also called terminal feedback) and provides
sit and return to stand. Once that is accomplished, he will the patient information about the success of his or her
continue to practice sit to stand to sit to stand as a whole actions with respect to the activity. KR can be classified as
activity. faded, delayed, or summary. In faded feedback the therapist
According to the sequence in which component tasks are provides more information in the beginning stages of learn-
practiced, blocked or random practice may be used. In ing of the skill and slowly withdraws that information as
blocked practice the patient first practices a single task over the patient demonstrates improvement in the performance of
and over before moving to the next task. On the other hand, the task. With delayed feedback, information is given to the
in random practice, the component tasks are practiced with- patient when a period of time has elapsed after the task has
out any particular sequence. The contextual interference ef- been completed. The intent of this pause between the termi-
fect explains the difference in motor performance found nation of task and feedback is to give the patient some time
when comparing these two types of practice. Studies have to process the activity and generate possible solutions to the
shown performance may be enhanced by using blocked difficulties encountered in the previous performance of
practice; however, learning is not enhanced by using this the task. In contrast, summary feedback is provided after the
type of practice. Random practice has been shown to en- patient has performed several trials of a particular task with-
hance learning because this type of practice forces the learn- out receiving feedback. Previous studies showed that sub-
ers to come up with a motor solution each time a task is jects who were given more frequent feedback performed
performed.61,62 better during the task acquisition stage of learning but worse
Feedback. The use of feedback is another important on retention tests compared with those who received sum-
variable related to motor learning. Feedback is defined as mary feedback.63,64
the use of sensory information—visual, auditory, or somato- Additional concepts related to long-term learning are
sensory—to improve performance, retention, or transfer of a presented in Figure 4-7.
CONCEPTS IMPORTANT TO LONG-TERM MOTOR LEARNING
Variability in practice
• Variable practice increases the applicability or generalizability
Block vs. random practice

Blocked design: Random design:
Initial Performance
outcome Retention initially Retention Learning
Mental practice
Guidance
• Immediate reinforcement = high performance/decreased retention
• Intermittent reinforcement = lower performance/higher retention
Thus... error is necessary for learning to occur.
There is a need to use inherent mechanisms to self correct.
Figure 4-7 n Concepts important to long-term learning.

Variables Related to the Environment performance, require the client to solve problems to meet
Therapists can alter the environmental conditions to opti- the demands presented, and allow a level of success that
mize motor learning. Gentile44,45 described the manipulation inspires continued motivation to practice and achieve a
of the environment in which a task is performed to make an higher standard of skill.
activity more appropriate for what the patient is able to do.
A closed environment is stationary; it allows the patient to Systems Interactions: Motor Responses
practice the skill in a predictable manner, with minimal Represent Consensus of Central Nervous
distractions from the environment. On the other hand, an System Components
open environment is one that is in motion or unpredictable. Motor behavior reflects not only motor programming but
In patients with neurological dysfunctions, clinicians may also the interaction of cognitive, affective, and somatosen-
decide to have a patient practice a skill in a closed environ- sory variables. Without a motor system, neither the cogni-
ment to allow the patient to plan the movement in advance tive nor the emotional systems have a way to express and
and to perform the movement with minimal distractions or communicate inner thoughts to the world. The cognitive
challenges. An example of this would be performing gait and emotional systems can positively or negatively affect
training in a quiet and empty therapy gym. As the patient motor responses. The significance of the somatosensory or
improves, it may be important to practice this activity in an perceptual-cognitive cortical system must be emphasized.
open environment to provide a real-world application of a The somatosensory association areas play a critical role in
task. Going back to the previous example, the therapist may the ideational and constructional aspects of the MP itself.
have the patient ambulate in an open environment such as a When there are deficits within this system, clients will often
busy gym with crowds and noise, a crowded cafeteria, or a demonstrate significant distortions in motor control even
moving walkway. without a specific motor impairment. An example of this
If prior procedural learning has occurred, then creating problem might be an individual who had a stroke and devel-
an environment that allows the program to run in the least oped a “pusher syndrome.” The motor behavior shown by
restrictive environment should lead to the most efficient this client would be pushing off vertical generally in a lateral
outcome in the shortest time.51,52 If a patient needs to learn or posterolateral direction.67 Physically correcting the cli-
a new program, such as walking with a stereotypical exten- ent’s posture to vertical or asking the patient to self-correct
sion pattern, then goal-directed, attended practice with will not eliminate the original behavior. Pusher syndrome
guided feedback is necessary. It may be easier to bring back does not stem from a motor problem but rather from a per-
an old ambulatory pattern by creating an environment to ceptual problem of verticality from thalamic nuclei radiating
elicit that program than to teach a client to use a new inef- false information to the somatosensory cortices. Although a
ficient movement program.53-55 therapist might want to augment intervention by trying to
A therapist must identify what MPs are available and un- push the patient to vertical, the patient will resist that move-
der what conditions. This allows the therapist to (1) determine ment pattern. Functional training becomes frustrating to
whether deficits are present, (2) anticipate problems in perfor- both the patient and the therapist because the impairment
mance, and (3) match existing programs with functional does not fall within the motor system itself. Reliance on the
activities during training. Similarly, knowing available MPs use of vision and environmental cues might be the best in-
and the component body systems necessary to run those tervention strategy for this type of problem because the im-
programs aids the therapist in the selection of intervention pairment is within the sensory processing centers.68 Asking
procedures. the patient to find midline and reach across midline, then
If the client has permanent damage to either the basal gan- acknowledging success, along with a lack of falling help the
glia or the cerebellum, then retaining the memory of new MPs somatosensory system to relearn and thus begin to inher-
may be difficult and substitution approaches may become ently correct to vertical. Verbalizing to the patient that you
necessary. Through evaluation the clinician needs to deter- (the therapist) acknowledge that she or he feels as if she or
mine whether anatomical disease or a pathological condition he is falling when placed in the vertical position demon-
is actually causing procedural learning problems and whether strates to the client that you have accepted the patient and
identifying and teaching a substitution pattern or teaching his or her perceptions. Simultaneously maintaining tactile
the patient to compensate with an old pattern will allow the contact to prevent the patient from falling effectively lets the
individual to succeed at the task. However, therapists should limbic system relax and reduces its need to trigger motor
never forget that the plasticity of the CNS can promote reactions. This example creates conflict between the cogni-
significant recovery and adaptation through the performance tive system’s information from the thalamus and motor
of attended, goal-directed, repetitive behavior.65,66 system feedback. The thalamus is saying vertical is “X,” and
Providing an appropriate level of challenge to the learner the motor system is saying “if X then I am falling.” When
optimizes motor learning. The clinician must learn to ex- the goal is not to fall, then the cognitive system will gener-
pertly manipulate the environment to best facilitate learning. ally override the thalamic information and learn to accept a
A task that is too difficult for the client will result in persis- new concept of vertical. Taking all these variables into the
tent failure of performance, frustration, and lack of learning, treatment environment optimizes the potential that the pa-
and the only option will be to compensate through available tient will self-correct during a functional activity such as
patterns of movement that limit function. An activity that reaching with weight shift.
is too easy and routinely results in 100% success also does If a patient’s insult falls within the limbic or emotional
not result in learning because the learner becomes bored system, then motor behavior could also be affected. The mo-
and no longer attends to the learning. The most beneficial tor dysfunction will be different from the dysfunction re-
level of challenge for training will create some errors in flecting damage either in the sensory cortices or associated
with information sent to them. For years it has been com- of basic science findings with patients.69-71 Clinicians cannot
mon knowledge that individuals who are depressed will simply provide the same, familiar treatment of yesterday
demonstrate motor signs of withdrawal (e.g., flexion). If the because it is comfortable and easy and requires minimal
posture of flexion was created by a chemical response re- effort. Physical therapy professionals must be dynamic,
lated to depression, then somatosensory retraining would enthusiastic, evidence-based and committed to lifelong
have a limited effect on behavior. Similarly, functional train- learning, ready to accept the challenge and unique opportu-
ing may initially modify the impairments, but without nity to work with other members of the health care team to
changes within the limbic system itself no permanent change translate neuroscience to practice. Failure to translate basic
will be achieved. Instead, augmenting the input to alter the science findings into clinical practice will significantly
emotional system and then reinforcing self-control could impair the potential for patient recovery.
create the best potential outcome. During the last 45 years, three large conferences72-74
For many clinical problems, functional retraining of the focused on these issues in neuroscience. In 1966 the North-
motor system through attended, sequenced, repetitive prac- western University Special Therapeutic Exercise Project
tice could lead to greater functional gains, although the body (NUSTEP) conference in Chicago, Illinois, brought re-
system impairment(s) may never be eliminated. That is, searchers, basic scientists, educators, and master clinicians
muscle strengthening and programming coactivation to en- together for 6 weeks to identify commonalities in ap-
able joint stability could restore client independence. Given proaches to interventions and to integrate basic science into
the complexity of impairments and function in a patient with those commonalities. A huge shift from specific philoso-
a neurological insult, a therapist may need to use all three phies to a bodily systems model occurred in 1990 at
types of intervention procedures to affect all areas of the Norman, Oklahoma, the site of the Second Special Thera-
CNS simultaneously. The decision of which intervention is peutic Exercise Project conference (II STEP). During the
most appropriate or which should be emphasized falls next 15 years, concepts of motor learning and motor control
within the professional judgment of the clinician. There is were beginning to affect the methodology and intervention
no easy recipe to decide which intervention is best for all philosophies of both occupational and physical therapy.
people. It is the problem-solving skills of the therapist and Simultaneously, newer approaches such as locomotion train-
one’s keen analysis of movement function and dysfunction ing with partial weight bearing on a treadmill,75,76 task-
that lead to the best solution. Obviously, patient involvement specific training,77,78 constraint-induced movement train-
and desired outcomes are also critical components leading ing,79,80 neuroprotective effect of exercise,81 mental and
to this decision. physical practice,82,83 patient-centered therapy,84-86 and sen-
sorimotor training87 were frequently seen in peer-reviewed
literature. The third STEP conference, Summer Institute on
PRINCIPLES OF NEUROPLASTICITY:
Translating Evidence into Practice (III STEP), occurred in
IMPLICATIONS FOR NEUROREHABILITATION
July 2005 in Salt Lake City, Utah. At this conference, unique
Rehabilitation, Research, and Practice clinical models for intervention were embraced that will
Rehabilitation is the process of maximizing functional learn- direct professional education for decades. Changes in prac-
ing. The integration of basic neuroscience into clinical practice over the next 15 years will lead to embracing many
tice is critical for guiding the questioning of researchers and older intervention techniques with current evidence-based
maximizing the recovery of patients. The 1990s were re- practice.
ferred to as the “Decade of the Brain.” For the last 20 years, Four primary conclusions were summarized from the III
researchers have made enormous advances in understanding STEP conference: (1) client-centered, empowerment mod-
the adaptability of the CNS. Because of this revolution, cli- els needed to be the platform for all neurorehabilitation and
nicians must focus on recovery rather than compensation. postdisease models of care; (2) evidence-based practice
There is sufficient evidence that the CNS not only develops needs to start with the documentation of clinical effective-
and matures during adolescence, but also recovers from seri- ness based on reliable and valid measurement tools followed
ous disease and injury and maintains sensory, motor, and by efficacy studies; (3) a strong link is needed among basic
cognitive competency through spontaneous healing, appro- science, clinical science, and disease-specific motor dys-
priate medical management, physical exercise, balanced function research to develop the best patient management
nutrition, and learning. Across the life span, individuals can environments; and (4) movement science belongs to a broad
maximize independence and quality of life by taking advan- community that requires integration of the goals, cultural
tage of learning from enriched environments, task-specific beliefs, ethnic values, emotional understanding, and scien-
training, and attended, progressive, goal-oriented, repetitive tific knowledge of many individuals, including but not lim-
behaviors. In addition, the nervous system can adapt nega- ited to health care providers (physicians, PTs and OTs,
tively to repetitive and abnormal patterns of movement psychologists), clinical research practitioners, basic science
based on structural anomalies, pain, abnormal biomechan- researchers, educators, clients, families, and employers.
ics, or bad habits (see the section on motor learning in this There are a variety of challenges to implementing effec-
chapter). tive, neuroscience-based interventions. The first is the pa-
The paradigm shift in rehabilitative intervention strate- tient. Patient-centered therapy is critical for effective thera-
gies based on neuroplasticity has just begun. Basic science peutic outcomes. The patient can be both the obstacle to
researchers cannot ignore the impact of their findings on the successful recovery88,89 and the critical link to success.90,91
health and function of the consumer. Clinical researchers To achieve optimum neural adaptation, the patient must
must collaborate in clinical studies to determine the impact be engaged in attended, goal-directed, novel, progressive
behaviors. There is no measurable neural adaptation with to plan, coordinate, and execute movements. The task of the
passive movements or passive stimuli. For a change in neu- motor systems in controlling movement is the reverse of the
ral response to be achieved, the stimulus needs to be novel task of sensory systems in generating an internal representa-
or a surprise and the individual has to attend to the stimulus, tion. Perception is the end product of sensory processing,
make a decision about what to do, and receive some feed- whereas an internal representation (an image of the desired
back regarding the appropriateness or accuracy of the out- movement) is the beginning of motor processing.
come.92 This progressive decision making has to be done Sensory psychophysics looks at the attributes of a stimu-
repetitively and progressed in difficulty over time. These lus: its quality, intensity, location, and duration. Motor psy-
behaviors may be difficult to achieve when a person is chophysics considers the organization of action, the inten-
depressed, feels hopeless, lacks motivation or cognition, or sity of the contraction, the recruitment of distinct populations
has emotional instability or there is neglect of one or more of motor neurons, the accuracy of the movements, the coor-
parts of the body. dination of the movements, and the speed of movement. In
Another obstacle to bringing scientific evidence into both the sensory and motor systems, the complexity of
practice is the barrier created by living in a society in behaviors depends on the multiplicity of modalities avail-
which the economics of health care rather than the science able. In sensation, there are the distinct modalities of pain,
or the patient benefits drive the delivery of services (see temperature, light touch, deep touch, vibration, and stretch,
Chapter 10). When a physician or a therapist recommends whereas in the motor system can be found the modalities
a new approach to intervention, the third-party payer may of reflex responses, rhythmic motor patterns within and be-
deny payment for service because it is “experimental.” tween limbs, automatic and adaptive motor responses, and
Furthermore, third-party payers may deny the opportunity voluntary fine and gross movements.96-116 Although all
to apply findings from animal studies to human subjects. motor movements require integration of sensory informa-
Another example of constraint from the third-party payer tion for motor learning, once motor control is attained the
is the timing of intervention. Despite the evidence that the system can run on very little feedback. The relationship of
CNS can be modified under conditions of goal-oriented, incoming sensory information is particularly complex in
repetitive, task-relevant behaviors even years poststroke, voluntary motor movements that constantly adapt to envi-
insurance companies deny coverage of service late in the ronmental variance. For voluntary motor movements, the
recovery process. The insurance company may interpret motor system requires contraction and relaxation of mus-
“medically necessary services” as the services provided cles, recruitment of appropriate muscles and their synergies,
during the first 30 days postinjury, the time after a cerebro- appropriate timing and sequencing of muscle contraction
vascular accident when the greatest spontaneous recovery and relaxation, the distribution of the body mass, and ap-
occurs. Furthermore, even though neural adaptation re- propriate postural adjustments. As stated, once an MP is
search confirms that enriched environmental conditions learned, it does not take the same amount of sensory infor-
and sensory inputs can facilitate both greater and contin- mation to run the program in a feed-forward manner within
ued recovery, the insurance company may claim that the the motor system as long as the information to the cerebel-
services93-95 are simply for maintenance. Thus, as the sci- lum is able to run and adjust all aspects of the program. (See
ence of neuroplasticity continues to develop, it is critical to Chapter 21 and the section on motor control in this chapter.)
improve the interface among the scientist, the practitioner, To learn new programs, the CNS must go through the pro-
the patient, and the third-party payer. Clinicians and recess of receipt of sensory input, perceptual processing, com-
searchers must regularly inform third-party payers about munication with the frontal lobes, and relays to basal gan-
current research evidence. glia and cerebellum, followed by intentional, goal-directed
execution of the motor plan.
Integration of Sensory Information Within each movement, there must be adjustments to
in Motor Control compensate for the inertia of the limbs and the mechanical
Understanding neural adaptation must include attention to arrangement of the muscles, bones, and joints both before
sensory as well as motor systems. In virtually all higher-order and during movement to ensure and maintain accuracy. The
perceptual processes, the brain must correlate sensory input control systems for voluntary movement include (1) the
with motor output to assess the body’s interaction with the continuous flow of sensory information about the environ-
environment accurately. A problem in the somatic motor sys- ment, position, and orientation of the body and limbs and
tem affects the motor output system. Both systems are inde- the degree of contraction of the muscles; (2) the spinal cord;
pendently adaptive, but functional neural adaptation involves (3) the descending systems of the brain stem; and (4) the
the interaction of both sensory and motor processing. pathways of the motor areas of the cerebral cortex, cerebel-
The sensory system provides an internal representation of lum, and basal ganglia. Each level of control is based on
both the inside and outside worlds to guide the movements the sensory information that is relevant for the functions it
that make up our behavioral repertoire. These movements are controls. This information is provided by feedback, feed-
controlled by the motor systems of the brain and the spinal forward, and adaptive mechanisms. These control systems
cord. Our perceptual skills are a reflection of the capabilities are organized both hierarchically and in parallel. These
of the sensory systems to detect, analyze, and estimate the systems also control activation of sensations and motor
significance of physical stimuli. (See the section on aug- movements as well as inhibition (e.g., globus pallidus). Fur-
mented therapeutic intervention in Chapter 9 for a detailed thermore, some parts of the brain are needed for new learn-
discussion of each sensory system.) Our agility and dexterity ing (e.g., cerebellum) and others for maintained learning
represent a reflection of the capabilities of the motor systems (e.g., globus pallidus, hippocampus). The hierarchical but
interactive organization permits lower levels to generate To understand neural adaptation and to be able to apply the
reflexes without involving higher centers, whereas the paral- principles to practice, it is necessary to objectively measure
lel system allows the brain to process the flow of discrete the changes. Positive changes in neural structure can be mea-
types of sensory information to produce discrete types of sured by using a variety of imaging techniques (e.g., magnetic
movements.117,118 resonance imaging [MRI], functional MRI [fMRI], magneto-
Ultimately, the control of graded fine motor movements encephalography, magnetic source imaging [MSI]). The types
involves the sensory organ of the muscle, the muscle spin- of outcomes that can be expected electrophysiologically and
dle, which contains the specialized elements that sense functionally are summarized in Table 4-4. At this time, imag-
muscle length and the velocity or changes in spindle length. ing techniques are applied primarily for research purposes or
In conjunction with the tendon organ, which senses muscle to rule out other pathology. The specific type of intervention
tension, the muscle spindle provides the CNS with continu- to address the principles of neuroplasticity may vary, but the
ous information on the mechanical state of the muscle. outcomes must be clearly documented.
Ultimately the firing of the muscle spindles depends on both
muscle length and the level of gamma motor activation of Principles of Neural Adaptation
the intrafusal fibers. Similarly, joint proprioceptors relay To achieve maximum neural adaptation, there are some basic
both closed and open chain input and mobility (range) infor- principles to follow (Box 4-1). Learning is the key to neural
mation from within the joint structures to the CNS. This adaptation. Plasticity is the mechanism for encoding, the
illustrates the close relationship between sensory and motor changing of behaviors, and both implicit and explicit learning.
processing and the integral relationship between the two.119 During neural adaptation, the fundamental questions are
as follows: As we learn, how does the brain change its repre-
Foundation for the Study of Neuroplasticity sentations of inputs and actions? What is the nature of the
The principal models for studying cortical plasticity have processes that control the progressive elaboration of perfor-
been based on the representations of hand skin and hand mance abilities? In different individuals, what are the sources
movements in the New World owl monkey (Aotus) and the of variance for emergence of improved performance? What
squirrel monkey (Saimiri). These primate models have been changes in cortical plasticity facilitate the development of
chosen because their central sulci usually do not extend into “automatic” motor behaviors? Why are some behaviors hard
the hand representational zone in the anterior parietal (S1)
or posterior frontal (M1) cortical fields. In other primates the
sulci are deep and interfere with accurate mapping. Albeit n NEUROPROTECTIVE MOTOR
TABLE 4-4
there are differences in hand use among primates, in all of ENRICHMENT FACTORS AFFECTING OUTCOMES
the primates the hand has the largest topographical represen-
tation for the actual size of the extremity, the detail of this NEGATIVE PLASTICITY POSITIVE PLASTICITY
representation is distinct, and the hand has the greatest po-
Stimulation Disuse, unskilled Intensive, skilled
tential for skilled movements and sensory discrimination.
Quality of Noisy, nonspecific Appropriate, specific
However, the findings from studies of this cortical area are
sensory
applicable across the different cortices as well as the other
input
cornerstones of the brain such as the thalamus, basal gan-
Modulation Not challenging High stakes, novel,
glia, brain stem, and cerebellum.120,121 See Figure 4-8 to
challenging
identify specific anatomical locations and their respective
Outcome Negative behaviors Positive behaviors
classifications.
Figure 4-8 n Classification and anatomical locations of cortical map.

to change? What limits plasticity processes? What are the strategies contribute to or interfere with restoring lost neuro-
critical elements of brain circuitry, genes, synapses, neural nal function? How does the unaffected side contribute to or
chemistry, neuronal networks, and neural connections for interfere with neuroplastic changes and restoration of func-
restoration of lost function? What guidelines need to be fol- tion? Does damage to the brain alter the neuronal response
lowed to drive the greatest change in brain structure and to learning (e.g., cascade of cellular activity for healing
function? How do spontaneous compensatory behavioral altered circuitry, new neural connections)?
BOX 4-1 n NEUROPLASTICITY PRINCIPLES TRANSLATED TO GUIDE CLINICAL PRACTICE
BASIC PRINCIPLES INTEGRATE THE PRINCIPLES OF NEURAL

Translating basic science to clinical practice is a challenge ADAPTATION INTO NEUROREHABILITATION
over time. There is no exact protocol. Learning activities When embarking on a rehabilitation program with some-
need to be adapted and matched to the abilities, goals, and one, match the principles of neuroplasticity to interactions
objectives of each individual. Based on research evidence, with the patient, the family, the health care team, and the
the following principles can help guide training: job, emphasizing the importance of the following:
1. Use it or lose it—Stay active and keep challenging 1. Thinking positively about health and recovery;
learning. Failure to regularly engage specific and expect to get better
general brain functions can lead to functional 2. Setting clear goals and objectives for retraining
degradation. 3. Encouraging the family to be involved in the
2. Use it and improve it—Engaging in training behav- retraining activities.
iors that drive specific brain functions can lead to an 4. Creating learning activities that are attended-goal
enhancement of the function. directed, repetitive, progressed in difficulty,
3. Be specific—The training experience must match the increased in variety and depth, spaced over time,
desired outcome; the nature of neural plasticity is rewarded, and complemented with feedback on
dictated by the nature of the training. accuracy
4. Repetition is essential—Learning requires repetition 5. Linking activities temporally (in time) and spatially
progressed in difficulty and spaced over time. but progressively sequenced; making the stimulus
5. Intensity matters—Plasticity changes require a suffi- strength adequate for detection and appropriate to
cient training intensity to ensure durability of pathways. avoid abnormal behaviors
6. Salience is important—The training must be salient 6. Integrating training behaviors into meaningful
and match the outcome behavior desired and the functional activities
goals of the individual. 7. Making training activities age appropriate
7. Age must be addressed—Training-induced plasticity 8. Integrating training activities across multiple
occurs most readily in a young brain, but neural ad- sensory modalities appropriate for desired
aptation continues across the life span with learning- outputs
based training. With aging, greater efforts at variety, 9. Performing training activities in different postural
integration, and discovery may be needed. orientations and different environments, which
8. Transference—Plasticity in response to one training facilitate the best performance
experience can also enhance acquisition of similar 10. Matching training behaviors with progression of
behaviors and adaptation in other experiences and healing and recovery as well as development
other parts of the body. 11. Strengthening positive responses with meaningful
9. Interference—Plastic changes after one training ex- rewards
perience may interfere with the acquisition of 12. Making it difficult to use the unaffected side
changes in similar systems. (e.g., wearing a glove)
10. Patient expectation—Patient expectation can facili- 13. Avoiding activities that stimulate repetition of
tate the outcomes of training; patients who expect to abnormal movements
get better can enhance their learning. 14. Maintaining high levels of attention and cognitive
11. Reward or feedback—Feedback allows modification function within the context of all daily activities;
of training behaviors, correcting errors and improv- avoiding habitual unattended behaviors
ing accuracy of learning. 15. Maintaining self-esteem
12. Environment—Enrich the environment by simply 16. Avoiding an egocentric focus; thinking about how
noticing everything in the environment, expanding to help and be involved with others
the environment to include new opportunities and 17. Being fit, thinking “tall,” and challenging
interacting with others. balance by interacting in new unstable
13. Fun—Learning is greatest when it is associated with environments
discovery and fun. RESEARCH VALIDATED OUTCOMES FOLLOWING
14. Helping others—Maintaining the fitness of the brain CENTRAL NERVOUS SYSTEM TRAINING
is best when individuals look beyond themselves to With thoughtful, attentive regular physical exercise, inte-
help and involve others. grated learning-based activities, daily learning, and specific
Continued
BOX 4-1 n NEUROPLASTICITY PRINCIPLES TRANSLATED TO GUIDE CLINICAL PRACTICE—cont’d
practice to improve skills, there is scientific evidence 2. Growth in the number of neuron populations
confirming the following positive outcomes: excited with progressively greater specificity in
1. Strengthened and elaborated neuronal interconnections the neuronal representations, and stronger temporal
2. Improved health and vigor of nerve cell populations coordination
(including neurotransmitters, nerve brain growth 3. Strengthening of neural connections (synapses)
factors, dopamine) following important behavioral inputs
3. Increased physical size of brain centers and a 4. Increased oxygenation
slowing down of shrinkage and atrophy of the brain 5. Decreased atrophy of the brain
with aging and disuse 6. Shortening of the time between the stimulus and
4. Increased accuracy of neuronal processing neuronal activation (latency)
5. Improved strength of associative memory processes 7. Modification of the amplitude of neuronal firing
and the capacity for the brain to remember what is 8. Improvement in the ability to turn off neurons once
seen, heard, felt, or learned fired
6. Faster brain processing and more reliable connections 9. Increased ability to inhibit unwanted neuronal
to improve sharpness and completeness of how our firing in response to an input
brain represents and records information 10. Shortened integration time between processing
7. Improved coordination of neuronal activities across inputs and production of outputs
brain subsystems 11. Specialization of representational firing in response
8. Improved abilities to broaden and control our atten- to familiar inputs
tion, shift attention, and take in more information 12. Improved temporal sequencing of firing following
with better acuity familiar inputs
9. Improved integration in vision, listening, feeling, and 13. Increased myelination
awareness of joint and trunk position in space 14. Increased complexity of dendrites and change in
10. Improved ability to suppress noise and distractions to number and complexity of synapses
stay on track 15. Increased consistency of response ( e.g., density of
11. Improved security of mobility and more reliable neuronal responses)
postural reactions to protect from falling in familiar 16. Improved selective excitation
and stable as well as unfamiliar and unstable 17. Increased specificity of neuronal response
environments 18. Increased salience of the response
12. Reactivation of long underpracticed skills that 19. Change in cortical (and noncortical) topography
support independent mental and physical actions 20. Increased area of representation
(e.g., riding a bike, skipping, throwing and catching 21. Smaller receptive fields
balls, playing an instrument) 22. Increased density of receptive fields
13. Restoration of fluency, self-confidence, liveliness, 23. Improved precision and order of receptive fields
and happiness Clinical Documentation of Outcomes
14. Increased longevity after Learning-Based Training
15. Increased blood flow and oxygen to the heart and Basic science and clinical research studies report positive
nervous system correlations between functional outcomes and neural ad-
16. Physical exercise combined with attended learning- aptation. With timely prevention, appropriate management
based exercise for decreased risk of heart disease, of acute insults to the CNS, spontaneous recovery, and
cancer, metabolic failure, and Alzheimer disease thoughtful attention to activities of daily living (ADLs)
METHODS OF MEASURING NEURAL ADAPTATION and task practice, disabling CNS problems can be mini-
Neurophysiological and Neuroanatomical Outcomes mized. Furthermore, early treatment after CNS injury or
Neurophysiological and neuroanatomical changes can be onset of disease may prevent more extensive damage to the
measured in the central nervous system (CNS) with learning. brain. Learning activities may not only be neuroprotective
Measurements have been made with a variety of techniques but also drive more complete recovery of function.
(e.g., neurophysiological mapping after craniotomies, elec- Changes in neural adaptation can be measured clinically in
troencephalography, magnetic source imaging [MSI], func- terms of improvement in function including the following:
tional magnetic resonance imaging [fMRI], electromyogra- 1. Fine and gross motor coordination
phy, cortical response mapping with positron emission 2. Sensory discrimination
tomography, and spectroscopy with the potential for neuro- 3. Balance and postural control
chemical analysis of neurotransmitters, growth hormones, 4. Reaction time
inhibitors, corticosteroids). With learning it is possible to 5. Accuracy of movements
measure the following: 6. Rhythm and timing of movements
1. Achievement of specialized cortical representations 7. Memory storage, organization, and retrieval
of behaviorally important inputs 8. Alertness and attention
BOX 4-1 n NEUROPLASTICITY PRINCIPLES TRANSLATED TO GUIDE CLINICAL PRACTICE—cont’d
9. Sequencing 8. Keep hobbies alive; mix life with work and play.
10. Logic, complexity, and sophistication of problem 9. Consider learning to play a musical instrument
solving (e.g., take lessons, practice and carefully listen
11. Language skills (verbal and nonverbal) while playing).
12. Interpersonal communication 10. Sing along with music; sing out loud in the
13. Positive sense of well-being car (loud, clearly, and slowly), and consider
14. Insight joining a choir to share the joy of singing with
15. Self-confidence others.
16. Self-image 11. Take time and opportunities to dance; consider
17. Signal/noise detection; able to make finer distinctions taking some lessons.
18. Ability to “chunk” information for memory and use 12. Volunteer in the community to interact with
19. Learning skills including faster learning others.
20. Achievement of developmental milestones 13. Wear a hearing aid if one has been prescribed; wear
21. Appropriate sensitivity of the nervous system (e.g., glasses if they are needed.
reduction in hyperactivity and sensory defensiveness) 14. Improve everyday activities by learning something
22. Ability to perform a skill from memory new or by challenging observation and recall skills:
23. Flexible behaviors; variability in task performance have a puzzle out and add pieces, or have challeng-
24. Flexibility for experience-based learning ing crossword puzzles to work on.
PRACTICAL SUGGESTIONS FOR MAINTAINING 15. Play games that require fine motor skills (e.g., shuf-
PHYSICAL AND BRAIN HEALTH ACROSS fling cards, Ping-Pong, bowling, tennis).
THE LIFE SPAN35,128 16. After walking to the store, reconstruct all of the
Make living a learning experience by creating goal-directed things that were seen on the way and at the store
activities that require attention and can be progressed in dif- and what was accomplished.
ficulty or variety over time. Where possible, provide condi- 17. When waiting for scheduled appointments, review
tions where feedback about performance is received. Try to the details of the environment; examine what has
maintain variability in activities and vary the environments changed since the last visit.
for performing the same and different tasks. Take some risks 18. Before going to social gatherings, try to
by changing activities that are familiar and comfortable. remember the names of the people who are
Walk around on unstable surfaces as well as familiar sur- expected to be there; afterward, review who was
faces with the eyes closed to challenge balance and postural there by name.
reactions. Assume different positions to perform common 19. When idle or waiting, instead of sitting, walk
tasks. More specifically: around and mentally review items in the
1. Integrate low intensity to moderate physical exercise environment, organize these items, review tasks
into the day, balanced with healthy eating, good that need to be done (including steps required),
hydration, and stress management. play a game.
2. Stop all negative learning behaviors; minimize or 20. Find different ways to get to common places;
eliminate bad habits. evaluate which way is fastest, easiest, most
3. Be actively engaged at the cutting edge of all interesting, most fun.
activities; minimize habitual behaviors. 21. Constantly read and listen to the news, attend
4. Improve skills; progressively practice to perform lectures, listen to or watch educational programs.
each task better and use mistakes to guide practice. 22. When with others, especially with children or
5. Improve language listening skills and expand the grandchildren, play progressive or problem-solving
words and the language used. games (e.g., Boggle, chess, card games, checkers).
6. Be a lifelong learner; take classes, go to lectures, 23. Look beyond the self; think what you can to do
listen to audio books, and discuss what was learned make others happy.
with others. 24. Avoid stress; instead enjoy life and share joy with
7. Engage in conversational listening (review what is others.
remembered about a conversation right after the 25. Take a walk or ride a bike every day.
conversation ends). 26. Find something fun to do every day.
Data from Byl N, Merzenich MM, Cheung S, et al: A primate model for studying focal dystonia and repetitive strain injury: effects on the primary somato-
sensory cortex, Phys Ther 77:269-284, 1997; Kleim J, Jones TA: Principles of experience-dependent neural plasticity: implications for rehabilitation after
brain damage. J Speech Lang Hear Res 51(1):S225-S239, 2008; and Merzenich M: The brain revolution (in press), 2010.
The most informative studies on neuroplasticity are those changes in cortical responses. Consequently, specific as-
specifically directed toward defining the changes induced by pects of these changes in distributed neuronal response are
learning. One approach has been to document the patterns of highly correlated with learning-based improvements in per-
distributed neural response representation of specific inputs ception, motor control, and cognition.134-137 In these pro-
before and after learning. In particular, neuronal responses cesses the brain is not simply changing to record and store
have been measured in the primary auditory, somatosensory, content, but the cerebral cortex is also selectively refining its
and motor cortices in animals. These animal studies have processing capacities to fit each task at hand by adjusting its
been paired with behavioral studies in humans. Both the spectral or spatial and temporal filters. Ultimately it estab-
animal and the human studies provide strong evidence docu- lishes its own general processing capabilities. This “learning
menting the ability of the brain to functionally self-organize. to learn” determines the facility with which specific classes
This capacity for change occurs not only during develop- of information can be stored, associated, and manipulated.
ment but also in adulthood, specifically after learning-based These powerful self-shaping processes of the forebrain ma-
activities. The basic processes for neural adaptation are dis- chinery are operating not only on a large scale during devel-
cussed in the following paragraphs. opment but also during experience-based management of
1. Neural circuits must be actively engaged in learning-based externally and internally generated information in adults.
activities if degradation and atrophy are to be prevented. This self-shaping with experience allows the development
We know that if infants are deprived of sensory and motor of hierarchical organization of perception, cognition, motor,
experiences during development, the brain does not develop and executive management skills.
normally. For example, without exposure to light, there is a 5. In learning, selection of behaviorally important inputs is
reduction in the number of neurons in the visual cortex.122 a product of strengthening input coincidence-based con-
Similarly, if infants are not exposed to sound, there is a reduc- nections (synapses).
tion in the neurons in the auditory cortex.123 Even in adults, The process of coincidence-based input co-selection
when neural circuits are not used over an extended period of leads to changes in cortical representation. Coincident, tem-
time, they begin to degrade, and the unused area of the brain porally and spatially related events that fire together are
is allocated to serve another part of the body.124 Similarly, if strengthened together. In skill learning, this principle of
task performance is practiced, then the topography expands concurrent input co-selection results from repetitive practice
and becomes more detailed, as might occur in someone who that includes the following:
is blind and reads Braille.125 It is also interesting to note that a. A progressive amplification of cell numbers engaged
although a person is blind, the visual cortical areas may be- by repetitive inputs.136-138
come active when the individual is reading Braille.126 Simi- b. An increase in the temporal coordination of distrib-
larly a person who is deaf may demonstrate activation of the uted neuronal discharges evoked by successive events
auditory cortex when visual stimuli are presented. to mark features of behaviorally important inputs is a
2. With learning, the distributed cortical representations of consequence of a progressive increase in positive cou-
inputs and brain actions “specialize” in their representa- pling between nearly simultaneously engaged neurons
tions of behaviorally important inputs and actions in skill within cortical networks.136,139
learning. c. A progressively more specific “selection” of all input
There seems to be a minimal level of repetitive practice features that collectively represent behaviorally important
needed to acquire a new skill that will be maintained over inputs, expressed moment by moment in time.138,139 Thus
time. In fact, this may lead to specialization or change in the skill learning results in mapping temporal neighbors in
underlying neurophysiological processing.127-129 This spe- representational networks at adjacent spatial locations
cialization develops in response to selective cortical neuron when they regularly occur successively in time.65,140,141
responses specialized to demands of sensory, perceptual, Changes in activation patterns, dendritic growth, syn-
cognitive, and motor skill learning.130-133 This adaptation has apses, and neuronal activities may also be observed.
been clearly documented in animal studies. For example, if The basis of the functional creation of the detailed, rep-
an animal is trained to make progressively finer distinctions resentational cortical maps converting temporal to spatial
about specific sensory stimuli, then cortical neurons come to representations is related to the Hebbian change principle.142
represent those stimuli in a progressively more specific and The Hebbian plasticity principle applies to the development
progressively “amplified” manner. of interconnections between excitatory and inhibitory inputs
3. There are important behavioral conditions that must be within the cortical pyramidal neurons and their connections
met in the learning phase of plasticity. to extrinsic inputs and outputs. On the basis of the Hebbian
a. If behaviorally important stimuli repeatedly excite principle, the operation of coincidence-based synaptic plas-
cortical neuron populations, the neurons will progres- ticity in cortical networks results in the formation, strength-
sively grow in number. ening, and continuous recruitment of neurons within neuro-
b. Repetitive, behaviorally important stimuli processed nal “assemblies” that “cooperatively” represent behaviorally
in skill learning lead to progressively greater specific- important stimuli.
ity in the spectral (spatial) and temporal dimensions. 6. Plasticity is constrained by anatomical sources and
4. The growing numbers of selectively responding neurons convergent-divergent spreads of inputs. Every cortical
discharge with progressively stronger temporal coordi- field has the following:
nation (distributed synchronicity). a. Specific extrinsic and intrinsic input sources
Through the course of progressive skill learning, a more b. Dimensions of anatomical divergence and convergence
refined basis for processing stimuli and generating actions of its inputs, limiting dynamic combination Hebbian
critical to skilled tasks is enabled by the multidimensional input co-selection capacities143,144
Anatomical input sources and limited projection overlap differentiated perceptual, cognitive, monitoring, and
both to enable change by establishing input-selection reper- executive skills.
toires and to determine the limits for change. There are c. The sources of inputs and their field-specific spreads
relatively strict anatomical constraints at the “lower” sys- and boundary limits, the distributions of modulatory
tem levels, where only spatially (spectrally) limited input inputs differentiated by cortical layers in different cor-
coincidence-based combined outcomes are possible. In the tical regions, the basic elements and their basic inter-
“higher” system hierarchies, anatomical projection topog- connections in the cortical processing machine, and
raphies are more powerful, with neurons and neuronal as- crucial aspects of input combination and processing at
semblies developing that respond to complex combinations subcortical levels are inherited (see reference 147 for
of features of real-world objects, events, and actions. review). Although these inherited aspects of sensory,
7. Plasticity is constrained by the time constants govern- motor, and cortical processing circuit development
ing coincident input co-selection and by the time struc- constrain the potential learning-based modification of
tures and potentially achievable coherence of extrinsic processing within each cortical area, representation
and intrinsic cortical input sources. changes can occur as a result of environmental interac-
To effectively drive representational changes with coinci- tion and purposeful behavioral practice.
dent input-dependent Hebbian mechanisms, temporally coor- 9. Temporal dimensions of behaviorally important inputs
dinated inputs are prerequisite, given the short durations also influence representational “specialization.” In at
(milliseconds to tens of milliseconds) of the time constants least four ways, the cortex refines its representations of
that govern synaptic plasticity in the adaptive cortical machin- the temporal aspects of behaviorally important inputs
ery (see reference 145 for review). Consistently uncorrelated during learning.
or low–discharge-rate inputs induce negative changes in syn- a. First,
aptic effectiveness. In addition, stimuli occurring repetitively 1) The cortex generates more synchronous represen-
simultaneously can also degrade the representation. These tations of sequenced and coincident associative
negative effects also contribute importantly to the learning- input perturbations or events, not only recording
driven “election” of behaviorally important inputs. their identities but also marking their occurrences
8. Cortical field–specific differences in input sources, dis- (for examples, see references 132, 136, 139, and
tributions, and time-structured inputs create different 148 to 151). These changes in representation ap-
representational structures. pear to be primarily achieved through increases in
a. There are significant differences in the activity from positive coupling strengths between intercon-
afferent inputs from the retina, skin, or cochlea gen- nected neurons participating in stimulus- or action-
erated in a relatively strictly topographically wired specific neuronal cell assemblies.132,150,152-171 The
V1 (area 17), S1 proper (area 3b), or A1 (area 43) strength of the interconnectedness increases repre-
compared with the inferotemporal visual, insular sentational salience as a result of downstream
somatosensory, dorsotemporal auditory, or prefrontal neurons being excited as a direct function of the
cortical areas that receive highly diffuse inputs (see degree of temporal synchronization of their inputs.
Figure 4-8). In the former cases, heavy schedules of 2) Increasing the power of the outputs of a cortical
repetitive, temporally coherent inputs are delivered area drives downstream plasticity. Hebbian plas-
from powerful, redundant projections from relatively ticity mechanisms operating within downstream
strictly topographically organized thalamic nuclei cortical (or other) targets also have relatively
and lower-level, associated cortical areas. Whereas short time constants. The greater the synchronic-
neighboring neurons can share some response prop- ity of inputs, the more powerfully those change
erties, neurons or clusters of neurons respond selec- mechanisms are engaged. The strength of the in-
tively to learned inputs. These neurons are distrib- terconnections also helps protect against noise.
uted widely across cortical areas and share less For example, by simple information abstraction
information with neighboring neurons. In the “lower” and coding, the distributed neuronal representa-
levels, afferent input projections from any given tion of the “signal” (a temporally coordinated,
source are greatly dispersed. Highly repetitive inputs distributed neuronal response pattern represent-
are uncommon, inputs from multiple diffuse cortical ing the input or action) is converted at the entry
sources are more common as well as more varied, levels in the cortex into a form that is not as easily
and complex input combinations are in play. These degraded or altered by “noise.” The strength of
differences in input schedules, spreads, and combi- the interconnectedness also confers robustness of
nations presumably largely account for the dramatic complex signal representation for spatially or
differences in the patterns of representation of be- spectrally incomplete or degraded inputs.
haviorally important stimuli at “lower” and “higher” b. Second,
levels.146 1) The cortex can select specific inputs through learn-
b. Despite these differences in representational organi- ing to exaggerate the representation of specific
zation across the cortex, the cortex does progres- input time structures. Conditioning a monkey or a
sively differentiate cortical cells to accomplish spe- rat with stimuli that have a consistent, specific
cific operational tasks. There is a serial progression temporal modulation rate or interstimulus time, for
of differentiation to allow the development of func- example, results in a selective exaggeration of the
tional organization that allows an individual to pro- responses of neurons at that rate or time separa-
gressively master more and more elaborated and tion. In effect, the cortex “specializes” for expected
relatively higher-speed or relatively lower-speed b. These time constants govern—and limit—the cortex’s
signal event reception. ability to “chunk” (i.e., to separately represent by dis-
2) Both electrophysiological recording studies and tributed, coordinated discharge) successive events
theoretical studies suggest that cortical networks within its processing channels. Both neurophysiologi-
richly encode the temporal interval as a simple cal studies in animals and behavioral training studies
consequence of cortical network dynamics.172,173 in human adults and children have shown that the time
It is hypothesized that the cortex accomplishes time constants governing event-by-event complex signal
interval and duration selectivity in learning by representation are highly plastic. With intensive train-
positively changing synaptic connection strengths ing in the right form, cortical “processing times” re-
for input circuits that can respond with recovery flected by the ability to accurately and separately
times and circuit delays that match behaviorally process events occurring at different input rates can be
important modulation frequency periods, inter- dramatically shortened or lengthened.182-185
vals, or durations. However, studies on including 11. Plasticity processes are competitive.
excessive, rapid, repetitive fine motor movements a. If two spatially or spectrally different inputs are con-
can sometimes lead to serious degradation in rep- sistently delivered nonsimultaneously to the cortex,
resentation if the adjacent digits are driven nearly cortical networks generate input-selective cell assem-
simultaneous in time. This may be associated with blies for each input and actively segregate them from
negative learning and a loss of motor control.174 one another.139,184,186-188 Boundaries between such in-
c. Third, puts grow to be sharp and are substantially intensity
1) The cortex links representations of immediately independent. Computational models of Hebbian net-
successive inputs that are presented in a learning work behaviors indicate that this sharp segregation of
context. nonidentical, temporally separated inputs is accom-
2) As a result of Hebbian plasticity, it establishes plished as a result of a wider distribution of inhibitory
overlapping and neighboring relationships be- instead of excitatory responses in the emerging, com-
tween immediately successive parts of rapidly peting cortical cell assemblies that represent them.
changing inputs yet retains its individualized, dis- b. This Hebbian network cell assembly formation and
tinct cortical representation. 65,175 competition appear to account for how the cortex cre-
d. Fourth, ates sharply sorted representations of the fingers in the
1) The cortex generates stimulus sequence-specific primary somatosensory cortex.140,189 The Hebbian net-
(“combination-sensitive”) responses, with neuro- work probably accounts for how the cortex creates
nal responses selectively modulated by the prior sharply sorted representations of native aural language-
application of stimuli in the learned sequence of specific phonemes in lower-level auditory cortical
temporally separated events. areas in the auditory and speech processing system of
2) These “associative” or “combination-sensitive” humans. If inputs are delivered in a constant and ste-
responses have been correlated with evidence of reotyped way from a limited region of the skin or
strengthened interconnections between cortical cochlea in a learning context, that skin surface or coch
cell assemblies representing successive event ele- lear sector is an evident competitive “winner.”136,190 By
ments separated by hundreds of milliseconds to Hebbian plasticity, the cortical networks will co-select
seconds in time.176,177 The mechanisms of origin that specific combination of inputs and represent it
of these effects have not yet been established. within a competitively growing Hebbian cell assem-
10. The integration time (“processing time”) in the cortex bly. The competitive strength of that cooperative cell
is itself subject to powerful learning-based plasticity. assembly will grow progressively because more and
a. Cortical networks engage both excitatory and inhibi- more neurons are excited by behaviorally important
tory neurons by strong input perturbations. Within a stimuli with increasingly coordinated discharges. That
given processing “channel,” cortical pyramidal cells means that neurons outside of this cooperative group
cannot be effectively reexcited by a following pertur- have greater numbers of more coordinated outputs
bation for tens to hundreds of milliseconds. These contributing to their later competitive recruitment.
integration “times” are primarily dictated by the time Through progressive functional remodeling, the cor-
for recovery from inhibition, which ordinarily domi- tex clusters and competitively sorts information across
nates poststimulus excitability. This “integration time,” sharp boundaries dictated by the spectrotemporal sta-
“processing time,” or “recovery time” is commonly tistics of its inputs. If it receives information on a
measured by deriving a “modulation transfer func- heavy schedule that sets up competition for a limited
tion,” which defines the ability of cortical neurons to input set, it will sort competitive inputs into a corre-
respond to identical successive stimuli within cortical spondingly small number of largely discontinuous
“processing channels.” For example, these “integra- response regions.191,192
tion” times normally range from about 15 to about c. Competitive outcomes are, again, cortical level depen-
200 ms in the primary auditory receiving areas.178-180 dent. The cortex links events that occur in different
Progressively longer processing times are recorded at competitive groups if they are consistently excited
higher system levels (e.g., in the auditory cortex, they synchronously in time. At the same time, competi-
are approximately a syllable in length, 200 to 500 ms tively formed groups of neurons come to be synchro-
in duration) in the “belt cortex” surrounding the pri- nously linked in their representations of different parts
mary auditory cortex.181 of the complex stimulus and collectively represent
successive complex features of the vocalization Attentional control flexibly defines an enabling “window”
through the coordinated activities of many groups. for change in learning.182 Progressive learning generates pro-
d. Neurons within the two levels of the cortex surround- gressively more strongly represented goals, expectations, and
ing A1 (see Figure 4-8) have greater spectral input feedback196,197 across all representational systems that are
convergence and longer integration times that enable undergoing change and to modulatory control systems weigh-
their facile combination of information representing ing performance success and error. Strong intermodal behav-
different spectrotemporal details. Their information ioral and representational effects have also been recorded in
extraction is greatly facilitated by the learning-based experiments that might be interpreted as shaping expecta-
linkages of cooperative groups that deliver behavior- tions.198,199 These shaping expectations would be similar to
ally important inputs in a highly salient, temporally those observed in a human subject using multisensory inputs
coordinated form to these fields. With their progres- such as auditory, visual, and somesthetic information to create
sively greater space and time constants, still higher- integrated phonological representations, to create fine motor
level areas organize competitive cell assemblies that movement trajectory patterns that underlie precise hand con-
represent still more complex spectral and serial-event trol, or to make a vocal production.
combinations. Note that these organizational changes 14. The scale of plasticity in progressive skill learning is
apply over a large cortical scale. In skill learning over massive.
a limited period of training, participating neuronal a. Cortical representational plasticity must be viewed as
members of such assemblies can easily be increased arising from multiple-level systems that are broadly
by many hundredfold, even within a primary sensory engaged in learning, perceiving, remembering, think-
area such as S1, area 3b, or A1.136,139,174,184,193 ing, and acting. Any behaviorally important input
e. In extensive training in complex signal recognition, (or consistent internally generated activity) engages
more than 10% of neurons within temporal cortical many cortical areas. Repetitive training drives all
areas can come to respond highly selectively to a spe- cortical areas to change.131,144,200 Different aspects of
cific, normally rare, complex training stimulus. The any acquired skill are contributed from field-specific
distributed cell assemblies representing those specific changes in the multiple cortical areas that are remod-
complex inputs involve tens or hundreds of millions of eled in its learning.
neurons and are achieved by enduring effectiveness b. In this kind of continuously evolving representational
changes in many billions of synapses. machine, perceptual constancy cannot be accounted for
12. Learning is modulated as a function of behavioral state. by locationally constant brain representations; rela-
a. At “lower” levels of the cortex, changes are generated tional representational principles must be invoked to
only in attended behaviors.137,138,146,193-195 Trial-by-trial account for it.131,201 Moreover, representational changes
change magnitudes are a function of the importance must obviously be coordinated level to level. It should
of the input to the animal as signaled by the level of also be understood that plastic changes are also induced
attention, the cognitive values of behavioral rewards extracortically. Although it is believed that learning at
or punishments, and internal judgments of practice the cortical level is usually predominant, plasticity in-
trial precision or error based on the relative success duced by learning within many extracortical structures
or failure of achieving a target goal or expectation. significantly contributes to learning-induced changes
Little or no enduring change is induced when a that are expressed within the cortex.
well-learned “automatic” behavior is performed from 15. Enduring cortical plasticity changes appear to be
memory without attention. It is also interesting to accounted for by local changes in neural anatomy.
note that at some levels within the cortex, activity Changes in synapse turnover, synapse number, synaptic
changes can be induced even in nonattending subjects active zones, dendritic spines, and the elaboration of termi-
under conditions in which “priming” effects of nonat- nal dendrites have been demonstrated to occur in a behavior-
tended reception of information can be demonstrated. ally engaged cortical zone.144,202-207 Through many changes
b. The modulation of progressive learning is also achieved in local structural detail, the learning brain is continuously
by the activation of powerful reward systems releasing physically remodeling its processing machinery, not only
the neurotransmitters norepinephrine and dopamine across the course of child development but also after behav-
(among others) through widespread projections to the ioral training in an adult who has had a neural insult.
cerebral cortex. Norepinephrine plays a particularly 16. Cortical plasticity processes in child development rep-
important role in modulating learning-induced changes resent progressive, multiple-staged skill learning.
in the cortex.148,184,195 a. There are two remarkable achievements of brain
c. The cortex is a “learning machine.” During the learning plasticity in child development. The first is the pro-
of a new skill, neurotransmitters are released trial by trial gressive shaping of the processing to handle the ac-
with application of a behaviorally important stimulus or curate, high-speed reception of the rapidly changing
behavioral rewards. If the skill can be mastered and streams of information that flow into the brain. In the
thereafter replayed from memory, its performance can cerebral cortex, shaping appears to begin most pow-
be generated without attention (habituation). Habitua- erfully within the primary receiving areas of the
tion results in a profound attenuation of the modulation cortex. With early myelination, the main gateways
signals from these neurotransmitter sources; plasticity is for information into the cortex are receiving strongly
no longer positively enabled in cortical networks. coherent inputs from subcortical nuclei, and they can
13. Top-down influences constrain cortical representational quickly organize their local networks on the basis of
plasticity. coincident input co-selection (Hebbian) plasticity
mechanisms. The self-organization of the cortical reception, storage, and analysis of diverse and com-
processing machinery spreads outward from these plexly associated information.
primary receiving areas over time to ultimately refine e. The flexible, self-adjusting capacity for refinement
the basic processing machinery of all the cortex. The of the processing capabilities of the nervous system
second great achievement, which is strongly depen- confers the ability of our species to represent com-
dent on the first, is the efficient storage of massive plex language structures. This self-adjusting capac-
content compendia in richly associated forms. ity also allows humans to develop high-speed read-
b. During development, the brain accomplishes its func- ing abilities; remarkably varied complex modern-era
tional self-organization through a long parallel series motor abilities; and abstract logic structures charac-
of small steps. At each step, the brain masters a series teristic of a mathematician, software engineer, or
of elementary processing skills and establishes reliable philosopher. This nervous system refinement also
information repertoires that enable the accomplish- creates elaborate, idiosyncratic, experience-based
ment of subsequent skills. Second- and higher-order behavioral abilities in all of us.
skills can be viewed as both elaborations of more basic
mastered skills and the creation of new skills depen- Neuroplasticity and Learning
dent on combined second- and higher-order process- How Are Learning Sequences Controlled? What
ing. That hierarchical processing is enabled by greater Constrains Learning Progressions? Perhaps the most
cortical anatomical spreads, by more complexly con- important basis of control of learning progressions is repre-
vergent anatomical sources of inputs, and by longer sentational consolidation. Through specialization, the trained
integration (processing, recovery) times at progres- cortex creates progressively more specific and more salient
sively higher cortical system levels. This hierarchical distributed representations of behaviorally important inputs.
but integrating processing allows for progressively Growing representational salience increases the power of a
more complex combinations of information integrated cortical area to effectively drive change wherever outputs
over progressively longer time epochs as one ascends from this evolving cortical processing machinery are distrib-
across cortical processing hierarchies. uted (e.g., in “higher system levels distributed and coordi-
c. As the cortical machinery functionally evolves and nated [synchronized] responses” more powerfully drive
consequently physically “matures” through childhood downstream Hebbian-based plasticity changes).
developmental stages, information repertories are rep- A second powerful basis for sequenced learning is pro-
resented in progressively more salient forms (i.e., with gressive myelination. At the time of birth, only the core
more powerful distributed response coordination). “primary” extrinsic information entry zones (A1, S1, V1) in
Growing agreement directly controls the power of the cortex are heavily myelinated.208,209 Across childhood,
emerging information repertoires for driving the next connections to and interconnections between cortical areas
level of elaborative and combinatorial changes. It is are progressively myelinated, proceeding from these core
hypothesized that saliency enables the maturation of areas out to progressively “higher” system levels. Myelina-
the myelination of projection tracts delivering outputs tion in the posterior parietal, anterior, and inferior temporal
from functionally refined cortical areas. More mature and prefrontal cortical areas is not “mature” in the human
myelination of output projections also contributes to forebrain until 8 to 20 years of age. Even in the mature
the power of this newly organized activity to drive state, it is far less developed at the “highest” processing
strong, downstream plastic change through the opera- levels.
tion of Hebbian plasticity processes. Myelination controls the conduction times and therefore
d. As each elaboration of skill is practiced, in a learning the temporal dispersions of input sources to and within cor-
phase, neuromodulatory transmitters enable change in tical areas. Poor myelination at “higher” levels in the young
the cortical machinery. The cortex functionally and brain is associated with temporally diffuse inputs. They can-
physically adapts to generate the neurological repre- not generate reliable representational constructs of an adult
sentations of the skill in progressively more selective, quality because they do not as effectively engage input-
predictable, and statistically reliable forms. Ultimately, coincidence–based Hebbian plasticity mechanisms. That
the performance of the skill concurs with the brain’s ensures, in effect, that plasticity is not enabled for complex
own accumulated, learning-derived “expectations.” combinatorial processing until “lower” level input reper-
The skill can then be performed from memory, without toires are consolidated (i.e., become stable, statistically reli-
attention. With this consolidation of the remembered able forms).
skill and information repertoire, the modulatory nuclei Although myelination is thought to be genetically pro-
enable no further change in the cortical machinery. The grammed, some scientists hypothesize that myelination in
learning machine, the cerebral cortex, moves on to the the CNS is also controlled by emerging temporal response
next elaboration. In this way the cortex constructs coherence and is achieved through temporally coordinated
highly specialized processing machinery that can pro- signaling from the multiple branches of oligodendrocytes
gressively produce great towers of automatically per- that terminate on different projection axons in central tracts
formable behaviors and great progressively maturing and networks. It has been argued that central myelination is
hierarchies of information-processing machinery that positively and negatively activity dependent and that distrib-
can achieve progressively more powerful complex uted synchronization may contribute to positive change.210 If
signal representations, retrievals, and associations. the hypothesis that coherent activity controls myelination
With this machinery in a mature and thereby efficiently proves to be true, then the emerging temporal correlation of
operating form, there is a remarkable capacity for distributed representations of behaviorally important stimuli
is generated level by level. This is done by changes in “mature” modulatory selectivity and power. The progres-
coupling in local cortical networks in the developing cortex. sive maturation of the modulatory control system occurs
It would also directly drive changes in myelination for the naturally with development or training. This system can
outputs of that cortical area. These two events in turn would provide another important constraint on skill development
enable the generation of reliable and salient representational progression and regulation of axial or trunk postural and
constructs at that higher level. By this kind of progression, balance control and fine motor coordination.
skill learning is hypothesized to directly control progressive What Facilitates the Development of Permanent
functional and physical brain development through the “Automatic” Motor Behaviors? The creation and main-
course of child development. This is accomplished both tenance of cortical representations are functions of the
by refining (“maturing”) local interconnections through animal’s or human’s level of attention at a task. Cortical
response dynamics of information processing machinery at representational plasticity in skill acquisition is self-limiting.
successive cortical levels and by coordinated refinement Because the behavior comes to be more “automatic,” it is
(“maturing”) of the critical information transmission path- less closely attended, and representational changes induced
ways that interconnect different processing levels. in the cortex fade and ultimately disappear or reverse
Another constraint in the development of neural adapta- (unlearning effects). 215,216 The element of behavioral
tion may be the development of mature sleeping patterns, performance that enables maintenance of the behavior
especially within the first year of life.211 Sleep both enables with minimum involvement of the cortical learning
the strengthening of learning-based plastic changes and re- machinery is probably stereotypical movement sequence
sets the learning machinery by “erasing” temporary unrein- repetition. As a movement behavior is practiced, an effec-
forced and unrewarded input-generated changes produced tive, highly statistically predictable movement sequence
over the preceding waking period.212-214 The dramatic shift is adopted that enables the storage of the learned behavior
in the percentage of time spent in rapid-eye-movement sleep in a permanent form that requires only minimal or no
is consistent with a strong early bias toward noise removal behavioral attention. If behavioral performance declines
in an immature and poorly functionally unorganized brain. or behavioral or brain conditions change to render a task
Sleep patterns change dramatically in the older child, in more difficult, attention to the behavior will again need to
parallel with a strong increase in the daily schedule of increase, producing an invigorated cortical response to the
closely attended, rewarded, and goal-oriented behaviors. new learning challenge.
This research will need to be explored in greater detail when By this view, the cerebral cortex is clearly a learning
these data are related to patients with CNS damage. This machine. William James217 was the first to point out that the
population often has poor breathing habits and capabilities great practical advantage for a self-organizing cortex was
that lead to decreased oxygenation and often broken sleep the development of what he called “habits.” When a skill is
cycles. How much either impairment, breakdown, or the overlearned, it will engage pathways that are so reliable that
interaction of the two diminishes neuroplasticity has yet to they can be followed without attention.
be determined. Why are some habits retained and others lost? Can sen-
Top-down modulation controlling attentional windows sorimotor learning be sustained when the adaptive represen-
and learned predictions (expectations and behavioral goals) tations of the learned behavior “fade” in the cerebral cortex?
must all be constructed by learning. Delays in goal develop- These areas have not been well researched. However, there
ment could also create an important constraint for the pro- are several possibilities. Habits could come to be repre-
gression of early learning. In the very young brain, prediction sented in an enduring form extracortically. The cortex could
and error-estimation processes would be weakened because modify processing in the spinal cord, the basal ganglia, the
stored higher-level information repertoires are ill formed red nucleus, or the cerebellum. For example, the learning of
and statistically unreliable. As the brain matures, stored in- manual skills requires a motor cortex, but overlearned motor
formation progressively more strongly and reliably enables skills may not be significantly reduced by the induction of a
top-down attentional and predictive controls, progressively wide area 4 lesion.
providing a stronger basis for success and error signaling Another possibility is that behaviorally induced cortical
for modulatory control nuclei and progressively enabling changes endure in a highly efficient representational form
top-down syntactic feedback to increase representational that can sustain the representation of its key features on
reliability. the cortex itself, engaging only limited distributed popula-
Attention, reward and punishment, accuracy of achieve- tions of cortical neurons to represent the behavior with high
ment of goals, and error feedback gate learning through a fidelity. Thus, recall of past learning may take less time to
modulatory control system are critical for learning. The restructure than to reformat entirely new learning, whether
modulatory control systems that enable learning are also it be a cognitive or motor task. The fact that a monkey
plastic, with their process of maturation providing con- improves discriminative abilities or movement performance
straint or facilitation for progressive learning. These sub- after modifying the cortical neuron response with heavily
cortical nuclei are signaled by complex information feed- practiced behaviors supports this alternative. However,
back from the cortex itself. The salience and specificity many behaviors, such as musical performance, require
of that feedback information grow over time. The ability constant, attended practice at a highly cognitive level to
to provide accurate error judging or goal-achievement maintain both the representational changes and the perfor-
signaling must grow progressively. The nucleus basalis, mance. It also appears that continued learning with heavily
nucleus accumbens, ventral tegmentum, and locus coeru- practiced behaviors may be neuroprotective with aging,
leus must undergo their own functional self-organization maintaining function despite loss of cortical neurons as a
on the basis of Hebbian plasticity principles to achieve natural part of aging.
SUMMARY relevant to patients with a head injury or stroke, they are also
Over the years, learning has been tied to critical periods of relevant for aging adults,229-233 and those with neurodegenerative
development, with the assumption that if a particular skill or disease. These principles are not meant to be exhaustive or mu-
behavior was not learned during the critical period, the op- tually exclusive but to highlight the principles of experience-
portunity to acquire that skill was lost. In addition, after this dependent plasticity. However, they can serve as a reference for
critical period of development, aging was associated with therapists who are designing creative intervention programs
inevitable deterioration of brain and neurophysiological based on the translation of basic science to clinical practice and
function. However, today there is substantial evidence that to help organize the extensive research on neuroplasticity.
the brain is an incredibly specialized representational ma- These principles can be applied across a broad range of
chine that can adapt to meet the specific inputs that engage exercises—not just “brain exercises” to improve cognition and
it. The beauty of the brain is that it not only self-organizes intellect, but also physical exercise. For example, we know that
but stores the contents of its learning to create a foundation brain derivative neural factor (BDNF) is necessary for learn-
that increases in depth and breadth and makes predictions on ing. BDNF decreases with aging and is severely reduced in
even novel inputs to facilitate acute and efficient operations. animals with dementia. However, BDNF can be increased
The earlier the exposure to multisensory stimuli, the easier with moderate and aerobic exercise.234,235 Furthermore, it is
it is for the competitive neuronal processes to adapt and to clear that timing of enrichment (e.g., mental stimulation,
make extensive connections. With growing neuronal speci- physical exercise, sensory and motor training) is important, not
ficity and salience, more powerful predictions are continued only during development but across the life span. Initiating an
until there is greater learning and mastery. exercise program too early (e.g., in less than 24 hours acute
Among the important findings of the twentieth century was post neural injury) may be associated with an exaggeration of
the validation that the brain is a learning machine that operates cellular injury.236 However, waiting too long to intervene can
throughout life. The aging process can take a toll on the ability limit the efficacy of the learning-based training experience.237
to store information and may reduce both the complexity of It also appears that the efficacy of learning-based training may
the information that is processed and the individual’s ability to be enhanced with cortical stimulation,238 repetitive transcranial
remember. But if an individual is conscious of good hydration, magnetic stimulation (TMS),239 and imagery. It is critical to
balanced nutrition, physical exercise, and regularly goal- create a positive foundation to maximize learning (e.g., good
directed progressive learning, CNS pathways of representation hydration to maximize blood flow and oxygenation of tissues,
and prediction can not only be preserved but also continue to adequate nutrition to energize the body, and aerobic activity to
adapt. These activities can also slow the aging process. Thus it increase endorphins and BDNF, as well as positive expecta-
is possible to drive improvement in function in individuals tions of getting better [limbic system]). Rehabilitation special-
with abnormalities related to development, disease, injury, or ists must not only translate basic neuroscience into practice but
aging. Learning is not necessarily specifically staged, but participate in clinical research, serve as advocates for patients,
rather represents complex abilities developed mostly from ensure access to appropriate rehabilitative services, and be
systems interaction and integration. Therapists must develop politically active in health care reform.
the ability to determine what inputs are reliable and salient to To ensure maximum neural adaptation, rehabilitation
effectively create functional and physical brain maturation, programs must include strong, carefully outlined home
adaptation, and learning. In the face of different types of chal- programs. Therapists must educate patients and their fami-
lenges (structural, emotional, pathological), clinicians must lies about the principles of neuroplasticity to empower
develop more effective strategies that can be used to facilitate them to create progressive learning activities at home and
neural adaptation, learning, substitution, and representational in the community. Patients should revisit health care team
changes that will allow meaningful maintenance and improve- members to facilitate ongoing learning. Patients must
ment in function despite anatomical or physiological variances become their own best therapist, consistently motivating
in structure. Although strong behavioral events can be associ- themselves to learn something new, perform attended
ated with measurable neural adaptability, new, more perma- behavioral activities, observe and integrate new information
nent neural connections and synapses must be strengthened from their environment, have fun, stay engaged with family,
with repetition and increased complexity. Clients with CNS friends, and community and avoid habitual stereotypical
disorders may have damaged certain areas of the brain, which behaviors. Learning should be an excuse to travel to new
may not recover; however, with learning-based activities it is places and learn new skills. Every day should include a new
possible to reorganize the brain, stimulate neurons from adja- learning experience. Learning and aerobic exercise may not
cent areas, establish new synapses and dendritic pathways,218 only be neuroprotective but could be critical for slowing
and activate neurons in the contralateral, uninjured parts of the down the natural neurodegenerative aspects of aging. Com-
brain.219-225 Creating the best environment to learn a skill may puter gaming, new technology, and robotics can be inte-
initially need to be contrived, with limitations controlled exter- grated to expand daily learning-based activities at home
nally by the therapist’s hands or clinical arena. In time, those (see Chapter 38).
limitations must be eliminated and variability within the natu- The maximum attainment of skilled performance cannot
ral environment reintroduced to achieve true learning and ulti- necessarily be determined. The original injury can be used
mate neuroplasticity. only as an estimate of the damage with some indicators
The elements of neuroscience research on neural adaptation for prognosis and recovery. The rest of the success of reha-
have been summarized into 14 principles to guide rehabilitation bilitation and restoration of function will reside with the
programs designed to facilitate experience-dependent plastic- motivation and commitment of the individual. How that
ity.96,226,227 These principles, outlined in Box 4-1, are similar to motivation and commitment are initially established and
those suggested by Nudo129 as well as Kleim and Jones128 and continually reinforced is based on the patient, the thera-
Byl and colleagues.228 Although these principles are particularly pist’s interactive skills and emotional bond (see Chapter 5),
CASE STUDY 4-1 n PERSON WITH PARKINSON DISEASE

People with Parkinson disease develop an array of deficits production, which is evident in the acceleration and decelera-
in motor control that interfere with multiple ADLs and can tion phases of the movement. If the rate of force production is
eventually degrade quality of life. When motor control deficits altered; amplitude of force production may also be affected.
are evaluated in this population, the severity of the disease, the The person may have a decreased ability to predict and
activity level of the person, and the medication schedule are prepare the motor pattern for turning before the actual turn.
important considerations. Following are several motor control There appears to be a slow initiation of the turning task. This
deficits that are evident in this disease. phenomenon could be caused by an inability to sequence the
Motor control impairments of rigid tone and bradykinesia cre- motor behavior as a whole. Several researchers have observed
ate alterations in stride length, speed, and step frequency in the that the person completes one movement before starting the
gait pattern of persons with Parkinson disease. The person may next movement in the sequence rather than executing a smooth,
demonstrate a gait pattern characterized by decreased amplitude continuing movement pattern.25 Another reason for the
of leg movement, duration of the gait cycle, trunk rotation, and decrease in the ability to perform this task smoothly is the
arm swing. Difficulty initiating gait, or “freezing,” is an observ- patient’s dependence on visual feedback. Relying more heavily
able motor control problem, and small shuffling steps and a on visual feedback to accomplish a task slows the movement.
festinating gait pattern are also common.240 Impaired righting and The movement deficits observed may also be a result of the
balance reactions can contribute to gait instability. When the inability to effectively coordinate movements such as those
demands on gait velocity and frequency are altered, metabolic observed between postural and motile components of the task.
cost increases and the ability of the individual to safely complete Postural strategies may be classified on a continuum that in-
the functional movement with appropriate coordination of cludes postural preparations, postural adaptations, and postural
postural and motor control is challenged. reactions.243 The person with Parkinson disease may not predict
A practical outcome measure that can be used to capture the and make appropriate postural adjustments before the movement
effect of these motor deficits on functional mobility is the Timed and may have deficits in adaptive and reactive postural responses
Up-and-Go Test (TUG). This quick test can be used to capture (e.g., righting and equilibrium reactions, and adapting to envi-
the time it takes for a person to initiate sit to stand, transition ronmental demands). In the case of the TUG, movement and
from sit to stand, walk 3 meters, turn around, walk back, turn, balance strategies are assessed when the client stands up and
and sit down. During the performance of these sequential move- sits down. If the client does not use a controlled descent into the
ment patterns the clinician can observe gait deviations, postural chair but rather falls backward, what are the possible causes for
abnormalities, movement amplitude, and safety awareness. the sudden descent? The client’s goal may be to land in the chair,
Episodes and duration of “freezing,” or failed initiation, can be but the preferred pattern may be to fall into the chair. The indi-
accounted for, and the number of steps to turn 180 degrees can vidual may not be able to predict the time and force needed to
be documented.241 The overall time to complete the test can activate the muscles for a smooth descent, the individual may be
supply the clinician with objective data related to fall risk. deconditioned and not have the strength or endurance to perform
Overshooting or undershooting the 3-meter line before a smooth descent, or the individual may not have the balance
turning may be demonstrated. Although clients with Parkinson strategies required to perform this maneuver.
disease are able to prepare the motor strategy and use advance The client with Parkinson disease is one example of a client
information, the primary problem is slow onset of execution of with a neurological condition that affects motor output and
movement; therefore changing motor patterns (e.g., switching control. Regardless of the diagnosis, all aspects of motor
from walking to turning) can be quite difficult. control need to be examined and activity-based tests must be
To illustrate the multiple motor control deficits, imagine a conducted to determine how the impairments interact to affect
patient performing the TUG.242 The person may have difficulty the execution of functional tasks. A few key deficits in gross
accelerating to walk and decelerating to turn around and may and fine motor control and postural control were examined
have difficulty decelerating when approaching the chair and in this example. It is not within the scope of this section to
sitting down. Postural instability may be observed during present all the motor and postural control deficits but to
transfers and turning, and a loss of balance in the backward highlight the complexity of patients with neurological
direction without the activation of an automatic stepping pathology. Accurate identification of motor control problems in
response may occur. The motor control deficits exhibited in the clients assists the therapist and the client in the development of
patient with Parkinson disease are numerous and intertwined realistic functional goals and effective intervention programs
and their severity is influenced by the progression of the (see Section II for recommendations regarding specific diseases
disease. As mentioned earlier, the client cannot appropriately or pathological conditions and their related body system
control the increase and decrease in the rate of force impairments and activity-based functional limitations).
and the family and other support systems surrounding the References
client (see Chapter 6). To enhance this text and add value for the reader, all refer-
ences are included on the companion Evolve site that ac-
Acknowledgment companies this textbook. This online service will, when
All the present authors and the editors would like to thank both available, provide a link for the reader to a Medline abstract
Roberta Newton, PT, PhD, and Sharon Gorman, PT, DPTSc, for the article cited. There are 250 cited references and other
GCS for their commitment to this text’s evolution, as well as general references for this chapter, with the majority of
to the delivery of best practices to the elderly population. those articles being evidence-based citations.
CHAPTER 5 The Limbic System: Influence over
Motor Control and Learning
DARCY A. UMPHRED, PT, PhD, FAPTA, MARCIA HALL THOMPSON, PT, DPT, DSc,
and THERESE MARIE WEST, PhD, MT-BC, FAMI

amygdala After reading this chapter the student or therapist will be able to:
declarative memory 1. Understand the complexity of the limbic network and the influence of the limbic
emotional behavior network on behavioral and functional responses.
F2ARV (fear and frustration, anger, rage, violence/ 2. Describe the behavioral responses directly influenced by the limbic network.
withdrawal) continuum 3. Describe the structures of the limbic network.
general adaptation syndrome (GAS) 4. Describe the interaction between the limbic network and body systems responsible for
hippocampus behavioral responses.
hypothalamus 5. Differentiate between limbic-driven motor control responses and frontal, cerebellar,
limbic network and basal ganglia motor regulation.
motor learning 6. Differentiate between declarative and procedural learning.
MOVE (motivation or memory, olfaction, visceral, 7. Identify signs of both positive and negative limbic network influence on a client’s
autonomic nervous system, emotional) observable behavior and functional responses.
8. Describe appropriate treatment interventions or program modifications for both the
limbic high and limbic low client.
9. Understand the influence of the therapist over the limbic network and behavioral and
functional responses, and effectively integrate limbic network treatment techniques into
current treatment models.
S ince the publication of the fifth edition of this book, the

limbic network has emerged as a key component of
central nervous system (CNS) function, becoming one of the
for and compliance with home programs, levels of func-
tional activity, and empowerment over treatment planning
and life activities.
most researched areas of the CNS when analyzing behavior, Obviously, the human organism is a complex totality
learning, emotions, and their influence on activities and par- made up of many interlocking parts. The medical system has
ticipation. In the past, review of the literature on the limbic traditionally divided the body into systems and has forgotten
network was limited to investigating potential interactions that each specific system is co-dependent on many other
of other systems with nuclei within the limbic network. This systems for function. Today the medical profession is redis-
is no longer the case, as neuroscience research has helped to covering the importance of how the systems interact with
identify the critical nature of behaviors controlled or influ- and influence one another.22,23 It is very important that
enced by the limbic network. Based on research at a cellular movement specialists do not fall into the same trap as
level,1-3 a consciousness level,4-7 a bodily systems level,8-10 medicine in the past and look at movement from only a
and a quantum level11-13 it is now clear that the motor system biomechanical, muscular, neurological, cardiopulmonary, or
is just one of the many systems affected by the complex integumentary system perspective. They must consider the
limbic network.14-21 interaction among systems and their subsystems within an
Although far from yielding a complete understanding, individual. For example, the motor system is a system in and
this research and knowledge are increasing daily and force of itself. But cognitive impairment and limbic network
today’s therapist not only to recognize limbic behavior but involvement can lead to tremendous errors in motor responses
also to develop an understanding of how involvement of even when the motor system is intact. In our clients with
the limbic network will positively and negatively affect CNS dysfunction, impairments exist in the motor and limbic
each patient. Therapists can no longer think of motor con- network and in cognition, thus creating the potential for a
trol and motor learning as controlled exclusively by an complex set of behavioral responses to internal or external
anatomically unique motor system, nor can we understand environmental influences.
movement using motor control or motor learning principles Again, the totality of these problems is like interlocking
alone. Therapists must consider how emotions may influ- pieces of a complicated puzzle. The therapist learner must
ence selection of health care services and how the limbic always maintain clear visualization of the entire puzzle (the
network strongly influences treatment participation and out- client and all his or her systems) while analyzing any one
comes, such as motivation and cooperation, responsibility piece or component system. The process of unraveling the
99
multisystem “puzzle” and adding new pieces of learning is presented as a critical element in a student’s education or
the journey a therapist learner begins in school and can con- background for identifying the rationale for behavioral
tinue throughout his or her career. This is one example of the responses. This chapter has been written to provide the
limbic network’s influence in our own work as therapists. reader with the realization that without an understanding
The decision to continue on a learning journey is driven by of limbic interactions and modulations over motor expres-
desire to learn and answer questions regarding the unknown. sion, patient outcomes will always be variable even with
The emotions felt by the therapist learner in pursuit of mas- consistent and accepted interventions. Similarly, the reli-
tery and the ability to have the intellectual memory of the ability and validity of measurements of motor perfor-
learning are also limbic functions. These behavioral responses mance will always be in question and often inconsistent.
play an important role in all our lives and in the lives and And, given the limitations in today’s health care delivery
recovery of our patients/clients, as we will continue to inves- models, stresses, and the growing dependence on home
tigate in this chapter. programs, without a keen awareness of the limbic responses
A patient example of these interactions can be found in of both the patient and the provider, a therapist will have
a case description of a middle-aged woman admitted to the little guarantee of the best possible functional outcome for
intensive care unit (ICU) with multiple pelvic fractures and patients.
diagnosed with severe internal bleeding, kidney failure, For the student learner, the first section of the chapter is
pneumonia, pulmonary emboli, and severe clotting in the a discussion of limbic behavior and how to begin to dif-
lower extremities. The physician took her husband aside ferentiate true motor responses from those entangled in
to let him know that she was going to die, to which her limbic interactions. For the therapist learner desiring ongo-
husband replied, “I understand. Juggling one system prob- ing clinical mastery, the second section delves into the
lem is easy, juggling two systems takes a little practice, anatomy and physiology of the limbic network, the biology
and three-system involvement may challenge the best of learning and memory, neurochemistry, and neuroplastic-
medical skill. She is presenting four or five body system ity. The third section discusses the immediate relevance
failures and you are sure no one can juggle that many prob- to both the student and practicing clinician—how can we
lems.” The doctor said yes and the patient’s husband then apply our understanding of the limbic network to our patient
said, “Please keep juggling and don’t worry about me, be- assessments, treatment, and interactions? In other words,
cause if you do, I would then be one more ball to juggle.” how might it change what we do “come Monday morn-
And it did seem that every time the doctors got a handle on a ing”? And finally, in the last section, current advances and
body system problem, another system would fail. She re- future research possibilities in the role of the limbic net-
quired services from an endocrinologist, infection control work are explored.
specialists, interventional radiologists, a pulmonologist, a The concept of patient/client-centered therapy has
hematologist, a vascular surgeon, an internal medicine evolved to become an important aspect of health care deliv-
specialist, a urologist, and a nephrologist. Each specialist ery.24-34 The desire to improve or regain function can be
shared his or her limited experience with a complex clini- self-motivated, but very often it is instilled through the clini-
cal problem like this and that there was nothing in the lit- cian to the patient that his or her best interests and unique
erature to help his or her respective understanding. After goals are the focus of the health care team. This belief
21⁄2 months in the ICU, the woman survived. The physi- is based on trust, hope, and attainable steps toward desired
cian who had foretold her death met again with the patient and realistic goals. Patients know that their desires, interests,
and her family. He stated, “How are you still alive? I know and needs as unique and valued members of society are
what we did medically, but that was not enough to keep considered. They first believe and then recognize that they
you alive.” And he was right. No model within each re- are persons with specific problems and desired outcomes.
spective field could account for her recovery. However, the Although they may have specific medical diagnoses, be
piece not considered within her medical management was placed on clinical pathways, administered drugs, and sent
the beliefs and spiritual strength of the patient and her fam- off to the next facility in a couple of days, patients need to
ily, a positive limbic network influence on the function of feel that they, as individuals, have not lost all individuality
each failing body systems. This concept of limbic influ- and that someone cares. That need is a feeling of security
ence will be further discussed in the third section of this and safety that bonds a patient to a therapist along the
chapter. journey of learning.35-37
So why has this chapter been positioned so promi- Before understanding and becoming compassionate
nently within a textbook on basic neurological rehabilita- regarding the needs of other people, such as patients with
tion? In many curricula the limbic network is discussed signs and symptoms of neurological problems, therapists
only in a basic science course of neuroanatomy and neu- need to understand their own limbic network and how it
rophysiology. In others, the limbic role in declarative affects others who might interact with them.38-43 Because
memory and emotional responses is presented as part of a both occupational and physical therapy professions have
discussion on memory or cognitive function within a psy- evolved to using enablement models and systems interac-
chology course. Yet today’s curricula do stress and accept tions to explain movement responses of their respective
“motivation and attention” by the patient as key factors in client populations, separating limbic from true motor or
neuroplasticity and motor learning. Similarly, discussions cognitive impairments will help guide the clinician toward
about the negative effects of “fear of falling” on balance intervention strategies that will lead to the quickest and most
and function in the elderly population are stressed. Both effective outcomes.
components are controlled by the limbic network, yet The complexity of the limbic anatomy, physiology, and
the science behind how the system works is often not neurochemistry baffles the minds of basic science doctoral
CHAPTER 5 n The Limbic System: Influence over Motor Control and Learning 101
students. The changes in understanding of cellular metabo- Yet that individual needs to be willing to experience the
lism, membrane potentials, and the new mysteries of cell unknown to learn and adapt. The willingness, drive, and
communication and memory perplex the world of science adaptability of that individual will affect the optimal plastic-
and neuroscience.44-47 How this microcosm relates to the ity of the CNS.62 The limbic network is a key player that
macroworld and how the external environments influence drives and motivates that individual. The lack of awareness
not only consciousness but all levels of CNS function are of that variable or its effect on patient performance will ul-
slowly unraveling but still remain mysteries. Yet a therapist timately lead to questions and doubts about the effectiveness
deals with the limbic network of clients on a moment- and efficacy of both assessment and intervention results.
to-moment functional level throughout the day. Figure 5-1 Similarly, if this system is overwhelmed either internally or
illustrates the interlocking co-dependency of all major CNS externally, it will dramatically affect neuroplasticity and
components with the environment. At no time does any sys- motor learning as well as cognitive, syntactical learning (see
tem stand in isolation. Thus from a clinical perspective the Chapter 4). At the conclusion of this chapter it is hoped that
therapist should always maintain focus on the whole envi- therapists will comprehend why there is a need to learn to
ronment and all major interactive components within it, modulate or neutralize the limbic network so that patients
while directing attention to any specific component. How can functionally control movement and experience cognitive
the feedback (internal and external) to the patient’s CNS learning. Then therapists need to reintroduce emotions into
changes the neurochemistry and membrane potential, trig- the activity and allow the patient to once again experience
gers memory, creates new pathways, or elicits other poten- movement and cognitive success during various levels of
tial responses is not the responsibility of the clinician or emotional demands and environments. This change in the
therapist. The responsibility of the clinician is accurate emotional environment will create novelty of the task. This
documentation of changes and consistency of those changes novelty is a critical motivator for learning and will drive
toward desired patient outcomes. The professions that focus neuroplasticity.63-65
on movement science are interacting more closely with
the neurosciences and other biological sciences and many
THE FUNCTIONAL RELATIONSHIP
related professions to unravel many of these mysteries and
OF THE LIMBIC NETWORK TO CLINICAL
create better assessment and intervention procedures for
PERFORMANCE
future patients.
The primary purpose of this chapter is to discuss the The Limbic Network’s Role in Motor Control,
influence of the limbic network on motor learning, motor Memory, and Learning
performance, neuroplasticity, and functional independence It is not easy to find a generally accepted definition of the
in life activities. If a person is fearful or apprehensive, motor “limbic network or complex,” its boundaries, and the com-
performance and the ability to learn either a motor skill ponents that should be included. Mesulam66 likens this to a
or intellectual information will be very different48-55 from fifth-century bce philosopher’s quotation, “the nature of
that of an individual who feels safe, is given respect, and God is like a circle of which the center is everywhere and the
becomes part of the decision-making process and thus func- circumference is nowhere.” Brodal67 suggests that func-
tions inherently with control.52,56-61 tional separation of brain regions becomes less clear as we
An individual will naturally have feelings of loss and discover the interrelatedness through continuing research.
reservations or fears about the unknown future after injury He sees the limbic network reaching out and encompassing
to any part of the body, but especially the CNS (see Chapter 6). the entire brain and all its functional components and sees
no purpose in defining such subdivision. Although the ana-
tomical descriptions of the limbic network may vary from
author to author, the functional significance of this system is
widely acknowledged in defining human behavior and be-
havioral neurology.68
Brooks69 divides the brain into the limbic brain and the
nonlimbic sensorimotor brain. He also defines the two limbic
and nonlimbic systems functionally, not anatomically, because
their anatomical separation according to function is almost
impossible and task specific (Figure 5-2). The sensorimotor
portion is involved in perception of nonlimbic somatosensory
sensations and motor performance. Brooks defines the limbic
brain component as primitive and essential for survival, sens-
ing the “need” to act. The limbic brain is also responsible for
memory and the ability to select what to learn from each expe-
rience, either positive or negative. Thus the overall purpose of
the limbic network is to initiate need-directed motor activity
for survival, based on experience. The limbic network there-
fore initiates and can send neurons up to the frontal lobe or
down to the brainstem and thus regulates motor output.
Kandel and colleagues56 state that functional behavior
Figure 5-1 n Interlocking co-dependence of all major central requires three major systems: the sensory, the motor, and the
nervous system components. motivational or limbic systems. When a seemingly simple
Figure 5-2 n Divisions and interconnections between the limbic and nonlimbic cortices (sensory
and motor areas).
action, such as swinging a golf club, is analyzed, the sensory motor input, and (3) modifies motor expression accordingly,
system is recruited for visual, tactile, and proprioceptive influencing both the autonomic and the somatic sensorimo-
input to guide the motor systems for precise, coordinated tor systems. It thereby plays a role in controlling the skeletal
muscle recruitment and postural control. The motivational muscles through input to the frontal lobe and brain stem and
(limbic) system does the following: (1) provides intentional the smooth muscles and glands through the hypothalamus,
drive for the movement initiation, (2) integrates the total which lies at the “heart” of the limbic network (Figure 5-3).
Motor centers
BG + cerebellum
Internal
feedback
Figure 5-3 n Motivational system’s influence over the sensorimotor and autonomic nervous systems.
(Adapted from Kandel ER, Schwartz JH, Jessell TM: Principles of neural science, ed 4, New York,
2000, McGraw-Hill.)
Noback and co-workers70 state that the limbic network The complexity of the limbic network and its associative
is involved with many of the expressions that make us influence over both the motor control system and cortical
human; namely, emotions, behaviors, and feeling states. That structures are enormous. A therapist dealing with a client
humanness also has individuality. Our unique memory with motor control or cognitive learning problems needs
storage, our variable responses to different environmental to understand how the limbic network affects behavioral
contexts, and our control or lack thereof over our emo- responses. The knowledge base focuses not only on the
tional sensitivity to environmental stimuli all play roles in client’s deficits but also on the integrative function of the
molding each one of us. Because of this uniqueness, each therapist. This understanding should lead to a greater aware-
therapist and each client need to be accepted for their own ness of the clinical environment and the factors within the
individuality. environment that cause change. Without this knowledge
Broca71 first conceptualized the anatomical regions of the of how to differentiate systems, objective measurements of
limbic lobe as forming a ring around the brain stem. Today, motor performance or cognitive abilities may be inconsistent
neuroanatomists do not differentiate an anatomical lobe as without any explanation. Similarly, with excessive limbic
limbic, but rather refer to a complex system that encom- activity, clients’ ability to store and retrieve either declarative
passes cortical, diencephalon, and brain stem structures.56 or procedural learning may be negatively affected, thus lim-
This description is less precise and encompasses but is not iting the patients’ ability to benefit from traditional interven-
limited to the orbitofrontal and prefrontal cortex, hippocam- tions and from potentially regaining their respective highest
pus, parahippocampal gyrus, cingulate gyrus, dentate gyrus, quality of life.
amygdaloid body, septal area, hypothalamus, and some
nuclei of the thalamus.56,72-76 Anatomists stress the impor-
The Limbic Network’s Influence on Behavior:
tance of looking at the interrelated structures and segments Its Relevance to the Therapeutic Environment
or loops within the complex limbic region.77,78
These multiple nuclei and interlinking circuits play cru- Levels of Behavioral Hierarchies: Where Does the
cial roles in behavioral and emotional changes77,79,80 and Limbic Network Belong?
declarative memory.79-96 The loss of any link can affect the Strub and Black109 view behavior as occurring on distinct
outcome activity of the whole circuit. Thus damage to any interrelated levels that represent behavioral hierarchies.
area of the brain can potentially cause malfunctions in any Starting at level 1, a state of alertness to the internal and
or all other areas, and the entire circuit may need reorganiza- external environment must be maintained for motor or men-
tion to restore function. tal activity to occur. The brain stem reticular activating sys-
Researchers do not ascribe a specific single function to tem brings about this state of general arousal by relaying in
CNS formations but see each as part of a system participating an ascending pathway to the thalamus, the limbic network,
to various degrees in the multitude of behavioral responses and the cerebral cortex. To proceed from a state of general
(see Chapters 3 and 4 for additional information). Therefore arousal to one of “selective attention” requires the commu-
the loss of any part of higher centers or the limbic network nication of information to and from the cortex, the thalamus,
may not be clearly definable functionally, and the return of and the limbic network and its modulation over the brain
function is not always easy to predict. stem and spinal pattern generators.56,110
Recovery of function after injury may involve mecha- Level 2 of this hierarchy lies in the domain of the hypo-
nisms that allow reorganizing of the structure and function thalamus and its closely associated limbic structures. This
of cortical, subcortical, and spinal circuits. In very young level deals with subconscious drives and innate instincts.
infants, areas within opposite hemispheres may “take over” The survival-oriented drives of hunger, thirst, temperature
function, whereas in more mature brains reorganization of regulation, and survival of the species (reproduction) and the
existing systems seems to be the current accepted hypothe- steps necessary for drive reduction are processed here, as
sis within the expanding knowledge of neuroplasticity.97-100 well as learning and memory. Most of these activities relate
For complex behavior, such as in motor functioning requir- to limbic functioning. If an individual or patient is in a per-
ing many steps, the limbic network, cortex, hypothalamus, ceived survival mode, little long-term learning regarding
basal ganglia, and brain stem work as an integrated unit, either cognition or motor programming will occur. Thus,
with any damaged area causing the whole system to initially making the patient feel safe is initially a critical role for the
malfunction. Without change or encouragement of appropri- therapist. This approach may require placing the therapist’s
ate external and internal environmental changes that will hands on the patient initially to take away any possibility of
create neuroplasticity, the initial malfunction can become falling. The therapist would first deal with the emotional
permanent.101 The timing for optimal neuroplasticity has not aspect of the patient’s environment and then shift to the
yet been established. The medical use of drugs to alter cel- motor learning and control component, in which the patient
lular activity and plasticity after CNS damage has become is empowered to practice and self-correct within the pro-
a huge pharmaceutical research area (see Chapter 36). Early gram she or he can control.
as well as later drug therapy may encourage neuroplastic- On level 3 only cerebral cortical areas are activated. This
ity.102-108 The same questions must be asked about early level deals with abstract conceptualization of verbal or
instead of later rehabilitation intervention, as well as the quantitative entities. It is at this level that the somatosensory
limbic influence over the motor system. A loss of function and frontal motor cortices work together to perceptually and
or a change in behavior cannot necessarily be localized as procedurally develop motor programs. The prefrontal areas
to the underlying cause. A lesion in one area may cause of the frontal lobe can influence the development of these
secondary dysfunction of a different area that is not actually motor programs, thus again illustrating the limbic influence
damaged. over the motor system.111,112
Level 4 behavior is concerned with the expression of Emotion: feelings and attitude
social aspects of behavior, personality, and lifestyle. Again, Self-concept and worth
the limbic network and its relationship to the frontal Emotional body image
lobe are vital. The shift to the World Health Organization Tonal responses of motor system affected by limbic
International Classification of Functioning, Disability and descending pathways
Health (WHO-ICF) model, which reflects patient-centered Attitude, social skills, opinions
therapy, has actualized the critical importance of this level of As seen in this outline, the M (memory, motivation)
human behavior.24,28,31,113,114 depicts the drive component of the limbic network. Before
The interaction of all four levels leads to the integrative learning, an individual must be motivated to learn, to try to
and adaptable behavior seen in the human. Our ability to succeed at the task, to solve the problem, or to benefit from
become alert and protectively react is balanced by our previ- the environment. Without motivation the brain will not ori-
ous learning, whether it is cognitive-perceptive, social, or ent itself to the problem and learn. Motivation drives both
affective. Adaptability to rapid changes in the physical envi- our cortical structures to develop higher cognitive associa-
ronment, in lifestyles, and in personal relationships results tions and the motor system to develop procedures or motor
from the interrelationships or complex neurocircuitry of the programs that will enable us to perform movement with the
human brain. When insult occurs at any one level within least energy expenditure and the most efficient patterns
these behavioral hierarchies, all levels may be affected. available. Once motivated, the individual must be able to
As Western medicine is unraveling the mysteries behind pay attention and process the sequential and simultaneous
the neurochemistry of the limbic network58,115-117 and alter- nature of the component parts to be learned, as well as the
native medicine is establishing effectiveness and efficacy for whole. Thus there is an interlocking dependence among
various interventions and philosophies (see Chapter 39), a somatosensory mapping of the functional skills124 (cogni-
fifth level of limbic function may become the link between tive), attention (limbic) necessary for any type of learning,
the hard science of today and the unexplained mysteries. and the sequential, multiple, and simultaneous programming
Those medical mysteries would be defined as unexplained of functional movement (motor). The limbic amygdala and
yet identified events that have either been forgotten or been hippocampal structures and their intricate circuitries play a
hidden from the world by those scientists—mysteries such key role in the declarative aspect of memory.125-128 Once this
as why some people heal from terminal illnesses spontane- syntactical, intellectual memory is learned and taken out of
ously, various others heal in ways not accepted by tradi- short-term memory by passing through limbic nuclei, the
tional medicine,60,118 and still others just die without any information is stored in cortical areas and can be retrieved at
known disease or pathological condition.119-122 One critical a later time without limbic involvement.129
component everyone identifies as part of that unexplained The O refers to olfaction, or the incoming sense of smell,
healing is a belief by the client that he or she will heal. That which exerts a strong influence on alertness and drive. This
belief has a strong emotional component,120 and that may is clearly illustrated by the billions of dollars spent annually
be the fifth level of limbic function. How conscious intent on perfumes, deodorants, mouthwashes, and soap as well as
drives hypothalamic autoimmune function is being unrav- scents used in stores to increase customers’ desires to pur-
eled scientifically, and clinicians often observe these changes chase. This input tract can be used effectively by therapists
in their patients. Through observation it becomes apparent who have clients with CNS lesions such as internal capsule
that clients who believe they will get better often do, and and thalamic involvement. The olfactory system synapses
those who believe they will not generally do not. Whether within the olfactory bulb and then with the limbic structures
belief comes from a religious, spiritual, or hard science and then may go directly to the cerebral cortex without syn-
paradigm, that belief drives behavior, and that drive has a apsing in the thalamus. Although collaterals do project to the
large limbic component. thalamus, unlike all other sensory information, olfaction
does not need to use the thalamus as a necessary relay center
The Limbic Network MOVEs Us to access the cortical structures, although many collaterals
Moore123 eloquently describes the limbic network as the also project there.56,130 Other senses may not be reaching the
area of the brain that moves us. The word MOVE can be cortical levels, and the client may have a sensory-deprived
used as a mnemonic for the functions of the limbic network. environment. Olfactory sensations, which enter the limbic
Limbic Network Function. network, may be used to calm or arouse the client. The
Memory and motivation: drive specific olfactory input may determine whether the person
Memory: attention and retrieval, declarative learning remains calm or emotionally aroused.131,132 Pleasant odors
Motivation: desire to learn, try, or benefit from the external would be preferable to most people. With the limbic net-
environment work’s influence on tone production through brain stem
Olfaction (especially in infants) modulation, this is one reason aromatherapy causes relax-
Only sensory system that does not have to go through the ation and is used by many massage therapists.
thalamus as a second-order synapse in the sensory A comatose, seemingly nonresponsive client may respond
pathway before it gets to the cerebral cortex to or be highly sensitive to odor.133 The therapist needs to be
Visceral (drives: thirst, hunger, and temperature regulation; acutely aware of the responses of these patients because these
endocrine functions) responses may be autonomic instead of somatomotor and may
Sympathetic and parasympathetic reactions be reflected in a higher heart rate or an increase in blood
Hypothalamic regulation over autoimmune system pressure. Using noxious stimuli to try to “wake up” a patient
Peripheral autonomic nervous system (ANS) responses in a vegetative state has the possibility of causing negative
that reflect limbic function arousal, fear, withdrawal, or anxiety and an increase in base
tone within the motor generators.132 Using this type of input model in which consensus creates the observed behavior.
places the patient at level 2 in a “protective state of survival.” To illustrate this concept, think of an orchestra leader con-
Using a pleasant and personal desirable smell will more ducting a symphony. It would make no sense for the con-
likely place a client at level 2 “safety.” The former can lead ductor to ask the string section to play louder if half the
to strong emotions such as anger, whereas the latter often brass section got sick. Instead, the conductor would need to
leads to bonding and motivation to learn. Research has quiet the string section and all other sections to allow the
shown that retrieval processing and retrieval of memory brass component to be heard.
have a distinctive emotionality when they are linked to odor- E relates to emotions, the feelings, attitudes, and beliefs
evoked memories.134-136 that are unique to that individual. These beliefs include
The V represents visceral or autonomic drives. As noted psychosocial attitudes and prejudices, ethnic upbringing,
earlier, the hypothalamus is nestled within the limbic net- cultural experiences, religious convictions, and concepts of
work. Thus, regulation of sympathetic and parasympathetic spirituality.120 All these aspects of emotions link especially
reactions, both of the internal organ systems and the periph- to the amygdaloid complex of the limbic network and
ery, reflects continuing limbic activity. Obviously, drives orbitofrontal activity within the frontal lobe.140-142 This is a
such as thirst, hunger, temperature regulation, and sexuality primary emotional center, and it regulates not only our self-
are controlled by this system. Clients demonstrating total concept but our attitudes and opinions toward our external
lack of inhibitory control over eating or drinking or mani- environment and the people within it.
festing very unstable body temperature regulation may be To appreciate the sensory system’s influential interaction
exhibiting signs of hypothalamic-pituitary involvement or with the limbic network directly, the reader need only look
direct pathways from hypothalamus to midbrain struc- at the literature on music and how it interacts with emo-
tures.56 Today, this interaction of the hypothalamus with tions.143,144 Most people can give examples of instances
motor neurons that change or support movement has clearly where music has elicited immediate and compelling emo-
been established.137 tional responses of various types. Pleasant and unpleasant
Less obvious autonomic responses that may reflect lim- musical stimuli have been found to increase or decrease
bic imbalances often go unnoticed by therapists. When the limbic activity and influence both cognitive and motor
stress of an activity is becoming overwhelming to a client, responses. Although the neurological mechanisms are not
she or he may react with severe sweating of the palms or an yet well understood, the limbic network seems to be impli-
increase in dysreflexic activity in the mouth rather than with cated in both “positive” and “negative” emotions in response
heightened motor activity. A therapist must continually to musical stimuli.145-148 The clinical implications are huge.
monitor this aspect of the client’s response behaviors to as- Excessive noise, loudspeaker announcements, piped in
certain that the behaviors observed reflect motor control and music, and all the therapists’ voices can affect the CNS of a
not limbic influences over that motor system. client. These responses can be highly emotional, cause changes
If the sensory input to the client is excessive whether in visceral behavior, and affect striated motor expression.
through internal or external feedback, the limbic network Level of musical consonance or dissonance is just one ele-
may go into an alert, protective mode and will not function ment of the auditory stimulus that is subjectively experienced
at the optimal level, and learning will diminish. The client by the listener as pleasant or unpleasant. The implications
may withdraw physically or mentally, lose focus or atten- not only that listening to music affects limbic emotional
tion, decrease motivation, and become frustrated or even states but that the influence may direct the hypothalamus in
angry. The overload on the reticular system may be the regulation of blood flow within the CNS have also been
reason for the shutdown of the limbic network and not the shown.148 With music or sound being just one input system,
limbic network itself. Both are part of the same neuroloop the therapist must realize that sensory influence from smell,
circuitry. All these behaviors may be expressed within the taste, touch, proprioception, and vestibular and organ sys-
hypothalamic-autonomic system as motor output, no matter tem dysfunction can lead to potential limbic involvement in
where in the loop the dysfunction occurs. Having a func- all aspects of CNS function and directly affect the emotional
tional understanding of the neuroanatomy and their rela- stability of the patient.
tionships with each other helps therapists unravel some of Another very important concept linked to the emotional
the mysteries patients present after CNS insult.138,139 The system is the emotional aspect of body image or the concept
evaluation of this system seems even more critical when a of SELF. For example, assume that one morning I look in
client’s motor control system is locked, with no volitional the mirror and say, “The poor world, I will not subject it to
movement present. Therapists often try to increase motor me today.” I then go back to bed and eat nothing for the rest
activity through sensory input; however, they must cau- of the day. The next day I get up and look in the same mirror
tiously avoid indiscriminately bombarding the sensory sys- and say, “What a change, I look trim and beautiful. Look out
tems. The limbic network may demonstrate overload while world, here I come!” In reality, my physical body has not
at the same time the spinal motor generators reflect inade- been altered drastically, if at all, but my attitude toward that
quate activation. How a therapist might assess this overload body has changed. That is, the emotional component of my
would be to closely monitor the ANS’s responses such as body image has perceptually changed.
blood pressure, heart rate, internal temperature, and sweat- A second self-concept deals with my attitude about my
ing versus observing or measuring muscle tone. Although worth or value to society and the world and my role within
the somatosensory system and the ANS are different, they it.149 Again, this attitude can change with mood, but more
are intricately connected. The concept of massively bom- often it seems to change with experience. This aspect of
barding one system while ignoring the other does not make client-therapist interaction can be critical to the success of
sense in any learning paradigm, especially from a systems a therapeutic environment. The two following examples
illustrate this point, with the focus of bringing perceived has great impact on our learning and motor control. If a
roles into the therapeutic setting: patient is not motivated and places little value on a motor
Your client is Mrs. S., a 72-year-old woman with a left output, then complacency results and little learning will
cerebrovascular accident (CVA). She comes from a occur.159-161 On the other hand, if a therapist places an ex-
low socioeconomic background and was a house- tremely high value on a motor output as a pure expression
keeper for 40 years for a wealthy family of high social of motor control without interlocking that control with the
standing. When addressing you (the therapist), she patient’s limbic influence, the behavioral response may
always says “yes, ma’am” or “no, ma’am” and does lead to inconsistency, lack of compliance, and thus lack
just what is asked, no more and no less. It may be very of motor learning and carryover.159 Similarly, it can cause
hard to empower this client to assume responsibility extreme stress, which even the general public knows causes
for self-direction in the therapeutic setting. Her per- disease.162
ceived role in life may not be to take responsibility or Motivation and Reward. Moore123 considers motiva-
authority within a setting that may, from her perception and memory as part of the MOVE system. Esch and
tion, have high social status, such as a medical facility. Stefano163 link motivation with reward and help, illustrating
She also may feel that she does not have the right or how the limbic network learns through repetition and reward.
the power to assume such responsibilities. Success in They state that the concept of motivation includes drive and
the therapeutic setting may be based more on chang- satiation, goal-directed behavior, and incentive. They recog-
ing her attitudes than on her potential to relearn motor nize that these behaviors maintain homeostasis and ensure
control. That is, the concept of empowerment may the survival of the individual and the species. Although the
play a crucial role in regaining independent functional frontal lobe region appears to play an important role in self-
skill and control over her environment.24,28,31,150-153 control and execution activities, these functions seem to
Your client is a 24-year-old lumberjack who sustained require a close interlocking neuronetwork between cognitive
a closed-head injury during a fall at work. It is now representation within the frontal regions and motivational
1 month since his accident, and he is alert, verbal, and control provided by limbic and subcortical structures.140,164
angry and has moderate to severe motor control prob- An important aspect of motivated behavior is linked to
lems. During your initial treatment you note that he patient- and family-centered therapy.* “The most powerful
responds very well to handling. He seems to flow with force in rehabilitation is motivation.”167 These words are
your movement, and with your assistance is able to strong and reflect the importance of the limbic network in
practice a much higher level of motor control within a rehabilitation.
narrow biomechanical window; although at times he Motivated behavior is geared toward reinforcement and
needs your assistance, you release that control when- reward, which are based on both internal and external feed-
ever possible to empower him to control his body. At back systems. Repeated experience of reinforcement and
the end of therapy he sits back in his chair with much reward leads to learning, changed expectancy, changed be-
better residual motor function. Then he turns to you havior, and maintained performance.168 Emotional learning,
(the female therapist) and instead of saying, “That was which certainly involves the limbic network, is very hard to
great,” he says, “You witch, I hate you.” The inconsis- unlearn once the behavior has been reinforced over and
tency between how his body responded to your han- over.169,170 For that reason, motor behavior that is strongly
dling and his attitude toward you as a person may linked to a negative emotional response might be a very dif-
seem baffling until you realize that he has always per- ficult behavior to unlearn. For example, a patient who is
ceived himself as a dominant male. Similarly, he per- willing to stand up and practice transfers just to get the
ceives women as weak, to be protected, and in need of therapist off his back is eliciting a movement sequence that
control. If his attitude toward you cannot be changed is based on frustration or anger. When that same patient gets
to see you in a generic professional role, he will most home and his spouse asks him to perform the same motor
likely not benefit as much from your clinical skills and behavior, he may not be able to be successful. The spouse
guidance as a teacher. Before the accident the patient may say, “The therapist said you could.” The patient may
may have suppressed that verbal response but not respond, “I never did like him!” Thus repetition of motor
tone and body language. After a traumatic brain injury performance with either the feeling of emotional neutrality
affecting the orbitofrontal system, the inhibition of or the feeling of success (positive reinforcement) is a critical
the behavioral response itself may be lost, further element in the therapeutic setting. Consistently making the
embarrassing the patient emotionally. motor task more difficult just when the client feels ready
Preconceived attitudes, social behaviors, and opinions to succeed will tend to decrease positive reinforcement or
have been learned by filtering the input through the limbic reward, lessen the client’s motivation to try, and decrease the
network. If new attitudes and behaviors need to be learned probability of true independence once the patient leaves
after a neurological insult, the status of the amygdaloid the clinical setting. When pressure is placed on therapists to
pathways seems crucial. Damage to these limbic struc- produce changes quickly, repetition and thus long-term
tures may prevent learning154; thus, socially maladaptive learning are often jeopardized, which may have a dramatic
behavior may persist, making the individual less likely effect on the quality of the client’s life and the long-term
to adapt to the social environment. It is often harder to treatment effects once he or she leaves the medical facility.
change learned social behaviors than any other type of Motor control theory (see Chapter 4) coincides with limbic
learning.155-158 Because our feelings, attitudes, values, and
beliefs drive our behaviors through both attention and
motor responses, the emotional aspect of the limbic network *
References 24, 27, 28, 31, 34, 135, 150, 165, 166.
research regarding reinforcement. Inherent feedback within and the left nucleus accumbens and cerebellum. The
a variety of environmental contexts allowing for error with researchers compared these results with those from the ear-
correction leads to greater retention.171 Repetition or the lier studies by members of the same team147,148 and suggest
opportunity to practice a task (motor or cognitive) in which that areas such as the subcallosal cingulate are related to
the individual desires to succeed will lead to long-term the direct experience of occurrent emotions rather than dis-
learning.172 Without practice or motivation the chance of criminate processing for emotion and that different areas are
successful motor learning is minimal to nonexistent. specifically activated during the pleasant physical responses
Positive emotional states may create a limbic environ- known as “chills.” They go on to propose that the superior
ment in which the therapist can link reward and pleasure temporal pole and adjacent insula may serve as a point of
associations to new motor sequences. Although it is well bifurcation in neural circuitry for processing music. They
known that appropriate selections of music can stimulate also suggest that neurons from that region project to limbic
states of highly pleasant positive affects and physical relax- and paralimbic areas involved in emotional processing and to
ation, the neurological mechanisms for these effects are premotor areas possibly involved in discrimination and struc-
not well understood. In an early study by Goldstein173a tural processing of music. Although research has increased
subjects reported pleasant physical sensations of tingling our appreciation of the complexity of brain activation by
or “thrills” in response to music listening. After subjects music, much more study is needed to validate a model of
were injected with naloxone, which blocks opiate recep- limbic network activity in human emotional responses to
tors, thrill scores and tingling sensations were attenuated in musical stimuli. Clinically, music can be used to improve
some subjects. Although responses to music are highly mood and increase patient motivation to participate in reha-
individualized and this study has not been replicated, it bilitation treatment. Case studies174,175 suggest that music
suggests that endorphins may be released under certain can be used to decrease crying by infants and toddlers dur-
music listening conditions that elicit pleasant physical sen- ing physical therapy treatment. West has participated in both
sations. In a positron emission tomography (PET) study developmental and rehabilitation settings as a music thera-
of cerebral blood flow (CBF) changes measured during pist in co-treatment with physical and occupational thera-
highly pleasurable “shivers or chills” in response to sub- pists. The music therapist first does a thorough assessment
ject-selected music, Blood and colleagues147 found that as of the individual’s preferences and responses to music, then
the intensity of the chills increased, CBF increases occurred provides music selected or composed specifically to provide
in the left ventral striatum, dorsomedial midbrain, bilateral motivating energy, pleasant associations, and positive affec-
insula, right orbitofrontal cortex, thalamus, anterior cingu- tive states to accompany the motor activity. This individual-
late cortex, supplementary motor area, and bilateral cere- ized, live-music approach allows the music therapist to
bellum. As the intensity of chills increased, significant modify the musical elements as needed in the moment,
CBF decreases were also observed in the right amygdala, working in a real-time limbic partnership with both the cli-
left hippocampus and amygdala, and ventral medial prefron- ent and the physical or occupational therapist. Music or
tal cortex. The increases found in brain structures associ- pleasure sounds can be used to help neutralize or balance
ated with reward or pleasant emotions and decreases in the limbic influence on motor expression. Obtaining a limbic-
areas associated with negative emotional states suggest neutral impact is critical before evaluating functional move-
that music (1) must be carefully selected according to indi- ment in order to accurately determine true motor system
vidual preferences and responses, in order to reliably elicit involvement.
such highly pleasurable experiences as “shivers down the Many types of emotions create motivation, such as plea-
spine” and (2) might be used therapeutically to positively sure, reward processes, emotions associated with addiction,
affect limbic activity. appreciation of financial benefits, amusement, sadness, humor,
Other studies provide additional support for the notion happiness, and depression.163,173b,176-179 Some emotions tend to
that music may activate limbic and paralimbic areas associ- drive learning, whereas others may discourage learning,
ated with reward or pleasurable emotions. Brown and col- whether that learning be cognitive or motor.
leagues145 conducted a PET study of 10 nonmusicians who
listened passively to unfamiliar music, which they later re- Integration of the Limbic Network as Part
ported had elicited strongly pleasant feelings. Unlike previ- of a Whole Functioning Brain
ous studies of music, emotion, and limbic activity, this re- Motivation, alertness, and concentration are critical in motor
search design called for subjects to listen passively without learning because they determine how well we pay attention
engaging in any task such as evaluating affective compo- to the learning and execution of any motor task. These pro-
nents during the music. The authors noted that the music cesses of learning and doing are inevitably intertwined: “We
stimuli used was musically complex and strongly liked by learn as we do, and we do only as well as we have
the subjects. When the CBF during the music was compared learned.”180
with silent rest conditions in the same subjects, activations Both motivation (“feeling the need to act”) and concen-
were seen, as expected, in areas presumed to represent per- tration (“ability to focus on the task”) are interlinked with
ceptual and cognitive responses to music (primary auditory the limbic network. The amygdaloid complex with its mul-
cortex, auditory association cortex, superior temporal sulcus titude of afferent and efferent interlinkages is specially
bilaterally, temporal gyrus of the right hemisphere, in the adapted for recognizing the significance of a stimulus, and it
right superior temporal pole, and adjacent insula). In addi- assigns the emotional aspect of feeling the need to act.
tion, responses were found in limbic and paralimbic areas, These neuroanatomical loops have tremendous connections
which included the left subcallosal cingulate, the anterior with the reticular system. Hence, some authors call it the
cingulate, left retrosplenial cortex and right hippocampus, reticulolimbic network.56,157 The interaction of the limbic
network and the motor generators of the brain stem and parahippocampal gyrus /isthmus, cingulate gyrus, and septal
ultimate direct and indirect modulation over the spinal sys- nucleus) has even greater influence over the sensorimotor
tem lead to need-directed and therefore goal-directed motor cortices through the cingulate gyrus, both directly and indi-
activity. It also filters out significant from insignificant infor- rectly through association areas.182-184 The thalamus, cere-
mation by selective processing and storing the significant bellum, and basal ganglia contribute to the production of the
for memory, learning, and recall. These interconnected neu- specific motor plans. These messages of the general plan are
roloop circuitries reinforce the concept that areas have both relayed to the projection system. The limbic structures
specialization and generalization and thus work closely through the cingulate gyrus also have direct connections
together with other areas of the brain.169,181 with the primary motor cortex. These circuits certainly have
Goal-directed or need-directed motor actions are the re- the potential to assist in driving fine motor activities through
sult of the nervous system structures acting as an interactive corticobulbar and corticospinal tract interactions. The thala-
system. Within this system (Figure 5-4), all components mus, cerebellum, basal ganglia, and motor cortices (premo-
share responsibilities. The limbic network and its cortical tor, supplementary motor, and primary motor) contribute
and subcortical components represent the most important to the production of the specific motor plans.56 Messages
level. In response to stimuli from the internal or external regarding the sensory component of the general plan are
environment, the limbic network initiates motor activity relayed to the projection system, where they are transformed
out of the emotional aspect of feeling the need to act. This into refined motor programs. These plans are then projected
message is relayed to the sensory areas of the cerebral cor- throughout the motor system to modulate motor generators
tex, which could entail any one or all association areas for throughout the brain stem and spinal system.56 Limbic con-
visual, auditory, olfactory, gustatory, tactile, or propriocep- nections with (1) the cerebellum, basal ganglia, and frontal
tive input. These areas are located in the prefrontal, occipi- lobe56,185-189 and (2) the motor generator within the brain
tal, parietal, and temporal lobes, where they analyze and stem enable further control of limbic instructions over motor
integrate sensory input into an overall strategy of action or a control or expression. If the limbic and the cognitive sys-
general plan that meets the requirements of the task. There- tems decide not to act, goal-driven motor behavior will
fore these cortices recognize, select, and prepare to act as cease. An individual’s belief (emotional and spiritual) can
a response to relevant sensory cues when a state of arousal inhibit even the most basic survival skills, as has been
is provided by reticular input. The limbic cortex (uncus, clearly shown in history when individuals with particular
Figure 5-4 n Functional and dynamic hierarchy of systems based on both limbic and motor con-
trol interactions. (Adapted from Brooks VB: The neural basis of motor control, New York, 1986,
Oxford University Press.)
religious beliefs were pitted against vicious predators and requirements. This insight helps the motor system correctly
those people chose not to defend themselves. select strategies that will successfully initiate and support
Within the projection system and motor planning com- the appropriate movement for accomplishing the task. This
plexes, the specifics are programmed and the tactics are knowledge of results feedback is required for effective
given a strategy. In general, “what” is turned into “how” motor learning and for forming the correct motor programs
and “when.” The necessary parameters for coordinated for storage.197,198
movement are programmed within the motor complex as to The reader may better understand the role of the limbic
intensity, sequencing, and timing to carry out the motor network in motor programming through a nonmedical
task. These programs, which incorporate upper motor neu- example. Imagine that you are sitting in your new car. The
rons and interneurons, are then sent to the brain stem and dealer has filled the tank with necessary fuel. The engine,
spinal motor generators, which in turn, through lower mo- with all its wires and interlocking components, is totally
tor neurons, send orders regarding the specific motor tasks functional. However, the engine will not perform without a
to the musculoskeletal system. (See Chapters 3, 4, and 8 for mechanism to initiate its strategies or turn on the system.
more specific in-depth discussion.) The actions performed The basal ganglia or frontal lobe motor mechanism plays
by each subsystem within the entire limbic–motor control this role in the brain. The car has a starter motor. Yet the
complex constantly loop back and communicate to all starter motor will not activate the motor system without the
subsystems to allow for adjustments of intensity and dura- driver’s intent and motivation to turn the key and turn on
tion and to determine whether the plan remains the best the engine. The limbic complex serves this function in the
choice of responses to an ever-changing three-dimensional brain. Once the key has been turned, the car is running and
world.138,186,187 ready for guidance. Whether the driver chooses reverse or
The limbic network has one more opportunity to modify drive usually depends on prior learning unless this is a
and control the central pattern generators and control the totally new experience. Once the gear has been selected, the
body and limbs through direct connections to the spinal motor system will program the car to run according to the
neuronetwork.110,190-193 That is, the limbic network can alter driver’s desires. It can run fast or slow, but for the plan to
existing motor plans by modulating those generators up and change, both a purpose and a recognition that change is
down or altering specific nuclear clusters and varying the necessary are required. The car has the ability to adapt and
patterns themselves. Therapists as well as the general public self-regulate to many environmental variables, such as ruts
see this in sports activities when emotions are high, no mat- or slick pavement, to continue running the feed-forward
ter the emotion itself. Individuals who have excellent motor program, just as many motor systems within the CNS, espe-
control over a specific sport may find high-level performance cially the cerebellum, perform that function. The limbic
difficult as the stress of competition increases. Having con- network may emotionally choose to drive fast, whereas
trol over emotional variance as well as motor variance with one’s cognitive judgment may choose otherwise. The inter-
a functional activity is an accurate example of empower- active result will drive the pedal and brake pressure and
ment. Thus, for a therapist to get a true picture of a patient’s ultimately regulate the car. The components discussed play
motor system’s function, the limbic network should be flow- a critical role in the total function of the car, just as all
ing in a neutral or balanced state without strong emotions of the systems within the CNS play a vital role in regulating
any kind. Generally, that balance seems to reflect itself in a behavioral responses to the environment.
state of safety, trust, and compliance. Once the motor con- Brooks69 distinguishes insightful learning, which is pro-
trol has been achieved then the therapist must reintroduce grammed and leads to skills when the performer has gained
various emotional environments during the motor activity to insight into the requirements, from discontinuous move-
be able to state that the patient is independent. ments, which need to be replaced by continuous ones. This
In summary, the limbic complex generates need-directed process is hastened when clients understand and can demon-
motor activity and communicates that intent throughout the strate their understanding of what “they were expected to
motor system.110,191,194,l95 This step is vital to normal motor do.” Improvement of motor skills is possible by using pro-
function and thus client care. Clients need the opportunity grammed movement in goal-directed behavior. The reader
to analyze correctly both their internal environment (their must be cautioned to make sure that the client’s attention is
present and feed-forward motor plans and their emotional on the goal of the task and not on the components of the
state) and the external world around them requiring action movement itself. The motor plan needs programming and
on a task. The integration of all this information should practice without constant cognitive overriding. The limbic/
produce the most appropriate strategy available to the frontal system helps drive the motor toward the identified
patient for the current activity. These instructions must be task or abstract representation of a match between the motor
correct, and the system capable of carrying out the motor planning sequence and the desired outcome. The importance
activity, for effortless, coordinated movement expression to of the goal being self-driven by the patient cannot be over-
be observed. If the motor system is deficient, lack of adapt- emphasized.*
ability will be observed in the client. If the limbic complex Without knowledge of results, feedback, and insight
is faulty, the same motor deficits might present themselves. into the requirements for goal-directed activity, the learn-
The therapist must differentiate what is truly a motor system ing is performing by “rote,” which merely uses repetition
problem versus a limbic influence over the motor system without analysis, and meaningful learning or building of
problem. effective motor memory in the form of motor holograms
Schmidt196 stresses the significance of “knowledge of
results feedback” as being the information from the envi-
ronment that provides the individual with insights into task *
References 24, 28, 31, 111, 199, 200.
will be minimal. Children with cognitive and limbic emotional state (versus a pure reflection of motor control) is
deficits can learn basic motor skills through repetition an aspect of evaluation often overlooked.
of practice, but the insights and ability to transfer that
motor learning into other contexts will not be high (see Limbic Influence on Emotional Output: The F2ARV
Chapters 12, 13, and 14). and GAS Continua
Schmidt196 suggests that to elicit the highest level of Some of the earliest understanding of the limbic network
function within the motor system and to enable insightful was of its role in “fight or flight.” It is important that clini-
learning, therapy programs should be developed around cians not only understand but also recognize two powerful
goal-directed activities, which means a strong emotional limbic motor response programs: the fear and frustration,
context. These activities direct the client to analyze the anger, rage, and violence continuum (F2ARV) and the gen-
environmental requirements (both internal and external) by eral adaptation syndrome (GAS).
placing the client in a situation that forces development of F2ARV (Fear and Frustration, Anger, Rage, and
“appropriate strategies.” Goal-directed activities should be Violence or Withdrawal) Continuum. One sequence of
functional and thus involve motivation, meaningfulness, and behaviors used to describe the emotional circuitry of the
selective attention. Functional and somatosensory retraining limbic network through the amygdala is the F2ARV contin-
uses these concepts as part of the intervention (see Chapters 4 uum157,203,204 (Figure 5-5). This continuum begins with fear
and 9). Specific techniques such as proprioceptive neuro- or frustration. This fear can lead to avoidance behavior.205
muscular facilitation, neurodevelopmental therapy, the Rood If the event inducing the fear or frustration continues to
method, and the Feldenkrais method can be incorporated heighten, avoidance behaviors can continue to develop.205 In
into goal-directed activities in the therapy programs, as can a simple example, we recall or have seen these behaviors
any treatment approach, as long as it identifies those aspects in our teens and as young adults, when the challenges faced
of motor control and learning that lead to retention and fu- in high school can lead to avoidance of activities. Alternately,
ture performance and allows the patient to self-correct.196 extreme fear and frustration can also lead to anger. Anger
With insights into the learned skills, clients will be better is a neurochemical response that is perceived and defined
able to adjust these to meet the specific requirements of dif- cognitively (at the cortical level) as anger. If the neuro-
ferent environments and needs, using knowledge of response chemical response continues to build or is prolonged, the
feedback to guide them. The message then is to design exer- anger displayed by the person may advance to rage (internal
cise activities or programs that are meaningful and need chaos) and finally into violence (strong motor response). A
directed, to motivate clients into insightful goal-directed common societal example is in the case of domestic discord
learning. Thus, understanding the specific goals of the cli- and violence. Women who attain the level of rage may
ent, patient-centered learning, is critical and will be obtained become withdrawn and thus become victimized by a partner
only by interaction with that client as a person with needs, who is also in rage or inflicting physical or emotional vio-
desires, and anticipated outcomes.201,202 A therapist cannot lence.206 Another current example is posttraumatic stress
assume that “someone wants to do something.” The goal of disorder (PTSD), in which the prolonged stress of deploy-
running a bank may seem very different from that of bird- ment and unique challenges of warfare lead to limited adap-
watching in the mountains, yet both may require ambulatory tive reserves in warriors and returning veterans. Suicide and
skills. If a client does not wish to return to work, then a domestic violence have become a more common occurrence
friendly smile and the statement, “Hi, I’m your therapist and between deployments, necessitating a dramatic shift in men-
I’m going to get you up and walking so you can get back to tal health policy in the last 5 years.207-210
work,” may lead to resistance and decreased motivation. In How quickly and completely any individual will progress
contrast, a therapist who knows the goal of the client may from fear to violence is dependent on several factors. First,
help him or her become highly motivated to ambulate; that the genetic neurochemical predisposition (initial wiring)
client may be present in the clinic every day to meet the goal will influence behavioral responses.204 Second, “soft-wired”
of birdwatching in the mountains although never wishing to or conditioned responses resulting from experiences and
walk back into the office again. reinforced patterns will influence output. For example, it
is commonly known that abusive parents were usually
Clinical Perspectives abused children203,211; they learned that anger quickly leads
The Client’s Internal System Influences Observable
Behavior. At least once a year almost any local newspaper
will carry a story that generally reads as follows: “Seventy-
nine-year-old, 109-pound arthritic grandmother picks up car
by bumper to free trapped 3-year-old grandson.” Fear/Frustration Anger Rage Violence
We read these articles and at first doubt their validity, F2 A R V
questioning the sensationalism used by the reporter. But we
know that these events are real. That elderly lady picked up
the car out of fear of severe injury to her grandchild. Emo-
tions can create tremendously high tonal responses, either in
a postural pattern such as in a temper tantrum or during a Withdrawal
movement strategy such as picking up a car. Conversely,
fear can immobilize a person and make it impossible to cre-
ate enough tone to run a motor program or actually move. Figure 5-5 n Fear and frustration, anger, rage, and violence or
Evaluating muscle power or tone production in relation to withdrawal: F2ARV continuum.
to violence and that the behavior of violence was some- the skin may become hot. The patient will have no energy
how acceptable. Last, the quality and intensity of the to move, will withdraw, and again will exhibit decreased
stimulus initiating the continuum will influence the level of postural tone and increased flexion.
response. This stress or overstimulation syndrome is characterized
The neurochemistry within an individual’s CNS, whether by common symptoms as described earlier.140,224-231 If the
inherently active or altered through drugs or injury, will have acute symptoms are not eliminated, they will become
great influence on the plasticity of the existing wiring.40,212 chronic and the behavior patterns much more resistant to
Repetitive or prolonged exposure to negative environmental change.
stimuli may also lead to a chronically imbalanced neuro- GAS is often seen in the elderly, with various precipitat-
chemical state that results in a lowered threshold or tolerance ing health crises,221 and also in neonatal high-risk infants
to a given stimulus. Chemistry or wiring can become imbal- (see Chapter 11), victims of head trauma, and other clients
anced from damage, environmental stress, learning, or other with neurological conditions. The initial alarm can be pre-
potentially altering situations, changing an individual’s con- cipitated by moderate to maximal internal instability with
trol over this continuum.56,106-108,213,214 When neurochemical less intensive external stress, or by minimal internal instabil-
imbalance exists, these behaviors will persist, and balance ity with severe external sensory bombardment. For instance,
may be restored only through natural neurochemical activity in the elderly, stresses such as change of environment, loss
(e.g., sleep, exercise, diet, spirituality) or medication support of loved ones, failing health, and fears of financial problems
(chemical replacement). can each cause the client’s system to react as if over-
Therapists need to be acutely aware of this continuum in loaded.223 As another example, individuals with head trauma
clients who have diffuse axonal shearing within the limbic (Chapter 24), vestibular dysfunction (Chapter 22B), inflam-
complex. Diffuse axonal shearing is most commonly seen matory CNS problems (Chapter 26), and brain tumors
and reported in research on individuals with head trauma215,216 (Chapter 25) often possess hypersensitivity to external input
(see Chapter 24). Resulting lesions within the limbic struc- such as visual environments, noise, touch, or light. In these
tures may cause an individual to progress down this contin- individuals, typical clinical environments and therapeutic
uum at a rapid speed. This point cannot be overemphasized. activities may create a sensory overload and trigger a GAS
Patients with an accelerated F2ARV continuum may physi- response.
cally strike out at a clinician or caregiver out of simple Stress, no matter what the specific precipitating incident
frustration during care. Knowing the social history of the (confusion, fear, anxiety, grief, or pain), has the potential
client and the causation of the injury often can help the to trigger the first steps in the sequence of this syn-
therapist gain insight into how an individual patient might drome.224-229,232 The clinician’s sensitivity to the client’s
progress down this continuum. Not all head-injured patients emotional system will be the therapeutic technique that best
had prior difficulty with the F2ARV continuum; however, it controls and reverses the acute condition.
is important to note that many individuals received their Similarly, patients with dizziness and instability, particu-
head injuries in violent confrontational situations or in war- larly within visually stimulating environments, can develop
time conflict. Some individuals, primarily females, when feelings of panic, which can evolve into full attacks and
confronted with stress, anger, and potential violence from agoraphobic responses.233 These individuals avoid partici-
another, will withdraw and become depressed. This behav- pating in activities that put them within visually overstimu-
ior, similar to violence, will change the structure of the lating environments in an effort to control the dizziness and
limbic network.55 prevent the associated autonomic reactions. Similar types
GAS (General Adaptation Syndrome). The auto- of reactions have been documented, such as space-motion
nomic responses to stress also follow a specific sequence of discomfort (SMD),234 postural phobic vertigo,233 visual ver-
behavioral changes and are referred to as the general adap- tigo,235 and dizziness of “psychogenic” origin. Often these
tation syndrome.217-223 The sequential stages of GAS are a individuals are referred first to psychology or psychiatry.
direct result of limbic imbalance and can play a dramatic However, there is an underlying physiologic explanation
role in determining client progress. for these symptoms. In a majority of individuals with
Stress can be caused by many internal or external factors, SMD, there is a documented increase in vestibular sensitiv-
often unique to the individual. Examples include pain, acute ity (increased vestibulo-ocular reflex [VOR] gain) and an
or chronic illness or the ramification of illness, confusion, impairment in velocity storage (shorter VOR time constant).236
sensory overload, and a large variety of other potential In addition, the dorsal raphe nucleus (DRN of the midbrain
sources. The initial reaction to a stressor is a neurochemical and rostral pons) is the largest serotonin-containing nucleus
change or “alarm” that triggers a strong sympathetic ner- in the brain and directly modulates the firing activity of the
vous system reaction. Heart rate, blood pressure, respiration, superior and medial vestibular nuclei. It is this interaction
metabolism, and muscle tonus will increase. It is at this between serotonin and vestibular function that helps to ex-
stage that the grandmother lifts the car off the child as in our plain the link between vestibular and anxiety disorders. It
previous example. If the overstimulation or stress does not can also help explain how patients with sleep disorders or
diminish, the body will protect itself from self-destruction other serotonin-depleting disorders develop vestibular-like
and trigger a subsequent parasympathetic response. At this symptoms and anxiety.237,238
time, all the symptoms reverse and the client exhibits a Although there are physiological reasons underlying the
decrease in heart rate, blood pressure, and muscle tonus. The vestibular system disorder in a majority of these cases,
bronchi become constricted, and the patient may hyperven- the symptoms triggered are part of a spectrum of limbic
tilate and become dizzy, confused, and less alert. As the responses to aberrant vestibular, cerebellar, and brain stem
blood flow returns to the periphery, the face may flush and interactions. Normal clinic activity or typically appropriate
therapeutic activity may trigger an autonomic cascade ver- Decreasing stimulation versus increasing facilitation may
sus the desired somatomotor response. The rehabilitation of lead to attention, calmness, and receptiveness to therapy.
the resultant visual and postural movement dysfunction is When the client feels that control over her or his life has
typically more complicated in the absence of limbic network been returned, or at least the individual is consulted regard-
management. The clinician’s strategic prescription (or “dos- ing decisions (informed consent, forced choices), resistance
ing”) of therapeutic activities with careful monitoring of the to therapy or movement is often released and stress is
client’s emotional system and physiological response will reduced. Even clients in a semicomatose state can partici-
be one of the therapeutic techniques that best controls the pate to some extent. As a clinician begins to move a mini-
aberrant responses and allows vestibular adaptation and mally responsive client, resistance may be encountered. If
compensation to occur. This must be done to manage limbic slight changes are made in rotation or trajectory of the
network activity for successful motor learning to occur. movement pattern, the resistance is often lessened. If the
The F2ARV and GAS continua are often interrelated clinician initially feels the resistance and overpowers it, total
in individuals who have direct or indirect limbic network control has been taken from the client. Instead, if the clini-
involvement. The therapist needs to be aware that a patient cian moves the patient in ways her or his body is willing
may overrespond to stress, frustration, or fear of failure in to be moved, respect has been shown and overstimulation
both cognitive and motor activities. The initial response may potentially avoided.
be an escalation of the F2ARV continuum with what then No single input causes these limbic responses, nor does
seems like a rapid withdrawal or a heightened state of anger one treatment counteract their progression. Being aware of
(GAS). There are many ways to help the patient balance clinical signs is critical. In a time when therapists are often
these autonomic reactions and continue to learn within the rushed by the realities of a full schedule or stressed by third-
therapeutic setting. The Bonny Method of Guided Imagery party or short discharge demands, a clinician may inadver-
and Music is a music-centered psychotherapy method that tently miss key signs and opportunities to treat. In addition,
has been used extensively with individuals recovering from he or she may actually create a less optimal environment by
various types of trauma.239-242 In reviewing specific Bonny physically moving faster than the pace best tolerated by the
Method treatment approaches used with trauma patients, patient, who may need more time to process stimuli or to
Körlin242 describes a cyclical process whereby an important practice a target skill. The challenge for both the therapist
initial treatment period emphasizes the mobilization of inner and the patient is to find harmony within the given environ-
resources, alleviating vulnerability and increasing the ment to allow for optimal outcomes.
patient’s self-confidence. This phase uses carefully selected Developing limbic network assessment tools (or repur-
music that elicits positive limbic states and “bodily manifes- posing existing tools) for their ability to screen or identify
tations with qualities of warmth, energy, strength, move- the presence of direct or indirect limbic involvement is of
ment, nourishing, and healing, all belonging to the implicit critical value. In addition, the ability to discriminate the type
realm of positive vitality affects and mental models” (p. 398). of limbic involvement (decreased responsiveness and with-
The individual is then better equipped to face a period drawal from increased responsiveness or overresponsive-
of confrontation with painful or traumatic material or the ness) is important to treatment planning. Treatment techniques
challenges faced within a therapeutic rehabilitation environ- will be discussed later in the chapter. However, the specific
ment. Successful confrontation of difficult realities is then techniques appropriate for treating these syndromes are
followed by a new phase of resource mobilization and con- tools all therapists possess. These tools range from simple
solidation of healthier behaviors that begin to replace dys- variations in approach (e.g., lighting, sound, smell) to more
functional defenses such as avoidance, behavioral extremes, formal therapeutic techniques, such as the Feldenkrais
or substance abuse. approach, or The Bonny Method of Guided Imagery and
This process continues in repeated cycles of rest-resourcing Music.239 How each clinician uses those tools is a critical
and working-confronting. The clinical success of this link to success or failure in clinical interaction.
approach suggests that for some patients it may be advanta- Specific Limbic influences on Motor System Output.
geous to purposefully facilitate positive or pleasant physical Throughout the existence of humankind, emotions have been
and affective experiences before engaging in more challeng- identified in all cultures. A child knows when a parent is
ing work. Although it may be impractical to provide appro- angry without a word being spoken. A stranger can recognize
priate music selections on the basis of individual assessment a person who is sad or depressed. People walk to the other
in the therapeutic environment (e.g., physical, occupational, side of the road to avoid being close to someone who seems
music), other modalities such as heat, massage, or ultra- enraged. Emotions are easily recognizable as they are ex-
sound treatment may also elicit relaxed, receptive physical pressed through motor output of the face and body. Emotions
states. The treating professional can also become aware dur- similarly have an impact on functional motor control. The
ing assessments or treatments of environmental auditory effect and intensity of emotions and limbic influence on
input that may trigger stress responses in patients who have motor control are an important part of the therapy evaluation.
limbic network involvement or who may have experienced Table 5-1 helps differentiate the level of limbic activity with
traumatic physical or emotional injuries. These triggers can observed behavioral states cross-referenced with various
be something as simple as the therapist’s tone of the voice in medical conditions.
a sentence to the patient or as complex as the multiple-noise Fear. Fear is often associated with pain, be it somatic
environment of a busy rehabilitation setting. Some patients or emotional. To the individual in pain, pain is just pain.
may need to be scheduled for early morning, during lunch- Figure 5-6, A illustrates two people who are on a roller-
time, or in the late afternoon to provide a decrease in the coaster which could create automatic responses of fear. The
auditory environment. boy looks scared and obviously exhibits fear. The woman
TABLE 5-1 n INTERACTIONS AMONG LIMBIC STATE, MEDICAL CONDITIONS, AND BEHAVIORS*
LIMBIC STATE OFTEN ASSOCIATED WITH OBSERVED BEHAVIORS

Neutral Health and wellness Relaxed state
Low Depression, stroke Decreased eye contact, crying
Lack of motivation
High Anxiety and panic disorders Anxiety, anger, fear, increased respiratory rate,
Vestibular disorders higher blood pressure, high muscle tension
Mild or traumatic head injury or tone, hyperactivity
Blast injury, PTSD
Overload (F2ARV, GAS) Traumatic head injury Violence, extreme withdrawal, loss of inhibition,
Blast injury, PTSD, frail individual (either young reversal of expected behavior
or old), physiologically unstable individual
GAS, General adaptation syndrome; F2ARV, fear and frustration, anger, rage, violence; PTSD, posttraumatic stress disorder.
*Many individuals with neurological dysfunction fall into these categories.
A B
Figure 5-6 n A, Two individuals riding a rollercoaster. Individual on right looks scared; this facial
expression represents fear. The facial expression of the individual on the left could represent enjoy-
ment, with eyes open and a smile, or extreme fear, with hyperextension of her head causing her mouth
to open. B, Individual’s facial expression after the rollercoaster stopped. She was unable to relax her
facial muscles for over 2 minutes because she had been so scared or demonstrated extreme fear.
could be expressing joy or fear given her motor responses. fear of falling need to first acknowledge that the fear is
Her eyes seem fixed, which might lead to the assumption normal and then make sure that when the individual moves,
that she is truly in fear. She may not have control over her he or she does not fall. Trust will be discussed later in the
facial responses and could be exhibiting an extreme reaction chapter, but fear often precedes the development of trust.
to fear. If that were the case, this would be a limbic motor Fear is an emotional response and thus is initiated and con-
reaction, which could be semiautomatic. This extension pat- trolled by the limbic network.
tern, if limbic, could trigger hyperextension of the neck, Fear of pain is another emotional response housed within
causing opening of the mouth. In Figure 5-6, B, the roller- the limbic network that drives many individuals’ motor
coaster has stopped, and she still has the same expression. In responses. Whether individuals have fear of movement after
fact, it took over a minute before she was able to relax her a musculoskeletal injury,243 fear of going to the dentist after
face and regain the feeling that she had some control over a dental procedure,246 fear of pain intensity after a chronic
her emotional reaction. Her next reaction to occur was cry- pain problem,247 or fear of falling,248 fear will drive motor
ing and observable frustration in her inability to control her responses, and that fear will often lead to a lower quality
initial response. The amygdala nuclei plays a critical role in of life.244 For that reason alone, therapists need to differenti-
regulation of facial responses to fear, pain, and other incom- ate the limbic system’s and the motor system’s summated
ing stimuli.243 This is often observed in healthy normal indi- responses when observing the movement patterns of the
viduals such as seen in Figure 5-6 and similarly can be seen individual in therapy.
in patients who are extremely fearful, no matter the cause. Anger. Anger itself creates muscle tone through the
Fear of falling is a common problem with the elderly, amygdala’s influence over the basal ganglia and the sensory
especially the elderly who have various neurological diag- and motor cortices and their influence over the motor con-
noses.244,245 Therapists working with individuals who have a trol system. This is clearly exhibited in a child throwing a
temper tantrum (Figure 5-7) or an adult putting his fist unwilling, unaware, or unable to attend to these variables,
through a wall. How far a client or a friend will progress the reliability or accuracy of functional test results becomes
through the F2ARV continuum (discussed in the previous questionable.
section) depends on a large number of variables. When a For example, West was asked to consult with a rehabilita-
client loses control, the therapist must first determine tion team to devise a treatment program to address violent
whether the intervention forced the client beyond her or his rage episodes in a 40-year-old man with moderate physical
ability to control. If so, changes within the therapeutic envi- and severe cognitive functioning deficits resulting from a
ronment need to be made to allow the client opportunities to brain aneurysm. He would escalate very rapidly along the
develop control and modulation over that continuum. F2ARV continuum when presented with environmental chal-
Creating opportunities to confront frustration and fear or lenges such as passing another patient with his wheelchair
even anger in real situations while the client practices modu- in the hallway. Although his physical rehabilitation had
lation will lead to independence or self-empowerment. The progressed well and he had regained much independence in
client simultaneously needs to practice self-directed motor mobility, because his assaultive outbursts posed risks to
programming without these emotional overlays. Thus, true other patients as well as to caregivers, this patient appeared
motor learning can result. In time, practicing the same motor to be heading for placement in a locked facility, a more
control over functional programs when confronted with a restrictive environment than he would need considering his
large variety of emotional situations should lead to indepen- level of physical limitation. Although this unfortunate man
dence in life activities and thus meet a therapeutic goal. had no short-term memory function and no insight about his
Similarly, being unaware of a client’s anger may lead the behaviors, West found during her assessment that he was
therapist to the false assumption that that individual has ad- highly responsive to calming music and was able to access
equate inherent postural tone to perform activities such as some intact long-term memories that could be used to elicit
independent transfers. If the client is angry with the therapist a relaxation response. A highly positive limbic state of deep
and performs the transfer only to get the therapist “off relaxation was thus elicited, and simple verbal cues were
my case,” when the client is sent home she or he may be then presented to develop a conditioned response that any
unable to create enough postural extension to perform the staff member could then call forth with the verbal cue alone.
transfer. Thus this transfer skill was never functionally inde- The entire rehabilitation team was briefed on the use of this
pendent because the test measurements were based on intervention and reported success using this approach in the
limbic or frontal influence over the extensor component of milieu as well as during physical therapy and occupational
the motor system. The client needs to learn how to do the therapy treatments when the patient would become resistant
activity without the emotional overlay. When a therapist is and angry in response to therapist instructions. The patient
was trained to self-regulate by giving himself the same ver-
bal cue when confronted with challenging situations. This
treatment supported the patient’s ability to regain emotional
controls and allowed him the opportunity to be placed in a
less-restrictive community environment. West observed this
individual maintaining his progress in positive behavioral
adaptation in a group home environment more than a year
after the intensive inpatient rehabilitation treatment protocol
had been completed. The patient never recalled a previous
music therapy treatment session and asked each time to have
the purpose of the treatment explained to him. But his body
remembered the set of behavioral experiences, and he
quickly complied with the relaxation procedure. The success
of this approach demonstrates that even in the absence of
short-term memory and other cognitive functions usually
considered essential for new learning, the skillful engagement
of positive limbic states and intact areas of patient function-
ing (strengths) can support development of new adaptive
skills, which can be generalized to new environments.
Grief, Depression, or Pain. Emotions such as grief or
depression can be expressed by the motor system.56,249 The
behavioral responses are usually withdrawal, decreased
postural extension, and often a feeling of tiredness and
exhaustion (Figure 5-8). Sensory overload, especially in the
elderly, can create low muscle tone and excessive flexion.
Again, because of the strong emotional factor, these motor
responses are considered to be the result of the limbic net-
work’s influence over motor control.110 Learned helpless-
ness is another problem that therapists need to avoid.250
Figure 5-7 n Extensor behavior responses caused by anger. When patients are encouraged to become dependent, their
(“Angry Boy,” Vigelund Sculpture Grounds in the Frogner Park, chances of benefiting from services and regaining motor
Oslo, Norway. Adapted from photo by Normann.) function are drastically reduced.251,252
Figure 5-8 n A, Behavior responses elicited by concern, pain, and grief. B, Pain or grief elicits
flexion and can modify postural extension. (A from Vigelund Sculpture Grounds in the Frogner Park,
Oslo, Norway. Adapted from photo by Normann.)
Pain is a complex phenomenon, and the more it is under- In Figure 5-9 an entire spectrum of motor responses can
stood, the more complex it becomes253-257 (see Chapter 32). be observed in four statues. A client who feels safe can relax
The concept of pain and pain management is discussed and participate in learning without strong emotional reac-
in detail in both Chapters 18 and 32. Hippocampal volume tions. The woman being held in Figure 5-9, A is safe and
has been identified as a variable in pain ratings in the relaxed. The man and woman are interacting through touch
elderly.258 Whether the pain is peripherally induced or cen- with the warmth and compassion that are often observed in
trally induced because of trauma or emotional overload, the client-therapist interaction of an experienced or master
often the same motor responses will exist. A withdrawn clinician. In Figure 5-9, B, the client and clinician seem
flexor pattern from pain makes postural activities exhausting to flow together during the treatment as if they shared one
because of the work it takes to override the existing central motor system. When looking at the therapist and client or
pattern generators. Thus, daily living activities, which con- looking at the man and woman in the statue, it becomes
stantly require postural extension against gravity, may be obvious that the two figures seem to flow together. In the
perceived as overwhelming and just not worth the effort. statue, those two figures make one piece of art.
The therapist needs to learn to differentiate between periph- With clinical emphasis on clients generating and self-
eral physical pain and central or emotional pain and between correcting motor programming, it would perhaps seem rea-
mixed peripheral and central induced pain. To the patient, sonable for a therapist to conclude that he or she need not,
“pain is pain!”259-266 or should not, touch the patient. This conclusion may be
Client-Therapist Bonding. Bonding projects relaxation, accurate when considering the motor system in isolation and
whereas lack of bonding reflects isolation. Because of the assuming that patients can self-correct errors in motor pro-
potency of the limbic network’s connections into the motor grams. When correction by the therapist is through words
system, a therapist’s sensitivity to the client’s emotional rather than touch, external feedback through the auditory
state would obviously be a key factor in understanding the system has replaced internal feedback from the somatosen-
motor responses observed during therapy. This requires that sory system. The voice, as well as touch, can be soothing
a therapist first understand her or his own feelings, emo- and instill confidence.275 Yet language in and of itself will
tional responses, and communication styles that are being not replace the trust and safety felt both physically and emo-
used within any given clinical or social environment.267-274 tionally through the deep pressure of touch as illustrated in
A B
Figure 5-9 n A, Grief, depression, and compassion responses are seen in the center figures, and
rigid, stoic, distancing behaviors are observed in the two left statues. B, Compassion is easily recog-
nized in the clinic between a therapist and the client. (A from Vigelund Sculpture Grounds in the
Frogner Park, Oslo, Norway. Adapted from photo by Normann.)
Figure 5-9. Bonding and trust occur much more often may play a role in that gifted clinician’s skill. Although
through touch than through conversation.276 Recall, also, therapists are trained to be skilled observers of patient
that verbal instructions require intact auditory processing behavior, the development of “master clinician” capabilities
and translation from declarative to procedural information, a also requires self-awareness on the part of the helping pro-
cognitive ability that the client may not possess. fessional. “Behavioral activity can often tell us about the
Referring again to Figure 5-9, A, the two men in the inner state of another or ourselves” (p. 19).280 The willing-
statue on the left demonstrate a lack of bonding. In fact, if ness to be aware of one’s own internal state increases
the artist could have brought them closer together, they the therapist’s ability to perceive subtleties in the patient’s
might just have rejected or repelled each other with greater responses.
intensity. If one of the men were the therapist and one the Achieving a limbic neutral state in the client by carefully
patient, little interaction would be occurring, and thus an modifying the therapeutic environment will facilitate effec-
assumption that learning is occurring is probably false. The tive motor learning. There are many core tenets and tech-
therapist could do nothing to the other person (and vice niques necessary to effectively achieve this neutral state,
versa) without that person perceiving the act as invasive, internal and external therapeutic environment, and optimal
negative, or even disrespectful, with little consideration learning in the client.
of the person’s individual values. The therapist’s responsi- Trust. Trust is a critical component of a successful ther-
bility is to open the patient’s receptiveness to learning, not apy session.281 The therapist gains the client’s trust by his or
to close it.277,278 her actions. The therapist may also build trust through sin-
These pictures clearly illustrate two types of therapist- cere acknowledgment that the patient has life-limiting func-
client interactions. If an artist can clearly depict the tonal tional problems and that those problems are limiting normal
characteristics of emotion, certainly the therapist should be participation. Trust is further developed when the therapist’s
able to recognize those behaviors in the client.279 If a client is words can be supported by data. When the therapist can
frustrated or angry and simultaneously has rigidity, spasticity, illustrate the presence of functional limitations and generate
or general high tone, then a therapist might spend the entire a treatment plan with the patient using objective data, a bond
session trying to decrease the motor response. If the client and trust between the patient and the therapist are created.282
could be helped to deal with the anger or frustration during In today’s environment the use of reliable, valid, objective
the therapy session and neutralize the emotion and achieve a tests and measures allows for this form of communication,
limbic neutral state, then the specific problems could be which has not existed to the same extent in the past. Honesty
treated effectively. Differentiating the limbic network compo- and truth lead to trust.119,283-287
nent from the motor control system when establishing treat- A trusting relationship is strengthened when an agree-
ment protocols has not typically been within the spectrum of ment or “contract” can be established that sets the boundar-
a therapist’s skills. It is a skill that must be developed and ies for discomfort (fatigue, dizziness, nausea, imbalance)
practiced, as it is clear that the influence of an overactive or or pain that the patient will experience within a therapeutic
overloaded (limbic high) or underactive (limbic low) limbic session. As one example, telling a person that you will not
network state may drastically alter the consistent responses of hurt them is a therapist-patient contract. If the therapist con-
the motor systems and thus dampen the procedural learning tinually ranges a joint beyond a pain-free range, that behav-
and limit the success of the therapeutic setting. Carryover ior is dishonest and untruthful and will not lead to trust.
of procedural learning (Chapter 4) into adaptive motor Trust can be earned by stopping as soon as the client verbal-
responses needs to be practiced with consistency.56 izes symptoms or shows pain with a body response such as
Many factors in an interactive setting, such as therapy, a grimace. Being sensitive to a patient’s pain, no matter the
cannot be identified, but certain limbic or emotional factors cause, and working with the patient to eliminate that pain
often lead to very strong bonding and trust that will lead to of potential danger. Trust means acceptance that although
compliance and learning. Ignoring the pain may be per- the danger is present, the potential for harm, pain, or disaster
ceived as insensitivity and lack of caring, which can lead to is very slight and the expected gain is worth the risk (in this
distrust and often resistance to learning or performance. case, delay in intervention). In Figure 5-10, the student’s
As another example, a patient with vestibular dysfunc- trust that the instructor will not hurt her can be seen by her
tion associated with significant dizziness and nausea will lack of protective responses and by her calm, relaxed body
experience symptoms within the course of recovery (adap- posture. The student is aware of the potential of the kick but
tation and compensation), but those symptoms must be trusts her life to the skills, control, and personal integrity
carefully controlled in intensity and duration. Symptoms of the teacher. Those same qualities are easily observed
poorly controlled can trigger an ANS or GAS cascade and in patient-therapist interactions when watching a gifted cli-
elevate the limbic state, preventing learning and recovery nician treat clients. The motor activities in a therapeutic
and destroying trust. setting may be less complex than in Figure 5-10, but in no
Because these symptoms can be overt or covert, the way are they less stressful, less potentially harmful, or less
therapist needs to be aware of both the physical and emo- frightening from the client’s point of view.
tional responses of the patient. The use of analog or per- In addition, therapists must first trust themselves enough
ceived exertion scales can be a valuable way to make the to know that they can effect changes in their clients.7,288
covert more overt to the therapist. Symptoms are valuable Understanding one’s own motor system, how it responds,
to the therapist as well as the patient to create environments and how to use one’s hands, arms, or entire body to move
for change, but the intensity of those stimuli need close someone else is based partly on procedural skills, partly on
monitoring because they can dramatically affect motor declarative learning, and partly on self-confidence or self-
responses and ultimately overwhelm the CNS and prevent trust. Trusting that one, as a therapist, has the skill to influ-
learning. Compliance to participate is limbic, and the limbic ence the motor response within the patient has a limbic
system has tremendous control over intentional movement, component. If a therapist has self-doubts about therapeutic
no matter the context of the environment.119 skills, that doubt will change performance, which will alter
Once a client gives his or her trust, a clinician can freely input to the client. This altered input can potentially alter the
move with the client and little resistance caused by fear, client’s output and vary the desired responses if the client’s
reservations, or need to protect the self will be felt or motor system cannot run independently.
observed. When the patient is limbic neutral (the limbic Responsibility. Very close to the concept of trust is the
network is emotionally neutralized), the tightness or limita- idea of responsibility. Accepting responsibility for our own
tions in movement that are present on examination can be behavior seems obvious and is accepted as part of a profes-
considered true impairments within those systems or sub- sional role.289 Accepting and allowing the client the right to
systems. Examination and interventions at this time will accept responsibility for her or his own motor environment
more consistently reflect true motor performance. Once lim- are also key elements in creating a successful clinical envi-
bic neutral has been achieved and examination is complete, ronment and an independent person.*
it is recommended, for example, that if the pain is a result of Figure 5-11 illustrates the concept through the following
peripheral tightness or joint immobility, the therapist does example: The instructor asked the student to perform a
not elicit pain during that session. Deal with those issues in
the next session after gaining the trust of the client. Trust by
the therapist or the client does not mean lack of awareness *
References 24, 28, 31, 150, 290, 291.
Figure 5-10 n Trust relaxes the limbic network’s need to protect. A, The skill of the teacher is
obvious. B, The student trusts that she is in no danger.
motor act, in this case, to perform a kick to the teacher’s accomplishment of a goal.90,292,293 The limbic complex and
head. The kick was to be very strong or forceful and com- its interwoven network throughout the nervous system play
pleted. The student was instructed not to hold back or stop a key role in this behavioral drive.294 The task itself can be
the kick in any way, even though the kick was to come simple, such as a weight shift, or as complex as getting
within a few inches of the teacher’s head. This placed tre- dressed or climbing onto and off of a bus. No matter what
mendous responsibility on the student. One inch too far the activity, the client needs to accept responsibility for
might dangerously hurt the instructor, yet one inch too short her or his own behavior before independence in motor func-
was not acceptable. The teacher knew the student had the tioning can be achieved. Although the motor function itself
skill, power, and control to perform the task and then passed is not limbic, many variables that lead to success, self-
the responsibility to the student. The student was hesitant to motivation, and feelings of independence are directly related
assume the responsibility, for the consequence of failure to limbic and prefrontal lobe circuitry. The variance and
could have been very traumatic. However, the student self-correction within the movement expression also create
trusted that the teacher would not ask for the behavior unless novelty and motivation to continue to practice.90,292-295
success was fairly guaranteed. That trust reduced anxiety As another clinical example of responsibility, in a patient
and thus neutralized the neurochemical limbic effect on the with vestibular dysfunction and dizziness compounded by
motor system of the student, giving her optimal motor con- anxiety, symptoms of dizziness are necessary during treat-
trol over the act.292 Once the task was completed success- ment to drive CNS change. The therapist has a responsibility
fully, the student gained confidence and could repeat the to prescribe the appropriate activities, dosage (intensity, tim-
task with less fear or emotional influence while gaining ing, and so on), and environment to retrain sensory organi-
refinement over the motor skill. zation and balance (motor output). The patient can be given
Although the motor activities described in this example the responsibility of monitoring and managing her or his
are complex and different from functional activities practiced own symptoms within these activities, for instance, by
within the clinic by therapists and clients, the dynamics of agreeing on the maximal level of dizziness the patient and
the environment relate consistently with client-therapist roles therapist are willing to accept within the therapeutic activity.
and expectations. A gifted clinician knows that the client has A tool as simple as a verbal or visual analog scale can em-
the potential to succeed. When asked to perform, the client power the patient to manage symptoms, dampen the limbic
trusts the therapist and assumes responsibility for the act. The network response, and improve motor output for balance
therapist can facilitate the movement or postural pattern, control.
thereby ensuring that the client succeeds. This feeling of suc- Flexibility and Openness. Another component of a suc-
cess stimulates motivation for task repetition, which ulti- cessful clinical environment deals with learning and flexibil-
mately leads to learning. The incentive to repeat and learn ity on the part of the therapist. A master clinician sees and
becomes self-motivating and then becomes the responsibility feels what is happening within the motor control output
of the client. As the therapist relinquishes control and em- system of the client. Letting go of preestablished belief
powers the client to more and more of the function, novelty of what will happen is difficult.296,297 It is important for a
to the learning is occurring. clinician to be open to what is present as the motor expres-
Current literature has shown that people are more moti- sion. This openness is critical to actually identifying what
vated by novelty and change than by success at mastery or is being expressed by the nervous system of the patient.
Master clinicians do not get stuck on what they have been
taught but use that as a foundation or springboard for addi-
tional learning. Learning is constantly correlated to memo-
ries and new experiences.
To the therapist, each client is like a new map, sparsely
drawn or sketchy at the beginning, but one that is constantly
revised as the terrain (client) changes. The initial medical
diagnosis may link to many paths provided within the map,
but the comorbidities can result in great variance among
patients.298 That initial map might be a critical care pathway
for the client, given her or his neurological insult. That path-
way is a map, but only a sketchy one, and may not even be
a map that a particular patient falls within in spite of his or
her medical diagnosis. It is the therapist’s responsibility to
evaluate the patient and determine whether that pathway or
map will work or is working and when changes in that map
need to be altered. That is, the therapist must let go of an
outdated map or treatment technique and create a new one
as the environment and motor control system of the client
change. This transference or letting go of old maps or ideas
is true for both the client and therapist. If a position, pattern,
or technique is not working, then the clinician needs to
change the map or directions of treatment and let the client
Figure 5-11 n The teacher relinquishes the task to the student, teach the therapist what will work. The ability to change and
and the student trusts the teacher is right even if self-doubt exists. select new or alternative treatment techniques is based on
the attitude of the therapist toward selecting alternative figure (Figure 5-12) is a second map. That second map
approaches. Willingness to be flexible and open to learning might represent another professional’s interaction and goal
is based on confidence in oneself, a truly emotional strategy with the same client. It is during these overlapping interac-
or limbic behavior. Master clinicians have learned that the tions that both professionals can empower the patient to
answers to the patient’s puzzle are within the patient, not the practice, and that practice will help lead to those functional
textbooks. goals established by both practitioners. In some situations a
Figure 5-12 depicts two maps with a beginning point and clinician from one profession may guide a client toward
a terminal outcome or goal in each. The parameters of the obtaining the functional skill necessary for a member of the
first map illustrate the boundaries of that therapist’s experi- second profession to begin guiding the client toward the
ence and education. The clinician, through training, can expected outcomes of the second profession. These inter-
identify what would seem to be the most direct and efficient locking dependencies of the client and the professions are
way or path toward the mutually identified goal of the illustrated in Figure 5-12. If the client begins therapy striv-
therapist and client. When the client becomes a participant ing for the first goal and ends at the functional outcome of
within the environment or map, what would seem like a the second goal, then additional functional outcomes have
direct path toward a goal might not be the easiest or most been achieved and both professions interacted for the ulti-
direct path for the client. If empowerment of the client leads mate prognosis for the patient. That interaction requires
to independence, then allowing and encouraging the client respect and openness of both professionals toward each
to direct therapy may provide greater variability, force the other as well as toward the client. Those attitudes and ulti-
client to problem solve, and lead to greater learning. The mate behaviors are limbic driven.
therapist needs to recognize when the client is not going in Matching maps should be a collaborative effort instead
the direction of the goal. For example, the client is trying to of coincidence. These collaborative efforts include interac-
perform a stand-pivot transfer and instead is falling. If it is tions with all professions within the rehabilitation setting.
important to practice transfers, then practicing falling is Occupational and physical therapists are very familiar with
inappropriate and the environment (either internal or exter- collaboration, and both often approach interventions as a
nal) needs modification. Falling can be learned and prac- team effort. There are many additional therapists and indi-
ticed at another time. Once both strategies are learned, the viduals within that same setting who could also collaborate.
therapist must empower the patient to take ownership of the Recreational therapists, psychologists, nurses, family mem-
map. In the examples of transferring, if the therapist asks bers, and music therapists are but a few. Within a profession
the client to practice transfers and if the client starts to fall, such as music therapy, the existence of two maps may over-
a change in required motor behavior must be made and the lap within a multidimensional environment. When a physi-
opportunity given to the client to self-correct. In that way cal or occupational therapist needs to challenge a patient, the
the client is gaining independent control over a variety of music therapist may be able to calm the system at the same
environmental contexts and outcomes. Within the same time (overlapping maps). Research on affective responses
to consonance and dissonance in music supports the cre-
ation of a map within a rehabilitation environment that
could overlap with either physical, occupational, or speech
Outside Clinical Expertise of Two Disciplines therapy. Words such as relaxed or calm correlated positively
MAPPING
with higher levels of consonance in the music, whereas
adjectives associated with negative emotions (unpleasant,
tense, irritable, annoying, dissonant, angry) were found
to correlate positively with higher levels of dissonance.133
Creating a whole environment where potential frustrations
within motor learning could be balanced with higher levels
of consonance in the music would potentially balance the
limbic network emotional response within the overlapping
maps and bring balance or stability to the limbic network’s
influence on motor learning and control. A later study by
Peretz and colleagues148 related the same variables to a
happy-sad rating task. Given the research evidence for activ-
ity within the limbic network as it relates to music,144,299
motor learning,300 and cognitive enhancement,301 a natural
multiple map system would be easy to incorporate within a
therapeutic setting. The clinician needs to appreciate the
uniqueness of each map while holding onto the concept of
the interaction of the two maps.
Vulnerability. To receive input from a client that is mul-
tivariable and simultaneous, a therapist has to be open to that
information. If a clinician believes that he or she knows
what each client needs and how to get those behaviors
Figure 5-12 n Concept of clinical mapping including client and before meeting the client, then the client falls into a category
therapist and the interactions and importance of overlapping proof a recipe for treating the problem. Using the recipe does
fessional goals and staying within the professional expertise. not mean the client cannot learn or gain better perceptual
and cognitive, affective, or motor control, but it does mean drugs and alcohol can have dramatic effects on the CNS and
that the individuality of the person may be lost. A more often are associated with limbic behavior.307 Korsakoff syn-
individualized approach would allow the clinician to iden- drome, caused by chronic alcoholism and its related nutritional
tify through behavioral responses the best way for the client deficiency, is identified by the structural involvement of the
to learn how to sequence the learning, when to make diencephalon with specific focus on the mammillary bodies,
demands of the client, when to nurture, when to stop, when and the dorsal medial and anterior nucleus of the thalamus56
to continue, when to assist, when to have fun, when to laugh, usually shows involvement (see the anatomy section and
or when to cry. An analogy might be going to a fast food Figure 5-13). This syndrome is not a dementia but rather
restaurant versus a restaurant where each aspect of the meal a discrete, localized pathological state with specific clinical
is tailored to one’s taste. It does not mean that both restau- signs. The most dramatic sign observed in a client with
rants are not selling digestible foods. It does mean that at Korsakoff syndrome is severe memory deficits.252,308-310 These
one eating place the food is mass produced with some deficits involve declarative memory and learning losses, but
choices, but individuality, with respect to the consumer, is the most predominant problem is short-term memory loss.311
not an aspect of the service. Unfortunately, managed care, As the disease progresses, clients generally become totally
limited visits, reduced time for treatment, and therapists’ unaware of their memory loss and are unconcerned. Initially,
level of frustration all are pushing therapeutic interventions confabulation may be observed,312 but in time most clients
toward a “one size fits all” philosophy that may increase the with a chronic condition become apathetic and somewhat
time needed for learning, not reduce it. withdrawn and are in a profound amnesic state. They are
To be open totally to processing the individual differ- trapped in time, unable to learn from new experiences because
ences of the client, the clinician must be relaxed and non- they cannot retain memories for more than a few minutes and
threatened, and feel no need to protect himself or herself are unable to maintain their independence252,308-310,313; many
from the external environment. This environment needs to may become social isolates and homeless.
project beyond the therapist-client relations and envelope The use of alcohol affects not only adults but also children
all disciplines interacting with the client.302 In order for and adolescents. Still another population of children affected
these interactions to occur, the clinician’s emotional state by alcohol abuse has surfaced as a specific clinical problem.
requires some vulnerability, allowing him or her to be open These children are infants who have the effects of fetal alco-
to new and as-yet-unanalyzed or unprocessed input. This hol syndrome. A variety of researchers have investigated the
vulnerability implies the role not of an expert who knows effects of alcohol and other toxic drugs on neuromotor and
the answers beforehand but of an expert investigator. Being cognitive development.314-319
open must incorporate being sensitive not only to the vari- Alzheimer Disease (See Chapter 27). In Alzheimer
ability of motor responses but also to the variability of disease, the hippocampus and nucleus basalis are the most
emotional responses on the part of the client.303,304 This severely involved structures, followed by neurofibrillar
vulnerability leads to compassion, understanding, and degeneration of the anterotemporal, parietal, and frontal
acceptance of the client as a unique human being. It can lobes.252,320-323
also be exhausting. Therapists need to learn ways to allow Initially the symptoms fall into several categories: emo-
openness without taking on the emotional responsibility of tional, social, and cognitive. Usually the symptoms have
each patient. a gradual onset. Depression and anxiety often are seen dur-
ing the early phases because of the neuronal degeneration
Limbic Lesions and Their Influence within the prefrontal lobes and limbic network.322,324-326
on the Therapeutic Environment During the second stage, the emotional, social, and intel-
Many lesions or neurochemical imbalances within the lectual changes become more marked. Clients have diffi-
limbic network drastically affect the success or failure of culty with demands, business affairs, and personal manage-
physical, occupational, and other therapy programs. This ment. Their memory and cognitive processing continue to
chapter does not discuss in detail specific problems and their deteriorate, whereas their awareness of the problem is often
treatment, but instead it is hoped that identification of limbic
involvement may help the reader develop a better under-
standing of specific neurological conditions and carry that
knowledge into Section II, where the specific clinical prob-
lems are discussed.
Substance Abuse (See Chapter 24). The anterior
temporal lobe (especially the hippocampus and amygdala)
has a lower threshold for epileptic seizures than do other
cortical structures.56 This type of epilepsy is produced by
use of systemic drugs such as cocaine and alcohol. The sei-
zure is often accompanied by sensory auras and alterations
in behavior, with specific focus on mood shifts and cognitive
dysfunction.305 Obviously, the precise association between
behavior and emotions or temporolimbic and frontolimbic
activity is not understood, yet the associations and thus their
impact on a therapeutic setting cannot be ignored.217,306
Whether street bought, medically administered, or ingested Figure 5-13 n Anatomy of the limbic network: schematic
for private or social reasons (such as in alcohol consumption), illustration.
still insightful, causing additional anxiety and depression. able to continue to respond to music through the progression
During this phase clients may be unable to recognize famil- of the disease, and the response to rhythm may represent
iar objects and become scared because they are losing con- overlearned motor responses that are tied to positive limbic
trol of the environment both internally and externally. Thus states. For example, nonambulatory individuals, when pre-
the client may become combative out of a defensive (fight- sented with familiar and preferred music, may stand and move
or-flight) autonomic response. For that reason, therapists with the rhythm of the sound. Their bodies may remember
need to make sure the client feels safe during therapy to how to dance with the spouse whose name they no longer
optimize the learning and compliance. The third phase know.352 A physical or occupational therapist can instruct a
manifests itself with moderate to severe aphasic, apraxic, caregiver to use music as part of everyday activities. The
and agnosic problems. Object agnosia, the failure to recog- therapeutic effects of music to engage and maintain atten-
nize objects, is a typical sign of advancing Alzheimer dis- tion, activate long-term memory, and modulate emotion
ease. Distractibility and inattentiveness are also common states are well suited to the needs of both the person with
signs of this third stage. The final stage of Alzheimer disease Alzheimer disease and his or her caregivers.352
is marked by an individual who is uncommunicative, with Head Injury (See Chapter 24).
little meaningful social interaction, who often takes on the Traumatic Injury. One potentially severe limbic problem
features of the Klüver-Bucy syndrome (see Chapter 13). that can be present after traumatic closed head injury is dif-
Thus they exhibit emotional outbursts, inappropriate sexual fuse axonal injury.215,216,353-356 The long associative bundles
behaviors, severe memory loss, constant mouth movements, or fibers that transverse the cortex on a curved route can be
and often a flexor-type postural pattern. In this latter phase, sheared by an impact or a blow to the head. One of these
the client is virtually decorticate and clinically indistin- long associative bundles is the cingulate fasciculus, which
guishable from persons with other dementias. The prognosis coordinates the amygdala and hippocampal projections to
of Alzheimer disease was only a few years ago totally bleak, and from the prefrontal cortex. Many basic perceptual strat-
but today there are hopes that in the future, pharmacologi- egies, such as body schema, hearing, vision, and smell, are
cal interventions may slow and even reverse the damage linked into the emotional and learning centers of the limbic
inflicted by this disease.327-330 network through the cingulate fasciculus.357 Thus, declara-
In spite of future treatments, the continual degeneration tive learning through sensory and cognitive processing can
of the limbic network is a key distinguishing factor in become impossible. If the pathways to and from the hippo-
Alzheimer disease.109,331-333 Many clients in the past have campus and amygdala are sheared bilaterally, total and per-
been misdiagnosed as having other problems such as intra- manent global anterograde amnesia will be present.109,358,359
cranial tumors, normal pressure hydrocephalus, multiinfarct If destruction of both tracts on one side occurs, but the con-
dementia, or alcoholic or chronic drug intoxication.334-337 tralateral side is left intact, the individual can compensate,
Similarly, many clients with tumors, multifaceted demen- but learning will be slower or the rate of processing
tias, alcoholism, or heart attacks resulting in hippocampal delayed.157 If only one tract on one side is damaged, such
damage may be diagnosed with Alzheimer disease. When as the tract to and from the hippocampus, the amygdaloid
the disease is correctly evaluated and diagnosed, however, system on the same side will compensate but be slower
it becomes obvious that the limbic-cortical area involved than without the lesion.157 Thus the specific degree of
from phase 1 through the last phase is interacting with other involvement will vary and depend on the extent of shearing.
areas of the brain and constantly affecting the behavioral Those with total shearing on both sides will usually be in
patterns of the patient.338-341 Owing to the neurochemical a deep coma and will not survive the injury.360 Those with
sensitivity and production within the limbic network, drugs less severe insult will show signs ranging from total amnesia
are often used to prevent or slow the progression of to minor delays in declarative learning.361 The emotional
Alzheimer disease (see Chapter 36).342-346 Similarly, a problems of traumatic head injury can often be associated
genetic predisposition has been found in some patients with with other limbic problems such as posttraumatic stress
Alzheimer disease56,347-349; thus, gene therapy may prove to syndrome. This problem is especially apparent when treat-
have great therapeutic value.350 Because music is able ing soldiers injured on the battlefield who have returned
to activate many different brain areas, it is particularly valu- home.362
able in the treatment of persons at all stages of Alzheimer Integrating various professions becomes a critical aspect
disease351 and can effectively be used during physical or of an injured soldier’s rehabilitation. When the interaction of
occupational therapy. Long after declarative memory is lost, the limbic network and higher control is considered, addi-
individuals can sing entire songs (procedural memory), tional variables can be taken into consideration within the
dance with a loved one they no longer recognize (procedural therapeutic environment. In treating more than 200 patients
memory), or be soothed and calmed by hearing someone who who had trauma, Körlin242 found that certain kinds of musi-
cares about them singing familiar favorite songs or lullabies cal elements often triggered intrusive and traumatic reexpe-
(limbic response). This is why the power of music is so riences of the event. (For a theoretical discussion of this
great, especially as observed in individuals with Alzheimer phenomenon, see Goldberg.363) The phenomenon of audi-
disease who have lost declarative memory. In earlier phases tory triggering has implications for the rehabilitation setting,
of the disease, individuals who have lost words can recall where patients may be recovering from traumas related
words such as song lyrics through the linking with melody. to accident, injury, or difficult medical procedures. Both
When melody is lost, individuals still retain rhythmic re- environmental noise and “background” music may present
sponses. At the palliative stage of Alzheimer disease care, auditory triggers that elicit limbic network and ANS activity.
agitated patients are observed to calm to simple music such Thus the potential for eliciting the F2ARV continuum dur-
as familiar lullabies. Thus Alzheimer disease patients are ing a physical or occupational therapy treatment session is
always present, and the therapists need to be acutely aware all reflect a strong emotional or limbic component. Given
of background noise. Moore’s concept of a limbic network that MOVEs us and
Cerebral contusions (bruises) have long been a primary the F2ARV continuum regarding emotional control over
sign of traumatic head injury.364 Regardless of area of im- noxious or negative input, it is no wonder so many clients
pact, the contusions are generally found in the frontal and have difficulty with personal and emotional control over
temporal regions. There are long-term neuropsychological their reactions to the therapeutic world. If the imbalance
ramifications after mild traumatic brain injury even when were within the client, then the external environment would
there is no loss of consciousness.365 The regions most fre- be one possible way to help center the client emotion-
quently involved are orbitofrontal, frontopolar, anterotem- ally.374,375 This centering requires that the therapist be sensi-
poral, and lateral temporal surfaces. tive to the emotional level of the client. As the client begins
The limbic network’s connection to these areas would to regain control, an increase in external environmental de-
suggest the potential for direct and indirect limbic involve- mands would challenge the limbic network. If the demand
ment. The greater the contusion, the greater the likelihood is excessive, the client’s emotional reaction as expressed
that the limbic structures might simultaneously be involved. by motor behavior should alert the therapist to downgrade
Impulsiveness, lack of inhibition, and hyperactivity are a the activity level.
few of the clinical signs associated with orbitofrontal or Head injuries affect many areas of the CNS. A client with
limbic involvement.366 The dorsomedial frontal region, in- spasticity, rigidity, or ataxia may exhibit an increase in those
volved in the hippocampal-fornix circuit (once referred to as motor responses when the limbic network becomes stressed.
the Papez circuit),367 when damaged seems to induce a pseu- Learning to differentiate a motor control problem from a
dodepressed state, including slowness, lack of initiation, and limbic problem that influences the motor control systems
perseveration. requires that the therapist be willing to address the cause of
Nontraumatic Head Injuries: Anoxic or Hypoxic Brain the problems and their respective treatments.376 Each client
Injury. Lack of oxygen to the brain, regardless of the cause, is different, no matter the commonalities of the site or extent
seems not only to have a dramatic effect throughout of the lesions, because of prior learning, conditioning of the
the cortex but also selectively damages the hippocampal limbic network, and their respective perception of quality of
regions.368 The loss of hippocampal declarative memory life.377 The response of two clients to the same clinical learn-
systems bilaterally would certainly provide one reason for ing environment may have great variance and should not
the slowness in processing so commonly observed in head surprise the clinician. Thus the therapist needs to give undi-
injury.369 A hypothesis could also be made regarding the vided attention to the client at all times and be willing to
limbic network’s interrelation with other cortical and brain make moment-to-moment adjustments within the external
stem structures. In cases of hypoxia, many structures inter- environment to help the client maintain focus on the desired
connecting in the limbic network are potentially affected, so learning.
information sent to the limbic network may be distorted. Vestibular Disorders. The vestibular system has exten-
These distortions could cause tremendous imbalances within sive neuronal connections and commissural influences on
the limbic processing system, with not only attention and the limbic network and structures; conversely, the limbic
learning problems but also the hypothalamic irregularity network has significant influence on the vestibular nuclei.
often seen in head trauma. Individuals who demonstrate Details of the neuroanatomical connections are described
obstructive sleep apnea, another cause of hypoxia, have later in this chapter.
been shown to have an imbalance in the hippocampal It is generally accepted that vestibular dysfunction
area.370 This imbalance may lead to severe cognitive dys- results in erroneous input to the CNS. This erroneous sen-
function.371 This preexisting hypoxic environment certainly sory information creates a mismatch between the external
can have a long-term effect on any patient who has CNS (afferent) cues and the internal conceptual model for
damage at any age. movement contained by the cerebellum. This mismatch
A therapist always needs to understand the environ- creates an imbalance in vestibular and cerebellar signals to
ment within which the injury occurred as well as being the CNS, flooding the central limbic structures and result-
aware of preexisting complications. If the injury was sus- ing in symptoms such as vertigo, motion sickness, nausea,
tained in a violent confrontation, such as a fight or a or decreased postural control. Detection of this mismatch
frightful experience such as a near-drowning, the emo- results in an attempt by the cerebellum to compensate for
tional system had to be at a high level of metabolic activ- the imbalance, which becomes a core tenet of recovery.378
ity at the time of the insult. If the event was anoxic, then Alternately, this neural stimulation may create an internal
those areas with the highest oxygen need or at the highest stressor, and trigger an adverse limbic response, such as a
metabolic state might be the most affected or damaged GAS response.
after the event. Knowing that information, a therapist’s Newer evidence from animal research has demonstrated
analytical problem-solving strategies should guide her or that vestibular lesions result in dramatic changes in the
him toward limbic assessment. morphology and function of the hippocampus. Of note is
Summary of Limbic Problems with Head-Injured Clients. that bilateral vestibular lesions have been associated with
The behavioral sequelae after any head injury reflect many hippocampal atrophy. The hippocampus makes unique con-
signs of limbic involvement. In studies of both the pediatric and tributions to memory, both spatial and nonspatial. Thus
adult populations,353-355,357,365,372,373 behaviors of impulsive- vestibular lesions impair learning and memory, particularly
ness, restlessness, overactivity, destructiveness, aggression, those tasks that require spatial processing. In addition to the
increased tantrums, and socially uninhibited behaviors (lack more well known deficits in spatial and gravitational orien-
of social skills) are frequently reported. These behaviors tation for balance control, vestibular lesions can also result
in impaired cognition, learning, and memory through dam- often goes undetected within the initial medical workup
age to this connection. Decreased concentration, thought and management plan excepting in specialty vestibular
processes, and memory are among the most common com- practices.393-397 When the disorder is undetected and left
plaints in patients with vestibular disorders. In the past these unchecked, the patients do not respond to standard treatment
complaints were often attributed to competitive resources, interventions. They also complain of atypical symptoms or
suggesting that cognitive resources were being devoted responses to these typical treatments. When the patient does
to the basic tasks of staying balanced during function. It not respond in predictable ways to standard treatment, the
is now clear that there is a true physiologic explanation label “aphysiological” is applied, particularly in situations
for these secondary symptoms, which are quite limiting to where disability or secondary gain is a factor. Fortunately
activity and participation in normal daily activities, particu- there are well established performance criteria that can
larly working. It has also been suggested that treatment effectively differentiate true balance or vestibular impair-
activities that stimulate the function of the vestibular system ment from embellishment for secondary gain.398 (Refer to
also stimulate activity within the hippocampus and can Chapters 22A and 22B.)
improve memory, which has important implications for In treatment, recovery is based on long-term compensa-
treatment.379-387 tion mediated by the cerebellum, and symptoms must be
Thus vestibular dysfunction can influence the therapeutic reproduced for recovery to occur. However, stimulation of
environment both in the assessment and the treatment of this the vestibular system must be controlled, with every effort
system. However, the vestibular system is not a primary made to maintain a limbic (emotional) neutral state. Some
consideration of most physicians and therapists during patients have true vestibular dysfunction that affects only
evaluation. On the basis of benchmarking data from within motor responses, whereas other patients have true limbic
specialized balance centers, the average patient with a ves- psychiatric problems that do not manifest themselves with
tibular disorder (dizziness or imbalance) travels within the vestibular symptoms. These two behaviors are located at the
medical system an average of 52 months before finding a polar ends of the curve between limbic motor and vestibular
solution. During this time, he or she has seen on average motor dysfunction. Before prescribing appropriate interven-
four physicians. There is also at least one visit to the emer- tion strategies, the clinician must be clear regarding the
gency department in crisis and one visit to a psychiatrist. degree of limbic overlay on the vestibular dysfunction, and
Typically there has been no rehabilitation referral or inter- the question “What are the best vestibular and limbic inter-
vention during this time.388 active environments that will challenge and drive neuroplas-
The patient with a chronic vestibular disorder can have tic change?” must be answered. Although researchers233,390
myriad symptoms, including vegetative, autonomic, mo- have identified tools that differentiate the two extremes, today
toric, cognitive, psychological, and behavioral symptoms researchers are trying to clarify the midrange of patients
that are often misdiagnosed during this search for an out- who clearly have symptoms on the basis of the interaction of
come as other, more serious medical diagnoses. As an both systems.398,399 Development of tools that can further
example, of those patients diagnosed with dizziness or discriminate whether the behaviors are first driven by ves-
imbalance of a psychologic origin, evidence has deter- tibular and followed by limbic responses, or vice versa, is a
mined that more than 70% of these patients have underly- key to treatment planning.
ing vestibular dysfunction on key vestibular function tests Parkinson Disease. The motor impairments seen in
(electronystagmography and calorics, rotary chair, com- individuals with Parkinson disease are widely accepted,
puterized dynamic posturography, auditory brain stem understood, and treated by physical and occupational thera-
response, and acoustic reflexes).233,235,389-391 Conversely, of pists (see Chapter 20). What is not commonly synthesized
those patients with chronic dizziness and imbalance, only by physical and occupational therapists, no matter the work-
16% were found to have dizziness of a true psychogenic ing environment (hospital, outpatient, rehabilitation, home
origin.392 Acknowledgment of a patient’s symptoms, use health), is that individuals diagnosed with Parkinson disease
of data, and explanation (in understandable detail) that often have limbic involvement.
there is a physiologic explanation for his or her com- Masked expression is accepted as a motor sign of this
plaints builds the client-therapist relationship and begins disease and is linked directly to the rigidity expressed within
to neutralize the client’s abnormal limbic state (anxiety the motor system. Yet, a masked expression is also associ-
versus depression). It can lower the GAS or autonomic ated with fear as an emotion (see Figure 5-6). Similarly, the
cascade, maximizing the treatment time before the onset ability to extinguish this fear response or masked expression
of limiting symptoms (i.e., raise the symptom threshold). is also based on the infralimbic prefrontal lobe and the
Even patients with motion sickness have documentable number of dopamine receptors.400 These areas may not be
physiologic and functional changes. Some of the best cur- directly damaged by Parkinson disease, but the amount of
rent evidence is in our military personnel with symptoms of available dopamine is dramatically reduced. Given this
motion sickness. On examination, these soldiers have phys- interaction, patients with Parkinson disease may have
iological changes identifiable by results of rotary chair difficulty facially expressing what they are feeling. Thus,
(60% with abnormally long time constants) and computer- when a therapist sees a patient with a fixed facial expression,
ized dynamic posturography (70% with abnormal sensory that therapist cannot draw a conclusion from that facial
organization test [SOT] condition 5 and 6).393 expression.
Patients who have sustained a mild head injury, postcon- Similarly, depression is commonly associated with any
cussive syndrome, blast exposure or injury (positive or individual with a degenerative disease.401,402 Obviously,
negative pressure event), or whiplash often have concomi- depression from a neuroanatomical perspective is housed
tant involvement of the vestibular apparatus or nuclei. This with the limbic system. Depression from a motor response
perspective causes lower postural tone with increased flex- When the tumor is located within the frontal and temporal
ion in the neck (see Figure 5-8, B). This pattern within the lobes, associated with limbic structures, psychiatric problems
trunk is also often described as the postural patterns of an may manifest, ranging from depression to anorexia to psycho-
individual with Parkinson disease. The question arises, “Is sis.109,418 Obsessive-compulsive disorder resulting from lim-
the tone generated from the motor system alone, from the bic tumor has been used as a tumor marker for relapse.419
limbic influence on the motor system alone, or from a com- Amnesia has been reported in patients with dorsomedial
bination of the two?” thalamus, fornix, midbrain, and reticulolimbic pathway
Individuals working in a psychological setting (inpa- lesions. This again reinforces the importance of the limbic
tient and outpatient) may focus on the psychoemotional network’s role in storage.109,420,421
problems without addressing the functional motor involve- The neurochemistry within the limbic network is very
ment. It is not infrequent that an individual with this dis- complex and will be discussed within the next large section,
ease may simultaneously exhibit signs of psychosis and but even without a keen understanding of the specific chem-
other potential psychiatric problems.403-407 It is critical for istry, therapists need to recognize behavior and mood
therapists, despite the physical setting, to develop and un- changes within the client. These changes often signal neuro-
derstand the entire spectrum of the problems associated chemical problems affecting the individual’s motor system.
with this disease. If medical intervention includes medicine, pharmacists
Cerebrovascular Accidents (See Chapter 23). The should be able to explain how those behaviors are being
most common insult in CVA results in occlusions within regulated by pharmacological intervention. Literature is now
tributaries of the middle cerebral artery.56 When this occlu- reporting that what were once thought idiopathic seizures
sion is in the right hemisphere, studies have shown that cli- are now believed to be neurochemical imbalances with the
ents are often confused and exhibit metabolic imbalance.408 limbic structure and may someday be controlled with medi-
The primary problem of this confused state is inattention. cations that directly affect the immune system.422
After brain scans, it has been shown that focal lesions ex- Ventricular Swelling after Spinal Defects in Utero,
isted within both the reticulocortical and limbic cortical Central Nervous System Trauma, and Inflammation
tracts, suggesting direct limbic involvement in many middle (See Chapters 15, 24, and 26). Although the effects
cerebral artery problems.66 of ventricular swelling after trauma, inflammation, and in
With the use of magnetic resonance imaging (MRI), utero cerebrospinal malformations are not discussed in great
specific lesion deficits after CVA can help physicians and detail in the literature with respect to limbic involvement,
therapists identify specific motor and limbic behavioral the proximity of the lateral and third ventricle to limbic
problems that would limit quality of life of the pa- structures cannot be ignored. It is common knowledge that
tients.409-411 Many clients who have had a CVA do not have most people exposed to hot, humid weather begin to swell;
direct limbic involvement, yet the stresses placed on the become more irritable, less tolerant, and moody; and may
client,412,413 whether external or internal, are often reflected complain of headaches. Some people become aggressive,
in the limbic network’s influence over cognition and the others lethargic. All these behaviors are linked to some
motor control systems.414 Everyday existence as well as extent with limbic function. Thus, ventricular swelling caus-
performance of motor tasks required during therapy are ing hydrocephalus, whether caused by trauma, inflamma-
usually valued highly in the client’s life. This value or tion, or obstruction, would potentially affect the limbic
stress placed on the limbic network overflows into the structures. Reported behavioral changes such as seizures,
motor system and never allows it to relax, as observed memory and learning problems, personality alterations,
by noting the increase of tonus in the unaffected leg. The alertness, dementia, and amnesia can be tied to direct or
client is usually unaware of this buildup of tonus but can indirect limbic activity.56
release it once attention is drawn to it. If attention is never Summary of Clinical Problems Affected by Limbic
directed toward these tension buildups, a therapist trying Involvement. It is easy to identify limbic problems when
to decrease tonus in the affected arm or leg will always the behaviors deviate drastically from normal responses. It
be interacting with the associated patterns from the is much more difficult to determine subtle behavior shifts in
less-involved extremities. clients. The therapist should be sensitive to these minor
Tumor (See Chapter 25). Any brain tumor, regardless mood shifts because they may represent early signs of
of whether it directly affects the limbic structures, will cer- future problems. Similarly, noting that a particular client
tainly arouse the limbic network because of the stress, anxi- is always irritable and has difficulty learning on hot days
ety, and emotional overlays of the diagnosis. The degree of should help direct the therapist toward establishing a treat-
emotional involvement will obviously affect the declarative ment session that regulates humidity and temperature to
learning of the client as well as the limbic network’s influ- optimize the learning environment. The limbic network is
ence over motor response. not just a neurochemical bundle of nuclei and axons found
Tumors specifically arising within limbic structures415,416 within the brain. It is a pulsating center that links percep-
can cause dramatic changes in the client’s emotional behavior tion of the world and the way an individual responds to that
and level of alertness, especially with hypothalamic tumors.417 perception. Quality of life is a value, and that value has a
The behaviors reported include aggressiveness, hyperphagia, strong limbic component. If functional outcomes leading to
paranoia, sloppiness, manic symptoms, and eventual confu- maintaining or improving the quality of life of our clients is
sion.56 Tumors within the hypothalamus cause not only the goal of both physical and occupational therapy,291,423,424
behavioral abnormalities but also autonomic endocrine imbal- then the limbic network is no less important during exami-
ances, including body temperature changes, menstrual abnor- nation, evaluation, prognosis, and intervention than the motor
malities, and diabetes insipidus.109 system itself.
THE NEUROSCIENCE OF THE LIMBIC disconnected from other brain regions. Recent research
NETWORK has clearly identified the neurochemical precursors to this
aggressive behavior.53,56,428-430 This “septo-hypothalamic-
Basic Anatomy and Physiology mesencephalic” continuum, connected by the medial forebrain
A brief overview of the anatomy and physiology of the lim- bundle, seems to be vital to the integration and expression
bic network is presented in the following sections. The reader of emotional behavior.431 The linking of other brain struc-
is referred to a variety of textbooks and websites for a more tures to emotions came initially from the work of Papez,367
in-depth understanding of this system56,119 and how higher who first identified the hippocampal-fornix circuit. He saw
thought might be much more complex than previously identi- this as a way of combining the “subjective” cortical experi-
fied.44,68,425-427 ences with the emotional hypothalamic contribution. Earlier,
Broca71 labeled the cingulate gyrus and hippocampus “cir-
Basic Structure and Function cle” as “the great limbic lobe.” Today, the concept of the
The limbic network can best be visualized as consisting of limbic network and its interaction with sensory inputs and
cortical and subcortical structures with the hypothalamus motor expression has become extremely complex.432 Mood
located at the central position (Figures 5-13 and 5-14). The can change motor output, and motor activity can change
hypothalamus is surrounded by the circular alignment of the mood.421,433
subcortical limbic structures vitally linked with one another Klüver and Bucy434 linked the anterior half of the tempo-
and the hypothalamus. These structures are the amygdaloid ral lobes and the amygdaloid complex to the limbic network.
complex, the hippocampal formation, the nucleus accum- They showed changes in behavior, with specific loss of the
bens, the anterior nuclei of the thalamus, and the septal amygdaloid complex and anterior hippocampus input, re-
nuclei (see Figure 5-13). These structures are again sur- sulting in (1) restless overresponsiveness, (2) hyperorality of
rounded by a ring of cortical structures collectively called examining objects by placing them in the mouth, (3) psychic
the “limbic lobe,” which includes the orbitofrontal cortex, blindness of seeing and not recognizing objects and the pos-
the cingulate gyrus, the parahippocampal gyrus, and the sible harm they may entail, (4) sexual hyperactivity, and (5)
uncus. Other neuroanatomists also include the olfactory emotional changes characterized by loss of aggressiveness.
system and the basal forebrain area (see Figure 5-14). These changes have been named the Klüver-Bucy syndrome
Vitally linked and often included in the limbic network as (see Chapter 13).435 Myriad connections link the amygdala
the “mesolimbic” part is the excitatory component of the to the olfactory pathways, the frontal lobe and cingulate
reticular activating system and other brain stem nuclei of the gyrus, the thalamus, the hypothalamus, the septum, and the
midbrain. Some consider components of the midbrain a very midbrain structures of the substantia nigra, locus coeruleus,
important region for emotional expression.86 Derryberry and periaqueductal gray matter and the reticular formation. The
Tucker86 found that attack behavior aroused by hypotha- amygdala receives feedback from many of these structures
lamic stimulation is blocked when the midbrain is damaged it projects to by reciprocal pathways.
and that midbrain stimulation can be made to elicit “attack At the heart of the limbic network is the hypothalamus.
behavior” even when the hypothalamus has been surgically The hypothalamus, in close reciprocal interaction with most
Figure 5-14 n Limbic network circuitry with parallel and reverberating connections and with
medial forebrain bundle.
centers of the cerebral cortex and the amygdala, hippocam- These pathways course through the lateral hypothalamus to
pus, pituitary gland, brain stem, and spinal cord, is a primary terminate in the cingulate gyrus in its ascending limb and in
regulator of autonomic and endocrine functions and controls the reticular formation of the midbrain in its descending
and balances homeostatic mechanisms. Autonomic and so- part; these pathways have strong interconnections and con-
matomotor responses controlled by the hypothalamus are trol over the periaqueductal gray area.192 These links enable
closely aligned with the expression of emotions.429,436-438 the limbic network itself and the non–limbic-associated
In the temporal lobe, anteromedially is the amygdaloid structures to act as one neural task system. No portion of the
complex of nuclei, with the hippocampal formation situated brain, whether limbic or nonlimbic, has only one function.56
posterior to it. Located medial to the amygdala is the basal Each area acts as an input-output station. At no time is it
forebrain nuclei, which receive afferent neurons from the totally the center of a particular effect, and each site depends
reticular formation, the hypothalamus, and the limbic cortex. on the cooperation and interaction with other regions. For
From this basal forebrain, efferents project to all areas of the therapists the concept of neuroplasticity within the motor
cerebral cortex, the hippocampus, and the amygdaloid body, system is incorporated into our theories of motor learning,
providing an important connection between the neocortex but we still have difficulty integrating sensory, emotional,
and the limbic network. These nuclei represent the center of and motor components as interactive elements in motor per-
the cholinergic system, which supplies acetylcholine to lim- formance. Yet research is identifying that these neurocircuit-
bic and cortical structures involved in memory formation. ries are present and interactive.444
Depletion of acetylcholine in clients with Alzheimer disease The parvicellular reticular formation (PCRF, or lateral
relates to their memory loss.192,320,321,439 medullary reticular formation), together with the nucleus
tractus solitarius, receives both vestibular and nonvestibular
Interlinking the Components of the System input from the cortex, cerebellum, and limbic network and
The limbic network has many reciprocating interlinking is considered functionally as the vomiting center. It also re-
circuits among its component structures, which provide for ceives input from the area postrema (floor of the fourth
much functional interaction and also allow for continuing ventricle), which contains the chemoreceptor region for the
adjustments with continuous feedback (Figure 5-15).56,429 production of vomiting in response to noxious chemicals.
The largest pathway is the fornix.440 Commissural fibers from the vestibular nuclei complexes
Another limbic pathway is the stria terminalis, which run through the PCRF and connect the vestibular nucleus to
originates in the amygdaloid complex and follows a course the reticular formation through axon collaterals. The PCRF
close to the fornix to end in the hypothalamus and septal also projects fibers to the parabrachial nuclei that contain
regions. The amygdala and the septal region are also con- the respiratory centers and to the hypoglossal nucleus.378
nected by a short direct pathway called the diagonal band Visceral autonomic input from multiple sources, including
of Broca. A third pathway, the uncinate fasciculus, runs the vestibular nuclei, converges in the parabrachial nucleus.
between the amygdala and the orbitofrontal cortex.56,441,442 The locus ceruleus and autonomic brain stem nuclei also
The medial forebrain bundle and other parallel circuits (see receive vestibular nuclear input.234,445-448 Thus, cardiovascu-
Figure 5-14) are vital connections of the limbic network.443 lar activity and respiration (brain stem–mediated autonomic
Figure 5-15 n Interlinking neuron network within the limbic network. (Adapted from Kandel ER,
Schwartz JH, Jessell TM: Principles of neural science, ed 4, New York, 2000, McGraw-Hill.)
activity), as well as vomiting, are highly influenced by the the lateral portion originates in the limbic network (lateral
status of the vestibular system. If we could understand how hypothalamus, amygdala, and bed nucleus of the stria termi-
cold to the neck or forehead, pressure to the wrist, or taste nalis). The prefrontal area may be the master controller over
or olfactory input of ginger interacts with known autonomic this regulatory system.458-461 The functional motor implica-
reactions and nausea in response to chronic vestibular or tion of these tracts is determined by whether the fibers pro
interneuronal connections problems, the synthesis of many ject as part of a medial or lateral descending system. The
aspects of health care delivery would no longer be a mys- medial system, through the locus coeruleus, periaqueductal
tery. Obviously, these older treatment techniques are effec- gray matter, and raphe spinal pathways, plays a role in the
tive and have been for thousands of years, but to today’s general level of activity of both somatosensory and motor
researchers the “why” drives the desire to better understand neurons. Thus the emotional brain or limbic network has an
the neuromechanisms underlying the observable responses. effect on both somatosensory input and motor output. These
There are three different types of drugs that neuroanatomi- fibers can alter the level of excitation to the first synapse of
cally suppress or modulate vestibular input and thus have a somatosensory information, thus altering the processing
dramatic effect on dizziness and nausea.449 or importance of that information as it enters the nervous
Research involving functional MRI (fMRI) supports the system. Similarly, it can alter the level of motor generators
concept that there is increased activity within the inferior involved in motor expression, which may account for the
frontal cortex when nausea is induced by either vestibular extension with anger and flexion with depression. The lat-
stimulation or ingestion of an emetic.450 This research veri- eral system seems to be involved in more specific motor
fies that there is a strong interconnection among vestibular output related to emotional behavior and may explain some
input, limbic nuclei, and autonomic responses.451 of the loss of fine motor skill when one is placed in an emo-
There are also connections between the parabrachial tional situation such as competition. To differentiate whether
nucleus and higher brain centers, including the amygdala, the tonal conditions of a client are a result of limbic imbal-
which is known to be critical in the development of condi- ance or problems within the traditionally accepted motor
tioned avoidance, such as found in agoraphobia, as an ex- system, the clinician would need to observe the emotional
ample. Thus, vestibular input results in a sensory stimulus state and how it changes within the client. If the abnormal
that may induce a state of general autonomic discomfort as state consistently alters with mood shifts, then limbic involve-
a trigger of avoidance that precedes the onset of a panic ment causing motor control disturbances would be identi-
attack.378,445,451,452 fied. Human social behavior requires motor expression, yet
Vestibular firing rates are modulated and regulated from that behavior is driven through the limbic circuitry.444,462-464
the DRN of the midbrain and rostral pons. The DRN is the Neuroimaging has helped to reduce uncertainty concerning
largest producer of serotonin in the brain and explains the the anatomical pathways, and neurochemistry has widened
significant linkage between vestibular dysfunction and anxi- the possibilities of variations across synaptic connections.465-467
ety, and sleep deprivation and anxiety.237
Vestibular lesions in animals result in dramatic changes Neurobiology of Learning and Memory
in the morphology and function of the hippocampus. Of note
is that bilateral vestibular lesions have been associated with Functional Applications for an Intact System
hippocampal atrophy. The hippocampus is responsible for “Ultimately, to be sure, memory is a series of molecular
spatial and gravitational orientation, cognition, learning, and events. What we chart is the territory within which those
memory (spatial and nonspatial).379-387 events take place.”123 Although expressed more than four
During the past decade anatomical pathways have been decades ago, these words are still accurate. They were ex-
identified that are descending motor tracts that terminate in pressed by a master clinician and researcher, a clinician who
the caudal brain stem and spinal cord.453-455 These pathways watched behavior, emphasized neuroscience, stressed accu-
help modulate the activity level of somatic and autonomic rate documentation, and always was respectful and aware of
motor neurons. Some of these tracts receive direct and indi- patient interaction and how that affected motor behavior.
rect afferent information from the periphery and are part of The brain stores sensory and motor experiences as mem-
the interneuronal projection system to motor neurons. They ory. In processing incoming information, most sensory
are found in the caudal brain stem, in the spinal cord, and pathways from receptors to cortical areas send vital infor-
between the two and play a role in the generation of fixed mation to the components of the limbic network. For
action patterns such as biting and swallowing, which have a example, extensions can be found from the visual pathways
strong emotion context linked to the motor program.456,457 into the inferior temporal lobe (limbic network).56,468,469
Some of the pathways are linked with the ventromedial and Visual information is “processed sequentially” at each syn-
lateral systems, identified for many years as part of the proxi- apse along its entire pathway, in response to size, shape,
mal and axial and distal motor control system, modulated by color, and texture of objects. In the inferior temporal cortex,
a variety of structures.192,457 They connect the limbic net- the total image of the item viewed is projected. In this way
work to the brain stem and spinal neuronal pools. These the sensory inputs are converted to become “perceptual
tracts do not seem to synapse on what would be considered experiences.” This also applies to other sensory stimuli, such
true motor nuclei of the brain stem (e.g., red nucleus, ves- as tactile, proprioceptive, and vestibular. The process of
tibular nuclei, lateral reticular nuclei, interstitial nuclei translating the integrated perceptions into memory occurs
of Cajal, or inferior olive). However, these pathways do bilaterally in the limbic network structures of the amygdala
connect with raphe nuclei, periaqueductal gray matter, and and the hippocampus.56,470-481
locus coeruleus. The medial components of these tracts Before the limbic network’s impact on learning and
originate within the medial portion of the hypothalamus, and memory can be delved into, a clear understanding of what is
meant by these functions is needed. Current theories support the limbic network may become a key element in the
a “dual memory system” that uses different pathways in the success or failure of that movement.491,492 Most functional
nervous system. Terms such as verbal and nonverbal, habit tasks or activities practiced in a clinical setting have value
versus recognition, intrinsic and extrinsic, and procedural attached to them. That value can be clearly seen by observ-
and declarative have been given to these two memory sys- ing the emotional intent placed on the activity by the
tems. These systems do not operate autonomously, and client.493
many therapeutic activities seem to combine these memory The two reverberating or reciprocal pathways, or circuits,
systems to achieve functional behavior.56 In reality, the com- within the limbic network most intimately involved in declara-
plexity of memory is not a two-category system. Verbal and tive learning are (1) the amygdaloid, dorsomedial thalamic
nonverbal memory both interact with declarative function.482 nucleus, and cortical pathways and (2) the hippocampal, for-
Even within spatial memory, additional areas of integration nix, anterior thalamic nucleus, and cortical pathways.
and parallel circuitry have been identified.483,484 The hippocampus may be more concerned with sensory
For this discussion, two specific categories of learning— and motor signals relating to the external environment,
procedural and declarative—will be used, although in today’s whereas the amygdala is concerned more with those of the
neuroscience environment, the terms implicit and explicit internal environment. They both contribute in relation to the
memory are used as frequently. Both categories of learning significance of external or internal environmental influ-
have been correlated to limbic function.485-487 Declarative ences.475,494-499 The hippocampus is rich in stem cells and
(explicit) memory entails the capability to recall and verbally may be a primary nuclear mass that directs the bodily sys-
report experiences. This recall requires deliberate conscious tems to heal after injury. This is especially true when the
effect, whereas the procedural counterpart is the recall of external environment is enriched and nurtures the emotional
“rules, skills, and procedures (implicit),”56 which can be environment for that healing.500,595
recalled unconsciously. The amygdaloid circuits seem to deal with strongly emo-
Procedural learning is vital to the development of motor tional and judgmental thoughts, whereas the hippocampal
control. A child first receives sensory input from the various circuits are less emotional and more factual. The amygdala
modalities through the thalamus, terminating at the appropri- may be more involved in emotional arousal and attention, as
ate sensory cortex. That information is processed, a functional well as motor regulation, whereas the hippocampus may
somatosensory map is formulated,124,488 and the information deal with less emotionally charged learning. These limbic
is programmed and relayed to the motor cortex. From there, circuits seem crucial in the initial processing of material that
it is sent to both the basal ganglia and the cerebellum to leads to learning and memory. Once the thought has been
establish plans for postural adaptations, refinement of motor laid down within the cortical structures, retrieval of that
programs, and coordination of direction, extent, timing, force, specific intermediate and long-term memory does not seem
and tone necessary throughout the entire sequence of the to require the limbic network, although new associations
motor act. Storage and thus retrieval of memory of these will need to be run through the system.56,471,473,475
semiautomatic motor plans are thought to occur throughout A third component in the memory pathway involves the
the motor control system.56 The complexity of this process medial diencephalon, a structure that contains the thalamic
has had an impact on the study of motor control and variables nucleus. When this region is destroyed by neurotrauma such
that might affect that control.489 as strokes, neoplasms, infections, or chronic alcoholism,
The frontal lobe, basal ganglia, and cerebellum are criti- global amnesias result, owing to the destruction of the amyg-
cal nuclei for changing and modulating existing programs.56 dala and hippocampus. The amygdala and hippocampus send
Many interlocking neuronetworks establish pathways allow- fibers to specific target nuclei in the thalamus, and the de-
ing for the conceptualization of research on motor theory struction of these tracts also causes the same amnesic effect.
concepts of reciprocity, distributed function, consensus, and It appears that the limbic network and the diencephalon co-
so on (see Chapter 4). Procedural learning and memory operate in the memory circuits. The medial diencephalon
do not necessitate limbic network involvement as long as seems to be another relay station along the pathway that
an emotional value is not placed on the task. This memory leads from the specific sensory cortical region to the limbic
deals with skills, habits, and stereotyped behaviors. This structures in the temporal lobe to the medial diencephalic
motor system is involved in developing procedural plans structures and ends in the ventromedial part of the prefrontal
used in moving us from place to place or holding us in a cortex (Figure 5-16).56,501,502
position when we need to stop.56 As shown in Figure 5-16, memories may be stored in the
Unlike procedural learning and memory, declarative sensory cortex area, where the original sensory input was
(explicit) learning and memory require the wiring of the interpreted into “sensory impressions.” Today, concepts
limbic network. Recent literature has clearly identified that regarding memory storage suggest that declarative memory
the basal ganglia and cerebellum both play roles in cognitive is stored in categories similar to a filing system. Those cat-
function, especially as it relates to category learning tasks.490 egories or files seem to be stored in several cortical areas
This type of learning is closely associated with limbic func- bilaterally depending on the context.503,504 This system
tion, further identifying the complexity of what was consid- allows for easy retrieval from multiple areas. Memory has
ered two entirely separate systems. Declarative thought stages and is continually changing. It was once thought that
deals with factual, material, semantic, and categorical aspects the hippocampus only dealt with long-term memory, but it
of higher cognitive and affective processing. A strong emo- is now accepted that it also supports multi-item working
tional and judgmental component is linked with declarative memory.505 To go from short-term to long-term memory, the
thought. Thus as soon as a motor behavior has value placed brain must physically change its chemical structure (a plas-
on the act, it becomes declarative as well as procedural, and tic phenomenon). Memory first begins with a representation
Figure 5-16 n The basal forebrain closes the circuit and causes changes in sensory area neurons,
which could lead to correct perception and stored memory. This is neurochemical dependent.
of information that has been transformed through processing Klüver-Bucy syndrome. For clients with this neurological
of perceptual systems. The transferring of this new memory problem, familiar objects do not bring forth the correct
into a long-lasting chemical bond requires the neuronetwork associations of memories experienced by sight, smell,
of the limbic complex. Owing to the multiple tracts or paral- taste, and touch and relate them to objects presented.516
lel circuits in and out of the limbic network and throughout Association of previously presented stimuli and their
neocortical systems, clients, even with extensive lesions, responses appear to be lost. Animals without amygdaloid
can often learn and store new information.56,506 This may input had different response patterns that ignored previous
also explain why damage to the limbic network structures fears and aversions. Thus the amygdala adds the “emotional
does not destroy existing memory nor make it unavailable weight” to sensory experience. Loss of the amygdala takes
because it is actually stored in many places throughout away many positive associations and potential rewards,
the neocortex. The circular memory circuit illustrated in thereby altering the shaping of perceptions that lead to
Figure 5-16 shows only one system. The reader must memory storage.
remember that many parallel circuits function simultane- When stimuli are endowed with emotional value or sig-
ously. The circular memory circuit shown reverts to the nificance, attention is drawn to those possessing emotional
original sensory area after activation of the limbic structures significance, selecting these for attention and learning. This
to cause the necessary neuronal changes that would inscribe would give the amygdala a “gatekeeping” function of selec-
the event into retrievable stored memory.507 This informative filtering. The amygdala may enable emotions to influence
tion can be recognized and retrieved by activation of storage what is perceived and learned by reciprocal connection with
sites anywhere along the pathway.56,508 the cortex. Emotionally charged events will leave a more sig-
The last station or system to be added to the circuit is the nificant impression and subsequent recall. The amygdala
“basal forebrain cholinergic system,” which delivers the alters perception of afferent sensory input and thereby affects
neurochemical acetylcholine to the cortical centers and to subsequent actions.126,517,518
the limbic network, with which it is richly linked. The loss In the human, memory functioning has been associated
of this neurotransmitter is linked to memory malfunctioning with the phenomenon of long-term potentiation observed
in Alzheimer disease. Currently, many chemicals are being in hippocampal pathways.56 This potentiation of synaptic
studied for their influence on brain structures and specially transmission, lasting for hours, days, and weeks, occurs
limbic structures.509,510 Similarly, loss of this cholinergic sys- after brief trains of high-frequency stimulation of hippo-
tem plays a key role in dementia problems in Parkinsonism.511 campal excitatory pathways. Whether this phenomenon is
Performance of visual recognition memory can be aug- caused by alteration at the presynaptic or postsynaptic ter-
mented or impaired by administration of drugs that enhance minals has not been established, and the complexity contin-
or block the action of acetylcholine.512-514 ues to evolve.250 The question remains whether there is an
It has also been shown that the amygdala and hippocam- increased amount of neurotransmitter released presynapti-
pus are interchangeably involved in recognition memory.515 cally (glutamate) or whether the expected amount is produc-
The hippocampus is vital for memory of location of objects ing a heightened postsynaptic response. Or, are both sites
in space, whereas the amygdala is necessary for the associa- involved56? Even a third hypothesis regarding nonsynaptic
tion of memories derived through the various senses with a neurotransmission or exocytoses with receptor sites on the
specific recognition recall. For example, a whiff of ether surface of neurons beyond postsynaptic sites may help guide
might bring to mind a painful surgical experience or the our understanding of memory and memory storage in the
sight of some food may cause a recall of its pleasant smell. future.56,192 Recent literature has linked a neurotropic factor
Removal of the amygdala brings out the behavior shown in usually considered for long-term potentiation within the
hippocampus as a factor in amygdala-dependent learning, It appears that limbic involvement in the declarative
thus reiterating the interaction between these two nuclei and memory creates a chemical bond that allows cortical storage
their role in memory and learning.126 of “stimulus representation” necessary for subsequent rec-
Learning and memory evoke alterations in behavior that ognition and recall of the information.56,471,473,474,494
reflect neuroanatomical and neurophysiological changes.56,115 When declarative and procedural learning from a clinical
These alterations include the phenomenon of long-term reference is analyzed, a separation of functional mediation
potentiation as an example of such changes. The hippocam- can be observed. Clients with brain lesions localized in the
pus demonstrates the importance of input of long-term limbic network components of the amygdala and hippocam-
potentiation in associative learning. In this type of learn- pus have the ability to acquire and function with “rule-
ing, two or more stimuli are combined. Tetanizing of more based” games and skills but have lost the capacity to recall
than one pathway needs to occur simultaneously. When how, when, or where they gained this knowledge or to give
only one pathway is tetanized, the effect is decreased syn- a description of the games and skills learned. Relating this
aptic transmission. Long-term potentiation, requiring the to clinical performance, clients may develop the skill in a
cooperative action of numbers of coactive fibers, is engen- functional activity but not the problem-solving strategies
dered and formed by the “associative” interaction of affer- necessary to associate danger or other potentially harmful
ent inputs. Thus, long-term potentiation serves as one aspects of a situation that may develop once out of the
model for understanding the neural mechanism for asso- purely clinical setting.212,428,523-525 Similarly, if a client needs
ciative learning. Interacting with this neural mechanism to learn a procedural task such as walking, transfers, eating,
are hormones,which, combined with stress, can change the and so on, it may be extremely important to direct the atten-
specific circuitry active during the experience.519 As our tion off the task while the task is being practiced procedur-
understanding of the complexity of the limbic network ally. As knowledge about the complexity of memory evolves,
evolves, limbic responses to input stimuli need to be the clear dichotomy between explicit and implicit learning
differentiated from limbic memory and initiation of a or declarative and procedural learning is being questioned
response without the stimuli. Recent research has shown by current research.526 This study clearly demonstrates that
that the amygdala is not only involved in learning related anterograde amnesia affects learning that is dependent on
to emotional experiences but is also responsible for chang- combining a novel association with the development of
ing motor expression or conditioned response generated as memory compared with its accessibility to consciousness.
part of an autonomic fear expression.444,520 As the specificity and generalizability of memory come un-
der scrutiny, a question arises regarding the differentiation
Learning and Memory Problems after Limbic of semantic memory from music perception, music produc-
Involvement tion, and music memory.527 If emotional and associational
For initial declarative learning and memory, the combina- aspects of music memory are different from declarative
tion of hippocampus and amygdala of the limbic network memory and if both are different from procedural memory,
is required.56 For memory formation to occur, there must then perhaps music may be used to activate existing robust
be a storing of the “neural representation” of the stimuli in and rich neural networks linking different kinds of memory
the association and the processing areas of the cortex. This and learning, and/or elicit neuroplasticity potentials, to ad-
storage occurs when sensory stimuli activate a “cortico- dress therapeutic goals.528
limbo-thalamo-cortical” circuit.56 Although there is not one
single all-purpose memory storage system, this circuit Neurochemistry
serves as the “imprinting mechanism,” reinforcing the path- Discussion of the limbic network’s intricate regulation of
way that activated it. On subsequent stimulation, a stimulus many neurochemical substances is not within the scope of
recognition or recall would be elicited. In associative recall, this chapter. Yet therapists need to appreciate how potent
stored representations of any interconnected imprints could this system can be with respect to neurochemical reactions.
be evoked simultaneously.56 The amount of research reflecting new understanding
A vital processing area for all sensory modalities is of the role of neurochemistry in brain function is inundat-
located in the region of the anterior temporal lobe. This area ing the pharmacological research literature on a monthly
is directly linked with the amygdala and indirectly with the basis.479,529-536
hippocampus. The hippocampus and amygdala are also The hypothalamus, the physiological center of the limbic
linked both structurally and functionally to each other and network (see Figures 5-2 and 5-14), is involved in neuro-
to specific thalamic nuclei. Clients with temporal epileptic chemical production and is geared for passage of informa-
seizures and whose temporal lobes have been surgically tion along specific neurochemical pathways.
removed develop global anterograde amnesia—that is, Squire and colleagues537 consider it the major motor out-
amnesia develops for all senses, and no new memories can put pathway of the limbic network, which also communi-
be formed. Experimental removal of only the hippocampus cates with every part of this system. Certain nuclei of the
does not bring about these changes, although processing is hypothalamus produce and release neuroactive peptides that
slowed down. When both the hippocampus and the amyg- have a long-acting effectiveness as neuromodulators. As
dala are removed bilaterally, the amnesia is both retrograde such, they control the levels of neuronal excitation and
and global. It is postulated that the amygdala is the area of effective functioning at the synapses. By their long-lasting
the brain that adds a “positive association,” the reward part effects, they regulate motivational levels, mood states, and
to stimuli received and passed through processing. In this learning. These peptide-producing neurons extend from
way, stimulus and reward are associated by the amygdala, the hypothalamic nuclei to the ANS components and to
and an emotional value is placed on them.521,522 the nuclei of the limbic network, where they modulate
neuroendocrine and autonomic activities. The importance of behaviors observed in some clients with CNS damage may
these neuropeptides is being recognized as research begins directly reflect these mesolimbic dopaminergic systems.
to unravel the mysteries of the limbic network’s role in the The specific roles of the noradrenergic pathway are
regulation of affective and motivated behaviors.56,140,192,538-540 numerous and affect almost all parts of the CNS. The center
Lesions in the medial hypothalamus affect hormone produc- for the noradrenergic pathways is located within the caudal
tion and thus alter regulation of many hormonal control midbrain and upper pons. Its nucleus is referred to as
systems.56 For example, clients with medial hypothalamic the locus coeruleus. This nucleus sends at least five tracts
lesions may have huge weight gain because of the increase rostrally to the diencephalon and telencephalon.56 Of spe-
of insulin in the blood, which increases feeding and converts cific interest for this discussion are the projections to the
nutrients into fat. Similarly, this weight gain may be caused hippocampus and amygdala. The axons of these neurons
by hyperphagic responses resulting from the loss of satiety. modulate an excitatory effect on the regions where they
General hyperactivity and signs of hostility after minimal terminate.56 Thus the activation of this system will heighten
provocation can also be observed. These problems are often the excitation of the two nuclei within the limbic network
encountered in patients with head trauma. intricately involved in declarative learning and memory.
Lesions in the lateral hypothalamus lead to damage of Hyperactivation may cause overload or the lack of focus
dopamine-carrying fibers that begin in the substantia nigra of attention.544 Decreased activity may prevent the desired
and filter through the hypothalamus to the striatum. Lesions, responses. Attention to task may depend on continuing
either along this tract or within the lateral hypothalamus, lead noradrenergic stimulation. These tracts from the midbrain
to aphagia and hypoarousal. Decreased sensory awareness rostrally play a key role in alertness. The correlation of alert-
contributing to sensory neglect is also present in lateral ness and attention to performance of motor tasks as well as
hypothalamic lesions. The decreased awareness may be to learning can be demonstrated.56 Again, these research
caused by a decrease of orientation to the stimuli versus findings reiterate previous statements regarding a therapist’s
awareness of the stimuli once they are brought to conscious role in balancing the neurochemistry within the client’s lim-
attention. These lesions cause the client to exhibit marked bic network. From a clinical perspective, a therapist will
passivity with decreased functioning. Bilateral infarcts within observe a relaxed, motivated, alert participant in the learning
the mammillothalamic tract create an acute Korsakoff environment and will observe better carryover because the
syndrome.541 chemical interactions will only enhance the learning.
As noted earlier, depression is clearly identified as a More than 200 neurotransmitters have been identified
limbic function. A functional deficiency in monoamines, within the nervous system.56 How each transmitter and the
especially serotonin, is hypothesized to be a primary cause interaction of multiple transmitters on one synapse affect
of depression.542,543 The serotonin systems originate in the any portion of the CNS is still unclear. Certainly, some rela-
rostral and caudal raphe nuclei in the midbrain. Ascending tionships have been identified. Novelty-seeking behavior of
serotonergic tracts start in the midbrain and ascend to the the limbic network seems to be dopamine dependent,545
limbic forebrain and hypothalamus; they are concerned with whereas melatonin receptors seem to coordinate circadian
mood and behavior regulation. Damage with direct or indi- body rhythm.546 Adrenal corticosteroids modulate hippo-
rect limbic involvement results in the client exhibiting campal long-term potentiation.547 The complexity of this
depression. Descending pathways to the substantia gelati- system still challenges many researchers.
nosa are involved in pain mechanisms and have also been In conclusion, the neurochemistry of the limbic network
linked through a complex sequence of biochemical steps is intricately linked to the neurochemistry of the brain and
to the increased sensitization of the presynaptic terminals the body organs regulated by the hypothalamus. All systems
of the cutaneous sensory neurons, leading to a hyperactive within the limbic circuitry seem to be interdependent, with
withdrawal reflex or hypersensitivity to cutaneous input.56 the summation of all the neurochemistry being the determi-
This would account for the behavior patterns seen in clients nants of the specific processing of information. Similarly,
with head trauma, when the therapist sees a flexed posture the interdependence of the limbic network with almost all
with a withdrawn or depressed affect yet with an extremely other areas of the brain and the activities of those areas at
sensitive tactile system. any time reflect the complexity of this system.
It is hypothesized that the underlying pathophysiological
mechanism of one form of schizophrenia involves an exces- THE LIMBIC CONNECTIONS TO THE “MIND,
sive transmission of dopamine within the mesolimbic tract BODY, SPIRIT” PARADIGM
system.56 The dopaminergic cell bodies are located in the As neuroscientists, safe and deep within a Western allo-
ventral tegmental area and the substantia nigra. Some of pathic model of linear research, establishing efficacy and
these neurons project to the limbic network. These projec- evidence-based practice for what is taught to new learners is
tions go to the nucleus accumbens, the stria terminalis critically important.290,291,548 Yet there are too many unex-
nuclei, parts of the amygdala, and the frontal entorhinal and plainable behavioral unknowns occurring daily in the clini-
anterior cingulate cortex. It is the projection to the nucleus cal environment that cannot be researched using standard
accumbens that seems critical because of its influence over Western research tools common to physical or occupational
the hippocampus, frontal lobe, and hypothalamus. This nu- therapists. Identifying with treatment approaches that base
cleus may act as a filtering system with respect to affect and their philosophy on energy fields, flow patterns of those
certain types of memory, and the dopaminergic projections fields through the body, rhythms that do not seem to be
may modulate the flow of neural activity.56 The masked proven as existing, or planes of consciousness and belief
facies caused by the impaired motor activity seen in clients seems to contradict that comfortable groundedness of basic
with Parkinson disease and the paranoid-schizophrenic science. Thus for many health care practitioners, denial of
all those potential parameters that might affect evaluation neuroplasticity than have yet been identified. Physicians and
and intervention outcomes is an easy way to feel safe and neuroscientists studying the effects of disease and neuro-
linked to what is believed to be efficacy- or evidence-based plasticity after trauma549 or application of drugs552-554 are
practice within respective professions. Most allopathic med- trying to unravel a complex maze of chemical and electrical
ical physicians within the clinical environment are the first reactions at a level of the cell membrane.44 Quantum physi-
to reject what seem like irrational claims or ideas regarding cists are studying the universe and the electromagnetic pull
philosophical approaches. Therapists are not far behind of suns on planets and solar systems on one another.555-557
those physicians with their attitudes and verbal expressions Science is a long way from unraveling the mysteries
toward both patients and colleagues who bring in ideas explored by cellular biologists and quantum physicists and
regarding potential approaches that seem to be outside of how they might relate to each other. But many scientists
our reductionistic, linear research models used to establish trust that there is a relationship. As humans, we are made
efficacy. In the clinical arena, clinicians are realizing that up of billions of these cells; each cell has a membrane
effectiveness of practice with objective outcome measures potential and the ability to adapt and change; and they play
is another way to establish evidence. Similarly, effective- an important role in the existence of our species. Similarly,
ness can be subdivided into variables that pose questions. the universe is made up of billions of masses; each has
Researchers might be able to select variables that can be some relationship to energy pull, whether that be one solar
researched to establish efficacy of treatment approaches system in relation to another, one planet in relationship to a
used within a clinical environment. Western medicine has sun, one moon in relationship to a planet’s oceans, one
taught both medical practitioners and therapists to strongly person in relationship to the gravity on a planet, or one
question anything that reflects concepts of energy, healing, person in relationship to another person. If those cells are
or spiritual beliefs with regard to outcomes of therapy. Yet what makes a person human, and if what holds the person
electromagnetic tools have been embraced by physicians together is electromagnetic energy, then it is hard to ignore
and neuroscience researchers in the form of computed the possibility that one person might affect another person
tomography, MRI, PET, and fMRI to diagnose and study just by being present.553
neurological damage and neuroplasticity. These evaluation Therapists want to study the interaction of brain responses
and research tools create their own electromagnetic field between a practitioner and the client during a therapeutic
while the human body is placed within that field.549,550 treatment session.558 It is obvious that this interaction cannot
Practitioners can still deny that there is a natural energy be explained by one variable within linear space, nor that it
field and that this field has anything to do with health, but it is one variable alone that is causing all change over a linear
is getting harder and harder to deny the presence of such a set in time. Establishing efficacy on what seems to be a mul-
force. All of us have received an electric shock between our tidimensional construct using a basic science research model
body and a metal surface. That shock is called an electro- is not realistic. Thus efficacy research on the totality of the
magnetic charge, and the voltage depends on the inherent mind, the physical body, and the human spirit eludes basic
voltage of that individual. Where did this voltage come scientist researchers in a manner similar to the way that re-
from? What is meant by inherent voltage of an individual? searching the effectiveness of intuition eludes psychiatric
We all have learned that there is a static electromagnetic researchers.
field around us, but it is very hard to identify how that charge As research practitioners we use various tools to manipu-
might affect our body systems. As long as practitioners do late both the internal and external environments within which
not inquire about the physics of these energy fields and the our clients function to measure effectiveness of specific or
bioelectric or biochemical reactions of our human cells to generalized outcomes. Each person is so complex and unique
these fields, the idea that the electromagnetic and electro- that finding the best combination of tools and environments
chemical fields have nothing to do with neuroplasticity and has a very person-specific answer.554,559 Thus what we as
changes with patients after neurological insults unfortu- researchers are trying to do is find evidence that shows that
nately can remain a myth. Over a decade ago, some allo- one treatment paradigm has a better chance of creating
pathic physicians stepped out of their established model change than another, without placing rigid restrictions that
and developed a subspecialty in psychoneuroimmunology. say all persons will optimally benefit from any one particular
Checking PubMed for articles from 2012, a reader can find approach.560-567 MRI, PET, and fMRI tools are certainly
over 1120 published articles under the term psychoneuroim- capable of identifying changes in the CNS after interven-
munology. This subspecialty incorporates the relationships tions. Even when researchers or clinicians try to control as
among emotion, the endocrine and immune systems, and the many variables as possible, many additional external and
CNS and peripheral nervous system.22,113,114,551 As the lim- internal input possibilities exist.
bic neuronetwork intricately links various nuclei that deal This brings us full circle to the question regarding addi-
with emotion, endocrine production, and autoimmunity, tional variables that might affect health, well-being, and
there is little doubt that this system is involved with belief recovery outcomes from therapeutic interventions.568-575
in healing, emotions, and spirituality. “Despite such con- After 30 years of clinical practice and hearing Western
ceptual progress, the biological, psychological, social and physicians say, “Physical therapy and occupational therapy
spiritual components of illness are seldom managed as an just make the patients feel better,” it is obvious, first, that
integrated whole in conventional medical practice.”23 many physicians do not understand the depth and breadth of
Fortunately or unfortunately, there are scientists and our professions or what is provided to the clients. Second,
therapists who are “myth busters” and challenging the rigid those physicians do not understand the limbic interconnec-
paradigm of linear research, stating that there are many more tions to “feel better” and how that might drive the neuroplas-
variables and multiple systems involved in neurorecovery or ticity of the CNS and the autoimmune system’s response to
disease or pathology.575-582 Similarly, after many patients the body and the mind did the healing. Similarly, when the
have been observed over the last 40 years regaining con- drug itself aided in neuroplasticity and change, is it the drug
sciousness, whether the vegetative state lasted 6 months, itself or the individual’s belief that the drug will work that
9 months, a year, or 4 years, the fact remains that each indi- creates the change or both? How these changes occur is yet
vidual shifted from what might be considered a level 2, 3, or to be totally understood, but research substantiates that both
4 on the Rancho Levels of Cognitive Functioning to a 6, 7, neurochemical and neuroelectrical changes occur within an
or 8 on the same scale after 5 to 20 minutes. This reality individual’s physical body when the individual believes that
made me ask the question from the beginning of my profes- change is possible.119,120,570,576,587,590
sional career, “What are the variables that cause changes in When I was a novice therapist, a nurse once said, “I am
these clients?” The answers are not yet fully understood, very glad you are not a nurse because you are so idealistic.
although the behavioral outcomes keep presenting them- You believe these patients in comas are going to wake up
selves. Every time a patient comes out of this vegetative and walk out of here. And what is even worse is that most of
state, I [DAU] feel wondrous, emotional, and humbled. them do!” That moment should have told me that I would be
Something happens that is far beyond our scientific under- clashing with allopathic doctrine throughout my profes-
standing, something simple but extremely complex, cellular sional career, but instead I was confused about the nurse’s
and universal, all at the same time. Similarly, the bond be- use of the term idealistic. If the patients awoke and walked
tween that therapist and that client is very strong and deeply back into life with function and quality, then should that not
spiritual. The memory of those patients stays forever em- be considered a realistic expectation? In that same job situ-
bedded in the mind of the therapist even if the clinical envi- ation, my boss asked me to treat all the patients who were
ronment existed for only 30 minutes. All the words used to considered vegetative; once they were awake, my colleagues
explain such clinical experiences link closely to the limbic would treat them from there. My response was, “Emotion-
network and its role in creating change, both within the ally for both the patient and myself, I could not do that.
therapist and the patient; certainly at this time, these experi- Once I bonded with a person, gained his or her trust, and
ences fall outside the paradigm of Western medical science. found the patient was willing and capable of regaining con-
According to a report on the BBC, the use of appropriate sciousness, I could not just abandon the patient and go on to
fMRIs shows that many individuals in a vegetative state are another person.” The significance of that statement took
awake but still have little to no awareness because of the many years to understand, and it was not until I began my
severe brain injury.583 This explains the fact that a therapist study of the limbic network that I truly comprehended the
“feels” that a patient is aware but does not explain how the accuracy of that perception, once considered na-
therapist-patient interaction brings that client to a conscious ïve.570,578,586,592,593 It was not until the writing of this edition
state of attention. that I could shrug off comments such as “This has nothing
Medical schools and health science programs are becom- to do with physical therapy.”3,22,23,60,594
ing increasingly aware of the need to train the practitioners After 45 years of practice and often treating individuals
of the future to enter into a healing relationship with the in front of colleagues in workshop situations, I cannot deny
whole patient,584 a relationship that empowers the patient to that something more than just “feeling good” occurs during
engage endogenous healing capacities, even while we work physical or occupational therapy interventions although that
to better understand these mechanisms through both basic feeling good is certainly a limbic response. When working
and applied research. Master clinicians have long appreci- with clients, I find myself feeling very open and bonding in
ated this dynamic healing relationship, which affects both some way that is neither “physical” nor “mental”—and thus
the therapist and patient. Thus even when our patients can the only option left is a definition of “spiritual.” If, when
verbally communicate with the therapist, it is still important treating a patient in a vegetative state, that bond tells me that
to listen directly to the body, and on a deeper level, to more the patient is lost within another plane of consciousness and
subtle input that we do not yet have the ability to describe wants to regain consciousness as defined by healthy people,
and quantify with scientific method. It has been demon- and the physical body of the patient seems capable, then the
strated that even for persons in very low awareness and treatment is goal directed, the direction of the intervention is
response states, appropriately selected music can provide identified, and thus the outcome is selected by the patient.
time-organized and emotionally meaningful stimuli to gen- The map has been established, and together the patient and
tly activate intact neural networks, and to communicate with the therapist proceed. As with all therapists, the intervention
the person still alive inside the disabled body.585 The sounds will be guided by the motor responses and control of the
we make and the way we touch communicate to the deeper patient and the window within which the patient can run
levels of being and do not require words to convey caring, those programs independently. At times, when treating cli-
instill hope, and motivate the will to keep trying to get ents in a vegetative state, I feel unable to locate the “spirit”;
better. at other times it feels as if that person has not decided
The success or failure of many forms of alternative medi- whether to venture to an awake state, but more often I sense
cal practice, and for that matter Western allopathic medicine a frightened, confused individual who just wants to find her
and therapeutic practices, may depend on the limbic net- or his way back to what we call “life or reality.” Those pa-
work.557,573,586 At times research can prove unequivocally that tients often gain consciousness during therapy. It is not a
certain variables do not show a healing effect, after double miracle, nor can I ever say, “I healed something.”595 The
blind studies.587 If a patient “believes” an intervention will term healing refers to a concept of “whole.” The only person
work, even if it is a placebo, the chances of success far ex- who can regain the structure of the whole is the patient.596
ceed those when the patient does not think it will work.588-591 As a therapist, I am a teacher or a guide, helping others
If it is a placebo and the body heals, then logic dictates that relearn and regain control over their respective lives. If after
a 30-minute treatment session a person regains feeling and that humans bring to consciousness about 10% of all incom-
control of an extremity 18 years after a CVA or regains func- ing information. Yet the human brain is making decisions
tional use of a hand 6 years after incomplete spinal cord using 100% of the input information. Given that relation-
injury, there is more to the intervention than merely follow- ship, quite a bit of human decision making may be based on
ing a clinical pathway or a treatment regimen geared to nonconscious information regarding the external and inter-
all individuals at a specific stage of a disease process or a nal world.119 Thus the word all neuroscientists shudder
specific motor impairment. over—intuition—may to a large extent be the unraveling
One variable that always seems to be present when of that nonconsciously received data.619 I have effectively
clients achieve dramatic recovery is strong motivation by the taught colleagues how to feel blood pressure and heartbeats
patient to retain the control and an appreciation for the in- of clinical partners by just barely touching the top of the
struction on how to do that. A strong bond or compassionate hand, which might be explained by the high level of sensi-
appreciation for each other always seems to be present as tivity of Meissner corpuscles within our skin.618 If a clini-
another interlocking variable. Thus the clinical question cian can sense an autonomic response such as heart rate
“What is spirituality?” presented itself to me more than when touching a patient’s skin, then knowing how the lim-
30 years ago. It is a variable that is very difficult to define. bic network is interacting within a motor response can also
That variable, when researched, has been shown to affect be deduced. This would allow the clinician to modulate the
health and healing in individuals with health problems. rate used to move the patient during an activity such as bed
Spirituality and healing are both words that each individual mobility, while maintaining a consistent state of the motor
defines according to her or his own beliefs, cultural experi- generators. That steady state should decrease any need for
ences, and use of verbal language.425,426,597-601 The literature limbic fear by the patient. Fear has been shown to be very
is available for those who wish to pursue this topic.360,516,602-609 detrimental to motor performance.620-622 Therapists may in-
Over the last 5 years since the fifth edition was published, terpret these tactile responses as intuitive, but they are not.
thousands of articles dealing with health, healing, spiritual- When one clinician seems to know how fast to move the
ity, energy fields, quality of life, energy medicine, and emo- patient and another clinician has no idea how to determine
tional balance have been published in a large variety of types that decision or control that variable, we say it is the art of
of journals.610,611 Within this chapter a system that affects therapy and not the science. Yet it is the science of therapy.
all areas of the CNS and peripheral function has been dis- Similarly, helping someone shift consciousness levels seems
cussed. How this system is affected by or affects one’s similar to hypnosis. The exact identification of these vari-
spirituality is open for many lifetimes of future study.612,613 ables is very hard, let alone finding reliable and valid re-
Yet if spirituality affects healing and an individual believes search tools. This may just be a case of one clinician being
that this potential is available, then this variable may play open to receiving information and processing it. The other
a critical role in patient compliance, neuroplasticity, and therapist, for some reason, is either not receiving or not pro-
the limbic interface with other treatment procedures.614,615 cessing the available information. This is not an example of
Ignoring this variable is no different from ignoring cognitive “intuition.”
perceptual deficits when dealing with abnormal motor Intuition has been a source of fascination over centuries.
behavior. Owing to the strong emotional foundation for an Recently, with consumer dissatisfaction with health care and
individual’s spirituality, one could easily assume that the the assurgency of alternative medical practices, intuition has
limbic network plays a strong role in establishing and stor- again sparked the interest of scholars and the public. To many
ing memories that reflect these beliefs. it reflects mystery, magic, and even voodoo. Individuals
Until we can measure simultaneous synaptic activity of with a strong ethnic, cultural, and even religious bias may
all interactions within the therapist’s and the client’s CNS, find it hard to scientifically analyze this human strategy. For
we will not, from a grounded neuroscience efficacy base, be more than 35 years my husband has answered questions I
able to demonstrate exactly what occupational, speech, have posed in my mind. It took at least the first decade for
music, cognitive, or physical therapists do, even though we my left brain to actually accept that I was not subvocalizing
know they play a role.616 Until then, outcomes need to be the thought or that he could not have extrapolated the
measured objectively. Even if interactions seem unmeasur- thought from an environmental stimulus. Yet he consistently
able and subjective, clinicians still need to record the event has told me he hears me ask the question or state a fact.
change in the patient record and not bury that outcome deep Obviously, my thoughts have traveled to the primary and
somewhere in the subconscious level of the therapist’s associative receiving areas of his left temporal lobe and he
mind. The mind, the body, and the spirit are connected as a “hears” the thought in my voice. The dilemma that con-
whole. If therapists treat only one part, it may help the fronted me as a scientist is, if the information was not input
whole, but if the whole is treated simultaneously, the out- through his eighth cranial nerve, how did it enter into his
come is more likely to change the whole.617,618 The concept system? The answer would seem to be intuition. A definition
is no different from focusing on strengthening an isolated might be knowing something without entering the data
muscle and hoping it will lead to functional use versus through traditional input systems. The next question is
strengthening that muscle in functional patterns and in rela- “What is intuition?”
tion to other muscles that also work together within that Unfortunately, after 35 years of study, I cannot answer
movement sequence. that question. I do acknowledge that it is something, it can
After years of clinical experience and thousands of be learned, and master clinicians use it as a part of their
patients responding positively to various interventions, the clinical decision making, even if they choose not to verbal-
question arises regarding clinical decision making and ize it to their colleagues or even acknowledge it within their
choice of interventions. There is not a “variable” that has conscious mind. Much research and literature are available
been identified that guides that decision. It has been shown regarding intuition.119,623-646 Yet the answer to that simple
question “What is intuition?” is unavailable and does not create the first questions that lead to hypotheses and later to
seem so simple. No answer exists that has shown to be research that establishes efficacy.
definitively efficacious and reliable, although research over Until 40 years ago, I [DAU] hid from most people that
the last 10 years has begun to identify components of intu- aspect of my person because I was becoming a neuroscien-
ition.623-642 It may be that intuition is more than one variable tist and wanted to be grounded in scientific efficacy like all
and can be accessed in more than one way. In fact, after my colleagues. Unfortunately, my clinical experiences did
studying various alternative medical practices, all using very not allow me to hide that intuitive aspect of my clinical deci-
different interventions based on different philosophies and sion making from my family or close colleagues or those
belief systems, it seems as if all approaches may be tapping colleagues who recognized that something had happened
into the same human system, just opening to that system during a treatment that made no sense whatsoever. Those
through different paradigms. individuals recognized changes within the patient that,
In the late 1960s, I [DAU] was beginning to present an although very positive, should not have happened or were
integrated approach to neurological disabilities and integrat- very far from our basic scientific understanding. I treated
ing various treatment philosophies using the behavioral re- a woman who had a severe head injury and who after
sponses of patients and known science to guide intervention. 6 months was at a Rancho level III. After 30 minutes of in-
I was told at that time that integrating approaches could not tervention the woman volitionally moved all of her limbs
be done and that I would potentially injure patients by using and trunk without cognitive confusion. That motor function
approaches from different philosophical techniques. Today, and cognition might be partially explained by recent re-
of course, with our understanding of motor control, motor search using fMRI.583 But something that goes far beyond
learning, and neuroplasticity, an integrated approach from today’s fMRI followed the intervention. I innocently stepped
the 1960s based on a systems model is what we do. I now out of the safety of scientific understanding. I shared with
present the same model when looking at complementary my colleagues this woman’s medical and social history. That
approaches to intervention and the concept of intuition. information was critical to their understanding the course of
There are a number of variables that seem to open one’s progression of this woman through the rehabilitation pro-
intuition: bonding, being dedicated to the patient, having cess. I discussed the patient’s social background, her educa-
openness in listening to the patient, letting preconceived tion, her family, her children, and her husband, who had shot
knowledge be a springboard from which to expand that her in the head. This all made perfect sense, until the head
knowledge, having not only a willingness to learn but an of the department asked me how I knew that information.
insatiable appetite to continue learning, and possessing the I said, “I read it in the chart.” The director informed me that
ability to let go of one’s importance and just be another I had not seen the chart. I said, “You told me?” The director
person within the environment. These variables may be the responded with, “We did not discuss the case!” I asked if
best place for a learner to begin learning how to develop this I had been wrong, and the director said “no.” In fact, she was
skill. It would seem as if intuition is like an aptitude. Some amazed at how accurate I had been and just wondered how
individuals come into this life already with a high level of I had known that about the patient.
potential, others are nurtured to develop that potential, and At that moment, my life was changed. I could no longer
still others never have an opportunity or an environment in hide whatever this “intuition” was, nor could I truthfully tell
which to develop those strategies. Some individuals have colleagues what I had done during interventions without
had strong intuitive senses from childhood but share those bringing up this topic. Also, I could only tell them ways to
experiences with few, if any, other people. Experiencing in- develop intuition but had no understanding of the basic neu-
tuition is an all-knowing experience. One knows something roscience behind its function. I could not tell anyone exactly
first, then one becomes emotional regarding that knowledge. what it was because I did not know. That unknown is still
It is a knowledge that has a “wholeness” component and present. although some of the variables may have been identi-
then has a strong emotional base. For example, I knew I was fied. The future will unravel those answers. What I have found
going to lose a parent. Which parent, I did not know, but since that day is that “masters,” whether they are physicians,
I moved home for a year to make sure there wasn’t anything therapists, or teachers, often use this additional source of
I should have said to either parent before I went on with my information gathering to help them in their clinical reason-
life. A year later I was married and home for a holiday. ing. I do not make this statement lightly nor without tremen-
When I left I cried all the way back to my and my husband’s dous professional risk. I will leave you with an interaction
dwelling across the country because I knew I would never that solidified my belief that this direction of scientific study
see my father again. I was right; he passed 2 weeks before needs to be pursued. Two decades ago, I was a keynote
I was to return home. My father had been a very healthy man speaker at an international neurosurgical conference on
with no health issue that would indicate any life-threatening brain tumors. I was the token “other,” and the only speaker
health problems. I had known what was going to happen as who was not a neurosurgeon. I presented the topic “The
a whole (intuition) and then had had a very strong emotional Limbic System’s Influence on Motor Output.” With this
response to that intuition. Also, when my brother called to audience of 500 neurosurgeons and 50 token others, I, of
say that our father was critically ill, I had already adjusted course, used charts and pictures and based every sentence on
to the probability of his loss and was the one individual efficacy-based scientific research. At dinner that night when
within the family who could make cognitive decisions or all the speakers were together, the master neurosurgeon
answer press questions by phone. If I were to hazard a guess, whom everyone acknowledged asked if he could sit next to
the intuitive center is probably in the right anterior temporal me. I was aghast—a little nervous but honored nonetheless.
lobe owing to the “whole” understanding and its strong He opened by saying, “I think many physical and occupa-
emotional connection. If that proves true, it will solidify the tional therapists are intuitive.” With that, I knew him, his
limbic network’s connection to intuition. Experiences often life, his experiences, and so on. I let my left brain validate
my intuition and said, “Yes, it is like walking into a room, Some individuals believe that spirit means belonging to a
looking at a patient, knowing where and what type of tumor religious order. Others define spirit or spirituality as beyond
he has, and using instruments such as PET studies to vali- religion—the essence that links the person to a greater energy
date what you already know!” He responded with a smile force. For decades this last category has been considered
and said, “Yes, it is exactly like that!” I do not need to con- outside the domain of responsibilities of Western allopathic
tinue to discuss the fascinating interactions of that night but health care delivery and was comfortably relegated to reli-
leave the reader with the thought that even the master of the gious leaders or spiritual guides.
masters in neurosurgery uses intuition as a variable in clini- Today, everything is changing. Some scientists refer to
cal decision making, and it gave this man one additional bit energy fields around cells; others talk about energy fields
of information that his colleagues could not use in clinical around solar systems. Complementary practitioners talk
reasoning. No physician or therapist uses intuition as the about energy fields around the living organisms. Physicians
only variable; intuition just gives additional information that are being taught cultural sensitivity training while in medi-
helps in the process of clinical reasoning. It would seem as cal school to be more empathetic to the populations of
though intuition is highly integrated into the limbic network. people they will service. Physical therapy curricula are
As said previously, intuition is knowing something and as responsible for creating culturally sensitive professionals.653
a result experiencing great emotion, such as “I know, thus Occupational therapy programs are responsible for includ-
I fear.” If the sequence of events begins with an emotion or ing spirituality as one of the competencies a graduate is to
fear and leads to what is perceived as knowledge or truth, one have met.654 None of these professions has identified how
might question whether intuition was the driving force behind these competencies relate to evaluation and intervention
the belief. When emotions become elevated an individual outcomes after treatment, but even the accrediting bodies
may progress with “I fear, thus I think I know.” Research believe they are important. Thus even at the entry level, for
that looks at intuition assumes that when the limbic network student therapists, emphasis is placed on making sure not
is involved, the experience is highly emotional, and one only that the therapists’ limbic systems have become sensi-
might argue that an individual can be highly emotionally tive to spirituality, but also that they be able to identify its
charged and still be neutral as far as a balance in emotions. significance in their clients’ lives. Where does the interaction
Fear is not what drives intuition; instead it is emotional bal- of the mind, body, and spirit play a critical role in quality-
ance. Emotional balance or centering is not a state of being of-life issues and empowerment of the patient? The answers
without emotion but rather a heightened state of emotional to that question cannot be found within this text or any other
awareness without emotion, all at the same time. To become text in print today. Individuals with strong beliefs in a spe-
truly intuitive, one needs to become emotionally centered. In cific paradigm that includes spirituality can project the an-
our everyday world where each of us is overstimulated as a swer to this question, but establishing efficacy is an entirely
day-to-day experience, this emotional balance is extremely different issue. Our professions are tethered to research, sci-
difficult to achieve. It is even harder to find that balance in a ence, behavioral observations, and current knowledge. We
clinical arena where patients are arriving with more acute as clinicians can stretch that tether. Much of our early treat-
diseases along with chronic secondary problems, often ments developed from behavioral observations that included
patients’ schedules overlap with other patients’ time, and individuals’ beliefs that clearly required the limbic network
therapists not only are limited with time for intervention but for processing, storage, and direct effect on bodily system
also find that the number of allowed visits falls well short for reactions. If a patient lacks motivation, a therapist knows
optimal opportunity for learning by the patient. That reality part of the job is to motivate the person. If the person
does not mean that the therapists’ responsibility has changed. believes his “God” will heal him, the therapist should never
It is always up to the therapist to find those avenues by undermine that belief because everyone knows it cannot hurt
which better care may be provided within the existing envi- and often creates a positive change.655 How that interaction
ronment. This reality just says that the challenges and ques- occurs is unknown today, but clinical observation would
tions are enormous. Finding emotional balance within that reinforce that it does help. Because spirituality uses belief
environment is very hard. Yet intuition seems to be a vari- and hope, memory of those feelings must be processed and
able that gives some colleagues additional information that later stored with the help of the limbic network.
is then used as part of the clinical reasoning process. These dilemmas exist with every professional dealing
Intuition as a variable needs to be identified, studied, with health and wellness and quality-of-life issues. I [DAU]
researched, and taught once it is clearly understood. It is up to will leave you with one additional example. I spent over
all of us to find the answers to these questions and the solu- 2 months in the ICU a few years ago after a severe fall that
tions to today’s clinical problems and develop evidence-based caused two fractures to the pelvis, followed by severe inter-
practice to progress into the twenty-first century.647,648 nal hemorrhages. To summate the medical problems, I had
The concept of integration of mind, body, and spirit as a 18 initial arterial ruptures treated with radiological interven-
critical element in maintaining or regaining quality of life tional surgery, followed by four more ruptures 1 week later
between birth and death is not new.649-652 Western society leading to more surgery. I also had bilateral kidney failure, a
has tried to separate this concept into three distinct catego- large pulmonary embolism on the right side, pulmonary col-
ries. The mind is made up of perception, cognition, and lapse in the left inferior lobe, massive internal infections,
emotion. The body is made up of all systems external to the infusion of 12 units of blood, thrombophebitis, fevers of
nervous system such as peripheral organs, muscles, bones, over 105° F, very low blood pressure, and low oxygen ab-
and skin. Both the peripheral and central motor systems, sorption, along with external bleeds through most external
which control the body, are also included in the concept of orifices. In addition, the two bleeds destroyed my adrenal
body. The last component, the spirit, is a transcendental glands bilaterally, throwing me into another life-threatening
concept and is thought to depend on individuals’ beliefs. imbalance of chemistry within my body. The doctor kept
telling my husband with confidence that I would die. As my defeated, which is a true limbic emotion. Thus this chapter
husband had been told this many times before, he kept tell- has been presented in three parts. The first part introduces
ing the doctor he would wait for 3 days after I had been the system and its potential clinical application. This sec-
declared dead before he would accept that conclusion. Two tion, in and of itself, has many interwoven components, for
weeks after the initial hospitalization, the doctor came into nothing in the limbic network functions in isolation. Yet the
the room. He shut the door, sat down in a chair, addressed mysteries of this complex neurological network, when iden-
both of us and asked, “I know what medical problems you tified, may hold the answers to many clinical questions
have had, I know that we did everything medically that we regarding the art and gift of a master clinician. The second
could, but it was not enough, so how come you are still part introduces in more detail the basic anatomy and physi-
alive?” I responded with, “There is a lot more to healing ology of the limbic network. It is hoped that once the student
than what we understand, and that is what is fun about being or clinician has been drawn to the conclusion that this sys-
a health professional.” Life has taught me the lesson, tem may be a key to clinical success, she or he might be
whether as an intuitive, as a neuroscientist, or as a therapist, willing to delve into the science of the system. This path of
that there will be unknowns or mysteries along one’s life exploration is challenging, difficult, and frustrating at times
journey. Sometimes one can solve the problems or answer but certainly worth the effort once understanding has been
the questions, but more often than not one just has to file achieved. The last section opens up the minds of the readers
them in memory with the hope that one will sometime find when and if they so choose to address these unknown vari-
an answer. The unknowns are always present even as an- ables. The limbic network is very complex, is very interac-
swers are discovered. Having those unknowns creates an tive with all parts of the human body, and may hold many
exciting challenge and adventure for every clinician who has answers about patients’ responses and recovery. The reader’s
or will have the opportunity to interact with individuals who journey has just begun, and the future will open up many
have been brought into the health care delivery system be- more avenues of research and clinical study as well as many
cause of a CNS problem. Those individuals want to be con- more questions.
sidered as a whole human being even if part of their physical
body is dysfunctional. A circle has been drawn, and this References
chapter needs to end with a question. What is that whole? To enhance this text and add value for the reader, all refer-
Refer to Case Study 5-1 as a clinical example. ences are included on the companion Evolve site that
accompanies this textbook. This online service will, when
SUMMARY available, provide a link for the reader to a Medline abstract
The complexity and interwoven neurological arrangement for the article cited. There are 659 cited references and other
of the limbic network may seem overwhelming. A reader general references for this chapter, with the majority of
who tries to grasp all parts on first study will feel lost and those articles being evidence-based citations.
CASE STUDY 5-1

A 25-year-old first-grade teacher with a history of whiplash has patient’s goal is to eliminate the dizziness and imbalance and
been referred by the neurology department 5 months after a return to normal activity and work.
motor vehicle accident with complaints of severe dizziness and PHASE I: EVALUATION
imbalance. She is unable to recall the accident; however, there Unaware of being observed, the patient walks into physical ther-
was evidence to suggest that she struck her head on the steering apy extremely slowly, holding the wall, watching the ground,
wheel and briefly lost consciousness. Results of diagnostic testing and stopping periodically to close her eyes. Her color is pale,
(MRI, electroencephalography) are inconclusive. Medical man- her build small and thin, and her clothing loose. She is, however,
agement to date has been limited to central depressant medica- well groomed. Her steps are shortened in length, widened in
tions (alprazolam [Xanax], diazepam [Valium]; see Chapter 36). width, and limited in swing time. She demonstrates no segmental
She has received physical therapy since the accident for neck and movement of the head or trunk, walking en bloc (rigidly) with-
back pain, which exacerbated her symptoms. She denies specific out arm swing. As she sits down to begin the evaluation session,
assessment or treatment of her dizziness or imbalance until this she smiles. She periodically closes her eyes as people move
time. Her medical history includes a hospitalization 3 months around her. There is visible extraneous eye movement, although
after the motor vehicle accident with “intractable migraine, no immediate visualization of nystagmus (eyes opened or
postconcussive syndrome.” Of note is a previous head injury closed). She is pleasant and cooperative with no overt signs of
2 years before with moderate to severe postconcussive syndrome, anxiety in quiet sitting.
including vertigo and migraines. She has been referred for psy- System Impairment
chological assessment and management and was recently diag- n Dizziness with severe nausea and vomiting (at least once
nosed with obsessive-compulsive disorder. She is now referred weekly)
to physical therapy for a full postural control and vestibular n Dizziness Visual Analog Scale 7.5/10 and Dysequilibrium
assessment. The differential medical diagnosis is postconcussive Visual Analog Scale 7.0/10 (with 10 representing the most
syndrome, rule out aphysiological performance (psychogenic, severe symptoms imaginable)
secondary gain). The physician believes that a large part of her n Decreased concentration and memory; forgetfulness
problem is based within the medical psychiatric domain, but he is n Visual diploplia and blurriness with visual headaches
willing to widen his paradigm to include other possibilities and n Depression of central vestibular function (treated with
obtain additional data to assist in his patient’s management. The medication)
Continued
CASE STUDY 5-1—cont’d

n Decreasing body mass as observed by loose clothing and Balance Stability
supported by reports of severe nausea n SOT of sensory balance function658 showed an across-
n Emotional stability: fight or flight; in both active F2ARV the-board dysfunction pattern,14 although results were
and active GAS (autonomic) states: incomplete because the patient was not able to complete
n She reported that she was capable of rapid change from all 18 trials of the test protocol as a result of extreme
a state of calm to fits of rage with her family and other symptoms (nausea, respiratory, and anxiety symptoms,
support systems. particularly on conditions 2, 3, 5, and 6).
n Anger and rage alternated with reports of depression and n Total dependence (overreliance) on visual information for
avoidance. balance stability.
n She reported photophobia with hypersensitivity to light n Center of gravity position shift significantly leftward and
and sound. anterior of midline.
Activity: Client Report n Excessive use of a hip strategy for basic equilibrium (versus
n Impaired balance for function, with near falls in dark and ankle), even with stable surfaces or the smallest perturbation.
eyes-closed environments. Patient reports one true fall in n Inability to effectively:
the shower with her eyes closed. n Use somatosensory or vestibular sensory cues on
n Impaired balance within visually challenging environments. functional demand (reweight)
n Sleep deprivation and extreme fatigue. n Organize the sensory inputs to the CNS to facilitate
n Long-term stress and sensory intolerance and overstimulation appropriate motor output
It is important for a clinician to remember that the long-term n Dampen ANS/vegetative response, particularly in visual-
stress associated with a chronic disability of this nature can result vestibular mismatch conditions (SOT 3 and 6)
in a decrease in serotonin, which influences the hypothalamus n Aphysiological criteria—Aphysiological responses on SOT
and modulation of the vestibular nuclei through the DRN. Loss of raw data traces (exaggerated sway frequency and lateral
sleep can alter levels of serotonin and other neurotransmitters. A sway responses). Motor Control Test (of automatic motor
decrease in serotonin results in further depression and loss of responses) results would have strengthened conclusions made
sleep, resulting in a physiological fatigue. It also can result in an regarding an aphysiological component but were unavailable
increase in sensitization of presynaptic terminals of the cutaneous to this clinician at the time of the examination.658
sensory nerves, contributing to the sensory bombardment and Function and Gait
overstimulation.656 Self-selected velocity of 1.82 ft/sec (normal preferred gait
Participation in Life (Client Report) speeds in a 20- to 30-year-old woman should be closer to
n She attempted to return to work as a first-grade teacher but 3.47 ft/sec).14 The patient watched the floor for the entire
had a severe exacerbation of all symptoms in the classroom. distance with no head or trunk or arm movement. Thus vision
n She is unable to drive and requires assistance for shopping. was clearly directing each step and was adjusted to decrease
n She lives alone in an apartment and is independent in extraneous visual flow or input. When she was encouraged to
function, modified by her symptoms. focus on a distant object, velocity declined to 1.34 ft/sec and
n Disability Rating is 4⁄5 (recent severe disability, medical the patient veered consistently leftward 100% of the distance.
leave).657 She could be encouraged to walk at 2.86 ft/sec (normal encour-
n Dizziness Handicap Inventory (DHI) score is significant for aged gait speeds should approach 6.43 ft/sec), with an increase
physical and emotional impact of her dizziness, including in instability and a leftward loss of balance, not requiring
depression. (Total disability score is 78⁄100, functional assistance to regain necessitating assistance.
subscore 28⁄32, emotional subscore 30⁄40, and physical The interactions of the patient and therapist (limbic bonding,
subscore 20⁄28.390) as referred to previously within this chapter) became a critical
Based on the signs and symptoms obtained in the intake phase element in the examination. The patient had to trust the thera-
and subjective reporting, the preliminary physical therapy hypoth- pist that if she followed the therapist’s direction in examination,
esis would be the presence of a probable vestibular dysfunction she would not fall or incur additional symptoms outside her
of a mixed central and peripheral cause, a sensory integration control. The therapist, during the evaluation, empowered the
dysfunction, and anxiety overlay. The patient clearly has limbic patient to take responsibility for her functional movement while
network overload. It will be the therapist’s responsibility to making sure the patient was successful if willing to take the
differentiate that from physical motor system problems with the risk. This aspect of the therapeutic interaction is a limbic-
assessment and treatment. The examination phase is designed to neutral technique, and its success or failure will be reflected
confirm or refute and redirect this hypothesis. (See Chapter 22b in the motor responses of the patient.
for clarification of these specific vestibular tests and measures.) Confirmatory Tests and Measures
Oculomotor n Intact sensation but extreme hypersensitivity to vibratory
Oculomotor examination was performed, with results support- input (with strong ANS response)
ive of the hypothesis of vestibular involvement, although in- n Normal strength and range of motion
conclusive for peripheral versus central versus combination. Multiple rests were required throughout the examination to
Gaze instability—Clinical dynamic visual acuity test decrease symptomatology (nausea, increased respiration, sweating)
revealed a significant five- to six-line deterioration in dynamic to patient tolerance. Testing reproduced all subjective dizziness,
visual acuity when head was moving, with loss of postural and there was a resultant gross instability with loss of balance in
control in posterior-left direction and symptom exacerbation the posterior direction, necessitating assistance. Imbalance, nausea,
during testing. and anxiety were residual for 10 minutes after testing.

In the evaluative phase, the working hypothesis after the n The correct patient-therapist pairing for effective
examination phase was as follows: execution of the plan. Safe clinician contact may
n Diagnoses as identified by therapist: actually be part of the rehabilitation plan, with
n Medical—mixed central and peripheral vestibular pre- gradual reduction based on limbic and functional
sentation without confirmatory medical diagnostics or improvements.
diagnosis. Further medical workup required. n The correct environment to effect change.
n PT Rehabilitation291—Primary Problem: Practice Pattern Use appropriate voice (timber, pitch, volume),
5D: Acquired impairment of the central nervous system; appropriate pacing (onset of sound or other
Secondary Problem: Practice Pattern 5A: Primary stimuli), sound, light, consistency, and
prevention of falls predictability.
n Characterized by (1) SMD with VOR impairment, b. Balance retraining using sensory reorganization
probable high gain, with gaze instability, (2) central (reweighting).
processing impairment, (3) postural control impairment Gaze stability (VOR) retraining
with somatosensory dependence, and (4) limbic high Goal: Appropriate timing and predictability of sensory
state with autonomic response to testing procedures and treatment: “dose” to achieve desired limbic and
frequent rests required. functional outcomes.
n Prognosis: Fair for modified community level indepen- n Expose the patient to the problem sensory condi-
dence, physiologically complicated by history and chro- tions identified during the examination, present-
nicity. Improvement intratrial is a positive physiological ing first the easier conditions and progressing the
sign. However, anxiety overlay and history may have difficulty and complexity on the basis of patient
psychogenic versus physiological impact on recovery. response. Force the development of sensory inte-
n Red Flags: Watch for (1) additional central signs, (2) gration, compensation, or substitution, as well as
signs of secondary gain, (3) F2ARV and GAS limbic the development of new and appropriate move-
cascade, or (3) social service issues. ment strategies.
PHASE II: INTERVENTION n Provide for selective attention through predict-
1. There are three objectives of the treatment phase driven by able, short segments of sensory integrative
findings in the clinical assessment: challenge.
a. Monitor and manage limbic symptoms through environ- n Avoid sensory overload through proper exercise
ment change of input systems (vision, auditory, kines- prescription (intensity, duration, repetitions,
thetic) with the goal of the limbic network going neutral frequency) during sensory integrative treatment.
if possible. n Provide for maximal motor learning environment
b. Maximize sensory integration, central processing, com- by keeping the limbic network quiet while achiev-
pensatory impairments of the vestibulospinal reflex ing the correct balance between error detection and
(balance control) and VOR (gaze control). correction versus demotivation through making
c. Integrate gains in balance and gaze control into functional mistakes.
activity with and without emotional overlays. n Provide knowledge of performance and knowledge
2. Treatment approach: of results frequently. As one example, computerized
a. Patient-oriented (limbic) approach. visual biofeedback provides direct one-on-one
Goal: Maximize internal locus of control and trust; quiet feedback of body position in space and motor
the limbic influence (facilitate limbic neutral) to set performance.
an environment appropriate for motor learning and c. Functional activity requires complex integration of
functional change. balance and gaze control.
Techniques: n Gait training at controlled pacing in stable environ-
n Awareness and validation of the problem. Provide ments, progressing to changing pace within predictable
the patient with objective findings of organic and visual environments, to unstable visual environments
functional involvement (sensory organization, dy- and variable surface environment.
namic visual acuity, and other testing). Many of n Hippotherapy (an activity meaningful to the patient),
these patients have been told for years that it is at a controlled cadence provided by the animal,
“in their head.” The statement is accurate but the provides predictable sensory input (somatosensory,
intent is condescending and implies some psycho- vestibular, auditory, and olfactory), controlled rhyth-
logical dysfunction. mic visual flow, and neutral warmth in a meaningful,
n Awareness of and participation in the plan and goal-directed activity (as identified by the patient).
approach. The treatment plan should have strong This can be a very limbic-neutralizing activity when
emotional meaning to the patient to turn the fear is controlled as a factor.
“limbic key” to maximize involvement and moti- d. If the patient does not progress in a physiologically
vation. It should be goal directed intrasession normal manner or limbic signs remain unchanged (or
and intersession. Achieving proper motivation increase) then psychological management may take
and reward maximizes neuroplastic change.659 precedent over (or be required before) recovery in
Continued

rehabilitation (motor learning and neuroplasticity practice, and (5) allow the patient to improve at a pace her
within the CNS) can be achieved. CNS could manage.
n Successful life outcome is affected by early manage- After 4 months of intervention the therapist moved and thus did
ment. The extent to which a mild dizziness problem not follow up on the long-term outcome of this case. Although the
becomes chronic is dependent mainly on the psychotherapist does not know whether the patient reached her ultimate
logical reaction to the symptoms.233 goals, it was obvious after 4 months that the client was changing in
n There are specific management strategies associated the direction of functional control and participation in life.
with anxiety-type disorders within psychology, includ- The long-term permanent changes that may or may not have
ing use of medications. One theory of recovery from occurred with this patient’s vestibular, motor, or limbic net-
psychology problems is referred to as exposure. works are not known. The essential role of complete history
Although exposure is meant to cause habituation of the taking and dedication to reality by the therapist is obvious. The
patient to the triggering events, in our case exposure therapist’s success within this case was dependent on her abil-
actually will lead to forced use of the appropriate sen- ity to listen and watch (visually, auditorily, and emotionally
sory system(s) as required in activities of daily living. [limbic]) as the patient unfolded the mystery of her CNS prob-
CONCLUSION lems. The patient was the key to successfully unlocking her
What makes this a clinical problem within the domain of the complex subsystem problems. In the health care world of
limbic network is that this woman’s limbic network was stress, limited visits, and expected outcomes after intervention,
overriding all other systems. At first glance this person was it is far too easy to blame the patient for our failure as clini-
referred to therapy with typical vestibular and balance dys- cians. It is also easy to quickly identify that the patient has
function. She was anything but typical and could not be problems in other system areas outside our scope of practice
approached with a “standard protocol,” or failure for both and thus infer that it is those areas that are limiting improve-
the therapist and the patient was inevitable. The role of the ment. The difficulty is that all professions are doing the same
patient within this setting was to gain an appreciation and thing, and the patient is drowning in the repercussions of the
integration of how her vestibular, motor, and limbic networks waves. Partnering interventions both with other professionals
were interacting and when she went into system overload and with the client should optimize an environment that nur-
and why. The therapist’s role was to (1) help the patient gain tures long-term learning and plastic changes within the CNS.
this body awareness, (2) empower the patient with regard The limbic network drives our attention, our motivation, and
to her potential for recovery, (3) design interventions that our willingness to take risks into unknown environments. How
would nurture patient success, (4) collaborate with the you as a clinician accompany those patients throughout the
patient on needed interventions regarding practice and learning experience will depend on your limbic network as
novelty within the environment along with consistency of much as theirs.
CHAPTER 6 Psychosocial Aspects of Adaptation
and Adjustment during Various Phases
of Neurological Disability
ROCHELLE McLAUGHLIN, MS, OTR/L, MBSR, and GORDON U. BURTON, PhD, OTR/L

adaptation After reading this chapter the student or therapist will be able to:
adjustment 1. Describe adaptation and adjustment as parts of a flexible and flowing process, not as
bonding static stages.
coping 2. Describe elements of the grief process that deal with age, cognition, and developmental
family network level.
loss and grief 3. Integrate the elements of mindfulness, problem solving, loss, cognitive functioning, and
mindfulness coping, as well as significant others’ coping and learning styles, into the treatment
problem solving process to encourage adaptation.
sexuality 4. Integrate the family of the client and the client’s styles of coping into therapeutic
support systems treatment strategies to be used in the clinic.
5. Respect aspects of sexuality in treatment and consider them when treating the client.
6. Accept the role of patient advocate and the responsibility to report any abuse. Each
clinician is responsible for knowing state law as part of her or his state licensure
requirements.
I t is a part of the human condition to have intense life

experiences. There are a variety of ways in which we can
respond and adjust to these kinds of life experiences. Ad-
empowerment of the client and the support system. In this
chapter we will pursue topics that cover important aspects
of the adjustment process for the individual with a disabil-
justing to a disability is an ongoing process, just as adjust- ity and his or her support system.
ing to all other aspects of life is for everyone. This process
of moving forward is a lifelong one. In moving forward PSYCHOLOGICAL ADJUSTMENT
with a disability it is important to turn toward and confront In clinical practice, theoretical foundations for adjustment to
the situation, thereby opening up to the potential hidden disability appear to be elusive because they represent a fluid
within the situation. If we turn away and deny the situation, process: all people are constantly changing. This is espe-
we are at risk of never fully coming to terms with and ad- cially true for people who have recently become physically
justing to the life experience. Adjusting to a disability is not disabled. They do not reach a certain state of adjustment and
unilateral. The family and support system must be involved stay there but progress through a series of adaptations.
in this process. Therapists may be tempted to treat the im- Therapists see clients in a crisis state1-6 and therefore iden-
pairment in isolation and not be involved in the adjustment tify their adjustment patterns from this frame of reference.
process for the individual or their support system. This How well the individual adjusts to the crisis, however, does
would be a major mistake. Technicians address the me- not necessarily indicate how well he or she will adjust to
chanical (technical) aspects of treatment, but clinical pro- all aspects of the disability, or the rate of progress from
fessionals must treat the whole person and must be involved one point of adaptation to another.6-15 Disabilities are an
in the process of adjustment at all times. It is a fatal flaw to unimaginable insult to an individual’s self-perception.16-19
reduce the individual to just the impairment and not see or A month or even a year after the injury may not be long
try to understand the bigger picture with regard to what is enough to put the disability into perspective.10,16,17,19-22
really needed during treatment intervention. A technician For most people, progressing from the shock of injury
may obtain good physical results, but if the individual has to the acceptance of and later adaptation to disability is a
not adapted to the life-altering event, the physical results process filled with psychological ups and downs. Several
may never be maximized. If the individual’s support system authors have discussed the possible stages of adjustment
has not adjusted to the impairment, those individuals may and grieving.10,14 The research of Kübler-Ross23 into death
hold the client back from optimal functioning or put un- and dying has application to this topic of adjustment to dis-
necessary pressure on the individual simply out of a lack of ability. She discussed the concept of loss and grief in rela-
knowledge. Proper training and practice can allow for the tion to life; loss of function may result in just as profound
141
a reaction. The practice of mindfulness may be important in If we haven’t endured great suffering personally, we have
disengaging individuals from automatic thoughts, habits, borne witness to it—“9/11” is a perfect example of this. If
and unhealthy behavior patterns and thus could play a key we acknowledge this fact, then maybe we can acknowledge
role in fostering informed and self-endorsed behavioral regu- that we are more connected to our clients than we once
lation, and adjustment to catastrophic life events.24 Peretz25 thought and that we have more to offer our clients in terms
and others26-28 discuss the grieving process in relation to the of their ability to adjust to their disability than we once
loss of role function as well as loss of body function. These imagined.
losses must be grieved for before the client can fully benefit
from therapy or adjust to a changed body and lifestyle. AWARENESS OF PSYCHOLOGICAL
Therapists must be aware that the client can and must deal ADJUSTMENT IN THE CLINIC,
with the death of certain functional abilities. SOCIETY, AND CULTURE
Some authors have questioned the concept of stages of Working with individuals with functional limitations re-
adjustment,1,29 and call for more empirical research into quires that we cultivate a holistic and all-encompassing
adaptation and adjustment; this has been started.30 One alter- perspective: to visualize how they might best participate
native concept that has been developed is cognitive adapta- within their own homes and communities, and in the context
tion theory.31 This concept examines self-esteem, optimism, of their society and a given time. This is a dynamic and
and control. In this theory, if the individual feels good about constantly changing process. The clinician must develop an
himself or herself and has an optimistic view of life and a intervention that will appropriately stimulate the individual
sense of control over life, the individual will adapt to the and all their potential caregivers to maximize the potential
functional limitations and will participate in life. Cognitive for the highest-quality life possible. The skilled clinician
adaptation theory does not consider the organic changes that initially evaluates the individual’s physical and cognitive
may take place when brain damage has occurred, but the capabilities depending on the type of functional limitations.
basic goals are very much worth taking into consideration. The more subtle psychological aspects of the client’s ability
These should be examined in relation to the limbic system to function need to be assessed at some level. These include
(see Chapter 5) because limbic involvement is crucial to the individual’s support system and/or family network and
reaching all goals and plays a key role in establishment of its ability to adjust to the imminent changes in lifestyle. It
motivation. would be a tragic situation for a clinician to ignore the indi-
The components of successful psychological adjustment vidual’s psychological adjustment or consider it to be less
to a physical disability (activity limitations) are varied. To important in any way.19,33-36
bring a client to a level of function that is of the highest qual- Livneh and Antonak37 have introduced a consolidated
ity possible for that individual, therapists must look holisti- way to look at adaptation as a primer for counselors, which
cally at the psychosocial aspects and at the adjustment pro- should be examined by therapists. They use some of the
cesses involved, evaluate each component, and integrate the same basic concepts, such as stress, crisis, loss and grief,
processes into the therapeutic milieu to promote growth in all body image, self-concept, stigma, uncertainty or unpredict-
areas. There is much more to evaluation and treatment than ability, and quality of life, to frame their approach. They also
just the physical component; the mind and body have incred- consider the concepts of shock, anxiety, denial, depression,
ibly interrelated influences, and both must be understood, anger and hostility, and adjustment in a format that is usable
evaluated, and treated individually and as a whole. by the therapist.
Livneh and Antonak37 mention that one of the aspects
WE UNDERSTAND MORE ABOUT that the therapist must watch out for is a form of coping
SUFFERING THAN WE THINK called disengagement. This style of coping may be dem-
Clinical professionals have a wellspring of knowledge to onstrated through denial or avoidance behavior that can
draw from beyond their extensive traditional education. We take many forms. It can result in substance abuse, blame,
are all human beings, and being human comes with a great or just refusal to interact. Research regarding people with
deal of innate suffering. If we bring awareness to the fact head injuries has demonstrated that if a premorbid coping
that we have all suffered in our lives, we may not feel so style for a person was to use alcohol or other drugs, the
separate from our clients. We may realize that we have more client may revert to these same styles of coping, which
to offer our clients than just the knowledge we have gained can result in poor physical and emotional rehabilitation.38
about their disability and how we might help them gain It is important to help the individual out of this quagmire.
function. The more we allow ourselves to slow down and be The skills of a therapist are likely not enough to do this in
present with suffering—our own or that of another—the the short time that the client is in treatment, so a referral
more we will be able to be open to the mystery and joy of to social work, psychology, or psychiatry is required to
our lives just as they are without requiring them to be any help support the long-term process. It is still the thera-
different.32 It may be our lifetime’s journey to be servants of pist’s job to understand the process of adjustment, the in-
the healing arts; this is our job, and it also takes enormous dications regarding how an individual is adjusting, key
skill and bravery to bear witness to the full catastrophe of concepts for how to engage with an individual who is ad-
the human condition.32 One of the benefits of our profession justing, and how to set personal boundaries so that the
is the stimulus to examine our lives through the experiences clinician is less likely to be overwhelmed by the process
of others. This can improve our function and help us grow of adjustment and disability. In light of all this, it is still
as professionals and individuals, but if we are not open to the primary job of the therapist to help promote and
the clients’ experiences we may not find a reason to examine maximize the engagement in functional activities. These
and grow from our own experiences. activities are behaviors that must be goal oriented (patient,
CHAPTER 6 n Psychosocial Aspects of Adaptation and Adjustment during Various Phases of Neurological Disability 143
family, and therapist driven), demonstrating problem solv- Focusing on how to participate, move, and function in the
ing and information seeking and involving completion of world is one of the keys to helping the client and the family
steps to positively move forward into life with the disabil- work toward its future.33,49,51-54
ity and to maximize independence (promoting function). The therapist needs to help the client focus on the direc-
The rest of this section introduces the reader to some of tion of treatment objectives and to demonstrate how therapy
the psychological change components that may be assessed translates into meeting the client’s goals.19,55 To discover the
and acknowledged. The last section will attempt to demon- client’s true goals, the therapist must gain the trust of the
strate possible ways that these components can be taken into client and establish sound lines of communication. Distrust
account as an aspect of therapy. from health professionals may obstruct the adjustment pro-
cess and lead to negative consequences.56 Whenever possi-
Growth and Adaptation ble, the client’s support system should be enlisted to help
The clinician must keep in mind the context from which the establish realistic support for the client and the goals of both
client is coming. Just days or even hours ago the individual the client and the family. It has been found that if the client
may have been going about daily life without difficulty. The trusts the health professional, the client will be more adher-
trauma may be multifaceted: (1) physical trauma, (2) emo- ent and will seek assistance when it is needed (see Chapter 5
tional trauma occurring to the individual’s support system, for additional information).57,58
and (3) trauma of each of these systems interacting (the sup-
port system trying to protect the individual, and the client A New Normal
trying to protect the support system). The interaction of When we experience a decline in our ability to carry out our
these multifaceted components of the trauma may lead to everyday routine tasks, regardless of the cause of our “dis-
posttraumatic distress syndrome. This syndrome usually ability,” we may experience incredible degrees of despair.
happens within the first 6 months after the injury. This syn- Many societies emphasize a very specific idea of what it
drome may be observed more often in women39 but because means to be normal. There doesn’t appear to be a great deal
of cultural barriers it can be hidden in men. It happens more of flexibility in what this standard of normal is, regardless of
often when there has been a near-death situation.40,41 The one’s cultural background. When an individual fails to live
client may blame others, try to protect others, or be so self- up to or no longer fits this norm, there can exist a tremen-
absorbed that little else in the world may be seen or heard. dous amount of mental and emotional suffering. Because
It may be helpful to get psychological help for the individual our bodies and minds are so intricately connected, our
early in therapy if this is preventing optimal outcomes or physical being is adversely affected by the mental and emo-
creating obstacles in therapy.4,15,42-45 tional anguish. On top of what the individual may already be
It is the therapist’s job to develop a trusting relationship experiencing physically, suddenly there is another layer of
with the client. Through this relationship the individual can mental-physical anguish that is far too easily ignored and
be guided to focus on the goals of therapy and work on a unattended to by clinical professionals. However, once we
positive perspective about the future. One of the errors of are aware of the multifaceted potential for human suffering
the medical system is that of focusing on the disease out- with regard to adjusting to a disability, we may be empow-
comes and pathology and not on the person and the positive ered to assist the individual with a nonlinear, multifaceted
capabilities still within the individual’s grasp.19 This focus approach. Researchers and theorists from various psycho-
on the negative or loss may cause the individual to see only therapy traditions have begun to explore the potential value
the injury, disease, or pathological condition and nothing of the therapeutic relationship by making direct references
else. In a Veterans Administration hospital, spouses of to different levels of validation as a means of demonstrating
people with spinal cord injuries formed a group in which warmth, genuineness, empathy, and acceptance and reiterate
the group’s focus was on why the partners got married in how important it is for therapists to reflect back to the pa-
the first place; the group never looked at the physical limita- tient that their feelings, thoughts, and actions make sense in
tions as disabling. After a little while people came to the the context of their current experience. The therapist articu-
conclusion that they did not marry their spouses for their lates an expectation that the treatment collaboration will be
legs and the fact that the legs no longer worked was not a effective in an attempt to convey hope and confidence in
major issue after all. This started the decentering from the their ability to work together.59
medical disability model and the focus started to be placed We can guide our clients in identifying a new normal for
on the people and the families’ future. If we can help clients themselves, all the while allowing them and their support
focus on their functions and not their dysfunctions, the system to grieve the loss of the old normal. As the Harvard
effect of therapy after treatment will be much better. More psychologist Ellen Langer described in her book Mindful-
work needs to be done to help clients see the potential they ness, “if we are offered a new use for a door or a new view
will have in the future to live their lives with the highest of old age [or disability], we can erase the old mindsets
quality possible.34,46-50 The World Health Organization without difficulty.”60 We can offer our clients a new view of
developed a model that differentiates the disease pathology themselves by showing them what they are capable of as
model of medicine and focuses on individuals’ activities in they rebuild their lives. We can also help them acknowledge
life and the ability to participate in those interactions. This what is present in this moment and what the reality of the
model, the International Classification of Functioning, Dis- situation is. This does not have to be a problem to be suf-
ability and Health (ICF), has been enthusiastically accepted fered over but is a situation to be dealt with carefully and
by the therapy world, and the professions of both occupa- fully in the present moment. A woman who has lived with
tional and physical therapy use it as a reference model for multiple sclerosis for over 30 years described how the relief
practice. of suffering does not require restoring physical function to
some perceived level of normality. “Suffering is relieved to do you think this happened to you?” can lead to an enlight-
the extent that patients can learn to integrate bodily disorder ening experience. “Causes” may range from “God is punish-
and physical incapacity into their lives, to accommodate to ing me” to “I deserved it” to “life is against me.”
a different way of being” (p. 591).61 According to research From an early age, people in our society are exposed to
by De Souza and Frank,61 their subjects with chronic back misconceptions regarding the disabled person.70-73 If in the
pain expressed regret at the loss of capabilities and distress therapeutic environment, however, the client and family
at the functional consequences of those losses. They found have their misconceptions challenged constantly, they may
that facilitating “adjustment” to “loss” was more helpful start reformulating their concept of the role of the disabled
than implying the potential for a life free of pain as a result person. As this process progresses, therapists and other staff
of therapeutic interventions. can help make the expectations of the disabled person more
Guiding the individual through practice and repetition of realistic. Therapists can schedule their clients at times when
basic functional activities will allow the client to identify for they will be exposed to people making realistic adjustments
himself or herself how to live successfully in this world to disabilities. Use of individuals who have been success-
again and cultivate this “different way of being.” At the same fully rehabilitated as staff members (role models) can help
time we can encourage these clients to mindfully plan for to dispel the misconception that people with disabilities are
and visualize their future (see practice later) during specific not employable.74-76
times of their day so that their minds are not in constant This process of adaptation to a new disability can be
worry mode or rehearsing, which can cause a great deal of considered as a cultural change from a majority status (able
anxiety about the future. We can assist our clients in plan- bodied) to a minority status (disabled). Part of the adaptation
ning for that future, especially in a medical environment process can be considered as an acculturation process, and
where shorter rehabilitation stays are the norm. the therapist can help facilitate this process.16,72,77,78
Without any need to apologize for their loss, just simply The cultural background of the individual also contrib-
being with them in the moment in a nonjudgmental way and utes to the perception of disability and to the acceptance
allowing them to grieve can be a powerful tool for healing. of the disabled person. Trombly79 states that perception and
Acknowledging the loss and the suffering may help clients expression of pain, physical attractiveness, valuing of body
move forward with their lives in a new way. “Acceptance parts, and acceptability of types of disabilities can be cultur-
[of what is] doesn’t, by any stretch of the imagination, ally influenced. One’s ethnic background can also affect
mean passive resignation. Quite the opposite. It takes a intensity of feelings toward specific handicaps, trust of staff,79
huge amount of fortitude and motivation to accept what and acceptance of therapeutic modalities.80-84
is—especially when we don’t like it—and then work wisely The successful therapist will be sensitive to the cultural
and effectively as best we possibly can with the circum- values of the client and will attempt to present therapy to
stances we find ourselves in and with the resources at our the client in the most acceptable way. For example, in the
disposal, both inner and outer, to mitigate, heal, redirect, Mexican culture it is not polite to just start to work with a
and change what can be changed.”62 client; rapport must first be established. Sharing of food
may provide the vehicle to accomplish rapport. Thus, the
Practice: Mindful Planning therapist might schedule the first visit with a Mexican cli-
and Visualization of Future ent during a coffee break. The therapist must remember
n Find a time when you are alone; you need only a few that the dysfunctional client may be the one who can least
minutes every day for this practice. be expected to adjust to the therapist and that the therapist
n Allow this time to be specifically for future planning and may need to adjust to the client, especially in the early
visualization, not worrying. stages of therapy.
n If you find yourself worrying about the future at other Gaining trust is one of the crucial links in any meaningful
times during the day, acknowledge that there will be a therapeutic situation.58,85,86 Trust will create an environment
specific time devoted to planning and visualizing. Worry- that facilitates communication, productive learning, and ex-
ing throughout the day will bring a great deal of mental change of information.75,86 Trust is important in all cultures
anguish during times when you need to focus attention on and will be fostered by the therapist who is sensitive to
an important task or rehabilitation intervention. the needs of the client. This sensitivity is necessary with
n Use a journal to record thoughts and ideas on paper so every client but will be manifested in many different ways,
that the thoughts do not have to stay in the mind and be depending on the background and needs of the individual
rehearsed. Write down concerns as well as plans. in therapy. A client of one culture may feel that looking
n Try to let go of planning during daily activities and tasks another person in the eyes is offensive, whereas in another
until the next scheduled Mindful Planning Session, or, if culture refusal to look into someone’s eyes is a sign of weak-
necessary, allow this moment to be the next planning ses- ness or lack of honesty (shifty eyed).87 Thus although it is
sion but be sure to stop whatever else you are doing and impossible to know every culture or subculture with which
be fully present in the planning process. the therapist may come into contact, the therapist must
attempt to be sensitive to the background of the client. Even
Societal and Cultural Influences if the therapist knows the cultural norms, not every person
Culture, subcultures, and the culture and beliefs of the given follows the cultural patterns, and thus every client needs to
family are all aspects of the client that the therapist must be be treated as an individual in the therapeutic relationship. It
aware of.22,52,63-69 This concept gets into the beliefs about the should be the therapist’s job to be sensitive to the subtle
world and maybe a belief about the cause of the disability or nonverbal and verbal cues that indicate the level of trust in
at least how the client is viewing the disability. Asking “why the relationship. The therapist will obtain this information
by being open to the client, not open to a textbook. The are presented and structured to make therapy more relevant
client is the owner of this information and will share it with to the client’s values and needs. Value groups or exercises98
everyone he or she trusts. can be another means used by the therapist for evaluation
Trust is often established in the therapeutic relationship and understanding of the client.
through physical activities. The act of asking a client to Beliefs and values of cultures and families can play a
transfer from the chair to the bed can either build trust or profound role in the course of treatment. Such things as
destroy the potential relationship. If the client trusts the physical difficulties, which can be seen, are usually better
therapist just enough to follow instructions to transfer but accepted than problems that cannot be seen, such as brain
then falls in the process, it may take quite some time to re- damage that changed an individual’s cognitive abilities or
establish the same level of trust, assuming that it can ever be personality.99 A person with a back injury may be seen as
reestablished. This trusting relationship is so complex and lazy, whereas a person with a double amputation will be
involves such a variety of levels that the therapist should be perceived as needing help. At the same time, in some cultures
as aware of attending to the client’s security in the relation- a person who has lost a body part may be seen as “not all
ship as to the physical safety of the client in the clinic.58,85 If there” and should be avoided socially. Therefore being at-
the client believes that the therapist is not trustworthy in the tuned to the culture and beliefs of the client is imperative in
relationship, then it may follow that the therapist is not to be therapy. The reader is encouraged to refer to texts on cultural
trusted when it comes to physical manipulation of a disabled issues in health care such as Culture in Clinical Care by
body. If the client does not know how to use the damaged Bonder, Martin, and Miracle100; Cultural Competence in
physical body and thus cannot trust the body, then lacking Health Care: A Practical Guide by Rundle, Carvalho, and
trust in the therapist will only compound the stress of the Robinson101; and Caring for Patients from Different Cultures
situation.58,85,88 Chapter 8 provides more information on the by Galanti102 for more detailed discussions on how culture
neurological components of this interaction during the inter- and beliefs affect health care.
vention process.
The client’s culture may be alien to the therapist, even Establishment of Self-Worth
though both the clinician and client may be from the same and Accurate Body Image
geographical region. A client’s problems of poverty, unem- “The true value of a human being is determined pri-
ployment, and a lack of educational opportunities76,86,89,90 marily by the measure and the sense in which he has
can all result in the therapist and client feeling that therapy attained liberation from the self.”
will be unsuccessful, even before the first session has begun. —Albert Einstein
Such preconceived concepts held by both parties may not
be warranted and must be examined. These preconceived Self-worth is composed of many aspects, such as body im-
concepts can be more reflective of failure of rehabilitation age, sexuality, and the ability to help others and to affect the
than any physical limitation of the client. environment. The body image of a client is a composite of
Cultural and religious values may also result in the client past and present experiences and of the individual’s percep-
feeling that he or she must pay for past sins by being dis- tion of those experiences. Because body image is based on
abled and that the disability will be overcome after atone- experience, it is a constantly changing concept. An adult’s
ment for these sins. Such a client may not be inclined to body image is substantially different from the body image of
participate in or enjoy therapy. The successful therapist does a child and will no doubt change again as the aging process
not assault the client’s basic cultural or religious values but continues. A newly disabled person is suddenly exposed to
may recognize them in the therapy sessions. If the therapist a radically new body, and it is that individual’s job to assess
feels that the culturally defined problems are impeding the body’s capabilities and develop a new body image.
the therapeutic process, the therapist may offer the client Because the therapist is at least partially responsible for
opportunities to reexamine these cultural “truths” in a non- creating the environmental experiences from which the client
judgmental way and may help the client redefine the way learns about this new body, the therapist must be aware of
the physical limitations and therapy are seen.91 Religious the concept. In the case of an acute injury, the client has a
counseling could be recommended by the therapist, and new body from which to learn. The therapist can promote
follow-up support in the clinic may be given to the client to positive feelings as the therapist instructs the client how to
view therapy not as undoing what “God has done” but as a use this new body and to accept its changes.1,16,20,27,103,104
way of proving religious strength. Reworking a person’s Because in “normal” life we slowly observe changes in
cultural and religious (cognitive) structure is a sensitive our bodies, such as finding one gray hair today and watching
area, and it should be handled with care and respect and with it take years for our hair to turn totally white, we have the
the use of other professionals (social workers and religious luxury of slowly adapting to the “new me.” Change usually
and psychological counselors) as appropriate. does not happen quite so slowly and “naturally” when
The hospital staff can be encouraged to establish groups trauma or a disease affects the nervous system. This sudden
in which commonly held values of clients can be examined loss of function creates a void that only new experiences and
and possibly challenged.16,91-97 Such groups can lead the new role models can fill.
client to a better understanding of priorities and may help The loss of use of body parts can cause a person to per-
the person see the relevance of therapy and the need to ceive the body as an “enemy” that needs to be forced to work
continue the adjustment process. This can also prepare the or to compensate for its disability. In all cases the body is the
client to better accept the need for support groups after reason for the disability and the cause of all problems. The
discharge. The therapist may be able to use information need for appliances and adaptive equipment can create a sense
from such group sessions to adjust the way therapy sessions of alienation and lack of perceived “lovability” resulting from
the “hardness of the hardware.” People tend to avoid hugging The last aspect of self-worth is often overlooked in the
someone who is in a wheelchair or who has braces around health fields. This aspect is the need that people have to help
the body because of the physical barrier and because of the others.107 People often discover that they are valuable
person’s perceived fragility; a person with physical limita- through the act of giving. Seeing others enjoy and benefit
tions is certainly not perceived as soft and cuddly.20,27,52,102,103 from the individual’s presence or offering increases self-
Both the perception that these individuals are not lovable and worth. Situations in which others can appreciate the client’s
their labored movements can sap the energy of the disabled worth may be needed. Unless the client can contribute
and discourage social interaction or life participation. To to others, the client is in a relatively dependent role, with
accept the appliances and the dysfunctional body in a way everyone else giving to him or her without the opportunity
that also allows the disabled person to feel loved is surely a of giving back. Achieving independence and then reaching
major challenge. out to others, with therapeutic assistance if necessary, facili-
In the case of a person who will be disabled for the long tates the individual’s more rapid reintegration into society.
term, such as the person with cerebral palsy or Parkinson The therapist should take every opportunity to allow the
disease, the therapist is attempting to teach the client how to client to express self-worth to others through helping.
change the previously accepted body image to one that would The ability to expand one’s definition of oneself is a key
allow and encourage more normal function. In short, the factor in adjusting to a disability. Expanding the definition of
therapist has two roles. One role is to help lessen the disabled oneself in terms of all the roles and responsibilities one has
body image. The second is to teach a functional disabled body can help the individual comprehend the enormity of who he
image to a newly disabled person. The techniques may be the or she is. The individual may begin to understand how he or
same, but in both cases the client will have to undergo a great she is so much greater than just the job he or she once per-
amount of change. The person with a neurological disorder formed and so much greater than the role he or she once
or neurologically based disability may assume that he or played. This practice can cultivate understanding of how
she will not be capable of accomplishing many things with complex our species is and how much we have to offer the
his or her life. The therapist is in a unique position to world, differently abled or not (see journal activity, Box 6-1).
encourage development of and maximize the client’s level
of functional ability. The individual may then expect more Sense of Control
of himself or herself. The newly disabled person must “Oh, I’ve had my moments, and if I had to do it over
change the expectations; however, he or she has little con- again, I’d have more of them. In fact, I’d try to have
cept of what is realistic to expect of this new body. At this nothing else. Just moments, one after another, instead
point, role models can be used to help shape the client’s of living so many years ahead of each day.”
expectations. If the client is unable to adjust to the new body —Nadine Stair, 85 years old, Louisville, Kentucky
and change the body image and self-expectations, life may
be impoverished for that individual. Pedretti105 states that As Drs. Roizen, and Oz stated in their book You: The
the client with low self-esteem often devalues his or her Owner’s Manual, we can control our health destiny.108
whole life in all respects, not just in the area of physical Although we can’t always control what happens to us (no
dysfunction.1,16,20,27,103,104 matter how fit we are), there are some things we can con-
One way the client can start exploring this new body is by trol: our attitude, our determination, and our willingness
exploring its sensations and performance. Dr. Jon Kabat-Zinn to take our own health into our own hands.108
developed a guided “body scan” meditation that can help in- Adjusting to a disability can make clients feel as though
dividuals learn how to become more connected and in tune they have very little control over their lives; they may feel
with the sensations of the body.62 This kind of practice is
about learning to pay attention to the body in a new way and
can be very helpful in developing an accurate body image and
improve self-awareness. The client with a spinal cord injury BOX 6-1 n JOURNAL ACTIVITY
may also use the sensation of touch to “map out” the body to
see how it reacts.106 They may ask themselves the following Write down three words that someone who loves you would
questions: Is there a way to get the legs to move using choose to describe you. Choose qualities that you think oth-
reflexes? Can positioning the legs in a certain way aid in rollers appreciate about you, such as intelligent, funny, kind,
ing the wheelchair or make spasms decrease? What, if any- organized.
thing, stimulates an erection or lubrication? Such exploration Write down the ways that you express those qualities
will start the client on the road to an informed evaluation in your life. Maybe it is when you garden, write, draw, or
of his or her abilities. listen.
The therapist’s role is to maximize the client’s perceptions Write down your ideal vision of the world. Maybe it
of realistic body functioning. Exercises can be developed that is, “I envision a world that is free of violence” or “where
encourage exploration of the body by the individual and, if everyone has access to knowledge about how to keep our
appropriate, the significant partner. Functioning and building oceans clean” or “where children are well cared for.”
an appropriate body image will be more difficult if intimate Put it all together in one statement: “I will use my intel-
knowledge of the new body is not as complete as before in- ligence, humor, organization, and kindness, through writ-
jury.9 The successes the client experiences in the clinical ing, drawing, gardening, and listening to help create a world
setting coupled with the client’s familiarity with his or her that is peaceful, where children are well cared for, and
new body will result in a more accurate body image and will where the oceans are clean.” Then do it!
contribute to the client’s feelings of self-worth.
helpless, as though their health is in everyone else’s hands According to Drs. Oz and Roizen,108 these emotions can
but their own. This feeling can cause incredible suffering cause high blood pressure, as well as disrupting the body’s
and emotional anguish on top of their physical or cognitive normal repair mechanism, and also constrict our blood ves-
disability. If we focus solely on treating the disability and sels, making it even harder for enough blood to work its
ignore what may be going on for our clients mentally and way through the body. They go on to say that learning
emotionally, we may be creating even more suffering for relaxation techniques such as yoga and meditation can
them. Clinical professionals have the opportunity to guide help us handle these damaging feelings in a healthier way.
their clients toward a new way of relating to their disability We know now that these mind states affect our bodies
by focusing on what they do have control over as well as profoundly—for example, a feeling of helplessness appears
identifying ways in which they may relate differently to to weaken the immune system.108 If we can teach our clients
those situations over which they do not have control. to be mindful of and pay attention to their “mind states”—
Dr. Jill Bolte-Taylor says this eloquently in a passage also known as “thoughts”—at any given moment during
from her book: therapeutic intervention, we may be able to encourage a
greater sense of control and facilitate greater mental and
“I’ve often wondered, if it’s a choice, then why would emotional adjustment to the individual’s disability. Accord-
anyone choose anything other than happiness? I can ing to a 2008 article by Ludwig and Kabat-Zinn in JAMA,
only speculate, but my guess is that many of us don’t “the goal of mindfulness is to maintain awareness moment
exercise our ability to choose. Before my stroke, I by moment, disengaging oneself from strong attachment to
thought I was a product of my brain and had no idea beliefs, thoughts, or emotions, thereby developing a greater
that I had some say about how I responded to the emo- sense of emotional balance and well-being.”113 Anat Baniel,
tions surging through me. On an intellectual level, in her book Move into Life, describes how research shows
I realized that I could monitor and shift my cognitive that the moment we bring attention and awareness to our
thoughts, but it never dawned on me that I had some movements moment by moment, the brain resumes growing
say in how I perceived my emotions. . . . What an new connections and creating new pathways and possibili-
enormous difference this awareness has made in how ties for us.114
I live my life.”109 According to a research study by Dr. Jon Kabat-Zinn115
of the Stress Reduction Program at the Center for Mindful-
As Dr. Bolte-Taylor describes, all of us have the choice to be ness in Medicine, Health Care, and Society, the practice of
in relation to the present moment fully, or we can allow our mindfulness meditation used by chronic pain patients over a
thoughts and emotions to “take us for a ride” as though we 10-week period showed a 65% reduction on a pain rating
were on automatic pilot.109 If we allow our minds and emo- index. Large and significant reductions in mood disturbance
tions to take over our experience of the present moment, we and psychiatric symptoms accompanied these changes and
can easily be dragged along into rehashing our past events were stable on follow-up. Another study looked at brain
that led up to the disability, which can create more suffering imaging and immune function after an 8-week training pro-
and emotional anguish. We also may be rehearsing what our gram in mindfulness meditation.116 The study demonstrated
lives will be like without allowing the dust to settle, without that this short program in mindfulness meditation produced
waiting until we have a clearer picture of what implications demonstrable effects on brain and immune function. The
the disability may have for us. An unacknowledged rehash- results of a clinical intervention study by Brown and Ryan112
ing and rehearsing can create an incredible sense of lack of showed that higher levels of mindfulness were related to
control over one’s life, thereby increasing anxiety and de- lower levels of both mood disturbance and stress before
pression. Approximately 70% of our thoughts in any par- and after the Mindfulness-Based Stress Reduction (MBSR)
ticular waking state can be considered to be daydreams, and intervention. Increases in mindfulness over the course of
they can often be unconstructive.110 In an experience sam- the intervention predicted decreases in these two indicators
pling method, Klinger and his colleagues found that “active, of psychological disturbance. Evidence has indicated that
focused problem-solving thought”111 made up only 6% of those faced with a life-threatening illness often reconsider
the waking state. According to Baruss, “it would make more the ways in which they have been living their lives, and
sense to say that our subjective life consists of irrational many choose to refocus their priorities on existential issues
thinking with occasional patches of reason”110 while we are such as personal growth and mindful living.117
participating in our daily activities. Especially when one is These findings suggest that meditation may change
participating in menial, basic self-care activities, our mind is brain function and immune function in positive ways.
often in another place. If an individual is frequently discon- “Meditation” as it is taught in this 8-week program is simply
nected from the present moment, tending to ruminate over an awareness and attention training: a way of learning how
the past or future events, he or she may experience signifi- to pay attention in the present moment to our thoughts and
cant negative effects from this distraction. Rumination, ab- emotions and coming to understand how our thoughts and
sorption in the past, rehashing, or fantasies and anxieties emotions affect our bodies. It may sound simple but actually
about the future can pull one away from what is taking place can be incredibly challenging. However, an instant stress
in the present moment. Awareness or attention can be di- reliever can be bringing awareness to the breath. Deep
vided, such as when people occupy themselves with multi- breathing can act as a mini-meditation and from a longevity
ple tasks at one time or preoccupy themselves with concerns standpoint is an important stress reliever.108 Shifting to
that detract from the quality of engagement with what is fo- slower breathing in times of tension can help calm us and
cally present, and this can increase anxiety and depres- allow us to perform, whether mentally or physically, at
sion.112 higher levels.108
Another study, focused on Coping Effectiveness Training without judging them or reacting to them, is healing our view
(CET), consisted of weekly 60-minute psychoeducational of the body and allowing it to come to terms, at least to some
group intervention sessions focused into six topic areas degree, with conditions as they are in the present moment in
and was adapted from the protocol Coping Effectively with ways that no longer overwhelmingly erode our quality of life,
Spinal Cord Injury.118 The treatment protocol was structured even in the face of pain or disease.”62
to provide education and skill building in areas of awareness “Mystery surrounds every deep experience of the hu-
of reactions to stress; situation appraisal; coping strategy man heart: the deeper we go into the heart’s darkness or
choice; interaction among thoughts, emotions, and behav- its light, the closer we get to the ultimate mystery of God
iors; relaxation; problem solving; communication; and social [the Universe].”121
support.119 There was a significantly positive correlation Religious and spiritual beliefs can be assistive in the
between the learned coping strategies and the disabled indi- process of adjusting to a disability. Johnstone, Glass, and
vidual’s ability to adjust in a healthy way. Oliver highlight that religion and spirituality are important
coping strategies for persons with disabilities.67 According
Hope and Spiritual Aspects to Adjustment to Dr. Jill Bolte-Taylor in her book My Stroke of Insight: A
Through great suffering there is incredible potential for us to Brain Scientist’s Personal Journey, “Enlightenment is not a
transcend the mental and emotional limits of the physical process of learning but a process of unlearning.”109 Western
body. As clinical professionals we need to be aware of society rewards the skills of the “doing” left brain much
this capability. As described by Dr. E. Cassel in the New more than the “being” right brain, which can significantly
England Journal of Medicine, “Transcendence is probably hinder our process of spiritual growth. The focus of our lives
the most powerful way in which one is restored to whole- becomes more about obtaining positions, roles, and “stuff.”
ness after an injury of personhood. When experienced, tran- We begin to identify ourselves with all of this when in real-
scendence locates a person in a far larger landscape. The ity the positions, roles, and stuff can be taken from us at any
sufferer is not isolated by pain but is brought closer to a moment. “When we are obsessed with . . . productivity,
transpersonal source of meaning and to the human commu- with efficiency of time and motion, with projecting reason-
nity that shares those meanings. Such an experience need not able goals and making a beeline toward them, it seems
involve religion in any formal sense; however, in its transper- unlikely that our work will ever bear fruit, unlikely that we
sonal dimension, it is deeply spiritual.”120 Parker Palmer, will ever know the fullness of spring in our lives.”121
a writer and teacher, describes it this way: “Treacherous There is a much deeper definition of ourselves that
terrain, bad weather, taking a fall, getting lost—challenges goes beyond all of the material possessions and the roles
of that sort, largely beyond our control, can strip the ego that we may ever play. According to Eckhart Tolle,122
of the illusion that it is in charge and make space for true self when forms that we identify with, that give us a sense of
to emerge.”121 Eckhart Tolle describes the ego as complete self—such as our physical bodies—collapse or are taken
identification with form—physical form, thought form, emo- away, it can lead to a collapse of the ego, because ego
tional form.122 The more we are identified with the physical is identification with “form.” When there is nothing to
realm, the more we will suffer when our attachment to stuff identify with anymore, who are we? When forms around
or “form” becomes torn. us die, or death approaches, Spirit is released from its
“For all of us, our willingness to explore our fears, to live imprisonment in matter. We can finally understand that
inside helplessness, confusion, and uncertainty, is a power- our essential identity is formless, spiritual.122 Cultivating
ful ally. Acknowledging our repeated exposure to human greater understanding of these concepts and delving more
suffering—our own and others’—and the seductive draw of into the spirit can provide a great deal of relief for all of
numbness and melancholy that provides temporary escape is us who are suffering.
necessary if we are to be renewed.”32 Dr. Santorelli goes on There is a wonderful quote by former Secretary-General
to say that “there is no way out of one’s inner life, so one of the United Nations U Thant, as he describes how he envi-
had better get into it.”32 “On the inward and downward sions the spiritual:
spiritual journey, the only way out is in and through.”121
“Spirituality is a state of connectedness to life.
Practice: The Willingness to Embrace What Is It is an experience of being, belonging and caring.
1. Become aware of the moments when “resistance to what It is sensitivity and compassion, joy and hope.
is” is noticed. This may manifest itself as anxiety, sad- It is harmony between the innermost life and the
ness, fear, depression, anger. outer life or the life of the world and the life of the
2. As soon as anger arises (for example), notice how it universe.
manifests itself physically in the body. It may be tension It is the supreme comprehension of life in time
in the muscles, a quickened or palpitating heartbeat, or and space, tuning of the inner person with the great
sweating. mysteries and secrets that are around us.
3. Note what the sensation feels like in the body without It is the belief in the goodness of life and the possi-
trying to make the moment different than it is. Acknowl- bility for each human person to contribute goodness
edge whatever is present in the moment. to it.
4. Note that we are not the anger, we are the awareness of it. It is the belief in life as part of the eternal stream of
5. Note what the awareness does. Journal any thoughts or time, that each of us came from somewhere and is
feelings about the practice. destined to somewhere, that without such belief there
Dr. Jon Kabat-Zinn, in his book Coming to Our Senses, could be no prayer, no meditation, no peace, and no
states, “It seems as if awareness itself, holding the sensations happiness.”123
Spirituality is something that provides hope, connection signify either a lack of mental health or an inability to cog-
with others, and reason or meaning of existence for many (if nitively realize the situation.
not most) people. It is amazing that the medical community
has been slow to accept the power of spirituality because Expectancy for Recovery
this is an area that gives meaning to so many peoples’ lives. The client in the stage of expectancy for recovery is aware
Spirituality has been linked to health perception, a sense of that he or she is disabled but also believes that recovery will
connection with others, and well-being.66,67,124-130 be quick and complete. The person may look for a “miracle
Anything that helps the client put the disability into per- cure” and may make future plans that require total return of
spective and helps the client move on with life in a healthy function. Total recovery is the only goal, even if it takes a
way is good. The Western medical system was based on great deal of time and effort to achieve. Key signs of this
diagnosis of pathology and how best to cure disease, but stage are resentment of loss of function and the feeling that
there has been a slow but fruitful shift toward a more holistic the whole body or mind is necessary to do anything worth-
view of the healing process and prevention. The National while. The staff can stimulate a change from this stage by
Institutes of Health now has a National Center for Comple- giving clear statements to the client that the damage is per-
mentary and Alternative Medicine (http://nccam.nih.gov). Al- manent (if in fact that is true), by transferring the person
most every major hospital and university in the country now home or to the rehabilitation unit, or by discontinuing ther-
has an integrative health center (e.g., http://stanfordhospital. apy. Any one of these occurrences can help make the client
org/clinicsmedServices/clinics/complementaryMedicine realize the permanence of the disability. It is also important
and www.osher.hms.harvard.edu). Although this small but to not take away an individual’s hope. In the case of an indi-
steady shift in the focus of medicine has gained momentum, vidual who has experienced a stroke or a brain injury, we
one of the dangers of the medical system is still the entrap- know now that the brain is capable of repairing itself
ment in pathology to the point where the client may not see throughout a lifetime—though we need to be clear that we
anything but his or her pathology. Spirituality can help the do not know how much recovery will occur, if any. This all
client and the family to see that there is more to life than depends on the severity of the damage and the lifestyle of
pathology, stimulate interaction with others, put the func- the client—for example, smoking, stress, and/or lack of
tional limitations in perspective, give meaning to life (and participation in meaningful activity, all of which impede
the disability), and give the person hope and a sense of well- progress.
being.* This is what we all want for the client and the family.
Refer to Chapters 1, 5, and 39 for additional content. Mourning
During the stage of mourning the individual feels that all is
Adjustment Using the Stage Concept lost, that he or she will never achieve anything in life. Sui-
Each person has his or her own coping style, and each should cide is often considered. The individual may feel that char-
be allowed to be unique. Kerr133 describes five possible acteristics of the personality (such as courage or will) have
stages of adjustment: also been lost and must be mourned as well. Thus, motiva-
Shock: “This really isn’t happening to me.” tion to continue therapy or the will to improve may be im-
Expectancy for recovery: “I will be well soon.” peded. The prospect of total recovery may no longer be held,
Mourning: “There is no hope.” but at the same time there appears to be no other acceptable
Defense: “I will live with this obstacle and beat it.” alternative. This feeling of despair may be expressed as hos-
(healthy attitude) “I am adjusted, but you fail to see tility, and as a result therapists may view the individual as a
it.” (neurotic attitude) “problem patient.” It is possible for a client to remain at this
Adjustment: “It is part of me now, but it is not necessarily stage with feelings of inadequacy, dependence, and hostility.
a bad thing.” However, it is also possible for therapeutic intervention to
In light of current research, it is important for the facilitate movement to the next stage by creating situations
therapist to realize that these are not lockstep stages and in which the client may feel that “normal” aspirations and
are to be thought of as concepts to help with the under- goals can be achieved. In this circumstance, normal would
standing of common reactions of all individuals.134,135 not include such “low-level” activities as dressing or walk-
Some individuals may settle in one stage for quite some ing; these are all activities that were taken for granted before
time or may even skip stages altogether, whereas others the injury. Normal, though, would include performing the
may move through the stages quickly. This is an incredi- job the client was trained to do. Such activities would also
bly individual process. include playing with or caring for a child or family. This
would be seen as self-actualization by Maslow.136
Shock
The individual in shock does not recognize that anything is Defense
actually wrong. The client may totally refuse to accept the The defense stage has two components. The first represents
diagnosis. The client may even laugh at the concern ex- a healthy attitude in which the client actually starts coping
pressed by others. This stage is altered when the person has with the disability. The individual takes pride in his or her
an opportunity to test reality and finds that the physical or accomplishments and works to improve independence and
cognitive condition is actually limiting the ability to partici- become as “normal” as possible. The person is still very
pate in functional activities. If this stage continues, it may much aware that barriers to normal functioning exist and is
bothered by this fact but also realizes that some of the barri-
ers can be circumvented. This healthy stage can be under-
*References 66, 67, 124, 126, 127, 131, 132. mined and possibly destroyed by well-meaning family,
friends, and therapists who encourage the individual to see White140 stated that without some participation, there can
only the positive aspects and who do not allow the client to be no affecting the environment and thus no sense of self-
examine feelings about the restrictions and barriers of the satisfaction. Fine141 and King142 point out that without satis-
condition. Conditions that lead to the final stage of adjust- faction from affecting the environment, reinforcement is
ment are the realization that the whole body or mind is not insufficient to carry on the behavior, and the behavior will
needed to actualize his or her life goals and that needs can be extinguished. Thus satisfaction and performance must be
be actualized in other ways. A therapist should watch for linked. If the patient has not adjusted to his or her new body,
opportunities to facilitate this transition. There is a fine however, little satisfaction can be gained from such every-
line between hopelessness and hope of regaining function. day activities as walking, eating, or rolling over in bed.143
Taking away any hope of regaining quality of life leads to To define adjustment on a purely performance basis is to
helplessness and may take away the motivation for neuro- run the risk of creating a “mechanical person” who might
plasticity within the patient’s central nervous system. Thus, be physically rehabilitated but, once discharged, may find
helping the patient be realistic and reality oriented while not that he or she lacks satisfaction, incentive, and purpose.
taking away hope is a skill all therapists need to cultivate. The psychological state of adjustment is what makes self-
The negative alternative during the defensive stage is the satisfaction possible.
neurotic defensive reaction. The client refusing to recognize
that even a partial barrier exists to meeting normal goals Body Image
typifies this. The client may try to convince everyone that he “Self-care is never a selfish act—it is simply good steward-
or she has adjusted. ship of the only gift I have, the gift I was put on this earth
to offer to others. Anytime we can listen to true self and give
Adjustment it the care it requires, we do so not only for ourselves but
In the final stage, adjustment, the person sees the disability for the many others whose lives we touch.”121
as neither an asset nor a liability but as an aspect of the per- Body image is an all-encompassing concept that looks at
son, much like a large nose or big feet. He or she is accept- how the person and to some extent the support systems
ing what is, not resisting what is. Functional limitation or view the person and roles that are expected to be assumed.
inability to participate in any life activity is not something to Taleporos and McCabe20 found that clients had negative
be overcome, apologized for, or defended. Kerr133 refers to feelings about their bodies and general negative psycho-
two aspects or goals of this stage. The first goal is for the logical experiences after injury. Even when clients do not
person to feel at peace with his or her god or greater power: have disfigurements that are readily observable, they often
the client does not feel that he or she is being punished or still report changes in body image and negative feelings of
tested. The second goal is for the client to feel that he or she self-worth.
is an adequate person, not a second-class citizen. Kerr137 One of the issues that may arise relating to body image is
believes that “It is essential that the paths to those more sexuality. This concept may take many behavioral forms:
‘abstract goals’ be structured if the person is to make a flirting, harassment, questions about fertility, or questions
genuine adjustment.” She also believes that it is the health regarding whether the client is capable of performing the sex
care professional’s job to offer that structure. act at all. Flirting may be a sign that clients have had assaults
Acceptance or adjustment is at least as hard to achieve on their femininity or masculinity. By flirting, clients are
and maintain in life for the disabled person as happiness and often trying to determine whether they still are seen as a
harmony are for all people.138 Adjustment connotes putting sensual being. In this case the therapist may need to set
the disability into perspective, seeing it as one of the many boundaries by saying that he or she is not allowed to date or
characteristics of that person. It does not mean negating the flirt with clients. This is to make sure that the client does not
existence of or focusing on the condition. Successful adjust- think that it is something about the disability that is the
ment may be defined as a continuing process in which the “turnoff.” Sensitivity must be used because the client could
person adapts to the environment in a satisfying and efficient think that “if a medical person finds me repulsive then no
manner. This is true for all human beings, able-bodied one will ever see me as attractive.” It is important for the
or disabled. There are always obstacles to overcome in therapist to try to ascertain the intent behind the behavior.
attempting the goal of a happy and successful life.16,92,138,139 Usually this can be accomplished by evaluating how he or
People and circumstances change. Maintaining a bal- she feels about the interaction. If the therapist feels unthreat-
anced state of adjustment is not easy, especially for the ened and does not feel demeaned when the client is flirting,
person with limitations. I recall a woman who had achieved he or she still needs to report this to the therapist of record.
a stable state of acceptance of her quadriplegic condition. If the therapist feels defensive, demeaned, or very uncom-
One day she called in a panic because, as she saw it, she fortable, then he or she may be experiencing harassment. It
“wasn’t adjusted anymore.” She had moved into a college is never warranted or “part of the job” to be harassed, and
dormitory and wanted to go out for a friendly game of the client’s behavior must be stopped immediately by alert-
football with her new friends but suddenly saw how physi- ing the client that the behavior is making the therapist feel
cally limited she was. She had grown up in a hospital and uncomfortable and that it must stop now. Again, the thera-
had never had to face this situation. After discussing this, pist needs to go to the supervisor or team to mention this
she was able to put things into perspective and was able to behavior. It can often be the case that other team members
talk over her feelings of isolation with her friends, who, are experiencing the same behavior and it can be dealt with
without hesitation, altered the game to include her. Keep- as a team. If the behavior is considered a chronic problem
ing a balanced perspective is hard in a world that changes by the team, a treatment plan needs to be designed to stop
constantly. the behavior. It is important to remember that sexual health
should not be a neglected area of client treatment. It may as other parts of the body, allows the individual to develop
take time for the appropriate questions to be asked by the communication, self-gratification, and a feeling of compete
client.28,144-148 nce.106,140,167
Questions about any physical performance are within the This feeling of competence is derived from the effective
domain of therapy. If the client is asking for information use of the body to meet its needs and to accomplish tasks.
regarding sex (e.g., positioning options) it is a subject that By the age of 8 years, body parts and body processes are
needs to be addressed in a respectful manner. If the ques- usually named and the child perceives the body as good. At
tions are regarding fertility, capability, and the like, then this time intimacy between the self and another person is
these should be referred to an appropriate medical person. further refined, as are roles. During puberty, body changes
None of these questions should be discouraged or neglected, and sexual tension are heightened. Self-acceptance is based
because this area is important for your clients’ motivation on the person’s perception of how effectively he or she has
and sexual health.149,150 It is important for the therapist to accomplished the previous tasks.106,167-169
know that in spinal cord injury, fertility will generally not be The preceding is an oversimplification of the first 20 years
impaired for a woman, but issues of lubrication before sex of life, but the role of sensuality and sensation cannot be
should be addressed by the appropriate person. Men may overemphasized. This is especially true for those profession-
have erection problems and ejaculation issues, but these too als who constantly interact with clients in a physical manner
can be addressed by the appropriate person. It is now known such as handling. The intervention the therapist provides
that fertility in spinal cord–injured men may be possible and when the client is, or feels he or she is, in a dependent state
should not be ruled out.146,151-154 can have a direct impact on how the client may perceive
himself or herself in the future.
Awareness of Sexual Issues
Sexuality is usually one of the last areas to be assessed by Pediatric Sensuality
clinical staff, but it is one area mentioned as having great The child needs to learn to enjoy the body. The therapist
importance to family members and the client.83,104,155,156 should help the client to distinguish between therapeutic
Sexuality involves more than just the sex act; it incorporates touch and “fun” sensual touch, such as tickling or cud-
characteristics such as sexual attraction, sexual identifica- dling. It is important for clients to distinguish between the
tion, sexual confidence, and sexual validation.104,155,156 It is two so that they do not “turn their bodies off” to touch. For
a predictor of adjustment to disability, of success in voca- example, a woman with cerebral palsy stated during an
tional training, and of marital satisfaction when the woman interview that therapy was either painful or so clinical that
is disabled.28,73,147,148,156-162 she disassociated herself from sensations in her body dur-
Sexuality (sensuality) is representative of how the person ing therapy. Later in life this became a problem when she
is dealing with his or her world. If the person feels inade- was married. She stated that it took 7 years of marriage
quate as a sexual, sensual, and lovable human being, there before she could enjoy the sensations of being touched by
is little chance that the person will also feel motivated to her husband.
pursue other avenues of life.83,156,163 This area of function The therapy session should also help the client develop a
must be assessed with great sensitivity to the individual’s sense of personal ownership of the body.81,155,170 This aspect
feelings.143,148,163-165 is often neglected when working with children.81,167 The
The framework for assessing sexuality differs with the therapist often does not ask permission to touch a client,
therapist. Some therapists see sexuality as an activity of thus suggesting that the client lacks the right to control
daily living and incorporate it into the evaluation. Others being touched by others. The last thing the therapist would
feel the client needs information about body mechanics to desire to communicate, especially to a child, is that any
perform the sex act; thus positioning and reflex inhibiting person has the right to handle and touch the client’s body.
patterns are assessed. Still others have found it a motivating Child molestation with a disabled population is just begin-
force when range of motion and muscle control are worked ning to be recognized as a problem in this country, with
on. A further discussion of these concerns follows in the possibly one third of the female and male population being
section on adult sexuality. victimized.81 It is hard to think of a more likely victim than
a person who has (unintentionally) been taught that he
Development of Sensuality (Sexuality) or she does not have the right to say “No” to being touched
Even before birth, the sense of touch166 and the ability to and who cannot physically resist unwanted advances and
distinguish pleasurable and unpleasurable tactile sensations in some cases cannot even communicate that abuse has
begin to develop. Pleasurable feelings are comforting, and taken place. The effects of this can be seen in adults. When
attempts are made to prolong them; for example, a baby one client was asked why tone increased in her lower
cries when nursing is stopped. If satisfaction is not derived extremities when she was touched, her response was, “I was
from this interaction on a regular basis, a feeling of anxiety sexually abused by my father in the name of therapy, and
may develop, the child may withdraw from interaction with therapy and sexual abuse are synonymous at this point.” No
others, and distrust may develop.166 If pleasure in interaction wonder she had not wanted to reenter therapy!
with others is obtained in the first 3 years, the ability to One way of helping clients “own” their respective bodies
maintain the warmth of being close and being nourished is (besides asking permission to touch) is by naming body
translated into trust (that all needs will be satisfied by the parts and body processes using correct terminology (as
caretaker) and lovability (bonding). It is here that a sense of opposed to baby talk), thus making it possible for the client
intimacy is initiated.106,167 By the age of 5 years, the ability to communicate and relate appropriately.81,167,170,171 This can
to explore the world by using the hands and mouth, as well be accomplished as the need arises, or it can be encouraged
through the use of anatomically correct puzzles or dolls dur- interactions should be directed toward creating an environ-
ing therapy sessions. ment that will promote a stronger and more well-adjusted
One goal of therapy may be to develop the concept that client.106,170,171,173
the body (in the case of persons with the congenital dis- The therapist’s response to sexual advances must be tem-
abilities) or the “new body” (in the case of those with ac- pered with an understanding of the possible cause for the
quired disabilities) is acceptable and good,167-171 thus giving behavior. The client may be cognitively impaired and may
the client a more positive attitude toward his or her body and not even be aware of the inappropriateness of some forms of
toward therapy. Pointing out a particularly positive aspect of sexual behavior, or the client may be trying to control others
the client’s body and mentioning this regularly can encour- through acting-out behaviors. The client may have been
age this attitude. This feature could be the hair, the eyes, or sexually aggressive even before the injury. At no time should
a smile, but it should be an aspect of the client that can be the therapist allow himself or herself to be sexually ha-
seen and commented on by others as well. Commenting on rassed. If the therapist feels harassed, the therapist must take
how well the body feels when it is relaxed or how good the control of the situation and find a way to stop the client’s
sun feels on the body helps the client recognize that the behavior. This is usually achieved by confronting the issue.
body can be a positive source of pleasure. Not dealing with inappropriate behavior will allow it to con-
Another message that can negatively affect the client in tinue and may be detrimental to the medical team and to the
later life is the concept that individuals with movement dys- client’s normal participation in life.145,156,170,171
function are asexual and will never have sexual needs or The therapist can assist the client in moving through the
partners.73,148,169-173 Although it may not be appropriate to stages of self-awareness to appreciate that the client is still
deal directly with the concept in therapy with a child, the sensual, sexual, and huggable. This process can be done
therapist might mention that he or she knows of a person through everyday interaction; it may entail encouraging the
with a functional problem such as the client’s movement family to embrace the client and may even call for the thera-
limitations who is married or who has children. In this way pist to role model these behaviors at times.174 The therapist
the therapist communicates that there is a possibility that the may provide reading materials to the client and family
“normal” sex roles of the child may be fulfilled in the future. directly by reviewing and answering questions or indirectly
Without this possibility being presented, the child may think by having such books as Reproductive Issues for Persons
that there is no chance that all the movies, books, and televi- with Physical Disabilities,175 Sexuality and the Person with
sion programs that deal with normal adult interactions apply Traumatic Brain Injury: A Guide for Families,176 and Sexual
to individuals with functional limitations, a belief that leads Function in People with Disability and Chronic Illness177
to poor socialization and further alienation from participating available for their reading. In this way, the individual and
in life.* significant others are made aware of possible options for the
expression of intimacy and of the fact that this part of life is
Adult Sexuality not over.
Discussing positioning to reduce pain and spasticity or Because the therapist is in a situation of one-to-one treat-
to enable the client to more comfortably engage in sexual ment involving touching, moving, and handling the client’s
relations will help the client deal with problems before they body, he or she may frequently be the natural person from
reveal themselves. Because sexual hygiene may be consid- whom the individual may seek information. If this natural
ered as an activity of daily living, it may fall within the curiosity does not appear to be forthcoming, however, the
domain of therapy. therapist can give the client an opening. For example, during
The client may feel that his or her sexual identity is an evaluation of motor skills, the person may be asked if
threatened by a newly acquired disability and may try to as- there are any problems in such areas as sexual positioning.
sert sexuality through jokes, flirting, or even passes toward The topic need not be pursued any further by the therapist,
the therapist. In these cases it is important for the therapist but when the client is ready to deal with the subject area, he
to realize that what is often being looked for is the confirma- or she will probably remember that the therapist brought it
tion that the client is still a sexual and sensual human being; up and may be a person to approach when dealing with these
thus the therapist’s response is very important.106,170,171,173,174 issues.163,170,178
If the therapist rejects or even ridicules the client, it may be Other ways of presenting sexual information are to have
a very long time before the client can even think of attempt- literature available on the client’s ward so that those who are
ing such a confirmation of personal attractiveness. The client interested may pursue the topic in private, to have a group
may feel that because the therapist rejects the client and the discussion (interested clients, clients and significant others,
therapist is familiar with the disabled, there is little chance or whatever group the client and therapist might choose to
anyone who is not familiar with the disabled could accept assemble), or to have literature in the department waiting
the client as lovable.175 The therapist should not be surprised room.
by such advances and should deal with the situation in a It is important for the therapist to be aware of some of the
professional manner. The therapist should also realize that aspects of sexuality that may or may not affect the client as
approximately 10% of the population is homosexual and be a result of trauma or disease. Fertility is seldom affected in
prepared for advances from clients of the same sex. The women.179-183 Men, on the other hand, may experience dys-
therapist needs to be as professional as possible in acknowl- function of the penis and testicles and/or fertility.55,69,184-186
edging this client as with any other. All of the therapist’s Devices may be used and adapted to allow for sexual
gratification of the client (masturbation) or significant others.
Stimulant drugs such as sildenafil citrate (Viagra) or other
*References 73, 106, 148, 171, 173, 174. aids may be used to enhance a person’s sex life. Sensation
should be checked and sexual activities modified (or the changes in relationships, and facilitate philosophical, physi-
client should be alerted to the problem) to avoid breakdowns cal and spiritual growth.”198 Turner and Cox198 also felt that
or medical complications. Positioning modifications may be the medical staff could facilitate “recognizing the worth of
needed to allow for better energy conservation, joint protec- each individual, helping them to envision a future that is full
tion, motor control, maintenance of muscle and skin integ- of promise and potential, actively involving each person in
rity, and pleasure. Clients may have questions regarding their own care trajectory, and celebrating changes to each
modifications that may be needed for the use of birth control person’s sense of self.”198 Man199 observed that each family
devices or contraindications regarding the use of such copes differently in relation to a brain-injured family mem-
devices. Clients may also need equipment (e.g., vibrators) ber and that the family’s structure should be explored to
modified if hand function is involved. Complications that develop intervention guidelines. It has also been noted that
may affect function and mobility of the client may arise as a health care professionals should view the situation from the
result of pregnancy. Delivery may present some unique situ- family’s perspective to approach and support the family’s
ations that may also need to be addressed. After delivery the adaptation.200 This should be done to help the client and
disabled parent may require modifications to the wheelchair, the family accept the disability but at the same time to help
or consultations may be needed to achieve an optimal level them keep the negative views of society in perspective.70
of function in the parenting role. All of these possibilities In general, it has also been found that family support is a
point to the fact that sexual issues must be dealt with significant factor in the client’s subjective functioning201,202
throughout the treatment of all individuals with disabilities, and that social engagement is productive.89,203 According
whether the functional limitations are progressive, stable, or to Franzén-Dahlin, Larson, and colleagues,204 enhancing
correctable.175,179,187 The therapist may approach these needs psychological health and preventing medical problems in
or aspects of function while taking a client’s sexual history. the caregiver are essential considerations to enable individu-
Clients have repeatedly called for more attention to be paid als with disabilities to continue to live at home. Their
to sexual concerns. This is not sex counseling or therapy, and research found that evaluating the situation for spouses of
the therapist should not try to deal with deep psychosexual stroke patients was an important component when planning
issues. The therapist must be informed and needs to provide for the future care of the patient.
information that relates to the therapist’s areas of expertise, When working with children it is important to realize that
especially because other medical personnel may not have the they often feel responsible for almost anything that happens
knowledge to correctly analyze the components of some of in life, such as divorce, siblings getting hurt, or general argu-
these activities.45,55,69,175,182,183,188-190 ments between parents. It is important that the therapist help
Any of these issues may present themselves during the the client and the siblings realize that they are not respon-
medical screening phase of evaluation, whereas others be- sible for the client’s condition. Part of this magical thinking
come issues as the patient is adjusting to and questioning that often appears is the concept that “bad things happen to
functional limitations caused by the disease or condition. bad people.” Thus, the child is bad because a bad thing has
Once the patient has identified the need for this informa- happened or the adult is bad just because the disease or
tion, the therapist, whether through referral, group work, or trauma has occurred. It is important to be sensitive to this
individual discussions, needs to address the questions and ideation and help dispel this maladaptive thought pattern
must not deny the patient answers because the therapist is because it is not true or productive for the client, the sib-
uncomfortable. lings, parents, or spouses within a family and may cause
All the clinical problem areas that need assessment and further adjustment problems later in treatment. Siblings of
evaluation and that have been mentioned previously are the client should be helped to see their roles as good siblings
examined in relation to treatment planning in the clinical and should not be placed in the role of caretakers of a sibling
setting in the following sections. with special needs. In this way all children can grow natu-
rally without any one of the children being overly focused
Support System on. At the same time, it is a fact of life that the disabled child
Earlier literature hinted that partner relationships may be will probably need physical assistance, therapy, increased
negatively affected by a member being disabled. Within the medical care, and thus more time devoted to him or her, and
last few years this concept has been questioned in regard to this is just a fact of life.
some disabilities such as adult-onset spinal cord injuries,189 It should always be noted by the medical establishment
whereas pediatric spinal cord injury and other disabilities that having a disability is expensive in ways that we are
may result in relationship problems.191,192 It has been shown often not aware of. There are the obvious medical costs of
that adjustment and quality of life can be adversely affected therapy, surgery, drugs, wheelchairs, or orthoses, but there
by the physical environment being inadequate, thus making are other costs such as the possibility of extra cost of trans-
the person more dependent. The result of the dependence portation, catheters for urination, wheelchair maintenance,
appears to be poor relationships.193-195 This can also be seen adaptive clothing, and the like that are continuing costs not
with the families in which a member has had a brain in- covered by most insurance plans. These costs add up and
jury.196,197 In studies on muscular dystrophy it was found contribute to the emotional costs and demands on the family.
that physical dependence is not the only variable needing to The significant others may feel the need to work more to
be considered. Psychological issues need to be identified have the money to cover such expenses, but then that person
and considered as part of intervention.198,199 Recent litera- is not around to help out. This is but one of the many dilem-
ture has identified a number of elements that the client and mas that must be acknowledged for the support system of
the family may need help to work on, such as “to assist them the disabled person. The family may be encouraged to con-
to develop new views of vulnerability and strength, make tact such groups as the Family Caregiver Support Network
(www.caregiversupportnetwork.org) to get information and Parental Bonding and the Disabled Child
assistance with such diverse topics as being a caregiver, The parental bonding process is complicated and is still
legal and financial aid, and communications (this group being studied.48 The process may start well before the child
tends to focus on the adult but still may be a wonderful aid). is even conceived. The parents often think about having a
Such groups will give information to all who need it and child and plan and fantasize about future interactions with
help to empower the family. This takes the focus off of the the child; after conception the planning and fantasizing
medical condition and may help the family to gain a better, increase. During the pregnancy the mother and father
more balanced perspective on the condition. accept the fetus as an individual, and after the birth of
the child the attachment process is greatly intensified. The
Loss and the Family “sensitive period” is the first few minutes to hours after the
In this chapter the client’s support system is referred to as the birth. During this time the parents should have close
family. The family may be composed of spouses, parents, physical contact with the child to strongly establish the
children, lovers (especially in gay and lesbian relationships), attachment that will later grow deeper.216,217 There is an
friends, employers, or interested others such as church almost symbiotic relationship between mother and child
groups, civic organizations, or individuals. The people in the at this time: infant and mother behaviors complement each
support system may go through the same stages of reaction other (e.g., nursing stimulates uterine contraction). It is
and adjustment to loss that the client does.1,9,141,205-207 important at this point for the child to respond to the par-
ents in some way so that there is an interaction. In the early
Family Needs stages of bonding, seeing, touching, caring for, and inter-
The family will, at least temporarily, experience the loss of acting with the child allow for the bonding process. When
a loved family member from the normal routine. During the this process is disturbed for any reason, such as congenital
acute stage the family may not have concrete answers to malformations or hospital procedures for high-risk infants,
basic questions regarding the extent of injury, the length of problems may occur later.
time before the injured person will be back in the family When the parents are told that their child is going to be
unit, or possibly whether the person will live. malformed or disabled, it is a massive shock. The parents
During this phase, the family network will be in a state of must start a process of grieving. The dream of a “normal”
crisis.9 New roles will have to be assumed by the family child must be given up, and the parents must go through the
members, and the “experts” will not even tell them for how loss or “death” of the child they expected before they can
long these roles must be endured. If children are involved, accept the new child. Parents often feel guilty. Shellabarger
they will probably demand more attention to reassure them- and Thompson218 state that parents feel the deformed child
selves that they will remain loved. Depending on the child’s was their failure.1 The disabled child will always have a
age, the child will have differing capabilities in understand- strong impact on the family, sometimes a catastrophic
ing the loss (see the section on examination of loss). Each one.1,8,9,218,219 A study by Ha, Hong, Seltzer, and Green-
member of the family may react differently to bereavement, berg220 found that compared with parents of nondisabled
and each may be at a different stage of adjustment to the children, parents of disabled children experienced signifi-
disability (see the section on adjustment). One member may cantly higher levels of negative affect, poorer psychological
be in shock and deny the disability, whereas another mem- well-being, and significantly more somatic symptoms.
ber may be in mourning and may verbalize a lack of hope. Older parents were significantly less likely to experience
The family crisis that is caused by a severe injury cannot be the negative effect of having a disabled child than younger
overstated.98,206-209 parents.
Role changes in the family may be dramatic.64,74,92,210-212 In a study by Arnaud, White-Koning, and colleagues221
Members who have never driven may need to learn that greater severity of impairment was found to not always be
motor skill; one who has never balanced a checkbook may associated with poorer quality of life; in the moods and
now be responsible for managing the family budget; and emotions, self-perception, social acceptance, and school
those who have never been assertive may have to deal environment domains, less severely impaired children
forcefully with insurance companies and the medical appeared to be more likely to have poor quality of life. Pain
establishment.9,57,173,213,214 was associated with poor quality of life in the physical and
The family may feel resentment toward the injured mem- psychological well-being and self-perception domains.
ber. This attitude may seem justified to them because they see Parents with higher levels of stress were more likely to
the person lying in bed all day while the family members must report poor quality of life in all domains, which suggests
take over new responsibilities in addition to their old ones. In that factors other than the severity of the child’s impair-
a study by Lobato, Kao, and Plante,215 Latino siblings of chil- ment may influence the way in which parents report quality
dren with chronic disabilities were at risk for internalizing of life.
psychological problems. The medical staff may not always Parents must be encouraged to express their emotions,
understand the stress that family members are under and may and they must be taught how to deal with the issues at hand.
react to the resentment expressed either verbally or nonver- Techniques for accomplishing these goals are discussed in
bally with a protective stance toward the client. Siding with the later sections.48,217,219
“hurt” client may alienate the family from the medical staff
and may also drive a permanent wedge between family mem- The Child Dealing with Loss
bers. This long-term situation may undermine the compliance If a parent is injured, the young child may experience an
of family members’ involvement in home programs and ulti- overwhelming sense of loss. Child care may be a problem,
mately the successful outcome of long-term intervention. especially if the primary caregiver is injured. The child will
probably feel deserted by the injured parent and may de- approach emphasizing life skill development can, however,
mand the attention of the remaining parent. This will in- be used to acquire daily living skills.
crease the strain on all family members.64 The adolescent appears to react differently from other age
If the child is the client, his or her life will have under- groups to the knowledge of his or her own terminal illness.
gone a radical change: every aspect of the child’s world will The adolescent often feels that he or she has gone through a
have altered. Loved objects and people will help to restore very painful process (initiation) that will soon lead to the
the child’s feeling of security. It is of major importance to “joys and rights” of adulthood. Unlike persons in older age
explain to the child in very simple terms what is going on groups, who might feel that they can look back and gain
and to allow the child the opportunity to express feelings solace from the past, the adolescent feels that he or she will
both verbally and nonverbally. It is important to use play and have what Britto and colleagues222 term “death before fulfill-
art as the medium of communication for children. ment” and thus may react by feeling cheated by life. This
The hospital setting is threatening to all people, but same pattern may occur with the disabled adolescent.223 The
children are especially susceptible to loss of autonomy, therapist must be acutely aware of these feelings so that
feelings of isolation, and loss of independence. Senesac therapy may be presented in the most effective manner for
(see Chapter 12) has stated that the severity of the disabil- the client to find challenge and fulfillment in life.224
ity is not as important a variable in the emotional develop-
ment of the child as are the attitudes of parents and family.2,8 Family Maturation
Parents must attempt to be aware of the child’s inability to The family also has a maturational aspect. If the injured
understand the permanence (or transience) of the loss of person is a child and if the family is young with dependent
function.8 They will also need to help the child feel secure children at home, the adjustment may not be the problem
by bringing in familiar and cherished objects. A schedule that it would be for a family whose children are older. In the
should be established and kept to promote consistency. latter case, parents have begun to experience freedom and
Play and art should be encouraged, especially types that independence, and they may find adjusting to a return to a
allow the child to vent feelings and deal with the new restricted lifestyle difficult or even intolerable. They may
environment. Any procedures or therapies should be pre- have the feeling that they have already “put in their time”
sented in a relaxed and playful way so that the child has and should now be free. If the disability interrupts the
time to think and to feel as comfortable as possible about child’s developmental process, future conflict may arise
the change. The parents may often need to be reminded because the parents will eventually want retirement, relax-
to pay attention to the children in the family without dis- ation, and freedom. Parents may feel guilty and try to
abilities during this acute stage. repress this normal response.
The reverse may also be true. The parents may be feeling
The Adolescent Dealing with Loss that the children have left them (“empty nest syndrome”), and
The adolescent is subject to all of the feelings and fears that they may be too willing to welcome a “dependent” family
other clients express. Adolescents are in a struggle to achieve member back into the home. This may lead to excessive de-
autonomy and independence, and they often are ambivalent pendence or anger toward the parents on the part of the client.
about these feelings. When an adolescent is suddenly injured All these factors must be taken into consideration by the
and has to cope with being disabled, it can be a massive therapist when therapy is presented to the client and family.
assault on the individual’s development.139,222 According to The therapist can develop a greater understanding of
research conducted by Kinavey,50 findings imply that youth the client and family by being aware of the normal human
born with spina bifida face biological, psychological, and developmental patterns. These patterns identify some of the
social challenges that interfere with developmental tasks of major hurdles that must be overcome in the client’s life.
adolescence, including identity formation. Therapists are urged
to direct intervention toward humanizing and emancipating Coping with Transition
the physical and social environment for youth with physical In the acute stage of a family member’s injury, the family
disabilities to maximize developmental opportunities and must be helped to deal with the crisis at hand. During this
potential while fostering positive identity. phase, the family must first be allowed to cope with the
Kingsnorth, Healy, and Macarthur49 stated that with emotional impact of what is happening with a loved one.
advances in health care, an increasing number of youth with Second, the family should be helped to see the situation as a
physical disabilities are surviving into adulthood. For youth challenge that if overcome will facilitate growth. Third, ad-
to reach their full potential, a number of critical life skills aptation within the family unit must occur for the situation
must be learned. Specific learning opportunities are impor- to be overcome.
tant, as youth with physical disabilities may be limited in the Brammer and Abrego224 have developed a list of basic
life experiences necessary to acquire these skills. Therapists coping skills that they have broken into five levels. In the
are in the unique position of fostering these kinds of envi- first level the person becomes aware of and mobilizes skills
ronments to encourage adolescents to engage in critical life in perceiving and responding to transition and attempts to
skills such as problem solving, decision making, goal set- handle the situation. In the second level the person mobi-
ting, critical thinking, communication skills, assertiveness, lizes the skills for assessing, developing, and using external
self-awareness, and skills for coping with stress. Life skills support systems. In level three the person can possess, de-
differ from instrumental daily living skills. Daily living velop, and use internal support systems (develop positive
skills are the activities required to function independently in self-regard and use the situation to grow). The person in
the community and include skills such as financial manage- level four must find ways to reduce emotional and physio-
ment, meal preparation, or navigation in the community. An logical distress (relaxation, control stimulation, and verbal
expression of feelings). In level five the person must plan information to clients on their medical condition, in-
and implement change (analyze discrepancies, plan new op- cluding its present status, prognosis, anticipated future
tions, and successfully implement the plan). Using this functional limitations, and when applicable, vocational
model, the therapist and family can evaluate the coping skill implications.” This may be done by helping the client
level of the family. The therapist and staff can then help and family access resources such as PubMed (www.
promote movement toward the next level of coping with the ncbi.nlm.nih.gov/pubmed) online or find medical refer-
transition. These levels are also broken into specific skills ences in the library.
and subskills so that the therapist can grade them further. 3. Providing clients with supportive family and group
One of the more damaging aspects of hospitalization to experiences. “These strategies permit clients (usually
all involved is that the hospital staff focuses on the disability with similar disabilities or common life experiences)
rather than on the individual’s strengths.76,206,225 Centering and, if applicable, their family members or signifi-
on the disability can lead to a situation in which client, fam- cant others, to share common fears, concerns, needs,
ily, and staff see only the functional limitations and not the and wishes.” This can be done in rather unobtrusive
potential ability of the client. ways such as scheduling clients with the same
Decentering from the loss of function will be examined disability at the same time so that they meet in the
further in this chapter. If the family relationship was positive waiting room or while doing group mat activities.
before the insult and if the client is cognitively intact, then Another option is hiring individuals with limitations
the focus must be directed toward the relationship’s strengths who are health care professionals and can discuss
as well as toward the client’s and family’s individual cogni- and role model positive behaviors and answer rele-
tive and emotional strengths.91 In the initial acute stage of vant questions from the client’s perspective. Remem-
adjustment, crisis intervention may help the family use its ber that clients are all potential teachers for you as
strengths and at the same time deal with the situation at well as other clients.
hand. 4. Teaching clients adaptive coping skills for successful
To adequately deal with the crisis, the family should do community functioning. “These skills include assertive-
the following: ness, interpersonal relations, decision making, problem
1. Be helped to focus on the crisis caused by the disabil- solving, stigma management, and time management
ity; identify the situation to stimulate problem solv- skills.” This would entail role-playing situations that
ing; identify and deal with doubts of adequacy, guilt, may occur in the community, such as an able-bodied
and self-blame; identify and address grief; identify person asking why the client is in a wheelchair; preach-
and deal with anticipatory worry; be offered basic ining to the wheelchair user because he or she must have
formation and education regarding the crisis situation; offended God in some way—otherwise the person
and be helped to create a bridge to resources in the would not be in a wheelchair; or telling a woman that it
hospital and in the community for support and to see is such a shame that she is disabled because she is so
their own family resources.48,226-229 good looking and could have found a man if it were not
2. Be helped to remember how they have dealt success- for the disability. Role playing can also be used to help
fully with crises in the past and to implement some of a person deal with the possibly awkward experience of
the same strategies in the present situation. going to bed with a new partner and having to explain
3. Work with the family as a unit during crisis to help how to be undressed, or what those tubes coming out of
strengthen the family and facilitate more positive the body are for, or what positions are best for someone
attitudes toward the client. These attitudes by the with this condition.
family will improve the client’s attitudes or feelings
toward the injury and hospitalization.92,212,230-233 En- ROLE OF THE THERAPEUTIC ENVIRONMENT
couraging family-unit functioning in this situation Whenever possible in therapy the functional activity should
will decrease the amount of regression displayed by be presented and structured to promote empowerment, prob-
the client. If the family is encouraged to function lem solving, and adjustment. Adjustment and adaptation
without the client, however, more damage than good to life form a dynamic process that allows for the person to
may be done.1,139 interact with life in a meaningful and productive way that
encourages the person to enjoy life (Figure 6-1).17,80,234-237
TREATMENT VARIABLES We see the client at a very stressful time, and we need to
IN RELATION TO THERAPY make this time as productive for the client and the family as
Skilled therapy intervention focuses on maximizing par- possible.
ticipation in functional activities, participation in life, and This section examines issues the therapist and staff
behavioral change. Livneh and Antonak134 promote the should know to create a therapeutic environment that will
following activities for the health professional: facilitate psychological adjustment and independence of
1. Assisting clients to explore the personal meaning of the the client with activity limitations. The physical and the
disability. “Training clients to attain a sense of mastery attitudinal environment of the treatment facility plays a
over their emotional experiences.” A way of doing this major role in the way the client views the services that are
would be to help the client not to demonstrate emo- rendered.
tional outbursts or to help the client look at his or her Recall a time before you became a member of the medi-
emotions and to put them into perspective. cal community. Think about how awe inspiring the people in
2. Providing clients with relevant medical informa- white coats were, how strange the smells of the hospitals
tion. “These strategies emphasize imparting accurate were, how busy it all seemed, and how puzzling the secret
True BOX 6-2n QUESTIONS TO HELP GAIN INSIGHT

adaptation INTO THE CLIENT AS A PERSON
Adaptation
Client is trying
Looks good The following questions can be asked:
and failing but
accepting What would this person be doing if he or she did not
have this condition?
What is stopping the person from reaching these goals
Disability now?
Superficial
Who will marry this person and why?
Looks good and Growth and
sounds good but acceptance
What are his or her positive traits?
is not dealing of change What will this person do for a living?
with change What will this person do for enjoyment?
How will this person bring others enjoyment?
What would this person be doing if there was no
disability?
How is the disability stopping the person from actual-
Maladaptation
Functional izing their goals? (These are the goals that need to be
Shows inability
flexibility and worked on.)
to function due
enjoyment Similar questions can be asked of the client to explore ways
to emotional
of life of helping the client have a meaningful life:
disability
What do you look like and function like now?
Figure 6-1 n Possible directions of the adaptive process. What will you look like after therapy?
What important things would you be doing now if you
were not in need of treatment?
What activities or forms of productivity were you in-
medical language was. It all seemed overwhelming then, volved in before, and which were important to you?
and it still is to newcomers, especially newly admitted pa- Which of these things do you still do?
tients and their families. The hospital usually appears imper- What if anything is preventing you from doing these
sonal,238 sterile, monotonous, and confusing, and all status things now?
accumulated outside the hospital means little inside. How does this condition affect your being a lover of life,
The therapist needs to take the setting into account when family, significant other, and so on?
dealing with the client. The environment can be altered in a How will this condition affect your important life goals,
variety of ways. Therapy staff could wear street clothes, your activities, and your ability to do meaningful
decorate the department or hospital with posters and lively activities?
colors, and allow clients to bring some personal items into How much different would your life look if it were not
the hospital. for this condition?
The nature of the therapy process can often lead the Will any of the above be stopped by your condition, and
therapist to see only the disability and not the person—as if so how?
occurs, for example, when a client is referred to by his or her
disability rather than by name. This stereotyping of those
with disabilities can lead the therapist to concentrate on the
lack of abilities rather than on the strengths of the clients. and the therapist deals with the individuals with similar
The real danger is that the client and family will also start to problems all the time, then there must not be anything
focus on the functional limitations of the client and feel that good about me.” Positive, sincere comments to client and
their family relationship is now permanently altered. The family can add a motivational factor to treatment that may
accuracy of this perception may have to be evaluated as part have been missing.76,232
of the adjustment process. The wife of a man with paraple- Providing opportunities to be outdoors can help clients
gia said with a sudden burst of insight, “I didn’t marry him cultivate a sense of connection with something that is larger
for his legs—this doesn’t change the relationship.” Often so than themselves. At the most basic level there is more oxy-
much attention is directed toward the disability that tunnel gen outside, and in general the air is fresher than indoors.
vision develops. One way to try to get a better perspective is “And looking at a faraway horizon or sky can help us gain
to look at the bigger picture. A variety of questions can be needed perspective on our small world, bounded by our bod-
asked that may help the therapist gain a greater insight into ies and our lives.”241
the client as a person (Box 6-2). The last and possibly the most important aspect in creat-
After the therapist is aware of the strengths of the cli- ing an environment that will foster growth and adjustment in
ent, these strengths may be capitalized on in therapy to the client is a staff whose members are well adjusted and
help the client realize them and build confidence. Clients aware of their own personal needs. Just as coping skills are
often reported that they were not complimented in therapy necessary for the client, the staff, too, must be capable of
and especially that they never received feedback that their coping with the stresses of the emotional and physical pain
bodies were desirable239 or that they were doing things of the client and the client’s family. The therapist must also
correctly.11,76,175,176,240 A logical thought by the client is, deal with his or her own personal reactions to the sometimes
“If the therapist cannot see anything desirable about me, devastating situations others are in.76,232,242 Exposure to such
situations often elicits introspection on the part of the staff Rogers and Figone143 developed the following sugges-
that can result in emotional turmoil for staff members and tions that could benefit the therapist when trying to create a
affect their own personal relationships. This emotional en- supportive environment:
ergy needs to be directed in a productive way so that the 1. It is helpful to use the same staff member to de-
energy does not turn into chaos within the staff interaction velop the relationship and to provide continuity
or become a destructive force for the client. of care.
To decrease the possibly distractive nature of this 2. Concerned silence is most appreciated, although push-
emotional energy, the staff should be made aware of their ing is sometimes necessary.
own coping styles, and they should be allowed to vent 3. Staff members should anticipate the need to repeat
their reactions to particularly distressing client case loads information graciously.
in a positive, supportive group. Group meetings can be 4. Cumbersome, hard-to-repair adaptive equipment should
used to handle some of the inevitable tension, especially not be used after discharge.
if there is a respected member who is skilled in group 5. Give clients responsibility so that they feel they have
work. This is not a psychotherapy session (although psy- some control over therapy.
chotherapy may be warranted in some situations) but a. The client should be allowed to pick his or her own
rather an opportunity to test reality and remove tension advocate from the team.
before it is incorrectly directed toward fellow staff mem- b. The client should be given a choice of activities
bers. These sessions can make use of the four elements (e.g., which exercise comes first).
of crisis intervention mentioned in the previous section, c. Professionals should avoid placing the client
as well as information from others.177,232 Other times in an inferior status. In time the client starts
that this stress reduction can be achieved are in supervi- thinking this way (feeling like a “second-class
sion or during coffee breaks, as long as the sessions are citizen”).
productive. 6. Psychological support is attributed to noncounseling
The staff can use these sessions to better understand their personnel. Personal matters are better discussed with
various reactions to stress and to explore their coping staff members with whom the client has developed
styles.211,232,243,244 Ideally, this knowledge of coping styles a relationship.2,173,227
and stress reduction will decrease staff burnout and aid the 7. Willingness to allow the client to try and fail is more
staff to help clients and their families deal with stress more helpful than controlling the client.
successfully.139,240-244 There are also MBSR courses held in Bolte-Taylor109 developed “forty things I needed the most”
most hospitals, universities, and communities and can be during her rehabilitation for her stroke. Here are a few:
found online at www.umassmed.edu/cfm/mbsr. 1. I am not stupid, I am wounded. Please respect me.
The need to have a staff that is supportive is of para- 2. Come close, speak clearly, repeat yourself if neces-
mount importance because the attitude of rehabilitation sary, and enunciate.
personnel has emerged as one of the chief motivating 3. Approach me with an open heart, slow your energy
factors in rehabilitation.1,92,137 In fact, the use of humor down, take your time.
has been found to be assistive in the process of adjust- 4. Be aware of what your body language and facial
ment. In a study by Solomon,245 aging well was related to expressions are communicating to me.
aspects of humor. It seemed to affect aging well through 5. Make eye contact with me, encourage me.
its relationship with perceived control. Physical health, 6. Honor the healing power of sleep.
satisfaction with housing, and relationships with family 7. Protect my energy. No talk radio, TV, or nervous
and friends were also positively influenced by humor. One visitors! Keep my visitations brief.
suggestion by McCreaddie and Wiggins246 is that stress 8. Speak to me directly, not about me to others.
can be coped with through distraction, which lessens the 9. Clarify for me what the next step or level is so I
negative physical effects of stress. Humor is also known to know what I am working toward.
have a number of potential benefits in relation to interper- 10. Celebrate all of my little successes. They inspire me.
sonal skills or social support.247 Specific aspects such as
empathy, intimacy, and interpersonal trust have all been CONCEPTUALIZATION OF ASSESSMENT
positively correlated with a sense of humor and subse- AND TREATMENT
quently with interpersonal relationships. According to
McCreaddie and Wiggins,246 a degree of rapport with the Assessment
patient is necessary before humor can be used, and humor The one component that weaves through all of Rogers
should be used only after a level of empathy, caring, and and Figone’s143 seven points is the need for the therapist
competence has been clearly demonstrated. This interac- to be involved with the client in a therapeutic relation-
tion of therapy and societal interactions explains why the ship—that is, to know where the client is “coming from.”
World Health Organization model went from a disability To know where the client is coming from is to be aware
or handicap model to a model of functional ability and of and sensitive to the person’s total psychosocial frame
participation in life. Although the International Classifica- of reference.2,227
tion of Diseases (ICD-9 or ICD-10) deals with physicians The therapist who knows his or her own beliefs, refer-
and disease categories, therapy clearly separates itself into ence points, and prejudices can evaluate whether an
a clear model that stresses the strength of an individual assessment result or treatment sequence reflects the cli-
and his or her potential to participate in and have quality ent’s needs and values or those of the therapist. In the first
of life (www.who.int/classifications/icf). half of this chapter, several assessments were discussed
that could be summarized into the following three major Problem-Solving Process
components: The family unit, including the client, should be encouraged
1. Preinjury to take active control over as much of the client’s care and
a. Values and prejudices (value systems, culture, and decision making as possible.* This can be done in every
prejudgments) of the client and family members phase of the rehabilitation process. A family conference
before the injury with the rehabilitation staff should actively involve the client
b. Developmental stage of the client and family members and family in all stages of planning and treatment, up to and
c. Cognitive level of the client and family members including discharge. The family (including the client) should
d. Ability of the client and family members to handle be briefed ahead of time to prepare questions that they want
crisis answered or problems that need to be addressed. Rogers and
2. Components to be evaluated leading to adjustment Figone143 report that conferences with family members that
a. Loss and grief process for the client and family excluded the client engendered suspicion2,211; therefore if
members the client is capable, the client may educate the family in
b. Adjustment process for the client and family members regard to what is happening in the hospital and in rehabilita-
c. Transitional stages for the client and family members tion. Conversely, family involvement facilitates and shortens
d. Role changes for the client and family members the rehabilitation process and encourages reintegration into
e. Age or cognitive level of client and family mem- the community.8,207,249,253 The family can also be educated
bers9,139,227,248 regarding the side effects and interactions of medication
f. Sexual adjustment for the client and spouse with publications such as the Physicians’ Desk Reference.258
3. Techniques used to elicit adjustment and independence Later in the rehabilitation process the client and family can
a. Crisis intervention strategies be encouraged to arrange transportation services, find and
b. Letting the client and family take control evaluate housing, and supervise attendant care. All these
c. Expression of emotion, both verbally and nonverbally activities allow the client and the family to be more in con-
d. Problem solving trol of the environment and thus to feel independent.
e. Role playing In the context of one-on-one therapy, giving choices can
f. Praise foster client responsibility and independence. Making a de-
g. Education cision about the order of treatment activities (such as on
h. Support groups which side of the bed to transfer out of or which direction to
Once an assessment has been made of the client and fam- roll one’s wheelchair first) can give the individual a sense of
ily members’ stages of psychological adjustment, the cli- self-worth that can continue to grow. This will cultivate a
ent’s occupational history and roles, and their preinjury at- belief by the client and family that they are strong, with
titudes and beliefs, a treatment protocol can be established. rights that need to be met. Moving out of the role of the
This protocol will need to incorporate steps toward stage victim, the client begins to exercise responsibility and to
change and possibly attitudinal change. Because these take action, such as applying for extended health benefits or
changes require learning on the part of the client and family, getting a second consultation when an important medical
an environment that optimally facilitates these changes must decision needs to be made. If the client and family start to
be established.* realize that they do not have to be a casualty of the medical
Therapy can be seen as a form of education in which the establishment and if they find ways to control the medical
client and the client’s family are taught how the client establishment,92,234,259 they are better able to discard the role
should use his or her body. The education process is not of victim.
limited to the physical aspects of therapy, however. The cli- In some centers, such as the occupational therapy clinic
ent is also taught how to look at and think about the body at San Jose State University, clients have been taught the art
and the disability. If the staff is nonverbally telling the client of self-defense to make sure that they never have to fall into
and the family that the client is not capable of making deci- the victim (dependent) role. It should be noted, however,
sions and of being independent, it follows that the client that this knowledge on the part of the client and family can
may indeed feel dependent and incapable of making deci- be used in ways that the therapist may not always agree
sions. Giles211 and others207,211,249,252,253 stated that there was with. At such times it may help to adopt a philosophical
an inverse relationship between independence and distress. attitude toward the situation and to view it as a positive
Distress causes further anxiety and decreases the learning direction for the client in terms of moving from victim to
potential of the client. There are ways, however, for the advocate in the rehabilitation process.
therapist to encourage independence on the part of the client The steps of crisis intervention, which were mentioned in
and his family. the previous section, can be used to help the family under-
stand and analyze their needs in the crisis situation. Once the
Specific Therapeutic Interventions family has discovered that they are in crisis, they will then
“Engagement in leisure-like activities may not only help be able to create strategies that they can use to overcome
people ‘feel better’ in the immediate context of coping with present and future problems.
rehabilitation treatments, but may help sustain coping ef- Problem solving is another element the therapist may use
forts as individuals learn to live with ongoing functional to help the client and family gain independence and control.†
limitations.”254
*References 19, 22, 52, 208, 211, 248-250, 252, 253, 255-257.
*References 9, 75, 92, 98, 195, 210, 249-251. †
References 19, 52, 207, 211, 249, 250, 252, 253, 255, 256.
Persson and Rydén22 acknowledged the importance of this have been called for throughout the literature.* Throughout
when they found that there were a few significant categories the therapeutic process, the client and the family need to be
to adjustment: self-trust, problem-reducing actions (problem praised frequently, and credit needs to be given for the gains
solving), change of values, and social trust. Acknowledge- made by the client and family members. Granted, the thera-
ment of reality and trust in oneself was found to be sig- pist may have engineered the gains, but the family and client
nificant, and they identified the importance of understand- are the ones who need the reinforcement. As Bolte-Taylor109
ing coping processes from the disabled person’s point of suggested, celebrate all of the little successes—they can
view. In a phenomenological study by Bontje, Kinébanian, help inspire the client and their family. Through gratifying
Josephsson, and Tamura,260 participants stated they used experiences the family will unite to overcome the disability.
already familiar problem-solving strategies and personal They need to know that they can survive in the world with-
resources as well as resources in their social and physical out having the medical staff constantly there to solve the
environments to identify prospects of potential solutions and family’s problems. In short, they need the strategies and re-
to create solutions to overcome constraints on occupational sources that will allow them to be independent outside the
functioning. medical model.
Rather than having the client routinely learn how to ac- Yet another way to encourage independence can be ap-
complish a specific task, the client or family must be en- plied to working with parents of disabled children.13 The
couraged to think through the process from the problem to parents should be educated about normal and abnormal
the solution and to accomplishment of the task. To achieve growth and development, including physical, cognitive,
this activity analysis, the client would have to know the and emotional growth, so that the family can maintain
basic principles behind the activity143 and may then be some perspective and objectivity about their child’s vari-
responsible for educating the family. An example of this ous levels.8,92,167,236 The parents can then better understand
would be a transfer from the wheelchair to the toilet. If the the needs of those children with disabilities and those
therapist simply has the client memorize the steps in the without in the family. Armed with this knowledge, the
task, the client or family members will not necessarily be parents and children will not be frustrated with unreal ex-
able to generalize this procedure to a transfer to the car. If pectations or unreal demands. Education of the parents
the client learns the principles of proper body mechanics, could take place at local colleges, at the hospital, or even
work simplification, and movement, the client or family in a parent’s group.
member may be more able to generalize this information to
almost any situation and to solve problems later when the Support Systems
therapist is unavailable.252 Rogers and Figone143 have Groups are often used to increase motivation, provide sup-
noted that although the client and family may fail at times port, increase social skills, instill hope, and help the client
during these trials, the therapist should let them be as inde- and family realize that they are not the only ones who have
pendent and responsible as possible: let them try it their a disabled family member. This will help the client and fam-
way, even if they are not successful the first time. ily establish a more accurate set of perceptions about the
Pictures or slides of a restaurant, movie theater, or public disabled individual and allow for greater independence of
building can be used to facilitate discussion and problem the client and family.1,9,92,135,261,265 Problem solving can be
solving by the family unit when analyzing potential archi- encouraged and value systems can be clarified. Client or
tectural barriers in the environment. Thus in the future family support groups can be used to relieve pressure that
when the family is presented with a problem or a barrier, might otherwise be vented in therapy. Lawrie Williams, a
they will have the resources to overcome it rather than be mother of two daughters who experienced serious medical
devastated by it. challenges, is the author of a series of articles about parental
Role playing in combination with support groups can roles in family-centered care. One article in particular high-
also be used to defuse potentially painful situations and lights the role parents can play in helping other families
operate independently. While the client is still in the safe through parent-to-parent support programs. Williams first
environment of the rehabilitation setting, simulations of experienced the support of another parent when one of her
incidents can be created for them to practice problem solv- daughters was young, and later realized she could use
ing with supervision to help anticipate potential situations. her own experiences professionally. For the past 6 years,
They can be asked what they would do when a stranger Williams has been the coordinator of the Parent Support
(possibly a child) approaches the client and asks why he or Program at the Center for Children with Special Needs,
she is in a wheelchair or is disabled or what they would do Children’s Hospital and Regional Medical Center in Seattle,
when a waiter asks the family member to order for the dis- Washington.266
abled client. All of these situations are potentially devastat- Livneh and Antonak134 found that in a chronic-care
ing for all involved; however, if role playing and support ward family involvement helped the client and the family
groups are used in advance to help all members of the fam- improve their status. Schwartzberg249 and Schulz128 and
ily (client included) to satisfactorily handle and feel in others1,98,107,248,267-269 have reported great success in the use
control of the situation, the family will not be as likely to of support groups with individuals who had brain damage.
be traumatized by a similar occurrence. The result is that Support groups can also be used to educate the client about
the family will not be as inclined to be overwhelmed by the client’s disability to increase independence.†
social situations and will be able to socialize in a much
freer, more gratifying way.77,79,261
Cognitive-behavioral therapy has been used for clients *
References 91, 226, 229, 236, 249, 262-264.
and spouses with success.93-95 Psychosocial support groups †
References 1, 207, 248, 253, 270, 271.
Kreuter and colleagues271 and Taanila and colleagues13,270 therapy situations to reteach the client appropriate behav-
found that independent physical functioning and knowledge ioral and social interaction skills. A technique called dialec-
about one’s condition were exceedingly important in mov- tical behavioral therapy has been used with people with
ing through the phases of the rehabilitation process.75,234,265,272 mental health disorders, and it appears to be a promising
A guide to facilitating support groups has been published by approach. One study by Miller and colleagues278 found sig-
Boreing and Adler,274 and it has been found to be useful, nificant reductions in suicidal symptoms; the most highly
especially by laypeople establishing such groups.* rated skills included distress tolerance and mindfulness
skills. The goals of a dialectical behavioral therapy program
The Adult Client with Brain Damage designed for individuals with mild traumatic brain injury
The adult client with brain damage and the needs of the fam- include decreasing the individual’s self-defeating behaviors
ily will be specifically, yet briefly, examined here. Brain and cognitions, cultivating understanding of the individual’s
damage can affect the cognitive, perceptual, emotional, so- abilities and impairments, and increasing behavioral and
cial, and neurological systems of the individual and can be cognitive skills that will lead to a greater sense of self and
incredibly disruptive and catastrophic to the client’s and feelings of self-esteem. The program is designed to improve
family’s lives. When a person sustains a brain injury and is each patient’s ability to accept his or her life as it is and to
hospitalized, emotional support for the family (client in- function independently.279
cluded) is the primary need to be met initially. The therapist Better follow-up care needs to be implemented when
should attempt to convey warmth and a caring attitude, es- dealing with the adult with brain damage.1,98,107,248,267-269 In
pecially during the family’s initial contacts.275 Typical com- some areas there are outpatient, privately funded programs
plaints about the acute period involve impersonal hospital that can help support the brain-injured individual and his or
routines and lack of definite information about the patient’s her family on discharge from hospital settings. These re-
status.13,92,175,229,276 Unfortunately, definite information is sources must be recommended for follow-up care as needed.
usually not available at the earliest stages. It may not be possible for the client and family to con-
Later the family must deal with the physical changes in stantly come to the clinic for support and follow-up, but
the client’s body; what may be even more injurious to the telephone conversations can be scheduled on a periodic ba-
family are the psychological, cognitive, and social changes sis, or the exchange of letters or audiotapes can also be used.
in the client.† People with cerebrovascular accidents have With the increased availability of video recorders, the day
been found to be more clinically depressed than orthopedic may come when a follow-up may be performed on video-
patients are. The libido263 and the emotional systems are tapes and sent via the Internet by clients living in rural areas.
also affected.75,176-178,226 It has further been shown that per- Support groups are being used increasingly to facilitate cli-
sons who survive a cerebrovascular accident or other im- ent and family adjustment and accommodation to disability,
pairment and who have a full return of function do not return as well as reentry into the community.*
to normal life because of a lack of social and emotional
skills.‡ Families of cerebrovascular accident victims have References
also reported that social reintegration is the most difficult To enhance this text and add value for the reader, all refer-
phase of rehabilitation.277 Lack of socially appropriate be- ences are included on the companion Evolve site that ac-
haviors has been one of the most troublesome complaints of companies this textbook. This online service will, when
people who deal with the person with a traumatic brain in- available, provide a link for the reader to a Medline abstract
jury.176 Therapists may be able to help alter this syndrome for the article cited. There are 281 cited references and other
by encouraging appropriate behavior and by structuring general references for this chapter, with the majority of
those articles being evidence-based citations.
* *
References 1, 16, 92, 232, 248, 239, 270, 280.
References 75, 92, 98, 207, 249, 270-274.
†
References 1, 8, 9, 16, 18, 19, 262.
‡
References 1, 9, 92, 135, 261, 265.
CASE STUDY 6-1 n PUTTING EVALUATIONS AND TECHNIQUES INTO PRACTICE

Joan, a married 30-year-old woman, has had a T2 spinal cord family members are probably in Sheehy’s281 “catch-30”
injury. She has worked as a computer programmer for the past stage, in which the person reevaluates his or her life and
8 years, except for a short maternity leave when she gave birth relationships. Joan already “knows” that the physically
to her daughter, who is now 6 years old. Joan was always very disabled cannot enjoy a physically active life and is also
active physically and often stated that she felt sorry for her feeling that everything she has worked for in her career is
physically disabled neighbor because the neighbor could not lost. She appears to be in the mourning stage of adjustment.
hike, be active, or enjoy the outdoors. Joan’s husband, age Her daughter and husband have to adjust to radical role
33 years, is attempting to visit Joan regularly and care for their changes. Cognitively, Joan’s young daughter is not going
daughter, a role that is new for him. to understand the permanence of the disability and may be
The therapist has assessed several things regarding Joan’s inclined to act out as the result of the turmoil. The husband
developmental stage, adjustment stage, social and cultural will have to be assessed to determine his stage of adjustment
influences, and family adjustment reactions. The two adult to her disability.
Continued
CASE STUDY 6-1 n PUTTING EVALUATIONS AND TECHNIQUES INTO PRACTICE—cont’d

The therapist has determined that Joan’s transfers need further self-reinforcing. Within this treatment session, the therapist has
work but would like to use the adaptive process to stimulate ad- used the recreational therapist as a role model to change Joan’s
justment. The therapist has devised a treatment session to meet prejudice against the disabled being active in the outdoors and to
the goals of promoting the defense stage of adjustment, decreas- show Joan that she can still be a parent although she is disabled.
ing Joan’s prejudice against the disabled, encouraging problem The therapist may also increase Joan’s knowledge of how to
solving, increasing her feelings of self-worth, proving to her that interact with children from a wheelchair by giving a few hints
she can take care of her daughter through interacting with chil- and then having Joan transfer up a set of stairs to reach one of
dren, and having her decenter her focus from her disability to her the children.
ability. The therapist has contacted the recreational therapist (who If we want to carry this scenario further, the therapist could
has paraplegia) to plan a collaborative session at the park across introduce Joan to a child who is interested in computers and
the street from the hospital. Because the recreational therapist who needs help with a programming problem (Joan’s computer
works in the pediatrics ward, it is determined that the children background will be used, which will increase Joan’s feelings of
with spina bifida should come and play tag, transferring from log self-worth and help her focus on her abilities rather than her
to log in the playground. disabilities). On the way back to the ward, the therapist and
The stage is now set. Joan will be asked to help supervise the Joan may discuss how the family is dealing with the crisis they
children. The adaptive process will be used to teach Joan how to are in and help her realize how the family has made it through
transfer using the environment. The transfer will be organized other crises in the past and how those previously successful
subcortically because she will be attending cortically to the chil- strategies could be used in this situation. Support groups and
dren’s needs and to the game itself. Joan will be actively affecting psychological counseling are mentioned as resources. The ses-
her personal environment, and if everything goes well, the act of sion may end with Joan planning the next therapy session and
helping the children will increase her self-worth and will also be thus starting to take control of her life.
CHAPTER 7 Differential Diagnosis Phase 1:
Medical Screening by the Therapist
WILLIAM G. BOISSONNAULT, PT, DHSc, FAAOMPT, FAPTA,
and DARCY A. UMPHRED, PT, PhD, FAPTA

Differential Diagnosis Phase 1 After reading this chapter the student or therapist will be able to:
Differential Diagnosis Phase 2 1. Identify the difference between Differential Diagnosis Phase 1, medical screening,
Medical screening and Phase 2, diagnosis of impairments and functional limitations.
Patient referral 2. Analyze the concept of body system and subsystem screening.
Review of systems screening 3. Develop a mechanism for body system screening to be used with clients with
Causational systems interaction preexisting neurological dysfunction.
4. Analyze the significance and importance of performing a medical screening for all
clients who interact in a therapeutic environment with occupational or physical
therapists.
5. Differentiate between direct causation of a clinical symptom as opposed to system
causation of a clinical problem.
T raditionally, the term differential diagnosis has referred

to a process used by physicians to diagnose disease.
This process typically involves three distinct steps. Step 1 is
wellness not only has changed the words used by therapists
but also incorporates external societal limitations that our
clients face.4 These changes do not affect the way a thera-
taking a thorough history, including an investigation of the pist should medically screen before formulating a clinical
patient’s medical history, presenting complaints, and a re- diagnoses based on movement dysfunction. These conditions
view of systems. Step 2 is the performance of the physical are inherent in the interrelationships among impairments,
examination. This history and the findings of the physical functional or activity limitations, and participation in life
examination will lead to a diagnosis or to step 3, the identi- and are appropriate for physical or occupational therapy
fication of necessary tests, including laboratory tests, diag- interventions.2,3,5,6
nostic imaging modalities, and so on. The goal of the three The process of differentiating the cluster of findings that
steps is the formulation of a specific diagnosis that will lead warrant communication with a physician regarding concerns
to the implementation of the appropriate medical treatment about a patient’s health status compared with those that do
and an accurate prognosis. not will be called Differential Diagnosis Phase 1.7 In this
For the professions of physical and occupational therapy scenario a physician will ultimately diagnose the patient’s
the concepts associated with and use of the term differential illness, but the PT’s and occupational therapist’s (OT’s)
diagnosis are still evolving and under debate. A recent edito- examination findings and subsequent patient referral con-
rial describes diagnosis in physical therapy as complex and tribute to the diagnosis being generated. For many of these
controversial, with diverse views existing.1 For physical illnesses, the use of advanced imaging, laboratory testing,
therapists (PTs), the guiding premise is that the differential and/or tissue biopsy is necessary for the diagnosis to be
diagnostic process fits within the Patient/Client Management made.8 Numerous examples exist in Physical Therapy
Model described in the Guide to Physical Therapist Practice2 Journal and Journal of Orthopaedic and Sports Physical
(Figure 7-1) and within The Guide to Occupational Therapy Therapy of published case reports and case series describing
Practice.3 The therapist attempts to organize the history and such action taken by PTs.
physical examination (including tests and measures) findings If the decision is reached that the symptoms and signs do
into clusters, syndromes, or categories. There are certain fall within the scope of practice of PTs and OTs, a second
clusters of findings that suggest the presence of disease or level of differential diagnosis occurs. Now the therapist at-
an adverse drug event and warrant communication with tempts to categorize the examination findings into the specific
a physician. There are other symptoms and signs that are diagnostic categories that will specifically guide the choice of
consistent with conditions that still fit into the older disable- treatment interventions and the development of a prognosis.
ment framework. In the world today, the model of choice This second level of diagnosis is called Differential Diagnosis
of all therapists is the World Health Organization (WHO) Phase 27 and is the focus of Chapters 8 and 9. Figure 7-2
International Classification of Functioning, Disability and illustrates where Differential Diagnosis Phase 1 and Phase 2
Health (ICF), which moves away from the consequences of fit into the Patient/Client Management Model.
disease classification to a health focus classification. Thus a The purpose of this chapter is to discuss the medical
shift in how one looks at disease and its impact on health and screening components associated with Differential Diagnosis
163
Diagnosis
Define clusters, syndromes, or
categories to determine the most
appropriate intervention
Prognosis
Evaluation Determine the level of
Clinical judgments based improvement and the
on data collected required time
Intervention
Methods and techniques
Examination Outcomes to produce changes
Data collection: Results of patient/client consistent with the
History, tests, and measures management diagnosis and prognosis
Figure 7-1 n Patient/client management model. (Adapted from American Physical Therapy
Association: Guide to physical therapist practice. Phys Ther 81:43, 2001, with permission of the
American Physical Therapy Association.)
Phase 1, including identification of patient health risk factors, the differences between direct causation of movement
recognition of atypical symptoms and signs, review of sys- dysfunction pain syndromes arising from disease versus a
tems, and within-systems review. Methods to collect this in- system causation that may or may not be directly connected
formation during a patient examination are also presented. to a specific disease. The therapist referral often plays a
The critical importance of therapists developing these visual critical role in providing the doctor the patient behaviors
and analytical skills is that they can lead to identification of observed as system causation with or without a disease
DIFFERENTIAL
DIAGNOSIS
PHASE 1 PHASE 2
Refer/Consult Diagnosis
Therapist initiates commun- Data organized into defined
ication/referral of patient to clusters, syndromes, or
physician. categories.
Prognosis
Evaluation Intervention
Examination Outcomes
Figure 7-2 n Patient/client management model showing Differential Diagnosis Phase 1 and Phase 2.
(Modified from Umphred DA [Chair]: Diagnostic Task Force, State of California, 1996–2000, California
Chapter of American Physical Therapy Association.)
CHAPTER 7 n Differential Diagnosis Phase 1: Medical Screening by the Therapist 165
classification. Patient case scenarios are used to illustrate the

Review medical history/patient profile
important medical screening principles.
DIFFERENTIAL DIAGNOSIS PHASE 1: HISTORY/INTERVIEW
MEDICAL SCREENING Symptom investigation/functional limitations

The Guide to Physical Therapy Practice2,9 and The Guide to
Occupational Therapy Practice3 clearly describe the thera- Review medical history
pists’ responsibility to refer patients/clients with health con-
cerns to other practitioners. The emphasis of the following Review of systems
discussion is detecting clinical manifestations that suggest the (general health)
specific need for physician intervention. Typically the initial

warning signs associated with these scenarios include a recent Review of systems
(specific systems)
onset or exacerbation of symptoms such as pain, weakness,
numbness, dizziness, falls, confusion, and so on—common Cardiovascular system Psychological disorders Nervous system
Endocrine system
complaints of patients with neurological disorders. Therapists Pulmonary system
Urogenital system Rheumatic disorders
Integumentary
system
may also detect symptoms or signs unrelated to the primary Gastrointestinal
system
medical neurological condition but that could be related to an PHYSICAL EXAMINATION
existing comorbidity or a medication side effect. In addition,
a general health and wellness screen may reveal a need for Alteration of symptoms Impairments Cardiovascular system
Pulmonary system
a psychological, dermatological, or other nonneurological Nervous system
medical consultation. Integumentary
system
As opposed to Phase 2, the goal of Differential Diagnosis EVALUATION OF FINDINGS
Phase 1 is not to formulate a specific diagnosis on the basis

of these clinical manifestations. A therapist’s Phase 2 diag- Treat Treat and refer Refer
nosis is primarily a group of motor behaviors representing

movement dysfunction and how it limits independence in
life activities and an individual’s ability to participate in life. Response for treatment
The Phase 1 process identifies signs and symptoms that are Figure 7-3 n Patient examination scheme. (Taken from notes
health or disease and pathology driven and, when they have from course by W. G. Boissonnault, 1998.)
been identified, directs a referral to a medical specialist. In
fact, providing a specific diagnosis or labeling a cluster of
examination findings when referring a patient to a physician Identifying Patients’ Health Risk Factors
because of health status concerns (e.g., peptic ulcer disease, and Previous Conditions
endometriosis, new or progressive neurological problems) Owing to the considerable overlap in symptomatic presenta-
could place the therapist outside the scope of his or her tion of impairment-related conditions and those requiring
practice. Having the ability to formulate such a specific physician examination, identifying existing health risk fac-
systemic, neurological, or visceral disease or pathology diag- tors for occult diseases is important. Numerous factors have
nosis is not necessary to meet the responsibilities described an effect on the patient’s risk for compromised health status,
in the Guides to Practice. Once the therapist’s concerns have including age, sex, race, occupation, leisure activities, pre-
been communicated, it is then up to the physician to diag- existing medical conditions, medication usage (over-the-
nose the presence of such disease entities. counter and prescription drugs), tobacco use, and substance
The purpose of the therapist’s medical screening is to abuse or the interaction of some of these conditions, and
(1) identify existing medical conditions, (2) identify symp- family medical history.
toms and signs suggesting that an existing medical condition Of these, a personal history of a current or recent medical
may be worsening, (3) identify neurological manifestations condition, current medication use, and a positive family his-
that suggest an acute or life-threatening crisis, and (4) iden- tory (e.g., mother and aunt with a history of breast cancer,
tify symptoms and signs suggestive of the presence of an father diagnosed with prostate cancer at the age of 58 years)
occult disorder or medication side effect. This medical screen- are the most relevant risk factors for the potential presence
ing has always taken place within the clinical framework of of an occult condition. For example, the history of a previ-
PTs’ and OTs’ practices, but as practitioners become more ous episode of depression significantly increases the risk of
autonomous, this screening must become more comprehen- a second episode compared with the risk that someone who
sive, requiring tools and documented evaluation results. has never had an episode of depression will have his or her
Figure 7-3 is an example of an examination scheme leading to first such episode.10 The greater the number of existing risk
the decision to treat the patient, to treat and refer the patient, factors, the more vigilant the therapist should be for the
or to refer the patient. Phase 2 may also include the decision presence of warning signs suggestive of disease and the
to refer the patient to another practitioner (e.g., dietician, more extensive the other medical screening components will
social worker, clinical psychologist) for services augment- need to be. Those increased risk factors, whether within one
ing the therapy or to social programs such as wellness clin- system or multiple systems, can lead to clinical behaviors
ics that will encourage the patient to participate in move- that are the summation of the systems problems and their
ment activities even though he may need individualized interactions that affect movement. Physicians should be able
therapeutic intervention. The following material focuses on to depend on the therapist to recognize these interactive
the components of this scheme most directly related to the symptoms and refer the patient back to either the referring
medical screening process leading to a patient referral. physician or to another specialist.
There are different methods to collect this medical his- be overlooked by therapists working with patients who have
tory and patient profile information, including a review of neurological involvement and signs and symptoms (e.g.,
the medical record and use of a self-administered question- weakness, numbness) that are much more debilitating and
naire, depending on the practice setting and patient popula- cause more functional limitations than the pain complaints do.
tion. Figure 7-4 is an example of a self-administered ques- Although investigating pain complaints may not be the initial
tionnaire that could be completed by the adult patient, a priority for these therapists, at a later visit such questioning is
family member, or a caregiver. As noted in Figure 7-3, a very important, especially if it continues, increases in intensity,
quick scanning review of this information should occur, if shifts, or enlarges its region with no causation. Effective
possible, before the patient interview is begun. The therapist medical screening involves the interpretation of a patient’s
will have a head start in organizing the history and physical description of symptoms, functional limitations, and the cor-
examination, knowing what to prioritize and at least initially responding physical examination findings. Descriptions of
what parts of the examination can be deemphasized. The symptoms associated with neuromusculoskeletal impairments
utility and accuracy of a self-administered questionnaire in (loss or abnormality of physiological, psychological, or ana-
patient populations germane to therapists’ practice, similar tomical structure or function) generally reveal a fairly con-
to the one illustrated in Figure 7-4, have been described, with sistent and predictable pattern of onset and change over a
the conclusion that such a tool can be a valuable adjunct to defined period of time. In addition, the neurological and
the oral patient interview.11 MSK impairments noted during the physical examination
Affirmative answers to previous or current illness ques- should match with the functional limitations described by
tions should direct the therapist to consider what the poten- the patient or the caregiver. If these expectations are not met,
tial impact may be on the patient’s symptoms, choice of it does not necessarily mean the patient has cancer or an
examination and treatment techniques, rehabilitation poten- infection, but doubt should be raised on the therapist’s part
tial, and risk for additional illness. For example, the pres- whether therapy is indicated.
ence of existing chronic kidney disease (e.g., renal failure) Patients many times are not aware that presenting symp-
should alert the therapist to numerous potential complica- toms or signs suggest a condition better addressed by a
tions including patient fatigue, weakness, and impaired physician as opposed to a PT or an OT. For example, Mr. S.
concentration, all of which could interfere with rehabilita- had a cerebrovascular accident 6 months ago with resultant
tion efforts. Chronic renal failure is also marked by pares- mild residual left hemiplegia. At the time of discharge from
thesia and muscle weakness, which could mistakenly be rehabilitation services he was independent in all activities
associated with other neurological conditions. Renal osteo- of daily living, but residual left upper extremity weakness
dystrophy is yet another complication associated with remained. When visiting his internist for a routine checkup,
chronic renal failure. The concern of compromised bone he complained that over the prior 3 weeks he had lost some
density should direct the therapist to use techniques that functional skills and was having difficulty with self-care.
carry a reduced risk of skeletal injury. A series of follow-up The physician then referred Mr. S. to the therapy clinic for
questions for the affirmative answers will assist the thera- evaluation and treatment. Mr. S. states he has been less
pist in determining the relevance (if any) of each item (see active and just needs some help regaining his motor func-
Figure 7-5 for examples of follow-up questions for selected tion. During the history taking he states that he is experienc-
information categories). ing a deep, dull, aching sensation in the lower lumbar spine
Having the self-administered questionnaire completed and right buttock. He assumes it has developed as a result
before the scheduled time of the initial visit will improve the of his inactivity and thus saw no reason to bother the physi-
therapist’s efficiency. Mailing the questionnaire to the patient cian with this problem. As Mr. S. continues to describe his
before the visit or having the patient arrive 10 to 15 minutes difficulties, he also notes a constant deep ache in the right
before the appointment would allow for the form’s comple- shoulder that he relates to increased use of his right arm
tion without taking time away from the actual examination to compensate for the left arm weakness. The physical
itself. Once the questionnaire has been completed, taking examination of the low back, pelvis, and right shoulder re-
1 to 2 minutes to scan it before the interview should be all veals that the existing symptoms do not vary with active
that is necessary for the therapist to begin formulating ques- or passive range of motion, resisted testing, or postural
tions and organizing the physical examination. The inability holding. In addition, quantity of motion is normal for these
of the patient to recall information or complete the question- regions and motor programming appears intact. At this point
naire may be another sign that medical clearance is necessary the therapist cannot explain the symptoms from an impair-
before progression to Phase 2. ment standpoint; therefore, depending on other examination
findings, including the patient profile and medical history,
Symptomatic Investigation of Functional communication with the internist may be warranted. The
Restriction following information describes some of the subcategories
The chief presenting symptoms or functional restriction associated with symptom investigation.
typically provides the reason for therapy services being
sought and can provide the initial warning sign(s) of potential Location of Symptoms
medical issues needing to be addressed. Despite pain not A body diagram can be a valuable tool to document the
typically being the chief complaint of many patients with pri- location of symptoms expressed verbally or nonverbally by
mary neurological conditions, a relatively mild pain is often patients with identified neurological deficits. Besides pain
the initial complaint associated with a serious pathological and altered sensation, patterns of abnormal tone, asymmetri-
condition; a dull diffuse ache is often the initial presenting cal posturing, and areas of weakness can also be noted on
complaint associated with tumors of the musculoskeletal the body diagram (Figure 7-6). Numerous body structures
(MSK) system.12 This relatively minor complaint can easily are potential pain generators, including visceral structures.
Medical History Questionnaire

Name:____________________________________________ Age ___________________________
SS#: ________________________ Occupation: __________________________________________
Leisure activities: _______________________________________________________________________
Has anyone in your immediate family (parents, sisters,

I. Are you currently being seen by any of the following professionals:
A. General medical doctor (MD) Yes No
brothers) ever been treated for any of the following:
B. Medical specialist (MD) Yes A. Stroke
No Yes No
If yes: please specify ____________________________
B. Seizure disorders Yes No
C. Osteopathic doctor Yes No
C. Parkinson’s disease Yes No
D. Physical/occupational therapist Yes No
D. Multiple sclerosis Yes No
E. Chiropractor Yes No
E. Other neurologic problems _____________ Yes No
F. Psychiatrist/psychologist Yes F. Mental illness
No Yes No
G. Alternative medical practitioner Yes G. Cancer
No Yes No
If yes: please specify ____________________________
H. High blood pressure Yes No
If you have been seen by any of the above practitioners within the
I. Heart condition Yes No
last year, please discuss the reasons: J. Breathing problems Yes No
_______________________________________________________ K. Diabetes Yes No
_______________________________________________________ L. Arthritic disease Yes No
_______________________________________________________ M. Kidney disease Yes No
N. Anemia Yes No
II. Have you EVER been diagnosed as having the following O. Vascular problems Yes No
condition(s)? P. Thyroid problems Yes No
A. Stroke Yes No Q. Skin problems Yes No
B. Seizure disorders Yes No R. Chemical dependency (e.g., alcoholism) Yes No
C. Migraines Yes No S. Learning disabilities Yes No
D. Other neurologic problems: Yes No T. Cognitive dysfunction Yes No
Specify ________________________________________ U. Genetic disorders Yes No
E. Depression Yes No
F. Cancer: Specify ____________________ Yes No Please list any PRESCRIPTION medications you are currently
G. High blood pressure Yes No taking (include pills, injections, patches, etc.)
H. Heart condition Yes No __________________________________________________
I. Emphysema Yes No __________________________________________________
J . Asthma Yes No __________________________________________________
K. Tuberculosis Yes No __________________________________________________
L. Diabetes Yes No
M. Rheumatoid arthritis Yes No Please list any OVER-THE-COUNTER MEDICATIONS you are
N. Other arthritic disease Yes No taking:
O. Kidney disease Yes No __________________________________________________
P. Anemia Yes No __________________________________________________
Q. Hepatitis Yes No __________________________________________________
R. Circulatory problems Yes No __________________________________________________
S. Thyroid problems Yes No
T. Skin problems Yes No Please list any prescriptions or over-the-counter medications
U. Digestive problems Yes No you were taking prior to your current problems
V. Bowel or bladder problems Yes No __________________________________________________
W. Chemical dependency (e.g., alcoholism) Yes No __________________________________________________
X. Unexplained falls Yes No
Y. Cognitive dysfunction Yes No
Z. Genetic disorders Yes No How much caffeinated coffee or other caffeinated beverages
AA. Other ___________________________ Yes No do you drink per day? (number of cups/cans/bottles) ________
Please list all surgeries/hospitalizations including dates and reasons. Do you smoke? Yes No
Date Surgery/hospitalization/reason If yes: How many packs per day? ________
_____ _____________________________________________
_____ _____________________________________________ Do you drink alcohol?
If yes: How many days per week
Are you being or have you been treated for musculoskeletal injuries do you drink? ________ days/week
(fracture, dislocations, repetitive strains, joint instability)? If so, If yes: How many drinks per sitting? ________ drinks/sitting
please state: (Note: one beer or one glass of wine equals 1 drink)
Date Injury
_____ _____________________________________________ If you use marijuana or other
_____ _____________________________________________ substances, how often? ________ days/week
Are you being or have you been treated for neuromuscular problems
(weakness, pain, spasticity, incoordination, dizziness, tremor)?
If so, please state:
Date Injury
_____ ________________________________
_____ ________________________________
Figure 7-4 n Self-administered questionnaire to collect medical history information. (Modified

from Boissonnault WG, Koopmeiners MB: Medical history profile: orthopaedic physical therapy
outpatients. J Orthop Sports Phys Ther 20:2–10, 1994, with permission of the Orthopaedic and
Sports Sections of the American Physical Therapy Association.)
Medical History Follow-up Form

Figure 7-7 and Table 7-1 illustrate local and referred pain
patterns from various visceral organs. Although the pre-
1. Have you EVER been diagnosed as having the following condition(s)? sented pain patterns illustrate those most commonly noted,
Heart condition Yes No clinicians should be aware of other potential patterns. For
Follow-up questions:
example, ischemic heart disease—the complaint of left chest
• Describe your heart condition?/What is your heart condition? wall and left upper extremity pain, pressure, or tightness—is
• Current problem or previous problem and now fully resolved?
• If a current problem not the classic presentation for women and many of the
-Which doctor is following you for this condition?
-When is your next follow-up visit?
elderly. Besides what is noted in Figure 7-7 and Table 7-1,
-What symptoms do you experience when your heart problem acts up? pain from the heart can also be experienced in the right
-How do you treat your problem if it’s acting up?
-How does your heart problem interfere with your daily activities/lifestyle? shoulder or biceps, jaw and tooth, epigastric, and interscapu-
-Has the MD placed you on any restrictions owing to your heart problem?
lar regions.13,13a
2. Has anyone in your immediate family (parents, sister[s], brother[s]) ever been treated Because there is so much overlap between pain locations
for any of the following:
associated with visceral disease and neuromusculoskeletal
Cancer Yes No
conditions, the results obtained in and of themselves have
Follow-up questions: minimal use in differentiating MSK from non-MSK condi-
• Who in your family has the history of cancer?
• What type of cancer? tions. Being familiar with the visceral pain patterns will be
• At what age were they diagnosed?
• What is their current health status?
extremely important, however, when deciding which body
• Does anyone else in your family have cancer? systems to screen during the review of systems. Besides not-
ing where symptoms are located, it is equally important to
Figure 7-5 n Potential follow-up questions for affirmative answers document areas of no complaints (see Figure 7-6). Once the
on the self-administered questionnaire. (From W. G. Boissonnault, patient has reported symptoms (e.g., low back and right but-
course notes, 1998.) tock aching, see Figure 7-6), therapists should clarify.
Screening to eliminate the possibility of symptoms being
present down the back and up the front of the legs; in the
pelvis, stomach, chest, neck and face areas; or between the
shoulder blades and in the arms is critical. If there is one
Figure 7-6 n Body diagram illustrating symptom location. Body areas with no known symp-
toms or abnormalities are marked with a checkmark. (From Boissonnault WG, editor: Examina-
tion in physical therapy practice—screening for medical disease, ed 2, New York, 1995, Churchill
Livingstone.)
Figure 7-7 n Possible local and referred pain patterns of visceral structures. (From Boissonnault
WG, editor: Examination in physical therapy practice—screening for medical disease, ed 2, New York,
1995, Churchill Livingstone.)
body area so involved that all the patient’s and practitioner’s thinking whether physical or occupational therapy is what
attention is focused on it, a relatively mild but potentially the patient truly needs.14
serious symptom may be overlooked elsewhere. Placing a In general, when symptoms such as weakness or numb-
checkmark over each body region devoid of symptoms or ness associated with primary neurological conditions are
other abnormal findings is one way to document such infor- investigated, the 24-hour reference point to assess symptom
mation and record change over time. change is not realistic. Except for an acute onset or exacer-
bation, these symptoms tend not to fluctuate that quickly
Symptom Pattern with change in posture or position. Understanding the
Aspects of the patient’s chief complaint other than symptom pathogenesis of primary neurological disorders will allow
location are very relevant to the process of differential diag- for detection of symptom change unusual for the patient.
nosis, in particular a description of how and when the symp- This will lead to follow-up questions to determine whether
toms changed over a defined period of time. Complaints of this change may represent a medically serious situation.
pain, paresthesia, and numbness associated with primary Similarly, a change in the biomechanical alignment of a
MSK conditions typically change in a consistent manner joint (e.g., the shoulder), may immediately alter the patient’s
over a 24-hour period. The patient will report that the symp- pain response, indicating a direct relationship between MSK
tom intensity increases with the assumption of specific imbalance in joint stabilization and gravitational pull, for
postures such as left side lying or sitting or with specific which therapy would be appropriate.
activities such as walking, driving, or 2 hours of computer
work. Conversely, patients typically can relate paresthesia or History of Symptoms
pain relief with avoiding certain postures or activities, the The therapist must also scrutinize the patient’s report of
assumption of certain postures, wearing an arm sling, and so the onset of the symptoms. Pain and paresthesia or numb-
on. Night pain investigation also falls under this subcategory ness associated with neuromusculoskeletal impairments
of patient data. Pain that wakes an individual from sleep and typically can be related to trauma, either on a macro or a
for which changing positions in bed does not provide relief micro level, or to a medical event such as a cerebrovascular
is more concerning than if the pain is positionally related. accident. More often than not it is repetitive overuse or
If the pattern of symptom aggravation and alleviation is cumulative trauma that leads to tissue breakdown and
that there is no consistent pattern, such as pain that comes inflammation (see Chapter 18). Patients with neurological
and goes independently of the patient’s posture, activities, impairments resulting in postural abnormalities and abnor-
or time of day; night pain is the patient’s most intense pain; mal movement patterns are at risk for such conditions. If a
or paresthesia or pain moves from one body region to patient’s symptoms are truly insidious, meaning not related
another inconsistently with common pain referral patterns or to macro or micro trauma, or there has not been a signifi-
identified medical conditions, then the therapist should start cant change in activity level that reasonably accounts for
TABLE 7-1 n VISCERAL PAIN PATTERNS
STRUCTURE SEGMENTAL INNERVATION POSSIBLE AREAS OF PAIN REFERRAL

PELVIC ORGANS
Uterus including uterine ligaments T10-L1, S2-4 Lumbosacral junction
Sacral
Thoracolumbar
Ovaries T10-11 Lower abdominal
Sacral
Testes T10-11 Lower abdominal
Sacral
RETROPERITONEAL REGION
Kidney T10-L1 Lumbar spine (ipsilateral)
Lower abdominal
Upper abdominal
Ureter T11-L2, S2-4 Groin
Upper abdominal
Suprapubic
Medial, proximal thigh
Thoracolumbar
Urinary bladder T11-L2, S2-4 Sacral apex
Suprapubic
Thoracolumbar
Prostate gland T11-L1, S2-4 Sacral
Testes
Thoracolumbar
DIGESTIVE SYSTEM ORGANS
Esophagus T6-10 Substernal and upper abdominal
Stomach T6-10 Upper abdominal
Middle and lower thoracic spine
Small intestine T7-10 Middle thoracic spine
Pancreas T6-10 Upper abdominal
Lower thoracic spine
Upper lumbar spine
Gallbladder T7-9 Right upper abdominal
Right middle and lower thoracic spine, including
caudal aspect scapula
Liver T7-9 Right middle and lower thoracic spine
Common bile duct T6-10 Upper abdominal
Middle thoracic spine
Large intestine T11-12 Lower abdominal
Middle lumbar spine
Sigmoid colon T11-12 Upper sacral
Suprapubic
Left lower quadrant of abdomen
CARDIOPULMONARY SYSTEM
Heart T1-5 Cervical anterior
Upper thorax
Left upper extremity
Lungs and bronchi T5-6 Ipsilateral thoracic spine
Cervical (diaphragm involved)
Diaphragm (central portion) C3-5 Cervical spine
Modified from Boissonnault WG, Bass C: Pathological origins of trunk and neck pain, I. Pelvic and abdominal visceral disorders. J Orthop Sports Phys Ther
12:192–207, 1990, with permission of the Orthopaedic and Sports Sections of the American Physical Therapy Association.
the complaints, the therapist should again be concerned movements and functional loss. Often, that means a thera-
about the source of the symptoms. A worsening of symptoms pist must have a working professional relationship with a
(e.g., numbness, weakness, spasticity, swelling) associated clinical pharmacist (see Chapter 36). Use of a general health
with an existing medical condition should be investigated by checklist (Box 7-2) can assist the therapist in prioritizing the
the therapist with the same scrutiny. The therapist always inclusion of the checklists in the systems review checklists
needs to ask, “Is there a reasonable explanation for the box during the initial visit. The symptoms noted in this check-
worsening?” An increase in the intensity of the complaints list can be associated with disease of most of the body’s
or the involvement of additional body regions could signal systems, as well as with systemic disease and adverse drug
a progression of the disease. events.
If the patient or caregiver (on the patient’s behalf) replies
Review of Body Systems yes to any review of systems question, the therapist must
By design, review of systems screening allows the therapist to determine whether there is a reasonable explanation for the
detect symptoms secondary (and maybe unrelated) to the complaint, whether the physician is aware of the complaint,
reason therapy has been initiated.15 The review of systems and, if so, whether the complaint has worsened since the
allows for a general screening of body systems for symptoms patient last saw the physician. When the given explanation
suggesting the presence of an adverse drug reaction, occult is not satisfactory, the physician is unaware of the com-
disease, or worsening of an existing medical condition. Sus- plaint, or the symptom is worsening, communication with
picions of any of these scenarios would warrant communica- the physician is warranted. Similarly, most physicians look
tion with a physician. Checklists of symptoms and signs for at direct causation: complaint to disease. Therapists need to
each body system can be used by the PT or OT during the look at system causation because we see the end result of the
patient interview (Box 7-1). To keep the checklists manage- combinations of the problems: disease, maturation, environ-
able in length, the therapist should investigate presenting mental factors, and other nondisease causations. All the
complaints and symptoms and the patient’s medical history checklists do not need to be covered during the initial visit.
before the review of systems, as noted in Figure 7-3. For If the patient says “no” for each of the general health items,
example, on review of the cardiovascular and peripheral vas- the patient’s health history is uneventful, and the therapist is
cular system checklist items associated with heart conditions comfortable with the description of the chief complaints
in Box 7-1, important items appear to be omitted, such as (including pattern and onset), then the therapist can proceed
chest pain, claudication, a history of heart problems, hyper- with the evaluation of specific impairments and functional
tension, high cholesterol levels, and circulatory problems. If limitations with some confidence that Differential Diagnosis
symptoms have already been investigated by use of a body Phase 2 and therapy intervention are very likely appropriate.
diagram, the therapist would already know whether the The review of systems then takes a lower priority. The result
patient has chest pain. If symptom change (aggravation or is that the therapist could decide to delay the use of the ap-
alleviation) over a 24-hour period has already been investi- propriate systems review checklists until the patient’s sec-
gated, the therapist would know whether claudication is an ond or third visit. If the patient answers “yes” to general
issue. Finally, if the patient’s medical history has already health items and has an inconsistent pain pattern, the appro-
been discussed, the therapist would know whether heart priate review of systems then takes a higher priority and
problems, hypertension, or circulatory problems existed.13a should be covered during the initial visit.
All of the checklists in Box 7-1 need not be used for
every patient. The location of symptoms will direct the Musculoskeletal System
therapist in deciding which checklists should be included in Box 7-3 provides the checklist for the MSK system. In
the initial examination. Figure 7-7 and Table 7-1 can be used addition, as with all other body systems, the general health
to link pain location with visceral systems that could be the checklist also provides a level of screening for conditions of
source of the complaints. Table 7-2 provides a summary of the MSK system such as infections, metastatic cancers, and
potential pain locations and diseases of the pulmonary, car- rheumatic disorders (e.g., rheumatoid arthritis). Identifying
diovascular, gastrointestinal, and urogenital systems. Other patient risk factors for these conditions is a key for recogniz-
symptom characteristics can also alert the therapist to the ing when to be suspicious. For example, those at highest risk
possible involvement of the endocrine, nervous, and psycho- for MSK cancers are those (1) over the age of 50 years and
logical systems. Symptoms, including pain and paresthesias under 20 years, (2) having a previous history of cancer (e.g.,
that come and go irrespective of posture, activity, or time breast, lung, prostate, thyroid, and kidney—the most com-
of day and that appear to move among the various body mon cancers to metastasize to the axial skeleton), (3) having
regions, can be associated with these systems as well as the a positive family history of cancer, and (4) having had expo-
visceral systems. In addition to the identification of the loca- sure to environmental toxins. Those individuals at highest
tion and characteristics of symptoms, a patient’s medical risk for MSK infections report or demonstrate (1) current
history will also help the therapist decide which systems to or recent infection (e.g., urinary tract, tooth abscess, skin
screen. A positive medical history, such as a heart problem, infection), (2) history of diabetes with use of large doses of
would direct the therapist to investigate the patient’s condi- steroids or immunosuppressive drugs, (3) elderly age, and
tion, including possible use of the cardiovascular and periph- (4) spinal cord injury with complete motor and sensory
eral vascular checklist as well as the questions listed in loss.16 Last, the primary risk factors for rheumatoid arthritis
Figure 7-5. The therapist also needs to be aware of the include (1) female sex, (2) age (peak) 30 to 40 years, and
medications taken by the patient to medically manage these (3) positive family history.17
pathological conditions. Similarly, therapists need to be The other category of MSK conditions for which thera-
able to analyze how the drugs potentially affect functional pists need to be vigilant is fractures. The pain and deformity
BOX 7-1 n REVIEW OF SYSTEMS CHECKLISTS
CARDIOVASCULAR AND PERIPHERAL VASCULAR PSYCHOLOGICAL (DEPRESSION)

dyspnea depressed or irritable mood
orthopnea psychomotor agitation or retardation
palpitations apathy
pain with sweating sleep disturbance
syncope weight gain or loss
peripheral edema fatigue
cold feet or hands feelings of worthlessness
peripheral sensory loss impaired concentration
skin discoloration suicide ideation (recurrent)
open wounds or gangrene recent loss of family member
cough PULMONARY
GASTROINTESTINAL dyspnea
difficulty with swallowing onset of cough
heartburn, indigestion change in cough
specific food intolerance sputum
bowel dysfunction hemoptysis
color (black and tarry, light slate colored) clubbing of the nails
frequency stridor
shape, caliber (flat ribbon-like, thin-caliber pencil-like) wheezing
constipation or diarrhea SENSORY AND MOTOR (NERVOUS)
incontinence sudden or slow onset of sensory loss
UROGENITAL impaired balance
urinary unexplainable frequent falls
frequency impaired gross movement patterns
urgency decrease in or difficulty with fine motor skill
incontinence impaired mentation
reduced force of stream tremors: intentional or unintentional
difficulty initiating or attention needed to urinate muscle atrophy: symmetrical vs asymmetrical
dysuria asymmetrical facial features
color (hematuria, dark, brownish tea color) asymmetrical tongue patterns
REPRODUCTIVE: MALE facial contour
urethral discharge ptosis
impotence pupil abnormalities
dyspareunia strabismus
REPRODUCTIVE: FEMALE ENDOCRINE
vaginal discharge arthralgias
dyspareunia myalgias
change in menstruation neuropathies
frequency and length of cycle cold or heat intolerance
dysmenorrhea skin or hair changes
blood flow fatigue
date of last period weight gain or loss
number of pregnancies polyuria
number of deliveries polydipsia
menopause
associated with most sudden-impact, traumatic fractures malabsorption syndrome, and long-term corticosteroid, hep-
make for an obvious presentation. However, trauma suffi- arin, anticonvulsant, and cytotoxic medication use. The most
cient to cause a fracture may not be so obvious in a patient common locations for such fractures include vertebral bod-
with decreased bone density. Lifting a gallon of milk, expe- ies, the neck of the femur, and the radius. Observation of
riencing a mild slip or bump, or trying to open a window posture and body position may provide a clue that some-
that is stuck may be sufficient to cause a fracture in a patient thing may have changed structurally. For example, with
with a history of chronic renal failure, multiple sclerosis, vertebral compression fractures the thoracic kyphotic curve
rheumatoid arthritis, hyperparathyroidism, gastrointestinal may be accentuated, accompanied by a very pronounced
TABLE 7-2 INKING PAIN PATTERNS

L n patient may report a specific event or time of onset of
AND VISCERAL SYSTEMS symptoms, and a pain pattern of increasing pain with
weight bearing on the involved extremity over time and
PAIN LOCATION VISCERAL SYSTEMS lessoning relief of pain with assumption of non–weight-
bearing positions—all typical findings with impairment-
Right shoulder (including shoulder Pulmonary
driven symptoms. The therapist may also be able to pro-
girdle) Cardiovascular
voke symptoms during the physical examination as the
Gastrointestinal
involved bony area is mechanically loaded. When the his-
Left shoulder (including shoulder Cardiovascular
tory and physical examination findings are evaluated, an
girdle) Pulmonary
unusual finding or pattern will emerge, or the patient will
Upper thoracic or midthoracic spine Cardiovascular
not respond to treatment as expected, making the therapist
Pulmonary
step back and consider alternative hypotheses regarding
Gastrointestinal
the origin of patient symptoms, especially if the risk fac-
Lower thoracic and upper lumbar Peripheral vascular
tors listed earlier are present.
or midlumbar spine Pulmonary
Gastrointestinal Integumentary System
Urogenital
Screening of the integumentary system is not typically
Lumbopelvic region Gastrointestinal
based on the presence or absence of pain, paresthesia, or
Urogenital
numbness. As with the nervous system, some degree of
Peripheral vascular
screening of the integumentary system occurs with every
patient regardless of the presenting diagnosis. Skin cancer
has the highest incidence of all the cancers,19 and therapists
generally see a number of exposed body areas during the
BOX 7-2 n GENERAL HEALTH CHECKLIST postural assessment and regional examination that make up
the physical examination. In fact, as noted in Figure 7-3,
Fatigue
screening the skin begins during the patient interview.
Malaise
During the interview the therapist can be looking at areas of
Fever, chills, sweats
exposed skin such as the face, neck, arms, and feet. As with
Nausea
screening of the other body systems, the therapist’s goal is
Unexplained weight change
not to identify a melanoma or differentiate squamous cell and
Dizziness, lightheadedness
basal cell carcinoma but simply to identify skin lesions with
Unexplained paresthesia, numbness
atypical presentations. Once the patient has been referred to
Unexplained weakness
the physician, disease will be ruled out or diagnosed. Box 7-4
Unexplained cognitive and emotional changes
can be used to assess any mole or other skin marking. The
items noted are atypical for a benign lesion, more suggestive
of a pathological condition.20 Selected items from Box 7-4 have
been highlighted, resulting in an acronym—A (asymmetry),
B (borders), C (color), D (diameter), and E (evolving)—that
BOX 7-3n MUSCULOSKELETAL SYSTEM has been used to educate the public for self-screening.21
SCREENING If the therapist notes any of these findings and the patient
reports a recent change in the size, color, or shape of the
Insidious onset of symptoms lesion and that a physician has not looked at the lesion, a
Atypical pain pattern (aggravating or alleviating factors) referral would be warranted.
Night pain (progressive and/or nonpositional) Besides skin lesions, abnormal general skin color can be
Early morning stiffness lasting longer than 30 to 60 minutes a manifestation of a number of conditions. Table 7-3 sum-
Inadequate relief of symptoms with rest or rehabilitation marizes abnormal skin color changes. Occasionally, some of
Inability to alter symptoms during the physical examination
Lack of impairments that match patient’s functional
limitations
Atypical physical examination findings (e.g., masses, unex-
n SKIN LESION SCREENING—
BOX 7-4
plained atrophy, or weakness)
PATHOLOGICAL CHARACTERISTICS
Multivariant color
Black or blue-black color
Irregular borders
apex of the curve that was not present before. With femoral Nondistinct (“fuzzy”) borders
neck fracture the lower extremity is often positioned in Size: 6 mm or larger in diameter
external rotation and appears shortened compared with its Asymmetrical shape
counterpart.18 Friable tissue
Causing potential confusion for the clinician are dis- Ulcerations
eases (especially in the early stages) of the MSK system, Evolving (changing size, shape, color)
which may mimic mechanical MSK conditions. The
TABLE 7-3 n ABNORMAL COLOR CHANGES OF THE SKIN
COLOR CHANGE PHYSIOLOGICAL CHANGE COMMON CAUSES

White, pale (pallor) Absence of pigment or pigment changes Albinism, lack of sunlight
Blood abnormality Anemia, lead poisoning
Temporary interruption or diversion of blood Vasospasm, syncope, stress, internal bleeding
flow
Internal disease Chronic gastrointestinal disease, cancer, parasitic disease,
tuberculosis
Blue (cyanosis) Decreased oxygen in blood (deoxyhemoglobin) Methemoglobinemia (oxidation of hemoglobin), high
blood iron level, cold exposure, vasomotor instability,
cerebrospinal disease
Yellow Jaundice, excess bilirubin in blood, excess bile Liver disease, gallstone blockage of bile duct, hepatitis
pigment (conjunctivae are also yellow)
High levels of carotene in blood (carotenemia) Ingestion of food high in carotene and vitamin A
Gray High level of metals in body Increased iron, bronze-gray; increased silver, blue-gray
Brown (hyperpigmentation) Disturbances of adrenocortical hormones Adrenal pituitary
Addison disease
From Shapiro C, Skopit S: Screening for skin disorders. In Boissonnault WG, editor: Examination in physical therapy practice—screening for medical disease,
ed 2, New York, 1995, Churchill Livingstone.
the most obvious abnormalities are the most difficult to note the observed abnormalities. Covering the items in the
when one is so focused on items more directly related to nervous system checklist should add little time to the
therapeutic intervention. therapist’s initial examination. Assessing for facial asym-
metries and tremors can take place during the interview.
Nervous System Observing balance, movement patterns, and muscle atro-
As with the integumentary system, the nervous system is phy can occur while watching the patient ambulate into the
screened to a degree for all patients. The systems review examination area, during the interview, and as the patient
checklists in Box 7-1 include items that provide a very changes positions during the physical examination. Last,
gross, general screening of the nervous system. The thera- impaired mentation may become apparent during the inter-
pist should be vigilant for the presence of any of these view or the physical examination as the patient struggles
items in all patients during the initial and subsequent to appropriately answer questions or follow directions.
visits. For patients with preexisting findings from this Case Studies 7-1 and 7-2 illustrate the importance of this
checklist, the therapist must be vigilant for a worsening of general screening.
CASE STUDY 7-1

A 55-year-old elementary school teacher was referred with a and the patient consented to allow her primary care physician
diagnosis of cervical degenerative disk disease at C5-6 and (the referring physician) to be called to discuss the finding. The
C6-7. Her chief complaint was posterior cervical aching and a physician facilitated a referral of the patient to a neurologist.
sense of neck weakness. Functionally, the patient’s primary Approximately 1 month later, after the neurology consultation
concern was her increasingly difficult time making it through and tests, the patient was diagnosed with Parkinson disease.
her workday. She taught first-grade students, so much of her During that month the patient continued to receive physical
workday was spent with her neck and trunk in a forward therapy care for her cervical complaints. In this example,
flexed position. The patient stated that this persistent flexion performance of Differential Diagnosis Phase 1 showed the
posturing was a significant factor for the worsening of her presence of a new symptom (tremor of the right hand) that
symptoms as her workday progressed. As the interview was not consistent with the medical diagnosis of degenerative
continued, a tremor of the patient’s right hand and forearm disk disease. This symptom triggered the decision by the
was observed as the arm rested on her thigh. When questioned therapist to refer the patient back to the physician for that
about the observed tremor she stated it started 4 or 5 months specific clinical sign, which led to the additional diagnosis of
ago. She admitted the tremor appeared to be getting worse and Parkinson disease. With the patient having been referred to the
that she did not mention it to her physician. No other positive physician, therapy was also initiated. Differential Diagnosis
neurological findings were noted. After the initial examination Phase 2 was performed, which resulted in the decision to treat
was completed, the concern about the tremor was discussed the cervical complaints of the patient.
CASE STUDY 7-2

A 75-year-old woman was sent to physical therapy with the beyond strengthening and stretching muscles, and the patient
diagnosis of moderate to severe osteomalacia of the spine. The was very excited about therapy and how much improvement
physician referred her to a PT. The therapist evaluated her she was making. At the next treatment session her neurological
spine and noted weakness, pain, and tightness. The plan of care signs were still subtle but enhanced, so the patient was told that
included strengthening and stretching, with the assumption that she needed to see her primary care physician for examination
the pain would subside once the muscles could better support and consideration of diagnostic imaging. She continued with
the spine. The therapist did not do a medical screen, and for PT for three more sessions with her pain almost resolved.
much of the therapy program the patient exercised without Per the therapist’s recommendation she saw her physician after
supervision. Both the patient and the husband felt physical the second treatment. The physician ordered magnetic reso-
therapy was not helping at all. The patient and her husband nance imaging, and it revealed a grapefruit-sized nonmalignant
discussed this problem with a neighbor who was also a PT. tumor in her right lower frontal-temporal area. The tumor was
The neighbor referred them to another PT who had extensive removed and the patient recovered after 2 weeks of rehabilita-
manual therapy background and used the Guide to Physical tion. The woman and her family believe that it was the PT that
Therapy Practice as a cornerstone to practice. The new thera- saved not only her quality of life, but also her life itself. The
pist performed a medical screen as part of the examination first PT, by not doing a medical screening examination, did not
and noticed that the woman had some general weakness in her identify the occult neurological problem. If the second thera-
left side that did not coincide with the original diagnosis. The pist’s medical screening and referral to the doctor had not been
symptoms were very subtle and the therapist asked her if she performed, the first therapist could have been deemed negligent
was having any difficulty with daily living activities. She said and cited in a liability suit; importantly, the patient’s tumor may
“no,” so the therapist treated her for her back impairments have caused more permanent damage as it grew undiagnosed
but monitored her neurological signs. The treatment went far within her cranium.
Depression. Depression is a commonly encountered

psychological disorder that is associated with significant
morbidity and mortality.10,22-24 The systems review checklists BOX 7-5 n SUICIDE WARNING SIGNS
in Box 7-1 contain items the therapist can use to help make
the decision to refer a patient for consultation. If the patient History of major clinical depression, chemical dependency,
has suicide ideation, the physician should be contacted be- schizophrenia, or previous suicide attempt
fore the patient leaves the clinic. For the first eight items on Expressions of hopelessness
the depression checklist, concern should be raised when the The sense that the patient is “giving up”
therapist detects four or five of the items present daily for a An abrupt improvement in patient mood
minimum of 2 weeks and resulting in the patient having dif-
ficulty functioning at home, work, or school, socially, or in
rehabilitation. Of the four or five items, one of them should
be depressed or irritable affect or apathy. An exception to the
2-week time frame is during periods of bereavement. When PHYSICAL EXAMINATION
people are faced with a significant loss, it is not uncommon In addition to this discussion of observation screening for
for them to experience a number of the checklist items as the integumentary and nervous systems, other screening
they work through the grieving process (refer to Chapter 6).10 principles are associated with the physical examination.
It is reasonable for these people to experience these symp- The therapist should have expectations of physical exami-
toms for up to 2 months. A neurological event such as a nation findings based on the existing medical diagnosis and
cerebrovascular accident could easily trigger a major clinical data from the history. There should be a correlation be-
depressive disorder, and the depression could significantly tween the described functional limitations and the noted
impede rehabilitation progress. The therapist may be in a impairments. Using the clinical example previously de-
position to facilitate a psychological consultation. scribed, the right shoulder pain Mr. S. was experiencing
Considering that approximately 15% of people with true would be expected to increase or decrease in intensity with
major clinical depression commit suicide,10 therapists need palpation, movement assessment, or special tests. Not only
to be vigilant for warning signs that the patient may be was the therapist unable to alter the ache, but the shoulder
considering this action. See the suicide screening shown in motion and motor control also appeared intact. Essentially
Box 7-5 for a list of warning signs. Once the patient ac- there is nothing for the therapist to treat. The inability to
knowledges suicidal ideation, follow-up questions would alter a patient’s complaints and the lack of neuromusculo-
be appropriate to investigate the patient’s plan and how skeletal impairments one would expect with the medical
readily available the resources are regarding the reported diagnosis and the reported functional limitations should
method of attempt. This is all-important information to be again raise concern about the source of the symptoms. The
reported when the therapist contacts the physician. Therapists physical examination also includes elements of the systems
should be very familiar with their facility’s “suicide proto- review.
col or procedure” in terms of what information should be The Guide to Physical Therapy Practice describes the
collected from the patient and who should be contacted. systems review, in part, as a brief or limited examination
of the anatomical and physiological status of the cardio- to consider when examining a patient. A 30-second monitoring
vascular and pulmonary, integumentary, MSK, and neuro- period after a 2- to 5-minute rest period is recommended
muscular systems.1 For the purposes of this chapter the to obtain baseline rate values.25 Resting blood pressure val-
discussion will focus on assessment of height and weight ues can also provide important screening information. As
and assessing heart rate and blood pressure. Being over- with assessing pulse rate, resting blood pressure should be
weight or obese can significantly increase the risk of assessed after a 5-minute rest period. Variations from the
development of a number of serious conditions (Table 7-4). normative values may lead therapists to additional assess-
Using patient height and weight to calculate body mass ment of the vascular system and the central autonomic
index (BMI) can be a valuable measure to identify patients nervous system and then to a patient referral.
who may need a dietary consultation to prevent disease
states or minimize morbidity associated with current Examination Summary
illnesses. BMI is calculated by dividing body weight (in For many patients a single red flag finding does not warrant
kilograms) by height (in meters). Table 7-4 provides a a referral, but a cluster of history and physical examination
summary of disease risk associated with BMI and waist findings does increase disease probability to the point where
circumference. a referral is indicated. Two examples that are germane to a
Resting blood pressure and pulse rate and rhythm are also number of individuals with neurological conditions are deep
important values to be routinely measured. See Table 7-5 for venous thrombosis (DVT) and pulmonary embolus (PE).
a summary of blood pressure values for adults. Table 7-6 DVT affects approximately 2 million individuals in the
presents normal resting pulse rate parameters for therapists United States annually, making it the third most common
TABLE 7-4 n DISEASE RISK RELATIVE TO NORMAL WEIGHT AND WAIST CIRCUMFERENCE
MEN #102 CM (#40 INCHES) .102 CM(.40 INCHES)

BMI (KG/M2) OBESITY CLASS WOMEN #88 CM (#35 INCHES) .88 CM (.35 INCHES)
Underweight ,18.5 — —
Normal 18.5-24.9 — —
Overweight 25.0-29.9 Increased High
Obesity 30.0-34.9 l High Very high
35.0-39.9 ll Very high Very high
Extreme obesity $40 lll Extremely high Extremely high
From the National Heart, Lung, and Blood Institute: Clinical guidelines on the identification, evaluation, and treatment of overweight and obesity in adults.
Available at: www.nhlbi.nih.gov/health/public/heart/obesity/lose_wt/risk.htm/. Accessed July 20, 2011.
BMI, Body mass index. Classification by Body Mass Index (BMI), waist circumference, and associated disease risks.
TABLE 7-5 n CLASSIFICATION OF BLOOD PRESSURE FOR ADULTS 18 YEARS OLD OR OLDER*†
CATEGORY SYSTOLIC BLOOD PRESSURE (mm Hg) DIASTOLIC BLOOD PRESSURE (mm Hg)
‡
Optimal ,120 and .80
Normal 120-129 and 80-84
High normal 130-139 or 85-89
Hypertension
Stage 1 140-159 or 90-99
Stage 2 160-179 or 100-109
Stage 3 $180 or $110
From The Sixth Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure. Hypertension 23:275–285,
1994, and The Sixth Report of the U.S. Department of Health and Human Services, Public Health Service, National Institutes of Health, National Heart, Lung
and Blood Institute, Bethesda, MD, 1997.
*
Not taking antihypertensive drugs and not acutely ill. When systolic and diastolic blood pressures fall into different categories, the high category should be
selected to classify the individual’s blood pressure status. In addition to classifying stages of hypertension on the basis of average blood pressure levels, clini-
cians should specify presence or absence of target organ disease and additional risk factors. This specificity is important for risk classification and treatment.
†
Based on the average of two or more readings taken at each of two or more visits after an initial screening.
‡
Optimal blood pressure regarding cardiovascular risk is less than 120/80 mm Hg. However, unusually low readings should be evaluated for clinical sig-
nificance.
TABLE 7-6 ESTING PULSE RATE IN BEATS

R n
in ambulatory patient populations (Table 7-7). Of note for
PER MINUTE the neurological population, a history of spinal cord injury
does not appear in this rule even though it is considered a
strong risk factor for DVT. The authors assume there were
AVERAGE LIMITS
very few patients with spinal cord injury in the validation
Norms 120-160 — research.
Fetal 120 70-190 Similarly for PE, a clinical decision rule exists for screen-
Newborn 120 80-160 ing (Table 7-8). PE is associated with high morbidity and
1 year old 110 80-130 mortality, highlighting the critical nature of timely detection.
2 years old 100 80-120 Hull describes PE as one of the “great masqueraders” of
4 years old 100 75-115 medicine because of the often nonspecific presenting symp-
6 years old 90 70-110 toms and signs.28 Wells and colleagues estimate that 50% of
8-10 years old PEs go undiagnosed.29
12 years old Clinician concern regarding the possibility of a DVT and/
Female 90 70-110 or a PE being present would warrant urgent communication
Male 85 65-105 with the patient’s physician.
14 years old
Female 85 65-105 RESPONSE TO TREATMENT
Male 80 60-100 Frequently during Differential Diagnosis Phase 1 the thera-
16 years old pist will decide referral of the patient to a physician is not
Female 80 60-100 warranted and will proceed to Differential Diagnosis Phase
Male 75 55-95 2 and determine whether physical therapy is warranted or
18 years old no intervention recommended. As treatment is initiated
Female 75 55-95 and progresses, the therapist must remain vigilant for the
Male 70 50-90 appearance of symptoms and signs discussed throughout
Well-conditioned athlete 50-60 50-100 this chapter. In addition, correlating subjective and objec-
Adult — 60-100 tive changes as treatment progresses will help the therapist
Aging — 60-100 decide whether further intervention is warranted or whether
Modified from Jarvis C: Physical examination and health assessment, ed 4, referral back to the physician or other health care practitio-
Philadelphia, 1992, WB Saunders. ner is appropriate. For example, if a patient reports a sig-
nificant improvement or worsening, one would expect the
therapist to note a corresponding change in posture, move-
ment ability, palpatory findings, or neurological status. If the
cardiovascular disease.26 A sobering estimation is that expected correlation between patient report and physical
approximately 50% of those with a DVT are asymptomatic examination findings is not found, the therapist should begin
in early stages.27 Clinicians are challenged to identify pa- considering that therapy may not be warranted. A careful
tients at greater risk for this condition who do not have the review of systems and symptom investigation would again
obvious signs and symptoms of calf pain, swelling, and red- be necessary as part of the return to Differential Diagnosis
ness. The following clinical decision rule has been validated Phase 1.
TABLE 7-7 n CLINICAL DECISION RULE FOR DEEP VENOUS THROMBOSIS (DVT)
CLINICAL CHARACTERISTIC SCORE

Active cancer (treatment ongoing, or within previous 6 months or palliative) 1
Paralysis, paresis, or recent plaster immobilization of lower extremities 1
Recently bedridden .3 days, or major surgery in past 12 weeks requiring general or regional anesthesia 1
Localized tenderness along distribution of deep venous system 1
Swelling of entire leg 1
Calf swelling .3 cm greater than asymptomatic side (measured at 10 cm below tibial tuberosity 1
Pitting edema confined to symptomatic leg 1
Collateral superficial veins (nonvaricosed) 1
Alternative diagnosis is as likely as or more likely than DVT 22
KEY
SCORE SIGNIFICANCE
22 to zero Low probability of DVT: 5% (95% confidence interval [CI], 4.0%-8.0%)
1-2 Moderate probability of DVT: 17% (95% CI, 13%-23%)
3 or greater High probability: 53% (95% CI, 44%-61%)
From Wells PS, Anderson DR, Bormanis J, et al: Value of assessment of pretest probability of deep-vein thrombosis in clinical management, Lancet 350(9094;
Dec 20-27):1795-1798, 1997.
TABLE 7-8 WELLS” CRITERIA FOR

“ n
The responsibilities of the PT and OT related to screen-
DETERMINING PROBABILITY OF PULMONARY ing for symptoms and signs that indicate the involvement
EMBOLISM (PE) of another health care practitioner are clearly stated in the
Guide to Physical Therapy Practice2 and The Guide to
Occupational Therapy Practice.3 The process associated
POINT VALUE
with Differential Diagnosis Phase 1 allows for the appro-
CRITERION FOR CRITERION
priate medical screening yet keeps therapists within their
Clinical signs of deep venous thrombosis 3.0 scope of practice. The therapist simply communicates to
(DVT) the physician the list of clinical findings. The physician
Heart rate .100 beats per minute 1.5 will determine whether new or additional medical tests are
Immobilization for 3 days or longer, or sur- 1.5 needed to rule out or diagnose specific diseases. Facilitating
gery in previous 4 weeks the timely referral of patients to physicians is an important
Previous diagnosis of PE or DVT 1.5 role for therapists working within a collaborative medical
Hemoptysis 1.0 model. It is this model that best serves the needs of our
Patients with cancer receiving treatment, 1.0 patients. For additional information related to the medical
treatment stopped in past 6 months, or re- screening process, the readers are directed to four other
ceiving palliative care textbooks.25,30-32
Alternative diagnosis less likely than PE 3.0 With changes in health care delivery and physicians also
Pretest probability of PE is low with a score ,2 points; moderate being asked to see more patients in less time, it is critical that
with a score 2 to 6 points; and high with a score .6 points. all health care practitioners include an adequate medical
Data from Wells PS, Anderson DR, Rodger M, et al: Excluding pulmonary screening component to their examinations. If quality-of-life
embolism at the bedside without diagnostic imaging: management of pa- issues are truly an important component of health care deliv-
tients with suspected pulmonary embolism presenting to the emergency ery, then Differential Diagnosis Phase 1, medical screening,
department by using a simple clinical model and a d-dimer. Ann Intern Med has and will continue to be a professional expectation and
135:98–107, 2001. responsibility placed on each PT and OT. Because consumers
are accessing therapeutic services through more direct means,
that responsibility will remain and grow in importance as
part of both professions’ education and practice. Over the
next few years PTs’ and OTs’ roles will continue to evolve in
CONCLUSION the arena of primary care. Medical screening performed by
If all diseases manifested with a high fever, coughing up the therapist will guide patients to a physician and could
blood, and blood in the urine, the medical screening process become a key component of maintaining the health and quality
would be a simple one. Unfortunately, many diseases initially of life of that consumer.
manifest with subtle complaints, intermittent symptoms or In the future another choice will have to be considered
mild pain, stiffness, subtle weakness or paresthesias, or acute as part of the role of a movement specialist. The results of
dementia. If these complaints are brought to a physician’s Phase 1 and 2 assessments may determine that neither
attention by the patient, they often are not severe enough to a medical referral nor therapeutic intervention itself is
warrant extensive diagnostic testing. Many patients or family appropriate. In this situation, the patient might benefit
members simply ignore symptoms or physiological changes, from community activities but would not need a movement
rationalizing that everything is okay, the family member is specialist, especially if the physician also has determined
just old, or he or she simply does not like to see physicians or that medical intervention is not necessary.
is too busy. All of the scenarios can account for patients with
occult disease seeing therapists. The fact that PTs and OTs References
tend to spend a moderate amount of time with patients over a To enhance this text and add value for the reader, all refer-
period of weeks or months can facilitate the detection of subtle ences are included on the companion Evolve site that
manifestations. In addition, as therapists develop rapport with accompanies this textbook. This online service will, when
patients and family members, information may be shared that available, provide a link for the reader to a Medline abstract
they were uncomfortable disclosing initially. Always remem- for the article cited. There are 32 cited references and other
ber that acute dementia is never normal and is reflective of an general references for this chapter, with the majority of
acute problem rather than simply of aging. those articles being evidence-based citations.
CHAPTER 8 Differential Diagnosis Phase 2: Examination
and Evaluation of Functional Movement
Activities, Body Functions and Structures,
and Participation
ROLANDO T. LAZARO, PT, PhD, DPT, GCS, MARGARET L. ROLLER, PT, MS, DPT, and
DARCY A. UMPHRED, PT, PhD, FAPTA

activity limitations After reading this chapter the student or therapist will be able to:
body system problems and impairments 1. Differentiate the medical diagnosis made by the physician from the diagnosis made by
evaluation a movement specialist.
examination 2. Identify the differences among activity limitations, participation restrictions, and
participation restrictions impairments in specific body structure and function.
3. Choose appropriate examination tool(s) from each category of the ICF model—body
system problems and impairments, activity limitations, and participation restrictions.
4. Identify resources used to analyze the usefulness and psychometric properties of
outcome measures that address body system impairments, activity limitations, and
participation restrictions.
5. Discuss the role of support personnel and assistants in the examination process.
6. Evaluate the results of the clinical examination to establish a therapy diagnosis that
drives intervention planning.
S ince the beginning of the evolution of practice for move-

ment specialists within the health care arena, clinicians
have been expected to examine a client’s functional perfor-
(Figure 8-1). In this phase it is important to examine and
identify the client’s strengths that will facilitate recovery of
functional movement, as well as what the client is unable to
mance and draw conclusions from the examination. The do functionally.
synthesis of information gathered has led to the establish- Numerous tools are used to examine clients with physical
ment of short- and long-term goals, a prognosis concerning complaints and problems with functional movement. Many
the likelihood of the goals being achieved, and the time it of these tools directly measure specific strengths and weak-
will take to achieve those goals. Similarly, the selection of nesses of body system structures and functions, helping the
the most effective and appropriate intervention strategies will clinician to identify specific impairments of a client that are
guide the therapist and patient toward the desired outcomes. causes of functional loss. Each body system or impairment
Today, clients are referred for physical and occupational tool is intended for a specific purpose and is designed to
therapy with “evaluate and treat” orders as the common re- supply the user with a given outcome measure in a predeter-
ferral pattern used by physicians or other health care provid- mined set of values.
ers. With direct access to physical and occupational therapy Other tools measure a client’s ability or limitation for
becoming a reality in many states across the United States performing functional activities. These tools are designed to
and other countries, many patients are walking into clinics examine the performance of a client during various func-
because they have decided that therapy is the best alternative tional skills and activities of daily living. Functional tools
to assist with their functional problems. Whether through also provide the user with a predetermined set of values.
self-referral or referral from another medical or health care These tools, however, do not directly supply information
practitioner, once a client enters into a therapeutic environ- about the cause of the client’s functional movement prob-
ment, clinicians must first determine whether the individual lems. The user must extrapolate information from the results
is medically stable at a body system level (see Chapter 7) of each functional test and then choose the appropriate body
and an appropriate candidate for therapeutic intervention. system or impairment measurement tools to determine the
Once medical screening has been completed and the thera- combination of impairments that may be contributing to
pist determines that there are no red flags to suggest that the the limitations in the client’s ability to perform daily living
client needs to be referred for additional disease or pathol- activities or participate in normal life interactions.
ogy examination or does not need therapeutic intervention, A third category of assessment is the administration
then the client enters into Phase 2 of the evaluation process of participation outcome measures. These are designed to
179
list of tests for body function and structure, activity, and

Patient is sent by a referral source or self-refers
participation in Appendix 8-A.
SELECTION OF APPROPRIATE TESTS

AND MEASURES
Phase One: Medical screening for disease and pathology After the patient history and interview and the review of
• System level screening only (Chapter 7). systems, the next step in the patient/client management pro-
• Refer to practitioner who diagnoses medical disease cess is the selection of appropriate tests and measures that
and pathology if red flags are present. will identify the most important patient problems that require
• Determine whether to refer only or to refer and intervene.
therapeutic intervention. In the clinical environment, the
therapist often approaches this process by first identifying the
functional activities that the patient is able and not able to do
(activity limitation or functional limitation), hypothesizing
Phase Two: Client remains within the scope of physical/ the possible impairments causing this activity or functional
occupational therapy practice and proceeds with the limitation, and then testing the body systems and subsystems
diagnostic process (Chapter 8). to identify the nature and extent of the impairments. Consis-
• Examine and evaluate to determine impairments,
activity limitations, and participation restrictions. tent with the International Classification of Functioning,
• Establish a clinical diagnosis and prognosis. Disability and Health (ICF) model, the client’s strengths
• Select and apply best available evidence-based are often emphasized as the foundation for this process. The
interventions (Chapter 9). approach is proactive and collaborative, truly capitalizing on
the client’s potential and goals, and highlights the therapist-
Figure 8-1 n The diagnostic process used for best practice by client partnership.
physical and occupational therapists.
TESTS OF ACTIVITY AND FUNCTIONAL
PERFORMANCE
As mentioned previously, the therapist typically starts with
assess a client’s involvement or restriction in domestic, com- the examination of functional activities. This step will
munity, social, and civic life situations. These tools examine allow the therapist to understand the specific functional
the client’s perspective on the effects of his or her health tasks that the patient can do, as well as those activities or
condition on the ability to function in major life areas, inter- functions that the patient is unable to perform. It is impor-
personal interactions and relationships, and quality of life. tant to observe the client during the performance of these
The process of examination and evaluation occurs tasks and to note the motor patterns used. Many functional
throughout the entire episode of care. The therapist must be assessment tools have been developed to provide a more
constantly assessing the patient’s health status during each objective assessment of everyday skills and tasks. Each tool
encounter. Initially the therapist may miss something impor- is designed to measure and score a specific type of func-
tant that does not become identified until more complex tional ability. Several tools test a range of skills from bal-
activities are introduced. Once the subtle body system prob- ance skills to walking ability during the performance of
lems become obvious, the therapist may need to go back to functional tasks. Some tests are quick and easy to set up and
identify more in-depth causes and fine-tune intervention perform, such as the Functional Reach Test and Timed Up
strategies. The intervention strategy may proceed in various and Go (TUG) Test, whereas other tools may take longer to
directions depending on whether the patient and therapist administer, such as the Berg Balance Scale or Fugl-Meyer
mutually agree that the goal should stress adaptation and Assessment. There are advantages and disadvantages to
compensation versus regaining function through motor both of these types of tests. Those that are the quickest to
learning. administer tend to measure fewer functional skills and
To be able to provide information that is meaningful in do not supply information on the total functioning of a
determining the best possible intervention for a particular client. However, if the clinician properly identifies the cli-
patient, the examination tools selected by the clinician must ent’s problems and is able to focus on the most efficient way
be objective, reliable, valid, and appropriately matched of measuring them, a quick functional test can be the most
with patient expectations. These tools should also commu- beneficial one to use. Tests that take a bit longer to adminis-
nicate necessary information in a language that is under- ter typically assess multiple skills and supply the user with
standable to all health care professionals and the payer a more comprehensive picture of the client’s functional
responsible for funding the services (see Chapter 10). With abilities including those in various domains such as gross
the limitations on visits within the clinical setting and the mobility, self-care, cognitive ability, and communication
critically important variable of motivation and adherence ability, among others. Often the decision on the complexity
of the patient, the setting of goals and the selection of of the assessment tool used is based on the client and
intervention strategies need to be established through family’s objectives and long-term goals.
patient-centered participation.1-10 Outcome scales for functional tests may be found in ordinal
This chapter has been developed to help the reader work format (e.g., Functional Independence Measure [FIM], Barthel
through the problem-solving and decision-making process Index, Katz Index of Independence in Activities of Daily
for selecting appropriate tests. It is not within the scope Living). Each has its own unique point value and range, vary-
of this chapter or text to explain each examination tool in ing from a two- to three-point scale (e.g., Tinetti Performance-
detail. However, the reader is presented with an extensive Oriented Mobility Assessment [POMA]) to a seven-point
C H A P T ER 8 n Examination and Evaluation of Functional Movement Activities, Body Functions and Structures, and Participation 181
scale (e.g., FIM). A few tools supply ratio scale data (e.g., the However, these tools typically require a large improvement
TUG, Functional Reach Test, and measures of gait velocity). in a client’s functional performance for a clinically signifi-
Test data presented in ratio scale format will more clearly cant change to be seen. Results obtained from impairment
show incremental changes, thereby facilitating the comparison tests can fill in the large gaps between numerical scores on
of pretherapy to posttherapy performance. functional scales, demonstrating objective measurements
and trends in the direction toward improvement before any
TESTS OF BODY FUNCTIONS change is demonstrated on the functional examination.
AND STRUCTURES Box 8-1 illustrates impairments that may be seen in
After identifying problems with functional performance of patients/clients with movement disorders caused by neuro-
activities, the clinician then focuses on the performance of logical dysfunctions. These impairments are further classified
appropriate tests for body functions and structures. Consis- as those that are within the central nervous system and those
tent with the ICF model, the intent is to identify which body that are outside the central nervous system and result from
systems or subsystems are intact and functioning normally interaction with the environment. These impairments are
and could be optimized as the patient works on regaining the further discussed in detail in various sections of this book.
ability to perform functional tasks or participate in life. Range of motion testing is one example of a common
In this step, it is also important to identify which body sys- neuromusculoskeletal system examination procedure. Clini-
tems and subsystems are not normal. These body system cians depend heavily on ROM measurements as an essential
impairments may be the cause of the functional loss. component of their examination and consequent evaluation
Impairment (ICF; International Classification of Impair- process. It is imperative that the data obtained from this pro-
ments, Disabilities, and Handicaps [ICIDH]; Nagi)11 is cedure be reliable. It has been suggested that the main source
defined as the loss or abnormality of physiological, psycho- of variation in the performance of this procedure is method
logical, or anatomical structure or function at the organ and that reliability can be improved by standardizing the
system level.12 The clinician needs to make the distinction procedure.13
between primary impairments, which are a direct conse- An impairment in ROM can be the result of other body
quence of the client’s specific disease or pathological system impairments. ROM measurements may be used to
condition, and secondary impairments, which occur as
sequelae to the disease or rehabilitation process or as the
result of aging, disuse, repetitive strain, lifestyle, and so on.
Moreover, the clinician must remember that, although func- BOX 8-1 n IDENTIFICATION AND
tional limitations are usually caused by a combination of CLASSIFICATION OF IMPAIRMENTS
specific impairments, it is possible that impairments may
IMPAIRMENTS WITHIN THE CENTRAL NERVOUS
not contribute to specific functional problems for a particu-
SYSTEM
lar client. If this is the case, the clinician should make a
1. Tone, reflexes, and abnormal state of the motor neuron
determination regarding whether these impairments, if left
pool
uncorrected, will result in the development of activity limi-
2. Synergies (volitional or reflexive)
tations at a later time. Simultaneously, the patient needs to
3. Sensory integration and organization
be a part of this discussion because the therapist may not
4. Balance and postural control
have the time to address all impairments. The correction of
5. Speed of movement
particular impairments may have more meaning or value to
6. Timing
the patient. To the consumer some impairments may lead to
7. Reciprocal movement
limitation of functions that are important to them, whereas
8. Directional control, trajectory or pattern of movement
other impairments may restrict an activity in which the
9. Accuracy
patient would never want to participate.
10. Emotional influences
The ultimate goal of any therapeutic intervention pro-
11. Perception
gram is to attain the highest level of health and wellness
12. Cognition, memory, and ability to learn
possible. Measurement tools that the clinician chooses to
13. Levels of consciousness
use also need to reflect this end result. For example, “tradi-
tional” impairment measurements may indicate that a client IMPAIRMENTS OUTSIDE OF THE CENTRAL
demonstrates shoulder range of motion (ROM) that is NERVOUS SYSTEM AND INTERACTION
decreased by 25 degrees. The more important question WITH THE ENVIRONMENT
should be how this decrease in ROM affects the client’s abil- 1. Range of motion
ity to perform a functional task such as dressing or any other 2. Muscle strength or power production
activity that the client perceives as important. The clinician 3. Endurance
is therefore encouraged to consider the functional implica- 4. Cardiac function
tions of these measurements to obtain results that are more 5. Circulatory function
meaningful for the client. 6. Respiratory function
The clinician is always faced with the challenge of iden- 7. Other organ system interactions
tifying and administering examination tools that will not 8. Hormonal and nutritional factors
only reflect the client’s level of health and wellness but also 9. Psychosocial factors
reflect the client’s functional improvement as a result of 10. Task content
the intervention provided. Functional measurement tools 11. Environmental construct
can be used as a baseline measure for those functional skills.
determine the effect of tone, balance, movement synergies, The clinician is also advised to investigate the interaction of
pain, and so forth on the neuromuscular system and ulti- other organs and systems as they relate to the patient’s func-
mately on behavior. Most important, the clinician needs to tional limitations. For example, electrolyte imbalance, hor-
remember that the ROM needed to perform a functional monal disorders, or adverse drug reactions (see Chapter 36)
activity is more critical than “normal,” anatomical, biome- may explain impairments and activity limitations noted in
chanical ROM values and must be considered when labeling other interacting systems.
and measuring impairments. For example, full ROM in the
shoulder is seldom needed unless activities of daily living, TESTS FOR PARTICIPATION
work, or leisure activities require it, such as performing a AND SELF-EFFICACY
tennis serve or reaching overhead to paint a ceiling. When In ICF terminology, participation is defined as an individu-
needed for specific tasks, goniometric measurements of al’s involvement in a life situation. Domestic life, interper-
ROM are appropriate, but at other times a functional range sonal interactions and relationships, and community, social,
measurement may be sufficient. and civic life are some examples of aspects of participation
Muscle strength testing is another commonly used that can be examined for each individual. Participation
examination procedure. Clinicians use various methods of restriction is the term used to denote problems that individu-
quantifying strength including “traditional” manual mus- als may experience in involvement in life situations. When
cle testing (MMT) and the use of a dynamometer. As with considering participation it is important to obtain the indi-
ROM, strength should be correlated with the patient’s vidual’s perception of how the medical condition, impair-
functional performance. Again, the clinician may find a ments, and activity limitations affect his or her involvement
client to have 3/5 strength in the shoulder flexor muscle in life and community. Therefore many of the tests for par-
groups or find grip strength to be 35 kg, but the more ticipation and self-efficacy are in self-report format. The
important question should be “What does this mean in Activities-specific Balance Confidence Scale (ABC), Short
terms of the client’s ability to perform activities of daily Form 36 (SF-36), and Dizziness Handicap Inventory (DHI)
living, and/or can he use that power in a functional activ- are examples of tests that can be used to gather information
ity?” The clinician is also advised to make the distinction under this domain. These tests allow an individual to assess
between muscle strength and muscular endurance as it his or her health quality of life after an incident that affected
relates to function. A client may have sufficient lower- activity and participation. Appendices 8-A, 8-B, and 8-D
extremity strength and power to get up from the seated include tools that measure participation and quality of life.
position; however, this does not necessarily mean that the
client has muscular endurance to perform the task repeat- CHOOSING THE APPROPRIATE
edly during the day as part of normal everyday activities. EXAMINATION TOOL
The status of the cardiac, respiratory, and circulatory The ability to choose the appropriate examination tool(s) for
systems significantly affects a client’s functional perfor- a particular client will depend on several factors:
mance (see Chapter 30). Blood pressure, heart rate, and 1. The client’s current functional status (ambulatory vs
respiration give the clinician signs of the patient’s medical nonambulatory)
stability and the ability to tolerate exercise. The clinician 2. The client’s current cognitive status (intact vs confused
may also obtain the results of pulmonary function tests for or disoriented)
ventilation, pulmonary mechanics, lung diffusion capacity, 3. The clinical setting in which the person is being evaluated
or blood gas analysis after determining that the client’s pul- for treatment (acute hospital, rehabilitation, outpatient,
monary system is a major factor affecting medical stability skilled care, or home care)
and functional progress. Various exercise tolerance tests also 4. The client’s primary complaints (pain vs weakness vs
attempt to quantify functional work capacity and serve as a impaired balance)
guide for the clinician performing cardiac and pulmonary 5. The client’s goals and realistic expectation of recov-
rehabilitation. ery, maintenance, or prevention of functional loss
A client who has difficulty performing activities of daily (acute injury, chronic problem, or progressive disease
living and who has neurological impairments in the central process)
motor, sensory, perceptual, or integrative systems needs to 6. The type of information desired from the test (discrimi-
undergo examination procedures to establish the level of native or predictive)
impairment of each involved system and to determine if and The evaluator should select examination tools that will
how that system is contributing to the deficit motor behav- measure the client’s primary problems (activity limitations,
iors. Functional evaluation tools used may include the FIM, impairments, and participation restrictions) and supply out-
the Barthel Index, the Tinetti POMA, or the TUG test. The come values that are needed to set realistic treatment goals
results of these tests will help to steer the clinician toward in accordance with those of the client and family and to plan
the most useful impairment tools to use to evaluate limita- efficient and effective intervention strategies. The clinician
tions in the various body systems. Impairment tools may is advised to select functional tools that contain component
include the Modified Ashworth Scale for spasticity, the skills that the particular client is having difficulty perform-
Upright Motor Control Test for lower-extremity motor con- ing. Skills the client performs poorly will disclose the activ-
trol, the Clinical Test of Sensory Interaction on Balance ity limitations. Skills the client performs well determine the
(CTSIB), or the Sensory Organization Test (SOT) for bal- client’s strengths and abilities. The evaluator must then
ance and sensory integrative problems, or computerized focus on the client’s functional activity limitations as
tests of limits of stability on the NeuroCom Balance Master, determined by the test(s) to determine the impairment
among others (see Appendices 8-A, 8-B, and 8-C). tests that will be performed next. For example, if the client
demonstrates difficulty in rising from a chair during a func- other impairments. The examiner must be able to come to a
tional test and scores low on this skill on the outcome conclusion regarding the relationship between the client’s
measure (the Tinetti POMA or Berg Balance Scale), the activity limitations and the existing body systems impair-
clinician must then closely examine the skill of coming to ments. Without an understanding of this relationship, it is
stand to determine the cause of the mobility limitation. The difficult to assess the effect of the treatment intervention(s)
problem may be that the client cannot generate adequate on an individual.
muscle power to push up from the chair, does not have The interactions and interrelationships of the identified
adequate ROM in the hip or the ankle joints to rise from the functional problems and impairments provide the clinician
chair, or no longer sees a reason to get out of the chair, or with an initial status or problem list specific to that individ-
that it hurts too much to even try. It may be a problem with ual. That list helps the clinician formulate a diagnosis for the
dynamic balance during or after the transitional movement. movement dysfunction. Through consideration of the objec-
Any impairment that is hypothesized by observing perfor- tive values obtained during the examination process, a target
mance of the functional skill needs to be measured more status to be reached at the conclusion of therapeutic inter-
specifically. It is up to the examiner to determine the next vention can be estimated. That target status is both impair-
best steps to take to target the client’s problems as efficiently ment and function driven and traditionally would be consid-
as possible, to measure and record the needed outcomes as ered a list of outcome goals. The interactions between
objectively as possible, and then to set treatment goals in impairments and their related activity and participation
consultation with the client to design the best intervention to limitations make up the unique problem map of that indi-
remediate or manage the problems. vidual and direct the clinician toward selecting optimal
Many of the examination tools that measure a client’s interventions.
ability to perform functional activities have been accepted as The prognosis made by the clinician is based on the
valid, reliable, and useful for the justification of payment for assessment of the likelihood that the patient will achieve the
services rendered. The number of activity limitations and target outcome in a given time frame and estimated number
the extent of the client’s participation limitation are often of visits needed to reach the treatment goal. Once the clini-
reasons why an individual either has accessed therapy ser- cian has measured and identified specific activity limitations
vices directly or was referred by a medical practitioner. For and their respective impairments, he or she then has an
this reason, the third-party payer expects to receive reports excellent opportunity to conceptually understand how vari-
concerning positive changes in the client’s functional status ous impairments affect multiple functional problems and
for therapeutic services to be justifiable (see Chapter 10). which impairments are activity specific.
The initial list of functional or activity limitations or par- The following case scenario synthesizes the clinical
ticipation restrictions helps the therapist determine the examination and evaluation process used by physical and
extent of, expectations for, and direction of intervention, but occupational therapists.
it does not determine why those limitations exist. This is the Assume that a clinician has been called in to examine a
question that is critical to answer as part of the evaluation client who has sustained an anoxic brain injury during heart
process. Examination tests and procedures that identify spe- surgery. The client’s cognitive ability is within normal
cific system and subsystem impairments help the therapist limits, and he is highly motivated to get back to his normal
determine causes for existing participation and activity limi- activities. He is retired; he loves to walk in the park with his
tations. These tools need to be objective, reliable, and sensi- wife and to go on birdwatching experiences in the moun-
tive enough to provide needed communication to third-party tains with their group of friends. The clinician must select
payers to explain the subsystem’s baseline progress during which functional tests to use to obtain an objective initial
and after the intervention. These tools should also supply status and target the client’s problems. Currently the client
explanations for residual difficulties in the event that the requires assistance with all gross mobility skills and is
functional problems themselves do not demonstrate signifi- demonstrating difficulty balancing in various postures and
cant objective change or show progress within the time performing activities of daily living. Results of functional
frame estimated. testing reveal that the client demonstrates significant limita-
tions, requiring moderate assistance in the activities of com-
USING THE EVALUATION PROCESS TO LINK ing to sit, sitting, coming to stand, standing, walking, dress-
BODY SYSTEM PROBLEMS, ACTIVITY ing, and grooming. Assume that the client also displays
LIMITATIONS, AND PARTICIPATION impairment limitations in flexion ROM at the hip joints
RESTRICTIONS TO INTERVENTION caused by both muscle and fascia tightness and hypertonic-
After objective measures have been obtained for activity ity within the extensor muscle groups. He has compensated
limitations, body system and subsystem impairments, and to some degree and is able to perform bed mobility indepen-
participation restrictions, clinicians must determine whether dently. Upper-extremity motor control is within normal
the impairments or the functional problems are changeable limits, and thus the client is capable of performing many
to a more independent, safe, and functional level. In certain activities of daily living as long as his lower trunk and hips
situations a mobility limitation may be remediated and are placed in a supportive position and hip flexion beyond
become more functional, although the contributing compo- 90 degrees is not required. The client has general weakness
nent impairments may remain unchanged. In other situa- from inactivity, and power production problems in his
tions, impairment measures may significantly improve but abdominals and hip flexor muscles owing to the dominance
the functional problem may remain unaltered. This is espe- of extensor muscle tonicity. Once he is helped to stand, the
cially true when one impairment is significantly improved extensor patterns of hip and knee extension, internal rota-
but functional progress is masked by the contribution of tion, slight adduction, and plantarflexion are present. He can
actively extend both legs after being placed in flexion, but he tion and evaluation is the acceptance of the movement dys-
is limited in the production of specific fine and gross motor function or impairment by the client. A mobility problem or
patterns. Thus a resulting balance impairment is present impairment may be clearly identified by a functional test or
owing to the inability to adequately access appropriate bal- impairment test; however, the client may deny that the prob-
ance strategies caused by the presence of tone, limb synergy lem even exists. Acceptance of the problems by the client and
production, and weakness in the antagonists to the trunk and a willingness to change are critical to the client’s adherence
hip extensors. Through the use of augmented intervention to the intervention strategy.
(see Chapter 9) the client is noted to possess intact postural As mentioned earlier, the identification of potential
and procedural balance programming; however, both func- impairments was done after functional testing to streamline
tions are being masked by existing impairments. The decision the examination process. After performing the functional
is made to perform impairment measures, including assess- examination, the therapist postulated that the client might
ments of ROM at the hip, knee, and ankle joints; the ability to have impaired motor control, muscle weakness, sensory
produce strength in both the abdominal and hip flexor muscle deficits, pain, and decreased endurance that may have been
groups; and volitional and nonvolitional synergic program- causing the functional limitations. MMT revealed lower-
ming, balance, and posture, and volitional control over extremity strength of 1/5 in both ankle motions, 2/5 in both
muscle tone. The demand on ROM, power production, and knees, and 32/5 in both hips. Upper extremities tested as
specific synergic programming will vary according to the 1/5 finger flexors (incomplete grip), 2/5 wrist motions, and
requirements of the functional activities performed. 31/5 in both elbow motions. Shoulder and trunk strength
Using a clinical decision-making process, the clinician were within functional limits for all motions. Sensory test-
will conclude that the impairments that are being targeted to ing indicated absent touch and proprioceptive sensations
measure will vary from one functional activity to the next. from the foot to the knee of both lower extremities, with
For instance, if this client is demonstrating difficulty rising impaired sensation from the thighs to the hips. Both hands
from a chair, the target impairment may be a ROM measure- and wrists tested absent to touch and proprioception, with
ment. This same ROM impairment may also contribute to the elbows and shoulders testing intact. The client’s endur-
problems with moving about the base of support in func- ance was limited to short bouts of activity (3 to 5 minutes),
tional sitting. The clinician makes the determination as to with rapid muscular and cardiovascular fatigue. The pres-
the extent to which the impairment interferes with each ence of these impairments helped to explain the resultant
functional problem for that particular client. functional limitations tested earlier.
These objective measurements help the clinician explain In terms of standardized functional tests, the multidisci-
which outcomes would be expected to be achieved first and plinary FIM could give insight into this patient’s ability to
why. These measurements are recorded as part of interven- function in multiple domains and categories. Baseline scores
tion charting and help to objectively demonstrate that the on the Tinetti POMA and the Berg Balance Scale could be
client is improving toward functional independence. They collected because this client is expected to regain further
also give an indication of what the client still needs to reach function in balance and postural control as recovery from
the desired outcome, the rate of learning that is taking place, the condition occurs. As the client regains strength and
and an estimation of recovery time that is still required. peripheral sensory ability, he may be able to perform the
These objective measurements give to the clinician and the TUG and the 10-Meter Walk Test. These functional assess-
client a better avenue to discuss expectations with family ments paint a better picture of what the client can and cannot
members, other medical practitioners, and third-party payers. do, as well as providing a way to measure functional
In this example, assume that, after intervention, functional progress in various activities throughout rehabilitation.
ROM in the hip was achieved. However, this improvement When determining an appropriate tool to examine a
did not result in an improvement in the activity problems client’s functional status, the clinician must also consider
because synergic programming prevented adequate hip flex- the “ceiling and floor effect” of the functional tools. In this
ion during one or more functional activities. Understanding particular case, the patient is probably unable to perform
and measuring the difference between lack of ROM as a the Functional Gait Assessment (FGA) but may be appro-
result of muscle or fascia tightness versus lack of range from priate for beginning the balance portion of the Tinetti
abnormal synergic patterning helps the clinician communi- POMA. As the patient progresses, the predictive and dis-
cate why a client is successful in one activity and may still criminative properties of some of these tests could provide
need assistance in another. information regarding the patient’s likelihood of falling,
Scores obtained from tests of activity, participation, or ability to safely perform selected functional tasks.
and impairments supply statistically important measure-
ments that can then be used to discuss the limitations
placed on the therapeutic environment by fiscal intermedi- References
aries. Therapists must be clear when documenting the To enhance this text and add value for the reader, all refer-
initial status and the target status for clients so that the ences are included on the companion Evolve site that
recommended intervention and length of stay may be accompanies this textbook. This online service will, when
justified (see Chapter 10). available, provide a link for the reader to a Medline abstract
When making a determination of the potential impact of for the article cited. There are 209 cited references and other
an intervention on improving a client’s problems, clinicians general references for this chapter, with the majority of
must remember that a key factor in this process of examina- those articles being evidence-based citations.
CASE STUDY 8-1*

The patient is a 30-year-old man who was referred to outpatient The Gait Abnormality Rating Scale (GARS) was performed,
physical therapy after a 1-week stay in an acute care facility fol- and the patient scored a 32/48, indicating increased fall risk.
lowing an exacerbation of relapsing-remitting multiple sclerosis The Tinetti POMA was also administered. The patient scored
(MS). His height was 2.05 m (6 feet 9 inches) and his weight 8/28, indicating a high risk for falls. Several tests were per-
was 133.81 kg (295 pounds). The patient was first diagnosed formed using the NeuroCom Balance Master. The sit-to-stand
with MS 4 years ago. He developed optic neuritis during the test showed that the patient had difficulty maintaining balance
exacerbation and was treated with corticosteroid pulse therapy. immediately after rising and had more weight on his left lower
The patient’s past medical history included depression, gastro- extremity. The results were abnormal, based on the norms for
esophageal reflux disease (GERD), migraine headaches, and the patient’s gender and age. Next, the weight-bearing squat
hyperlipidemia. After being diagnosed with MS, the patient had test was done. During this test the patient was not able to
to stop working. Before the most recent hospitalization, the maintain equal weight through bilateral lower extremities, with
patient lived with his sister in a single-story home with five the patient bearing weight more on the left side. The patient
stairs to enter with a railing. At that time he was able to ambu- then performed the limits of stability test, which revealed an
late independently without an assistive device and to complete inability to lean his center of gravity (COG) over his right lower
all activities of daily living without any assistance. extremity, or forward onto his toes. The patient then performed
An outpatient physical therapy initial examination was the rhythmic weight-shift test; he was not able to complete the
conducted 1 week after the patient was discharged from the forward-backward component of the rhythmic weight-shift test
hospital. He reported that he was using a wheelchair to get to without falling, and he also had difficulty with directional
and from appointments, and inside his home. He was alert and control and velocity during lateral weight shifting.
oriented to person, place, and time, although responses were Last, tests for participation and self-efficacy were
delayed and speech was slightly slurred. There were no administered. The Activities-specific Balance Confidence
complaints of pain, and the patient stated that fatigue and (ABC) Scale questionnaire was given to the patient to assess
temperature had not affected him. the patient’s balance self-efficacy. The patient had a score
Several tests of functional movement activities were performed of 20%, indicating a low level of physical functioning. He
first. The patient was able to roll to the right and left with minimal scored 10% on being able to bend down, pick a slipper up
assistance, with rolling to the right being less difficult for the off the floor, and reach for a can on a shelf at eye level with
patient. He needed supervision to move from supine to sitting. the use of a FWW.
The patient required moderate assistance to perform a The data collected at initial examination revealed limita-
sit-to-stand transfer. He was able to ambulate five steps with tions in functional performance resulting from impairments in
a front-wheeled walker (FWW) and moderate assistance of balance, gait, strength, and motor control, giving the therapist
one person. the various movement diagnoses that reflected problems. The
After examination of the patient’s functional movement Guide to Physical Therapist Practice was used to classify the
activities, several tests of body function and structures were patient in the neuromuscular practice pattern E (impaired
then administered. Passive ROM for all joints in both upper motor function and sensory integrity associated with progres-
and lower extremities was within normal limits. The patient sive disorders of the central nervous system). The Guide
presented with 3/5 (fair) strength of the right upper and lower indicates a range of 6 to 50 visits needed to reach anticipated
extremities and good (4/5) strength of the left upper and lower outcomes for patients who are classified in this practice pat-
extremities during MMT. Light touch and superficial pain tern. Intervention frequency and duration was set at three
sensations tested intact from C4-S2 dermatomes bilaterally. times per week for 8 weeks. The prognosis that the patient
Sitting balance was scored as 3/4 (good), as the patient was would be able to ambulate independently in the community
able to accept moderate challenges. Standing static balance was with an assistive device in 8 weeks was good, given the
11/4 (poor plus); the patient was able to maintain balance with patient’s willingness to participate in physical therapy, positive
handheld support and occasional minimal assistance. outlook, family support, and positive response to medical
Observational gait analysis was performed, and impairments interventions. The plan of care that was developed focused
in gait included the following: decreased step length bilaterally, on improving activity limitations such as transfers and gait
wide base of support, decreased weight bearing through the and impairments such as weakness and imbalance. The long-
right lower extremity, and lack of toe-off. It was also noted that term goals were set to be achieved in 8 weeks, and short-term
ataxic type movements were present with ambulation. goals were set to be achieved in 4 weeks.
*Case study modified from Larsen-Merrill J, Lazaro R: Use of the NeuroCom balance master training protocols to improve functional performance in a person
with multiple sclerosis. J Stud Phys Ther Res 21:1–16, 2009.
APPENDIX 8-A Outcome Measures from the Neurology Section of the American
n
Physical Therapy Association (APTA) Special Interest Groups (SIGs) from
Neurologic Practice Essentials: A Measurement Toolbox,* Organized by Categories
of the World Health Organization (WHO) International Classification of
Functioning, Disability and Health (ICF)14-17
ICF Category Outcome Measures ICF Category Outcome Measures
Balance and Falls SIG Activity 2- or 6-Minute Walk Test
Body Structure/Function None submitted 360-Degree Turn Test
Berg Balance Scale
Activity Berg Balance Scale
Dynamic Gait Index (DGI)
Fregly-Graybiel Ataxia Test Battery
Functional Independence Measure (FIM)
Functional Reach Test
Functional Reach Test
Gait Abnormality Rating Scale, Modified
Gait Speed—Self-Paced and Fast
(mGARS)
Modified Gait Abnormality Rating Scale
Gait Speed—10 Meter Walk Test
Schwab and England Scale
Limits of Stability Test (LOS)
Timed Up and Go Test (TUG)
Physical Performance Battery
Tinetti Performance-Oriented Mobility
Sensory Organization Test (SOT)
Assessment (POMA)
Tinetti Performance-Oriented Mobility
Assessment (POMA) Participation Fatigue Severity Scale
Walky-Talky Test Modified Falls Efficacy Scale
Modified Fatigue Impact Scale
Participation Activities-specific Balance Confidence Scale
Parkinson’s Disease Questionnaire–39 (PDQ-39)
(ABC)
Short Form 36 (SF-36) or Short Form 12 (SF-12)
Tinetti Falls Efficacy Scale (FES)
Spinal Cord Injury SIG
Brain Injury SIG
Body Structure/Function American Spinal Injury Association (ASIA)
Body Structure/Function Agitated Behavior Scale
Impairment Classification Scale
Awareness Questionnaire
Manual Muscle Testing (MMT)
Coma/Near Coma Scale
Modified Ashworth Scale
Disorders of Consciousness Scale
Myometry
Glasgow Coma Scale (GCS)
Penn Spasm Frequency Scale
JFK Coma Recovery Scale, Revised
Activity Functional Evaluation in Wheelchair (FEW)
Patient Competency Rating Scale
Quadriplegia Index of Function (QIF)
Rancho Levels of Cognitive Functioning
Spinal Cord Injury Functional Ambulation
Activity Berg Balance Test
Inventory (SCI-FAI)
Brunel Balance Test
Spinal Cord Injury Measure (SCIM)
Walking Index for Spinal Cord Injury–II
Functional Independence Measure/Functional
(WISCI-II)
Assessment Measure (FIM/FAM)
Wheelchair Assessment Tool (WAT)
High-level Mobility Assessment Test (HiMAT)
Participation Craig Handicap Assessment and Reporting
Participation Community Integration Questionnaire
Technique (CHART)
Craig Handicap Assessment and Reporting
Impact on Participation and Autonomy (IPA)
Technique (CHART)
Life Habits and Handicap (LIFE-H)
Disability Rating Scale
Mayo Portland Adaptability Inventory Stroke SIG
Participation Objective, Participation Subjective Body Structure/Function Fugl-Meyer Assessment of Sensorimotor
Recovery After Stroke (FMA)
Degenerative Diseases SIG
Hand-Held Dynamometry
Body Structure/Function ALS Functional Rating Scale
Mini-Mental State Examination (MMSE)
Hoehn and Yahr Stage
Kurtzke Extended Disability Status Scale
National Institutes of Health Stroke Scale (NIHSS)
Neurobehavioral Cognitive Status Examination
Modified Mini-Mental State Examination
Postural Assessment Scale for Stroke (PASS)
(MMSE)
Stroke Rehabilitation Assessment of Movement
Unified Huntington’s Disease Rating Scale
(STREAM)
(UHDRS)
Trunk Control Test
Unified Parkinson’s Disease Rating Scale
Trunk Impairment Scale
(UPDRS)
APPENDIX 8-A Outcome Measures from the Neurology Section of the American
n
Physical Therapy Association (APTA) Special Interest Groups (SIGs) from
Neurologic Practice Essentials: A Measurement Toolbox,* Organized by Categories
of the World Health Organization (WHO) International Classification of
Functioning, Disability and Health (ICF)14-17—cont’d
ICF Category Outcome Measures ICF Category Outcome Measures
Activity 10-Meter Walk Test (10MWT) Activity Dynamic Gait Index (DGI)
6-Minute Walk Test (6MWT) Functional Gait Assessment (FGA)
Barthel Index Timed Up and Go Test (TUG)
Berg Balance Scale Participation Dizziness Handicap Inventory (DHI)
Chedoke-McMaster Stroke Assessment Scale Physical Activities Scale for the Elderly
Frenchay Activities Index (FAI) Short Form 36 (SF-36)
Functional Independence Measure (FIM) Vestibular Disorders Activities of Daily Living
Modified Rankin Handicap Scale Scale (VADL)
Motor Assessment Scale (MAS)
Generic Measures
Rivermead Motor Assessment (RMA)
Body Structure/Function Mini-Mental State Examination (MMSE)
Timed Up and Go Test (TUG)
Activity 5- or 10-Meter Walk Test
Participation Euro Quality of Life–5D (EuroQol-5D)
Short Form 36 (SF-36) 6-Minute Walk Test
Clinical Test of Sensory Interaction on
Stroke Impact Scale (SIS)
Balance (CTSIB)
Stroke Specific Quality of Life (SS-QOL)
Four Square Step Test (FSST)
Stroke-Adapted Sickness Impact Profile
Functional Ambulation Categories
(SA-SIP30)
Functional Gait Assessment (FGA)
Vestibular SIG Trunk Impairment Scale
Body Structure/Function Clinical Test of Sensory Interaction on Participation Activities-specific Balance Confidence
Balance (CTSIB) Scale (ABC)
Romberg Test and Sharpened Romberg Test Short Form 36 (SF-36)
Sensory Organization Test (SOT)
Single-Leg Stance Test
Nystagmus Tests
Gaze-Evoked Nystagmus
Post–Head Shaking Nystagmus Test
Spontaneous Nystagmus
Vibration-Induced Nystagmus
Positional Testing
Dix-Hallpike Test
Motion Sensitivity Quotient (MSQ)
Tests of Voluntary Eye Movement
Saccades
Smooth Pursuit
Vergence
VOR Cancellation Test
Vestibular Ocular Reflex Tests (VOR)
Dynamic Visual Acuity Test (DVA)
Gaze Stabilization Test (GST)
Head Thrust Test (HTT)
*Neurologic Practice Essentials: A Measurement Toolbox Development Team: Jane Sullivan (lead), Bill Andrews, Richard Bohannon, George Fulk, Desiree
Lanzino, Aimee Perron, Peggy Roller, Kirsten Potter, Yasser Salem, Teresa Steffen. Neurology Section Support and Coordinators: Nancy Fell, Karen McCulloch,
Dorian Rose.
APPENDIX 8-B Sample Outcome Measures According to the Examination Areas

n
in the Guide to Physical Therapist Practice, from: Neurologic Practice Essentials:
A Measurement Toolbox*
Body Structures and Functions— n Voluntary motor
List of Measures n American Spinal Injury Association (ASIA) Scale (spinal
Cognition cord injury)
n Ability to follow multistep commands n Coordination tests
n Alert, oriented 3 4 o Detection of tremor
n Kokman Short Test of Mental Status o Finger-to-nose and rapid alternating movement tests (and
n Mini-Mental State Examination (MMSE) other nonequilibrium coordination tests)
n Montreal Cognitive Assessment o Fregly-Graybiel Ataxia Test Battery
n Scales to detect behavioral and cognitive impairments often o Tandem walking (and other equilibrium coordination tests)
seen in patients with brain injury: n Five-Times Sit-to-Stand Test (FTSST)
n Agitated Behavior Scale n Hand-held dynamometry
n Apathy Evaluation Scale n Manual muscle testing (MMT)
n Awareness Questionnaire n Motor Assessment Scale (MAS)
n Cognitive Log n Motricity Index
n Coma Recovery Scale–Revised n Rivermead Motor Assessment
n Coma/Near Coma Scale n Stroke Rehabilitation Assessment of Movement (STREAM)
n Confusion Assessment Protocol n Tests of endurance
n Disorders of Consciousness Scale o 2-Minute Walk Test
n Glasgow Coma Scale (GCS) o 3-Minute Walk Test
n Glasgow Outcome Scale (GOS) o 6-Minute Walk Test
n JFK Coma Recovery Scale–Revised o Borg Scale of Perceived Exertion
n Neurobehavioral Cognitive Exam o Maximum distance ambulated or propelled in wheelchair
n Neurobehavioral Functioning Inventory n Trunk Control Test
n Patient Competency Rating Scale n Trunk Impairment Scale
n Rancho Los Amigos Levels of Cognitive Functioning Posture
n Tests for depression n Observational description of posture
n Beck Depression Inventory Range of Motion
n Geriatric Depression Scale (GDS) n End feel assessment
n Tests for inattention and neglect n Goniometry
n Behavioral Inattention Test n Neural tension tests
n Clock Drawing Test Sensory Integrity
n Line Bisection Test n Sensory organization
n Motor-Free Visual Perceptual Test n Modified Clinical Test of Sensory Interaction on Balance
n Star Cancellation Test (mCTSIB)
Fatigue n Sensory Organization Test (SOT)
n Fatigue Severity Scale n Somatosensory
n Modified Fatigue Impact Score n Exteroception
Joint Integrity and Mobility o Monofilament testing
n Glenohumeral joint positioning o Superficial pain (sharp or dull)
n Palpation of subluxation o Superficial touch (light touch)
Muscle Performance and Motor Control o Temperature (can be left out if pain is normal per Goodman
n Reflexive motor and involuntary responses and Snyder)
n Babinski o Two-point discrimination
n Clonus (ankle, wrist) o Vibration
n Deep tendon reflexes (DTRs) n Proprioception
n Modified Ashworth Scale (tone, spasticity) o Joint position sense testing
n Motor Control Test (MCT) on force platform o Kinesthesia Test (Mirroring Test)
n Penn Spasm Frequency Scale o Thumb-finding test
n Automatic motor n Rivermead Assessment of Somatosensory Performance
n Adaptation Test (ADT) on force platform n Cortical Sensory Tests
n Nudge-push test (postural reactions to perturbations) o Graphesthesia
n Shoulder tug test (STT) (Parkinson disease) o Stereognosis
APPENDIX 8-B Sample Outcome Measures According to the Examination Areas

n
in the Guide to Physical Therapist Practice, from: Neurologic Practice Essentials:
A Measurement Toolbox*—cont’d
n Vestibular system testing n NIH Stroke Scale
n Dix-Hallpike Test (for benign paroxysmal positional vertigo n Stroke Impact Scale (also addresses activity and participation
[BPPV]) on ICF)
n Fukuda Step Test—50-step protocol n Unified Huntington’s Disease Rating Scale (UHDRS)
n Head-Shaking Nystagmus Test n Unified Parkinson’s Disease Rating Scale (UPDRS)
n Head Thrust Test Activity—List of Measures
n Motion Sensitivity Quotient (MSQ) (positional testing) Balance and Mobility
n Vibration-Induced Nystagmus n Berg Balance Scale
n Visual system testing and vestibuloocular reflex (VOR) tests n Dynamic Gait Index (DGI)
n Dynamic visual acuity (DVA) (clinical or computerized) n Functional Gait Index (FGA)
n Gaze stabilization test (GST) (clinical or computerized) n Tinetti Performance-Oriented Mobility Assessment (POMA)
n Oculomotor tests (saccades, smooth pursuit, vergence) (balance and gait)
n Perception time test (computerized) n Gait speed—10-meter walk test
n Static visual acuity Participation—List of Measures
n Visual field test (i.e., confrontation testing) n Activities-specific Balance Confidence Scale (ABC)
Pain n Craig Handicap Assessment and Reporting Technique (CHART)
n FACES Pain Scale n CHART-19—SF
n Visual Analog Pain Scale (0 to 10 numerical scale versus line n Dizziness Handicap Inventory (DHI)
bisection) n Geriatric Depression Scale
n Wheelchair User’s Shoulder Pain Index (WUSPI) n Modified Fatigue Impact Scale (mFIS)
Multi-Categorical Measures of Body Structure n Multiple Sclerosis Quality of Life–54 (MSQOL-54)
and Function n Parkinson’s Disease Quality of Life Questionaire-39 (PDQ-39)
n ALS Functional Rating Scale n Satisfaction With Life Scale (SWLS)
n Canadian Neurological Scale (stroke) n Short Form 36 (SF-36)
n Chedoke-McMaster Stroke Assessment Scale n Stroke Impact Scale (SIS)
n Fugl-Meyer Assessment of Motor Recovery After Stroke n Stroke Impact Scale–16 (SIS-16) Physical Performance Short Form
n Hoehn and Yahr Staging of Parkinson’s Disease n Tinetti Falls Efficacy Scale (FES)
n Kurtzke Expanded Disability Status Scale (multiple sclerosis) n Vestibular Activities of Daily Living Scale (VADL)
*Neurologic Practice Essentials: A Measurement Toolbox Development Team: Jane Sullivan (lead), Bill Andrews, Richard Bohannon, George Fulk, Desiree
Lanzino, Aimee Perron, Peggy Roller, Kirsten Potter, Yasser Salem, Teresa Steffen. Neurology Section Support and Coordinators: Nancy Fell, Karen McCulloch,
Dorian Rose.
APPENDIX 8-C n Pediatric Tools

Ages and Stages Questionnaire18,19 Harris Infant Neuromotor Test (HINT)140
Alberta Infant Motor Scale20-25 Health Utilities Index Mark 3141
Assessment, Evaluation, and Programming System for Infants and Infant Motor Screen142,143
Children26 Infant Neurological International Battery144-146
Battelle Developmental Inventory27-31 Milani-Comparetti Motor Development Screening Test147
Bayley Infant Neurodevelopmental Screener32-36 Movement Assessment of Infants148-153
Bayley Scales of Infant Development37-59 Neurological Assessment of the Preterm and Full-Term Newborn
Bruininks-Oseretsky Test of Motor Proficiency60-69 Infant154
Canadian Occupational Performance Measure2,70-84 Neurobehavioral Assessment of the Preterm Infant155
The Carolina Curriculum for Infants and Toddlers with Special Neonatal Behavioral Assessment Scale156-168
Needs85 Neonatal Neurobehavioral Examination169
The Carolina Curriculum for Preschoolers with Special Needs86 Neonatal Oral Motor Assessment Scale170
Child Health Questionnaire87-99 Peabody Developmental Motor Scales54,171-175
Childhood Health Assessment Questionnaire100 Pediatric Clinical Test of Sensory Interaction for Balance176
Children’s Orientation and Amnesia Test101 Pediatric Evaluation of Disability Inventory136,177-185
Clinical Observation of Motor and Postural Skills Test102 Pediatric Outcomes Data Collection Instrument (PODCI)186
DENVER II103-112 Pediatric Quality of Life Inventory187
Energy Expenditure Index (EEI)113 Infant/Toddler Sensory Profile188-191
Early Intervention Developmental Profile114 School Function Assessment192
Erhardt Developmental Prehension Assessment115 Sensory Integration and Praxis Test193,194
Functional Independence Measure for Children (WeeFIM)116-124 Sensory Profile195
Gillette Functional Assessment Questionnaire125 Test of Infant Motor Performance21,22,196-198
Goal Attainment Scaling126 Test of Sensory Functions in Infants199
Gross Motor Function Measure127-139 Toddler and Infant Motor Evaluation200,201
APPENDIX 8-D n Quality-of-Life Tools from the World Health Organization

World Health Organization Quality of Life instrument (short form) (WHOQOL-BREF)202-206
World Health Organization Quality of Life instrument (long form) (WHOQOL-100)205
World Health Organization Disability Assessment Schedule 2.0—36 items202,207-209
Special note: Condition-specific examination tools can be found in the respective chapters:
Spinal Cord Injury: Chapter 15, Chapter 16
Neuromuscular Diseases: Chapter 17
Multiple Sclerosis: Chapter 19
Basal Ganglia Disorders: Chapter 20
Cerebellar Disorders: Chapter 21
Balance and Vestibular Disorders: Chapter 22
Hemiplegia: Chapter 23
Head Injury: Chapter 24
Aging, Dementia, and Disorders of Conditions: Chapter 27
CHAPTER 9 Interventions for Clients with Movement
Limitations
DARCY A. UMPHRED, PT, PhD, FAPTA, NANCY N. BYL, PT, MPH, PhD, FAPTA,
ROLANDO T. LAZARO, PT, PhD, DPT, GCS, and MARGARET L. ROLLER, PT, MS, DPT

augmented intervention After reading this chapter the student or therapist will be able to:
evidence-based practice 1. Appreciate the complexity of motor responses, and discuss methods used to influence
functional training body systems and their effects on functional behaviors.
impairment training 2. Outline the differences in recovery related to healing, compensation, substitution,
neuromuscular retraining habituation, and adaptation.
3. Analyze the similarities and differences among impairment training of specific body
systems, functional training, augmented feedback training, and learning-based
sensorimotor retraining.
4. Select appropriate intervention strategies to optimize desired outcomes.
5. Analyze variables that may both positively and negatively affect complex motor
responses and a patient’s ability to participate in functional activities.
6. Identify procedures and sequences required to attain the most successful therapeutic
outcome that best meets the needs and goals of the client and the family.
7. Consider the contribution of the client, the client’s support systems, research evidence,
neurophysiology, and the best practice standards available to optimize outcomes.
B efore discussing therapeutic intervention procedures, the

therapist must identify the learning environment within
which the client will perform. As discussed in Chapter 1, that
Both the American Occupational Therapy Association
(AOTA) and the American Physical Therapy Association
(APTA) have developed guides to practice that help to direct
environment is made up of the therapist and the client, all therapists entering the professions and should help to guide
internal body control mechanisms of the client, and the exter- practice throughout their working lives.2,3 APTA, through
nal restraints and demands of the world. Although this text the initiation of the California Physical Therapy Associa-
focuses on relearning functional movement, the reader must tion, has been collecting and classifying evidence-based
always consider all aspects of the client including how other articles through the Hooked on Evidence project.4 Through
organs or body systems will be affected by or will affect the the use of current evidence-based practice; sensorimotor
therapeutic outcome both during rehabilitation and in relation processing, motor control, motor learning, and neuroplastic-
to long-term quality of life. An examination and evaluation ity theories (see Chapter 4); and body systems models, the
(see Chapter 8) are performed before intervention to establish therapist must determine the flexibility or inherent motor
movement diagnoses. These examinations lead to movement control the client demonstrates while executing functional
diagnoses that must link to functional limitations or restric- activities and participating in life. This chapter or other
tions in activities and their causations (body system problems). chapters in the book cannot establish for the reader the exact
Movement diagnoses and the degree and extent of the system treatment sequence that should be used for every patient, but
or subsystem dysfunction or impairments determine prognosis an example of a decision-making pathway has been given in
of the outcomes on the basis of the client’s potential for func- Box 9-1. Functional goals must be established that lead to
tional improvement. Factors such as motivation, family sup- the client’s ability to participate in life within his or her
port, financial support, and cultural biases must be considered environment and whenever possible lead to or maintain the
as part of the prognosis.1 This process guides the selection of quality of life desired by the client. Similarly, the therapist
intervention strategies. Although it could be assumed that must differentiate whether the observed motor problems
some of these impairments would be directly correlated to are based on acute or longstanding impairments before
the central nervous system (CNS) trauma experienced by the establishing timelines for prognosis.
client, it must also be determined whether some or most of Before beginning any intervention, the therapist must
these impairments have developed over a lifetime as a result of determine the treatment strategies that will be used to help
small traumas and adjustments to life. This insidious cause the client attain the desired functional outcomes. The spe-
of impairments needs to be differentiated from acute causation cific environment used by the therapist to optimize patient
of activity limitations because goal setting and expectations performance will depend on the functional level and amount
related to prognosis and recovery can be different. of motor control exhibited by the patient. The following
191
BOX 9-1 n TREATMENT STRATEGY CATEGORIES
COMPENSATION TRAINING
Use of an assistive device or orthotic to compensate for a permanent impairment or lost body system function.
SUBSTITUTION TRAINING
Teaching the client to use a different sensory system or muscle(s) group to substitute for lost function of another system.
An example of sensory substitution might be teaching the client to use vision to substitute for an impaired vestibular system or
somatosensory system for balance function. Substitution within the motor system might be teaching hip hiking to substitute for
lack of dorsiflexion of the ankle during swing phase of gait.
HABITUATION TRAINING
Activity-based provocation of symptoms with the goal of symptom reduction with repetitive practice. An example would be teaching
head movement to a patient who has a chronic labyrinthitis and severe nausea with any head movement.
NEURAL ADAPTATION
Driving changes in structure and function of the central or peripheral nervous system with repetitive, attended practice. This
category would be considered neural plasticity. This category of treatment strategy takes the greatest repetition of practice and
requires a strong desire by the individual to gain the functional ability and realize the potential of the central nervous system to
change.
classifications can be used to document the specific role of Thus, this perturbation moves each foot reciprocally back-
the therapist within the training session (refer to Chapter 4 wards and the body forward, triggering a stepping reaction.
for additional detail): In the case of an individual after a cerebrovascular accident
Functional training: Practice of a functional skill that is (CVA), one leg will still respond normally, thus helping to
meaningful, goal directed, and task oriented. Patient trigger a between-limb reciprocal stepping action of the in-
will experience errors and self-correct as the program volved leg. In the case of bilateral involvement, both legs
becomes more automatic and integrated. An example may need placement, requiring two people to assist. The
would be gait training on a tile surface, rugs, inclined activity may be classified as impairment training, with the
surfaces, compliant surfaces such as grass, and so on focus on appropriate power production or cardiovascular
to practice ambulation. fitness, leading to functional training to trigger normal
Body system or impairment training: Treatment focus motor programs necessary for gait. Simultaneously, aug-
is on correcting a body system problem during an mented training done by a therapist includes manual assis-
activity (e.g., pure muscle strengthening, stretching, tance in the direction, rate, and placement of the involved
sensory training, endurance training). leg throughout the gait cycle. In this previous example,
Augmented feedback training: Patient needs external therapists need to make sure they are aware of the patient’s
feedback (auditory, visual, kinesthetic) and control center of gravity and do not move the foot before it should
over the motor program running the target task. This be at “push off” during the gait cycle. This activity would
will limit the response patterns (e.g., reducing degrees not be considered functional training until the client could
of freedom, reduction or enhancement of tone) for reciprocally move both legs during the gait pattern without
successful performance of the desired movement the need of the harnass for postural support and the therapist
(e.g., handling techniques, body-supported treadmill to guide the movement.
training, constraint-induced training). When selecting from a variety of treatment interventions
Learning-based sensorimotor retraining: Treatment focus (neuromuscular retraining, functional training, impairment
is placed on improving sensory discrimination dys- training, and augmented feedback training), it is important
function as a consequence of somatosensory, premotor, for the therapist to consider that each one is based on differ-
and motor cortical disorganization resulting from ent strategies and rationales that contribute to the expected
trauma, degeneration, or overuse. outcome. All interventions should address the needs of
Clients with CNS damage often benefit from combining the patient and must consider any emotional and cognitive
interventions from the above categories. An example of this restraints. Although these intervention methods can be used
might be the early phase of partial body-weight supported simultaneously or in various combinations, the clinician
treadmill training. In the early phases, a therapist or assistant needs to consider which aspect of the intervention falls into
is guiding the client’s leg during swing and stance phases which treatment classification. Although various treatment
while the body harness supports a proportion of the client’s outcomes can be measured, if classification of each treat-
total weight (augmented feedback) to assist the postural ment variable is not identified, the determination of how and
system in running appropriate programs to maintain balance why the outcomes were influenced by the intervention
and decrease the power needed to generate a more normal becomes confusing and difficult to distinguish. Without
gait pattern. This augmented intervention is being done in a understanding the interactions of intervention methods and
functional pattern within an environment that perturbs the the outcome, treatment effectiveness and future clinical
client’s base of support under the normal center of gravity. decision making remain unpredictable, and unique practice
CHAPTER 9 n Interventions for Clients with Movement Limitations 193
patterns and pathways are hard to identify with consistency. whereas OTs were trained in activity analysis and treatment
A master clinician who is effective with all patients but does that identified and optimized the functional activities that
not know how and why the decisions are made along the resulted from the impairments. Few clinicians seemed to
intervention pathway cannot leave a legacy of effectiveness focus on the sequential or interactive aspect of lack of func-
that will ever lead to efficacy. Although not all graduates or tion with specific impairments. Thus after the onset of
inexperienced clinicians may have the innate aptitude or a stroke the PT would strengthen and evaluate range of
potential to become master clinicians, if professionals motion (ROM) of the leg and trunk, whereas the OT would
understand the verbal, spatial, cognitive, fine and gross encourage the patient to try to functionally use the arm. The
motor, and emotional sensitivity variables that play a role in PT would be preparing the patient to transfer out of bed and
the evolution toward mastery, educational experiences might get into and out of a chair and then helping the patient walk,
be able to nurture future colleagues along this pathway and whereas the OT would be preparing the patient to use the
help those with mastership potential reach that level of arm in functional activities such as grooming or eating. Both
function earlier in their professional careers. therapists hoped the patient would accept responsibility for
The reader must also remember that intervention encom- continued improvement through practice. What both profes-
passes multiple interactive environments where intervention sions discovered was that the patient generally did not
decisions are often made moment by moment during any regain normal motor control. He or she might be able to
treatment period. The challenge to the educated clinical walk and might be able to move the shoulder, but the move-
professional is to determine what is being done, why it ment strategies were generally stereotypical, were abnormal
is working, how to continue its effectiveness, and how to in patterns, and took tremendous effort by and energy from
determine the progress of the successful intervention. The the patient to perform. Over time, clients lost the motivation
clinician must also determine how to empower the client to even try, and thus what had been gained through therapy
(emotionally, cognitively, and motorically) to take over the may have been lost from lack of practice once they got
intervention with inherent, automatic mechanisms that lead home. There was also minimal recovery of functional hand
to fluid, flexible, functional outcomes independent of use, often because of the tremendous effort a patient had to
both the therapist and the environment within which the use to move the shoulder to place the hand somewhere.
activity is occurring. It is not until clinicians can determine Once that effort had been used the tightness and increased
effective treatment outcomes from various interventions that tone in the hand prevented functional use. Although func-
efficacy within a research laboratory can be studied without tionally independent skills as measured on the Functional
speculation and hypothesis formation based on speculation.1 Independence Measure were achieved, normal movement
Effectiveness is the first way to determine evidence-based patterns and normal motor control were rarely restored, and
practice. Once effectiveness has been established through quality of life was clearly affected for the patient and family.
case studies and larger controlled studies within the clinical During the decade or two before the 1960s, some talented
environment, researchers can begin to tease out separate and intelligent clinicians began to question the traditional
variables and establish efficacy as part of evidence to justify intervention strategies used by the OT and PT. These pio-
clinical decision making. neers5-29 in neurological rehabilitation set the stage for the
development of new concepts that allowed basic science to
HISTORY OF DEVELOPMENT OF infiltrate the clinical arena. The intervention strategies of
INTERVENTIONS FOR NEUROLOGICAL Jean Ayers, Berta Bobath, Signe Brunnstrom, Margaret
DISABILITIES Johnstone, Susanne Klein-Vogelbach, Margaret Knott,
In the mid 1900s the interventions by physical therapists Dorothy Voss, Margaret Rood, and others became popular.
(PTs) and occupational therapists (OTs) were separate. Gen- Colleagues observed these master clinicians and could
erally, PTs worked on gross motor activities with specific easily see that the “new” interventions were much more
emphasis on the lower extremities and the trunk, whereas effective and provided better outcomes than previous inter-
OTs worked on the upper extremities and fine motor activi- ventions. Each approach focused on multisensory inputs
ties. Both professions focused on daily living skills, with introduced to the client in controlled and identified se-
those involving the arms falling within the domain of the OT quences. These sequences were based on the inherent nature
and those involving the legs falling within the domain of the of synergistic patterns5,21,30,31 and motor patterns observed in
PT. Activities that required gross motor skills such as sitting, humans5,7,32 and lower-order animals33 or a combination of
coming to stand, walking, walking with assistive devices, the two.19,21 Each method focused on the individual client,
and running fell within the purview of the PT, whereas the specific clinical problems, and the availability of alterna-
grooming, hygiene, and eating were the responsibility of the tive treatment approaches within an established framework.
OT. Today, this approach is considered ridiculous owing to Some of these approaches focused on specific neurological
our understanding of motor learning, neuroplasticity, and medical diagnoses. The treatment emphasis was then on
motor programming and control. In the past it was also specific patients and their related movement disorders. Chil-
accepted that the PT worked on specific system problems dren with cerebral palsy and head injuries7,23,28 and adults
such as weakness, inflexibility, lack of coordination, and with hemiplegia8,9,21,32 were the three most frequently identi-
voluntary control, whereas the OT worked on functional fied medical diagnostic categories. In 1968 at Northwestern
activities integrated within the environment (such as dress- University a large conference was held and laid the founda-
ing) and the patient’s emotional needs and desires (occupation for the first STEP conference (Northwest University
tional expectations). According to the terminology of the Special Therapeutic Exercise Project [NUSTEP]). Most of
mid to late twentieth century, PTs were trained to identify these master clinicians, along with research scientists of the
and correct impairments that caused functional limitations, day, came together to try to (1) identify the commonalities
and differences between these approaches, and (2) integrate hypertonic pattern. One method may have been better than
and use the neuroscience of the day to explain why these the others given a particular patient, but in truth improved
approaches worked.34 Since the 1970s, substantial clinical patient performance may have stemmed not from the method
attention has also been paid to children with learning and itself, but rather from the preferential CNS biases of the
language difficulties.5,13,35 Now these concepts and treatment client and the variability of application skills among the
procedures have been applied across the age spectrum for all clinicians themselves. That is, when a therapist intentionally
types of medically diagnosed neurological problems seen uses specific augmented feedback to modulate the motor
in the clinical setting (refer to Section II of this text). This system’s response to an environment but does not identify
expansion of the use of any of the methods for any patho- the other external feedback present within that environment
logical condition manifested by insults from disease, injury, (e.g., lighting, sound, touch, environmental constraints),
or degeneration of the brain seems to be a natural evolution therapeutic results will vary. Because of variance, efficacy
given the structure and function of the CNS and commonali- of intervention is often questionable, although the effective-
ties in system problems and activity limitations that take the ness of that therapist may be easily recognized.
individual away from participating in life. Because of the overlap of treatment methods and the
Fortunately, most dogmatism no longer persists with infiltration of therapeutic management into all avenues of
respect to territorial boundaries identified by clinicians neurological dysfunction, various multisensory models were
using some specific intervention methods. A conference in developed during the early 1980s.13,38-41 These have contin-
199036 played a significant role in challenging the relevance ued to evolve into acceptable methods in today’s clinical
of these territorial boundaries and stressed the adoption of a arena. Although these models attempted to integrate existing
systems model when looking at impairments, activity limita- techniques, in reality they have created a new set of holistic
tions, and participation in life interactions.37 As the boundar- treatment approaches. In July 2005 the III STEP confer-
ies for interventions began blurring, intervention approaches ence42 was held in Utah to again bring current theories and
such as proprioceptive neuromuscular facilitation (PNF) evidence-based practice into today’s clinical environment.
were then integrated into the care of clients with orthopedic The history of the three STEP conferences demonstrates the
problems and patients with neurological impairments. evolution of evidence-based practice from the first confer-
Today, few universities within the United States teach sepa- ence, where basic science was the only evidence to justify
rate sections or units on specific approaches, but rather teach treatment, to the second conference, where evidence in
students to identify problems, when they are occurring in motor learning and motor control began to bring efficacy to
functional programs, and what bodily systems might be the intervention. By the time the third conference was held, the
cause of those activity limitations. research in neuro/movement science regarding true efficacy
For example, assume that a client with hemiplegia exhib- within practice and the reliability and validity of our exami-
ited signs of a hypertonic upper-extremity pattern of shoul- nation tools set the stage for standards in practice.43 Where
der adduction, internal rotation, elbow flexion, and forearm the next conference will take the professions and how soon
pronation with wrist and finger flexion. Brunnstrom8 would that will occur is up to colleagues in the future. No proceed-
have identified that pattern as the stronger of her two upper- ings from that third conference were published, but over the
extremity synergies. Michels,21 although using an explana- preceding years articles covering most of the presentations
tion similar to Brunnstrom’s to describe the pattern, would had been published in the Journal of Physical Therapy. The
have elaborated and described additional upper-extremity ultimate goal would be to develop one all-encompassing
synergy patterns. Bobath would have asserted that the client methodology that allows the clinician the freedom to use
was stuck in a mass-movement pattern resulting from abnor- any method that is appropriate for the needs and individual
mal postural reflex activity.30 Although the conceptualiza- learning styles of the client as well as to tap the unique indi-
tion of the problem certainly determined treatment proto- vidual differences of the clinician. Although intervention
cols, the pattern all three clinicians would have worked today is based on an integrated model, the influence of third-
toward was shoulder abduction, external rotation, elbow party payers, the need for efficacy of practice, and time
extension, forearm supination, and wrist and finger exten- constraints often factor into the therapist’s choice of inter-
sion. The rationale for the use of this pattern within an intervention. Visionary and entrepreneurial practice ideas that
vention period would vary according to the philosophical have the potential to be effective will always be a challenge
approach. One clinician might describe the pattern as a to future therapists. Those ideas generally originate within
reflex-inhibiting position (Bobath).31 Another would de- the clinical environment and not the research laboratory.
scribe the pattern as the weakest component of the various For that reason, clinicians need to communicate ideas to the
synergies (Brunnstrom),8 whereas still another might iden- researcher, and then those researchers can develop research
tify the pattern as producing an extreme stretch and rota- studies that test the established efficacy or refute that effec-
tional element that inhibited the spastic pattern (Rood).25 tiveness. Few researchers are master clinicians, and few
How those master clinicians sequenced treatment from the clinicians are master researchers; thus collaboration is
original hypertonic pattern to the opposite pattern and then needed as the professions move forward in establishing
to the goal-directed functional pattern would vary. Some evidence-based practice.
would facilitate push-pull patterns in the supine and side- Today’s therapists have replaced many of the existing
lying positions and rolling. Others would look at propping philosophical approaches with patient-centered therapeutic
patterns in sitting clients or at weight-bearing patterns of intervention. Patient performance, available evidence, and
clients in the prone position, over a ball or bolster, or in the expertise of the clinician often play a key role in the
partial kneeling. All have the potential of improving the specific decision regarding an intervention. When con-
functional pattern of the upper extremity and modifying the fronted with an abnormal upper-extremity pattern, today’s
therapist may choose to work on improving the movement understandable and repeatable. As new scientific theories
pattern using a functional activity. Control of the combina- are discovered, new information must be integrated to con-
tion of movement responses and modulation over specific tinue to modify treatment approaches.
central pattern generators or learned behavior programs will
allow the patient opportunities to experience functional INTERVENTION STRATEGIES
movement that is task oriented and environmentally specific.
With goal-directed practice of the functional activity, neuro- Functional Training
plastic changes, motor learning, and carryover can be Functional training is a method of retraining the motor
achieved.44 With a better scientific basis for understanding system using repetitive practice of functional tasks in an
the function of the human nervous system, how the motor attempt to reestablish the client’s ability to perform activi-
system learns and is controlled, and how other body ties of daily living (ADLs) and participate in specific life
systems, both internal and external to the CNS, modulate activities such as golfing, fly-fishing, basketball, or bridge.
response patterns, today’s clinicians have many additional This method of training is a common and popular interven-
options for selection of intervention strategies.45-54 Whether tion strategy used by clinicians owing to the fact that it is a
a patient would initially benefit best from neuromuscular relatively simple and straightforward approach to improving
retraining, functional retraining, or a more traditional deficits in function. A system problem such as weakness in
augmented or contrived treatment environment is up to the the quadriceps muscle of the leg can be treated by muscle
clinician and is based on the specific needs identified during strengthening in a functional pattern that can be easily mea-
the examination and evaluation process. sured. Because of its inherent simplicity, functional training
No matter what treatment method is selected by a clini- is sometimes misused or abused by clinicians. Most patients
cian, all intervention should focus on the active learning with neurological deficits have multiple subsystem prob-
process of the client. The client should never be a passive lems within multiple areas, which forces the CNS to use
participant, even if the level of consciousness is considered alternative movement patterns in order to try to accomplish
vegetative, nor should the client be asked to perform an the functional task presented. If the therapist accesses a
activity when the system problems only create distortion or motor plan such as transfers but allows the patient to use
demonstrate total lack of control of the desired movement. programs that are inefficient, inappropriate, or stereotypical,
With all interventions requiring an active motor response, then the activity itself is often beyond the patient’s ability.
whether to change a body system impairment such as by The patient may learn something, but it will not be the
increasing or reducing the rate of a motor response, modu- normal program for transfers. This activity often leads to
late the tonal state of the central pattern generators and additional problems for the client.
learned motor behaviors, or influence a functional response In Chapter 8 the steps involved in the examination pro-
during an activity, the client’s CNS is being asked to process cess are explained in detail. The intricate relationship of
and respond to the external world. That response needs to body system problems, impairments, and functional limita-
become procedural and controlled by the patient without tions that decrease participation in the rehabilitation process
any augmentation to be measured as functionally indepen- are discussed. Functional training can be implemented once
dent. In time, the ultimate goal is for the client to self- the clinician has identified the client’s activity limitations.
regulate and orchestrate modulation over this adaptable and The clinician must first answer the questions “What can the
dynamic integrated sensorimotor system in all functional client do?” “What limitations does the client have when
activities and in all external environments. engaging in functional activities?” “Are there motor pro-
A problem-oriented approach to the treatment of any grams that are being used to substitute for normal motor
impairment or activity limitation implies that flexibility and function?” and “Can the therapist use functional training to
neural adaptation are key elements in recovery. However, improve body system problems within the context of the
adaptation should not be random, disjointed, or non–goal functional skill?” Once the therapist has an understanding of
oriented. It should be based on methods that provide the best the reasons for any activity limitation and can alleviate sub-
combination of available treatment alternatives to meet stitution and compensation for the deficit, functional tasks
the specific needs of the individual. Development of a should be identified and practiced.
clinical knowledge bank enables the therapist to match treat-
ment alternatives with the patient’s impairments, activity The Effect of Functional Training on Task
limitations, objectives for improved function, and desired Performance and Participation
quality of life. A professionally educated therapist no longer The main focus of functional training is the correction of
bases treatment on identified approaches, although specific activity limitations that prevent an individual from partici-
aspects of those approaches may be treatment tools that will pating in life. However, through repetitive practice of func-
meet the client’s needs and assist him or her in regaining tional tasks and gross motor patterns, many of the client’s
functional control of movement. Treatment is based on an impairments can also be affected. For example, if a therapist
interaction among basic science, applied science, the thera- practices sit-to-stand transfers with a client in a variety of
pist’s skills, and the client’s desired outcomes.49-52,55,56 environments and performs multiple repetitions of each type
In most cases, multiple intervention strategies must be of transfer, not only can learning be reinforced, but the client
included, but the therapist needs to be able to identify why can also gain strength in the synergistic patterns of the lower
those selected treatments will lead to system improvement extremities that work against gravity to concentrically lift
as well as documenting those findings using reliable the client off of the support surface and eccentrically lower
standardized and acceptable clinical methods and terminol- him or her down. Weight bearing through the feet in a vari-
ogy. These intervention strategies must be dynamic yet also ety of degrees of ankle dorsiflexion during transfer training
will effectively place the ankles in functional positions. The may result in the development of abnormal, stereotypical
act of standing also helps the trunk and neck extensors to movement and potentially create additional impairments. An
engage in postural control. Varying the speed of the activity example of this is using transfer training when the patient is
during the treatment can stimulate cerebellar adaptation to unable to keep the program within the limits that define it as
the movement task. Moving from one position to another a transfer. What instead happens is that the patient would
with the head in a variety of positions stimulates the ves- begin to fall. Once in that situation, the patient is then work-
tibular apparatus and may assist in habituating a hypersensi- ing on approaches to prevent from falling, not activities that
tive vestibular system, allowing the client to change body allow the patient to safely transfer. The therapist’s decision
positions without symptoms of dizziness, resulting in a regarding what functional patterns or activities to practice,
higher quality of life. Repetitive practice also affects the and in what order, will depend on several factors. The thera-
vasomotor system and may assist in habituating postural pist must choose functional activities that are necessary for
hypotensive responses. the client to perform independently or manage with less help
A good example of the misuse of functional training is before being discharged home. For PTs, safe transfers and
the “nag-and-drag” method of gait training in the parallel ambulation are generally the focus of functional training.
bars. This method finds the therapist literally dragging the For OTs, independent bathing, dressing, and feeding are
client through the length of the parallel bars in an attempt to major foci. Yet both PTs and OTs also need to be sensitive
elicit some sort of movement response from the client. The to the activities that the patient or the patient’s family want
therapist then labels this procedure “gait training.” Clearly, to improve to enhance the quality of life for everyone
this approach will result in the client eventually learning involved in the person’s case. The ability to get in and out of
dysfunctional, inefficient motor programs. Before long, as a car might be the most important activity for the client to
the client learns to run these dysfunctional programs proce- learn because he or she needs to make frequent trips to
durally, the clinician will realize that he or she has created the physician’s office and the primary caregiver has
a bigger problem, and a considerable amount of time and cardiac problems and is unable to assist the patient in trans-
resources may be required to undo the damage that was cre- ferring without placing his or her own cardiac system at
ated by limiting the available movement strategies, limiting extreme risk.
the variability within practice, and ultimately restricting the It is suggested that the clinician modify or “shrink” the
plasticity of the nervous system. Similarly, forcing the axial environment to allow normal motor programs to run. An
trunk musculature to compensate for lack of motor control example of this might be to limit the ROM an individual is
within the elbow and wrist will result in dysfunctional allowed while performing a rolling pattern. The therapist
upper-extremity movement patterns. may opt to start this movement with the patient in a side-
Functional training is the best method of intervention lying position. The amount of patient movement may be
when the client can run normal programs that have some even further limited by the therapist stabilizing the patient’s
limitation such as poor ROM or inadequate muscle power hips by using the therapist’s one leg in kneeling position
from disuse. In that way, functional training will run normal against the patient’s posterior pelvis and the therapist’s other
programming until fatigue sets in, which may be after only leg in half-kneeling position with the top leg of the patient
one or two repetitions. Increasing the repetitions and/or the over the therapist’s half-kneeling leg. In this way the indi-
power necessary to run the programs will lead to functional vidual’s body can be totally controlled by the therapist; the
improvement. In using functional training, accurate stan- patient can be encouraged to roll the upper part of his trunk
dardized measurement tools that clearly illustrate change both backward with the arm reaching back and then forward
will quickly tell the therapist whether the change is in the with the arm coming across the body toward a weight-
direction of more functional control or additional limitation. bearing pattern on the hand. The therapist can change the
An intervention approach in the early 1990s that evolved rate of movement and also use his or her knees to control the
as an offshoot of functional training was labeled clinical range that the patient is allowed. The environment can be
pathways. These pathways were established by health progressively “enlarged” to allow the client to perform the
care institutions to improve consistency of management of activity in a functional context. Although this narrowing of
patients who met specific medical diagnostic criteria. It the functional environment would be considered a contrived
has been proven that the implementation of these pathways environment and must not be recorded as functional as
reduces variability in clinical practice and improves patient defined in a functional or activities-based examination, it
outcomes.67 Health care practitioners also became aware may allow the nervous system the opportunity to control and
that some individuals do not fall into these pathways and modify the motor programs within the limitations of its
need to be treated according to the specific clinical problems plasticity at the moment. Therefore this therapeutic tech-
that the patients were presenting. nique could be used within a functional training environ-
ment or may fall into an augmented treatment approach
Selection of Functional Training Strategies category, given an individual who has neurological prob-
What is the “ideal” procedure for effectively and efficiently lems that prevent normal movement.
using functional training as a treatment intervention? First, The goal of therapy is to move toward functional training
it is suggested that the clinician identify and select proce- as quickly as the client’s motor system can control the
dures that will use the client’s strengths to regain lost func- movement. As learning and repetition assist the CNS in wid-
tion and correct system limitations—“What can the client ening the response pattern during a functional activity, the
do?” The clinician is also advised to avoid activities that client’s ability to respond to variance within the environment
may be too difficult and elicit compensatory strategies that will enlarge and assist in gaining greater independence.
An example of this application of functional training might

be asking a client to perform a stand-to-sit transfer. The CASE STUDY 9-1 n FUNCTIONAL
client is first guided down to sitting onto a large gym ball, a TRAINING: AMBULATION
high-low table, or a stool that allows the client to sit only
Teaching a client to ambulate can be approached in many
one fourth to one half of the way down before returning
ways. Assume that the objective for a particular session is
to stand. As the client develops increased strength and bal-
ambulation. First, the client may be asked to ambulate in the
ance and improved control over abnormal limb synergies
parallel bars using the upper extremities to assist in forward
and tone in this pattern, then a smaller gym ball or a lower
progression of the movement to decrease fear and to assist in
point on a high-low table can be used. Finally, the client is
maintaining balance. Once the patient can perform this ambu-
asked to sit down onto a ball/mat or chair that results in the
latory activity, the therapist might decide to progress the
patient sitting with the hips and knees at 90 degrees. Once
patient’s ambulation by introducing a walker, which has four
the client can sit down and return to a vertical position, the
points of support. Ambulating with the walker will again
next task will be to sit down, relax, and then stand up. Once
increase power production in the legs and create an environ-
that activity is done easily, the client will be functionally
ment of safety for the client. Once walking with the walker
able to stand to sit and to reverse the movement pattern to sit
can be performed at various speeds and distances, the therapist
to stand.
may advance the activity to using two canes, then one cane
Although many clinicians understand the importance of
depending on the client’s balance, coordination, and need.
running motor tasks within an appropriate biomechanical,
While the patient is practicing ambulating with cane(s), he
musculoskeletal, and sensorimotor window in which the cli-
may also be walking on a treadmill to increase endurance,
ent has the ability to perform procedures functionally, it may
velocity of gait, and power. Once the patient can ambulate
be argued that in many cases this particular type of treatment
safely with a cane, the therapist may decide to transition to
strategy is simply not possible in a real-world situation. For
walking without any assistive devices. Again the patient may
example, given the current health care environment, if the
first be asked to walk on a treadmill while holding on with his
client is given a limited number of visits to achieve the
arms until he feels safe walking and no longer needs an assis-
desired outcome, the clinician may conclude that there is no
tive device. The therapist could transition to ramps, obstacles,
choice but to “allow as many degrees of freedom as possi-
uneven ground, and so on. All these activities would require
ble” or, in other words, to “force the window open” no mat-
the individual to begin with functional control over the pro-
ter the abnormal movement patterns used or the limitations
gram for ambulation. All the activities are focused on regain-
in independent functional control that they may produce.
ing independence in the functional activity of walking, using
In summary, the clinician should first identify and
repetitive practice. These therapeutic devices assist the patient
emphasize the client’s strengths (“What can the client
in successfully practicing the entire gait cycle on both legs.
do?”) and use those strengths to efficiently and effectively
In time, the patient is asked to continue walking without the
achieve functional change. Next, the clinician must priori-
need of the assistive devices and will continue to practice that
tize what systems or activities the client truly needs to
activity as functional movement or is considered functionally
change. The choice of what activities to emphasize during
independent with the use of an assistive device. The therapist
therapeutic training always poses a dilemma to therapists.
must also remember that when introducing an assistive device,
Although it may be ideal for the client to eventually be
that device itself will usually limit the environments within
able to ambulate independently on all surfaces without any
which a patient can ambulate independently.
assistance or reach for any object in and from any spatial
position, it may be more important initially for the client to
be able to safely transfer from the bed to the wheelchair, sit
independently while someone assists with dressing, or Conclusion
walk and transfer onto and off of the commode indepen- One important variable that has clearly been identified
dently at home. One should keep in mind that although with respect to functional training is “task specificity.”47,68-76
several skills may be learned by training them simultane- Although it is important that a patient be independent in as
ously, it may make more sense to concentrate on the safe many ADLs as possible, often the therapist, the patient, and
performance of one or two necessary functional tasks the family need to prioritize which activities are most impor-
rather than having the client end up being able to perform tant to the quality of life of the patient. If walking into the
multiple tasks that require considerable outside assistance mountains to do “birdwatching” is one important goal to the
for safety. The need to work functionally on additional patient, then creating an environment that would closely
activities may also be an opportunity for the clinician to resemble the environment of that activity is crucial. Simi-
request additional therapy visits for the client, arguing that larly, practice within that environment is a key to successful
there is a reasonable expectation that more intervention carryover (see Chapter 4). If the patient wants to walk into
would result in a greater increase in function and a greater the mountains and the family expects the patient to walk into
decrease in the risk for potential injury than if the interven- his or her old job, a therapist must accept that motivation
tion were not continued. The use of valid and reliable will drive behavior and task specificity will drive learning.
functional outcome measures becomes critically important Carryover into any other functional activity such as walking
in case management. These tools objectively measure the into the office building in order to go back to work may not
effect of the intervention, help predict the potential risks be the motivating factor that will guide that individual’s
if the therapy is not continued, and ultimately aid in the desire to perform that motor task. Whether the patient ever
justification to continue therapeutic intervention. goes back to work is not the variable that should be used as
part of the motivational environment for task-specific gait environment will result in more meaningful changes in func-
training geared to walking in the mountains and is not a tion. Impairment training can be a very effective treatment
decision for which the therapist is responsible. Therapists approach. It can lead to functional gains after an improvement
need to allow the patient to tell them what will be the most in a specific body system problem. This can lead to improved
important task and the specificity of that task to optimize participation in not only normal functional activities but also
motor learning and functional recovery. activities that should lead to a better quality of life.
Often, clients with neurological trauma or disease cannot
Body System and Impairment Training begin therapy with functional or impairment training
As mentioned in Chapter 8, the therapeutic examination because of the degree and extent of impairments within the
results in the identification of activity limitations and pos- entire CNS. Therapists must then choose augmented
sible body system and subsystem impairments that are caus- therapeutic interventions that externally guide the client’s
ing the functional movement disorders. Impairment training learning through hands-on and environmentally controlled
is another intervention strategy that involves the correction techniques such as a body-weight–supported treadmill train-
of impairments with the expectation that improving these ing (BWSTT). It is cautioned that the therapist should not
impairments will result in a corresponding improvement in consider these interventions as functionally independent
function. For example, when a client has the inability until the individual’s success is based on internal self-
to stand up without assistance (activity limitation) and the regulation of movement. The clinician must continually
clinician determines the cause to be lower-extremity weak- strive to transfer control to the client by widening the win-
ness, an appropriate approach may be to strengthen the dow of independence and limiting the manual or verbal
lower extremities (impairment training). Numerous studies guidance used during therapy.
have shown the effectiveness of impairment training in
improving the functional performance of individuals with Augmented Therapeutic Intervention
neurological conditions such as cerebral palsy,77,78 stroke,79-87 As discussed in the previous section, some treatment alter-
multiple sclerosis,88-93 Parkinson disease,94-98 and other neu- natives require little if any hands-on therapeutic manipula-
romuscular diagnoses.99-110 The strengthening intervention tion of the client during the activity. For example, the patient
selected should reflect the task and the environment within practices transfers on and off many support surfaces with
which the impairment was identified. The clinician should standby guarding only. Thus the client self-corrects or uses
attempt to create a training situation so that the client may inherent feedback mechanisms to self-correct error to refine
be able to run the necessary motor programs with all the the motor skill. This ultimate empowerment of the client
required subsystems in place. For example, training sit to allows each individual to adapt and succeed at self-identified
stand with weakness in the hip and knee extensors is much and self-motivated objectives first with augmented interven-
less likely to automatically result in the improvement of tion and finally without any assistance. Often, allowing the
sit-to-stand function if the therapist begins the activity in client to try to succeed without assistance enables the thera-
sitting where generation of extension is most difficult, than pist to evaluate what components of the task the client can
if the strengthening training was performed with repetition control and what components are not within the client’s cur-
of practice starting in standing and going to sit and back rent capabilities, especially if normal, fluid, efficient, and
again to stand. By decreasing the degrees of freedom of the effortless movement is the desired outcome. In some cases
eccentric control of the hips and knees when going from the therapist may use hands-on skills or augmented aids
stand to sit, the functional training activity has turned into such as BWSTT, which would substitute for many aspects
specific impairment training. The therapist can ask the of the environment and allow the client to succeed at
patient to eccentrically lengthen the extensors only in a lim- the task—but the control and feedback during the activity
ited range and then concentrically contract back to standing. would be considered augmented feedback and fall into that
As the power increases, the degrees of freedom can also be classification.
enlarged until the patient is able to complete the task of These augmented techniques make up a large component
stand to sit while simultaneously regaining the sit to stand of the therapist’s specific interventions tool box. The differ-
pattern. In pure impairment training a patient might also be ence between augmented and functional training might be
asked to straighten the knee when sitting or to extend the hip the need for the therapist or piece of equipment to be part of
when prone. These three exercises have the potential of the client’s external environment for the client to succeed
training impaired strength, but only the first example forces at the task. For example, in BWSTT a harness is used to take
the training within a functional pattern. Similarly, the thera- away the demand of gravity on the limbs during gait and
pist could train the sit-to-stand pattern using various seat the demand of the postural trunk and hip muscles for stabil-
heights that encompass many of the components that force ity. Before the therapist or the patient can consider the
the use of normal movement synergies and postural control, movement as independent, those aspects must be removed
using the environment in which that activity is typically from the environment. In the previous example, the indi-
performed, versus performance of strengthening exercises vidual needs to transition from maximal body weight sup-
against resistance in an open chain exercise program. port during ambulation to not needing any external support
The decision to treat the impairments causing the activity during ambulation. The client must assume total ownership
limitations or to correct the functional problems themselves of the functional responses. Then and only then has indepen-
is influenced by myriad factors. It would appear that dence been achieved. At that time, functional retraining can
for certain tasks to be completed the client must possess be used with the intent of enlarging the environmental
the “threshold amount” of basic movement components parameters to allow for maximal independence. Figure 9-1
required for the task. Task specificity within this limited illustrates this concept of functional versus contrived
Contrived
1. Therapist guides activity
2. Therapist uses extrinsic

feedback
a. Altering hypertonicity
b. Increases or alters
hypoactivity
c. Manual therapy
d. Positioning out of synergy
e. Use of theraband, weight
belts, resistance, tapping
f. Modification of visual
auditory, tactile environment
g. Body weight support
treadmill training
h. Constraint induced
movement therapy
Figure 9-1 n Contrived versus functional therapeutics. (Modified from the original work of Jan Davis, OTR, San Jose State University.)
intervention, which must be constantly considered through- must determine why. Often, it is because the therapist did
out any treatment session. Augmented techniques are often not identify the correct body system problems. Many correct
the early choices for treatment of patients who have neuro- solutions may answer the question. Which solution is best
logical insults. It cannot be emphasized enough that once the may be more client than approach dependent. Yet if flexibil-
client has the ability to perform without augmented methods ity means that the therapist selects any component of any
and does so in functional, efficient ways, those augmented method that helps the client reach an objective, then the
techniques need to be selectively eliminated. therapist is confronted with hundreds—if not thousands—of
Once a clinician has chosen to augment the clinical envi- various treatment choices. If the treatment procedures used
ronment, the client needs to learn efficient motor behaviors introduce information to the client through sensory systems,
within the limitations of that environment. The client influ- then from a neurological perspective a limited number of
ences the therapist’s decision-making strategies by selecting input systems or modalities are available. The myriad treat-
inefficient or ineffective motor responses to a given task ment procedures are transformed into neurochemical and
demand. If the response is effortless, efficient, and noninju- electrophysiological responses that must travel along a lim-
rious to any part of the body and meets the client’s expecta- ited number of pathways in the nervous system. Thus, many
tions and goals, then the therapist knows the strategies different treatment procedures may produce similar types of
selected were effective even if the therapist augmented the neurotransmission. The temporal and spatial sequencing or
intervention. If the movement itself is available to the client, timing of the input will vary according to the technique and
then there is a high probability that the client will be able to the specific application. The clinician has little basis for
regain that movement control, regardless of the need for decision making without a comprehensive understanding
early augmentation to achieve the skill. If the response does of the neurophysiological mechanisms of (1) the various
not meet the desired goal for any reason, then the therapist techniques introduced to modify input, (2) where that
information will be processed and how that might affect to the environment to achieve functional motor output
motor output, (3) prior learning and the ability for new toward a goal. Both external and internal feedback are criti-
learning, and (4) the client’s willingness and motivation to cal for adaptation and change. External feedback in this
adapt. The reader is referred to Chapter 1 (Figure 1-1); chapter is considered a mechanism to help the client’s CNS
Chapter 4 on motor control, motor learning, and neuroplas- optimally learn and adapt. Obviously, as the patient learns,
ticity; and Chapter 5 for a discussion on motivation. internal feedback will allow the person to run feed-forward
The number of available contrived or augmented feed- motor programs without the need for external feedback for
back techniques is almost infinite. This section presents an control. External feedback will, it is hoped, be used only
overview of a classification system that can be used to help when the outside surrounding needs the feed-forward pro-
the reader develop a greater understanding of why certain gram to change to adapt to a new environment (refer to the
responses occur and why the selection of certain techniques Chapter 4 section on motor learning). Therapists must real-
is appropriate and should positively affect the desired motor ize that even if the primary goal may be to facilitate or
responses. This section focuses on intervention strategies dampen a motor system response, diverging pathways may
that have been accepted, have been used within the tradi- also connect with endocrine, immune, and autonomic
tional Western health care model, and are efficacious. Some systems. According to motor control theory, the clinical
alternative approaches to intervention that are not necessar- picture is a consensus of all interacting body systems (see
ily classified as traditional within this chapter are introduced Chapter 4). Research tools are not yet available to measure
in Chapter 39. There are other classification systems a clini- those systems interacting simultaneously, although func-
cian might use when analyzing movement problems seen in tional magnetic resonance imaging (fMRI) studies are begin-
patients with neurological dysfunction. For example, a ning to help researchers and clinicians identify what happens
therapist may see in a patient a problem primarily with tone, to the nervous system with input from the environment and
such as hypertonicity, hypotonicity, rigidity, dystonia, flac- how that information is processed. Efficacy using reliable
cidity, intentional and nonintentional tremors, ataxia, and and valid measurement tools must then be based on out-
combinations of or fluctuations in the total movement strate- comes, with an understanding of the best available scientific
gies. Given this specific classification schema, one still uses knowledge as a rationale for why the outcome is present.
the available treatment strategies or uses an input modality This classification system is based on identified input,
that may modify the specific tone problem that was causing observed responses, current research on the function of the
the movement dysfunction. CNS, and the various systems involved in the control and
The primary goal of this section is to help the reader modification of responses. An understanding of normal pro-
develop a classification system based on the primary input cessing of input and its effect on the motor systems helps the
modality used when introducing an augmented treatment clinician evaluate and use the intact systems as part of treat-
technique to facilitate a sensory system and provide feedback ment. Research with fMRI is now allowing greater insight
to the CNS in order to help a client learn or relearn motor into specific brain regions that are being used during various
control. The reader has been provided with an in-depth refer- cognitive and motor activities.113-128 Yet the specific interac-
ence to the specific neurophysiological approaches in the tive nature of multisensory input, memory, motivation, and
past also discussed in Chapter 1, and only a brief overview motor function is still unknown. When the response to cer-
has been included within this chapter. In-depth discussion of tain stimuli does not help the client select or adapt a desired
some basic treatment strategies, explanations of less familiar motor response, then the classification schema for aug-
techniques, and current approaches gaining popularity within mented input provides the clinician with flexibility to select
the clinical area of movement analysis are found within the additional options. This can be done by spatially summating
body of this section. input, such as using stretch, vibration, and resistance simul-
When the primary input system for a technique is identi- taneously, or temporally summating input, such as increas-
fied, at no time do we suggest that it is the only input system ing the rate of the quick stretch or increasing the time
affected. For example, when a proprioceptive technique is between inputs to give the system ample time to respond.
introduced, tactile cutaneous receptors are also simultane- Many factors can influence motor behavior, such as the
ously firing. If there is a “noise” component (such as with methods of instruction, the resting condition of the nervous
vibration or tapping with the fingers), then auditory input system, synaptic connections, cerebellar or basal ganglia or
has been triggered as well. There is evidence that a given cortical processing, retrieval from past learning, motor
sensory modality may “cross over” or fuse with a com- output systems, or internal influences and neuroendocrine
pletely different modality, helping in the synthesis of motor balance. Figure 9-2 illustrates and simplifies this total sys-
responses. In addition, there is evidence that the principles tem. Its clinical implications become clearer if the therapist
of neuroplasticity are applicable across modalities (e.g., retains a visual image of the client’s total nervous system,
auditory, visual, vestibular, somatosensory). Sometimes including afferent input, intersystem processing, efferent
responses occur in a modality that does not appear to be response, and the multiple interactions on one another. At
related. For example, olfaction may improve tactile sensitiv- any moment in time, multiple stimuli are admitted into a
ity of the hand. This concept is called cross-modal training client’s input system. Before that information reaches a level
or stimulation.111,112 Yet a classification schema based on a of primary processing, it will cross at least one if not many
primary modality promotes logical problem solving because synaptic junctions. At that time the information may be
the therapist can select from available treatment procedures inhibited, excited, changed or distorted, or allowed to con-
that theoretically provide similar information to the CNS tinue without modification. If the information is at the first
and help in the organization of appropriate motor responses. synapse, the patient will have no sensation. If it is inhibited
The motor system and its various motor programmers adapt at the thalamus, again the patient will not perceive sensation,
Figure 9-2 n Model of possible interactive effects among methods of treatment, input systems, processing and output systems, internal
influences, and feedback systems.
but that does not mean other areas of the brain will not be normal processing somewhere in the CNS or an insufficient
sent that information, because sensory information is also amount of input was used. One way to differentiate motor
sent to a variety of areas after that initial synapse. Research problems from problems with other systems is to use other
studies have found that sensory input information may even functional activities that have programs similar to the body
affect gait and other movement patterns even if the patient system program identified as impaired. If a program, such as
has no perception of the input.129,130 If the input is changed, posture, demonstrates deficiencies in one functional pattern,
then the processing of the input will vary from the one nor- then the therapist must determine if it is also deficient in
mally anticipated. The end product after multiple system other patterns. If the postural motor problem affects all
interactions will be close to, will be farther away from, or motor performance, then the therapist had determined that a
will seem to have no effect on the desired motor pattern. motor program deficit exists and will have to determine how
Furthermore, sensory processing can take place at many to correct that problem. If, on the other hand, the program
segments of the nervous system. Although the CNS is not runs smoothly and effortlessly when certain demands are
hierarchical, with one level in total control over another, taken away, such as resistance from gravity, position in
certain systems are biased to affect various motor responses. space, need for quick responses, and so forth, then it may be
At the spinal level the response may be phasic and synergis- that the problem is within another subsystem such as cogni-
tic. Brain stem mechanisms may evoke flexor or extensor tion, perception, the biomechanical system, or the cardio-
biases, depending on various motor systems and their modu- pulmonary system or is a power-production problem that
lation. Cerebellar, basal ganglia, thalamic, and cortical can be corrected by slowly increasing the demand on the
responses may be more adaptive and purposeful.130-133 Thus postural system through repetitive practice using various
the therapist must try to discern where the input or the additional input interventions. Differentially screening motor
feedback is being affective or short circuited. impairments as pure CNS motor problems (muscle recruit-
Remembering input as a possible option for intervention ment, firing rate, balance) versus problems with another
will always allow the therapist to differentiate the same five system (perception of vertical) becomes critical in a man-
alternatives—no response, facilitating (heightening), inhib- aged-care system that funds only a certain number of treat-
iting (dampening), distorting, or normal processing. These ment sessions. Internal influences also need to be considered
alternatives can occur anywhere in the system at synaptic because they affect each aspect of the system. Once normal
junctions. Finally, motor output is programmed and a re- processing has been identified, understanding of deficit sys-
sponse is observed. If the response is considered normal, the tems and potential problems can be analyzed more easily.
clinician assumes that the system is intact with regard to the To reiterate, this requires awareness of the totality of the
use and processing of the inputs. If the response is distorted individual—that is, the client’s personal preference of stim-
or absent, little is known other than there is a lack of the uli and the uniqueness of processing and internal influences.
A systems model requires simultaneous processing of mul- the clinician a choice of various procedures and promotes a
tiple areas, with interactions being relayed in all directions. learning environment that is flexible, changing, and interest-
A client’s CNS and peripheral nervous system (PNS) are ing. The therapist must, again, make the transition from
doing just that, and the therapist must develop a sensitivity applying contrived therapeutic procedures during functional
toward the client as a whole while interacting with specific tasks to allowing the client to practice the task without the
components (see Chapters 1, 4, 5, 6, and 39 for additional therapist interceding and without external feedback.140
information). With input from the client and family, it is the In that way the client uses inherent feedback to self-correct
therapist’s responsibility to select methods most efficacious feed-forward motor programming and then to continue
and effective for each client’s needs in relation to that running the appropriate movement strategies. This self-
person’s specific neurological problems. (See all clinical correction leads to independence, adaptability, and long-
chapters in Section II.) This viewpoint, based on a variety of term learning (see Figure 9-2).
questions, leads to a problem-oriented approach to interven- To avoid confusion about which peripheral sensory nerve
tion. Because the output or response pattern is based on fiber coming from the surface of the body or extremities is
alpha motor neuron discharge and thus extrafusal muscle being discussed, the two primary methods of classifications
contraction, the first question is posed: what can be done to (Gasser-Erlanger and Lloyd), along with a description of the
alter the state of the alpha motor neuronal pool or motor functional component, have been included in Table 9-1 for
generators? Second, what input systems are available, either easy referral. The other sensory systems will be presented
directly or indirectly, that will alter the state of the motor separately to help the reader establish an appropriate
pool? Third, which techniques use these various input sys- classification scheme. The primary sensory input systems
tems as their primary modes of entry into the CNS? Fourth, presented include proprioception, exteroception, vestibular,
what internal mechanisms need modification or adaptation vision, auditory, taste, and smell. These sensory inputs have
to produce a desired behavior response from the client? the potential to influence CNS structures including the
Fifth, which input systems are available to alter the internal thalamus, sensory and motor cortices, the cerebellum, the
mechanism and what outcomes are expected? Sixth, what reticular formation, and the basal ganglia and thus to affect
combination of input stimuli will provide the best internal the descending fibers under their control.
homeostatic environment for the client to learn and rehearse
a more optimal response pattern? For example, assume that Proprioceptive System Integration of Stretch,
a client with a residual hemiplegia resulting from an anterior Joint, and Tendon Receptors
cerebral artery problem has a hypertonic lower extremity Proprioception as an input system has a direct effect on pro-
that produces the pattern of extension, adduction, internal gram generators at the spinal level.141 Because of its impor-
rotation of the hip, extension of the knee, and plantarflexion tance in motor learning and motor adaptation to new or
inversion of the foot. The answers to the first two questions changing environments, however, proprioception also has
are based on the knowledge that the proprioceptive and significant connections to the cortical and cerebellar neural
exteroceptive systems can drastically affect spinal central networks. Its divergent pathways have synapses within the
pattern generators and that these input systems are intact at brain stem, diencephalon, and spinal system. Proprioceptive
spinal, brain stem, cerebellum, and thalamic levels and may input can potentially influence multiple levels of CNS func-
even project to the cortex. tion, and all those levels can potentially modulate the
Appropriate selection of specific techniques—such as intensity or importance of that information through many
prolonged stretch using the tendon organ to modulate the different mechanisms.141,142 Proprioceptors are found in
hypertonic pattern, quick stretch or light touch to the three peripheral anatomical locations: the stretch receptors,
antagonistic muscle, or any other treatment modality within the tendon, and the joint. The afferent receptors responsible
the classification schema—will provide viable treatment for relaying sensory information through those sites are
alternatives. Awareness that a client’s response pattern is an discussed in the following subsections.
inherent synergistic pattern and that it is further elicited by Muscle Stretch Receptors
pressure to the ball of the foot leads to a better understand- Stretch. Stretch, quick stretch, and maintained stretch
ing of the clinical problem. Knowing that the client is unable are all sensory input systems that use the stretch receptors
to combine the alternative patterns, such as hip flexion with in the muscles and heighten the motor pool.143-145 Stretch
knee extension needed for the late stage of swing phase simultaneously heightens both the muscle response to that
through the early aspects of stance phase during gait, the stretch and potentially heightens the sensitivity of the ago-
therapist can use the other inherent processes to elicit these nistic synergy. It will also lower the excitation of the
and other patterns. BWSTT is an example of an augmented antagonistic muscle and those muscles that are part of the
treatment intervention in which the clinician assists the antagonistic synergy. Stretch information will be sent to
patient to place the leg and foot with each step while the higher centers for sensory integration and perception. The
apparatus controls balance and posture to provide an experi- cerebellum uses this incoming feedback to maintain and/or
ence of normal gait while requiring the patient to have only regulate motor nuclei in the brain stem that will influence
the strength to manage partial body weight.134-139 Finally, the state of the alpha and gamma motor neurons. This allows
techniques such as combining standing and walking with the for cerebellar feed-forward regulation (refer to Chapter 21).
application of quick stretch, vibration, or rotation, or having There are many ways to apply stretch to the muscles. The
the client reach for a target or follow a visual stimulus while therapist can use (1) the hands and their respective muscle
walking, provide a variety of combinations of therapeutic power to apply a stretch, (2) a manual weight system of
procedures to help the client learn or relearn normal some sort that maintains the stretch through the range, (3) a
response patterns. Furthermore, combining techniques gives suspension system such as used in Pilates exercises (see
TABLE 9-1 n CLASSIFICATIONS OF PERIPHERAL NERVES ACCORDING TO SIZE
MOTOR (FUNCTIONAL
GASSER-ERLANGER LLOYD COMPONENT) SENSORY (FUNCTIONAL COMPONENT)
A fibers: large myelinated fibers with a high conduction rate
A alpha Ia Large, fast fibers of alpha motor Muscle spindle; primary afferent endings (primary
system (large cells of anterior stretch or low threshold stretch; Ia tonic fibers
horn to extrafusal motor fibers) respond to length, Ia phasic fibers respond to
rate)
Ib Tendon organ for contraction; respond to tendon
stretch or tension
A beta II Muscle spindle; secondary afferent endings; tonic
receptors responding to length
Exteroceptive afferent endings from skin and
joints; respond to light or low threshold stretch
A gamma 1 and 2 II Gamma motor system (small Bare nerve endings; joint receptors, mechanoreception
cells of anterior horn to of soft tissues; exteroceptors for pain, touch, and
intrafusal motor fibers) cold (low threshold)
A delta III
B fibers: medium-sized myelinated fibers with a fairly rapid conduction rate
B beta Preganglionic fibers of
autonomic system (effective
on glands and smooth muscle;
motor branch of alpha):
unknown function
C fibers: small, poorly myelinated or unmyelinated fibers having slowest conduction rate; augmentation and recruiting occur within the
nervous system after stimulation of these fibers has ceased
IV Postganglionic fibers of Exteroceptors; pain, temperature, touch
sympathetic system
Chapter 39), (4) the patient’s own body weight against grav- flexors, benefit from isometric exercise, as well as isotonic
ity, (5) a complex robotic system that computerizes the exercise in both eccentric and concentric modes. Under
amount of stretch depending on the individual’s specific data normal circumstances, the flexors are used for repetitive or
(see Chapter 38), or many other creative ways to apply rhythmical activities. The extensors, on the other hand, usu-
stretch to muscle fibers within the belly of the muscle tissue. ally remain contracted in an effort to act against the forces
As stated previously, stretch can also be applied to the of gravity. Therefore the extensor groups benefit best from
antagonist muscle or muscle synergy in order to dampen ago- isometric and eccentric resistance.146
nist function. Thus stretch can be used to enhance tone in the When resistance is applied to a voluntary muscle, spindle
agonist or to decrease tone of the agonist through the antago- afferent fibers and tendon organs fire in proportion to the
nist. The therapist should always remember that even though magnitude of the resistance. Resistance is more facilitative
a response may not look obvious, as long as the peripheral to an isometrically contracted muscle than in an isotonic
nerves and motor neurons within the spinal system are intact, contraction.35 As isometric resistance is increased or contin-
these approaches will change the state of the motor pool. ued, more motor units are recruited, thereby increasing the
Table 9-2 lists a variety of treatment procedures believed strength of extrafusal contraction.26 Eccentric isotonic con-
to use proprioceptive input from the muscles as a primary traction refers to the lengthening of muscle fibers with resis-
mode of sensory stimulation. The varying intensity, amount tance added to the distal segment, as in lowering the arms
of tension, or rate of the stimuli, in addition to the original while holding a heavy weight. Eccentric contraction uses
length of the muscle before application of the stimulus, will less metabolic output and promotes strength gains in less
determine its firing. Remember, afferent information is time.26 However, all types of muscle contraction will pro-
projecting to many areas above the spinal system, and the mote increased strength. Resistance is an important clinical
result will be regulation or modulation, ultimately affecting treatment and has been used and will continue to be used
activity.141 by clinicians within multiple treatment philosophies over
Resistance and Strengthening. Resistance is often used the next millennium.8,19,25,29,77,147-153 The complexity of
to facilitate intrafusal and extrafusal muscle contraction. neural adaptation after resistive exercises may lead to a dif-
Resistance can be applied manually, mechanically, and by ferent training environment depending on age, athletic
the use of gravity. Resistance recruits more motor units in status, and specific body system deficits.154 Combining
the target muscles. Although muscles can contract both in an resistive training with guided imagery or other types of
isometric and an isotonic fashion, most contractions consist adjunct interactions has conflicting results.154-156 Yet there
of a mixture of the two. Certain muscle groups, such as the are still questions regarding optimal resistive training and
TABLE 9-2 n PROPRIOCEPTIVE STRETCH RECEPTORS
RECEPTOR STIMULUS NATURE OF RESPONSE

Ia tonic Length Monosynaptic and polysynaptic facilitation of agonist
Ia phasic Rate of change in length Polysynaptic inhibition of antagonist and antagonistic synergy
Polysynaptic facilitation of agonistic synergy
Input to cerebellum
Input to opposite parietal lobe
Specific parietal lobe responses open for question
II Length Monosynaptic facilitation of agonist
Polysynaptic facilitation of specific muscle groups, depending on muscle
function of tissue where II fibers originate
Transmittal of information to higher centers
POSSIBLE TREATMENT ALTERNATIVES
1. Resistance
2. Quick stretch to agonist
3. Tapping: tendon and/or muscle belly
4. Reverse tapping: gravity stretches; tapping agonist into shortened range
5. Positioning (range)
6. Electrical stimulation
7. Pressure or sustained stretch
8. Stretch pressure
9. Stretch release
10. Vibration: facilitatory frequency for small vibrator, relaxation for total body vibration
11. Gravity as a prolonged stretch
12. Active motion
whether one resistive technique is better than another.157,158 obtain a shortened range. An example of reverse tapping
Research certainly has shown that resistance training does would be tapping the triceps muscle when the client is bear-
enhance functional abilities across age groups,150,159,160 but ing weight on the extended elbow and actively trying to
again the specifics regarding resistive training techniques achieve full elbow extension. Gravity quickly stretches the
are often not identified. The terms resistive training, weight triceps. Timing of this technique is important. If the thera-
training, and strength training are often used synonymously, pist taps the elbow toward extension when the flexors’ motor
and thus specifics are yet to be identified in the research. neurons are sensitive, then those flexor muscles may
How all these uses of resistive exercises will play out in the respond to the stretch and contract, taking the arm farther
future is up to future researchers in the field of movement into flexion. If the timing follows the quick stretch to the
science. Very costly high-technology tools have been added extensor, then the flexors will be dampened and active
to aid in resistive training (see the discussion of Pilates in extension more likely a motor response.
Chapter 39 and robotics in Chapter 38).161,162 Given the Positioning (Range). The concept of submaximal and
needs of individuals after neurological insults, cost becomes maximal range of muscles is highly significant to clinical
a major factor, and finding creative and cost-efficient ways application. Bessou and colleagues164 monitored the neuronal
to apply resistance may become a common research ques- firing of muscle spindles at different ranges of motion. Upper
tion in the future. motor neuron lesions can alter the sensitivity of the spindle
Tapping. Three types of tapping techniques are com- afferent reflex arc fibers by not using presynaptic inhibition to
monly used by therapists. Tapping of the tendon is a fairly normally dampen incoming afferent activity.165 Therefore
nondiscriminatory stimulus. Physicians use this technique ROM should be carefully assessed on an individual basis,
to determine the degree of stretch sensitivity of a muscle. particularly in a patient with an upper motor neuron lesion, to
A normal response would be a brisk muscle contraction. determine the maximal or submaximal range for an individ-
Because of the magnitude of the stimulus and the direct ef- ual. Therapists always need to determine whether the differ-
fect on the alpha motor neuron, this technique is not highly ence between optimal range and functional ROM is different.
effective in teaching a client to control or grade muscle con- If a patient will never need to use full ROM, then spending
traction.163 Instead, tapping of the muscle belly, a lower- long periods of time trying to stretch a shoulder or hip may
intensity stimulus, is more satisfactory. Reverse tapping is a not be the best decision with regard to intervention. As well
less frequently described technique, but it can be used. The as the ROM itself, therapists need to carefully evaluate exces-
extremity is positioned so gravity promotes the stretch, sive range resulting from hypermobility and hypotonicity. In
instead of the therapist manually tapping or actively induc- those situations, external support of the affected joint or limb
ing muscle stretch. Once the muscle responds, the therapist needs to be considered in all functional positions in order to
taps or passively moves the extremity to help the muscle prevent complications such as pain.166-168
Electrical Stimulation. For an in-depth discussion of the responses within specific muscle function has been used to
use of electrical stimulation both as an evaluation and a treat- show how proprioception can be used to alter upright stand-
ment modality, see Chapter 16 and Chapter 33. Electrical ing.179,180 The second type of vibratory method is a total-
stimulation has the potential to be an excellent muscle spin- body vibration to facilitate postural tone and balance and is
dle facilitatory technique, especially if additional therapeutic applied through the feet in a standing position.181-184
tools, such as resistance, are included. Electrical stimulation Bishop185,186 wrote an excellent series of articles on the
delivered to create muscle contraction is beneficial, but elec- neurophysiology and therapeutic application of vibration
trical stimulation as a sensory stimulus is less effective as a in the 1970s. High-frequency vibration (100 to 300 Hz or
learning tool because there are no sensory receptors for elec- cycles per second) applied to the muscle or tendon elicits a
trical currents and thus they are not represented as a unique reflex response referred to as the tonic vibratory response.
stimulus on the somatosensory cortex. Functional electrical Tension within the muscle will increase slowly and progres-
stimulation (FES) is a technique that applies electrical stimu- sively for 30 to 60 seconds and then plateau for the duration
lation during functional movement. Chapter 16 discusses this of the stimulus.187 Some researchers found that at cessation
technique with traumatic spinal cord injury, but the applica- of the input the contractibility of the muscle was enhanced
tion has gone beyond those individuals diagnosed with spinal for approximately 3 minutes.187,188 The discrepancy in the
injury. Individuals poststroke have also been studied using research may reflect the way the individual is using the
FES. The results were inconsistent. Some studies showed input, both from a direct effect on the motor generator and
there was no difference in the stroke groups during or from supraspinal modulation over the importance of the
directly after intervention but that the long-term effect input, which may affect the overall learning and plasticity of
remained with those individuals who received FES, whereas the CNS. To facilitate hypotonic muscle, the muscle belly is
those who did not regressed in function.169,170 Studies have first put on stretch, and then vibratory stimuli are applied.189
shown that FES training increased walking ability and speed To inhibit a hypertonic muscle, the antagonistic muscle
during and after the training.171,172 Studies that have looked at could be vibrated.185,189 The use of vibration can be
other neurological problems have also used FES and cer- enhanced by combining it with additional modalities such as
tainly are showing that this type of intervention may become resistance, position, and visually directed movement. Vibra-
a standard of practice in the future.173-175 Combined modulation also stimulates cutaneous receptors, specifically the
tion of voluntary movement, proprioceptive sensory feed- Pacinian corpuscles, and thus can also be classified as an
back, and electrical stimulation might play an important role exteroceptive modality.190 Because of its ability to decrease
in improving impaired sensorimotor integration by power- hypersensitive tactile receptors through supraspinal regula-
assisted FES therapy.176 The use of FES over acupressure tion, local vibration is considered an inhibitory technique
points has been shown to significantly reduce pain.177 (it is also discussed later in the section on exteroceptor-
Stretch Pressure. The muscle belly is the stimulus focus maintained stimulus). Therapists have reported that vibra-
of stretch pressure. The therapist slowly applies pressure to tion over acupressure points can modulate localized pain
the muscle belly. It is used to decrease or release tone in the syndromes. It seems to trigger A delta exteroceptive fibers,
target muscle, allowing for the (temporary) recovery of vol- which in turn dampen the effect of C fibers. (See Chapter 32
untary movement.111,178 Generally this type of stimulus for more information on the treatment of pain.)
is applied and maintained for a period of time (e.g., 5 to Farber111 summarized the use of vibration and clearly
10 seconds). It is not a quick stimulus and may be using the identified precautions that must be taken. Frequencies
tendon organ to dampen tone. This type of pressure tech- greater than 200 Hz can be damaging to the skin. We have
nique is also used in a variety of complementary approaches found frequencies greater than 150 Hz to cause discomfort
(see Chapter 39). and even pain. Therefore it is recommended that vibrators
Stretch Release. This technique is performed by placing registering 100 to 125 Hz be used. Most battery-operated
the fingertips over the belly of larger muscles and spreading hand vibrators function at 50 to 90 Hz.11 Frequencies less
the fingers in an effort to stretch the skin and the underlying than 75 Hz are thought to have an inhibitory effect on nor-
muscle. The stretch is done firmly enough to temporarily mal muscle,187 although a study showed that some muscle
deform the soft tissue so the cutaneous receptors and Ia affer- groups, especially the lateral gastrocnemius, do respond
ent fibers may produce facilitation of the target muscle. It is positively to frequencies of 40 to 60 Hz.191 Another
easy to determine quickly whether the response is efficacious researcher192 studying vibration found similar results that
by just feeling and looking at the response of the patient. frequencies of 50 Hz generated more neuromuscular facili-
Manual Pressure. Manual pressure can be facilitatory tation than lower frequencies (30 Hz) when studying
when it is applied as a brisk stretch or friction-like massage improvements in upper body resistance exercise perfor-
over muscle bellies. The speed and duration at which the mance. Cutaneous pressure is also known to cause inhibi-
manual pressure is applied determine the extent of recruit- tion, so if it is combined with a vibration technique that is
ment from receptors. Paired with volitional efforts, manual being used to augment a muscle contraction, it can only
pressure can lead to motor function, and with repetition, serve to cancel the desired effects.
motor learning. Amplitude or amount of displacement must also be con-
Vibration. There are two types of vibratory methods sidered when vibration is analyzed as a modality. It has been
used therapeutically. The first deals with the use of a hand- reported that high amplitude causes adverse effects, espe-
held vibrator to facilitate Ia receptors to enhance agonistic cially in clients with cerebellar dysfunction.186 Vibration is
muscle contraction in hypotonic muscles or to facilitate not recommended for infants because the nervous system is
Ia receptors of antagonistic muscle fibers to inhibit hyper- not yet fully myelinated and the vibration might cause too
tonic agonists. Currently the use of vibration to facilitate Ia much stimulation. The reader is also cautioned about using
vibration over areas that have been immobilized because of with the stretch receptors to inform higher centers of con-
the underlying vascular tissue potential for clotting. Vibra- tinuing environmental demands to modulate or change exist-
tion on or near these blood vessels could dislodge a clot, ing plans; these higher centers in turn regulate tonicity and
causing an embolism. Vibration also needs to be used cau- the state of the motor pool.43,141 The tendon (Ib) signals not
tiously over skin that has lost its elasticity and is thin (e.g., only tension but also the rate of change of tension and pro-
that in older persons) because the friction itself from the vides the sensation of force as the muscle is working.198
vibration can cause tearing. The therapist must always keep A fundamental difference between the tendon organ and the
in mind the environment and the functionality of an inter- stretch receptors is that the stretch receptors detect length,
vention procedure. The use of vibration may assist the client whereas the tendon monitors tension and force. Sensory
in contractions and somatosensory awareness, but it is an input from the stretch receptors and the tendon are mostly
unnatural way to facilitate either system and thus needs to be opposites.43,202 The stretch receptors regulate reciprocal
removed as part of an intervention as soon as the patient dem- inhibition, whereas the tendon modulates autogenic inhibi-
onstrates some sensory awareness and/or volitional control tion. Table 9-3 lists a variety of known treatment approaches
over a movement component. that use the tendon to inform higher centers regarding
Within the last decade the use of vibration of specific needed change and regulation over spinal generators.
muscle groups of the neck has been studied in order to Maintained Stretch to the Tendon Organ. Maintained
determine its effect on upright standing and the interaction stretch to a muscle has the potential for triggering the tendon
with and without eyes open.179,180 These studies showed that organ if tension is great enough. Once the maintained stretch
by vibrating specific muscle groups, those muscles would fires the tendon organ, autogenic inhibition of the same
actively contract and change the position of the head in muscle occurs. A therapist will feel a release of the agonist
space but that with eyes open the effect was minimized in muscle, allowing for elongation of the contractile compo-
relation to global postural control. A similar study examined nents. Simultaneously, the tendon organ’s sensory neurons
the effect of vibration on various muscles within the will facilitate motor neurons to the antagonist muscle, thus
lower extremities and how that affected various postural heightening its sensitivity and potential for activity. This is
responses.191,193 These researchers found that different fre- the technique used when a joint has developed range restric-
quencies affected different muscle groups. The one consis- tion. The clinician always needs to differentiate whether the
tent thing all studies have shown is that vibration does tightness found within the joint is caused by compensatory
facilitate Ia muscle fibers, which in turn affect muscle con- muscles considered movers protecting injured postural
traction of the agonist receiving the vibration. Other sensory muscles beneath or by tightness just from positioning,
systems can assist or override the effect of vibration, but that disuse, or fear.
is because of superspinal influence over motor generators. Inhibitory Pressure. Pressure has been used therapeuti-
Total-body vibration is currently being used to determine if cally to alter motor responses. Mechanical pressure (force),
it affects motor performance. Studies have shown that whole- such as from cones, pads, or the orthokinetic cuff developed
body vibration can enhance motor performance in high-level by Blashy and Fuchs,204 provided continuously is inhibitory.
athletes performing sprints and jumps,181,182 as well as That pressure seems most effective on tendinous insertions.
improve trunk stability, muscle tone, and postural control in It is hypothesized that this deep, maintained pressure acti-
individuals after stroke while in geriatric rehabilitation.184 Its vates Pacinian corpuscles, which are rapidly adapting recep-
application for individuals with neurological dysfunction is tors. A variety of researchers have studied these receptors
inconclusive.194,195 Studies specifically directed toward the and their relationship to regulating vasomotor reflexes,205
elderly again show promise, but further research is needed modulating pain,206-210 and dampening other sensory system
for specificity.196,197 Future research will need to determine influence on the CNS.188,209
the effect of total-body vibration when introduced to all This inhibitory pressure technique also works when pres-
populations of individuals with neurological dysfunction. At sure is applied across the longitudinal axis of a tendon. The
that time both amplitude and magnitude will need to be iden- pressure is applied across the tendon with increasing pressure
tified in order to replicate studies. Total-body vibration cer- until the muscle relaxes. Constant pressure applied over the
tainly falls under primarily proprioception but also could be tendons of the wrist flexors may dampen flexor hypertonicity
classified under combined proprioceptive techniques or mul- and elongate the tight fascia over the tendinous insertion (see
tisensory classification techniques because the input affects Chapter 39 for additional information).
the muscle spindles, the joints, the vestibular system, and Pressure over bony prominences has modulatory effects.
possibly the auditory system with the low frequency noise. A common example is pressure on the medial aspect of the
And every time vibration is applied, the skin receptors will calcaneus, which dampens plantarflexors and allows con-
initially fire although most will adapt quickly to prolonged traction of the lateral dorsiflexor muscles. Pressure over the
use of any stimuli. lateral aspect of the calcaneus also dampens calf muscles to
The Tendon. The tendon receptors are specialized allow for contraction of the medial dorsiflexor muscles.25
receptors located in both the proximal and the distal muscu- Localized finger pressure applied bilaterally to acupuncture
lotendinous insertions. In conjunction with the stretch recep- points has been shown to relieve pain and reduce muscle
tors, the tendon plays an important role in the mediation of tone.210-214 This technique has also been found to be particu-
proprioception.141,142,198-203 larly effective when used in a low-stimulus environment and
The principal role of the tendon is to monitor muscle ten- when combined with deep breathing.
sion exerted by the contraction of the muscles or by tension This combination of pressure (manually applied), environ-
applied to the muscle itself. Research has demonstrated that mental demands (low), and parasympathetic activity (slow,
the tendon is highly sensitive to tension and acts conjointly relaxed breathing) illustrates various systems interacting
TABLE 9-3 n PROPRIOCEPTIVE RECEPTORS OF TENDONS AND JOINTS
RECEPTOR STIMULUS RESPONSE

TENDON
Tendon organ lb Tension on extrafusal muscle Polysynaptic inhibition of agonist,
facilitation of antagonist spinal level
circuitry; supraspinal regulation
Possible Treatment Suggestions
1. Extreme stretch
2. Deep pressure to tendon
3. Passive positioning in extreme lengthened range
4. Extreme resistance: more effective in lengthened and shortened range
5. Deep pressure to muscle belly to put stretch on tendon
6. Small repeated contractions with gravity eliminated
TYPE OF JOINT
I (6-9 m) Static and dynamic joint tension: Thought to facilitate postural holding and
muscle pull joint awareness
II (9-12 m) Dynamic: sudden change in joint tension Thought to facilitate agonist and
awareness of joint range of motion
III (13-17 m) Dynamic: linked to Golgi tendon organ Thought to inhibit agonist
traction; activates in extreme range
IV (5 m .2 m) Pain Thought to inhibit agonist
Possible Treatment Suggestions
1. Manual traction (distraction) to joint surfaces to facilitate joint motion
2. Manual approximation (compression) to joint surfaces to facilitate co-contraction or postural holding
3. Positioning: gravity used to approximate or apply traction
4. Weight belts, shoulder harnesses, and helmets to increase approximation
5. Wrist and ankle cuffs to increase traction
6. Wall pulleys, weights, manual resistance
7. Manual therapy20
8. Elastic tubing to provide compression during movement
together to create the best motor response. The real world can facilitate the postural coactivation needed during stand-
requires the client to respond to many environmental condi- ing or walking.216-218 At times, approximation can be used to
tions while relaxed or under stress. Thus, once a client heighten normal postural tone while simultaneously dampen-
begins to demonstrate normal adaptable motor responses, ing excessive tone in the other leg. For example, clients who
the therapist needs to change the conditions and the stress have CNS insult often have an imbalance in function within
level to allow the client to practice variability. That practice the two lower extremities. This can be very frustrating for the
should incorporate motor error, especially error or distortions therapist because bringing the patient to standing to assist in
in the plan, yet still achieve the desired goal. As the regaining normal postural extension of one leg triggers the
client self-corrects, greater demand and variability should be other into a strong extensor pattern, causing plantarflexion
introduced.215 and inversion of that foot. One way to use approximation in
Joint Receptor Approximation. Approximation of treating both legs simultaneously might be to first bring the
the joint mimics weight bearing and facilitates the postural patient from sitting onto a high-low mat. Then the therapist
extensor system. Gravity creates approximation and its great- can raise the mat high enough that the patient can be lowered
est force is produced down through the body in vertical into standing on the normal-functioning leg. At the same
postures. Approximation should help to stabilize any joint time the patient’s other leg can be bent at the knee, and that
that is in a load-bearing situation by eliciting coactivation of knee placed on a stool or chair. This allows approximation
the muscles around the joint in question. In standing, gravity down through the entire leg that is in standing position while
creates approximation down through the entire spine, hips, approximating the trunk, hip, and knee of the other leg in the
knees, and ankles. When in a prone position on elbows, the kneeling position. The therapist can work on standing and
load goes down again through the upper spine while simulta- weight shifting in one leg while dampening abnormal tone in
neous going down through the shoulder girdles of both arms. the kneeling leg. As the kneeling leg starts to regain postural
If a therapist increases that load by adding pressure down coactivation in its hip, postural function will often be felt in
through the joints in question, then an augmented interven- the knee and ankle.
tion has been added to the therapeutic environment. Using One very effective way to apply approximation and resis-
weight belts around the waist or a weighted vest on the trunk tance simultaneously is to use the product similar to a cut
large elastic rubber band: Thera-Bands. The rubber material technique should be incorporated into functional training to
is attached under the heel on the right and left side; both achieve better sensorimotor responses, improved cortical
ends of the band are brought up across the ankle and then representation of the involved body part, and greater
crossed over the lower leg, once more over the back of the functional carryover.
thigh, and then anchored onto a belt around the patient’s Ballistic Movement. Ballistic movements are effective
waist. A similar pattern can be used for the arm; the band is because of their combined proprioceptive interaction. The
first placed across the palm and then crossed in the forearm client is asked to initiate a movement, such as shoulder flex-
and then the arm. Finally one end is brought across the ion while prone over a table with the arm hanging over the
upper chest and the other comes around from the back of the side. This component is volitional, but the client then main-
arm. Then the two ends of the band are tied together across tains a passive role. As the patient relaxes, the movement
the neck.These techniques can be graded by the elasticity of patterns become automatic. The physiology behind the auto-
the material.219-221 matic movement is easy to understand. As the muscle
Traction and Distraction. One or more joints are dis- approaches the shortened range, the amount of ongoing
tracted by a force that causes it or them to separate or pull gamma afferent activity decreases. Thus both the agonist
apart, similar to the swing phase of the leg during ambula- alpha motor neuron bias and the inhibition of Ia and II
tion or the arms in a reciprocal pattern to each leg. This receptors of the antagonistic alpha motor neurons decrease.
distraction of the joint receptors also puts stretch on the Simultaneously, the antagonistic muscle is being placed on
muscles, which combines to facilitate the pattern into which more and more stretch. This stretch, as well as the lack of
the limb is moving. Simultaneously, distraction dampens the inhibition on the antagonistic alpha motor neurons, will
antagonistic movement pattern, which allows the agonist encourage the antagonistic muscle to begin contraction and
movement to continue. A therapist will often use manual reverse the movement pattern. The tendon organs also play
traction to get relaxation of hyperactive extensor muscles or a key role in ongoing inhibition. As the muscle approaches
for limited mobility.222 Often therapists do not think of the the shortened range and tension on the tendon becomes
traction when applying resistance to a limb. For example, a intense, the tendon organ increases its firing, thus inhibiting
mistake made is placing ankle weights to facilitate limbs the agonistic muscle in the shortened range while facilitat-
that are ataxic. Ataxia is an imbalance in coactivation and ing the antagonistic muscle. This technique is highly move-
smooth movement of both agonist and antagonist muscle ment oriented, and the traction applied by gravity to the
groups.223 The weight itself slows down the excessive move- shoulder joint while swinging the arm further facilitates the
ment by the resistance. However, weight on the ankle cre- movement. These ballistic movements are part of the pro-
ates traction that will facilitate only the flexor group and gram generators within the spinal system that facilitate
often creates an additional imbalance in the ataxic leg.224 reciprocal movements of the limb. As the client performs the
When the weights are removed, the patient often is more movement, there is little need for conscious attention to
ataxic. drive the movement; it will run automatically. The role of
Combined Proprioceptive Input Techniques. Many the Ib fibers during this open chain or movement pattern is
techniques succeed because of the combined effects of mul- definitely different from its role in a closed chain or weight-
tiple inputs. Some of these combined techniques include bearing environment.199 Supraspinal influence over pro-
jamming; ballistic movements; total-body positioning; PNF grammed activity also plays a role in the effectiveness of
patterns; postexcitatory inhibition (PEI) with stretch, range, this treatment.229 The specific rationale for why ballistic
rotation, and shaking; heavy work patterns; Feldenkrais (see movements have functional carryover may be explained by
Chapter 39)225-227; and manual therapy.20,208,228 recent research into cerebellar function and the importance
Jamming. Jamming is usually applied to the ankle and of mechanical afferent input in regulation of movement (see
knee with the intent of dampening plantarflexion while Chapter 21).
facilitating postural co-contraction around the ankle. The The clinician using this technique must exercise caution.
client can be placed in a side-lying position, can sit on a ROM can easily be obtained through ballistic movement.
chair or mat, or can be positioned over a bolster with the hip Consequently, the clinician must always determine before
and knee in some degree of flexion. This flexion dampens therapy the reasons for specific clinical signs and whether
the total extension pattern, including the plantarflexor mus- the total problem will be corrected through an activity such
cles. With release of plantarflexion these muscles are placed as a ballistic movement. This is the diagnostic responsibility
on extreme stretch to maintain the modulation. In this posi- of the professional. If one component of the problem is
tion, intermittent joint approximation and compression of alleviated, such as limitation of range, while other compo-
considerable force is applied between the heel and knee. If nents are ignored, this can be a dangerous technique. If the
the client is sitting, this approximation can easily be applied lack of range is a result of muscle splinting because there is
by pounding the heel on the floor and controlling a counter- lack of postural tone or joint stability, then ballistic move-
force at the knee. Once coactivation is minimally palpated, ment has the possibly increasing the problem. For example,
the clinician should initiate a movement pattern such assume that the rotator cuff muscles are slightly torn and the
as partial weight bearing to further encourage the CNS to movers of the shoulder are superficially splinting to prevent
readapt with postural control. This technique can also be further tearing. Instructing the client to perform ballistic move-
used to dampen flexion of the wrist and fingers by applying ment that causes relaxation of more superficial muscles will
force to the appropriate upper-extremity patterns, modulat- then place more responsibility for shoulder stabilization on
ing flexor reflex afferent activity, and applying a large the rotator cuff muscles. If those stabilizers are torn, traction
amount of joint approximation between the heel of the along with relaxation of muscles that are splinting may in-
hand and the elbow. To augment functional outcomes, the crease the tear on the rotator cuff muscles and thus increase
the problem. The patient may never return to therapy, but if At a certain point in the range, if the muscle is not limited
he does, he will complain of more pain than before. by fascial tightness, the hypertonic muscle will become
Total-Body Positioning. Total-body positioning implies dampened and tone will disappear. It is thought that at this
the use of positioning and gravity to dampen afferent activ- time either the tendon organ activity takes over and main-
ity on the alpha motor neurons and thus cause a decrease in tains inhibition or flexor reflex afferents are modified, thus
tone, or relaxation.230 Today, the rationale for why relaxation creating an inhibitory range in which antagonistic muscles
of striated muscle occurs after this treatment implies that the can be more easily initiated and controlled by the client.
effect of the flexor reflex afferents is being dampened by a If this technique is performed in a pure plane of motion,
combination of input and interneuronal activity. These the clinician will find it a time-consuming procedure. Range
changes in the state of the muscle tone will not be perma- can be achieved quickly by integrating a few additional
nent and will revert to the original posturing unless motor techniques, that is, incorporating rotational patterns of
learning and adaptation within the central programmer movement. For example, if the spastic upper extremity is
occur simultaneously. Thus for this treatment to effect per- positioned in the pattern of shoulder adduction, internal
manent change, a large number of systems need modifica- rotation, elbow flexion, forearm pronation, and wrist and
tion. This modification can be augmented by techniques that finger flexion, then a pattern in the opposite direction can be
facilitate autogenic inhibition, reciprocal innervation, laby- incorporated to include external rotation of the shoulder and
rinthine and somatosensory influences, and cerebellar regu- supination of the forearm. Every time the clinician begins to
lation over tone.231 Changing the degree of flexion of the lengthen the spastic extremity, those rotational patterns
head also alters vestibular input and the state of the motor should be used. This should be done both on initial stretch
pool. But again, the CNS of the client needs to be an active and when resisting movement during excitation and then
participant and will ultimately determine whether perma- lengthening (allowing movement) during the inhibitory
nent learning and change are programmed. phase. Rotation seems to lengthen the inhibitory phase and
Proprioceptive Neuromuscular Facilitation. To analyze allows additional range. If the clinician adds a quick stretch
and learn the principles, techniques, and patterns that consti- to the antagonistic muscle during the inhibitory phase of the
tute PNF, a total approach to treatment, refer to the texts agonistic muscle, then further facilitation of the antagonistic
by Adler,232 Voss,233 and Sullivan and colleagues.29 This muscle will occur. Because the agonistic muscle is in an
approach is being used extensively for patients with muscu- inhibitory phase, movement in and out of its spastic range
loskeletal and neuromuscular problems, with research on this should not affect it. Yet the quick stretch facilitation of the
method encompassing more populations with lower motor antagonistic muscle inhibits the spastic agonistic muscle and
neuron and musculoskeletal problems than upper motor neu- again lengthens the inhibitory phase. This entire procedure
ron lesions.154,228,234-242 When proprioceptive techniques are occurs very quickly. An observer might say that the clinician
packaged in specific movement patterns, it may be referred “shakes the hypertonicity out of the arm.” The shaking
to as PNF. When individual proprioceptive techniques are action is thought to be the quick stretch as well as joint oscil-
discussed alone, the specific sensory function is being ac- lations. The degree of success depends on the therapist’s
knowledged, and these techniques can be integrated into sensitivity to the tonal shifts or phase changes occurring in
many rehabilitation intervention strategies. the client. These tonal shifts are automatic at the hundredth-
Postexcitatory Inhibition with Stretch, Range, Rotation, of-a-millisecond level and not under the client’s conscious
and Shaking. The concept of PEI is based on the action control. But the sensitivity of the Meissner corpuscles are at
potential or electrical response pattern of a neuron at the time approximately 2 hundredths of a millisecond and provide
of stimulation and on the entire phase response until the neu- adequate input to the therapist. If a master clinician responds
ron returns to normal. At the time of stimulation, the action to each inhibitory phase, it will look like the tone melts
potential will build and go through an excitatory phase. The away. Most clinicians do not have that keen sensitivity, and
neuron then enters an inhibitory phase or refractory period the interventions will look more jerky because not every
during which further stimulation is not possible. This is inhibitory phase is sensed and thus there will be a lot of
referred to as the PEI phase or postsynaptic afferent depolar- stop-and-go movement in very small ranges of movement
ization.111 These phase changes are extremely short and, in out of synergy until the hypertonic muscles finally relax.
normal muscle, asynchronous with respect to multiple neuro- Rood’s Heavy Work Patterns. Rood’s concepts of co-
nal firing. In a hypertonic muscle more simultaneous firing contraction in weight-bearing positions such as on elbows, on
occurs. When the muscle is lengthened, and thus tension is extended elbows, kneeling, and standing blend with today’s
created, more fibers will be discharged. It is hypothesized that concepts of motor learning. Concepts explain why postural
if the hypertonic muscle is placed at the end of its spastic holding in shortened range for periods of time are valid treat-
range and a quick stretch is applied and held, then total ment procedures. Rood stressed the need for patients to work
facilitation followed by total inhibition will occur because of in and out of those shortened ranges in order to gain postural
PEI. As the inhibition phase is felt, the therapist can passively control as well as to practice directing the limbs during both
lengthen the spastic muscle until the facilitatory phase sets in closed and open chain activities.
repolarization. At that time the clinician holds the lengthened Feldenkrais. The Feldenkrais concepts225,226 of sensory
position. Increased tone will ensue, followed by inhibition awareness through movement place emphasis on relaxation
and continued lengthening. Holding the range (not allowing of muscles on stretch, and distracting and compressing
concentric contraction during the excitatory phase) is critical. joints for sensory awareness. Both techniques reflect com-
If the muscle is held as the tone increases, the resistance and bined proprioceptive techniques. Taking muscles off stretch
stretch are then maximal and probably further facilitate the slows general afferent firing and thus overload to the CNS.
inhibitory phase. Compression and distraction of joints enhance specific input
from a body part while simultaneously facilitating input tests look for adverse responses to physical examination of
of a lesser intensity from other body segments. This com- neural tissues. These adverse responses are muscle tone
bined proprioceptive approach enhances body schema increases as a result of painful provocation of sensitized
awareness in a relaxed environment. It also integrates em- neural tissue nociceptors attempting to prevent further pain
powerment of the client by use of visualization and asking by limiting the movement of the neural tissue.248 Pain
for volitional control. (See Chapters 27 and 39 for additional increases tone and leads to limited range of passive move-
information.) ment.248,249 Pain-free range suggests CNS sensitivity to the
Manual Therapy, Specifically Maitland’s. “The periph- large, highly myelinated alpha fibers and functions in a dis-
eral and central nervous systems need to be considered as criminatory manner. Pain range encompasses the degree of
one because they form a continuous tissue tract.”208,225,243-246 joint motion where neural length, as well as nociceptors in
Manual therapy or mobilization of joint or soft tissue struc- the skin, fascia, muscles, and joints, plays a primary role in
tures is not specific to orthopedic conditions, nor are neuro- CNS attention and protection. Inflammation of neural tissue
logical treatment principles ineffective on orthopedic can also cause the nociceptors to become hypersensitized or
patients. Regardless of the diagnosis or pathological body more reactive to mechanical or chemical changes. This
system leading to joint immobility, the functional conse- is particularly true in the joint when the nociceptors react
quences can be synonymous. Joint immobility can cause the significantly to movement at the end ranges.248
peripheral nerves to lose their adaptability to change in the Treatment will be based on the degree of immobility, the
length of the nerve bed. This change in neural elasticity then pain range, the site of the irritability, and the degree of pain.
creates additional problems in connective tissue function, Butler228 not only looks at joint problems but also considers
which in turn may affect the function of the motor system’s many joint problems as having adverse neural dynamics (ten-
control over the musculoskeletal component.228,247 For this sion on the PNS). Treatment still incorporates Maitland’s
reason alone, discussion of musculoskeletal mobilization grades of passive movement, but with consideration across
needs to be included in this section as a component of the length of the neural tissue across multiple joints.
classification. Butler247,250 divides treatment of the joint into three
“Pathological processes may interfere with both of these categories: limitations, pain, and adverse mechanical ten-
mechanisms: extraneural pathology will affect the nerve/ sion. When analyzing selective nervous system mobilization
interface relationship and intraneural pathology will affect as identified by Butler, the therapist needs to mobilize the
the intrinsic elasticity of the nervous system.”247 Patient nervous system and its surrounding fascia rather than
complaints of pain that limits functional movements consti- stretching it. These techniques may be either gentle (grade I)
tute the primary reason clients are referred to a therapist for or strong (grade IV), through the range (grades II and III),
a musculoskeletal evaluation. During the physical examina- or at end range only (grade IV). Different disorders (irritable
tion, tension tests can be used to determine the degree of compared with nonirritable) will require different treatment
pain and joint limitation, to differentiate between somatic approaches (Figure 9-3).
and radicular symptoms, and to identify adverse neuro- Treatment must interface with related tissues. When joint
physiological changes in the PNS.247 “The increased muscle immobility is interfaced with muscle and fascia tightness, all
tone (in a peripheral injury) is considered to be a protective components must be treated simultaneously. If the focus of
mechanism for the inflamed tissue.”248 This increase in tone treatment is the correction of joint and muscle signs, then
may be caused by a dampening of presynaptic activity of the constant reassessment of the effect on the nervous system is
flexor reflex afferent by supraspinal mechanisms. This same crucial. This aspect would seem even more crucial in clients
mechanism may be triggered by a CNS injury. The differ- with CNS and PNS injuries. The treatment may be direct or
ence between the orthopedic patient and the neurological indirect. Direct intervention involves procedures aimed at
patient may be the trigger to the CNS. In a central lesion the rebalancing the neuromusculoskeletal system through
motor generators are often not adequately maintained after strengthening and increasing ROM to improve motor con-
injury, which results in hypotonicity. The hypotonicity trol. Indirect treatment includes the use of movement pat-
causes peripheral instability, stretches peripheral tissue, and terns, especially posture-based patterns. When individuals
potentially causes peripheral damage. In both orthopedic have nervous system changes, static and dynamic postural
and neurological cases, there is peripheral instability, patterns often emerge as compensatory reactions to the
the first the result of peripheral damage and the second the problem state. Pain posturing, tension, or stiffness from
result of hypotonicity. The CNS response to the instability prolonged positioning, and forced postures that are the
may be the same: an increase in muscle tone by dampening result of synergy patterns, to name a few, all seem to
of presynaptic inhibition. A decrease in presynaptic inhibi- respond well to indirect treatment with or without passive
tion on incoming afferents would cause an increase in spinal CNS mobilization. The use of posture-based movement pat-
generator activity. With an isolated musculoskeletal problem terns during functional activities also provides for variability
and an intact CNS, the motor system would have the adapt- and repetition and thus should lead to greater carryover in
ability and control to modulate the spinal generators and motor learning.
isolate only those components in which an increase in tone Many manual therapy approaches affect and use the pro-
might directly affect the problems. The client with CNS prioceptive system as a means to change motor responses.
involvement may lose some of the flexibility of the motor The reader is again reminded that the proprioceptive system
system’s control over the pattern generators, and thus affects all systems within the CNS and vice versa. The end
high-tone synergistic patterns may develop. effect of all system interactions will be intrinsic reinforce-
In either case, the peripheral system needs to be evalu- ment of existing behavior or changes in and adaptations
ated and intervention provided when necessary. Tension of behavior to meet intrinsic and extrinsic demands. The
Treatment Alternatives Using the Exteroceptive

System. The function of the exteroceptive system is to
inform the nervous system about the surrounding world. The
CNS will adapt behavior to coexist and survive within this
environment. Although many protective responses are pat-
terned within the motor system, these patterned responses
can be changed or modulated according to momentary
inherent chemistry, attitude, motivation, alertness, and so
on. Different from some of the other treatment approaches,
the function of the exteroceptive input system is not reflex-
ive but rather informative and adaptable.
Quick Phasic Withdrawal. The human organism reacts
to painful or noxious stimuli at both conscious and uncon-
scious levels. If the stimulus is brief and of noxious quality,
it will elicit a protective reaction of short duration with use
of the long-chain spinal reflex loops. Simultaneously, affer-
ent impulses ascend to higher centers to evoke prolonged
emotional-behavioral responses. Stimuli such as pain,
extremes in temperature, rapid movement, light touch, and
hair displacement are the most likely to cause this reaction
by activating free nerve endings. These stimuli are per-
ceived as potentially dangerous and communicate directly
with the reticular-activating system and nonspecific tha-
lamic nuclei. These structures have diffuse interconnections
with all regions of the cerebral cortex, ANS, limbic system,
cerebellum, and motor centers in the brain stem. Research
has shown that children who exhibit hyperactive with-
drawal reactions also develop negative emotional reactivity
and show significantly more avoidance behavior and in
time show right frontal asymmetry.251 These alerting stim-
uli have been linked to motor seizures in critically ill
patients.252 As indicated by these research studies, thera-
pists need to be aware of these potential responses, espe-
cially in patients with severe neurological insult that has
resulted in a lower level of consciousness. These low-
functioning clients cannot express their feelings nor how
their nervous system is reacting to the input. Thus therapists
need to be very aware of any motor response a patient may
express and try to avoid using stimuli that might trigger
Figure 9-3 n Grades of movement. (Modified from Maitland’s these avoidance behaviors. From observance of the behav-
theory of joint and tissue mobilization by John Sievert, PT, GDMT. ior of clients with chronic pain, these responses seem to
From Course notes, Graduate Diploma in Manipulative Therapy, become habitual and may lead to somatosensory remap-
Curtin University of Technology, Perth, Western Australia, 1990; ping, making it hard to differentiate protective from dis-
and from Maitland GD: Peripheral manipulation, ed 3, Boston, criminatory information. Thus, any movement or touch
1991, Butterworth Heinemann.) triggers pain. Patients need to be taught to discriminate
between tightness and true pain, and therapists need to feel
when the muscle response has shifted from muscle gliding
to muscle restriction. Therapists need to gain trust, and one
behavior observed by the therapist as the client initiates way is to not elicit a lot of pain. For example, if a therapist
motor strategies in response to functional goals will be a tells a patient to say something when it hurts, and the
consensus of all these interactions. patient says, “Now,” the therapist should never respond
with “Well, just a little more.” In that instant the patient has
Exteroceptive or Cutaneous Sensory System learned that the therapist lied (because the patient was told
Differentiation of Receptor Site as Augmented to tell the therapist when it hurt, suggesting that the thera-
Intervention. Humans have many different types of tactile pist would stop then) or that the therapist is a masochist. If
receptors. Some are superficial, and others are deep within the the therapist had stopped when the patient said that it hurt,
layers of the skin. These receptors have been identified within the patient would then know that he does not need to tell the
the chapter on motor learning. Their use as augmented inter- therapist to stop 10 degrees before it hurts because the thera-
vention strategies is discussed in the following section. pist is not going to range him that 10 extra degrees. Often
A list of treatment techniques using the exteroceptive the therapist will find that without any effort the patient
(tactile) input system as their primary mode of entry can be now has that extra range and has no need to splint the limb
found in Table 9-4. because it is not going to hurt to have therapy.
TABLE 9-4 n EXTEROCEPTIVE INPUT TECHNIQUES
RECEPTORS STIMULI RESPONSE*

Free nerve endings: C 1 A fibers Pain, temperature, touch Seem to protect and alert, perception of
temperatures, protective withdrawal
Hair follicles Mechanical displacement of hair receptors Increased tone of muscle below stimulus site
Merkel disk Touch: pressure receptors Touch identification
Meissner corpuscles Discriminative touch Postural tone; two-point discrimination
Pacinian corpuscles Deep pressure and quick stretch to tissue, vibration Position sense, postural tone and movement
Ruffini corpuscles Touch mechanoreceptor Touch and spatial discrimination
SUGGESTED TREATMENT PROCEDURES USING CUTANEOUS STIMULI
Quick Phasic Withdrawal
1. Stimulus
a. Pain
b. Cold: one-sweep with ice cubes, Rood’s quick ice
c. Light touch: brush (quick stroking), finger, feather
2. Response
a. Stimulus applied to an extensor surface: elicits a flexor withdrawal
b. Stimulus applied to flexor surface: may elicit flexor withdrawal or withdrawal from stimulus into extension
Prolonged Icing (Repetitive Icing Should Be Used with Caution Because of Rebound Effect)
1. Stimulus
a. Ice cube
b. Ice chips and wet towel
c. Bucket of ice water
d. Ice pack
e. Immersion of body part or total body
2. Response: inhibition of muscles below skin areas iced
Neutral Warmth
1. Stimulus
a. Air bag splints
b. Wrapping entire body or individual body part with towel
c. Tight clothing such as tights, fitted turtleneck jerseys, Lycra clothing
d. Tepid water or shower
2. Response: inhibition of area under which neutral warmth was applied
Light Touch, Rapid Stroking
1. Stimulus
a. Light intermittent tactile stimulus to an identified dermatome-myotome interaction area
2. Response: facilitation of muscle(s) related to the stimulus area
Maintained Pressure or Slow, Continuous Stroking with Pressure
1. Stimulus
a. Slowly rubbing the target area with a towel
b. Wearing Lycra or spandex clothing
2. Response: sensory receptor adaptation and decrease in afferent firing
*Response: adaptation of many cutaneous receptors to stimulus, thus decreasing exteroceptive input, decreasing reticular activity, and decreasing facilitation
of muscles underlying stimulated skin.
There are some real therapeutic limitations to using gelatinosa to close the gate and thus block transmission of
stimuli that “load” the spinothalamic system. A painful pain messages to the brain. Studies have demonstrated that
stimulus will be excitatory to the nervous system and pro- physical activity (types of physical stress) stimulates the
duce a prolonged reaction after discharge. According to production of endorphins, which in turn release opiate
Wall’s gate-control theory,253-257 all sensory afferent neurons receptors and act as the body’s own morphine for pain
converge and synapse in the dorsal horn in an area called the control20,212,259-262 (see Chapters 18 and 32).
substantia gelatinosa. Curiously, the large, more discrimina- Because light touch has both a protective and a discrimi-
tory fibers do outnumber the small fibers.258 Therefore, natory function, techniques such as brushing or stroking the
physical activity, frequent positioning, deep pressure, and skin with a soft brush have the potential of informing
proprioceptive and cutaneous stimulation should cause the CNS about (1) texture, object specificity, and error in
enough impulses to converge on cells within the substantia fine motor responses or (2) danger (eliciting a protective
response). If a protective response is triggered, the specific primary rami located along the midline of the dorsum of the
withdrawal pattern will depend on a variety of circum- trunk have sympathetic connections to internal organs. The
stances. If the stimulus is applied to an extensor surface, cold stimulus may alter organ activity and perhaps produce
then a flexor withdrawal will be facilitated. If the stimulus is vasoconstriction, causing increased blood pressure and
placed on a flexor surface, one of two responses occurs. reduced blood supply to the viscera.268,269
First, the client might withdraw from the stimulus, thus go- Brief administration of ice can have beneficial effects if
ing into an extensor pattern. Second, the stimulus may elicit the nervous system’s inhibitory mechanisms are in place.
a flexor withdrawal and cause the client to go into a flexor For instance, in children with learning disabilities or adults
pattern. Which pattern occurs depends on preexisting motor with sensorimotor delays, the application of ice to the pal-
programming bias as a result of positioning and the predis- mar surface of the hands will cause arousal at the cortical
position of the client’s CNS. Both responses would be con- level because of the increased activity of the reticular acti-
sidered normal. The condition or emotional state of the vating system. This arousal response presumably produces
nervous system and whether the stimulus is considered increased adrenal medullary secretions, resulting in various
threatening also determine the sensitivity of the response, metabolic changes. Therefore icing should be used selec-
again reinforcing the systems’ interdependence. These tively. If the patient has an unstable ANS, icing should be
responses are protective and do not lead to repetition of eliminated as a potential sensory modality.270
movement or motor learning. For that reason, along with the Prolonged Use of Ice. Physicians have used therapeutic
emotional and autonomic reactions, a phasic withdrawal to cold for the treatment of individuals with high fever and/or
facilitate flexion or extension is not recommended as a treat- intracranial pressure with the intent of reducing the body
ment approach unless all other possibilities have been temperature or brain swelling to prevent brain damage.271
eliminated. This procedure is done with cooling pans or blankets.
Short Duration, High-Intensity Icing. Cold is another Whole-body cryotherapy has been used to reduce inflamma-
stimulus that the nervous system perceives as potentially tion and pain and overcome symptoms that prevent normal
dangerous. The use of ice as a stimulus to elicit desired movement. This type of therapy consists of the use of very
motor patterns is an early technique developed by Rood. Her cold air maintained for 2 minutes in cryochambers. A recent
technique was referred to as repetitive icing. An ice cube is study looked at this type of therapy for injured athletes. It
rubbed with pressure for 3 to 5 seconds or used in a quick- was found that the procedure did not cause harm to the indi-
sweep motion over the muscle bellies to be facilitated. This vidual.272 This approach does not seem realistic for use in
method activates both exteroceptors and proprioceptors and occupational or physical therapy clinics.
causes a brief arousal of the cortex. This method can A variety of approaches that incorporate prolonged icing
produce unpredictable results. Although initially a phasic techniques have been used in therapy clinics for decades.
withdrawal pattern generator response will be activated The PNF approach may be the most common.19 Inhibition of
immediately after the reflex has taken place, the “rebound” hypertonicity or pain is the goal for the use of any of these
phenomenon deactivates the muscle that has been stimulated methods. With prolonged cold the neurotransmission of
and lowers the resting potential of the antagonistic mus- impulses, both afferent and efferent, is reduced. Simultane-
cle.263 Therefore a second stimulus to the same dermatome- ously the metabolic rate within the cooled tissue is reduced
myotome neural network may not elicit a second response. (see Chapter 32). Caution must be exercised with regard to
But, because of reciprocal innervation, the antagonistic the use of this modality. However, for effective treatment
muscle may effect a rebound movement in the opposite results, the client (1) should be receptive to the modality,
direction. Icing may also cause prolonged reaction after (2) should be able to monitor the cold stimulus (sensory
discharge because of the connections to the reticular system, deficits should not be present), and (3) should have a stable
limbic system, and ANS. Thus the ANS would be shifted autonomic system to prevent unnecessary adverse effects of
toward the sympathetic end. Too much sympathetic tone hypothermia. Research of the last decade has consistently
causes a desynchronization of the cortex.264 Although the shown that cryotherapy is an effective tool for reducing pain
resting state of the spinal generator may be altered briefly, if and has helped individuals regain integration of axial mus-
the heightened state persists the cause is most likely fear or culature after neurological insults.273-276 Individuals of all
sympathetic overflow (see Chapter 5). This state is destabi- ages seem to respond similarly, which allows therapists to
lizing to the system and most likely will not lead to any use this therapeutic tool across generations.277
motor learning. Because of unpredictable response patterns Ice immersion of the contralateral limb was used decades
to Rood’s repetitive icing, this technique is seldom used. ago in order to get a reflexive decrease in temperature in the
The therapist is cautioned not to use short-duration, high- affected limb. It was believed that this intralimb reflex was
intensity icing to the facial region above the level of the lips, an effective way of treating pain without directly treating the
to the forehead, or to the midline of the trunk. These areas limb. Recent research has validated that belief.278
have a high concentration of pain fibers and a strong connec- Ice massage is another form of prolonged icing and is
tion to the reticular system.10,265 often used to treat somatic pain problems.279 It is also used
Ice should not be used behind the ear because it may over high-toned muscles to dampen striated muscle contrac-
produce a sudden lowering of blood pressure.266 The thera- tions. Caution must be used when eliminating pain without
pist should also avoid using ice in the left shoulder region in correcting the problem causing pain. For example, if insta-
patients with a history of heart disease because referred pain bility causes muscle tone and pain, then icing might
from angina pectoris manifests itself in the left shoulder decrease pain while causing additional joint instability and
area, indicating that the cold stimulus might cause a reflex- potential damage. The end result would be an increase, not
ive constriction of the coronary arteries.267 In addition, the a decrease, in pain and motor dysfunction.
Neutral Warmth. Like icing, neutral warmth alters the the therapist. Using boxes with an opening so the individual
state of the motor generators, either directly or indirectly can insert a hand and arm but cannot see what is inside, a
through afferent input. According to Farber,12 the length of patient can work on discriminating textures, objects, letter,
application depends on the client. A 3- to 4-minute tepid numbers, and so on while working on higher-order process-
bath may create the same results as a 15-minute total-body- ing. Once this touch has been integrated, the patient can also
wrapping procedure. As with any input procedure, the use light touch to determine balance, position in space, and
effects should be incorporated into the therapeutic session various other types of perceptual tasks.283
to maximize the results and promote client learning. The
Johnstone approach uses air splints effectively as a neutral Vestibular System (Refer to Chapter 22B)
warm treatment intervention while clients work on func- Vestibular Treatment Techniques. The vestibular
tional activities.17 If neutral warmth is applied as an isolated system is a unique sensory system, critical for multisensory
intervention, the client may feel relaxation or a decrease functioning, making it a viable and powerful input modality
in discomfort, but neuroplastic CNS changes are unlikely, for therapeutic intervention (see Chapter 22B). Any static
owing to the lack of repetition, attention, and error correc- position and any movement pattern will facilitate the laby-
tion by the client during activities. A recent study looked at rinthine system; therefore vestibular function and dysfunc-
blood pressure, heart rate, and other autonomic mechanisms tion play a role in all therapeutic activities. To conceptualize
in subjects using compression hose. The researchers did not vestibular stimulation as spinning or angular acceleration
look at neutral warmth as a mechanism to maintain a minimizes its therapeutic potential and also negates an en-
homeostatic state of the nervous system. Yet the use of com- tire progression of vestibular treatment techniques.12,41,284-286
pression hose does create a state of neutral warmth, and the Linear movements in horizontal and vertical postures and
link to homeostasis can easily be made.280 forward-backward directions occur early in development
Maintained Stimulus or Pressure. Because of the rapid and should be considered one viable treatment modality.
adaptation of many cutaneous receptors, a maintained stim- These movements seem to precede side-to-side and diagonal
ulus will effectively cause inhibition by preventing further movements, which are followed by linear acceleration and
stimuli from entering the system. This technique is applied end with rotational movements. All these movements can be
to hypersensitive areas to normalize skin responses. Vibra- done with assistance or independently by the client in all
tion used alternately with maintained pressure can be highly functional activities. It is important to remember that the
effective. It should be remembered that these combined rate of vestibular stimulation determines the effects. A con-
inputs use different neurophysiological mechanisms. It is stant, slow, repetitive rocking pattern, irrespective of plan or
often observed that low-frequency maintained vibration is direction, generally causes inhibition of total-body responses
especially effective with learning-disabled children who via the alpha motor neuron but not the spindles,287 whereas
have hypersensitive tactile systems that prevent them from a fast spin or fast linear movement tends to heighten both
comfortable exploration of their environment. When chil- alertness and the motor responses. Again, the vestibular
dren themselves use vibration on the extremities, their mechanism is only one of many that influence the motor
hypersensitive systems seem to normalize and they become system. Thus, the system interaction must be constantly
receptive to exploring objects. If that exploration is accom- reassessed.
panied by additional prolonged pressure, such as digging in As already indicated, constant, slow, repetitive rocking
a sandbox, the technique seems to be more effective because patterns, irrespective of plane or direction, generally cause
of the adaptive responses of the nervous system. inhibition of the total-body responses. Yet any stimulus
Maintained pressure approaches using elastic stockings, has the potential of causing undesired responses, such as
tight form-fitting clothing (e.g., wet suits, expanded polytet- increased or decreased tone. When this occurs, the proce-
rafluoroethylene [Gore-Tex] biking clothing), air splints, dure should be stopped and reanalyzed to determine the
and other techniques can be incorporated into a client’s daily reason for the observed or palpated response. For example,
activity without altering lifestyle. The use of TheraTogs in assume that a client, whether a child with cerebral palsy, an
children with various hyperactivity conditions has become adolescent with head trauma, or an adult with anoxia, exhib-
an accepted therapeutic tool. They add some resistance, its signs of severe generalized extensor hypertonicity in the
some support, and maintained pressure.281 TheraTogs have supine position. To dampen the general motor response, the
also been shown to be effective in assisting individuals with therapist decides to use a slow, gentle rocking procedure in
hemiplegia to regain abductor control.282 supine position and discovers that the hypertonicity has
In this way clients can self-regulate their systems, allow- increased. Obviously, the procedure did not elicit the desired
ing greater variability in adapting to the environment. response and alternative treatment is selected, but the reason
Owing to the multisensory and multineuronal pathways for the increased hypertonicity needs to be addressed.
used when peripheral input is augmented, traditional linear, It is possible that the static positioning of the vestibular
allopathic research on human subjects is extremely difficult system is causing the release of the original tone and that
to design or measure with control. But outcome studies through increasing of the vestibular input the tone also
demonstrating efficacy are possible. Initially, efficacy con- increases. It may also be that the facilitatory input did
firmed by observation was acceptable. Now it is time to indeed cause inhibition, but the movement itself caused fear
repeat studies and use objective measures to demonstrate the and anxiety, thus increasing preexisting tone and overriding
same outcome. the inhibitory technique. Instead of selecting an entirely new
Light Discriminatory Touch. Once an individual can treatment approach, a therapist could use the same proce-
discriminate light touch both for protection and for discrimi- dure in a different spatial plane, such as a side-lying, prone,
natory learning, a lot of therapeutic tools become available to or sitting position. Each position affects the static position of
the vestibular system differently and may differentially Pelvic mobilization techniques in sitting use relaxation
affect the excessive extensor tone observed in the client. The from slow rocking to release the fixed pelvis. This release
vertical sitting position adds flexion to the system, which allows for joint mobility and thus creates the potential for
has the potential of further dampening extensor tone. This pelvic movement performed passively by the therapist, with
additional inhibition may be necessary to determine whether the assistance of the therapist, or actively by the client. This
the slow rocking pattern will be effective with this client. It technique often combines vestibular with proprioceptive
would seem obvious that if a vestibular procedure was inef- techniques, such as rotation and elongation of muscle
fective in modifying the preexisting extensor tone, then use groups, which physiologically modify existing fixed tonal
of a powerful procedure, such as spinning, would be inap- response through motor mechanisms or systems interac-
propriate. Selection of treatment techniques should be deter- tions. Simultaneously, slow, rhythmic rocking, especially on
mined according to client needs and disability. Clients either diagonals, is used to incorporate all planes of motion and
with an acoustic tumor that perforates into the brain stem or thus all vestibular receptor sites to get maximal dampening
with generalized inflammatory disorders may be hypersensi- effect, whether directly through the vestibulospinal system
tive to vestibular stimulation, whereas other clients, such as or indirectly through the cerebellum and reticular spinal
a child with a learning disability, may be in need of massive motor system. The same pelvic mobility can be achieved by
input through this system. Heiniger and Randolph41 and placing the patient (child or adult) over a large ball. The ball
Farber12,111 present in-depth analyses of various specific must be large enough for the patient to be semiprone while
vestibular treatment procedures commonly used in the arms are abducted and externally rotated and legs relaxed
clinic. A general summary of the treatment suggestions is (either draped over the ball or in the therapist’s arms).
summarized in Table 9-5. Again, this position allows for maintained or prolonged
The literature clearly establishes the causation of one stretch to tight muscles both in the extremities and in the
vestibular imbalance, dizziness, for all age groups.288-291 trunk while doing slow, rhythmical rocking over the ball.
Certainly individuals can have vestibular problems and will The pelvis often releases, and the patient can be rolled off
present themselves as being dizzy or hyperactive to move- the large ball to stand on a relaxed pelvis preliminary to gait
ment of the head. There is a lot of literature discussing treat- activities. A word of caution must be given regarding use of
ment of dizziness, and only a few publications are listed a large ball for relaxation. It is much easier to control the
here.292-294 There is certainly evidence to show how the ves- ball when someone is assisting that control from the oppo-
tibular system links to the autonomic nervous system and site direction (in front of the patient). If slow rocking is done
especially the sympathetic pathways.295 In Chapter 22B the and the therapist is keeping his or her voice monotonous for
reader will be able to find in-depth discussion of vestibular further relaxation, the individual assisting will also relax.
rehabilitation and the role movement scientists play in that One author has had family members fall asleep and slowly
rehabilitation. or quickly fall to the floor.
General Body Responses Leading to Relaxation. Any Techniques to Heighten Postural Extensors. Any tech-
technique performed in a slow, continuous, even pattern nique that uses rapid anteroposterior or angular acceleration
will cause a generalized dampening of the motor output.296 of the head and body while the client is prone will facilitate
During handling techniques, these procedures can be per- a postural extensor response. Scooter boards down inclines,
formed with the client in bed, on a mat while horizontal, rapid acceleration forward over a ball or bolster, going down
sitting at bedside or in a chair, or standing. The movement slides prone, and using a platform or mesh net to propel
can be done passively by the therapist or actively by the cli- someone will all facilitate a similar vestibular response of
ent. Carryover into motor learning will best be accom- righting of the head with postural overflow down into the
plished when the client performs the movement actively, shoulder girdle, trunk, hips, and lower extremities. Rapid
without therapeutic assistance. In a clinical or school set- movements while on elbows, on extended elbows, and in
ting, a client who is extremely anxious, hyperactive, and a crawling position can also facilitate a similar response.
hypertonic may initiate slow rocking to decrease tone or feel Depending on the intensity of the stimulus, the response will
less anxious or hyperactive. The reduction of clinical signs vary. In addition, the client’s emotional level during intro-
allows the client to sit with less effort and to be more atten- duction to various types of stimuli may cause differences in
tive to the environment, thus promoting the ability to learn tonal patterns. Clinical experience has shown that facilita-
and adapt. tory vestibular stimulation promotes verbal responses and
It is the type of movement, not the technique, that is affects oral-motor mechanisms. Children with speech delays
critical. The concept of slow, continuous patterns is used in will speak out spontaneously and respond verbally.
Brunnstrom’s rocking patterns8 in early sitting, in PNF mat Because facilitatory vestibular stimulation biases the
programs, and in therapeutic ball exercise programs; the use sympathetic branch of the ANS, drooling diminishes and a
of these patterns can be observed in every clinic. Although generalized arousal response occurs at the cortical level.
the therapist may be unaware of why Mr. Smith gets so Therefore the appropriate time to teach adaptive rehabilita-
relaxed when slowly rocked from side to side in sitting, this tive techniques is after vestibular stimulation.297
procedure elicits an appropriate response. The nurse taking Facilitatory Techniques Influencing Whole-Body Re-
Mr. Smith for a slow wheelchair ride around the hospital sponses. Tactile, vestibular, and proprioceptive inputs also
grounds may do the same thing. Once the relaxation or inhi- assist in the regulation of the body’s responses to move-
bition has occurred, the groundwork for a therapeutic envi- ment.35,111 As stated previously, the vestibular system, when
ronment has been created to promote further learning, such facilitated with fast, irregular, or angular movement, such as
as learning of ADL skills. The technique in and of itself will spinning, not only induces tonal responses but also causes
relax the individual but not create change or learning. massive reticular activity and overflow into higher centers.
216
TABLE 9-5 n COMBINED INPUT SENSORY SYSTEMS: TREATMENT MODALITIES
INHERENT RESPONSE
SECTION I
PROPRIOCEPTIVE:
JOINT, TENDON NOT
TECHNIQUE SPINDLE EXTEROCEPTIVE VESTIBULAR GUSTATORY OLFACTORY AUDITORY VISUAL ANS LABELED LABELED
Sweep tapping620 X X
n
Brunnstrom rolling X X Automatic extension
Foundations for Clinical Practice in Neurological Rehabilitation

(hand)40 of hand
Raimiste sign40 X X ?
Stretch pressure620 X X
Digging in sand, X X ?
and so on
Gentle shaking620 X X
Prone activities X X X X Automatic righting of
over ball51,620,621 head (tectospinal or
vestibulospinal)
Sitting activities on X ? X X X OLR and balance
ball51 (all systems)
Mat activities X X X ? ?
Resistive exercises X X
1. Resistive X X X If verbal If visual leads Rotatory
rolling command integra-
tion
2. Resistive X X Depends on X X
patterns: pattern
PNF622-624
3. Resistive gait625 X ? Depends on If verbal X
pattern command
4. Isokinetics626,627 X Some X
5. Wall pulleys X X (if done X (if guided
in body toward
rotation) target)
6. Rowing40 X ? X If verbal X Body
command rotation
Feeding51,99,620
1. Maintained X X ? ?
pressure:
walking to
back of tongue
2. Resistive
sucking
a. Straw X X ? ?
b. Popsicle X X X X X
3. Use of textures X X X X X
a. Peanut butter
b. Applesauce
4. Maintained X X X Automatic
pressure to closing of
top lip mouth
Inverted TLR620,628 X X ? X X
Touch X X X X Decreased hypersensi-
bombardment620 tive tactile system
and thus withdrawal
pattern: stereognosis
1. Tactile discrim-
ination in sand,
and so on
2. Pool therapy
Joint compression X X X
more than body
weight629,630
Throwing and
catching
1. Balloon ? X X ? ? (withdrawal to light
touch)
2. Heavy ball X ? X Result of
CHAPTER 9
light
touch
Variance in
movement
1. Quick action X X X
n
directed by
Interventions for Clients with Movement Limitations

vision
2. Postural X ? X
activities in
front of mirror
3. Therapist using X X X
voice command
to assist client
with movement
High-level
movement
1. Walking X X ? ? If visually X Labyrithine righting
balance beam corrected and equilibrium;
possible OLR
2. Trampoline X X If visually X OLR and equilibrium
activities corrected
3. Running, X X
jumping, skipping
217
OLR, Optic and labyrinthine righting reactions; PNF, proprioceptive neuromuscular facilitation; TLR, tonic labyrinthine reflex.
Thus increased attention and alertness are often the outcome. the inverted position produces three major changes. First,
The tracts going from the spinal cord, brain stem, and higher because of the gravitational forces on circulation, the carotid
subcortical structures must be sufficiently intact to permit the sinus sends messages to the medulla and cardiac centers that
desired responses from this type of input. If a lesion in the ultimately lower heart rate, respiration, and resting blood
brain stem blocks higher-center communication with the pressure through peripheral dilation, creating a parasympa-
vestibular apparatus, then massive input may cause a large thetic response pattern. This position may be contraindi-
increase in abnormal tone. The therapist needs to closely cated for certain patients with a history of cardiovascular
monitor any distress or ANS anomalies.295 disease, glaucoma, or completed stroke. Clients with unsta-
Total-Body Relaxation Followed by Selective Postural ble intracranial pressure—for example, those with traumatic
Facilitation. The use of the inverted position in therapy has head injuries, coma, tumor, or postinflammatory disorders—
become very popular as a way to relax postural muscles and and many children with congenital spinal cord lesions
decrease compression between vertebrae.298 Not only does would also be at high risk for further injury if the inverted
this decrease pain, but it also causes relaxation. Earlier position were used. However, this position has been used
research on the labyrinth’s influence on posture and the with some success for adult patients with hypertension.
influence of the inverted position showed that total inversion In any case, scrupulous recording of blood pressure and
(angle of 0 degrees) produced maximal postural extensor other ANS effects should be taken before, during, and after
tone, and the normal upright position elicited maximal positioning.
flexor tonicity.230 There seems to be confusion in the litera- Another benefit of the inverted position is generalized
ture about the clinical effects of inversion. The initial relaxation. Farber12 recommends its use as an inhibitory
research was performed on anesthetized animals and cannot technique. Because the carotid sinus stimulates the para-
be representative of how the human CNS responds to inver- sympathetic system, the trophotropic system is influenced
sion as a system. Kottke299 reports that the static labyrinthine and muscle tonicity is reduced. This has been found to be
reflex is maximal when the head is tilted back in the semire- beneficial to patients with upper motor neuron lesions and
clining position at an angle of 60 degrees above the horizon- also to children who exhibit hyperkinetic behavior. Heiniger
tal. Conversely, minimal stimulation occurs when the head and Randolph41 report that severe hypertonicity in the upper
is prone and down 60 degrees below the horizontal position. extremities is noticeably reduced.
Stejskal297 studied the effects of the tonic labyrinthine posi- The third benefit of the inverted position is an increased
tion in hypertonic patients. This study failed to show laby- tonicity of certain extensor muscles. This phenomenon is
rinthine reflexes in subjects with hypertonia. The problem not purely a function of the labyrinth; it is also a result of
with use of the inverted position is its lack of permanency. It activation of the exteroceptors being stimulated by the
is a contrived technique used to relieve pain or to achieve body’s contact with the positioning apparatus.305 Therapists
total relaxation. The effectiveness of this approach comes have capitalized on this reaction to activate specific extensor
with the next set of therapeutic activities that allow the CNS muscles of the neck, trunk, and limb girdles.27,297,299
to maintain that relaxation for a period of time and hopefully Because the inverted position decreases hypertonicity
indefinitely over a series of multiple treatments. and hyperactivity and facilitates normal postural extensor
The explanation for the incongruity in the literature over patterns, the responses to the technique should be incorpo-
decades seems to be one of interpretation. Any time a sub- rated into meaningful functional activities. For example, if
ject is put on a tilt table or even a scooter board, the weight the position of total inversion over a ball is used, then pos-
bearing of the body on the surface must cause firing of the tural extension of the head, trunk, and shoulder girdles and
underlying exteroceptors while gravity pulls on the proprio- hips should be facilitated next. Additional facilitation tech-
ceptors. This position also has the potential to create fear.300 niques, such as vibration or tapping, could help summate the
As the body shifts and presses onto the underlying surface, response. Resistance to the pattern in a functional or play
stretch reflexes associated with posture and movement must activity would be the ultimate goal. If the inverted position
contribute some bias to muscle tone.301 In addition, if is used in a squat pattern, then squatting to standing against
the subject is in supine and the neck flexors are activated resistance would probably be a primary goal. This can be
eccentrically (being lowered to supine) or concentrically accomplished by the therapist positioning his or her body
(being pulled toward sitting or actively lifting the head), or behind and over the child, not only to direct the child
if the subject is in prone and the neck extensors are activated initially into the inverted position but also to resist the child
eccentrically (lowering the head toward the ground) or con- coming to stand. If the inverted position is used in sitting,
centrically (holding the head up in prone), the propriocep- activities of the neck, trunk, and upper extremities would be
tors of the neck could alter the muscle tone of the limbs.302 the major focus after the initial responses.
Another factor that contributes to tonal changes in the Because the inverted position elicits both labyrinthine
extremities is the cervicoocular reflex.303,304 Reflex eye and ANS responses, this technique needs to be cross-
movements to center the eyes as the body or neck rotates referenced within the classification schema. Because of its
also exert influences on the muscles of the limbs. Because ANS influence, close monitoring is important for all clients
all the influences brought about by gravity and postural placed in an inverted position. As with all labyrinthine treat-
mechanisms in a clinical situation cannot be controlled, the ment techniques, this approach, considered a normal, inher-
inverted position appears to be an interplay of cutaneous ent human response, is used outside the therapeutic setting.
receptors, proprioceptors, and tonal changes in the labyrin- For example, standing on one’s head in a yoga exercise
thine system.305 causes the same physiological state as that observed in
Several highly recognized therapists have reported using the clinic. In many respects the yoga stance is done for the
the inverted position as a therapeutic modality.12,28,41 Generally same reasons: decreasing hypertonicity (generally caused by
tension), achieving relaxation, and increasing postural tone sympathetic nervous system. The technique is performed
and altered states of consciousness. Clients can certainly be while the client is in the prone position. The therapist
taught to control their own ANS activity and hypertonicity begins by stroking the cervical paravertebral region in the
by placing their hands between their legs when they need a direction of the thoracic area, using a slow, continuous
generalized dampening effect on motor generators. Thus, motion with one hand. Usually a lubricant is applied to the
when accessing and incorporating other approaches, the skin, and the index and middle fingers are used to stroke
therapist analyzes each specific technique with use of a both sides of the spinal column simultaneously. Once the
critical neuroscientific frame of reference. first hand is approaching the end of the lumbar section, the
This section has described procedures that use the second hand should begin a downward stroking at the cervi-
vestibular system as a primary input modality to alter the cal region. This maintains at least one point of contact with
client’s CNS. If the client’s vestibular system itself is dys- the client’s skin at all times during the procedure. The tech-
functional, this dysfunction has the potential to alter the nique is applied for 3 to 5 minutes—and no longer—
functional state of the motor system. See Chapter 22A for because of the potential for massive inhibition or rebound
additional information on balance and Chapter 22B for of the autonomic responses.35,296 It is also recommended
information on the vestibular system. that at the end of the range of the last stroking pattern, the
The therapist must always remember that in combining therapist maintain pressure for a few seconds to alert both
vestibular and proprioceptive input or asking the CNS to the somatic and visceral systems that the procedure has
process this information, a variety of results can develop. concluded. Eastern medicine recognizes the importance of
When the two input systems are congruent, the response will the ANS in total-body regulation to a greater extent than
be summated and the CNS will not need to make a lot of Western medicine does. The concepts of meridians and
adjustment. However, if the inputs are in conflict, then the acupressure and acupuncture points are all intricately inter-
CNS needs to update the differences and weigh which twined with the ANS (see Chapter 39). For that reason, a
stimulus is more relevant. Then the updating and response technique such as slow stroking would potentially interact
will be in direct proportion to how both inputs were with meridians and does extend over the row of acupunc-
weighted.306 ture points referred to as shu points and relates to visceral
reflexes connecting smooth muscle and specific organ sys-
Autonomic Nervous System tems. It is believed that this continuous, slow, downward
The ability to differentiate tone created by emotional responses pressure modulates the sympathetic outflow, causing a shift
versus tone resulting from CNS damage is a critical aspect of to a parasympathetic reaction or relaxation. Whether a
the evaluation process. Emotional tone can be reduced when result of the pressure on the sympathetic chain, some
stress, anxiety, and fear of the unknown have been reduced. energy pressure over meridian points, a pleasant sensation,
This is true for all individuals. The client with brain damage is or something unknown, slow stroking does elicit relaxation
no exception. Six treatment modalities307 that normally pro- and calming.41,111 Clients with large amounts of body
duce a parasympathetic or decreased sympathetic (flight or hair or hair whorls are poor candidates for this procedure
fight) response are as follows: because of the irritating effect of stroking against the
1. Slow, continuous stroking for 3 to 5 minutes over the growth patterns and the sensitivity of hair follicles.
paravertebral area of the spine Slow, Smooth, Passive Movement within Pain-Free
2. Inversion, eliciting carotid sinus reflex along with Range. Increasing ROM in painful joints is a dilemma
other somatosensory receptors (refer to the discussion frequently encountered by therapists caring for clients with
of vestibular system earlier in the chapter). neurological damage. Having the client communicate the
3. Slow, smooth, passive and active assistive movement first perception of pain and then moving the limb in a slow,
within a pain-free range (refer to Maitland’s grade II smooth motion toward the pain range elicits a variety of
movements (see Figure 9-3)20 behaviors. First, the client generally gestures or verbalizes
4. Deep breathing exercises (see Chapter 18) that pain is present 10 to 15 degrees before it may, in reality,
5. Progressive muscle relaxation exist. This behavior may occur because the patient during
6. Cranial sacral manipulation (see Chapter 39) previous treatment interventions learned that therapists
When pressure is applied to both the anterior and posterior often responded to the client’s statement of pain by saying,
surfaces of the body, measurable reductions may be recorded “Let’s just go a little farther.” That additional range is usu-
in pulse rate, metabolic activity, oxygen consumption, and ally 10 to 15 degrees. If the therapist stops at the stated point
muscle tone.266,308 These pressure techniques are identified as of pain, retreats back into a pain-free area, and approaches
an intricate part of the many intervention approaches such as again, possibly with a slight variation in rotation or direc-
therapeutic touch,24,267 Feldenkrais,225-227,309 Maitland,20 mas- tion, the client will often relinquish the safety range and a
sage,310,311 and myofascial release.6,212,312-314 Although not true picture of the pain range will be obtained. The second
verbally identified, other techniques (e.g., neurodevelopmental finding is that if the motion toward the pain range is slow,
treatment (NDT),31,32 Rood,29,41,111 Brunnstrom,8 and PNF29) smooth, and continuous, then frequently much of the range
also place an important emphasis on the response of the patient that was initially painful becomes pain free. The hypothesis
to the therapist’s touch. is that slow, continuous motion is critical feedback for the
Treatment Alternatives Using the Autonomic Ner- ANS to handle imminent discomfort. The slow pattern pro-
vous System vides the ANS time to release endorphins, thus modifying
Slow Stroking. Slow stroking over the paravertebral the perception of pain and allowing for increased motion.
areas along the spine from the cervical through lumbar If the therapist stabilizes the painful joint and prevents
components will cause inhibition or a dampening of the the possibility of that joint going into the pain range, rapid,
oscillating movements can often be obtained within the procedures. Odors such as vanilla and banana have been
pain-free range. This maintains joint mobility and often, as used to facilitate sucking and licking motions.329,330 Ammo-
an end result, increases the pain-free range. This technique nia and vinegar have been used clinically to elicit with-
is not unique to the treatment of clients with neurological drawal patterns and increase arousal in semicomatose
problems; it is often used as a manual therapy proce- patients.331 When odors are used as a stimulant, the therapist
dure.212,253,315 Furthermore, one can move slowly into a must be aware of all behavior changes occurring within the
range that actually shortens muscles. If held for 30 seconds, client. Arousal, level of consciousness, tonal patterns, reflex
the muscle that is too short can relax, promoting greater behavior, and emotional levels all can be affected by odor.
motion in the opposite direction. This can be called Because of limited research in this area, caution must be
strain-counterstrain—inhibiting firing by maintaining a exercised to avoid indiscriminate use of the olfactory sys-
position of active insufficiency, making the muscle too short. tem. Odors such as body odor, perfumes, hairspray, and
Manual therapy20,148,316-319 can be used to describe the urine can affect the client’s behavior although the smell was
pain and joint changes occurring at the joint level. As not intended as a therapeutic procedure. Some clients, espe-
the fields of orthopedics and neurology merge into one cially those with head traumas and inflammatory disorders
system,228 with the brain acting as an organ controlling the of the CNS, often seem to be hypersensitive to smell. In
entire system and its components, the question of whether these cases the therapist needs to be aware of the external
the pain reduction is centrally or peripherally triggered may olfactory environment surrounding the client and to make
be an important one. The answer is probably both. For sure those odors that are present facilitate or at least do not
example, thumb pain can increase the sensation of the hinder desired response patterns.332
nervous system to the point that even cutaneous and proprio- Many clinical questions arise regarding smell as a thera-
ceptive receptors act as nociceptors. peutic modality. If the choice of odors is between pleasant
Maintained Pressure. Farber12 discusses a variety of and noxious, a pleasant odor will theoretically be perceived
techniques that facilitate a reduction of tone or hyperactiv- in a way that should be enjoyable, relaxing, and thus poten-
ity. Pressure to the palm of the hand or sole of the foot, to tially tone reducing. On the other hand, noxious odors
the tip of the upper lip, and to the abdomen all seem to pro- should cause a sympathetic reaction and, although produc-
duce this effect. The pressure need not be forceful, but it ing alertness, may also create a fight-or-flight internal reac-
should be firm and maintained.320 This same technique is tion that if repeated frequently could cause an adverse
defined as inhibitory casting when applied through the use response to the client’s perception of the world. This has the
of an orthosis (see Chapter 34). potential for having a profound effect on her or his feelings
Progressive Muscle Relaxation. Progressive muscle toward the therapist and the therapeutic environment. The
relaxation is practiced during both meditation and treatment effect may not be observable until the client reaches a
approaches such as Feldenkrais.309,320,321 These methods of level of consciousness or motor skill in which there is some
relaxation tend to trigger parasympathetic reactions, which ability to react.
in turn slow down heart rate and blood pressure and trigger Individuals’ perception of smell is not correlated to their
slow, deep breathing (see Chapters 18 and 39). The Alexander actual olfactory ability.333 Because of the complex neuronet-
technique has also been shown to cause relaxation while work of the olfactory system, the specifics between emo-
simultaneously increasing postural tone.322 tional responses and olfactory environment cannot be estab-
Cranial Sacral Manipulation. Summarizing the com- lished, and determining which olfactory input will drive a
plexity of cranial sacral theory is not within the scope of this pleasant, unpleasant, or neutral response is variable. There
book. The reader is referred to references to gain a global may be a cultural sensitivity to various smells that would
understanding of the treatment interactions and the ANS suggest a cultural learning linked with emotional responses
response to cranial therapy as well as a brief discussion in to smell.334-336 Therefore if a therapist is going to use smell
Chapter 39.307,312 This treatment approach needs to be more as part of therapy, identification of the individual’s prior
intensively researched in terms of physiological effects and likes and dislikes is very important. Family members and
clinical effectiveness. close friends will be the best people to consult in order to get
this information.
Olfactory System: Smell Without a sense of smell an individual may not be able to
The complexity of the olfactory system and how it interacts respond appropriately to various olfactory environments,
with nuclei that direct emotion in humans is still not totally which may increase a client’s feeling of isolation and lack of
understood. Yet quality of life in patients without smell (dys- social interactive skills.337-339 Smell is intricately linked to
osmic) is often impaired. How the neuroanatomy and neuro- the sense of taste. Without these sensory systems, individu-
physiology of human smell lead to a decreased quality of als tend to stop eating, thus creating an entirely different
life is still under investigation.323-326 health care issue.340,341
Smell evokes different responses by means of the limbic
system’s control over behavior. Pleasant odors, such as Gustatory Sense: Taste
vanilla or perfume, can evoke strong moods. Unpleasant Gustatory input is generally used as part of feeding and
odors can facilitate primitive protective reflexes, such as prefeeding activities. As already mentioned, the oral region
sneezing and choking. Sharp-smelling substances such as is sensitive not only to taste but also to pressure, texture, and
ammonia can elicit a reflex interruption of breathing.327,328 temperature. For that reason feeding would be classified as
As a result of arousal, protective reflexes, and mood a multisensory technique that uses gustatory input as one of
changes caused by odors, the use of smell as a treatment its entry modalities. Specific input modalities are based
modality has been implemented, especially during feeding on the combined taste, texture, temperature, and affective
response pattern—that is, both a banana and an apple may may prove to relate to one of those innate talents some thera-
be sweet, yet the textures vary greatly. When mashed, both pists have that distinguish them as gifted therapists.
fruits may have a pudding-like texture, yet the client’s emo- The emotional inflections used by the clinician certainly
tional response may differ. Disliking the taste of banana but have the potential to alter client response.348,349 For example,
enjoying apple may cause startling differences in the client’s assume the therapist asks Tim, a child with cerebral palsy, to
response during a feeding session. Thus the importance of walk. The specific response from the child may vary if the
the clinician’s sensitivity to the client’s response patterns clinician’s voice expresses anger, frustration, encourage-
within each sensory modality cannot be overemphasized.111 ment, disgust, understanding, or empathy. Knowing which
Similarly, a therapist needs to take into consideration normal emotional tone best coincides with a client’s need at a par-
changes with taste and smell that occur as a result of aging ticular moment may come with experience or sensitivity to
and adjust the input threshold appropriately.342,343 The inter- others’ unique needs.
relationship of taste and smell leads to the perception of Extraneous Noise. The varying level of sound or extra-
flavor. Current research has shown that the role of taste neous noise in a clinical setting can at times be overwhelm-
may be guided more by taste than by smell, but with each a ing. Dropping of foot pedals, messages over loudspeakers,
client will not be able to differentiate flavors of food.344 conversations, computers, printers, telephones, moans, a
Understanding this sensory system will lead to a greater jackhammer outside the clinic, water filling in a tank, a drip
understanding of some patient problems that follow CNS in a faucet, whirlpool agitators, a burn patient screaming,
damage.345 and a child crying all are encountered in the clinical environ-
ment, and all could be occurring simultaneously. A therapist
Auditory System whose CNS is intact usually can inhibit or screen out most
Treatment Alternatives with Use of the Auditory of the irrelevant sound, although his or her voice may rise
System. Because of the complexity of the auditory system, according to the surrounding noise and the therapist may not
a potentially large number of types of input modalities even be aware of the vocal change.347 Clients with CNS
exists. Although some of them might not be considered tra- damage may not have the ability to filter sensitivity to all
ditional therapeutic tools, they are nonetheless techniques these intermittent noise sensations.361 The protective arousal
that affect the CNS. Some treatment alternatives focus on responses these sounds might produce in a client could
the following: certainly elevate tone, block attention to the task, heighten
n Quality of voice (pitch and tone)346 irritability, and generally destroy client progress during a
n Quantity of voice (level and intensity)347 therapy session. Awareness of the noisy environment and the
n Affect of voice (emotional overtones)348,349 client’s response to it not only is important for treatment
n Spatial and temporal sound (how fast a stimulus modalities but also is critical to the problem-solving
occurs, and how frequently)350-354 process.
n Extraneous noise (sound)355 Decreasing auditory distracters or sudden noises can
n Auditory biofeedback356-362 drastically improve the client’s ability to attend to a task or
n Language363 to succeed at a desired movement.343,373 The therapist is
n Volume, level, and affect of voice364-366 reminded that if the environment has been externally adapted
n Auditory perception367-369 for a client to procedurally and successfully practice the
The therapist’s voice can be considered one of the most goal, then independence in that functional skill has not been
powerful therapeutic tools. Even constant sound has the achieved. Reintroduction of the noises of the external world
ability to cause adaptation of the auditory system and thus must be incorporated into the client’s repertoire of responses
inhibition of auditory sensitivity.141,355 Similarly, intermit- so that the individual can feel competent in dealing with any
tent, changing, or random auditory input can cause an auditory environment the world might present.
increase in auditory sensitivity.346,370 Because of auditory Music. Music as an adjunct to therapy has been sug-
system connections, an increase or decrease in initial input gested as a viable way to help clients develop timing and
or auditory sensitivity has the potential for drastically affect- rhythm in a movement sequence (see Chapter 20 for a dis-
ing many other areas of the CNS.371 The connections to the cussion of basal ganglia disorders and Chapters 5 and 39 for
cerebellum could affect the regulation of muscle tone. a discussion of music therapy). Consistent sound waves and
The collaterals projecting into the reticular formation could tempos, such as soft music, allow the patient to develop a
affect arousal, alertness, and attention, in addition to muscu- neuronal model or an engram for the stimulus. The use of
lar tone. The importance of voice level has been acknowl- background music during therapy sessions enables the
edged by colleagues for decades with respect to encouraging patient to make an association to the sounds, producing an
clients to achieve optimal output or maximal effort. The use autonomically induced relaxation response to a particular
of voice levels is a critical aspect of the entire PNF ap- musical composition.374-376 Therapists must remember that
proach.29 Yet the volume or intensity of a therapist’s voice is music has a very strong emotional link to all other areas of
only one aspect of this important clinical tool. Through the nervous system.377 For that reason, the use of music
clinical observation, it has been observed that clients respond needs to be discriminative and not randomly introduced
differently to various pitches.346 The response patterns and because the therapist likes the sound. Similarly, the music
specific range of comfortable pitch seem to be client depen- selected should be a piece that assists the patient and does
dent. The concept that each individual may have a range not become a deterrent to succeeding at the current motor
within the musical scale or even a specific note that is optimal task. The clinician will easily tell the difference by the tone
for biorhythm function has been proposed by one composer- the music creates (increase or decrease) and the success
musician.372 This concept needs research verification but made toward achieving the desired task.
Music is used for encouraging not only motor function to affect a client’s CNS. That input not only reaches the
but also memory378,379 and socialization.380-382 Rhythmic optic cortex for sight recognition and processing but also
sound perceived as an enjoyable sensation certainly has the projects to the brain stem and to the cerebellum through the
effect of creating motor patterns in response to that rhythm. tectocerebellar tract. Simultaneously, these afferents activate
Individuals, young and old, will tap their fingers or feet to a the reticular-activating and limbic spinal generators through
beat. If the beat has words, people will often sing along, the tectospinal tract.296,398 Thus, as long as light is entering a
recalling from memory the appropriate words. The move- client’s CNS, it has the potential to alter response patterns
ment, memory, and willingness to interact are all critical either directly—through the tectospinal system or the
aspects of the therapeutic environment. Having clients dance corticospinal system through occipitofrontal radiations—or
with a significant other twice a day to music they have en- indirectly through the influence of the ANS and limbic
joyed in the past encourages both the physical function and system on muscle tone resulting from emotional responses
the social bonding so important for quality of life.383 Music to light.399
affects heart rate, blood pressure, and respiration.384,385 It has The five categories of visual-system treatment alterna-
even been suggested that easy listening music may bolster tives should not be considered fixed, all-inclusive, or with-
the immune system.386-390 out overlap. The first three categories (color, lighting, and
Auditory Biofeedback. Biofeedback as a total therapeu- visual complexity) are common everyday visual stimuli.
tic modality is discussed under the treatment sections in Combined, they make up the visual world.
Chapters 33 and 39. Auditory biofeedback is generally Colors. When colors, hues, tones, the type of lighting,
thought of as a procedure in which sound is used to inform and the degree of complexity of the combined visual stimuli
the client of specific muscle activity.360,362 The level or pitch are varied, the treatment modality and the way the CNS
may change in relation to strength of muscle contraction or processes it change.400-407 Because the visual system tends to
specific muscle group activity. Yet auditory biofeedback also adapt to sustained, repetitive, even patterns, any input falling
encompasses feedback as simple as a foot slap that commu- under those parameters should elicit visual adapta-
nicates that a client’s foot is on the floor or verbal praise tion.141,408,409 This adaptation response will lead to decreased
after a successful therapeutic session.359 The importance of firing of sensory afferent fibers and have an overall effect of
the auditory feedback system as a regulatory mechanism decreasing CNS excitation. A clinician would expect to see
between internal and external homeostasis cannot be over- or palpate a decrease in muscle tone, a calming of the cli-
looked. However, the clinician should not assume that this ent’s affective mood, and a generalized inhibitory response.
system is intact and can automatically be used as a normal Cool colors, a darkened room, and monotone color schemes
feedback mechanism for clients with CNS damage.112,361,391 all seem to have an inhibitory effect. What a therapist might
Language. Although most therapists thoroughly appre- look for is a change in a patient’s behavior. For example,
ciate the complexity of the language system as a whole, they four days ago Patient A was placed on the green mat for
have little if any in-depth background to help them under- therapy and he seemed interactive, calm, and involved in
stand the components or the sequences leading to the devel- producing motor function. On the next day, he came to
opment of language.364,392,393 Thus many therapists are therapy and the red mat was available. When Patient A got
extremely frustrated when confronted with clients who show on the mat he became agitated and inattentive. The next day
perceptual or cognitive deficits involving the auditory pro- again Patient A was placed on the red mat and again was
cessing system. agitated and distracted. On day four, Patient A was placed
Therapists easily identify language comprehension diffi- on the green mat and had a great therapy session. On
culties with adults who have first language differences and this fourth day he was calm, interactive, and involved in
with young children because of their age and lack of regaining motor function. It would be easy for a therapist to
language experience. Nevertheless, many clients have a miss behavioral changes occurring when a patient is placed
language processing dysfunction that leads to communica- on a green or a red mat. These problems should be antici-
tion difficulties, both in reception and appropriate expres- pated when treating patients with emotional instability (see
sion.351 The elderly often can understand a conversation in a Chapters 5, 14, 23, 24, and 26).
quiet room but have difficulty in rooms that are noisy.371,394,395 In contrast, intermittent visual stimuli, bright colors,
The environment within which communication occurs can bright lights, and a random color scheme seem to alert
drastically affect both reception and the ability to express to the CNS and have a generalized facilitatory effect.410-412
the world inner feelings and thoughts.387 Creating an envi- Research in the 1980s in the area of criminology has pro-
ronment conducive to that exchange will dramatically affect duced evidence to suggest that specific shades of colors can
the motivation and drive of a patient within the therapeutic produce either a sedating response (such as certain pinks) or
setting.388 The complexity of auditory reception, processing, general arousal (certain blues).413 Although a tremendous
and responses is extremely extensive and could be over- amount of research is required to substantiate these results if
whelming to a PT or OT, but developing an understanding of the clinician is to apply them with confidence, research is
how auditory information affects motor performance will beginning to show that specific shades of colors and hues
certainly enhance the therapist’s analysis of movement may drastically affect a client’s general response to the
problems.396,397 world and specific response to a therapy session.403,404,407,414
Within the next few years, many facts regarding the reaction
Visual System of the CNS to specific visual stimuli may be uncovered, and
Treatment Alternatives with Use of the Visual the clinician will be responsible for integrating this new
System. Because light is an adequate stimulus for vision, information into the present categorization scheme.415
any light, no matter the degree of complexity, has the potential Although a person without body system problems may react
in specific ways to color, intensity, and visual distracters, Using rooms that have been stripped of such stimuli as fur-
individuals with CNS may not respond with the same niture and pictures can reduce not only distractibility but
behavior.416 In the Netherlands at the Institut de Hartenbuer, also hyperactivity and emotional tone. If this method of
playrooms have been designed in different colors.14 Except reduction of stimuli is used, the clinician must remember
for color, all rooms are exactly the same and originate from that this procedure has a sequential component. The client
a central hub or core.14 Children are allowed to select which must once again adapt to extraneous visual stimuli. Thus as
room they wish to play or be treated in. Children seem to the client’s coping mechanisms improve, the therapist needs
pick the color room that most suits their moods and alertness to monitor and change the visual environment. The therapist
and creates an environment in which they can learn.14 can monitor the amount of input according to the response
Lighting. Two types of lighting are found in a clinical patterns of the client but in time needs to have the client
environment. Fluorescent or luminescent lighting comes function in everyday environments and practice adaptation.
by definition from a nonthermal cold source. This type of Cognitive-Perceptual Sequencing with the Visual
lighting is generally emitted by a high-frequency pulse. System. In sighted individuals the visual system is impor-
Umphred (clinical observations, 1967 to 2005) has found tant for integrating many areas of perceptual development,
that many individuals within a normal population complain such as body schemes, body image, position in space, and
that this high-frequency flutter is irritating and causes spatial relationships.268,422,423 Vision as a processing system
distraction. For this reason, it is recommended that each is so highly developed and interrelated with other sensory
clinician observe clients’ responses to various types of light- systems that when intact it can be used to help integrate
ing to determine whether fluorescent visual stimuli cause other systems.395,424 Conversely, if the visual system is neu-
undesirable output.417 This is especially true with clients rologically damaged, it can cause problems in the process-
who already have an irritated CNS, such as those with ing of other systems.
inflammatory disorders (see Chapter 26), head trauma (see For example, assume that a child is asked to walk a bal-
Chapter 24), or seizure disorders.418,419 The clinician should ance beam while fixating on a target. The child is observed
also remember that clients frequently lie supine and look falling off the beam. On initial assessment vestibular-
directly at overhead lighting, whereas the therapist looking proprioceptive involvement would be primarily suspected.
at the client is unaware of that particular visual stimulus. On further testing the therapist might discover that the child,
The types of visual stimuli that may cause seizures and are while looking at the target, switches the lead eye in conjunc-
seen by clients within rehabilitation settings include com- tion with the ipsilateral leg. As the child switches from right
puters, videogames, television, and venetian blinds.417 For to left eye, the target will seem to move. Knowing the wall
that reason, any change in lighting should alert the clinicians is stationary, the child will assume the movement is caused
to watch for changes in their clients’ behavior. by body sway, will counter the force, and will fall off the
Incandescent lights by definition come from hot sources beam. The problem is a lack of bilateral integration of
and emit a constant light without a frequency. The bright- the visual system in contrast to other sensory modalities. The
ness of this type of lighting has the potential to alter CNS visual system deficit is overriding normal proprioceptive-
response. The visual system quickly responds to bright vestibular input to avoid CNS confusion. Unfortunately, the
lights with pupil constriction. After prolonged exposure to a client is attending to a deficit system and negating intact
bright environment, the visual system adapts and becomes ones. This visual conflict would be overriding the normal
progressively less sensitive to it.141,408 Similarly, when processing of intact systems.425
exposed to darkness the retina becomes more sensitive to An intact visual system can be overridden by deficits
small amounts of light. Because of the response of the visual in other systems. This can be seen in clients who are
system to incandescent lighting, it is recommended that a trying to relearn the concept of verticality. Clients with
therapist monitor the brightness of the lighting, especially hemiplegia who demonstrate a “pusher” syndrome illustrate
before any type of visual-perceptual training or visually this conflict. This clinical problem originates from a poste-
directed movement. rior thalamic stroke and less frequently with extrathalamic
Although the sun is a natural source of light, it is not lesions.426,427 An intact visual system can often be used to
generally the primary source in a clinical setting. The sun help reintegrate other sensory systems. First teaching clients
can effectively be used as indirect lighting, thus eliminating to attend to vestibular-proprioceptive cues while vision is
the problems produced by artificial lighting. Sunlight is occluded or visual stimuli tremendously reduced will help
also more acceptable psychologically. Some clinics have present a kinesthetic conflict. Individuals feel straight at
designed the buildings to allow for maximum use of natural 20 degrees or more to the ipsilesional side yet when not sup-
light.13 ported they fall. This conflict does not need to be verbally
Visual Complexity. The visual system is the primary discussed. The patients’ nervous systems will interpret the
spatial sense for monitoring moving and stationary objects conflict. The intent of the CNS is not to fall. If the patient
in space.420,421 An infant continually refines the ability to does not automatically self-correct, the therapist can add
discriminate objects in external space until capable of iden- reaching patterns across midline to assist. Then vision can
tifying specific objects amid a complex visual array.409 be reintroduced to assist orientation to vertical or upright
When brain damage occurs, the ability to identify objects, posture. The pusher syndrome is not just a posterior tha-
localize them in space, pick them out from other things, and lamic problem and can be combined with neglect. When
adapt to their presence may be drastically diminished.268 additional perceptual problems are added, the testing results
Because of the distractibility of many clients, reducing the and direction of the backward push can change.428 Once the
visual stimuli within their external space can help them cope orientation has been reestablished, visual input will often be
with the stimuli to which they are trying to pay attention. perceived in a more normal fashion. This syndrome has been
linked to the posterior thalamus as well as other integrative of cortical concentration and effort.418,456,457 Vision was
cortical areas within the brain.427,429-432 meant to lead and direct movement sequences.297,420,458 If it
Familiarity with the visual-perceptual system and its is used to modify each aspect of a movement, it cannot warn
interrelationships with all aspects of the therapeutic environ- or inform the CNS about what to expect when advancing to
ment is crucial if the clinician is to have a thorough concept the next movement sequence. Thus, using vision to compen-
of the client’s problem. (See Chapter 28 for specific infor- sate eliminates one problem but also takes the visual system
mation regarding visual deficits and treatment alternatives.) away from its normal function. For example, if a hemiplegic
Mental Imagery. As is mentioned in the discussion of man is taught to use vision to tell him the placement of his
neuroplasticity in Chapter 4, and as is discussed further in cane and feet, his need to attend to proprioceptive cues will
the section on somatosensory retraining within this chapter, decrease. When advancing to ambulatory skills such as
having patients visualize the sensory awareness of input crossing the street, the client may be caught in a dilemma.
from the environment has a positive effect on treatment As he is crossing the street, if he attends to the truck coming
outcomes. Similar positive effects have been shown to be rapidly down the road, he will not know where his cane or
effective when having patients practice motor imagery as foot is and thus will become anxious and possibly fall. If, on
part of the treatment protocol.137,433-436 It is known today the other hand, he attends to his foot and cane, he will not
that using mental imagery to retrieve past information or know if the truck is going to hit him. That may increase
experiences does use a variety of pathways within the CNS, emotional tone and make it difficult to move. If normal sen-
depending on the specific task.437 Having some cognitive sory mechanisms could be reintegrated, this client would
understanding of the correlation between cortical deficits in have freedom to respond flexibly to the situation. Thus cau-
specific patients and their visual-spatial problems helps the tion should be exercised to avoid automatic use of this high-
clinician avoid task-specific activities that will lead to fail- level system to compensate for what seem to be depressed
ure while introducing task-specific mental imagery that will or deficit systems.225,226,309,459,460
lead to success.438 Having the patient practice mental imag- Visual input should be used to check or correct errors if
ery of the functional activity practiced during a therapeutic other systems are not available. Movement should be pro-
session can be an excellent way to empower patients to prac- grammed in a feed-forward mode unless change is indi-
tice when they cannot perform the activity itself indepen- cated. Vision often recognizes the need for that change. If a
dently, without extreme effort and abnormal movement client is taught a motor strategy in which vision is used as
strategies.155,439 A therapist will know whether the patient feedback to direct each component of the pattern, the pattern
has mentally practiced the movement strategies by the itself will generally be inefficient and disorganized and will
carryover within the next session. The neurophysiological lack the automatic nature of feed-forward procedural motor
reason for this perceived contradiction may lie in plans. If the client is too anxious to practice the procedure
neuroanatomy, site of the lesion, specificity of the individual physically without overusing vision, then visual mental
client.156,439,440 Although imagery usually insinuates visual- practice can be introduced.
ization, there are also other forms of imagery that can be Internal Visual Processing: “Visualization Techniques.”
used as part of intervention.155,437,439,441-443 Refer to the A previous section discussed mental imagery as a substitute
music therapy section in Chapter 39 for information on in the presence of a sensory deficit or as a practice method for
mental imagery. when a patient cannot perform a motor task. The use of visu-
One extension of mental imagery that came into common alization of some aspect of bodily function goes far beyond
usage in the 1990s as a result of videogame popularity was just mental practice. Visualization has been and continues to
“virtual reality.” Over the last two decades the interface be used in many forms of therapy.459 In a randomized con-
between virtual reality and medical education has included trolled study that looked at normal bone healing versus the
the use of a virtual environment to teach surgeons fine motor use of a specific type of yoga that involves breath control,
skill without having them practice on a live subject.444 An chanting, and visualization as an adjunct treatment, the indi-
inevitable link has currently been identified between virtual viduals who practiced this yoga-based approach had acceler-
reality and motor rehabilitation.445-448 Today the literature ated fracture healing.461 It has been shown that individuals can
certainly reflects the potential advantage virtual reality may modulate their immune responses and that others can change
have with regard to not only motor learning but also the use that response through visualization.460,462 Smith and col-
of these environments as an adjunct to therapy in individuals leagues460 showed that individuals could exercise through
with CNS damage.449-455 The future realization of the poten- their thoughts and visualization various degrees of control
tial of this type of augmented intervention will be up to over what had been thought to be mindless internal processes.
visionary thinkers who “push the envelope” of traditional These concepts have been used therapeutically but usually
therapeutic interventions. when the client is resting or totally relaxed.225,226,309
Compensatory Treatment Alternatives with Use of the More recently, technology in neuroscience has allowed
Visual System. The visual system can be used effectively as for the measure of tissue metabolism (positron emission
a compensatory input system if the sensory component of transaxial tomography [PET])463 and changes in blood flow
the tactile, proprioceptive, or vestibular system has been lost (fMRI) while the brain is engaged in functional mental
or severely damaged. The procedure for using vision in a tasks.464,465 All areas of the brain except the cerebellum
compensatory manner should not be attempted until the appear to be activated during intense goal-directed mental
clinician is convinced the primary systems will not regain imagery. Given that the task is not motorically executed,
needed input for normal processing. Although vision can errors in rhythm and accuracy are not made, and thus the
direct and control many aspects of a movement, it is not cerebellum is not recruited for correction. This suggests that
extremely efficient and seems to take a tremendous amount mental imagery can be used to restore a function that might
have been lost as the result of a stroke or other type of injury

because the individual may be able to use kinesthetic mem-
ory to facilitate learning even if current kinesthetic recogni-
tion is impaired.466 Visual imagination has the benefit of
allowing correct task performance when physical limitations
may prevent normal task completion. This could prevent
abnormal learning (e.g., like that developing from abnormal
posturing in gait in a stroke patient who lacks the voluntary
control to ambulate and integrate a primitive synergy). For
additional information, see the section on somatosensory
discrimination.
Today these concepts can be integrated during active
treatment in a variety of ways. Before a client begins to
initiate a plan of movement, the therapist could ask the
client to close the eyes and imagine the movement and Figure 9-4 n Eye gaze: correlation with lobe and hemispheric
what it felt like in that functional activity before the CNS processing based on right-handed individuals. (Modified from a
injury. In this way, the patient is using prior memory and handout from New Learning Pathways, Denver, 1988. Illustrations
visualization to access the motor systems and hopefully by Ben Burton.)
initiate better motor plans. Similarly, if during a move-
ment plan the state of the motor generators builds to such
a level that the client is becoming dysfunctional, the
therapist can stop the movement; ask the client to visual- be to access the motor cortex to gain better motor function.
ize a calm, quiet place; and then continue with the If the client is asked to visualize the movement before and
movement pattern when the tone is reduced or extraneous during the activity, the head often comes to a posturally cor-
patterns cease.467 The client can be asked to practice men- rect position as the eyes gaze upward toward the occipital
tal imagery of the task until she or he can accomplish it lobe and the body automatically orients to vertical. If the
normally and then finally carry it over to the real environ- client is asked to walk while visualizing the movement,
ment.468,469 For example, a client may have practiced again the result may be a more upright, posturally efficient
transferring during an intervention session in which the pattern. Once the program is set and practice scheduling
therapist, using augmented treatment, kept the patient begun, the patient may no longer need to look down and into
within a biomechanical window or limits of stability. Dur- the frontal lobe. Thus in this case the client not only learned
ing the interval between sessions, the patient is asked to the procedure but also avoided practicing and learning a
visualize performing transfers initially from the same sur- posturally incorrect ambulation strategy.
face practiced and later to other surfaces at least a couple Combined Multisensory Approaches. Although all
of times an hour. At the follow-up session, the therapist techniques have the potential to be multisensory, the specific
will often be able to tell if the patient has done the visual- mode of entry may focus on one sensory system, as already
ization. If the patient did practice, there is often carryover described, or it may target two or more input modalities
into the skill performance. If the patient forgot to practice, along with automatic motor programming. As stated before,
often the skill has reverted back to the initial level of Table 9-5 categorizes a variety of treatment techniques that
learning, with little carryover from the last intervention. are clearly multisensory. The therapist, analyzing how
Another way to use the visual system to access the the summated effect of the combined input and automatic
processing strategies of the client is to observe eye gaze. responses influences client performance, gains direction in
Neurolinguistic theory postulates that the eyes gaze in the anticipating treatment outcomes in terms of the problem-
direction of brain processing.264,468 Figure 9-4 illustrates the solving process. Because the potential combinations of
eye gaze direction along with the suggested processing multisensory input classification are enormous, only a few
activity. For example, a client who needs to access and pro- examples of combinations are included in the text to illus-
cess motor plans through the frontal lobe will look down. A trate the process a clinician might use when classifying a
client who needs to visually construct an idea of something new technique or a new approach to intervention. When
new will look up and to the right. Various cortical lobes and clinicians select augmented treatment interventions to help a
hemispheres serve specific global processing functions. client as part of somatosensory retraining or functional
There are many ways to apply and interpret this theory. By retraining or to establish a procedural program, the basic
observing the patient’s eye gaze, the therapist can determine science understanding behind the clinical decision helps
whether processing is conducted in what would be believed develop questions for future research, determine a prognosis
to be the appropriate areas. Even more clinically relevant is regarding outcomes, and rationally explain why or why not
observing where the eyes are gazing before and during suc- an intervention was effective. Clinical decisions must ulti-
cessful functional activities. It may be that the area once mately be made regarding which techniques or component
used in processing is no longer available to do the function. of an approach should be eliminated first as the patient pro-
If gazing to the right and down always leads to motor gresses. These decisions must be based on understanding
success, then the therapist can empower the patient to look and integration of neurophysiological mechanisms, learning
down and right before dressing or transferring. Similarly, if environments, concepts of motor learning and control, and
a patient always looks down at the feet during ambulation, what motor impairment or body system problems are affect-
the reason may not be “to look at the feet” but instead may ing functional performance and on the client’s and family’s
needs, motivations, and goals. A simple rule a therapist pattern generators responsible for the extensor motor neu-
might follow would be to take away the least natural tech- rons controlling the wrist and finger musculature. Because
nique first. That technique would be the most artificial or the tone is felt in the client’s extensors and thus induces
contrived. An example using only one sensory system might relaxation of the hypertonic flexors, the therapist can more
help to clarify this point. For example, a therapist might easily open the client’s hand. As the client obtains volitional
assist a client with elbow flexion during a feeding pattern by control, some resistance can be added by the therapist to
(1) vibrating the biceps, (2) quickly tapping the biceps, or further facilitate wrist and finger extension. A hemiplegic
(3) quickly stretching the biceps a little beyond midrange by client can also be taught to use this combined approach to
using gravity. The first option would be the least natural and open the affected hand and give it increased range. This
obviously the least socially acceptable at a dinner party. The technique is a noninvasive, relaxing approach to opening the
third option is the most natural and closest to what might hand stuck in wrist and finger flexion hypertonicity. The
occur in the real environment in which the client will need technique itself also seems to trigger spinal generator pat-
to function. Remember, these contrived techniques are used terns that dampen the existing neuron network. It does not
to assist clients who cannot control or perform the motor teach the patient anything unless that individual begins to
programs or functional activities without assistance or who assist or take over control of the extensor pattern. This usu-
need assistance in learning to modulate motor control for ally occurs first when the therapist feels the flexors relax
greater functional adaptability. If the therapist added verbal while the patient is trying to extend the wrist and fingers
feedback or music as well as asking the patient to visually even if no active extension is palpated. Encouraging the
look at the target, the example would become multisensory. patient at this time, confirming that he or she is thinking cor-
Within the following section are examples of combined rectly, and urging him or her to continue doing it provide
multisensory approaches that might be used to augment important motivation for continued practice.
sensory feedback to obtain a better environment for regain- Withdrawal with Resistance. A therapist could com-
ing functional control. bine the technique of eliciting a withdrawal with resistance
Sweep Tapping. Sweep tapping is usually used to open to the withdrawal pattern. This can be an effective way to
a hypertonic flexor-biased hand. Many isolated techniques, release hypertonicity, especially in the lower extremities.
such as sweep tapping111 or rolling,8 would be considered The withdrawal can be elicited by a thumbnail, a sharp
primarily proprioceptive-tactile in sensory origin. During instrument, a piece of ice, or any adequate light-touch
sweep tapping the clinician first uses a light-touch sweep stimulus to the sole of the foot. As soon as the flexor with-
pattern over the back of the fingers of one of the hands. This drawal is initiated, the therapist must resist the entire
stimulus is applied quickly over the dermatome area that pattern. Once the resistance is applied, the input neuron
relates to muscles the client is being asked to contract. network changes and the flexor pattern is maintained through
Second, the therapist applies some quick tapping over the the proprioceptive input caused by resistance to the move-
muscle belly of the hypotonic muscle. The first technique is ment pattern. The one difficulty with this technique is the
tactile and believed to stimulate the reflex mechanism within application of resistance. The withdrawal pattern directly
the cord to heighten motor generators and increase the affects alpha motor neurons innervating those muscles
potential for muscle contraction of the hypotonic muscle or responding in the flexor pattern and simultaneously sup-
to dampen the hypertonic flexors. The second aspect, tap- presses alpha motor neurons going to the antagonistic mus-
ping, is a proprioceptive stimulus used to facilitate afferent cles. If the antagonistic muscles are hypertonic, then ini-
activity within the muscle spindle of the extensors, thus tially the hypertonicity is dampened within the alpha motor
further enhancing the client’s potential for muscle contrac- neurons’ neuronal pool. Because of the pattern itself, as
tion. At the same time the client will be asked to voluntarily soon as the flexor response begins, a high-intensity quick
activate the extensor motor system, which then automati- stretch is applied to the extensor muscles. If resistance is not
cally augments tactile, proprioceptive, and auditory input applied to the flexors to maintain inhibition over the antago-
with functional control. nistic muscles, the extensors will respond to the stretch. The
Rolling of the Hand. Before Brunnstrom’s rolling pat- client will quickly return to the predisposed hypertonic pat-
tern is implemented, the client’s upper extremity is placed tern and may even exhibit an increase in abnormal tone. This
above 90 degrees to elicit a Souque’s sign. This decreases extensor response is a complex reaction within the spinal
abnormal, excessive tone in the arm, wrist, and hand.8 This generators. The therapist should instruct the patient if ap-
phenomenon may well be a proprioceptive reaction of joints propriate to assist with the flexor pattern to recruit other
and muscle. The rolling technique consists of two alternat- components of the motor system to enhance the system’s
ing stimulus patterns. The wrist and fingers are placed on modulation over the spinal generators. This can be a way to
extensor stretch. The ulnar side of the volar component of generate the early component of rolling when leading from
the hand is the stimulus target. A light-touch sweeping the lower extremity and can get the patient out of an extreme
pattern is applied to the hypothenar aspect, which has the extensor pattern in the supine position.
potential to elicit an automatic opening of the hand begin- Touch Bombardment. Another example of a proprioceptive-
ning with the fifth digit.8 Immediately after the light touch, tactile treatment technique is modification of a hypersensi-
a quick stretch is applied to the wrist and finger extensors. tive touch system through a touch-bombardment approach.
These two techniques are applied quickly and repeatedly, The goal of this approach is to bombard the tactile system
thus giving the visual impression that the therapist is rolling with continuous input to elicit light-touch sensory adapta-
his or her hand over the ulnar aspect of the dorsum of the tion or desensitization. Deep pressure is applied simultane-
client’s hand. In reality, tactile and proprioceptive stimuli ously to facilitate proprioceptive input and conscious aware-
are being effectively combined to facilitate the central ness. Proprioceptive discrimination and tactile-pressure
sensitivity are thought to be critical for high-level tactile to protectively withdraw is an important process within the
discrimination and stereognosis. A hypersensitive light- CNS if normal stereognosis is to develop.
touch system elicits a protective, altering, withdrawal pat- Taping. Taping procedures normally used in peripheral
tern that prevents development of this discriminatory system orthopedic muscle imbalances and pain have the same
and the integrated use of these systems in higher thought. potential for patients with neurological problems. This adap-
This method of treatment can be implemented by having an tation would be a modification of both splinting and slings.
individual dig in sand or rice. The continuous pressure Research has been done to demonstrate efficacy of taping to
forces adaptation of the touch system, and the resistance and offset peripheral instability in individuals with neurological
deep pressure enhance the proprioceptive-discriminatory system impairments.282,470-474 The concepts and ideas remain
touch system by a complex adaptation process that most that taping has implications when treating individuals with
likely affects all areas involved in light and discriminatory neurological problems. Taping hypotonic muscle groups
touch, as well as the complex interaction of all motor system into a shortened range should effectively reduce the
components. Whereas sand is often used in the clinic or mechanical pull of gravity on both the muscle groups and
outside, rice can be used inside and vacuumed easily joints and prevent the CNS from developing the need for
whether in the clinic or in a patient’s home. compensatory stabilization or hypertonicity. If hypertonicity
Pool therapy can be used effectively for the same pur- is the result of peripheral instability, then taping a hyper-
pose, with the added advantage of neutral warmth, as long tonic muscle into its shortened range should stabilize the
as the temperature is in the neutral warmth parameters. peripheral system and eliminate the need for the CNS to
Heat increases the sensitivity of light touch, whereas cold create the hypertonic pattern. On the other hand, taping can
initially heightens the nervous system. In time cold can sup- also be used to heighten information about proprioception
press the state of the motor pool (refer to the section on and joint position, providing feedback to avoid hyperexten-
cold). Any client perceiving touch as noxious, dangerous, sion or hypermobility of a joint. This is especially true when
and even life-threatening will not greatly benefit from any there is an imbalance of intrinsics and extrinsics in the hand.
therapeutic session in which touch is a component. Touch Oral-Motor Interventions. There are more research
includes contacts such as touching the floor with a foot, articles available on specific oral-motor dysfunctions in
reaching out and touching the parallel bar railings, and patients with neurological problems475-480 than on interven-
touching the mat. The client may not respond with verbal tion. These are studies using fMRI of the CNS during oral-
clues such as “Don’t touch me” or “When I touch the floor motor activity, but the transition to intervention again is
it hurts” but will often respond with increased tone, emo- limited.481,482 Systematic reviews of potential oral-motor
tional or attitude changes, and avoidance responses. Never- interventions are even fewer.483
theless, this treatment approach has application in many When dealing with oral-motor intervention, the complex-
areas of intervention with clients having neurological defi- ity of combined proprioceptive-tactile input becomes
cits. As an adjunct to this method, a clinician should cau- enhanced by adding another sensory input, such as taste.
tiously apply light touch when in contact with the client. Implementation of one of a variety of feeding techniques
Deep pressure or a firm hold should elicit a more desirable clearly identifies the complexity of the total input system.
response for the client even if the light-touch system is When taste is used, smell cannot be eliminated as a potential
functional.212,320 The use of Gore-Tex material for clothing input, nor can vision if the client visually addresses the food.
can greatly enhance the client’s ability to tolerate the exter- The following explanation of feeding techniques is included
nal world, where light-touch encounters cannot be avoided. to encourage the reader to analyze the sensory input, pro-
Similarly, socks can decrease the hyperactive tactile system cessing, and motor response patterns necessary to accom-
in the foot and may allow the patient to stand or transfer plish this ADL task. The complexity of the interaction of all
without the feeling that he is standing on pins or that it is a the various systems within the CNS is mind-boggling, but if
noxious stimulus. the motor response is functional, effortless, and acceptable
The therapist may also consider systematic desensitiza- to the client and the environment, then the adaptation should
tion as a strategy to integrate the touch system. By allowing be facilitated after attended repetitive behaviors.
patients to apply the stimuli to themselves, they can grade Several feeding techniques have been developed in the
the amount that they can tolerate. In this respect they are past by master clinicians such as Mueller,301 Farber,111
empowered to control their own environment. They can Rood,25 and Huss.296 These techniques were not easily
practice adaptation in many situations. When the environ- mastered or understood through reading alone. Compe-
ment seems overwhelming, they have learned techniques to tence in feeding techniques is best achieved through
dampen the input both from within their own systems and by empirical experience under the guidance of a skilled
controlling the external world. For example, the therapist instructor. Today, some evidence base for implementation
may place a box containing objects of different textures of feeding techniques or related motor activities can be
before the patient and encourage exploration and active found in the literature.52,484,485
participation to learn which textures are acceptable or of- The facial and oral region plays an important role in sur-
fensive. A gradual exposure to the offensive stimuli will vival. Facial stimulation can elicit the rooting reaction. Oral
raise the threshold of the mechanoreceptors in the skin. stimulation facilitates reflexive behaviors, such as sucking
There are also the benefits to the patient of being in control and swallowing. Deeper stimulation to the midline of the
of the stimulus and having awareness of the treatment objec- tongue elicits a gag reflex. These reactions and reflexes are
tives. In addition, vibratory stimuli through a folded towel normal patterns for the neonate. When these reactions and
provide proprioceptive input to desensitize the touch reflexes are depressed or hyperactive, therapeutic interven-
system.188,268,320 Desensitizing the touch system from a need tion is a necessity. Oral facilitation is an important treatment
modality for infants and children with CNS dysfunction. A common problem seen in neurologically impaired
Therapeutic intervention during the early stages of myelina- infants and adults with head trauma is the “hyperactive
tion can be crucial to the development of more normalized tongue,” which is often accompanied by a hyperactive gag
feeding and speech patterns. reflex. To alleviate this problem, the receptors have to be
Similarly, adults with neurological impairment often systematically desensitized. The technique called tongue
have difficulty with oral-motor integration. Problems with walking has met with clinical success.12,41 It entails using an
swallowing, tongue control, and hypersensitive and desensi- instrument such as a swizzle stick or tongue depressor to
tive areas within the oral cavity and also with mouth closure apply firm pressure to the midline of the tongue. The pres-
and chewing are frequently observed in adults with CNS sure is first applied near the tip of the tongue and progres-
damage.475,476 sively “walked back” in small steps. As the instrument
Before basic feeding techniques are implemented, clini- reaches the back of the tongue, the stimulus sets off an
cians need to understand how the CNS and PNS work collab- automatic swallow response. The instrument is withdrawn
oratively with the musculoskeletal system to control and per- the instant the swallow is triggered. This technique is
form these complex oral-motor functional movements.141,486,487 repeated anywhere from five to 30 times a session, depend-
Feeding therapy is preceded by observation and examination. ing on individual responses.
With a pediatric client the therapist should observe breathing Another technique, which might be called deep stroking, is
patterns while the client is feeding to determine whether the used to either elicit or desensitize the gag reflex. Again, an
child can breathe through the nose while sucking on a nipple. instrument such as a swizzle stick is used to apply a light strok-
In addition, the child’s lips should form a tight seal around the ing stimulus to the posterior arc of the mouth. The instrument
nipple. Formal assessments should include functional assess- should lightly stretch the lateral walls of the palatoglossal arch
ments, developmental milestones, and behavioral manifesta- of the uvula. Normally, the palatoglossal muscle elevates the
tions. Medical charts and results from neurological examina- tongue and narrows the fauces (the opening between the
tions should be consulted for baseline data. mouth and the oropharynx). Just behind the palatoglossal arch
Postural mechanisms can influence feeding and speech lies another arch, called the palatopharyngeal arch. Normally,
patterns in clients with neurological dysfunction.28,485,488 A this structure elevates the pharynx, closes off the nasopharynx,
client with a strong extensor pattern may have to be placed and aids in swallowing. Touch pressure to either arc incites the
in the side-lying, flexed position to inhibit the forces of the gag reflex. This touch pressure should be carefully calibrated.
extensor pattern. The ideal pattern for feeding is the flexed A hyperactive gag reflex may be best diminished by prolonged
position, which promotes sucking and oral activity. Basic pressure to the arcs, whereas light, continuous stroking may be
reflexes such as rooting, sucking, swallowing, and bite and more facilitatory in activating a hypoactive gag reflex. A child
gag reactions should be elicited and graded in children and or adult who has been fed by tube for extended periods of time
evaluated in adults. The head needs to be in slight ventro- will often have both hypersensitive reactions in various parts
flexion to pull in the postural stabilization of the neck and of the oral cavity and hyposensitive areas in other locations.
tongue. This is necessary to effectively facilitate programs This problem needs to be assessed to formulate a complete
that provide functional swallowing and control of foods by picture of the client’s difficulties.
the tongue. The use of vibration over the muscles of mastication
The facial region and the mouth have an extraordinary appears to be physiologically valid. Muscle spindles have
arrangement of sensory innervation. Therefore oral tech- been identified in the temporal and masseter muscles.39
niques must be used with utmost care. Anyone who has Selected use of vibration on the muscles of mastication
visited the dentist can attest to the feeling of invasiveness enhances jaw stability and retraction. For protraction to be
when foreign objects are placed in the mouth. With this in facilitated, the mandible is manually pushed in.111
mind, the therapist should begin each treatment session by To promote swallowing, some therapists use manual fin-
moving the autonomic continuum toward the parasympa- ger oscillations in downward strokes along the laryngopha-
thetic end. Activation of the parasympathetic system should ryngeal muscles and follow up with stretch pressure. Ice is
lower blood pressure, decrease heart rate, and, more impor- beneficial as a quick stimulus to the ventral portion of the
tant, increase the activity of the gastrointestinal system. neck or the sternal notch. In addition, chewing ice chips
Neutral warmth, the inverted position, and slow vestibular provides a thermal stimulus to the oral cavity and a proprio-
stimulation should help to promote parasympathetic “load- ceptive stimulus to the jaw and teeth; it also increases saliva-
ing.” Another approach that is applicable to feeding tech- tion for swallowing.
niques is the application of sustained and firm pressure to It is recommended that a therapist work closely with a
the upper lip. An effective inhibitory device is a pacifier with colleague who has experience working with functional feed-
a plastic shield that applies firm pressure on the lips. Perhaps ing before independently beginning to work with clients. The
this is why a pacifier is a “pacifier.” Adults can acquire resis- possible complications that might develop with individuals
tive sucking patterns with a straw and plastic shield and aspirating food cannot be overemphasized.491
achieve the same results. The therapist can quickly realize that feeding as a proprio-
Sometimes children or adults are not cooperative and will ceptive, tactile, and gustatory input modality is extremely
not open their mouths.489,490 Rather than the mouth being complex and often incorporates other sensory systems.
pried open, the jaw is pushed closed and held firmly for a Breaking down the specific approaches into finite techniques
few seconds. On release of the pressure, the jaw reflexively helps the clinician categorize each component and then
relaxes. The receptors in the temporomandibular joint reassemble them into a whole. The job of dividing and reas-
and tooth sockets may be involved in the production of this sembling the parts becomes more and more difficult as the
response. number of input systems enlarges.267
Head and Body Movements in Space. Proprioceptive midline. The head is flexed 35 to 40 degrees to reduce the
and vestibular input is one of the most frequent combination influence of the otoliths and unnecessary extensor tone
techniques used by therapists. In fact, client success in through the lateral vestibulospinal tract. This flexed position
almost all therapeutic tasks depends on the coordinated should be maintained throughout the procedure. The thera-
input of these two sensory modalities. pist places one hand under the client’s occiput and the other
If the head is moving in space and gravity has not been on the forehead. Light compression is applied to the cervical
eliminated from the environment, vestibular and propriocep- vertebrae. This technique activates the deep-joint receptors
tive receptors will be firing to inform the CNS whether it (C1 to C3) and muscle spindles in the neck along with the
should continue its feed-forward pattern or adapt the plan vestibular mechanism, which in turn connects with the cer-
because the environment no longer matches the programmed ebellum and motor nuclei with the brain stem. If the tech-
movement. Depending on the direction of the head motion nique is performed slowly and continuously in a rhythmical
and the way gravity is affecting joints, tendons, and muscles, motion, total-body inhibition will occur. If the pattern is
the specific body response will vary according to the degree irregular and fast, facilitation of the spinal motor generators
of flexibility within the motor system. Bed mobility, trans- will be observed.
fers, mat activities, and gait all incorporate these two mo- Any one of these techniques can be implemented as
dalities. Although all these functional movements can be a viable treatment approach in considering vestibular-
performed without these feedback mechanisms, the CNS proprioceptive stimuli. The selection of an approach or a
cannot adapt effectively to changing environments without method will depend on client preference, client response,
input from these systems. For that reason alone, a thorough the clinician’s application skills, and the need for therapeu-
examination of the integrity of both systems and the effect tic assistance.
of their combined input seems critical if any ADL is to be
used as a treatment goal. Summary of Techniques Incorporating
The use of a large ball or a gymnastic exercise ball can Auditory, Visual, Vestibular, Tactile,
be classified under the category of proprioceptive-vestibular and Proprioceptive Senses
input. Many activities can be initiated over a ball. When a Most therapeutic activities activate five sensory modalities:
child or adult is prone on a ball, righting of the head can auditory, visual, vestibular, tactile, and proprioceptive.
often be elicited by quickly projecting the child forward Auditory and visual inputs are used as the therapist talks to
while the therapist exerts control through the feet, knees, or the client, asks the patient to look, and/or demonstrates the
hips. If the weight of the head is greater than the available various movement or response patterns to be accomplished
power, then a more vertical and less gravitationally demand- during an activity. As the client moves, vestibular, tactile,
ing position can be used. As the head begins to come up, and proprioceptive receptors are firing as inherent feedback
approximation of the neck can be added. Vibration of the systems. Thus the complexity of any activity with respect to
paravertebral muscles might also assist. Rocking forward or analysis of primary input systems is enormous. Even a sed-
bouncing the client who is weight bearing on elbows or entary activity such as card playing requires a certain
extended elbows will facilitate postural weight-bearing pat- amount of proprioception for postural background adapta-
terns through the two identified sensory input systems. Hav- tions, tactile input from supporting body parts and limbs,
ing a client sitting on a therapy ball doing almost any exer- and visual input for perception and cognition. When treating
cise will require vestibular and proprioceptive feedback for an individual with CNS damage, one or a number of sensory
appropriate adaptive responses to be made. The combination systems may not be processing at all or may be processing
seems to play a delicate role in the maintenance of normal incorrectly, which confounds the clinical problem even
righting and the equilibrium response so important in func- farther.
tional independence. Thus when the categorization of techniques—such as a
A trampoline, balance board, or similar apparatus has PNF slow reversal,19 a Brunnstrom marking time,8 marking
the potential to channel a large amount of vestibular- time with music,492 Feldenkrais’s sensory awareness through
proprioceptive input into the client’s CNS. In fact, a trampo- movement,225,226 NDT,31,493 Rood’s mobility on stability,25,28
line is so powerful it can often overstimulate the client and or any mat or ADL activity—is considered, the therapist
cause excitation or arousal in the CNS. must observe the sensory systems being bombarded during
The trampoline and balance board are generally used to the activity. At the same time, if the therapist has determined
increase balance reactions, orient the client to position in which sensory systems are intact, which are suppressed or
space and to verticality, and increase postural tone. A client dysfunctional, and which seem to be registering faulty data,
with poor balance, poor postural tone, or inadequate posi- then altering duration and intensity of the input environment
tion in space and verticality perception may be justifiably through any one system and the combined input through
fearful of these two apparatus because of the rate, intensity, multiple systems creates tremendous flexibility in the clini-
and skill necessary to accomplish the task. Because fear cre- cal learning environment. Understanding this diagnostic
ates tone and that tone may be in conflict with the motor process leads to more accurate prognosis and selection of
response from the client, caution must be exercised with appropriate interventions. Highly gifted therapists seem to
either modality. (See Chapter 22A for further discussion of instinctively go through this diagnostic process. One skill
the interactions of sensory systems and balance.) that seems consistent among master clinicians is a highly
Gentle Shaking. A specific technique of gentle shaking developed sensitivity to the client’s responses, which repre-
can be listed under a combined vestibular, muscle spindle, sents a summation of expression of all systems within the
and tendon category. This technique is performed while the CNS. Simultaneously, they adjust the quantity and duration
client is in a supine position and the head ventroflexed in of combined input to best meet the needs of the client. These
masters release external control and encourage the client to at various levels or areas within the CNS. These synergies or
use normal, inherent monitoring systems to adapt to chang- patterned responses are thought to limit the degrees of free-
ing environments as soon as the client is able to function dom available to programming centers such as the basal
independently, no matter if that is only 5 degrees of motion ganglia and cerebellum11,231 and to enable more control over
or an entire functional pattern made up of many motor pro- the entire body. Having soft-wired, preprogrammed, pat-
grams. Control may begin within a part of the range of a terned responses allows organizing systems to activate entire
functional skill and not necessarily the entire functional ac- sequences of plans and modify any components within the
tivity itself. Therapists must remember that when the control total plan. Modification and adaptation then become the
comes from the clinician and not the patient, it is then goal or function of the motor system in response to both
augmented. The key to carryover will be the client’s empow- internal and external goal-directed activities. The specific
erment over the motor control system and the degree of location of soft-wired programs is open to controversy, as is
practice, self-monitoring, and adaptation available to the the complexity of programming at any level within the CNS.
client. By analyzing and categorizing input and patient Recognizing that these neuronetworks exist with or without
responses, many therapists may develop skills that were external environmental influences would suggest that pat-
initially considered out of reach. Today, clinicians have terns can and will present themselves without an identified
the examination tools to validate changes in their patients’ stimulus. In the past, when an external influence was not
motor behavior (refer to Chapter 8). correlated with an identifiable stereotypical motor pattern, it
was referred to as a synergy. When a stimulus was identifi-
Innate Central Nervous System Programming able, the entire loop was called a reflex. Reflexes and prepro-
The responses of the PNS and CNS to various external grammed, soft-wired neuronetworks such as walking are
stimuli determine the individuality of an organism and its interactive or superimposed on one another to form the
survival potential within the environment. As organisms background combinations for more complex program inter-
become more and more complex, the types of external actions. This superimposed network may encompass spinal
stimuli and the internal mechanisms designed to deal with and supraspinal coactivity, which makes it difficult to spec-
that input also increase in complexity. As the CNS develops ify a level of processing. The exact control mechanisms that
structurally and functionally, inherent control over responses regulate the specific pattern may again be a shared responsi-
to certain common environmental stimuli seems to be mani- bility throughout the nervous system, thus providing the
fested. Different areas of the motor system play different plasticity observed when disease, trauma, or environmental
roles in the regulation of motor output. No area is dominant circumstances force adaptation of existing plans, as dis-
over another. Each area is interdependent on both the input cussed in the neuroplasticity section (see Chapter 4).
from the environment and the intrinsic mechanisms and One way to conceptualize this complex neuronetwork is
function of the nervous system. to picture a telephone system linking your home to any other
As mentioned earlier, the PNS is intricately linked to the home in any city in any country on the planet. If the relay
CNS and vice versa. Damage to one could potentially alter between a friend in New York and you in California devel-
the neuropathways, their function, and ultimately behavior ops static, the system may self-correct, relay through
anywhere along the dynamic loops. Nevertheless, although another area, or even route through a nonwired mechanism
researchers today emphasize the dynamic interactions of all such as a satellite. The options are infinite, but priorities for
components,494-501 clinicians have observed for decades dif- efficiency and adaptability exist within both the telephone
ferent motor problems when different areas of the brain are network and the brain. If the wires to your home are cut, the
damaged. Thus, when clients with neurological damage are phone will not ring. If your peripheral nerve is cut or the
discussed, it seems paramount to identify inherent synergy alpha motor neuron damaged, the muscle will not contract.
patterns available to humans, especially if those patterns If the relay centers at one end of your block are short-
become stereotypical and limit the client’s ability to adapt to circuited and not working properly, then your phone and
a changing environment. those of your neighbors may still function, but not in a fluid
The authors do not recommend or discredit the use of any or specific manner. That is, someone may be calling your
stereotypical or patterned response as a treatment procedure. neighbor but both your phone and your neighbor’s phone
Acknowledging the presence and stressing the importance might ring. Spinal involvement can create a similar problem.
of knowing how these motor programs affect clients’ func- The muscles are innervated and the input from the environ-
tional skills are important. Without this knowledge, thera- ment is accurate, but the neuronetwork is faulty. Regulation
pists working with either children or adults with CNS or modulation may be less efficient or controlled, but the
dysfunction limit their understanding of the normal CNS, system will use all available resources to try to respond to
the normal motor control mechanism and its components, internal and external environmental requirements. This rule
and the interactive effect of all systems on the end product: seems consistent throughout the nervous system, and the
a motor response to a behavioral goal. degree of plasticity is tremendous.503
To conceptualize a systems model, the reader must When specific patterned responses are observed, the
replace the hypothesis of a stimulus response–based concept reader must always hold simultaneously the interaction of
of reflexes308 with a theory of neuronetworks that may be all other motor programming options. In this way the thera-
more or less receptive to environmental influences (see pist can easily conceptualize the variations within one
Chapter 4).502 That sensitivity is modulated by a large num- response and the reason why, under different environmental
ber of interconnecting systems throughout the CNS and by and internal constraints, the motor response pattern may
the internal molecular sensitivity of the neurons themselves. show great variations within the same general plan. Simi-
Specific motor patterns seem to be organized or programmed larly, the expected motor response may not be observable,
although it would seem appropriate and anticipated. The The clinician needs to observe whether the specific
clinician must remember that the more complex the action patterned response is (1) triggered by afferent input,
(e.g., rolling compared with dressing compared with playing (2) triggered by volitional intent, or (3) activated without
hockey), the greater the need for integration and coordina- environmental input including position in space or cortical
tion over pattern generators. Similarly, the more complex intent. In the third case, the entire motor system needs to be
the desired action (especially in new learning), the greater evaluated to determine which portion might be modulating
the potential for needed perceptual-cognitive and affective the observable behavior. Differentiating these motor com-
interactions and the greater the potential for gratification and ponents will help in selecting appropriate examination
also for failure. tools, making the movement diagnosis, prognosing, and
Certain patterned responses or neuronetworks might be selecting interventions.
considered more simplistic or protective in function. These
patterns were once thought to be hard-wired spinal reflexes. Holistic Treatment Techniques Based
It is now known that these reflexes, as well as complex on Multisensory Input
pattern generators, exist at the spinal level and that their As already mentioned, a variety of accepted treatment
responses affect brain stem, cerebellar, and cortical actions. methods exist. Each approach focuses on multisensory
These centers simultaneously affect the specifics of the spi- input introduced to the client in controlled and identified
nal neuronetwork responses.129,130,504 With clients who have sequences. These sequences are based on the inherent
low functional control over the spinal or brain stem motor nature of synergistic patterns,5,30 the patterns observed in
networks, identifying existing patterns, optional patterns as humans5,7,249 and lower-order animals,33 or a combination of
a response to environmental demands, and obligatory pat- the two.19,28 Each method focuses on the total client, the
terns not within the control of the client’s intentional reper- specific clinical problems, and alternative treatment
toire of patterns becomes a critical evaluative component approaches available within each established framework.
before prognosing or identifying the most appropriate inter- Certain methods have traditionally emphasized specific neu-
ventions. rological disabilities. Cerebral palsy in children7,23,28,508-510
Recognizing specific patterns and how those patterns and and hemiplegia in adults8,9,21,31,511,512 are the two most
others might affect functional movement or positional pat- frequently identified. In the past two decades, substantial
terns has clinical significance. A child with spastic cerebral clinical attention has been paid to children with learning
palsy, for instance, shows extension and “scissoring” when difficulties.12,35,513-515 Yet the concepts and treatment proce-
the pads of the feet are stimulated. Sometimes the extension dures specific to all the techniques have been applied to al-
pattern is so strong that the child will arch backward. Sus- most every neurological disability seen in the clinical set-
tained positions that oppose pathological patterns are be- ting. This expansion of the use of each method seems to be
lieved to elicit autogenic inhibition. Contraction-relaxation a natural evolution because of the structure and function of
techniques also work on the autogenic inhibition principle.19 the CNS and commonalities in clinical signs manifested by
Just as afferent input can be used to alter tone and elicit brain insult. Literature in occupational and physical therapy
movement, it can also become an obstacle when the thera- management of individuals with various other neurological
pist tries to coordinate complex movement patterns. The problems has also enriched therapists’ identification of
human palmar and plantar grasp patterns are often thought efficacious interventions as well as those that should be
of as reflexive patterns, as seen in a newborn.505-507 A persis- removed from the toolbox.519,516-521
tent grasp pattern is a common occurrence in children and
adults with a CNS insult. This dominant grasp is often rein- Additional Augmented Interventions:
forced by the client’s own fingers and frequently prevents Today’s Focus
functional use of the hand. If a withdrawal pattern is elicited Four augmented therapeutic intervention approaches that
every time a client is touched, the client not only will be have become accepted over the last decade are (1) BWSTT,
unable to explore the environment through the tactile- (2) constraint-induced movement therapy (CIMT), (3) imag-
proprioceptive systems but also will experience arousal by ery (discussed in the section on the visual system) and vir-
the influence of the cutaneous system over the reticular acti- tual reality, and (4) robotic training. Each is discussed as
vating system. Severe agitation could likely be a behavioral a separate intervention philosophy, but the reader must
outcome from such a persistent reflex. remember that these are augmented intervention programs.
As with any treatment procedure, a clinician should Before an individual would be considered functionally inde-
determine whether the technique will help the client obtain pendent, the patient must be able to perform the functional
a higher level of function. The clinician must learn to recog- activity in a natural environment, such as ambulation within
nize not only specific patterns but also what combinations of a home setting or eating using the more involved extremity
responses of pattern generators would look like. If the reader without having the unaffected extremity restrained. A fourth
overlaid the map of the pattern generators for any combina- augmented intervention approach, robotics, will also be
tion of programs, a complex neuronetwork would result. To presented briefly within this chapter in order to illustrate
some it would verify chaos theory, and to others it would how therapists and patients have the capabilities to interface
verify the end result of multiple systems interacting. The with new and sophisticated technology. The reader is also
neuronetwork complexity of multiple input can be over- referred to Chapter 38 for more in-depth detail. One addi-
whelming. Thus a therapist must always be observant of the tional augmented approach, the Accelerated Skill Acquisi-
specific behavioral response and the moment-to-moment tion Program (ASAP), has been described here. This
changes in behavior during a treatment session, even if the approach is currently undergoing, and research is still
specific neuronetwork is not understood. needed to establish efficacy. This approach is impairment
oriented, emphasizes bimanual activities, and focuses on respective studies.136,532,535-537 The following are examples
active, patient-centered collaboration reinforced with self- of potential variables:
management and self-efficacy.522-525 This approach empha- n Walking speeds
sizes attended, repetitive task practice progressing in diffi- n Frequency of training
cult situations and meets the principles for neuroplasticity. n Length of training
Body-Weight–Supported Treadmill Training. Over n Aerobic levels of training
the last decade BWSTT has been accepted within the thera- n Type of unweighting
peutic community as an alternative approach to teaching n Endurance
gait training for individuals with CNS damage and residual n Type and severity of the patient’s neurological
motor dysfunction. Students are introduced to the treatment dysfunction
procedures and potential sequences from total dependence n Presence of hypertonicity
to independence of the patient. Colleagues take continuing n Age of patient
education courses to learn to position and drive the various n Time since injury
motor components of the gait program while using BWSTT. n Level of independence
Both a vertical support (harness) or air-distributed positive n Assistance needed during ambulation
pressure to unweight the body and a treadmill are combined There have been some excellent systematic reviews of
for BWSTT. The treadmill perturbs the feet backward or BWSTT in the literature that help identify many of the rea-
shifts the center of gravity forward, and the ground reaction sons the literature seems so inconsistent.136,538 The research
forces are reduced by the support. The clinical environment indicates that the two populations of individuals who most
unloads the CNS’s need to (1) provide protection from often benefit from use of BWSTT are people with incom-
falling; (2) trigger and control an effective and efficient plete spinal cord injuries and individuals poststroke.
postural system reaction; (3) reflexively drive the power Another problem in BWSTT research is that the harness
stepping reaction necessary to perform upright ambulation; systems can be uncomfortable at 20% to 30% unweight-
(4) control the balance strategy of stepping to prevent falling.539 Thus, as stated, the huge number of possible variables
ing; (5) facilitate rhythmic, symmetrical, bilateral stepping; and functional ways to measure outcomes using BWSTT
and (6) have a cognitive interface with the various motor or other types of training along with BWSTT has led to
programs necessary to run this functional activity. The tread- confusion in the literature.532,534,537,540,541 Even with all the
mill perturbation of the lower limb into extension facilitates confusion regarding these variables, this form of aug-
the transfer of weight to the forefoot. This forward transla- mented intervention seems to show promise as a protocol
tion forces the feet backward and optimizes the stepping for gait training. Future research studies will still need to
reaction forward. If the moving treadmill is not a sufficient determine which patients, their degree of motor involve-
stimulus to trigger a step, this component can be controlled ment, the optimal dosage, the time after insult, the best
by one or two therapists depending on whether it is a unilat- combination of other interactive interventions (e.g., pharma-
eral or bilateral problem. If the patient does not step, has a cological, robotic), the specific type of gait impairments,
delayed stepping response, or steps effectively with only one and where within the gait cycle the clients would most likely
foot, the therapist(s) can help to initiate the desired response benefit from this type of augmented intervention. It is im-
at the patient’s feet. The rate of movement or speed of the portant to continue to obtain evidence to more precisely
treadmill can also be controlled, as well as the length of time define the practice guidelines for BWSTT. As has been
spent on the affected leg. This treadmill strategy may en- shown in the past, new treatment ideas gain popularity and
courage more symmetrical and faster gait speed in patients become standards of practice without the rigor of establish-
after stroke526 and with Parkinson disease527-529 compared ing an evidence-based practice.35,36,42,542 Physical therapy
with standard physical therapy. This control by the therapist and occupational therapy need to establish that evidence as
helps to facilitate a patient’s response even if it is slow or proof of the evolving effectiveness of clinical practice.
inadequate for normal over-ground ambulation. The ques- Constraint-Induced Movement Therapy. CIMT (or
tion remains whether this type of augmented therapeutic CI therapy) is a type of treatment of clients with motor sys-
intervention does create the best environment to empower tem limitations that combines constraint or immobilization
the patient to learn or relearn normal locomotion after a of the unaffected arm with forced use of the affected limb.
neurological insult. A hand mitt or sling is used to constrain the use of the unaf-
The literature is mixed with regard to this question. The fected upper limb while the affected limb is engaged in a
literature supports BWSTT for individuals with incom- forced-use, mass practice meaningful motor task. The treat-
plete spinal injury,520 the elderly with Parkinson dis- ment focus of CIMT is on shaping behavior to improve
ease,521 and some individuals after stroke,138,522 but other functional use of the impaired upper limb.543,544 CIMT is
literature suggests that BWSTT is equivalent to or maybe based on the theory that impairment in hand and arm func-
less effective than over-ground gait training with a tion in clients after a stroke is compounded by learned
PT,533,534 and still other researchers report that there is no nonuse of that affected upper extremity, which leads to a
difference among different forms of ambulation train- physical change in the cortical representation of the upper
ing.534 With the literature so inconsistent, the clinician limb in the primary sensory cortex.545 Learned nonuse
could be confused as to the effectiveness of BWSTT and develops in the early stages after a stroke in humans as the
whether this type of augmented intervention should patient compensates for difficulty using the impaired limb
even be considered. One primary problem with the by increasing reliance on the intact limb. This compensation
research literature is the great variance in training and the has been shown to hinder recovery of function in the
identified variables selected by researchers within their impaired limb.546
CIMT and the learned nonuse theory are based on deaf- Subjects with chronic stroke hemiparesis who have par-
ferentation experiments in monkeys done by Dr. Edward ticipated in CIMT rehabilitation programs have demon-
Taub.547,548 Early primate studies demonstrated that if the strated significant gains in functional use of the stroke-
upper limb was surgically impaired by dorsal rhizotomy to affected upper extremity as measured by the Motor Activity
disrupt afferent input to the sensory cortex, the animal Log,575 significant reductions in motor impairment on the
stopped using the limb for function. Active mobility was upper-extremity motor component of the Fugl-Meyer
restored by immobilizing the intact upper limb for several Test,576 and more efficient task performance as measured by
days while training the animal to use the affected limb.546 the Wolf Motor Function Test.577-581 Fine motor improve-
The first report of CIMT for hemiparesis in humans was by ments have also been measured with use of the Grooved
Ostendorf and Wolf in 1981.549 Since then, investigations Pegboard Test and other dexterity tests.545,546 These
have demonstrated the effectiveness of CIMT with individu- improvements in impairment and function have been shown
als who have residual upper-extremity weakness as the to persist at follow-up evaluations up to 2 years after train-
result of an upper motor neuron lesion.549-559 CIMT has ing.545,559,573,580 Individuals participating in CIMT studies
been shown to be an effective therapy in persons with have demonstrated improvements in the amount of use
chronic stroke who have sufficient residual motor control and quality of movement in the more involved upper
to benefit from the exercises,550-552,557,560-565 in brain-injured extremity and carryover of skills from the clinic to real-
patients,566,567 in children with hemiplegic cerebral palsy,543,568-573 world activities.549-551,572 This functional improvement may
and in patients with Parkinson disease.574 The CI therapy be significant even if the patient has previously participated
approach has also been used successfully for the lower-limb in a conventional rehabilitation program.582
rehabilitation of patients with stroke hemiparesis, incomplete The question of when to begin CIMT after a stroke has
spinal cord injury, and fractured hip.553 Other diverse chronic not yet been definitively answered. CIMT has been applied
disabling conditions, including nonmotor disorders such as to clients with subacute strokes. This early use of CIMT is
phantom limb pain and aphasia, may also benefit from CIMT.553 based on the hypothesis that earlier intervention may pre-
The criteria for the inclusion of subjects in most CIMT vent learned nonuse and may have a greater impact on over-
research studies have focused on voluntary movement abil- all function. Investigators have found no adverse effects of
ity in the involved upper extremity.549,543-560,565 These criteria CIMT in the subacute phase and only slightly greater
included the ability to start from a resting position of fore- improvement in motor function of the affected upper
arm pronation and wrist flexion and actively extend each extremity.583 There is some evidence from animal studies to
metacarpal-phalangeal and interphalangeal joint at least suggest that if CIMT is introduced too early (e.g., 24 hours
10 degrees and extend the wrist at least 20 degrees through poststroke), it may be detrimental and potentially harmful to
a ROM.561 It is estimated that approximately 20% to 25% of humans. It may cause an increase in the size of the cortical
the population of patients with chronic stroke with residual lesion. This is based on studies of “forced overuse” in ani-
motor deficit meet this motor criterion.575 mals.584-587 Kozlowski and colleagues587 found that early
Not all patients with hemiparesis have been found forced overuse of the affected limb within the first 7 days
to benefit from CIMT. It has not been shown to be benefi- after a sensorimotor cortex lesion impeded motor recovery
cial for clients with severe chronic upper-extremity hemi- of the affected limb and enlarged lesion volume. Bland and
plegia after a stroke.576 Attempts to include individuals co-workers584 also forced overuse of the affected forelimb
who did not meet the minimal motor criteria (at least immediately after a focal cortical middle cerebral artery
10 degrees of finger extension and 20 degrees of wrist stroke, which increased the lesion size and impaired motor
extension) have failed to demonstrate significant or lasting recovery. The relative risks and benefits of “acute” CIMT,
functional improvements in the involved upper extremity and its optimal timing, remain to be determined.546
after CIMT.553,576 The neurophysiological mechanisms that are believed to
The criteria associated with successful therapeutic com- underlie the treatment benefit of CIMT include overcoming
ponents of CIMT therapy are (1) restraint of the unaffected learned nonuse and plastic brain reorganization.582,588 Stud-
arm with a mitt, sling, or glove for 90% of waking hours for ies have confirmed that CIMT produces use-dependent
a 2- to 3-week period; and (2) therapeutic sessions with cortical reorganization in humans with stroke-related paresis
physical and occupational therapy in which patients concen- of an upper limb.551,559,588,589 There is some question, how-
trate on intense, repetitive task training of the more affected ever, as to whether the improvements in upper-extremity
upper extremity for 8 hours a day.* motor function after CIMT are a result of the reduction of
Clients typically participate in 6 to 7 hours of therapy a learned nonuse or of overcoming a sense of increased effort
day; in addition, clients must reinforce this training in home during movement.545 Thus, task-specific, goal-oriented
activities and ADLs.546,564,572,575 The therapist-client ratio is training with the affected limb might be similarly beneficial,
typically 1:1, with the therapist present to give tactile and even without the constraint of the less affected side.
verbal feedback and instruction, along with assistance for Neuroimaging studies such as transcranial magnetic
the desired skill training. Clients also typically keep a daily stimulation (TMS), fMRI, and electroencephalography411,545
treatment diary to document the amount and intensity have been used to provide cortical evidence of neuroplastic-
of therapeutic intervention and the amount of time spent ity and cortical changes after CIMT.554,559,572,590 These stud-
wearing the mitt or sling each day for the duration of the ies have validated that massed practice of CIMT produces a
intervention.572 massive use-dependent cortical reorganization. This change
increases the area in which the cortex is involved during
voluntary movements of an affected limb, even in patients
*References 550, 551, 554, 555, 557, 558, 560, 565, 573, 574. with chronic stroke.546,591
The application of CIMT to real-life clinical environ- ago and currently demonstrates the following attributes
ments presents some challenges, including the time and regarding head control:
physical demands on therapists, the cost to the patient, and n Mild extensor hypertonicity is present in the supine
the resources required during rehabilitation. This limits its position, and Timothy is unable to flex and rotate his
cost-effectiveness and overall effect.546 Many patients in the head off the mat.
acute rehabilitation setting do not qualify for CIMT on the n In prone position, extensor hypertonicity is absent and
basis of limited motor function.546 CIMT, by its nature, can hypotonicity prevails. The client is able to briefly bob
prove to be difficult, frustrating, and intense, and progress his head off the mat in a hyperextension pattern. Mild
can be slow. It will create beneficial effects only if all par- tonal shifts occur to either side when the head
ticipants put in the time and effort to make it successful.572 is turned and when it is symmetrically flexed or
Many subjects who have been presented with the opportu- extended.
nity to participate in CIMT programs and studies have n Timothy is unable to roll or perform any functional
refused because of the intense practice schedule and the activity in the horizontal plane.
necessity of the restrictive device.592 Therapists have also n When placed in a long sitting position, he is unable to
voiced concerns about patient adherence and safety.592 hold the position or sit with flexed hips and extended
Although it has been shown to be effective in laboratory knees. His head remains in total flexion with his chin
research, CIMT may have limited practicality in some on his chest.
clinical environments.592 n When placed in a short sitting position on a mat table,
The future success of CIMT will depend on its ability to he is unable to hold the position. General hypotonicity
be modified according to disease factors, economic consid- prevails, although slightly more flexion is palpable.
erations, limitations of the practice setting, and the cognitive His head remains flexed. When asked to pick up
and physical status of the patient. Less intense practice his head, he extends into a hyperextension pattern
schedule models590,593,594 and combining CIMT with phar- followed by extensor relaxation into flexion.
macological interventions or robotic assistance may help n He is unable to hold the head in a neutral postural
increase its effectiveness and decrease costs without sacri- coactivation pattern in a vertical position.
ficing the benefits.546,595 Studies are now underway to deter- n Timothy does not mind being touched and responds
mine if massed task-specific practice without constraint can well to handling techniques.
be equally beneficial.596,597 Patient satisfaction, overall cost, From the analysis of these clinical signs, the following
and the impact on quality of life are other areas that require clinical interpretations are presented:
further evaluation.598 1. In the horizontal position, Timothy has persistence of
Robotics, Gaming, and Virtual Reality (See a motor program that is enhanced by the spatial posi-
Chapter 38). The most recent augmented intervention tion and its influence on the vestibular system. The
procedures involve the use of technology to regain con- result might be considered persistence of a tonic laby-
trol over functional movement and are the third and rinthine reflex (TLR). In this client the dominant
fourth approaches mentioned in the first sentence in this synergic pattern is extension. While he is supine,
section. The use of robotics,599-602 virtual reality,603-606 extension prevails. While he is prone, extension is
and gaming607-610 in the clinical environment continues to inhibited, although flexion tone is not dominant.
gain popularity as such technology continues to be more Because of the persistence of hyperactivity among the
affordable, and their applications are becoming more extensor motor generators, the ability to initiate roll-
widespread. A thorough discussion of these technologies ing using a neck-righting pattern is prevented. The
can be found in Chapter 38. presence of a mild, asymmetrical tonic neck reflex to
Summary of Augmented Intervention Strategies. both sides and a symmetrical tonic neck reflex has
As with many interventions, the therapist may need to start been noted. Because of his instability and low tone,
with augmented approaches to reduce impairments and/or Timothy seems to be using these stereotypical pat-
gain functional movement in a controlled environment. As terns volitionally to assist in gaining some control
the patient demonstrates improvement in this narrow win- over his motor patterns. In prone position, Timothy
dow of movement or function, the clinician could then in- has the ability to move into a neck extension or optic
crease the challenge with the goal of optimizing functional and labyrinthine righting (OLR) pattern but is unable
performance and improving quality of life. A summary of to hold it. Thus movement and range are present but
the augmented intervention strategies that facilitate neuro- postural holding is missing.
plasticity can be found in Box 9-2. 2. As a result of ventroflexion of the head in sitting, the
Case Examples: Using Augmented Intervention vestibular apparatus is placed in a position similar to
Strategies to Optimize Functional Performance that when prone. In a like manner, the total patterns
Case Study 1: Client with Lack of Head Control. remain fairly consistent. The increase in flexor tone
There is a potential for lack of head control in young, devel- may result from the positioning of hip and knee flex-
opmentally delayed children or in individuals who have ion and kyphosis of the back. The inability to flex the
sustained a severe injury to the CNS. For that reason it is a hips with knee extension suggests that total tonal pat-
common clinical problem. Furthermore, because of the terns or synergies are dominant. The client is unable
importance of head and neck control, virtually all functional to break out of those dominant patterns. Dominant
activities are affected by its absence. OLR is not present.
The client is Timothy, a 16-year-old adolescent male with 3. When asked, Timothy carries out the command to the
a closed-head injury. He had a lesion in his CNS 3 months best of his motor ability. This suggests the presence of
BOX 9-2 n SUMMARY OF INTERVENTION STRATEGIES TO FACILITATE NEUROPLASTICITY
There are many different intervention strategies to use when working with patients with neurological problems. These interventions
need to be matched to the needs of the individual patient and be consistent with the patient’s goals and objectives. All the interven-
tion strategies should be goal directed and repeated with attention to both the input mechanisms (motivation, sensory) and the
output mechanisms (movement). The input and output mechanisms are multifactorial, and they also involve all components of the
sensory, emotional, sensorimotor, and motor systems. Although evidence is increasing about the benefit of learning-based activi-
ties, research is still needed to help define more precisely when intervention should occur, how intense the intervention should be,
how much repetition is needed, how long the learning-based activities need to be continued and spaced, how specific the training
needs to be, how quickly behaviors can be progressed and the magnitude of gradation needed, how to keep patients interested,
motivated, and compliant in learning, and the magnitude of interference in learning relative to depression, stress, and loss of
self-esteem. The intervention strategies can be broadly classified as follows:
1. General body responses leading to quieting of the nervous system8,296
a. Slow rocking in a rocking chair or hammock.
b. Slow anterior-posterior, horizontal, or vertical movements (chair, hassock, mesh net, swing, ball bolster, riding in a
carriage, glider chair).
c. Rotating equipment such as a bed, chair, stool, hammock, or therapeutic or gymnastic ball (e.g., rhythmical bouncing).
d. Slow linear, undulating movements, such as in a carriage, stroller, wheelchair, or wagon.
e. Wrapping up tightly before rocking (e.g., roll self in sheet; put both arms inside tight tee shirt).
f. Listening to quiet music or natural environmental sounds (e.g., waves).
g. Repeating activities listed above first with eyes open and then closed.
2. Techniques to heighten postural righting reactions141
a. Rapid or unexpected anterior-posterior or angular acceleration.
i. Scooter board: pulled or projected down inclines.
ii. Prone over ball: rapid acceleration forward.
iii. Platform or mesh net: prone.
iv. Slides.
v. Any proprioceptive input that heightens postural extensors (e.g., quick stretch, tapping, resistance, vibration, joint
compression). Remember to use the most natural first, such as quick stretch versus vibration.
b. Rapid anterior-posterior motion in prone position, weight-bearing patterns such as on elbows or extended elbows while
rocking and crawling.
c. Weight-shifting in kneeling, half-kneel, or standing positions (first in vertical and then off vertical within limits of
stability by an activity itself [reaching]).
d. Do activities with eyes closed.
e. Create dual-task activities such as walking and talking, stepping over obstacles while on unstable surfaces, reading
while maintaining balance in a confusing environment.
f. Challenge balance in distracting environments (e.g., moving surround, multisensory stimuli in visual surround).
3. Facilitatory techniques to influence whole-body responses30,111,295
a. Movement patterns in specific sequences.
i. Rolling patterns.
ii. Prop on elbows (prone and side-lying positions) and extend and flex elbows as well as crawling (e.g., side by side,
or linear and angular motion).
iii. Coming to sit (side-lying to sit [using upper trunk and head rotation], prone to four-point position to sit [four-point
position to lower trunk rotation to side sit to sit], adult sit [full flexion leading with head]).
iv. Coming to stand (squat to stand, half-kneel to stand, standing from a chair or stool).
b. Spinning.
i. Mesh net.
ii. Sit and spin toy.
iii. Office chair on universal joint.
c. Any activity that uses acceleration and deceleration of head.
i. Sitting and reaching.
ii. Walking.
iii. Running.
iv. Moving from sit to stand.
v. Doing activities with eyes closed, head still, and then eyes closed, head turning.
d. Performing activities that require attention, memory, and cognitive processing at the same time.
4. Combined facilitatory and inhibitory technique: inverted tonic labyrinthine activities
a. Inverted tonic labyrinthine activities.
i. Semiinverted in-sitting (head between the legs).
ii. Squatting to stand (head below heart).
iii. Thirty degrees to total inverted vertical position beginning in supine.
Continued
BOX 9-2 n SUMMARY OF INTERVENTION STRATEGIES TO FACILITATE NEUROPLASTICITY—cont’d
b. Somatosensory and sensorimotor stimulation (refer to earlier in this chapter).

i. See detailed progressive learning-based sensorimotor training (Appendices 9-B and 9-C).
ii. Proprioceptive stimulation.
(a) Vibration over joints.
(b) Vibration in opposite direction of movement.
(c) Wear weights around ankles or on belt.
(d) Position the limbs and the trunk to match a position visually presented.
(e) Move slowly to the count of a metronome and then change speeds.
(f) Look at pictures and position the body to match the pictures.
c. Auditory discrimination (localization).
5. Techniques to facilitate specific task performance
a. Forced use.
i. Create training activities in which patients must use the affected extremity.
ii. Minimize the need to use the unaffected side.
iii. Use bilateral activities in which both hands and upper extremities are required.
b. Constraint-induced movement therapy (CIMT) (forced use)550-552,591,631,632 emphasizes the repetitive use of an
impaired limb in regular functional activities by restricting the movement of the less affected or unaffected side.
i. The patient is constrained from using the unimpaired limb on a concentrated task basis.
ii. The impaired limb is used on a concentrated basis.
iii. The theory is to reduce motor deficits early in the recovery period (learned disuse).
iv. The assumption is that the nervous system is adaptable and training for recovery should begin as soon as possible.
v. If the good arm is constrained, the patient must use the affected limb.
vi. Set time limits to use the constraint; in one large randomized clinical trial the patients were asked to wear a protective
safety mitt on the less affected upper limb for a goal of 90% of the waking hours for 14 consecutive days.
vii. During constraint, the individual works under supervision on designated functional tasks for 6 hours a day.
viii. The patient is encouraged to try to use the affected limb during waking hours.
ix. The constraint is paired with motor or behavioral objectives.
x. Tasks are practiced and progressed in difficulty or speed.
c. Mass task practice (see Chapter 4).
d. Mental imagery.
e. Mental practice.
f. Body-weight–supported treadmill training (BWSTT)524-527,633
g. Integration of robotics and technology (see Chapter 38)
h. Use of gaming (Wii Fit, Brain Fit)604,607-610
some intact verbal processing, which is translated into until the therapist has had an opportunity to augment
appropriate motor acts. Similarly, when asked to pick up the environment to determine how quickly he will
his head, he does just that, suggesting some perceptual regain control and retain the learning. The initial plan
integrity of body image, body schema, and position in of care is assumed to focus on development of head
space. Knowing where his head is in space and where to control as a preliminary and necessary motor program
reposition it also suggests that some proprioceptive- for all functional daily living activity. The estimated
vestibular input and processing are occurring. time it will take to regain this function will not be iden-
4. Timothy’s enjoyment of being moved in space as re- tified until after the first intervention session.
lated to handling techniques suggests proprioceptive- Movement Diagnosis. The client is unable to function-
vestibular integrity. Similarly, his tactile systems seem ally control his head in any position in space, which limits
to be functioning in a discriminatory manner and independence in all functional activities. Lack of postural
modifying negative responses of withdrawal and coactivation and adequate control over the motor generators
arousal. However, specific tactile perception would has led to imbalances in the tonal characteristics of flexor
need a great deal of further testing. Thus he demon- and extensor patterns with the compensatory development
strates functional strengths in cognition and percep- of stereotypical patterns of movement.
tion, in limbic motivation, in some areas of sensory Goal of Intervention Program. The goal is development
integrity, and in control over available but limited of independent head control, initially in a vertical midline
motor programming. Yet performance on any func- posture with the intent of enlarging that biomechanical
tional test would result in identification of an individ- window to include all positions in space.
ual whose functional limitations prevent him from in- Now that the clinical problem has been analyzed and
dependence in any activity. Prognosis must be guarded the goal of development of head control set, an intervention
sequence or protocol must be established. Timothy lacks flexion and rotation (Figure 9-5, C), the key to eliciting a
head control in all planes and in all patterns of movement. neck-righting, rolling pattern from supine to prone. The total
Thus, flexors and extensors must be facilitated to develop a flexed pattern can also be altered by adding more and more
dynamic coactivation or postural holding pattern of the extension of the extremities. This decreases the external
neck. The categorization scheme can now be of some assis- facilitation to the flexors and demands that Timothy’s CNS
tance. The therapist can ask, “Are there any inherent mecha- take more and more control (internal regulation). Additional
nisms that enhance flexors or extensors in a holding treatment procedures can be extracted from a variety of sen-
pattern?” The optic and labyrinthine righting (OLR) reac- sory categories. To add additional proprioceptive input, any
tion should elicit the desired response. Similarly, the clini- one of those listed techniques might be used. The rotation
cian can ask, “Are there any inherent motor programs that and speed of the rocking pattern affect the vestibular mecha-
would prevent righting of the head to face vertical OLR?” nism. Auditory and visual stimuli can be used effectively. If
The TLR would block or modify the facilitation of OLR. the therapist takes a position slightly below the client’s
Knowing that the TLR is most dominant in horizontal and horizontal eye level, the client (to look at the therapist) will
least dominant (if at all affected) in vertical is of clinical need to look down and flex his head, thus encouraging the
significance. It is also important to know that the OLR is desired pattern. Any type of visual or auditory stimulus that
most frequently tested in a vertical position and seems most directs the client into the desired pattern would be appropri-
active in that position. Awareness that the client is sensitive ate. The therapist must remember that neck flexion is one of
to total patterns (e.g., flexion facilitates flexion or extension the identified goals. Rotation was added to incorporate and
facilitates extension) gives additional treatment clues. set the stage for inherent programming that will lead to roll-
After all this information has been assimilated, the ing, coming to sit, and reaching while sitting. Because the
following treatment could be established. postural extensor component still needs integration, total
For enhancement of neck flexors, the client will be placed head control has not been attained. To facilitate neck exten-
in a totally flexed position in vertical, with the head posi- sion, a procedure similar to the one for flexion can be estab-
tioned in neutral. The client will be rocked backward toward lished. A vertical position, thus eliminating the influence of
supine, allowing gravity to quick stretch the flexors (Figure the TLR, would again be the starting position of choice. For
9-5, A). As soon as the neck flexors are stretched, the head additional visual feedback on the development of flexor
should be tapped forward and then back to vertical but not head control, refer to Chapter 3, Figures 3-15 through 3-18.
beyond. This avoids hyperextension, extreme stretch to the With extension facilitating extension, the client should
proprioceptors, and the horizontal supine position of the be placed in as much extension as possible without eliciting
labyrinths, all of which dampen the flexors and facilitate excessive extensor tone. An inverted labyrinthine position,
the extensors. The quick stretch and position should opti- a kneeling position, or a standing position would be viable
mally facilitate OLR, which should activate the neck flexors. spatial patterns to facilitate OLR of the head and coactiva-
The total flexion of the body similarly facilitates the neck tion of postural extensors. The vestibular system sensory
flexors. Once the neck flexors respond, Timothy can be category can be checked to identify the treatment procedure
rocked farther and farther backward while maintaining the for use with an inverted labyrinthine position. The kneeling
head in vertical or ventroflexion (Figure 9-5, B). Once or standing position places the client in a vertical position
Timothy can be rocked from vertical to horizontal and back with hip and trunk extension. Kneeling rather than standing
to vertical while maintaining good flexor neck control, his is used first because of the influence of the positive support-
CNS has demonstrated inherent control and modification ing reaction in standing and the massive facilitation of
over the stereotypical patterns, such as the TLR in supine total extension. Kneeling avoids total extension while
with respect to its influence over the neck musculature. maintaining a predominant extensor pattern. As a result
This rocking maneuver can be done on diagonals to practice of the gravitational pull of body weight through the joints,
Figure 9-5 n Development of flexor aspect of head control. A, Vertical position: head at midline and midrange (total-body flexion) to
optimally facilitate neck flexors. B, Facilitating symmetrical neck flexion, using position, gravity, and flexor positions. C, Facilitating flexion
and rotation to develop pattern necessary for neck-righting pattern.
approximation to facilitate postural extension is constantly cognitively trying to keep his head up. In this way automatic
maintained. The upper extremities can be placed in shoul- head control is facilitated, and often postural patterns follow.
der abduction and external rotation, which tends to inhibit In a partial kneeling pattern the client can be sequenced to
abnormal upper-extremity flexor tone and facilitate postural on-elbow over a bolster or ball or on a chair. These activities
tone into the shoulder. This extensor tone has the potential should be sequenced from vertical to prone to ensure both
through associated spinal reactions to facilitate neck and total postural programming in prone and optimal integration
trunk extension. The arms can be placed in this position of OLR, as well as to let the client experience control of
over a bolster or ball or by the therapist handling the client various motor strategies in many different environmental
from the rear (Figure 9-6, A). The head should begin again contexts. For more analysis of the development of extensor
in a neutral position. The client is rocked forward (Figure head control, refer to Chapter 3, Figures 3-19, 3-21, and 3-22.
9-6, B) to facilitate OLR of the head and to elicit a quick Once the client can maintain good flexor, extensor, and
stretch to the postural extensors. If the head begins to fall rotational components of head control, the activity should, if
forward, the therapist can tap the client’s forehead immedi- possible, be practiced with the client’s eyes closed. If the
ately after the quick stretch. This tapping action is the client can still maintain head control, labyrinthine righting
reverse tap procedure described under the proprioceptive would be adequate for any functional activity. If the client
stretch receptors category. The tapping is done to passively loses head control, then additional labyrinthine facilitation
move the head back to vertical. would be indicated. If a client uses only vision to right the
A variety of additional procedures can easily be com- head, then any time vision is needed to lead or direct another
bined to summate facilitation to the postural extensors. activity, head control might be lost. Because symmetrical
Tapping, vibration, and approximation through the head to vestibular stimulation plays a key role in activating the neck
the shoulders are only a few of the proprioceptive modali- muscles to hold the head in vertical, it also is a key element
ties. All would be facilitatory. A variety of auditory and leading to the perception of vertical and all the directional
visual stimuli could be used to orient the client to a position activities sequencing out of the concept of verticality. The
in space and thus righting of the head. Techniques listed postural extensor programming for head control needs to be
under the exteroceptive and vestibular systems could also be practiced in a standing position and a sitting position. The
part of the treatment protocol. The therapist would want to client needs to be able to stand quietly without excessive
sequence the client toward prone while the head remained in extension to run both postural and balance programs. Simi-
a vertical postural holding pattern. As the therapist rocks the larly, he needs to be able to sit with hip flexion while
client toward prone again, a rotational component should be coactivating postural extension in the trunk and neck.
added (Figure 9-6, C). The client will extend and rotate to Head control is a complex motor response. A therapist can
counterbalance the movement, thus incorporating the neck- facilitate inherent mechanisms to assist a client in regaining
righting pattern of extension and rotation necessary when function. Simultaneously, multitudinous external input tech-
rolling from prone to supine. Resistance to neck extension niques classified under the various sensory modalities and
with or without rotation is an important element in regaining combined modalities can be used to give the client additional
normal functional control. The client is alert and has some information. Awareness of one technique and the ability to
functional use of the arms and legs. This rocking pattern in categorize it appropriately allow easy identification and
kneeling can be done as a functional activity. The therapist implementation of many additional approaches. The thera-
asks the client to assist in reaching toward an object with pist always needs to remember that the client must practice
one upper extremity. The therapist can guide the client in the the behavior (head control) in a variety of spatial positions
reaching pattern in a forward, sideward, or cross-midline during various functional activities. This practice must be
direction. While reaching, the client can be rocked forward functional and no longer contrived.
to elicit right and equilibrium reactions. In incorporating The reader is referred to Chapter 3 in order to understand
an activity into the treatment of head control, the client not the normal development of head control and how the
only is entertained but also attends to the task rather than nervous system demonstrates motor learning and control.
Figure 9-6 n Development of extensor aspect of head control. A, Vertical position: head midline with long extensor in midrange and
postural extensors in shortened range; body in postural weight-bearing pattern. B, Facilitating symmetrical extension of head, trunk, and hips
while inhibiting abnormal upper-extremity tone. C, Facilitating head and trunk extension and rotation to encourage neck righting pattern;
client reaches for an object, which is then placed on the opposite side.
Case Study 2: Initial Augmented Intervention Transi- patient is actively assisting the therapist with the entire
tioning to Independence in Bed Mobility. Teaching the gait cycle of both legs. By the end of week 3, the patient is
client to roll in bed can be approached in a variety of ways able to walk on the treadmill independently. During the sec-
to accomplish the goal. The entire rolling pattern may be ond week, over-ground ambulation is begun to transfer the
practiced with enough assistance for the client to be able to treadmill learning into a functional activity. By the end of the
accomplish the goal, but also limiting help so that the client fourth week, the patient is independent on noncompliant
must use the maximum amount of power and ROM avail- surfaces. Over the next month the patient is in an outpatient
able within the key movement pattern. environment with the primary goal of independent ambula-
Rolling. The patient is a 73-year-old man, status post– tion on compliant surfaces such as sand, dirt hills, and gravel
ischemic infarct in the frontoparietal cortex with resultant environments.
left hemiplegia, hemisensory deficit, and left homonymous Specific Occupational Therapy Intervention with
hemianopia. The patient demonstrates visual-spatial inatten- Regard to Fly-Fishing. It is determined that the OT will
tion to the left environment. The client must learn to roll work on postural endurance of the trunk and lower extremi-
independently in bed for comfort and function. An example ties while facilitating the right upper extremity to practice
of a treatment session aimed at reaching the goal of indepen- fly-fishing. Initially the training is done in sitting to create a
dent rolling to the right and left may include the following stable environment for the right upper extremity. The arm is
sequence of activities: (1) begin in side-lying on one side; placed over a ball that the patient can roll back and forth as
(2) ask patient to tip back a few degrees and then return to he visualizes fly-fishing. His right hand is placed in a glove
the side-lying position (impairment training within limited that has a wrist support and is fastened to the rod with
ROM); and (3) progressively increase the degree the patient Velcro. The rod is placed in a bucket with a hinge joint that
must roll backward, assisting (augmenting) him as needed. allows for anterior and posterior movement of the rod
By the end of several repetitions the patient may be rolling attached to its base of support. Using this adaptation of the
from supine to side lying and the movement is functional ball, rod brace, and wrist support and glove, the patient is
because he is performing independently. The client will able to mimic one half of the range needed to fly-fish. He so
need to practice many times to relearn the activity before enjoys the activity that his family takes it up to the room to
that activity would be considered functional training within allow him to practice between therapy visits. After a week,
the environment practiced. Rolling on a therapeutic mat the patient is brought to stand, and the apparatus is adjusted
table is not the same as rolling on a soft mattress at home. for height. The ball is still used but placed on an adjustable
There may or may not be carryover. That needs to be identi- bedside table. As normal motor programs begin to be gener-
fied by the therapist and appropriate steps taken to ensure ated within the right upper extremity, modifications in size
that independence in all environments is obtained. of the ball, angle of the wrist and hand, and range allowed
Refer to the video for a demonstration of handling while within the hinge joint are made to allow for error and
working on rolling for bed mobility. self-correction. Within the 3-week period of inpatient reha-
Case Study 3: An Individual post Stroke. A 66-year-old bilitation, the patient becomes able to perform the activity
man after a stroke has mild extensor synergic hypertonicity normally with only the use of the ball for postural support
within the right lower extremity and hypotonicity within the within the shoulder girdle. The apparatus is taken home and
right upper extremity except within the shoulder girdle, which the patient adjusts all components depending on his fatigue
has weak but functional movement patterns. His stroke was level. Within a 2-month period of the patient working at
medically considered mild and his prognosis good in relation home, he goes from a totally augmented intervention
to the potential of the CNS regarding function. It has been program to functionally being able to stand by a river or lake
agreed that the therapeutic goals after physical rehabilitation and fly-fish independently. His endurance for this activity
are to ambulate independently and use the right upper extrem- improves as he continues to practice.
ity to fly-fish, an activity that he loves and has done daily
since he retired. SOMATOSENSORY RETRAINING
In terms of occupational and physical therapy interven- Somatosensory retraining is a multisensory approach to
tion, the patient would be taught to regain independent retraining target-specific skills for patients with movement
functional skills in dressing, feeding, hygiene, transfers, and dysfunction that manifests with measurable levels of sen-
other ADLs. To facilitate the patient’s goal of fly-fishing, his sory impairments. This type of therapy is based on the prin-
family is asked to bring in the rod and reel to augment a real ciples of learning and plasticity and progresses from a
situation with the functional skill he possessed within his strong sensory emphasis to sensorimotor practice to motor
right shoulder girdle. learning. This approach has been used with patients with
Specific Physical Therapy Task Training: Fly-fishing. In various types of hypertonicity resulting from congenital
addition to ADL training, it is decided to use BWSTT as a deficits (see Chapter 15) to degeneration (see Chapters 13,
training tool for his right lower extremity. Manual assistance 17, 19, and 20) and disease (see Chapters 21, 23, 25, and 26).
is used to guide the placement of the right foot into dorsiflex- It has been most commonly used in patients with dystonia
ion at heel strike. The training begins with a 30% weight and chronic pain. This approach combines a variety of the
reduction, and the patient is relaxed into the gait pattern. His strategies summarized in the augmented intervention section
right arm is suspended with the use of a shoulder harness and as well as Box 9-3. The principles for retraining can be
a robotic aid that swings through the arm in a reciprocal pat- found in Appendix 9-A. The progression of specific learn-
tern to the left leg. This intervention is performed twice daily ing-based sensorimotor training is summarized in Appendix
for 3 weeks. During weeks 2 and 3, the patient’s body weight 9-B. Additional ways to enhance sensorimotor training can
support is reduced to 15%. By the end of the second week the be found in Appendix 9-C.
n CONCEPTUAL GUIDELINES FOR CLINICAL DECISION MAKING: HOW TO SELECT

BOX 9-3
TREATMENT OPTIONS FOR PATIENTS WITH NEUROLOGICAL IMPAIRMENTS
After performing examination procedures in which you identify problems with activities and participation, you will then be able
to classify these into clusters or syndromes (i.e., the physical therapy diagnosis). You then need to formulate a prognosis and
determine the intervention options.
In order to determine the best treatment options for a patient with a neurological condition with movement problems, you must
simultaneously consider non–physical therapy–based as well as non–neurological system–based limitations along with the
specific neurological impairments.
Assume the best case scenario (which never exists) in which there are no limitations in health benefits, from cultural beliefs
or family, caused by conflict with other care providers, or in systems other than motor such as cognitive, emotional, vascular,
integumentary, pulmonary, cardiac, and so on.
First, what does the patient want to do compared with what his or her motor system can do? Can you work on improvement of
impairments and function within activities the patient is motivated to do? If so, do it!
Second, without altering the patient’s normal feedback (intrinsic) mechanisms, can he or she perform the functional activity
without causing program adaptations that are so stereotypical that those programs may limit future movement functions and
carryover?
For example: can you create an activity that will do the following without contriving the environment and while still running
flexible, malleable motor programs?
1. Improve range of motion (ROM) or
2. Improve power or
3. Improve coordination or
4. Improve balance or
5. Improve endurance or
6. Any or all of the above
7. Have any similar effect
If so, do it!
If not, you will need to contrive the environment in order to create functional change through treatment intervention.
ASK YOURSELF
1. Where in the activity can you optimize biomechanics, and where are biomechanics deoptimized? If the body was placed at a
better biomechanical advantage:
a. Would the client be able to run and power the program?
b. Would the motor program run more fluidly and procedurally?
c. Would there be greater endurance?
If your answer is yes, then try running the program that way initially and then increase the range and challenge all components
of the program.
2. Throughout the activity, where would the least and greatest power be needed? Does the program run differently at different
points throughout the activity depending on power production? Optimize what power you have, while maintaining fluid,
relaxed program generators. Hypertonicity will often be observed if you ask for more power than the generators can create
in a normal fashion.
3. Look at the program itself.
a. What central nervous system (CNS) components are missing (impairments or functional problems)? These are usually your
neurological diagnoses (physical therapy diagnoses).
b. Can you elicit through treatment intervention corrections of the impairments in any aspect of the program? If so, how?
o Caused by biomechanical advantage
o Caused by musculoskeletal advantage
o Caused by the program advantage itself
Optimize:
o Synergistic advantage
o Balance synergies
o Sensory processing
o And so on
These treatment answers will lay the foundations for your specific intervention strategies.
c. Can you elicit those components in any other movement programs? If so, these are treatment alternatives, although they will
not be task specific and have less immediate carryover.
4. Select movement activities that use existing components procedurally and facilitate or elicit function from impairment
component of subsystems.
5. Prioritize functional activities by identifying daily living needs of the patient, goals of the patient, and functional skill of the
patient. Determine which impairments affect the greatest number of functional activities. Similarly determine which
impairments can be quickly changed in order to gain functional skill. Decide within the limits of the environment which
activities to focus on first.
n CONCEPTUAL GUIDELINES FOR CLINICAL DECISION MAKING: HOW TO SELECT

BOX 9-3
TREATMENT OPTIONS FOR PATIENTS WITH NEUROLOGICAL IMPAIRMENTS—cont’d
For example: Assume the patient has poor balance in sitting and standing. He plans to sit in the lounge chair most of the
day but walk to the toilet when necessary. Although range, power, and postural control would need to be considered, you
might decide to work on standing balance and balance during walking before sitting balance owing to task specificity and
functional need. This is not stating that sitting balance is not important; it is prioritizing the activities according to need. Power,
range, posture, and so on may determine the specific intervention strategies used to work on standing and walking balance.
6. If normal programming cannot be elicited, look at adaptations and determine alternative interventions such as the following:
a. Adaptive equipment: biofeedback, orthotics, canes, and so on
b. Adaptive environments: ramps, rails, lights, changing walkways, changing surfaces (e.g., removing shag carpets)
c. Encouraging stereotypical and inflexible programs
d. Combination of a, b, or c
Make sure that when selection of alternative approaches or adaptations is made, consideration is given to what will be given up by
adapting the environment and CNS. Consider whether that decision is truly cost efficient and the best alternative to meet the needs
and goals of the patient, his or her family, and the physical and cultural environment within which he or she will function.
Neural Mobilization 21 years of age must receive educational experiences in the

Neural mobilization is often needed as an intervention strat- least restrictive environment. This amendment was made to
egy before somatosensory retraining is begun. Often there is the Individuals with Disabilities Educational Act (IDEA)
increased sensitivity in a limb from pain,611 neurovascular in 1997.619 These changes have been mandated within
restrictions, or soft tissue adhesions limiting the ability of the the school systems and have affected therapy environments
peripheral nerve to move through the tissue (see Chapter 18). for clinicians who work in those situations. It is realistic
This sensitivity can increase hypertonicity and further inter- to assume those changes will in time affect all therapy
fere with retraining motor control. In order to address this, environments.
it is important to quiet the nervous system and then gently As the World Health Organization has moved toward an
mobilize the neural tissue. One way to quiet the nervous individual-friendly focus and thus a focus on body system
system is by “swaddling.” This is often used in newborns strengths as well as impairments, participation in life, and its
to quiet their nervous system. For an older child or an adult, quality, the term patient may also need to be changed to
the patient is wrapped similar to the way a baby would be; participant. As can be seen in Chapter 8, examination tools
then gentle rocking in a rocking chair or a swing is added. have been included that deal with quality of life and not just
Patients can do this themselves by putting on a t-shirt with functional outcomes from therapy. Therapists, if they have
the arms tight to the trunk and then wrapping even further not done so already, are going to have to learn to deal with
with a blanket. This technique can be used periodically individuals coming to them for assistance as a partner in the
on days that the nervous system appears to be responding process and not a patient who receives services. Change will
primarily to adrenaline rather than purposeful heightened come. That is one of the exciting aspects of being an OT or
activity. There are a variety of ways to mobilize the PNS. PT in the coming decades.
Detailed examples of this can be found in textbooks on
the hand.612 CONCLUSION
There are treatment techniques that are universally applied to
NATURAL ENVIRONMENTS AND QUALITY the very young and the very old. As discussed in Chapter 4,
OF LIFE the CNS is in a constant state of change throughout life. The
Research has already been identified in the discussions of brain is unique to each individual. Each brain has idiosyn-
the various sensory input systems that recognizes that crasies but also has an enormous number of predictable
changes in external sensory input such as decreasing sound, responses. These factors affect the success or failure of a
light, tactile contact, or color of mats can change the pro- client-therapist interaction. In Box 9-3 the reader will find
cessing of CNS of the clients. The present and future guidelines that may assist in determining the type of inter-
research will recommend that therapists apply changes that ventions (functional, impairment, augmented, or somatosen-
affect not only the patient’s inherent sensory systems, but sory training) that will best match the patient’s functional
also the environments within which the therapy is done. movement capability. In answering the questions presented,
Therapists are going to have to adapt to change. At this time the therapist will gain a better idea of which examination
it is unrealistic to think that acute management of patients tools will best help objectively measure the progress of the
after a neurological insult will occur anywhere but in a large patient toward that patient’s specific goals. From that thor-
medical institution, but treatment needs after that acute stage ough evaluation process (see Chapter 8), the therapist must
may better be served in a more natural environment of the decide which treatment is appropriate and the most efficient
individual needing service.613-618 Not only has this conclu- course of intervention on the basis of the goals of the patient
sion been accepted conceptually in postacute pediatric and family, the movement diagnosis, the prognosis, the
settings, but federal law has ordered that individuals up to resources available, and the skills of the therapist. Once a
decision is made regarding whether the interventions or any combination of these four will provide the client
should be based on compensation, substitution, habitua- with the most environmentally effective, cost-efficient,
tion, neural adaptation, or a combination of the four, the and quickest map to functional independence or maximal
team must select the best options available given all the quality of life. How each therapist combines the interven-
resources. The options include functional retraining, im- tions with the client’s specific needs will vary according
pairment training, augmented and contrived interventions, to education, belief, skill, and openness to learning from
and somatosensory reintegration. No matter the specifics the total environment itself. Learning should lead to fur-
of the intervention selection, the therapist must cognitively ther learning. Answers to unknowns will be found, with
organize intervention options in a sequential process, be new unknowns coming to consciousness. The brain is still
willing to change direction or options as the patient more mystery than not, so for most OTs and PTs begin-
changes, and develop a greater clinical repertoire of inter- ning or ending their practice, the adventure has just begun.
vention strategies. Enjoy the experience.
When specific augmented interventions are needed, the
therapist must select specific treatments according to References
the needs of the client, the time available for therapy, the To enhance this text and add value for the reader, all refer-
level and extent of the functional involvement, the motiva- ences are included on the companion Evolve site that
tion of the client and family, the creativity of the therapist, accompanies this textbook. This online service will, when
and, of course, the existing pathology, whether it be stable available, provide a link for the reader to a Medline abstract
or an active disease process. A therapist must choose for the article cited. There are 636 cited references and other
whether somatosensory retraining, functional training, general references for this chapter, with the majority of
impairment training, augmented treatment interventions, those articles being evidence-based citations.
n Principles Used by Therapists for Retraining Clients with Pain

APPENDIX 9-A
and Motor Control Problems of the Hand
Nancy N. Byl, PT, PhD, FAPTA, Professor Emeritus, Department 3. When practicing tool use, let the sensation of the object teach
of Physical Therapy and Rehabilitation Science, School of your hand how hard to squeeze.
Medicine, University of California, San Francisco i. Modify the sensation of the object (e.g., very rough, slightly
A. Positive Foundation for Retraining rough, coarse, smooth, silky).
1. Carry out a regular exercise program, be well hydrated, eat ii. Take practice lifts of the object to determine how heavy it is.
balanced meals, get adequate sleep, make time to have fun, iii. Manipulate the object in your hands without visual moni-
minimize habitual repetitions, and effectively manage stress. toring before beginning functional use of the tool.
2. Engage in challenging balance to improve posture and integrat- 4. Avoid aggressive, precise, rapid, alternating, forceful finger
ing diaphragmatic breathing, neural mobilization, and core flexion and extension movements of the hand.
trunk strengthening to maintain a healthy posture. i. Transfer some of the work of the hand from the fingers to
3. Create learning strategies that emphasize sensory input and the forearm. For example,
feedback. You can do this by placing sticky, coarse, or rough o Lift the fingers by rotating the forearm into supination
surfaces on tools that are used in functional activities (e.g., pen, (e.g., turn palm up). If forearm rotation is limited, let the
keyboard, glass, hammer, utensils). shoulder externally rotate if necessary.
4. Set goals and objectives to guide your training. o When the hand needs to be palm down (pronated), let
5. Think positively about learning to be as good as you can be or the elbow swing away from the trunk if necessary to
to recover after injury; expect to regain function. keep the hand relaxed (e.g., internal rotation of the
6. Analyze and break down the tasks you want to learn or to shoulder can take the stress off the forearm).
improve into manageable components. ii. Use the hand in a natural functional position (e.g., rounded
7. Perform each component of functional tasks without abnormal palm from the base of the thumb to the base of the fifth
movements (e.g., pathological synergies, extraneous move- finger and rounded from the tips of the fingers to the wrist).
ments, excessive muscle firing, involuntary movements, Thus all the finger joints are slightly bent, the palm is
strain, pain). round, and the wrist is extended about 15 degrees. When
8. Be sure each activity is designed to require attention, repetition, your arms are at your side, this will usually be the position
progression of difficulty, feedback regarding accuracy of of the hand.
performance, and positive reinforcement (reward). iii. Do not let the joints of the fingers collapse or hyperextend
B. Stress-Free Hand Use Strategies when they are down on a surface. This can be difficult if the
1. Strengthen the small muscles inside the hand (intrinsic joints are hypermobile or the intrinsic muscles (muscles
muscles) to facilitate stability of functional hand use. inside the hand) are weak.
i. Give resistance to spreading fingers apart (try not to use o Practice dropping the hand onto a surface and maintain-
muscles that straighten the fingers). ing the roundness of the hand (a small soft ball under the
ii. Try to hold the fingers together while you use your other palm may be used for assistance).
hand to try and spread them apart. o Lean lightly onto the hand while it is on a flat surface,
iii. Bend the fingers at the large knuckle (metacarpophalangeal pronated and keep the round shape of the hand (e.g.,
joint) to 90 degrees by placing the back of the hand against may need to initially keep small round ball under palm).
the edge of a table. Now, one finger at a time, try to keep the o Thread the fingers of one hand through the fingers of the
fingers straight as you use the other hand to try and bend the other hand to help stabilize the hand when placing
finger, giving resistance at the distal segment of the finger. weight onto the hand as noted previously.
2. Concentrate on using the small muscles of the hands in all o Put a soft, rubber ball about 2 inches in diameter on
functional activities. the table; roll the palm of the hand over the ball while
i. Initiate bending the fingers from the base joint (the large letting the finger pads (not the tips) drop onto the
metacarpophalangeal joint that joins the finger to the palm); surface.
try to do this without bending the fingers at the other joints, C. Using the Computer Keyboard Safely
especially without using the muscles that bend the distal 1. Position yourself comfortably to use the computer.
finger joints. i. Sit with feet flat on the floor. Sit tall with hips about
ii. Avoid heavy gripping; squeeze the fingers in a power grip 90 degrees (vary this posture throughout the day).
only when necessary. For example, do not (1) squeeze the ii. Place the computer screen at or slightly below eye level.
steering wheel, (2) exercise while holding on to free iii. Keyboard height should be adjusted to maintain elbow
weights, or (3) squeeze a ball or strengthen the grip in flexion at about 80 degrees (positioned in approximately
other ways. 100 degrees of extension).
iii. Practice reaching for common objects with the eyes closed iv. Forearms should be angled toward the floor and not resting
and the hand relaxed. When you contact the object, let the on the table. If it is difficult to let your hands rest lightly on
sensation of the surface of the object open the hand. For the keyboard with the wrist floating, it may be helpful to
example, when you reach for your cup, let the cup open the have a pillow on your lap (or a lumbar roll around the
hand (e.g., do not actively spread the fingers first). Do not waist), where the forearms receive positive sensory infor-
use the handle of the cup. mation to help them relax.
Continued

APPENDIX 9-A
and Motor Control Problems of the Hand—cont’d
v. Place the screen about 2 feet away from the eyes for most vi. Consider interfaces other than a mouse (e.g., roller ball, a
work; pull the screen closer as necessary for close work. movement-sensitive pad, pen).
vi. Consider getting special antiglare glasses for computer vii. If it is not possible to use the hand in a stress-free way
terminal display work or use a screen glare protector. when on the computer, then consider voice-activated
2. Use your hands in a functional (e.g., round, not flat or angular) software to use your computer.
position on the keyboard. o Use your voice carefully and without excessive force
i. Look at the contour of the hand when it is at your side; or strain (e.g., loudness).
maintain that position as the finger pads (not the tips) are o Be careful to prevent co-contractions and stressful use
dropped on the keyboard. of the vocal cords.
ii. Place a rough surface on the keys (e.g., Velcro) to make it 9. Take regular breaks (e.g., every 15 minutes).
easier to feel the pads on the keys. i. Consider obtaining the software that forces a computer
iii. Avoid placing the tips of the fingers on the keys. This breakthrough screen reminder.
creates an obligatory co-coactivation of the finger flexors ii. Do diaphragmatic breathing continually while working
and extensors. on your computer to minimize tension and facilitate good
3. Keep the wrist in a neutral position (0 to 10 degrees of exten- oxygen exchange.
sion) while working on the keyboard (e.g., a floating wrist). iii. When taking a break and staying at the desk, get your
i. Do not rest the wrist on a “wrist rest.” Resting the wrist and hands off the computer and change your sitting posture
forearm on the work surface will increase the pressure in while doing gentle range-of-motion exercises. Occasion-
the carpal tunnel and force all the work to be done with the ally place the arms on the desk and bend the trunk over
fingers. the arms.
ii. If there is a wrist pad on the computer keyboard tray, think 10. At least every 20 minutes, stand up for a few minutes and
of the pad as a “sensory tickle” to let you know that your stretch.
wrists should be floating above the rest. D. Writing
4. Have all the fingers resting on the keyboard. 1. The fulcrum for the movement of writing should be the shoulder
i. Do not let any of the fingers fly up. and elbow, not the fingers.
ii. Continue to keep the fingers resting down even when one 2. The hand should be round and relaxed.
finger is engaged in depressing a key. 3. Try putting a sticky or a rough surface on the pen or pencil
iii. Avoid allowing the adjacent fingers to extend to get them before you begin to practice.
away from the finger actively pressing down. a. A sticky surface (e.g., tape with the sticky side facing out)
5. It is not necessary to actively lift the fingers after pressing can be strong enough to hold the pen in place
down. Usually it is sufficient to release the pressure without without any squeezing.
actively lifting up the digits. b. A fatter pen is not as helpful as a sticky or a rough
6. Avoid resting the fingers on the keyboard with the finger tips. surface. It is possible to excessively grip a large pen.
This leads to a contraction of the fingers and the wrist. 4. Practice writing when you are not at work or at a store when
i. Do not keep your fingers excessively curled. In that position you have to write your name.
it is impossible to keep the fingers on their pads. 5. Practice writing non–work-related words and sentences and
ii. Initiate the movement down from the base joint of the then progress to meaningful writing.
fingers. 6. Try holding the pen by different fingers or using different
iii. Imagine that you are using the muscles inside your hand movements.
and not the long muscles that bend the fingers. i. Try to hold the pen between the second (index) and third
iv. Avoid reaching one finger out in isolation from the others. (middle) finger rather than the thumb (D1), index finger
7. In general, change the primary fulcrum of movement from the (D2), and middle finger (D3). The hand should be open,
fingers of the hand to the elbow and shoulder. thumb resting down.
i. Allow the elbow to move freely in flexion, extension, and ii. If you must hold the pen in the traditional way, try to hold
rotation. the pen lightly among D1, D2, and D3, with D1 and D2
ii. Use the trunk with a little shoulder movement when reach- moving toward the thumb from the base joint with all
ing for an object or a paper or to move closer to or away joints of the digits extended.
from the computer keyboard or screen. iii. With a sticky surface on the pen, it is possible to control
8. Use the mouse by using forearm rotation rather than individual the pen with minimal squeezing.
finger movements. 7. Practice picking up the pen and putting it down without
i. Do not squeeze the mouse; drape your hand on the mouse. feeling any tension in your hand.
ii. Keep your wrist in neutral position. 8. Control the movement of the pen primarily from the elbow and
iii. Avoid clicking the button by lifting and bending the index shoulder; keep wrist and fingers quietly positioned on the pen.
finger. i. Let the arm rest lightly on the table and comfortably on
iv. Use rotation of the forearm to activate the button on the the ulnar (fifth finger) side of your hand. Avoid resting the
mouse. elbow on the surface. If there is inadequate pronation
v. Make sure the mouse is close to you and that the arm is not (e.g., it is uncomfortable to have the hand be palm side
extended to the side. Place a cover for the mouse over the down), allow the shoulder to move out away from the
number keys, if necessary, to keep the arm closer to your trunk. trunk (e.g., shoulder abduction or rotation).

APPENDIX 9-A
and Motor Control Problems of the Hand—cont’d
ii. Let all fingers rest down on the pen or the support surface. 2. Pull the seat close to the steering wheel so that you do not
Do not hold any fingers up off the pen or the support have to reach out so far for the gas pedal.
surface. 3. Sit tall to ensure good visibility, and try to drive without
iii. Mentally review relaxed writing before beginning to write stress.
with a new technique. 4. Consider putting a rough surface on the wheel so you do not
iv. Practice making circles, loops, large numbers, and letters. tend to squeeze it (you can buy ergonomic steering wheel
Consider practicing by writing in shaving cream, finger covers).
paints, or water. 5. When you need to look behind you, shift your weight in the
v. If you see your fingers moving and your knuckles turning opposite direction that you want to look. This will allow you
white, you are squeezing too hard and you are using only to turn your whole trunk in the desired direction and avoid the
your fingers. isolated neck strain that occurs when you only turn your head.
9. Use a mirror to get some feedback to retrain your style of 6. Mentally rehearse and review calm, alert driving.
writing. 7. Do not squeeze the wheel in a death grip. Hold the steering
i. Place a mirror in front of your affected hand as you write wheel by gently pushing your arms together. You only need to
and notice whether it appears relaxed. hold the wheel with a palmar squeeze when turning.
ii. Place the unaffected hand in front of the mirror and the 8. Keep your arms comfortably at your sides.
affected hand behind the mirror. Look at the image of the 9. Do not grip the shift knob; press the palm of your hand down
unaffected hand (e.g., looks like the affected side), and on the shift bar to change gears. You may even want to allow
then have the affected hand behind the mirror copy the your trunk to move with your arm while shifting.
mirror image. 10. If you continue to experience stress with driving, practice
10. Put the pen down if any signs of stress develop. braking and turning the wheel in your garage and imagine
E. Daily Activities in the Kitchen different scenarios.
1. Use two hands to hold a pot or a frying pan. 11. Also, if you need a diversion to avoid emotional confronta-
2. Use an electronic can opener and jar opener. tion with rude drivers, bring a plastic bag of buttons that you
3. Use an electronic blender rather than hand stirring. can manipulate and match to decrease your stress.
4. Use a chopper to avoid heavy cutting. G. Other Household Activities
5. Stand close to the sink and the work surface so you do not 1. As before, do not grip objects too firmly; keep hands open
have to have your arms out too far in front of you. and work with your arms close to the trunk.
6. Get close to the table for setting the table; avoid having to 2. Always bend your knees to pick up objects from the floor.
lean over; bend at the knees. 3. Be careful to avoid leaning over and straightening the bed-
7. If you are short, stand on a stool to work at the sink. ding (e.g., when making the bed, ask someone to do it with
8. If you are tall, consider raising the refrigerator up higher so you; otherwise, make one side of the bed at a time).
you do not have to lean over. 4. Put items at eye level; avoid putting things over your head for
9. Concentrate on eating and using utensils without stress in which you have to reach out and up.
your hands. 5. Walk close to the vacuum cleaner; try to hold it where you
i. Consider putting a sticky or a rough surface on the utensils do not have to reach your arms out (e.g., step forward and
(e.g., Velcro or flooring with a sticky back). backward with the movement of the vacuum cleaner).
ii. When eating, hold the utensils lightly, even when trying 6. Do not lean over from the waist for dusting; if necessary, dust
to cut. while kneeling or wipe the floor while you are on your knees;
iii. When cutting, move the whole arm from the shoulder; use hold the dust cloth lightly.
the weight of the trunk to assist putting force down on the
knife.
F. Driving
1. Use a lumbar roll in the back of your seat to support your
lower back. Also consider placing a wedge in your seat (vary-
ing the placement of the wedge with the high side in front and
then toward the back).
APPENDIX 9-B n Specific Learning-Based Sensorimotor Training

A. Instructions significant gains in performance for musicians with focal hand
Patients dystonia after 1 year of training focusing on task practice while
We use our hands for many skilled fine motor and functional tasks. controlling the fingers with a splint to improve isolated control of
It is important for these movements to be smooth, efficient, and the dystonic fingers. For patients who are stable after a stroke, Byl
accurate. When there is dysfunction in the central or peripheral and colleagues44 also reported significant gains in fine motor
nervous system from congenital anomalies, injury, disease, over- performance after a sensory retraining program similar to the
use, degeneration, or chronic pain, skilled and functional move- activities described here.
ments can be impaired. Although it is still important to strengthen Therapists and Family Members
the muscles, increase flexibility, and restore normal motor control, When giving these instructions to patients, it is important to
it is critical to improve sensory processing. The purpose of learning- supplement the written instructions with pictures or even videos.
based sensorimotor activities is to place demands on the sensory For patients with significant cognitive impairments, these instruc-
receptors of the skin, the muscle, and the joints to restore normal tions are almost more important for the family members who are
sensitivity and accuracy of sensory input and feedback. Your brain helping reinforce the supervised therapy program.
can change with training. By improving the accuracy of sensory B. Principles of Learning-Based Sensorimotor
discrimination under conditions of high levels of attention, repeti- Training
tive activities progressed in difficulty and reinforced with feedback 1. Learning strategies focus on improving the discrimination of
and reward should improve how the hand is mapped on your brain the somatosensory system in a range of tasks that focus pri-
(e.g., primary sensory cortex). When specific tasks involve motor marily on sensory processing during sensory discrimination
practice, topographical changes will also occur in other parts of tasks and fine motor tasks.
the brain (e.g., thalamus, motor cortex, limbic system, basal gan- 2. Successful recovery is contingent on being able to imagine
glia, prefrontal cortex, supplementary motor cortex, brain stem). using the hands normally again without abnormal movements,
Although most think about the motor requirements for performing apprehension, or pain.
a task, it is essential to have accurate sensory information and 3. The injured hand (affected limb) needs to recover laterality
feedback, which comes from accurate sensory differentiation of the (right and left).
hand. Dynamic sensory topography and function are requisite for 4. The patient needs to be able to look at a hand and imagine
the restoration of fine motor control. integrating the image of the hand into the movement or posi-
Research also suggests that positive expectations can facilitate tioning of his or her own hand.
recovery and maximize performance.634 Physical impairments can 5. The hand must be able to interface with the target surface
lead to significant handicaps and disability. In these cases it is chal- without creating tension, pain, or abnormal movement.
lenging to maintain a positive attitude and be motivated for recov- 6. It is essential to be able to mentally imagine performing
ery and rehabilitation. Depression, anxiety, loss of self-worth, and related and target tasks without abnormal movements
compromised self-esteem can significantly impair the recovery or pain.
process, especially when training activities are demanding, inten- 7. Sensory processing must achieve a minimum level of accu-
sive, and possibly associated with discomfort or frustration. It is racy before functional fine motor movements are integrated.
essential to progress activities without causing unnecessary anxi- 8. Functional fine motor tasks need to be mentally practiced
ety, apprehension, or pain. With these issues in mind, the initial before they are physically practiced.
steps in sensorimotor training may seem unusually simple and 9. Tasks must be divided into the smallest components that can
involve imagery in lieu of motor practice. Also, although the sugges- be normally executed (e.g., partial task performance), which
tions here focus on the hand, the principles apply to sensorimotor will serve as the foundation for building skill-based learning
retraining for other parts of the body as well. on the whole task.
Specific randomized clinical trials have not been carried out on 10. Learning requires attention and repetition of behaviors
this series of training activities. However, Moseley611 carried out progressed over time.
several studies establishing the procedures to perform recognition 11. Feedback and reward must be integrated into all learning
training of hand laterality, imagined hand movements, and mirror activities, either by mental imagery, mirror imagery, visual
movements. He also carried out a randomized clinical trial for reinforcement, auditory feedback, or objective, accurate task
patients with complex regional pain syndrome using these training performance.
techniques. He randomly assigned 20 subjects to one of three dif- 12. Feedback from error correction may be critical for enhancing
ferent groups: hand laterality recognition, imagined movements, learning.
mirror training, or imagined movements; hand laterality recogni- 13. Each component of a functional task must be performed as
tion, imagined movements, or hand lateral recognition; mirror normally as possible before progressing to a more difficult
movements or hand recognition laterality. At 6 and 18 weeks after task (e.g., without pathological synergies, extraneous move-
training for 2 weeks on these behaviors, subjects in all groups had ments, excessive muscle firing, involuntary movements,
a significant reduction in pain and disability (P ,.05), with strain, pain).
the group doing hand laterality recognition, imagined movements, 14. Repetitive activities must avoid stereotypical movements that
and mirror training making significantly greater gains than the occur nearly simultaneously in time.
other two groups. Byl and co-workers635,636 also reported signifi- 15. Sensory discriminative retraining should eliminate visual
cant gains in patients with focal hand dystonia after 6 weeks of cues to facilitate somatosensory learning (e.g., eyes closed,
learning-based training. Candia and colleagues634 also reported blindfolded, distorting lenses).
APPENDIX 9-B n Specific Learning-Based Sensorimotor Training—cont’d

16. Begin sensory training on nontarget surfaces or with easy D. Initiate Specific Learning-Based
tasks that do not trigger abnormal responses (e.g., nontarget Sensorimotor Training
tasks). 1. Retrain cutaneous, muscle, and joint receptors at nontarget tasks.
i. Practice on nontarget tasks until sensory processing is i. Develop a variety of active sensory discrimination activi-
improved and the task can be performed without any ties that you can do by yourself (e.g., actively exploring to
abnormal movement. interpret different object surfaces—stereognosis).
ii. Integrate sensory retraining in tasks that historically have o Take the opportunity to feel objects in your environment
been associated with abnormal movement (e.g., writer’s and identify the objects without looking at the object.
cramp, keyboarder’s cramp, hand functions associated o Put small objects in bowls of rice or beans and reach in
with abnormal synergies related to hypertonicity, tremors, and try to find and match the objects.
dystonia). o Hang different objects from a string on a door jamb;
C. Preliminary Activities to Improve start the objects swinging and allow them to stimulate
Readiness for Learning-Based Sensorimotor your hand. See whether you can differentiate the
Discrimination Training different objects as they move across your hand.
1. Restore hand laterality recognition. ii. Modify the difficulty of the sensory task.
i. Follow the guidelines developed by Moseley611 to be able o Change the intensity of the sensory stimuli (e.g., make
to quickly see the hand in different positions and identify the surfaces less distinct).
whether the hand is right or left. o Increase the challenge or the complexity of the stimuli
ii. See pictures of the hand in different orientations and you are trying to identify.
different positions of the wrist and fingers and identify o Change the environment in which you are exploring the
whether right or left. sensory stimuli (e.g., hand in water, still or agitated; in
iii. See the pictures in random order, faster and faster, and be shaving soap; in whipped cream as you discriminate an
able to accurately determine the side.611 object or manipulate a pen).
2. Restore ability to mentally imagine putting the affected hand o Change the position you assume when discriminating the
into different positions.611 stimulus (e.g., lie down on your back or your stomach,
i. See pictures of the appropriate hand (affected) in different stand instead of sitting).
positions. iii. Palpate objects in water or other media for identification;
ii. When picture is shown, mentally put your hand into the have the water be still and then agitate the water.
same position as the one in the picture. iv. Put pairs of coins and objects in your pocket (or a plastic
iii. Practice doing this while changing the order of the bag) and try to match them or discriminate between them.
positions and the time the position is visualized. v. Purchase clay that can be molded and shaped and then
3. Restore the ability to imagine performing normal movements heated until firm.
while observing a video of the hands of someone else o Place or draw different shapes on the clay.
performing target and nontarget tasks. o Always include a pair of designs that can be matched.
i. Record video of different people performing target and vi. Paste matched pairs of items on a card and try to find the
nontarget tasks. matched pairs.
ii. Watch the videos and imagine that the hands being o Paste stickers with shapes on cards and try to find
observed are your hands performing the tasks without matched pairs.
pain or abnormal movements. o Paste matched pairs of buttons on a card.
4. Learn how to copy a mirror image of the affected side.635 o Paste alphabet soup letters on a card and match letters
i. Place the unaffected hand in front of a vertical mirror and or spell words.
the affected hand behind the mirror (out of sight). o Put magnetic letters and other shapes on a card or a
ii. Look in the mirror and note that the mirror image of the refrigerator and move them to spell words.
unaffected hand looks like the affected hand. vii. Take construction paper and create pairs of letters, shapes,
iii. Do simple tasks using the mirror image to guide the or other designs by pressing heavily with the pen; this will
movement of the affected side. create a raised surface on the other side.
o Take the pictures from the visualization training and o With eyes closed, palpate and try to find matching pairs.
assume the position of the hand and wrist.635 o Turn the paper in different directions to make the
o Put different sensory objects within the reach of both exploration different.
hands; pick up an object and make the object feel the viii. Make a grab bag of items and reach into the bag and identify
same on both sides.635 the objects by gentle touch.
o Do simple functional tasks with both hands simultane- ix. Obtain Braille workbooks and learn to read Braille.
ously (e.g., turn hand up and down, tap a finger, bring o If you have trouble learning Braille with the affected
thumb to each finger, pick up a pen, circle the pen, side, try with the unaffected side.
pick up objects of different size or same size but o Do not tense your hand as you feel the letters, and do
different surfaces).635 not extend the adjacent digits away.
Continued

o Work your hands smoothly over the dots. You can iii. Create games in which objects have to be moved accurately
improve your skill, getting other workbooks for the across specific distances on a variable surface.
blind and ultimately purchasing books in Braille. iv. Create objects of the same weight and place different
o Obtain “Braille object cards” where the object is types of surfaces on the object (e.g., Velcro, sandpaper,
described in Braille. Palpate the letters and sentences. flooring). Then practice picking up, moving, and putting
x. Place raised numbers and designs on the computer key- down the object with minimal effort.
board and try to determine what the number or shape is v. Assemble puzzles by feeling the matching pieces rather
before striking the key; make some labeled letters match than looking with the eyes.
or mismatch the key itself. vi. Work with a friend and practice copying movements
2. Practice activities requiring the interpretation of sensory infor- together (first by looking and then by feeling).
mation delivered to the skin (interpretation of sensory inputs o Tap one finger while the other fingers are resting down.
without active exploration of the stimulus, graphesthesia). o Bring arms up over head and tap one finger at a time.
i. Ask a friend to stimulate your skin with different stimuli o Bend wrist with one arm and bend elbow with other arm.
(e.g., hot, cold, sharp, dull, rough) and try to identify the o Circle wrist to the right (right hand) and circle to the
stimuli. left with left hand.
ii. Ask this friend to draw numbers, letters, words (upper and vii. Have a friend give you some resistance as you move one
lower case or cursive), and designs on your forearm, finger, the wrist, or the forearm up and down.
hands, and fingers when you are not looking. viii. On a piece of paper, draw hand diagrams with different
o Identify the letters, numbers, words, and shapes angles of each finger and different angles of the wrist.
verbally (e.g., start with capital letters). Then put up a vertical screen where you cannot see your
o When it is easy to be correct on capital letters, have hand. Look at each picture and try to copy the pictures
your friend draw lowercase letters, including words. with your own hand. Look behind the screen to check to
o Progress to having designs drawn on your skin; repli- see how accurate you are.
cate the design by drawing it on a piece of paper or on ix. See if you can rent a continuous passive motion machine.
your own skin. o Set the machine at different speeds.
o Ask your friend to give you feedback about the o Try to follow the movements of the machine.
drawing to make sure the drawing matches the o Apply vibration to the skin over the joint in the direc-
stimulus. tion opposite to the movement.
o Check the angles where the lines meet. o Carefully time the movements to enable success.
o Note accuracy of detection of curves. x. Practice grasping objects with a light grip on the object.
o Note whether all parts of the design are placed in the Use a spherical group (thumb pad to the pads of other
right relationship and orientation (spatial accuracy). fingers). Practice this with objects of different size with
o Note whether the design is the correct size. minimum graded force.
o Check whether the drawing has some elaborate xi. Practice bending and straightening the elbow, wrist, or
components that were not actually drawn on the fingers while applying vibration to the appropriate joint.
surface of the skin. o When bending (flexing) the joint, apply vibration on
o Your friend should make the drawings smaller and the extensor surface.
smaller to increase the challenge of detection (e.g., o When straightening the joint, apply vibration on the
2 to 3 mm). flexor surface.
o The drawing or the stimuli should be delivered two E. Sensory and Fine Motor Activities at
or three times. If the design is still missed, look at the Nontarget Tasks
design. After viewing the design, repeat the design at 1. Move in normal patterns in desired directions without excessive
the next trial (or the alternate trial), and before pro- firing of the muscles.
gressing determine whether you can recognize the i. Consider a number of strategies to allow you to move the most
drawing). Use a friend to check on your accuracy. difficult finger more easily (e.g., stabilize adjacent digits).
3. Use other stimuli to reinforce somatosensory learning. o Use a soft splint to stabilize the fingers adjacent to the
i. Develop tasks to improve sound discrimination (either finger you want to move.
location or determination of whether you hear one or two o Mold a piece of clay; keep an area clear under the
sounds delivered). finger you want to move, and place a hole in the clay
ii. Have a visual stimulus provided at the same time an object for the other fingers to rest in.
is touched to the skin (on the affected and unaffected side); o Put a buddy strap on fingers adjacent to most dystonic
the goal is for you to accurately describe the cutaneous or painful finger.
stimulus (e.g., sharp, dull, smooth, rough, silky, hard, soft). o Put tape on the fingers on the surface that would be
4. Develop activities to emphasize proprioceptive and kinesthetic most likely to improve movement (e.g., on the flexor
learning. surface if the finger extends; on the extensor surface
i. Where necessary, use tape on the skin, use electrical or if the finger flexes; on the side of the finger if having
auditory biofeedback, or put weights around the wrist and difficulty with isolation).
ankle to increase feedback from joint, tendon, and muscle o Use a finger interphalangeal splint on fingers adjacent
receptors. to dystonic fingers.
ii. Create games in which a part of an object has to be ii. Increase sensory feedback on the finger you are trying to
accurately placed on a topographical picture. move (e.g., use tape on the finger).

2. With the eyes closed, play games that require discrimination of 4. Be sure to get reinforcement for performing all tasks normally
sensory information through the skin of the fingers. (e.g., use a mirror, use biofeedback, get verbal feedback).
i. Play dominoes. 5. Have someone make a video performing the target task with
ii. Play pick-up sticks. which you are having trouble. Then try to copy the movements.
iii. Play shape games (e.g., match a shape to an opening, such Watch the movements carefully and imagine that the move-
as in Perfection). ments are your hands moving.
iv. Put together puzzles that have a raised surface. 6. Perform the target task in different, nontraditional positions (e.g.,
v. Play Scrabble with raised or indented letters. practice in nontraditional positions such as lying on the back, lying
vi. Play games that require orientation in place without the on the stomach, reaching hand behind you or over your head).
benefit of vision. 7. Do the target task in different media (e.g., if having a problem
o Play pin the tail on the donkey. with writing, draw shapes and letters in shaving soap; draw big
o Walk through the house with your eyes closed and hands out letters and then small letters and then words).
to feel objects in your way and to catch yourself if needed. 8. Provide external support of the affected hand to appropriately
vii. Get a Braille deck of cards and play cards (e.g., Solitaire position the digits (e.g., a splint if necessary to prevent move-
can be played alone; play hearts, bridge, pinochle, or ment of adjacent digits) while doing sensory and sensorimotor
poker with others). tasks on the target instrument.
viii. Create other sensory games that require planning and i. Begin with a single digit adjacent to the most involved digit,
control and that can be played without vision. but not the most involved digit.
F. Learning-Based Sensorimotor Retraining (Praxis) ii. When you can do complex sensory exploration with a sin-
1. Feel objects and then define and demonstrate what to do with gle finger without abnormal movement, combine sensory
the objects. exploration with more complete target movements.
2. Have a friend provide a sensory stimulus and ask you to do iii. Add multiple digits to the sensorimotor tasks.
something that indicates you felt the stimulus (e.g., “when I tap 9. Without externally supporting the position of the digits (e.g., all
with this sharp object, I want you to tap once, but when I touch digits free) perform one simple movement on the target task.
you with this dull object, I want you to tap twice”). i. Integrate sensory exploration with the simple movements
3. Feel a number of items in a bag that are related to performing a and do the movements slowly, in time with a metronome.
task, and put the items together to do the task. ii. Increase the complexity of the sensory-driven motor tasks
4. Feel a number of objects put together in a specific design; have (e.g., tapping single note to playing scales and chords to
someone give you a second set of the objects to replicate or playing new music or performing new keyboard tasks).
match the design. iii. Increase the speed of the movements on the target task,
5. Practice throwing objects of different size; practice throwing keeping up with the metronome.
them to a particular spot. iv. Perform the target task normally for brief periods, and
6. Get accustomed to grading movements without uncontrollable progress the practice time slowly with frequent breaks.
contractions. H. Reinforcing Sensorimotor Learning with Feedback
i. Place the hand on a moving target and do not stop the movement. 1. Biofeedback can include visual, cutaneous, muscle, vibration,
ii. Manipulate objects without excessive force. auditory, or stretch stimuli.
iii. Put your hand on a record player and do not stop the 2. Biofeedback can be supervised by another person, facilitated with
record movement (e.g., do not change the sound). robotic movements, controlled by electronic contraction (activa-
iv. Put your hand on the moving belt of a treadmill and feel tion of muscles), or controlled by a physical constraint of a limb;
the moving belt. guided repetitive passive movements can be supplemented with
o Feel the belt moving under the hand. active movements to control motor output.
o Hold objects under the fingers. i. Put tape on the top of the skin over the extensor surface of the digits
o Pass objects back and forth between the fingers, and to limit motion or emphasize somatosensory input and feedback.
make the objects feel the same. ii. Use multichannel biofeedback to learn how to avoid abnormal
7. When it is possible to perform the sensory activities in nontarget movement strategies.
tasks, begin placing the hand on the target instrument without o Practice isolated movements and stop practice of
abnormal movements. unnecessary co-contractions of agonists and antagonists.
i. With the hand on the target instrument, mentally rehearse o Use the small muscles inside the hand (intrinsic mus-
the movements and the tasks you should perform. cles) to move the digits instead of the extrinsic muscles.
ii. Add rough surfaces to the target instrument if necessary to o Use imagery with mental rehearsal and practice to help
change the interface with the hand. restore the image of performing the task normally (see
G. Sensorimotor and Fine Motor Training Appendix 9-C).
at Target Tasks I. Return to Work
1. Emphasize the sensory aspects of the task even when beginning 1. Try to return to work part time.
to perform the target task. 2. Discuss other work options if you cannot resume the original
2. Perform a selected component of the task (e.g., drop one finger job tasks.
down on the keyboard). 3. Make ergonomic modifications at the workplace.
3. Progress the ability to complete more and more of a target task, 4. Integrate stress-free techniques.
emphasizing sensory exploration as long as the tasks can be 5. Take frequent brief breaks.
done normally. 6. Walk or do other exercises at lunch time.
n Enhancing Learning-Based Sensorimotor Training: Use of Imagery,

APPENDIX 9-C
Mental Rehearsal, and Mental Practice
A. General Comments about Imagery When there are conditions of chronic pain, there are changes
It is critical to restore confidence, a sense of wellness, and normal in the organization and representation of the painful part in the
control of the movements of the extremities and trunk. Initially this central nervous system (e.g., cortex, thalamus, prefrontal cortex,
may be difficult because of pain, lack of accurate sensory informa- supplemental motor cortex). Similarly, repetitive, abnormal pat-
tion, and difficulty with the control of movement or imagining that terns of movement also can dedifferentiate the representation
the hand or arm could be normal again. One way to begin to restore of the body part. Thus, intervention must focus on restoring
the accuracy of the information processing system so you can use the normal representation of the brain. Sometimes it is easier
your hand normally is to begin by changing how the hand and the to imagine normal movement or pain reduction than it is to
functional task you are trying to perform are represented on your actually change the pattern of movement or turn off the “on
brain (the internal representation of that injured part). cells” for pain.
It is important to be able to restore the normal image of the B. Suggestions for Goal-Directed Imaging
involved limb, that is, how it used to be and how it will be normal 1. Set goals for yourself to specifically improve the function of
again. In the process of restoring normal control, it is also impor- your hand.
tant to begin to use the hand normally and not increase the pain or 2. Follow a sequence for learning.
repeat the abnormal movements. Thus, visually imagine your hand a. Imagine that you are healthy and fit and have full normal
and how it looks. Making your hand look like the other hand is a control of all of your extremities.
good beginning. Then begin to create an image of the hand and the b. Focus on healing the involved tissues, particularly if you
task you want to perform. Imagine using the hand normally to have signs of inflammation and pain.
perform all the usual and target tasks. You can start by imaging i. Focus on diaphragmatic breathing and bringing blood
small parts of a larger task and then finally the whole task and to the tissues.
then related skills and activities that would be associated with ii. Imagine the blood carrying important elements to the
performing the task. area of injury (e.g., the growth factors and oxygen that
With advances in magnetic resonance imaging, we can more are requisites for healing tissues).
readily confirm the recruitment of brain processes with imagery. It iii. Imagine that an injury causes inflammation that triggers
is possible to activate functional, motor, and sensory representa- the healing response (e.g., laying down collagen [scar]).
tions of the hand with mental imagery. The area of the brain Also imagine that the body modifies the scar tissue and
recruited is dependent on the activities imagined by the individual. tries to keep it mobile.
For example, you have many different maps of your body. Some of c. Visualize the anatomy, physiology, and kinesiology of
the topographical maps may be redundant across different parts of the hand.
the central nervous system (e.g., motor cortex, sensory cortex, i. Imagine the bones gliding smoothly on one another.
prefrontal cortex, thalamus, basal ganglia). Well-learned functions ii. Imagine the muscles being strong, with a balance
are also mapped separately from sensory and motor topography. between the intrinsic and extrinsic muscles that serve
When you visualize a body part, you will activate the somatosen- the hand.
sory cortex. When you imagine doing the task (motor imagery), iii. Imagine normal movement patterns.
you will also activate the motor cortex. When you can visually and iv. Imagine normal sensation in the hand.
motorically imagine completing the task in your mind, you will d. Imagine pain-free movement.
activate the cortical areas representing the part of your body that is e. Imagine the hand being quiet and relaxed.
moving and the part of the brain that is devoted to completing that f. Imagine smooth control of the hand without involuntary
task (e.g., walking, writing, playing an instrument). The intensity extraneous movements.
with which the neurons fire when you are imaging is less than the g. Imagine that the affected hand is working just like the
intensity of firing when you are actually performing the task. Try unaffected hand.
to imagine performing your tasks without mistakes. This will rein- h. Imagine using the hand as you used to use it. Go back in
force the positive aspects of the sensorimotor feedback. You must time to when your hand felt good and you did not have any
imagine without interruption (e.g., attention), and you must repeat problems.
the imaging process with a high level of concentration to help the i. When mentally practicing and imaging, there should be no
nervous system learn. If you are imaging and you run into diffi- distractions. Spend at least 30 to 60 minutes a day normal-
culty completing a task normally, try to focus on the source of the izing the hand and imagining how good it feels.
difficulty, including asking your inner self what barriers are getting j. Mentally practice and perform the target task without any
in the way. Once you can get insight into these barriers, you should signs of strain or pain.
be able to break them down. k. Concentrate and mentally review each of the components of
During imaging or mental practice, approximately 30% of the the hand working normally.
neurons are recruited as would be recruited when the task is l. Concentrate on the free flow of rhythmic movements of the
physically executed. Furthermore, when learning a new task, more hand and arm as you walk.
neurons are recruited than when the task is learned. An impairment m. Recapture the excitement of using your hand while playing
of structure (e.g., neurological or musculoskeletal) could modify your instrument or working at your job without pain or
the ability to image performing a task normally. On the other hand, strain.
imaging normal function and task performance could be easier n. Reinforce the image of a normal hand by continuing to
than actually executing normal performance. In addition, repetitive progress learning, including more complex tasks and public
imaging could begin to drive neural adaptation and recovery. performances.
CHAPTER 10 Payment Systems for Services:
Documentation through the Care Continuum
BARBARA EDMISON, PT, and JOHN G. WALLACE, JR., PT, MS, OCS

capitation After reading this chapter the student or therapist will be able to:
CMS 1. Value the importance of documentation and its relationship to payment for services.
CPT 2. Synthesize different inpatient and outpatient payment systems.
HIPAA 3. Differentiate the continuum of care and documentation needed in different treatment
ICD-9-CM settings.
ICD-10-CM 4. Appropriately assign an ICD-9-CM code to medical and functional diagnoses.
Medicaid 5. Identify and select the CPT codes that best describe therapeutic interventions used in
Medicare treating patients.
prospective payment system 6. Analyze how payment policy can affect patient outcomes.
reasonable and necessary
skilled services
third-party payer
IMPORTANCE OF DOCUMENTATION Documentation is critical for success in the payment appeals

Physical therapists (PTs) and occupational therapists (OTs) process. For these reasons, documentation and payment for
are in the business of providing a health care service to services are tightly linked together. This chapter will look at
improve quality of life. Because of the myriad insurance the payer sources at the national level and their required
options available from both private and government-run documentation components for payment.
programs, people rarely pay cash (self-pay) for physical It is important to remember that all the federal programs
and occupational therapy. Therapists want to be paid a mentioned in this chapter are constantly changing. The pro-
“fair” amount for their skills and knowledge, but they gen- cess of legislating health care is dynamic and will be sig-
erally rely on a third party to provide this payment. Clinicians nificantly modified in the next several years because there
must convince the third-party payer, an entity that was not are not enough dollars available to cover the projected total
present and did not receive the therapeutic interventions, costs. The supply of funds is in direct conflict with both the
that the patient received valuable, unique, and worthwhile increased numbers of patients and their need for services.
services. Documentation is one method used to persuade Major changes must occur in the future to enable health
the third-party payers to pay for the professional services care, as expected by the public, to survive. One of the keys
provided. to these changes lies in documentation. A new national health
Documentation is a skill a therapist must acquire. Its policy plan for United States citizens was voted on and
importance is equivalent to other forms of therapy skills. accepted in the spring of 2010, and new payment schedules
Documentation creates a lasting impression of the practitio- or structure may be the outcome; but the need for documen-
ners who represent the profession. Occupational therapy and tation will remain constant, and documentation will always
physical therapy are an imperative and integral part of be a tool used for evaluating and justifying payment for
patient care; the documentation must reflect that. In addi- services.
tion, PTs and OTs are legally responsible for interventions Why document? Documentation provides baseline status,
provided by personnel under their supervision. Therapists records pertinent information, measures progress and suc-
then depend on other people to interpret their documentation cess, fulfills predictions, and declares the final outcomes. It
and, on the basis of contracted rates, determine how much creates a record of the appointments the patient or client
should be paid for each service. Third-party payers often had. It provides data for concurrent or retrospective audits as
submit documentation to peer reviewers to ascertain exces- well as evidence for research. It serves as an itemized bill for
sive, useless, or fraudulent treatments. services rendered. The medical record may also become
Securing payment for services rendered is, and will con- evidence in legal proceedings, which can either defend or
tinue to be, a crucial element for the therapist as a profes- incriminate the clinician. Documentation provides a snap-
sional as well as for the therapist’s livelihood. Documenta- shot of a period of time that gives the reviewer a full and
tion, which is a legal and professional responsibility, is the practical description of the status of patients and the impact
basis for billing and is the proof that treatment was provided. care has made on their quality of life.
251
Who reads the medical record? Although many therapists Part A and Part B claims within their geographical jurisdic-
seem to believe that documenting is a necessary evil with no tions. MACs have the ability to accept or deny claims made
particular purpose, the information that therapists provide is to them for payment on the basis of their interpretations of
vitally important. Physical and occupational therapy docu- the CMS guidelines. Medicare Parts A and B are discussed
mentation is read by colleagues in the same or related disci- in more detail later in this chapter.
plines to affect or continue the plan of care (POC). It is also COBRA (from the Consolidated Omnibus Budget
read by physicians and discharge planners to assist in deter- Reconciliation Act of 1985) refers to short-term interim
mining additional treatment or surgical options or placement insurance coverage. It allows people whose employment
opportunities. Insurance case managers rely on documenta- benefits have been terminated to have continuing employer-
tion for the assessment of proper use of services. OT and PT sponsored group health coverage temporarily. The American
documentation is read by employees of third-party payers Recovery and Reinvestment Act of 2009 (ARRA) has
who may be screening for proper dates and codes or for expanded premium assistance to some people who qualify.
predicted outcomes in a reasonable time frame. Therapists Workers’ compensation is coverage for people who have
do not want to have payment denied for any reason; there- been injured on the job. These regulations are determined at
fore it is extr emely important that the documentation both national and state levels. Workers’ compensation is
clearly present all the pertinent information in a manner that discussed in greater detail later in this chapter.
is easily understood by all parties. Correct billing and claims processing are also dependent
on accurately communicating treatment diagnoses and inter-
DEFINITION OF TERMS ventions to third-party payers. Three primary coding systems
There is an entire language of terms regarding payment are used to communicate diagnoses and interventions in
issues. Please refer to the Quick Reference Guide to Acro- health care. The International Classification of Diseases,
nyms (Appendix 10-A) for assistance. When therapy services Ninth Revision, Clinical Modification (ICD-9-CM) is a tabu-
are received, either the person pays the therapist directly lar list of medical diagnoses approved for use by CMS based
or someone else pays the bill. Generally a patient will pay on the World Health Organization’s ICD-9, originally pub-
directly for therapy in three circumstances: (1) having a need lished in 1977. Current Procedural Terminology (CPT) (a
for skilled services and not having insurance; (2) having had registered trademark of the American Medical Association
therapy interventions, understanding their value, and wish- [AMA]) is a coding system that describes health care inter-
ing to continue beyond what insurance is willing to cover; or ventions. CMS has developed its own coding system to meet
(3) having a preference for a specific therapist who accepts the specific requirements of the Medicare and Medicaid pro-
only cash payment or who is not a preferred provider of the grams. The Healthcare Common Procedure Coding System
insurance company. When someone else pays the bill, it is the uses CPT and alphanumerical codes developed by CMS in
third-party payer that is billed for the services. Third-party conjunction with the AMA to describe interventions, proce-
payers are usually insurance carriers who, by contract or dures, and supplies for the Medicare and Medicaid pro-
written agreement, may determine the maximum amount of grams.1 Use of these coding systems is discussed in greater
money paid and under what circumstances. detail later in this chapter.
Private health insurance is either purchased by a consumer
or provided to people as a benefit of employment. People may FEDERAL PROGRAMS
have additional coverage by paying for it or as a result of
being a dependent on someone else’s insurance plan. This Medicare and Medicaid
secondary insurance may pay for the portion of the bill that is “Medicare is a health insurance program for people age 65 or
unpaid by the patient’s primary insurance. In the case of older, people under age 65 with certain disabilities, and
Medicare coverage, Medicare beneficiaries can purchase sup- people of all ages with end-stage renal disease . . . (permanent
plemental insurance that will pay some or all of the charges kidney failure requiring dialysis or a kidney transplant).”2
that are not part of their Medicare benefit. As the federal gov- The Medicaid program provides medical benefits to groups
ernment is taking on a larger role in making sure individuals of low-income people, some of whom may have no medical
are insured by setting up a National Health Insurance System, insurance or inadequate medical insurance.3 Although the
the payer for the therapeutic services may change, but the fact federal government establishes general guidelines for the
remains that someone or a group of insurance carriers will program, the Medicaid program requirements are actually
pay for services rendered. established by each state. Whether or not a person is eligible
Health care services, for purposes of payment, are gen- for Medicaid will depend on the state where he or she lives.
erally divided into three groups: inpatient, outpatient, and “President Truman was the first President to propose a
home health services. Inpatient services are delivered to pa- national health insurance plan.”4 Congressional debate about
tients staying in a hospital or health care facility. Outpatient federal health care coverage continued for 20 years. In 1965,
services are delivered to patients who receive service by HR 6675, the “Mills Bill,” was introduced. “Congressman
going to a health care provider. Home health agencies Wilbur Mills, Chairman of the House Ways and Means
(HHAs) deliver services to patients in their own homes. Committee, created what was called the ‘three-layer cake’
Medicare services are processed and paid for by Medicare by starting with President Johnson’s Medicare proposal
Administrative Contractors (MACs). MACs are responsible (Part A), adding to it physician and other outpatient services
for administrating Medicare programs in 15 jurisdictions (Part B), and creating Medicaid which significantly ex-
comprised of two or more states. MACs are private compa- panded federal support for health care services for poor
nies that have been awarded contracts by the Centers for elderly, disabled, and families with dependent children.
Medicare and Medicaid Services (CMS) for processing all Medicare became Title 18 of the Social Security Act and
CHAPTER 10 n Payment Systems for Services: Documentation through the Care Continuum 253
Medicaid became Title 19.”4 Although HR 6675 passed the by allowing Medicare beneficiaries options for additional
House without a single amendment, the Senate version types of health plans. The BBA also reduced hospital pay-
required much more discussion and many amendments. ments, which had considerable consequences in the health
Finally, Medicare Part A, which involves basic hospital care industry. This was one reason that the Balanced Budget
benefits and other institutional services for the elderly; Refinement Act of 1999 (BBRA) was introduced. The BBA
Medicare Part B, a voluntary program; and Medicaid were was also designed to address fraud, abuse, and waste in the
approved by both the House and Senate. federal health care programs.
Medicare and Medicaid implementation did not begin The BBA also created the Children’s Health Insurance
until 1966. Initially, “Medicare was the responsibility of the Program (CHIP), also known as Title XXI of the Social
Social Security Administration (SSA), the agency that con- Security Act. “CMS administers this program, which helped
trolled the retirement social insurance program through states expand health care coverage to over 5 million of the
which most people became eligible for Medicare. Federal nation’s uninsured children. The program was reauthorized
assistance to the State Medicaid programs was adminis- on February 4, 2009, when President Obama signed into law
tered by the Social and Rehabilitation Service (SRS). SRS the Children’s Health Insurance Program Reauthorization Act
oversaw welfare programs including Aid to Families with of 2009 (CHIPRA or Public Law 111-3). CHIPRA finances
Dependent Children (AFDC), through which many people CHIP through fiscal year 2013. It will preserve coverage for
became eligible for Medicaid. SSA and SRS were agencies the millions of children who rely on CHIP today and pro-
in the Department of Health, Education, and Welfare vides the resources for states to reach millions of additional
(HEW). In 1977, HEW Secretary Joseph Califano reorga- uninsured children. CHIP is jointly financed by the federal
nized the department to create the Health Care Financing and state governments and is administered by the states.
Administration (HCFA). HCFA was designed to improve Within broad federal guidelines, each state determines the
administration of both Medicare and Medicaid by moving design of its program, eligibility groups, benefit packages,
both health programs together, to improve the staffing of payment levels for coverage, and administrative and operat-
the Medicaid program, and to create a new administrative ing procedures. CHIP provides a capped amount of funds to
structure to implement national health insurance. In 1980, states on a matching basis. Federal payments under CHIP to
HEW was divided into the Department of Education and the states are based on state expenditures under approved plans
Department of Health and Human Services (HHS). In 2001, effective on or after October 1, 1997.”6
Secretary Tommy G. Thompson renamed HCFA to become At least two other federal laws affect children who may
the Centers for Medicare and Medicaid Services (CMS) as not have sufficient health care coverage. The Elementary and
part of his initiative to create a new culture of responsiveness Secondary Education Act of 1965 (ESEA), reauthorized as
in the agency.”4 the No Child Left Behind Act of 2001 (NCLB), is standards-
“Coverage for Medicare Part A is automatic for people based education reform that is directed at disadvantaged
age 65 or older (and for certain disabled persons) who have students. IDEA, the Individuals with Disabilities Education
insured status under Social Security or Railroad Retirement. Act, provides for early intervention, special education, and
Most people don’t pay a monthly premium for Part A. related services to children with disabilities.7
Coverage for Part A may be purchased by individuals who
do not have insured status through the payment of monthly Health Insurance Portability
Part A premiums. Coverage for Part B also requires payment and Accountability Act of 1996
of monthly premiums. People with Medicare who have lim- The Health Insurance Portability and Accountability Act of
ited income and resources may get help paying for their 1996 (HIPAA) is a legislative effort to improve insurance
out-of-pocket medical expenses from their state Medicaid coverage of the work force and also to improve the contin-
program. There are various benefits available to ‘dual eligi- uum of care by switching health care records away from
bles’ who are entitled to Medicare and are eligible for some paper and into the computer age.
type of Medicaid benefit. These benefits are sometimes also Title I of HIPAA refers to health insurance reform. This
called Medicare Savings Programs (MSPs). For people who reform increases the opportunities for workers to maintain or
are eligible for full Medicaid coverage, the Medicaid pro- acquire insurance coverage when they lose or change jobs.
gram supplements Medicare coverage by providing services Title II of HIPAA relates to administrative simplifica-
and supplies that are available under their state’s Medicaid tion. These provisions are more closely associated with
program. Services that are covered by both programs will be documentation and payment for services. The purpose of
paid first by Medicare and the difference by Medicaid, up to administrative simplification is to create a national database
the state’s payment limit. Medicaid also covers additional for medical records to ease communication among health
services (e.g., nursing facility care beyond the 100-day limit care agencies. However, this led to concerns about privacy
covered by Medicare, prescription drugs, eyeglasses, and and security of vital information as a result of easily acces-
hearing aids). Limited Medicaid benefits are also available sible online medical records. This prompted HHS to also
to pay out-of-pocket Medicare cost-sharing expenses for include a privacy rule and a security rule. “The Standards
certain other Medicare beneficiaries. The Medicaid pro- for Privacy of Individually Identifiable Health Information
gram will assume their Medicare payment liability if they (‘Privacy Rule’) establishes, for the first time, a set of
qualify.”5 national standards for the protection of certain health infor-
The Balanced Budget Act of 1997 (BBA) made the most mation. HHS issued the Privacy Rule to implement the
significant changes to the Medicare and Medicaid programs requirement of HIPAA. The Privacy Rule standards address
since their implementation. One goal was to shift some of the the use and disclosure of individuals’ health information—
financial stress to the private sector, which was accomplished called protected health information (PHI) by organizations
subject to the Privacy Rule, called covered entities—as security of their information. The well-being of a person is
well as standards for individuals’ privacy rights to under- reflected not only in her or his treatment but also by the integ-
stand and control how their health information is used. rity of the system to keep personal information confidential.
Within HHS, the Office for Civil Rights (OCR) has respon- HIPAA and its consequences directly relate to documentation
sibility for implementing and enforcing the Privacy Rule standards and handling of PHI.
with respect to voluntary compliance activities and civil
money penalties. Prospective Payment Systems
“A major goal of the Privacy Rule is to [ensure] that indi- Years ago, people received therapy in hospitals, Medicare
viduals’ health information is properly protected while allow- was billed, and the hospital was paid. Physical and occupa-
ing the flow of health information needed to provide and tional therapy departments were among the highest money-
promote high-quality health care and to protect the public’s makers in the hospital. This, unfortunately, led to excessive
health and well-being. The Rule strikes a balance that permits billing and resulted in the need for improved accounting.
important uses of information, while protecting the privacy of More recently, CMS has established stricter requirements in
people who seek care and healing. Given that the health care an effort to control spending and to have money available for
marketplace is diverse, the Rule is designed to be flexible and future generations. These requirements also benefit patients
comprehensive to cover the variety of uses and disclosures today by accelerating the establishment of a medical diagno-
that need to be addressed.”8 sis, allowing for faster implementation of therapeutic inter-
“While the Privacy Rule mandates policies and proce- ventions and preventing billing or payment for unskilled
dures to protect patient information in all forms, the purpose services. Currently, under the prospective payment system
of the Security Rule is to adopt national standards to protect (PPS), hospitals are paid a set amount per patient. The
the confidentiality, integrity, and availability of electronic amount depends on the medical diagnosis and related mor-
protected health information. This Rule is directed at the bidities. Payments are no longer related to the length of stay
covered entities, which are health care providers, health care or procedures ordered. It is the hospital’s responsibility to
clearinghouses, and/or health plans, that transmit or main- maximize its income by minimizing the patient’s stay.
tain protected health information electronically [and] are The Social Security Amendments of 1983 were respon-
required to implement reasonable and appropriate adminis- sible for the plan to save taxpayers money by creating incen-
trative, physical, and technical safeguards. The Security tives to improve efficiency in acute-care hospitals. This
standards require that steps be taken to protect this informa- system applied to Part A Medicare beneficiaries and was
tion from reasonably anticipated threats or hazards. Built designed to give the hospitals a lump sum for patients who
into the Security Rule, however, is some flexibility that fit into certain categories.
allows covered entities to determine what is reasonable “Section 1886(d) of the Social Security Act (the Act) sets
and appropriate based on their size, cost considerations, forth a system of payment for the operating costs of acute-
and their existing technical infrastructure. This built-in care hospital inpatient stays under Medicare Part A (Hospital
flexibility also makes allowances for the rapid changes in Insurance) based on prospectively set rates. This payment
technology.”9 system is referred to as the inpatient prospective payment
“On July 27, 2009, Secretary of the Department of Health system (IPPS). Under the IPPS, each case is categorized into
and Human Services Kathleen Sebelius delegated authority for a diagnosis-related group (DRG). Each DRG has a payment
the administration and enforcement of the Security Standards weight assigned to it, based on the average resources used to
for the Protection of Electronic Protected Health Information treat Medicare patients in that DRG.”11
(Security Rule) to [OCR].” This action will improve HHS’s Use of the IPPS and DRGs, in which Medicare payments
ability to protect individuals’ health information by combining are established in advance and determined by the medical
the authority for administration and enforcement of the federal diagnosis at discharge, created the opportunity to transform
standards for health information privacy and security called for hospitals into more efficient and cost-effective organiza-
in HIPAA. The HIPAA Privacy Rule is also administered and tions. It also became essential to accurately determine the
enforced by OCR. discharge diagnosis of patients in the hospital. Appropriate
“Congress mandated improved enforcement of the “coding” of patients developed in the Health Information
Privacy Rule and Security Rule in the Health Information Management Departments of hospitals to determine the
Technology for Economic and Clinical Health (HITECH) correct DRG and corresponding payment.
Act, part of the American Recovery and Reinvestment Act Although the DRG is associated with an average hospital
of 2009. Privacy and Security are naturally intertwined, cost per diagnosis and is calculated on a per-case-at-discharge
because they both address protected health information. basis, the actual payment is affected by many factors. There
Combining the enforcement authority in one agency within are two different paths that contribute to the final payment: the
HHS will facilitate improvements by eliminating duplica- operating, or labor, expenses, and the capital, or nonlabor,
tion and increasing the efficiency of investigations and reso- expenses. On the operating expenses side, the wage index
lutions of failures to comply with both rules. Moreover, incorporates local labor costs. Cost of living adjustments are
combining the administration of the Security Rule and the made on the capital side. Also taken into account is the geo-
Privacy Rule is consistent with the health care industry’s graphical area (rural versus urban) where the hospital is lo-
increasing adoption of electronic health records and the cated. To adjust for case mix, each DRG is weighted relative
electronic transmission of health information.”10 to its complexity against the other individual DRGs. There are
The federal government is helping businesses to achieve several other possible factors contributing to the DRG pay-
the HIPAA-mandated goals of improved and efficient health ments. The indirect medical education adjustment is allo-
care while protecting the privacy of the recipients and the cated when the hospital is an approved teaching hospital
for graduate medical education. The new technology adjust- therapy documentation to reflect improvement and goal
ment is granted if the hospital is using expensive new tech- achievement. Because of the relative insensitivity and ordi-
nology that significantly improves clinical outcomes. The nal scales of these comprehensive instruments, a significant
disproportionate share of the hospital adjustment is provided amount of functional change is often required to document
to hospitals that treat a higher percentage of low-income improvement from one level to the next.
patients. An outlier is an exceptionally expensive course of It is expected that third-party payers will begin to use the
treatment that qualifies for additional funding. DRG pay- outcome measurement tools as a way of assessing the perfor-
ments may be reduced if the patient’s length of stay is short- mance of different facilities. With this information available
ened by a transfer to another acute-care hospital or post– for comparison, physicians and payers may choose to admit
acute-care setting. Fiscal year 2009 completed the transition patients to those facilities that provide the best outcomes in
to MS-DRGs, which are based on secondary diagnosis codes the fewest number of days.
and provide more specific information for resource allocation. The IRF-PAI, RAI, and OASIS were developed with
Medicare Severity (MS) divides cases into three levels. MCC, essentially the same goals in mind: (1) to measure patient
major complications with comorbidities, is the most severe. outcomes and (2) to improve quality of care. These tools are
CC refers to complications with comorbidities, and Non-CC, each used in conjunction with the Medicare PPS to deter-
or no complications with comorbidities present, is the least mine payments. However, the functional tools themselves
likely to require additional hospital resources. are not related and therefore there is no one system available
With the success of the IPPS in acute-care hospitals, addi- in the United States to provide “standardized, patient-centered
tional legislation mandated extension into other settings with outcome data that can provide policy officials and managers
Medicare Part A beneficiaries. The BBA, the BBRA, and the with outcome data across different diagnostic categories,
Benefits Improvement Act of 2000 (BIPA) moved the PPS into over time, and across different settings where post-acute
skilled nursing and inpatient rehabilitation facilities (IRFs), services are provided (p. 13).”12 For the future, it is hoped
HHAs, hospice, hospital outpatient, inpatient psychiatric fa- that “functional outcome data that [are] applicable to patients
cilities, and long-term care hospitals (LTCHs). Payments for treated across different clinical settings and applications,
each are based on different classification systems, although more efficient and less costly to administer, and sufficiently
therapy services remain included in the lump sum. The basic precise to detect clinically meaningful changes in functional
payment in each facility may also be adjusted by the factors outcomes (p. 23)”12 will be developed.
listed in the previous paragraph. Recent legislation instructed CMS to investigate this
The initial PPS has encouraged the use of modified ver- problem. By 2010, CMS had begun addressing the need for
sions of this payment system by nongovernment third- a standardized assessment tool that would be applied from
party payers. Today, most inpatient services are covered the acute-care hospital to four possible post–acute-care
by prospectively paid contracts with hospitals and health settings (IRFs, skilled nursing facilities [SNFs], HHAs,
care facilities. Services not covered by prospective pay- and LTCHs). Named the Continuity Assessment Record
ment arrangements are often covered by per diem contract and Evaluation, or CARE, tool, it was being used only in
arrangements that pay a flat rate per day for inpatient Demonstration Projects at the time of this writing. Similar
services. to the other instruments discussed (IRF-PAI, Minimum
Data Set 2.0 [MDS], and OASIS), the CARE tool is initiated
Outcome Measures at admission and completed at discharge. It incorporates
CMS has developed different methods of determining pay- demographics, medical status, cognitive status, and func-
ment in the PPS for the various settings. In almost every case, tional abilities. With the electronic medical record, a stan-
the initial status of the patient determines the amount of money dardized assessment tool across the continuum of care, and
the facility will receive. Generally, the more complicated the Web-based technology, CMS will then be able to deter-
patient’s condition, the higher the reimbursement rate. The mine and compare specific case-mix outcomes and costs
facility must then have a system to create a preliminary com- relative to the particular discharge status and setting. This
prehensive “snapshot” of patients within days of their arrival will ultimately be able to guide payment policy.
at that particular setting. To ensure that patients receive the
same standard of care and are treated equally, all patients are Inpatient Rehabilitation Facility–Patient
assessed by use of the Medicare preferred tools, even if they Assessment Instrument
do not have Medicare coverage. In an IRF, the IRF-PAI is required by CMS as part of its
In the inpatient acute rehabilitation facility, the preferred PPS. On admission to the IRF, the patient is assigned an
tool is the Inpatient Rehabilitation Facility–Patient Assessment Impairment Group Code (IGC), which is the condition
Instrument (IRF-PAI) to assist in determining the payment requiring a rehabilitation stay. “The IRF PPS uses data from
amount. The Resident Assessment Instrument (RAI) is the the IRF-PAI to classify patients into distinct groups based on
primary tool in subacute and skilled nursing facilities, and clinical characteristics and expected resource needs. These
OASIS (Outcome and Assessment Information Set) is used in distinct groups are called ‘case-mix groups’ or ‘CMGs.’ To
HHAs. These tools are discussed in more detail later in this classify a ‘typical patient,’ one who has a length of stay of
chapter. more than 3 days, receives a full course of inpatient rehabili-
With each of these outcome measurement tools and in tation care, and is discharged to the community, into a CMG,
each setting, therapy documentation in the medical record the admission IGC, the admission motor and cognitive
must validate the tool’s ratings. Each tool is completed when scores from the FIM,* and the age at admission are required.
the patient is admitted to the program and also at the time of The CMG and comorbidity tier determine the unadjusted
discharge. As the patient progresses, it is very important for federal prospective payment rate.”13
The Patient Assessment Instrument is best known for is a training manual available to assist the clinician in complet-
having incorporated the Functional Independence Measure ing this form.15
(FIM)14 along with function modifiers, quality indicators, A similar data or documentation form is used in pediat-
and additional patient information. “The FIM instrument is rics: the WeeFIM II System. “The WeeFIM instrument was
a basic indicator of severity of disability . . . . The need for developed to measure the need for assistance and the sever-
assistance (burden of care) translates to the time/energy that ity of disability in children between the ages of 6 months
another person must expend to serve the dependent needs of and 7 years. The WeeFIM instrument may be used with
the disabled individual so that the individual can achieve and children above the age of 7 years as long as their functional
maintain a certain quality of life. The FIM instrument is a abilities, as measured by the WeeFIM instrument, are below
measure of disability, not impairment. The FIM instrument those expected of children aged 7 who do not have disabili-
is intended to measure what the person with the disability ties. The WeeFIM instrument consists of a minimal data set
actually does, whatever the diagnosis or impairment, not of 18 items that measure functional performance in three
what (s)he ought to be able to do, or might be able to do domains: self-care, mobility, and cognition.”16
under different circumstances (p. III-1).”*15
Demographic, payer, medical, admission, and discharge Resident Assessment Instrument
information are included in the IRF-PAI. “The function modi- In SNFs, the PPS is designed to cover the costs of providing
fiers assist in the scoring of related FIM items and provide care on a daily basis. This includes payment for ancillary ser-
explicit information as to how a FIM score has been deter- vices. The BBA required that the payments be adjusted for
mined.”15 These modifiers apply to bowel and bladder control, case mix. Case mix refers to the diversity of patients/residents
tub and shower transfers, and distances covered by walking or on the basis of their complexity of medical problems or need
in a wheelchair. The FIM instrument specifically addresses the for resources. This accounts for the increase in costs of com-
amount of assistance required for the functional activities of plicated or involved cases. It ensures that facilities accept a
eating; grooming; bathing; upper body and lower body dress- variety of patients, rather than only those who require the least
ing; toileting; bladder and bowel management; bed, chair, and amount of services. In SNFs, a method of classifying each
wheelchair transfers; toilet transfers; tub transfers; shower resident was developed to adjust the payments relative to the
transfers; locomotion via walking or wheelchair; stairs; com- staff resources required to care for and to provide therapy to
prehension; expression; social interaction; problem solving, the residents. There is a higher cost associated with residents
and memory. Each has its own algorithm to determine the FIM who require more resources or one-on-one care by staff. The
score. Quality indicators include respiratory status, pain, pres- facility should be reimbursed at a higher rate for these resi-
sure ulcers, and safety (balance and falls).15 dents than for those who are more independent. Facilities are
The FIM instrument has a total of seven levels of also reimbursed at a higher rate for residents who are receiving
assistance. These are divided into two main categories, skilled services. All this information is acquired in the RAI,
Independent—No Helper, and Dependent—Requires Helper. which is composed of three parts: the MDS, the Resident
The two items in Independent—No Helper consist of Assessment Protocols (RAPs), and the Utilization Guidelines.
Complete Independence—7 and Modified Independence—6. The RAI provides a structured method for the facility to create
The highest score of 7 indicates that the patient completes the individualized care plans, to communicate on an internal and
task safely, in a timely manner, and without any assistive external basis, and to monitor quality performance. The MDS
devices. A score of 6 means that the patient requires a device indicators are factored into the calculations for the Resource
or takes extra time or safety is an issue. The Dependent— Utilization Groups, version III (RUG-III). RUG-III is the com-
Requires Helper category is further divided into two sections: plex classification system used by CMS to determine the daily
the Modified Dependence—5, 4, and 3 scores, in which the payment rate for the SNF PPS. RUG-III, in addition to many
patient provides 50% or more of the effort, and the Complete other categories, has a Rehabilitation category with five sub-
Dependence—2 and 1 scores, in which the patient’s effort is categories that describe the intensity of therapy received. The
less than 50%. Supervision or setup, 5, denotes no physical subcategories are determined by the number of minutes of
contact with the patient; the patient requires coaxing or some- therapy and the number of therapies each week.
one standing by, or a helper may need to set up the equipment. The MDS is completed on a set schedule. After the initial
Minimal contact assistance, 4, includes touching; the patient 5-day, then 14-, 30-, 60-, and 90-day reports, the MDS is
is doing 75% or more of the activity. Moderate assistance, 3, filed on a quarterly and annual basis. The MDS requires in-
indicates that more than touching is required, with the patient put from residents, their families, physicians, therapists, and
giving 50% to 74% effort. Maximal assistance, 2, has the dieticians. Facility staff from direct care, social services,
patient supplying 25% to 49% of the effort. In Total assistance, activities, billing, and admissions is also consulted. The
1, the patient performs less than 25% of the workload. There resident’s performance over the entire 24-hour day is re-
viewed and recorded to create an individual picture of
strengths and needs. The MDS includes a complete review
* of the resident’s health, sensory systems, activity levels,
Copyright © 2001, 2002 UB Foundation Activities, Inc. (UBFA, Inc.) for
compilation rights; no copyrights claimed in U.S. Government works included behaviors, continence, activities of daily living (ADLs),
in Section I, portions of Section IV, Appendices I and K, and portions of physical and functional status, medications, procedures, and
Appendices B, C, E, G, H, and J. All other copyrights are reserved to their discharge plans. Although the MDS assesses activities simi-
respective owners. Copyright © 1993-2001 UB Foundation Activities, Inc. for lar to those of the FIM, the format is quite different. The
the FIM Data Set, Measurement Scale, Impairment Codes, and refinements
thereto for the IRF-PAI, and for the Guide for the Uniform Data Set for
Functional Status section is composed of Activities of Daily
Medical Rehabilitation, as incorporated or referenced herein. The FIM mark Living Assistance, Bathing, Balance during Transitions and
is owned by UBFA, Inc. Walking, Functional Limitations in Range of Motion, Mobility
Devices, and Functional Rehabilitation Potential. The several variables, including geographical differences in
Activities of Daily Living Self-Performance subcategory of wages, outliers, and the health condition and care needs of the
Activities of Daily Living Assistance includes bed mobility, patient. The latter, also referred to as the case mix, is deter-
transfers, walk in room, walk in corridor, locomotion on mined by items in the Outcome and Assessment Information
unit, locomotion off unit, dressing, eating, toilet use, and Set. On January 1, 2010, HHAs began using OASIS-C
personal hygiene. The scoring system is based on an activity version 2.00 at the direction of CMS.
occurring three or more times. Use code 0 for Independent, “The Outcome and Assessment Information Set (OASIS) is
no help or staff oversight; 1 for Supervision—oversight, a group of data elements that represent core items of a compre-
encouragement, or cueing; 2 for Limited assistance if the hensive assessment for an adult home care patient and form
resident is highly involved in the activity; 3 for Extensive the basis for measuring patient outcomes for purposes of out-
assistance if the resident is involved in the activity and staff come-based quality improvement. The OASIS is a key compo-
members provide weight-bearing support; and 4 for Total nent of Medicare’s partnership with the home care industry to
dependence if full staff performance is required every time. foster and monitor improved home health care outcomes. The
This section has a separate but related area to record the goal was not to produce a comprehensive assessment instru-
ADL Support Provided. In this case, the coding is 0 for no ment, but to provide a set of data items necessary for measur-
setup or physical help from staff; 1 for setup help only; 2 for ing patient outcomes and essential for assessment—which
one-person physical assistance; and 3 for physical assistance home health agencies (HHAs) in turn could augment as they
from two or more persons. The MDS has a training manual judge necessary. Overall, the OASIS items have utility for
available to assist with completing the instrument.17 outcome monitoring, clinical assessment, care planning, and
The RAPs are used to identify problems and to create other internal agency-level applications.”19
individualized care plans. Certain responses from the RAPs The OASIS includes sections on patient demographics,
initiate triggers, which identify potential or actual problems. clinical record items, patient history and diagnoses, living
From the triggers, areas of concern are further researched arrangements, sensory status, integumentary status, respiratory
to determine complications and risk factors in addition to status, cardiac status, elimination status, neuro/emotional/
noting the need for referrals to appropriate health profes- behavioral status, ADLs and instrumental activities of daily
sionals. Utilization Guidelines are necessary to analyze the living (IADLs), medications, care management, and therapy
information gathered from the RAPs. need and POC. The ADL/IADL category is divided into
In response to providers, consumers, and others, CMS grooming, upper body dressing, lower body dressing, bathing,
implemented the new and improved MDS Version 3.0 effec- toilet transferring, toileting hygiene, transferring, ambulation/
tive October 1, 2010. This redesigned version incorporated locomotion, feeding or eating, ability to plan and prepare light
many significant changes. Based on a RAND/Harvard team meals, ability to use telephone, prior functioning ADL/IADL,
effort, the MDS 3.0 is much easier to read and accomplishes and fall risk assessment. In the OASIS format, choices to
several goals. These include improved resident input, im- describe patient function vary with the activity. Grooming,
proved accuracy and reliability, increased efficiency, and upper and lower body dressing, and toileting hygiene scales
improved staff satisfaction and perception of clinical utility. are 0 for independent; 1 for setup, no assistance; 2 if someone
A new development with MDS 3.0 is the addition of the must help with the activity; and 3 if the patient is totally
Care Area Assessment (CAA) Process to assist with the dependent. With bathing, the range is from 0, or independent,
interpretation of the information gathered from the MDS. As to 6, bathed totally by another person. For transfers, 0 is
of October 2010, the RAI components are the MDS 3.0, the independent and 5 is bedfast, unable to move self. Ambulation/
CAA process and the RAI utilization guidelines. An updated locomotion scores are from 0, able to independently walk on
classification system, RUG-IV, was scheduled to be intro- even and uneven surfaces, and negotiate stairs with or without
duced at the same time as the MDS 3.0. However, while railings and no device, to 6, bedfast, unable to ambulate or be
Section 10325 of the Affordable Care Act allowed CMS to up in a chair. Feeding or eating starts with 0 for able to inde-
implement the MDS 3.0 as scheduled, this same Section pendently feed self and extends to 5, unable to take in nutrients
mandated a delay of the implementation of the RUG-IV orally or by tube feeding. Ability to plan and prepare light
classification system by one year. Portions of RUG-IV were meals (make cereal or sandwich or reheat delivered meals
implemented on an interim basis on October 1, 2010. The safely) ranges from 0 for independent or was able to but did not
purpose of RUG-IV is to more accurately allocate payments. before this admission to 2 for unable. Ability to use telephone
RUG-III bases payments on predicted therapy minutes from is 0 for able to dial numbers and answer calls appropriately
the MDS, causing inaccurate classifications and payments to and as desired to 5, totally unable to use the telephone. Prior
SNFs in some instances. RUG-IV calculates the average functioning requests information about self-care, ambulation,
daily number of therapy minutes based on the actual number transfer, and household tasks. Finally, the fall risk assessment
of minutes provided to assign patients to Rehabilitation cat- asks if the patient is at risk for falls. A score of 0 means that no
egories. The number of minutes of therapy received affects multifactor fall risk assessment was conducted, 1 indicates that
the reimbursement rate. This is why it is very important to the fall assessment was completed but does not indicate a risk
correctly document the time spent treating the resident in for falls, and 2 indicates that the patient is at risk for falls. The
addition to the resident’s functional status.18 care management section assesses the level of caregiver ability
and willingness to provide assistance if needed in activities
Outcome and Assessment Information Set ranging from ADL assistance to patient advocacy. Note that
The home health PPS, introduced with the BBA, uses a simi- although the OASIS is very precise, it also makes it difficult
lar system as do the acute-care facilities, IRFs, and SNFs. to measure progress. For example, in the ambulation category,
There is a standard base payment rate adjusted according to a score of 4 indicates “chairfast, unable to ambulate but is
able to wheel self independently”; 3 indicates “able to walk appropriate. Fiscal intermediaries and Medicare carriers are
only with the supervision or assistance of another person at responsible for acceptance or denial of claims made to them
all times”; and 2 indicates “requires use of a two-handed by the acknowledged provider of services. The standards
device (e.g., walker or crutches) to walk alone on a level pertaining to “reasonable and necessary” are available from
surface and/or requires human supervision or assistance to individual fiscal intermediaries and Medicare carriers as
negotiate stairs or steps or uneven surfaces.”20 This is another local coverage determinations (LCDs).
example of the importance of documentation to report signifi- The LCD standards and other helpful information are
cant improvement in therapy. available through specific websites or through the Medicare
Coverage page of the CMS website (www.cms.gov). Non-
DOCUMENTATION RECOMMENDATIONS government third-party payers can follow guidelines of their
Documentation is communication of the professional judg- own design. These may or may not be similar to Medicare
ment used to establish a patient’s POC. Documentation guidelines. In general, when a therapist’s documentation
should demonstrate the integration of the elements of patient meets Medicare requirements, it satisfies the expectations of
management that determine the services that, in the profes- other third-party payers as well.
sional opinion of the therapist, will provide the best possible There are other regulating organizations, such as The Joint
outcome for the patient. Commission, licensing boards, or state departments of health
Medicare guidelines provide the minimum context stan- services, that set documentation standards to protect consum-
dards required for adequate documentation. Satisfying min- ers of health care services. It is important that therapists be
imum guidelines is not sufficient for the therapist who is aware of all documentation required by the regulatory agencies
thinking critically. This therapist should always be asking associated with their patients when documenting in the medical
determinative questions (Box 10-1). record. Because of the unique requirements of payers at the
When the answer to whether therapy is necessary is “no,” various state, county, and local levels, this section of the chapter
document the reason why services will not be rendered. This primarily addresses CMS guidelines for inpatient facilities.
will explain the therapist’s perspective. Generally this is an Medicare requires specific information with bills that are
obvious decision because the therapist is unable to establish submitted for payment. Following these rules will facilitate
any goals. reimbursement for services because any deviation may be used
When the answer is “yes,” the therapist must be able to as a reason for denial of payment. Proper documentation is al-
answer the additional questions in Box 10-1. These impor- ways necessary for the appeals process when a claim has been
tant questions justify treatment and payment. The patient denied. Medicare billing must include the following, which are
may have insurance or may be receiving federal, state, or appropriate for both inpatient and outpatient settings.
county aid. Either way, the therapist must not forget that The patient must be eligible for therapy services on the
someone is responsible for paying the bill and that some- basis of an active written POC. The POC must be ordered or
one deserves a meaningful and beneficial product in return. certified by a physician or by another licensed independent
The American Physical Therapy Association (APTA) has practitioner. Time periods for certification and requirements
published Guidelines: Physical Therapy Documentation of for return physician visits may vary. These requirements may
Patient/Client Management.21 These guidelines can be found be different in states with direct access to physical therapy.
on the APTA website (www.apta.org) under About Us— In addition, therapy must be a reasonable and necessary
Policies and Bylaws—Board of Directors Positions and treatment for the particular illness or injury. Reasonable
Policies, Section I—Practice. Although the general guide- and necessary allows a broad interpretation, which is why
lines in Box 10-2 were written as part of an APTA document, documentation becomes so important. The following are
they set a standard for therapists in the health care industry. components that establish medical necessity:
In addition to following APTA’s Guidelines: Physical 1. Intervention, as related to the specific profession, is an
Therapy Documentation of Patient/Client Management, the accepted standard of care for this diagnosis. There are
medical record must follow requirements set forth by other specific and effective interventions (evidence-based
agencies and regulating bodies. CMS sets minimum stan- practice) successfully used to treat the condition.
dards for documentation that are implemented on the local 2. The treatments require the skilled services of a profes-
level by fiscal intermediaries or Medicare carriers, as sional. Knowledge and judgment are required because
of the complexity of the problem and sophistication of
the therapist’s unique body of knowledge.
3. Therapeutic intervention creates significant improve-
BOX 10-1 n DETERMINATIVE QUESTIONS ment, demonstrated by measurable gains in range of
motion, strength, function, level of assistance, and so on.
Is there any therapy-related skilled service that this patient 4. The amount, frequency, and duration of treatment are
requires? reasonable. This is clarified by a POC with short- and
If yes, what is the unique professional contribution to this long-term goals, predicted end of treatment, and rea-
person’s rehabilitation? sonable potential to achieve the stated goals. Weekly
Are therapy services medically reasonable and necessary reassessments or changes in the patient’s condition
and able to be correctly administered in a timely and will require the plan to be modified as necessary.
beneficial way? Reasonable and necessary are key words for therapists
What are the therapy services and on what schedule will to synthesize as part of the critical thinking process. Two
they be administered? examples are given to assist the reader to further analyze the
meaning of these words.
BOX 10-2 n GENERAL GUIDELINES
Documentation is required for every visit or encounter. must be authenticated by the PT who provided the
All documentation must comply with the applicable service.
jurisdictional and regulatory requirements. n Documentation of intervention in visit or encounter
All handwritten entries shall be made in ink and will include notes must be authenticated by the PT or PTA who
original signatures. Electronic entries are made with ap- provided the service.
propriate security and confidentiality provisions. n Documentation by PT or PTA graduates or other
Charting errors should be corrected by drawing a single PTs and PTAs pending receipt of an unrestricted
line through the error and initialing and dating the chart license shall be authenticated by a licensed PT, or,
or through the appropriate mechanism for electronic when permissible by law, documentation by PTA
documentation that clearly indicates that a change was graduates may be authenticated by a PTA.
made without deletion of the original record. n Documentation by students in PT or PTA programs
All documentation must include adequate identification of must be additionally authenticated by the PT, or,
the patient/client and the physical therapist (PT) or when permissible by law, documentation by PTA
physical therapist assistant (PTA) (or occupational students may be authenticated by a PTA.
therapist or occupational therapist assistant): Documentation should include the referral mechanism by
n The patient’s/client’s full name and identification which physical therapy services are initiated.
number, if applicable, must be included on all offi- Examples include:
cial documents. n Self-referral or direct access
n All entries must be dated and authenticated with the n Request for consultation from another practitioner
provider’s full name and appropriate designation*: Documentation should include indication of no shows and
n Documentation of examination, evaluation, diagno- cancellations.21
sis, prognosis, plan of care, and discharge summary
*OT or occupational therapist assistant should use the same documentation system and protocol. Space prohibited using all professionals’ initials.
Recovery Audit Contractors

CASE STUDY 10-1 As part of the Medicare Modernization Act of 2003, Congress
A 60-year-old active, independent woman who falls and frac- initiated a Recovery Audit Contractor (RAC) demonstration
tures her humerus may be, understandably, a little wobbly from project to fight fraud, waste, and abuse in the Medicare sys-
the trauma, but she will not need therapy to achieve indepen- tem. “The demonstration resulted in over $900 million in
dent mobility. However, the situation changes completely when overpayments being returned to the Medicare Trust Fund
the same woman has an existing right hemiparesis, requires the between 2005 and 2008 and nearly $38 million in underpay-
use of a cane for balance, and then fractures her left humerus. ments returned to health care providers.”22 The RACs were so
Now therapy would be appropriate to address ADLs, safety, effective that Congressional legislation made them permanent
and gait to assist her in regaining her independence. Both in Section 302 of the Tax Relief and Health Care Act of 2006.
physical and occupational therapists may be treating this This Act expanded the RAC program to cover all 50 states
patient, and each professional needs to be following similar in January 1, 2010. These audits were designed with three
processes for thorough documentation. purposes in mind: first, to protect Medicare beneficiaries;
second, to protect taxpayer dollars used to make payments;
and third, to ensure that claims were paid only for services
that met Medicare requirements. The RAC teams, which are
required to include nurses and therapists, are paid on a contin-
gency fee basis in which the fee is returned if the provider
CASE STUDY 10-2 wins at any level of appeal.
“The goal of the recovery audit program is to identify
A patient who lives independently and is admitted to the
improper payments made on claims of health care services
hospital with a ruptured appendix would not usually require
provided to Medicare beneficiaries. Improper payments may
therapy services. The patient would need to spend time out
be overpayments or underpayments. Overpayments can occur
of bed and to ambulate in the hallways to regain endurance,
when health care providers submit claims that do not meet
but this may be done with nursing staff or family. If the
Medicare’s coding or medical necessity policies. Underpay-
same patient had comorbidities such as multiple sclerosis
ments can occur when health care providers submit claims for
or Parkinson disease that were exacerbated by the hospital-
a simple procedure but the medical record reveals that a more
ization, then therapy would be warranted. Therapy would
complicated procedure was actually performed. Health care
establish a POC to address the issues that prevent a return to
providers that might be reviewed include hospitals, physician
the prior level of function.
practices, nursing homes, home health agencies, durable med-
ical equipment suppliers and any other provider or supplier
that bills Medicare Parts A and B.”22 Because a complex ever before. The medical record is available to insurance
review requires the medical record, documenting comprehen- case managers and medical reviewers who are outside the
sible reasonable and necessary skilled services is critical. medical facility. Patients may share their records with their
families, new physicians and therapists, or even attorneys.
Skilled Services Because of the various interests and needs of these diverse
People who have experienced trauma or a disease process groups, it is necessary to be concise, legible, objective, and
that affects their ability to move or function would be read- professional when documenting. Remember that no docu-
ily labeled candidates for therapy services. Therapy inter- mentation can be released to others without a patient’s
vention should be easy to justify. The challenge is twofold. signed release of information form on file in the patient’s
The therapist must (1) be able to identify and then substanti- medical record.
ate the need for skilled services and (2) be sure that the Therapists should realize that it is very possible that their
documentation allows other parties to follow and understand notes may be subpoenaed in the future as part of a lawsuit.
what has been provided. The following example of docu- The person who is the keeper of the records at the time of
mentation compares two sentences that a reviewer might the case may have to go to court and explain, via another’s
read: “Gait training to facilitate weight shifting onto the documentation, what was done for the patient, or it is pos-
affected extremity with minimal assistance required for sible that the therapist may be reading her own notes several
safety” versus “The patient ambulated down the hall.” The years later while sitting in the witness box.
first sentence conveys the need for the unique and necessary When documenting, be aware of the following important
skills of a PT. The second fails to even suggest the presence and sensitive areas. Remember that therapists receive a long
of a therapist. Avoid referring to skilled physical or occupa- and expensive education to enable them to write in the offi-
tional therapy, which then infers that unskilled therapy is cial legal record. Reviewers are basing their decision to pay
also available. Unskilled therapy, for which a reviewer for therapy on what has been recorded; be mindful of the
should deny payment for services, could easily be repre- need to meet criteria for skilled services. Patients and their
sented by “The patient ambulated down the hall.” Skilled entire medical team appreciate professional interventions
services, on the other hand, reflect therapy provided by and professional documentation.
qualified therapists with clinical expertise and knowledge.
A list of the interventions provided does not demonstrate Patient Advocacy
skilled care. A therapist must include the level and type of The therapist is the patient’s advocate. As such, the therapist
skilled assistance given, clinical decision making or prob- should champion the best care for the patient. This may mean
lem solving involved, and continued analysis of patient consulting professionals in other disciplines or facilitating
progress. An explanation of why specific interventions are transfers to other facilities. It is the clinician’s responsibility
chosen and what makes them still necessary is also required. to ensure that the record reflects the patient’s best interests.
Documentation of the therapist’s observations of the patient’s Do not let therapy notes hinder the patient’s forward progress
movement and activity before, during, and after an interven- in any way. Patients with neurological conditions may have
tion, the patient’s specific response to the intervention, and deficits that affect their orientation, judgment, initiation, abil-
the relationship of progress to goals are additional examples ity to respond or comprehend, or insight. They may have vi-
of skilled service. sual-perceptual or other sensory problems that affect their
Duplication of services is also a concern when there is ability to participate in therapy. Their ability to process infor-
collaboration across the disciplines. Many patients will ben- mation may be delayed. None of these components are rea-
efit from treatments in which both OTs and PTs are present. sons to withhold treatment, but they may affect the time re-
However, if both therapists document, “Sat patient at edge of quired to achieve appropriate goals. These patients can and
bed to work on balance,” reviewers could easily question will progress with a creative, patient, and knowledgeable
whether both therapists did the same thing at the same time. therapist.
The reviewers might then have a problem approving payment
for the care provided, with a possibility that both services Timeliness
would be denied payment. There are no questions of duplica- Whenever possible, document immediately after seeing the
tion when the medical record states that the PT treatment ses- patient. This ensures that the session is recorded accurately.
sion included “instruction and demonstration of strategies for It is better to report the results of consultations, test results,
dynamic postural adjustments” and the OT treatment session or phone calls rather than writing that the therapist intends
was directed toward “ADL training with emphasis on dress- to take action. The latter leaves the reader wondering if any-
ing.” The same is true for speech pathologists, OTs, and/or PTs thing happened until the relevant findings are included in the
in a multidisciplinary approach. A treatment session may have medical record.
one therapist facilitating head control and midline orientation,
another addressing upper extremity function and coordination, Motivation
and a therapist from a third discipline focusing on the ability A therapist should believe that a patient is motivated to im-
to swallow. Be sure the documentation reflects the specific prove. Sometimes there is damage to the brain that affects
skills and knowledge related to each therapy. initiation, insight, or judgment; sometimes there is depres-
sion, pain, or another medical reason. It is the therapist’s
DOCUMENTATION: A LEGAL DOCUMENT responsibility to find the key to unlock the patient’s ability
The medical record is a legal document that is read by many to participate. Do not record that the patient is unmotivated.
people who are not therapists. Patients have much greater The lack of motivation usually belongs to the therapist. (See
access to and interest in their medical records today than Chapter 5 for additional information.)
Personal Opinion patient or caregivers must be able to understand the infor-

The therapist’s documentation must be absolutely objective. The mation. For example, documenting that the patient is blind
PT or OT may provide direct quotations or accurately record an and that he was given written handouts would be inappropri-
event that happened during a therapy session to allow the reader to ate, unless it was also documented that the family was
make his or her own judgment. It is very important to never let trained with the materials provided. With any teaching, it
personal feelings about the patient enter the medical record. Bias
and antagonism on the part of the therapist may leave the medical
is important to record what was taught, the response, and
record open to speculation and become a problem if litigation oc- how well the new information was comprehended, either by
curs. If the reader of the documentation senses animosity, certainly return demonstration or by correct responses to questioning.
the patient will too! The validity of the comments and the therapy Indicate whether the patient will be safe alone or with the
provided become questionable. Always keep personal opinions out specified caregivers after the training or whether additional
of official documentation. education is necessary.
Abbreviations Patient Rights
Be careful with abbreviations because the individual docu- Patients have many rights, including the right to be treated
menting may be the only one who understands what is with respect and dignity. Use terms that focus on the patient as
being stated. Most facilities have an approved abbrevia- a person; avoid labeling patients by their diagnosis. Informed
tions list; use it! The meaning of what has been written consent, risks and benefits of treatment, and confidentiality
will change according to the reader’s interpretation of the are extremely important, both ethically and legally. Patients
abbreviations used. It is possible that a reviewer will read always have the right to refuse treatment. Therapy cannot be
the medical record and either find it incomprehensible or forced on individuals against their will. Therapists offer a ser-
completely misunderstand the original intent. The purpose vice that medical and health care professionals know will be
of documentation is to provide information. Claims may beneficial, but patients are responsible for paying for their
be denied because the reviewer does not understand the health care and they must be given a choice.
abbreviations used.
Accountability
Pain Whenever possible, include references to other health care
The Joint Commission has brought the patient’s pain level to team members in order to demonstrate an interdisciplinary
the forefront, making a patient’s pain level the “fifth vital approach to patient care. Perform a thorough review of the
sign.” It is required that a comprehensive pain assessment medical record. Information from other team members can
appropriate for the patient’s age and condition be recorded at aid therapists in their understanding of the patient’s situa-
regular intervals. The most common pain scale is 0 to 10, tion. Inquire about the patient’s goals for therapy treatment
with 0 signifying no pain and 10 signifying the worst pain the to incorporate into the POC. Each case must be examined
patient can imagine. To further explain the scale to the pa- individually. No two cases should be assumed to have the
tient, the numbers 1 to 3 correspond to minimal pain, 4 to 7 same problems and the same plans for resolution. What
to moderate pain, and 8 to 10 to severe pain. A score of 4 or appears to be a routine assessment may present subtle and
higher requires immediate attention. It is important to ex- intricate challenges to both the patient and the therapist. Be
plain to the patient that his or her pain scale response is ac- sure to take a critical look at what has already been entered
cepted at face value and belongs only to the patient; the pa- into the medical record. Question any findings that do not
tient’s numbers are not compared with those of anyone else. make sense, especially if previous documentation does not
For children ages 3 years and older, the Wong-Baker FACES correspond to all the information and clinical symptoms
Pain Rating Scale23 may be easier to understand. The purpose present. Take the initiative to solve problems and investigate
of a pain scale is to ascertain the effectiveness of pain medi- inconsistencies. Patients depend on the skills and knowledge
cation or pain-reducing modalities. It is important to docu- of their therapists. Therapists must be accountable for their
ment the pain number at rest and during treatment, whether own documentation.
pain interferes with or prohibits participation in therapy, and
what the therapist has done to remedy the painful situation.
CONTINUUM OF CARE
(See Chapters 5 and 32 for additional information.)
Acute Care
Reassessments Different settings require a change in the focus of docu-
Reassessments are done on a weekly basis or sooner if short- mentation. It is important for the therapist to understand
term goals have been met or there has been a change in status. this concept and to modify documentation as necessary. In
The reassessment should describe the patient’s situation and the acute-care setting, discharge planning begins as soon
address the short-term goals, either explaining why the goals as the patient is admitted. The primary role of the therapist
have not been met or setting new goals when the previous ones is to assess the patient to determine the next level of care
have been achieved. In addition to addressing the short-term and to introduce therapeutic interventions to expedite that
goals, the reassessment provides the opportunity to clarify the process. Time is of the essence; the therapist may have
treatment plan, treatment frequency, and duration of treatment. only one or two visits to make a discharge recommenda-
tion and fewer than five visits to achieve initial short-term
Patient, Family, and Caregiver Training goals.
The education provided must be appropriate to the patient’s Depending on various circumstances, patients may trans-
abilities. It is important to assess the learning style and barri- fer from the acute-care hospital to home either directly or
ers to learning, then adjust the teaching accordingly. The indirectly by way of acute inpatient rehabilitation or an SNF.
Although the primary goal is to return the patient home and patient improves, acute inpatient rehabilitation may then be
continue therapy there or in an outpatient setting, some pa- considered.
tients may never leave the SNF. The emphasis is on safety.
The patient must be safe in her or his own environment. Home Health
Caregivers, if necessary, must be capable of safely assisting Patients who are discharged from hospitals and facilities
the patient. It is important to realize that patients may not may still require additional therapy. They may not have the
access every level of care, or they may require a combina- ability or the endurance to travel to an outpatient setting and
tion of settings. In each location, the treatment techniques then also participate in the various therapies. In these cases,
may vary and the short-term goals will be different, but the home health therapists provide the solution. To receive
same documentation guidelines apply. The following para- home therapy, a patient must be homebound. According to
graphs assume that the patient is initially admitted to an CMS, the definition of homebound is “Normally unable to
acute-care hospital and then describe the possible discharge leave home unassisted. To be homebound means that leav-
options. ing home takes considerable and taxing effort. A person may
leave home for medical treatment or short, infrequent ab-
Subacute Care sences for nonmedical reasons, such as a trip to the barber
A patient who is admitted to the hospital and then requires or to attend religious service. A need for adult day care
the use of both a ventilator and a feeding tube may benefit doesn’t keep you from getting home health care.”24 Docu-
from a subacute setting before moving on to acute inpa- mentation must explain why the patient is homebound. As
tient rehabilitation. In the subacute setting, respiratory the patient improves, this becomes more difficult and facili-
therapists and the nursing staff have key roles. Patients tates a decision for outpatient therapy or discontinuation of
who have had respiratory failure in addition to their neu- therapy services altogether.
rological deficits require a much slower pace to achieve
their rehabilitation goals (see Chapter 3). These patients, Transitional Living Centers
with extremely impaired endurance and low functional Some communities are fortunate to have a transitional liv-
levels, may stay in subacute settings for several months ing center (TLC) available for clients to move beyond IRFs
before they develop sufficient strength to progress to acute and into “real-world” situations. TLCs are community-
inpatient rehabilitation or return home. Short-term goals based neurocognitive rehabilitation programs where the
are set month to month, in contrast to acute-care hospitals, standard of care includes occupational, physical, and speech
where short-term goals may be met in a matter of visits therapy; case management; and neuropsychology services.
or days. This treatment team pulls weekly documentation into a
combined, goal-oriented individualized rehabilitation plan
Acute Inpatient Rehabilitation with summaries prepared for the payer source, physicians,
The Commission on Accreditation of Rehabilitation Facili- family, and team. TLCs provide “custom-designed” life
ties (CARF) monitors quality standards for acute inpatient plans to facilitate reentry into home, school, or vocational
rehabilitation care and is respected at an international level. settings. TLCs have been extremely successful as a way
Patients admitted to rehabilitation facilities accredited by for older adolescents and young adults with neurological
this commission must meet several requirements. First, the problems to progress from a rehabilitation center back into
patient must be medically stable and able to participate in at society.
least 3 hours of therapy throughout the day. The overall
medical stability must still require 24-hour nursing care Outpatient Therapy
and physician monitoring for medical diagnoses such as Patients who have progressed to a level where they can eas-
hypertension or diabetes. Second, the physical disability is ily leave home usually prefer to travel to therapy depart-
such that the patient must need at least two of the three ments or offices for treatment. Once in outpatient therapy,
rehabilitation disciplines of speech, occupational, and phys- patients receive the fine-tuning necessary to maximize their
ical therapy. Finally, the patient must have a community potential function. Usually these patients benefit from a
discharge plan. The discharge plan is imperative because gradually decreasing frequency with an increasing emphasis
acute inpatient rehabilitation is a dynamic process and on independent home programs. Although the guidelines for
patients will be discharged from this setting. A patient who outpatient and inpatient documentation are the same, the
was living alone before hospitalization but whose long-term payment systems for outpatient services are quite different
goals do not include independence may not be eligible for and will be covered in detail later in this chapter.
acute inpatient rehabilitation care.
Therapy and Discharge Planning
Skilled Nursing Facility Therapists in hospitals have the tremendous responsibility
If the patient does not meet the requirements for acute inpa- of seeing patients just a few times and making recommenda-
tient rehabilitation or if the patient does not have financial, tions that may affect the patients for the rest of their lives.
family, or other resources to enable him or her to live at These decisions are not made in a vacuum; other members
home with assistance, then an SNF may be a better option. of the health care team are involved and initial plans may be
The patient benefits by receiving rehabilitation services and amended. Often, however, the team looks to the therapists to
having a place to live. The facility is able to bill the third- determine the best discharge plan.
party payer at a higher rate than for someone who is not When discharge options are considered, there are ques-
receiving therapy services. The patient is allowed to receive tions a therapist should ask as part of the critical-thinking
therapy at a slower pace for a longer period of time. As the process (Box 10-3).
BOX 10-3 n CONSIDERING DISCHARGE n POINTS TO PONDER ABOUT

BOX 10-4
OPTIONS DISCHARGING A PATIENT
The questions a therapist should consider before making a TO HOME
discharge recommendation may include the following: Was the home environment safe before the patient entered
Is the patient capable of being successful at home, either the hospital?
alone or with selective assistance? Did the patient rely heavily on family, friends, or neighbors
If selective assistance is still needed, is outpatient for assistance?
therapy appropriate or would home health care be a Does the patient have a history of falls? (Some patients
better choice? know the paramedics by name.)
Is the patient medically and physically stable enough to Are there stairs with rails or elevators available?
proceed to an acute inpatient rehabilitation facility? Are the rooms and halls wheelchair or walker accessible?
Is the patient’s medical condition at a level that warrants What kind of assistive equipment will need to be in the home?
a skilled nursing facility? Are there healthy and available family members willing to
assist on a continuing basis?
Is there any money available to hire caregivers in the home,
and will the patient consent to this?
Are there cultural factors in the family unit that may affect
There are no simple answers to any of these questions, but caregiving?
the questions need to be asked to arrive at the best discharge Does the patient need dialysis, and if so, how will this be
plan for the patient. When trying to ascertain the best solu- accomplished?
tion, the therapist should remember that cognition is a major How compliant will the patient be with an independent
concern, as is the length of time expected for the patient to exercise program?
meet the long-term goals. The wishes of the patient and the Will it be possible and easy for the patient to travel to an
family must always be involved in the decision-making pro- outpatient program?
cess because sometimes they do not agree with each other or TO A RETIREMENT COMMUNITY
with the therapist’s recommendations. How far must the patient walk to reach the dining area, or
There are many aspects to consider. The patient’s prior level can meals be delivered to the room?
of function is essential information, followed closely by the Are assistive devices allowed in public areas of this
situation at home. The medical and surgical histories are also community?
pertinent factors. Contemplate the questions listed in Box 10-4. If not, what options have been provided or recommended by
The therapist should consider the level of responsiveness, this community?
the ability to follow commands, the prior level of function,
and the patient’s support system before making a recom-
mendation. To further challenge the therapist, insurance
coverage may affect the discharge plans. There will be cases
where particular insurance carriers will contractually man- living facility temporarily before returning to their inde-
date the patient’s discharge disposition. In rare instances, pendent living setting.
patients must wait in an acute-care hospital until they Be careful! The therapist may adversely affect a patient’s
become eligible for state or federal funding before moving disposition on the basis of the POC. For example, a therapist
on to the next level of care. in an acute-care hospital might routinely treat postoperative
In situations such as multiple fractures with non–weight patients with orthopedic problems who elect to have surgery.
bearing on bilateral lower extremities, the patient only needs These patients are expected to make major functional
time to heal before being able to participate in a rehabili- changes in just a few days. If a different patient arrives with
tation setting. Although the best-case scenario is for the a new subarachnoid hemorrhage and a maximum assist
patient to return home while recuperating, this is not always functional level, that same therapist may underestimate the
possible. The patient would then transfer to a facility for amount of assistance and the duration of care that will be
custodial care. needed for this severely involved patient. The therapist’s
Another possible discharge option is that of a retire- short-term goals might project independent mobility within
ment housing community. This plan usually includes three a 2-week time frame. If the therapist does not amend the
levels of care: the independent living setting, assisted liv- POC, then the discharge planner, insurance case manager,
ing, and a health center or SNF. People purchase a con- and physicians may decide that the patient is not making any
tract for a secure and predetermined health care future in progress at all. The patient is judged to have little to no re-
the retirement community. The contract specifies receiv- habilitation potential, when, in reality, the therapist’s POC
ing care at any and all of these levels. The members stay was inappropriate. This kind of error could essentially end
in independent living until they require medical interven- the patient’s chances for acute inpatient rehabilitation and
tion. They may slowly decline and move into assisted liv- affect the patient’s ultimate recovery level.
ing for a few years before finally settling into the skilled Less dramatic and possibly more common is the case of
nursing level of care, or they may have a medical emer- a patient who undergoes total hip arthroplasty for a hip
gency and be admitted to acute care. The hospital will fracture after an unwitnessed fall. The patient does not
then transfer them back to the community’s health center progress as quickly as the therapist would expect. The
for rehabilitation. These patients may stay in the assisted therapist must consider the possibility that this patient had
a mild stroke and then fell and fractured the hip. The patient assist the patient and influence the discharge plan by advo-
underwent workup for the obvious fracture, but the neuro- cating for a facility that offers both orthopedic and neuro-
logical symptoms went undetected by the orthopedic sur- logical rehabilitation.
geon. Sometimes the patient’s subtle medical problems are The third-party payer also has a say in the disposition of
realized only during evaluations that identify mismatches the case. Occasionally the discharge choice of the insurer,
between the medical diagnosis and the anticipated functional on the basis of the case manager’s review of the medical
skills and limitations. Open and clear lines of communica- records and the patient’s coverage, is not the therapist’s first
tion must be established between individuals working within choice for the patient. It may be possible to affect the deci-
the medical disease or pathology model and therapists sion regarding the patient’s future only if the therapist has
working on impairments, activity limitations, and participa- been a strong patient advocate and has consistently docu-
tion restrictions. The therapists have the opportunity to mented appropriately and thoroughly.
CASE STUDY 10-3

The following case illustrates the interaction of therapy on the trained and she is able to be discharged home. Because she
continuum of care and the various assessment instruments lives in a second-story apartment, is still using a wheelchair,
used in different settings. and continues to require occupational and physical therapy,
Ysabella D. is a 66-year-old woman, independent and Ysabella is eligible for home health therapy.
healthy, who has been diagnosed with atypical Guillain- Ysabella has Medicare Part A and B insurance coverage.
Barré syndrome. She is admitted to the acute-care hospital, This enables the acute-care hospital to be reimbursed on the
and subsequently respiratory failure, flaccid quadriplegia, basis of her diagnosis-related group. In this case, her long and
and cardiac arrhythmias develop. complicated stay would qualify her for the outlier adjustment,
Over the course of 5 months in the acute-care hospital, allowing the hospital to receive more money than it would
with more than one visit to intensive care, Ysabella undergoes have received for a patient with an uncomplicated Guillain-
tracheostomy and receives a feeding tube and a pacemaker. In Barré diagnosis. At the subacute facility, the initial Resident
the meantime, she also acquires pneumonia, dysphagia, and a Assessment Instrument Minimum Data Set (MDS)is com-
decubitus ulcer. Although Ysabella receives occupational, pleted after 5 days. The MDS is again completed after 14 days,
physical, and speech therapy while in the acute-care hospital, 30 days, 60 days, 90 days, and then quarterly until her transfer
she remains dependent in all areas. Because of the presence of to the short-term inpatient rehabilitation setting. Here the Inpa-
the tracheostomy and percutaneous endoscopic gastrostomy tient Rehabilitation Facility–Patient Assessment Instrument
tubes, Ysabella is transferred to a subacute setting, where she (IRF-PAI), including the Functional Independence Measure
stays for another 8 months. Here she gradually improves in (FIM) score, is completed after 3 days and at the time of dis-
strength, endurance, and function. charge. When Ysabella finally returns home, the home health
After her tracheostomy and feeding tubes are removed, therapist opens the case using the Outcome and Assessment In-
her skin has healed, and she has progressed to a regular diet, formation Set (OASIS). Each facility will be reimbursed after
Ysabella is strong enough to meet the criteria for an acute the submission of the appropriate information gathered from
inpatient rehabilitation facility and she is transferred there. each assessment and outcome instrument, assuming these tools
She stays in the short-term rehabilitation facility for another were completed correctly and there was no reason for payment
6 weeks before she reaches a minimal-assist level of care. to be denied. Proper documentation by the therapists would
At this point, she and her very supportive family have been justify all her therapy if there were to be an appeals process.
MEDICAL AND FUNCTIONAL DIAGNOSIS allow systematic codification and standardized naming of
AND INTERVENTION CODING: diseases and injuries and allows indexing of data for out-
DIAGNOSIS CODING come studies and for use in various payment, billing, and
Payment for rehabilitation services is dependent not only on electronic information formats. Health care insurance com-
the quality of the medical record produced during the course panies and government agencies require the use of ICD-9
of care but also on the accuracy of the codes used to describe for billing and payment processes and for medical records as
medical and functional diagnoses and therapeutic interven- a result of HIPAA. To track outcomes, especially functional
tions used in treatment. Third-party payers and other health outcomes, standardized diagnosis nomenclature is abso-
care system stakeholders rely on the accuracy of coding so lutely essential. In rehabilitation settings the treating thera-
that the appropriate payment policy can be applied during pist is responsible for accurate identification of the physical
the claims adjudication process. This section will introduce therapy (treating) diagnosis and any comorbidities that
the reader to the basics of diagnosis coding using ICD-9 could be factors during the course of care. Accurately iden-
codes and intervention coding using CPT codes. tifying these diagnostic codes is an essential part of the
The ICD-9-CM, or ICD-9 for short, is based on the advocacy role of the treating therapist because these coding
official version of the World Health Organization’s Ninth decisions can have significant effects on third-party payer
Revision of the International Classification of Diseases. ICD-9 decisions for paying claims for patients and clients with
classifies diagnosis, morbidity, and mortality information to potentially life-altering diseases and injuries.
Organization and Characteristics of ICD-9-CM Two terms need to be kept in mind when using ICD-9
ICD-9-CM is organized into two volumes. Volume 1 is the codes. The first is Not Elsewhere Classified (NEC). This
tabular list of ICD-9 codes and five appendices. Codes from term is used when the ICD-9-CM does not provide a code
Volume 1 are not usually used for medical and functional di- that may be as specific as the diagnosis the therapist is trying
agnoses involved with rehabilitation. Volume 2 is an alpha- to code, or when the clinician may not have enough infor-
betical list of ICD-9 codes. This listing contains a large num- mation to code to a more specific diagnosis requiring the
ber of medical and functional diagnoses that incorporate most fourth-digit subcategory. The second term is Not Otherwise
of the diagnostic terms currently in use. A group composed of Specified (NOS). This term is used when the diagnosis is
the American Hospital Association, CMS, National Center for unspecified. Again, the reader will have an opportunity
Health Statistics, and American Health Information Manage- to look at examples of both abbreviations for illustration
ment Association regularly updates ICD-9 codes, resulting in purposes.
annual editions that are updated throughout each calendar
year. When ICD-9 resources are consulted, it is important to Assigning ICD-9-CM Codes
always be sure that the most current edition is used. In most cases the therapist will start with the name of a
ICD-9 codes can be up to five digits long: at least three medical or functional diagnosis and will have to convert that
digits are to the left of the decimal and up to two digits to name to the numerical ICD-9 code. In rare cases the oppo-
the right of the decimal. The three digits to the left of the site occurs; a diagnostic code is provided and the code will
decimal define the diagnosis category, and the two avail- need to be converted to a name. For the purposes of this
able digits to the right of the decimal define more specific discussion it is assumed that a codebook is being used; how-
characteristics of the diagnosis by further defining site and ever, readers will find that many software and Internet ap-
location. We will look at several examples to illustrate the plications embed ICD-9 information within the application.
coding process (Box 10-5). When using embedded resources, it is important that the
reader refer to the text included in the codebook because
most of these applications use the “short language” form of
the code and do not tell you whether fourth- or fifth-digit
BOX 10-5 n ICD-9 CODING EXAMPLES modification is required.
Following are two examples of common neurological ICD-9 Coding Is a Five-Step Process
conditions. You will need an ICD-9-CM book to do this The following five-step process will guide the reader through
exercise. the ICD-9 coding process:
The name of the condition is the best place to begin. Step 1: Start by consulting the alphabetical index (Volume
For example, code the diagnosis complete paraplegia. 2) to identify the diagnostic category before using the
n Go to Volume 2 (alphabetical index) and look up tabular index (Volume 1). By identifying the correct
“Paraplegia, complete.” name of the diagnostic category in the alphabetical in-
n Review the listings under 344 of Volume 2 under dex, therapists will avoid coding errors that will result in
“Paraplegia.” There is no listing that matches the denied services.
term “complete.” Step 2: Identify the main medical or functional diag-
n Go to “344.1 Paraplegia” in Volume 1 (tabular nostic term or category. The alphabetical index is
index). The main entry is “344 Quadriplegia and arranged by condition. Conditions can be expressed
Paraplegia.” as nouns, adjectives, and eponyms. Some conditions
n Read the entries under 344 and find “344.1 have multiple entries under their synonyms. Be sure
Paraplegia.” Note what conditions are included to read any notes listed with the main term or cate-
and excluded by the listed codes. Read the note gory because these categories will help the reader
under “344.1 Paraplegia” that says “paralysis of identify the specific diagnostic code he or she is try-
both limbs.” This represents the closest match to ing to identify.
“complete paraplegia.” Step 3: Interpret abbreviations, cross-references, and
n “344.1 Paraplegia” is the diagnostic code. brackets. Cross-references used are “see,” “see cate-
Coding nonspecific encephalopathy: gory,” and “see also.” The abbreviations NEC and NOS
n Go to Volume 2 (alphabetical index) and look up follow main terms or subterms. Identify a tentative
“Encephalopathy.” code and locate it in the tabular index.
n Review the listings under “Encephalopathy.” There Step 4: By reading the entry in the tabular list, clinicians
is no listing that matches the term “nonspecific.” will be able to determine whether the code is at its
n Go to Volume 1 (tabular index) and look up “348.30 highest level of specificity. Assign three-digit codes
Encephalopathy.” (category code) if there are no four-digit codes within
n Read the notes and descriptions under “348.3 the code category. Assign four-digit codes (subcate-
Encephalopathy.” Note what conditions are gory codes) if there are no five-digit codes for that
included and excluded by the listed codes. category. Assign five-digit codes (fifth-digit subclas-
n “348.30 Encephalopathy, unspecified” matches sification codes) for these categories where they are
nonspecific encephalopathy most closely. available.
n “348.30 Encephalopathy, unspecified” is the Step 5: Assign the code.25
diagnostic code. Box 10-5 provides two ICD-9 coding examples.
Depending on the treatment setting, patients/clients may CPT is used to report health care provider services to
come to the therapist with diagnoses that are already coded. public and private or commercial insurance companies and
In other situations, such as in acute-care facilities and IRFs, payers. CPT codes are also used to report treatment encoun-
ICD-9 codes will be assigned by certified ICD-9 coders in ter information to government agencies and private compa-
the medical records department. In many outpatient settings nies for the purposes of research, outcome tracking, and
the therapist will be required to “match” ICD-9 codes for education.
Medicare patients with specific CPT codes to establish The AMA first published the fourth edition of CPT in
medical necessity for the rehabilitation interventions accord- 1977. CPT is continually updated to keep the codes current
ing to the Fiscal Intermediary or Carrier Local Coverage with the community standard of practice by a process led by
Decisions. In any case, the treating therapist should be abso- the AMA CPT Editorial Panel.26 For the rehabilitation disci-
lutely clear in the medical record about the treating diagnoses plines, the Health Care Professional Advisory Committee
and comorbidities that define the treatment program and develops CPT coding changes and updates. The Committee
POC of the patient or client. consists of representatives from 16 nonphysician provider
groups, including physical therapy, occupational therapy,
The Future of Diagnosis Coding: ICD-10-CM and speech and language pathology.
In 2013, changes in HIPAA regulations will replace ICD- The CPT code set is organized into six major sections:
9-CM with an updated diagnostic coding set: ICD-10-CM. Evaluation and Management, Anesthesiology, Surgery, Ra-
This updated system will enhance accurate payment for diology, Pathology/Laboratory, and Medicine. Each section
services and facilitate evaluation and tracking of medical is divided into subsections based on anatomical, procedural,
diagnoses and outcomes. ICD-10-CM will provide im- condition, and descriptor headings as appropriate to that
provements through more detailed diagnostic information specialty section. The AMA, in publishing the CPT code set,
and increased specificity of location and pathologies and recognizes that there may be significant overlap in the inter-
will have expanded ability to capture additional advance- ventions, procedures, and services performed by health care
ments in identification of pathology, diagnoses, and patient providers and makes the following statement in the intro-
problems. duction:
The ICD-10-CM classification system has been used in
other countries since the mid-1990s; it has been adapted by It is important to recognize that the listing of a service or proce-
dure and its code number in a specific of this book does not restrict
the Centers for Disease Control and Prevention for use in the
its use to a specific specialty group. Any procedure or service in
United States. The diagnostic coding under this system uses any section of this book may be used to designate the services
three to seven alphabetical and numerical characters and full rendered by any qualified physician or other qualified health care
code titles for each entry. Organization and format are very professional.26
similar to those of ICD-9-CM.
Because of the impact this change will have on electronic Typically, most codes used by rehabilitation profes-
data interchange and computer systems, the transitional plan sionals to describe treatment of neurological conditions
for this significant change is already underway. Therapists are in the 97000 series of the CPT; however, any code that
and other health care providers should be aware of this im- adequately represents the interventions or services per-
pending change and participate in training opportunities as formed by a provider with the appropriate qualifications
they become available. may be used.
INTERVENTION CODING Using the CPT Codes

Just as ICD-9 codes allow therapists to communicate to pay- Selecting the correct CPT code that most adequately de-
ers and other health care stakeholders the conditions and scribes the intervention performed is often very challeng-
injuries being treated, CPT codes allow therapists to identify ing because most therapists have not had formal CPT
and communicate the interventions being used in the course training. Although in-depth coding training is beyond the
of patient care. In a world where most billing information is scope of this text, this section will help the reader develop
transmitted electronically, it is essential for therapists to use some basic skills in applying sound coding techniques to
the most appropriate CPT codes to communicate the breadth, practice.
depth, and complexity of the treatment plans required in the Most of the codes used to describe therapy interventions
care of patients/clients. Appropriate intervention coding is are found in the Physical Medicine section of the CPT code.
also essential to the billing and claims adjudication process Although therapists use these codes, so do a large number of
and to maximize the health care benefits available to the other health care professionals and providers. For this rea-
patient with complex neurological conditions and injuries. son it is important for therapists to be able to adequately
describe their use of interventions using the correct CPT
Current Procedural Terminology codes so the codes reflect the complex nature of the treat-
Current Procedural Terminology,26 Fourth Edition, is main- ment plans implemented with their patients.
tained, updated, and published by the AMA and is a regis-
tered trademark of the AMA. It is a code set designed to Physical Medicine CPT Codes
identify the interventions and other services performed by The Physical Medicine codes are located in a subsection of
health care providers. Each intervention or service is de- the Medicine section of the CPT code set. Some CPT codes
scribed by a five-digit code. CPT is mandated by HIPAA as in the Physical Medicine section represent interventions that
the appropriate code set for use in health care transactions in occur in specified time intervals (e.g., 15 minutes) and are
the United States. considered “timed” codes. Timed codes generally require
constant attendance or direct (one-on-one) patient contact. made after care has been delivered and subject to reviews
Other codes are considered “occurrence” codes and do of medical necessity, appropriateness, and other policies.
not have a time period associated with them. Some occur- Financial class largely determines the types of policies and
rence codes require direct contact, whereas others do not. regulations that apply to any particular payer. There are
Occurrence codes are billed only one time during a visit or four primary financial classes: Medicare, Medicaid, and
treatment, but timed codes can be billed in multiple units as government programs; commercial insurance and private
justified by the time it takes to provide the intervention. coverage; automobile and accident insurance companies;
Consult a current CPT codebook for specific details, because and workers’ compensation. To be effective advocates for
these codes and their associated descriptions can change patient care, therapists must be vigilant regarding regula-
each year. tions and payment policies that determine how care is ap-
The Physical Medicine codes are organized into six proved, billed, and paid.
groups of codes. The codes in these subsections have specific
attributes.26 Medicare and Medicaid
Both the Medicare and Medicaid programs are overseen and
Evaluation/Reevaluation regulated by CMS. Medicare, as a federal program, is heav-
Evaluation/Reevaluation codes are the evaluation and reevalu- ily regulated. These regulations are readily available to
ation codes for physical therapy, occupational therapy, and providers through a number of resources, but the primary
athletic training. These codes are occurrence codes requiring access to information is through the Internet at http://cms.
direct contact between the therapist and the patient. gov. As previously discussed, Medicare pays for outpatient
services through MACs. Each of these entities must main-
Modalities tain a website for beneficiaries and providers to allow for
Modality codes are further divided into two groups: “Super- ready dissemination of pertinent information. MACs use
vised” modalities (occurrence codes that do not require direct Medicare’s national policies to process and adjudicate
contact) and “Constant Attendance” modalities (timed codes claims. Although Medicare has national policies, MACs
that require direct contact). have some discretion in how these policies are implemented
locally. Any MAC regulations or policies specific to particu-
Therapeutic Procedures lar services, interventions, or provider types are contained in
Therapeutic Procedure codes require direct patient contact LCDs that must go through a lengthy draft and approval
by the therapist. All but one of these codes are timed, so if process before they are made available to providers and
the time required for the intervention warrants, multiple implemented. Most MACs have LCDs specific to physical
units of a code can be charged. rehabilitation providers (physical therapy, occupational ther-
apy, and speech and language pathology) as well as specific
Active Wound Care Management services or interventions such as wound care, biofeedback
Active Wound Care Management codes are occurrence for incontinence, vestibular problems, and cardiac rehabili-
codes that require direct contact. tation. Because MACs have defined geographic coverage
Tests and Measures. Tests and Measures codes repre- areas, it is advisable for therapists to be sure they are famil-
sent specific assessment and testing interventions that are iar with Medicare’s payment policies in the areas where they
separate and distinct from evaluations and reevaluations. practice.
These interventions require separate written reports. Medicaid, as discussed earlier, is a health program for the
economically disadvantaged. Although it is partially funded
Other Procedures with federal dollars, it is also funded at the state level. Be-
The Other Procedures section consists of a single code used cause Medicaid is implemented at the state level, states have
to describe any “unlisted” physical medicine service or insignificant leeway in how their programs operate, approve
tervention. care, and pay for services. Consequently there are large
Each year therapists should review the codes and sections variations in the Medicaid program from state to state.
commonly used for changes and additions that will better Therapists should be aware within their individual work set-
describe the interventions performed with their patients. All tings of the regulations and policies that may apply to them
therapists should consult the CPT codes directly and avail as a result of their employer’s possible participation in the
themselves of training specifically designed to help them Medicaid program.
accurately describe their interventions. CPT coding resources Medicare and other payers often attempt to mitigate their
are available from a number of sources, including the AMA financial risk for costly episodes of rehabilitation by impos-
and professional associations such as APTA.27 ing arbitrary limits on care. These limits are often referred
to as “caps.” One example of such a limit is Medicare yearly
OUTPATIENT PAYMENT POLICY cap on rehabilitation services. This cap was created as part
The processes involved with billing, payment, and pay- of the BBA and went into effect in 1999. The cap was $1500
ment policy for outpatient services remains distinctly in payments per year for physical therapy and speech ther-
different compared with inpatient services. Although inpa- apy and a separate $1500 cap for occupational therapy ser-
tient rehabilitation services are primarily paid on a pro- vices. The cap applies in all outpatient settings except out-
spective basis, outpatient rehabilitation services continue patient hospital rehabilitation units. The therapy cap
to be paid primarily on a retrospective basis. This means is adjusted annually as a consequence of changes in the
that, although services may have been authorized before Medicare Economic Index that tracks health care costs and
delivery of care, the decision to pay for the services is inflation.
Another way Medicare and Medicaid attempt to mitigate patient sees a network provider (preferred provider) or an
their financial risk is to use outpatient service programs that out-of-network provider (a provider who is not a contracted
are prospectively paid. These programs operate by use of provider). These amounts can be based on a percentage of
capitation, a system by which health care providers are paid the charges, on a flat amount for each treatment (co-pay), or
in advance of rendering care to a defined group of beneficia- both. The required patient payment can have a significant
ries. In this payment system the capitated health care provid- effect on patients’ and clients’ financial abilities to partici-
ers provide care out of the prepaid pool of funds. These pate in their respective treatments. By increasing co-pay
programs use contracted insurance companies, using large amounts, payers know patients will have to make “harder”
groups of health care providers representing a wide array of decisions regarding how much care they can afford. This
specialties, to provide the anticipated health care needs of can play an important factor when a therapist and his or her
the covered patients. Capitation agreements must be care- patient agree on a POC, how much therapy the patient can
fully negotiated. If the negotiated prospective payment is too afford, and when the patient is discharged to a home pro-
low, or, if the therapist overtreats, the payment for services gram. For patients who pay cash for services, these deci-
rendered will be inadequate to cover the cost of providing sions can be even more difficult and come far sooner in the
care to the covered patient population. POC. In other situations, especially in long-term manage-
A number of smaller government programs also may ment of an individual after central nervous system (CNS)
have specific regulations and policies similar to those of injury, the therapist’s role may become consultative. When
Medicare. An example of such programs is CHAMPUS/ the patient or family identifies functional changes, the
TRICARE. This program provides health care insurance therapist may be asked to establish new goal interventions
coverage for members of the military and their dependents as a home program to be carried out by the patient’s support
and for military retirees. Other federal health care programs, system.
such as the Veterans Administration, may vary significantly Payers can also place limitations on the amount of ser-
from Medicare and Medicaid in their policies. vices a patient can receive each year by limiting the number
Coverage programs for children with congenital or ac- of visits, days, and dollars spent on therapy services. Thera-
quired conditions requiring extensive rehabilitation are fi- pists must be aware of these limitations and how these limi-
nanced through a number of federal, state, and local pro- tations may affect the potential interactions between long-
grams. Because of the huge diversity in the payment policies term care and patient potential. With this understanding, a
related to these programs, therapists should be aware of the therapist can help identify the best use of patients’ resources
particular program covering the care and should work and facilitate those individuals’ abilities to participate in
closely with parents and agencies involved to ensure that their own care. The nearly infinite number of ways payers
proper coverage for services is achieved. can shift risk and financial responsibility to patients and
providers makes it imperative that systems be in place in
Commercial Insurance and Private Coverage each clinical setting to check for limitations and alert the
Commercial insurance coverage is financed by traditional patient and therapist to potential financial challenges that
health insurance companies, self-insured employers, and can have chilling effects on treatment and the potential for
self-paying consumers. Commercial insurance companies recovery.
are regulated at the state level, and self-insured companies
are regulated at the federal level. Cash-paying consumers Automobile and Accident Coverage
must rely on their own understanding and self-education and Third-Party Liability
to make their purchasing decisions regarding therapeutic When individuals are injured in automobile and other acci-
care. dents, financial liability for care may become the responsi-
Commercial insurance companies operate by charging bility of others who were involved in or responsible for the
premiums to the beneficiaries (employers or individual accidents. In the case of automobile accidents, people are
consumers) and then paying for services delivered to their generally required by state law to carry some minimum
insured. Because these payers bear the risks associated amount of public liability insurance to cover such costs.
with the health of their beneficiaries, they use a number of Health insurance companies usually have stipulations in
strategies to mitigate their risks in this delivery model. their policies that allow them to recover any costs they incur
Many use preferred panels of health care providers to de- as the result of the liability of others.
liver services. These preferred providers agree to particular To further complicate matters related to accidents, many of
business processes, rates of payment, and utilization re- these cases end up in lawsuits and litigation. This represents
view and restrictions to have access to the beneficiaries of several challenges for the treating therapist. In terms of pay-
these payers. Some require the provider to obtain authori- ment for services, it is not always entirely clear who will be
zation before treatment is provided, whereas others pro- paying for services and when they will pay. Many patients
vide strict review of care after delivery to decide whether injured from the actions of others may feel that they are not
payment is warranted. These companies also have a num- responsible for paying for the care they receive, and they can
ber of mechanisms to shift their financial risk to the patient be unaware of the cost of treatment as it mounts. This can be
and to the provider, including capitation and case-rate re- problematic if the party the patient believed was liable is
imbursement. In case-rate reimbursement, a flat rate is paid exonerated or unable to pay.
for the entire course of care for a patient with a particular Nearly all health care facilities have a policy that states
medical diagnosis. that the patient, or his or her parent or guardian, is finan-
Insurance companies often require the patient to pay dif- cially responsible for the treatment received, although the
ferent amounts toward their care on the basis of whether the facility may be willing to bill other parties for those services.
Therapists should always be aware of the various possibili- who have been excluded by payer enrollment policies,
ties that can occur during the course of care that can affect inability to purchase health care coverage, or both. This
the ability of the patient to continue therapy. Therapists new coverage burden will be shared largely by employers
should also be aware that the medical records could end and by new state and federal programs financed through
up being examined by a number of attorneys and end up new taxes and efforts to curtail fraud and abuse in health
in open court. care. Although the details of providing funding for this
significant expansion in services are to be worked out, there
Workers’ Compensation will likely be significant downward pressure on payment
Of all the insurance classes reviewed, workers’ compensa- for services as well as an increase in efforts to compensate
tion has the highest degree of variability in regulation and health care providers on the quality of their clinical and fi-
payment policy. Each state legislates and regulates its treat- nancial outcomes. Implementation of evidence-based prac-
ment of injured workers independently of other states and tice and keeping current in “best practices” will be essential
federal involvement. This variability requires every facility for every therapist as compensation systems evolve to meet
treating workers’ compensation patients to maintain a the needs of patients and clients and the rising costs of
knowledge base of the laws and regulations governing the health care.
care of these patients as well as establishing procedures to
ensure that they are followed. Many states use fee schedules SUMMARY
that are based on CPT codes but are highly modified and Payment for rehabilitation services is a complex topic that
have significant variations from “normal” coding. These involves many legal, regulatory, and contractual details. To
types of fee schedules may require specific instruction to use completely explain the complexities involved in documen-
so that the therapist can accurately describe the interven- tation of patient care, medical billing, and claims adjudication
tions used with patients covered by these fee schedules. In would fill a volume similar to the size of this text. We have
addition, the nature of work-related injuries produces other attempted to provide the treating therapist with a basic
potential challenges for therapists. understanding of the payment systems involved in inpatient
Workers’ compensation coverage is provided through and outpatient services and the importance of documenta-
purchased insurance or through self-insurance programs set tion to the billing and payment process, provided basic
up by employers. Workers’ compensation cases are concur- steps for inclusion of diagnosis and intervention coding,
rently managed by insurance companies or by third-party and provided an overview of payment policy for outpatient
administrators who manage self-insured employer pro- services. Therapists must keep in mind that the regulatory
grams. Concurrently managed care means that the payer and legislative world of health care is in a continual state
requires the health care provider to preauthorize all proof flux and that there are a number of critical areas that
posed care and reviews documentation to ensure compli- affect payment for services that were not touched on in this
ance with state-mandated fee schedules and use guidelines. chapter. These would include the areas of Medicare and
Because of the assumed employer liability of work-related corporate compliance, the HIPAA privacy and security
injuries, some of these cases progress to lawsuits and litiga- rules, currently evolving issues related to the Medicare caps
tion as in the case of accidents. Therapists should remain on therapy services, and individual state practice acts for
aware, also, of the potential involvement of their patients’ various health care providers. The reader would be well
medical records in these legal proceedings. In the area of served to get specific questions and concerns addressed by
neurological rehabilitation, a workers’ compensation pack- knowledgeable individuals or to consult source documents
age may become very complex. If the injury results in per- on these important areas. The increasing reliance on elec-
manent CNS limitations, therapists are often asked to esti- tronic data interchange will necessitate improvements in
mate the long-term needs of the patient to establish potential the ICD-9 coding system to ICD-10, requiring the reader
costs of long-term therapeutic management over the life- to seek appropriate training. Emerging health care reform
time of the patient. initiatives will create new opportunities for coverage of indi-
viduals with chronic conditions but will place additional
Evolving Health Care Reform Efforts financial strain on the system and our economy, with possi-
and Effects on Payment Policy ble consequences on health care provider compensation and
In March 2010 President Barack Obama signed the Patient payment policies.
Protection and Affordability Act of 2010 (HR 3590) and
its companion legislation the Health Care and Education Acknowledgment
Reconciliation Act of 2010, ushering in the most sweeping With sincere appreciation for editorial contributions by Bob
regulatory changes in health care payment policy since the Niklewicz, PT, DHSc.
Medicare Act of 1965, which established the Medicare pro-
gram. The full effects of this legislation will not be fully References
implemented until 2016. The regulatory implications of this To enhance this text and add value for the reader, all refer-
new law will be promulgated over the coming years. The ences are included on the companion Evolve site that
reader should be forewarned that keeping current with accompanies this textbook. This online service will, when
major changes in health care policy is essential in providing available, provide a link for the reader to a Medline abstract
proper advice and counsel to patients requiring long-term for the article cited. There are 27 cited references and other
and intensive therapies to maximize their abilities to func- general references for this chapter, with the majority of
tion. This legislation will allow many to access services those articles being evidence-based citations.
APPENDIX 10-A n Quick Reference Guide to Acronyms

ADLs 5 Activities of Daily Living HMO 5 Health Maintenance Organization
AFDC 5 Aid to Families with Dependent Children IADL 5 Instrumental Activities of Daily Living
AMA 5 American Medical Association ICD-9-CM 5 International Classification of Diseases, Ninth Revision,
APTA 5 American Physical Therapy Association Clinical Modification
ARRA 5 American Recovery and Reinvestment Act of 2009 IDEA 5 Individuals with Disabilities Education Act
BBA 5 Balanced Budget Act of 1997 IGC 5 Impairment Group Code
BBRA 5 Balanced Budget Refinement Act of 1999 IME 5 Indirect Medical Education
BIPA 5 Benefits Improvement Act of 2000 IPPS 5 Inpatient Prospective Payment System
CAA 5 Care Area Assessment IRF-PAI 5 Inpatient Rehabilitation Facility—Patient Assessment
CARE 5 Continuity Assessment Record and Evaluation Instrument
CARF 5 Commission on Accreditation of Rehabilitation Facilities LCD 5 Local Coverage Decisions
CAT 5 Care Area Trigger LTCH 5 Long-Term Care Hospital
CHIP 5 Children’s Health Insurance Program MDS 5 Minimum Data Set
CHIPRA 5 Children’s Health Insurance Program Reauthorization MS-DRG 5 Medicare Severity Diagnosis-Related Group
Act of 2009 MSP 5 Medicare Savings Programs
CMG 5 Case-Mix Group NCLB 5 No Child Left Behind Act of 2001
CMS 5 Centers for Medicare and Medicaid Services NEC 5 Not Elsewhere Classified
COBRA 5 Consolidated Omnibus Budget Reconciliation Act of 1985 NOS 5 Not Otherwise Specified
CPT 5 Current Procedural Terminology OASIS 5 Outcome and Assessment Information Set
DRG 5 Diagnosis-Related Group OBQI 5 Outcome-Based Quality Improvement
DSH 5 Disproportionate Share Hospital OCR 5 Office for Civil Rights
EPHI 5 Electronic Protected Health Information PHI 5 Protected Health Information
ESEA 5 Elementary and Secondary Education Act of 1965 POC 5 Plan of Care
FI 5 Fiscal Intermediary PPS 5 Prospective Payment System
FIM* 5 Functional Independence Measure RAC 5 Recovery Audit Contractor
HCFA 5 Health Care Financing Administration RAI 5 Resident Assessment Instrument
HCPAC 5 Health Care Professional Advisory Committee RAP 5 Resident Assessment Protocols
HCPCS 5 Healthcare Common Procedure Coding System RUGs-III 5 Resource Utilization Groups, Version III
HEW 5 Department of Health, Education, and Welfare SCHIP 5 State Children’s Health Insurance Program
HHA 5 Home Health Agency SNF 5 Skilled Nursing Facility
HHS 5 Department of Health and Human Services SRS 5 Social and Rehabilitation Service
HIPAA 5 Health Insurance Portability and Accountability Act of SSA 5 Social Security Administration
1996 TLC 5 Transitional Living Center
HITECH 5 Health Information Technology for Economic and WHO 5 World Health Organization
Clinical Health Act
*
Copyright © 2001, 2002 UB Foundation Activities, Inc. (UBFA, Inc.) for compilation rights; no copyrights claimed in U.S. Government works included in
Section I, portions of Section IV, Appendices I and K, and portions of Appendices B, C, E, G, H, and J. All other copyrights are reserved to their respective
owners. Copyright © 1993-2001 UB Foundation Activities, Inc. for the FIM Data Set, Measurement Scale, Impairment Codes, and refinements thereto for
the IRF-PAI, and for the Guide for the Uniform Data Set for Medical Rehabilitation, as incorporated or referenced herein. The FIM mark is owned by
UBFA, Inc.
SECTION II
Rehabilitation Management of Clients

with Neurological System Pathology
CHAPTER 11 Neonates and Parents: Neurodevelopmental

Perspectives in the Neonatal Intensive Care
Unit and Follow-Up
JANE K. SWEENEY, PT, PhD, PCS, C/NDT, FAPTA, TERESA GUTIERREZ, PT, MS, PCS, C/NDT,
and JOANNA C. BEACHY, MD, PhD, FAAP

high-risk clinical signs After reading this chapter the student or therapist will be able to:
medical complications of prematurity 1. Discuss three theoretical frameworks guiding neonatal therapy services in the neonatal
neonatal intensive care unit environment intensive care unit.
neuromotor assessment 2. Identify the physiological and structural vulnerabilities of preterm infants that
neuromotor intervention predispose them to stress during neonatal therapy procedures.
parent instruction 3. Outline supervised clinical practicum components and pediatric clinical experiences to
physiological and musculoskeletal risks prepare for entry into neonatal intensive care unit practice.
subspecialty training 4. Describe how the grief process may affect behavior and caregiving performance of
parents of low–birth-weight neonates.
5. Differentiate the developmental course and neuromotor risk signs in infants with
emerging neuromotor impairment from the clinical characteristics of infants with
transient movement dysfunction.
6. Identify instruments for neuromotor examination of high-risk infants in neonatal intensive
care units and in follow-up clinics and compare psychometric features of the tests.
7. Describe program plans and follow-up for low–birth-weight infants in neonatal
intensive care unit and home settings.
P remature birth is associated with an increased preva-

lence of major and minor neurodevelopmental disabil-
ity. Advancements in newborn resuscitation and neonatal
and infant therapy approaches can serve these increasing
numbers of surviving neonates at neurodevelopmental risk
by (1) providing valuable diagnostic data through neuro-
intensive care have contributed to greatly improved survival logical and developmental examination, (2) participating in
of infants with low birth weight (LBW), but risk of neurodevelopmental and environmental interventions adapted
developmental sequelae remains high.1,2 to each infant’s physiological, motor, and behavioral needs,
Although brain injury can be documented by ultrasonog- (3) facilitating and coordinating interdisciplinary case man-
raphy, computed tomography (CT), and magnetic resonance agement for infants and parents, and (4) reinforcing preven-
imaging (MRI) in infants, prediction of subsequent neurode- tive aspects of health care through early intervention and
velopmental outcome is still relatively unreliable.3-6 Serial long-term developmental monitoring.
clinical examinations and careful monitoring of neuro Clinical management of neonates at developmental risk
developmental status are therefore critical during the neona- and their parents during the NICU and outpatient follow-up
tal period and discharge from the neonatal intensive care unit phases is the focus of this chapter. A theoretical framework
(NICU) through the outpatient phase of care. Pediatric thera- for neonatal practice is presented, and an overview of neo-
pists with mentored, subspecialty training in neonatology natal complications associated with adverse outcomes is
271
272 S E C T I O N II n Rehabilitation Management of Clients with Neurological System Pathology
provided. In-depth discussion in the neonatal section neonatal therapy. In dynamic systems theory, emphasis is
includes indications for referral based on risk, neurodevel- placed on the contributions of the interacting environments of
opmental examination instruments, high-risk profiles in the the NICU, home, and community in constraining or facilitat-
neonatal period, treatment planning, and therapy strategies ing the functional performance of the infant.8
in the NICU. The section on outpatient follow-up focuses on
critical time periods for neuromotor and musculoskeletal Synactive Model of Infant Behavior
reexamination, assessment tools, and clinical cases. The synactive model of infant behavioral organization is a
specific neonatal dynamic systems model for establishing
THEORETICAL FRAMEWORK physiological stability as the foundation for organization of
Concepts of dynamic systems, neonatal behavioral organi- motor, behavioral state, and attention or interactive behaviors
zation, and parental hope and empowerment provide a theo- in infants. Als and colleagues9-11 described a “synactive” pro-
retical framework for neonatal therapy practice. In this cess of four subsystems interacting as the neonate responds to
section are three models that provide a theoretical structure the stresses of the extrauterine environment. They theorized
for practitioners designing and implementing neuromotor that the basic subsystem of physiological organization must
and neurobehavioral programs for neonates and their parents. first be stabilized for the other subsystems to emerge and
allow the infant to maintain behavioral state control and then
Dynamic Systems interact positively with the environment (Figure 11-2).
Dynamic systems theory applied to infants in the NICU To evaluate infant behavior within the subsystems of func-
refers first to the presence of multiple interacting structural tion addressed in the synactive model, Als and colleagues10,11
and physiological systems within the infant to produce developed the Assessment of Preterm Infants’ Behavior
functional behaviors and second to the dynamic interactions (APIB). With the development of this assessment instrument,
between the infant and the environment. In Figure 11-1, a fifth subsystem of behavioral organization, self-regulation,
neonatal movement and postural control are targeted as was added to the synactive model. The self-regulation subsys-
a core focus in neonatal therapy, with overlapping and tem consists of physiological, motor, and behavioral state
interacting influences from the cardiopulmonary,7 behavioral, strategies used by the neonate to maintain balance within and
neuromuscular, musculoskeletal, and integumentary systems. between the subsystems. For example, many infants born pre-
A change or intervention affecting one system may diminish term appear to regulate overstimulating environmental condi-
or enhance stability in the other dynamic systems within the tions with a behavioral state strategy of withdrawing into a
infant. Similarly, a change in the infant’s environment may drowsy or light sleep state, thereby shutting out sensory input.
impair or improve the infant’s functional performance. The withdrawal strategy is used more frequently than crying
This theory guides the neonatal practitioner to consider because it requires less energy and causes less physiological
the many potential physiological and anatomical influences drain on immature, inefficient organ systems.
(dynamic systems within the infant) that make preterm infants Fetters12 placed the synactive model within a dynamic
vulnerable to stress during caregiving procedures, including systems framework to demonstrate the effect of a therapeu-
tic intervention on an infant’s multiple subsystems (Figure
11-3). She explained that although a neonatal therapy inter-
vention is offered to the infant at the level of the person,
outcome is measured at the systems level, where many
subsystems may be affected. For example, the motor
outcome from neonatal therapy procedures is frequently
influenced by “synaction,” or simultaneous effects, of an
COMMUNICATION infant’s physiological stability and behavioral state. Physi-
SYSTEMS
CHANNELS
Neuromuscular ological state and behavioral state are therefore probable
Autonomic (physiological)
Musculoskeletal
Motor organization
Cardiovascular
State system
Integumentary
Self-regulation
NEONATAL
FUNCTIONAL
ACTIVITIES
NEONATAL
ASSOCIATIVE MOVEMENT
LEARNING AND
AND MEMORY POSTURAL
CONTROL
NICU t
Environmen
Fa
mil nt
y an me
d Home Environ
Figure 11-1 n Dynamic systems within neonates and interacting

external influences on functional performance. (From Sweeney JK,
Heriza CB, Blanchard Y, Dusing SC: Neonatal physical therapy.
Part II: practice frameworks and evidence-based practice guidelines. Figure 11-2 n Pyramid of synactive theory of infant behavioral
Pediatr Phys Ther 22:3, 2010.) organization with physiological stability at the foundation.
C H A PTER 11 n Neonates and Parents: Neurodevelopmental Perspectives in the Neonatal Intensive Care Unit and Follow-Up 273
Figure 11-3 n Combined dynamic systems and synactive models. (Modified from Fetters L: Sensorimotor management of the high-risk
neonate. Phys Occup Ther Pediatr 6:217, 1986.)
confounding variables during research on motor behavior in

neonatal subjects. Neonatal therapists may find this com-
bined dynamic systems and synactive framework helpful in
conceptualizing and assessing changes in infants’ multiple
subsystems during and after therapy procedures.
Hope-Empowerment Model
A major component of the intervention process in neonatal
therapy is the interpersonal helping relationship with the fam-
ily. A hope-empowerment framework (Figure 11-4) may guide
neonatal practitioners in building the therapeutic partnership
with parents; facilitating adaptive coping; and empowering
them to participate in caregiving, problem solving, and advo-
cacy. The birth of an infant at risk for a disability, or the diag-
nosis of such a disability, may create both developmental and
situational crises for the parents and the family system. The
developmental crisis involves adapting to changing roles in the
transition to parenthood and in expanding the family system.
Although not occurring unexpectedly, this developmental tran-
sition for the parents brings lifestyle changes that may be
stressful and cause conflict.13 Because parents are experienc-
ing (mourning) loss of the “wished for” baby they have been
visualizing in the past 6 months, they often struggle with
developing a bond with their “real” baby in the NICU.14
A situational crisis occurs from unexpected external
events presenting a sudden, overwhelming threat or loss for
which previous coping strategies either are not applicable or
are immobilized.15 The unfamiliar, high-technology, often
chaotic NICU environment creates many situational stresses
that challenge parenting efforts and destabilize the family Figure 11-4 n Hope-empowerment (left) versus learned help-
system.16 The language of the nursery is unfamiliar and lessness (right) processes of the therapeutic partnership between
intimidating. The sight of fragile, sick infants surrounded by parents and the neonatal therapist.
medical equipment and the sound of monitor alarms are
frightening. The high frequency of seemingly uncomfort-
able, but required, medical procedures for the infant are of partnership with established interactive styles and varying
financial and humanistic concern to parents. No previous life and professional experiences, the initial contacts during
experiences in everyday life have prepared parents for this assessment and program planning set the stage for either a
unnatural, emergency-oriented environment. This emotional positive or a negative orientation to the relationship.
trauma of unexpected financial and ongoing psychological Despite many uncertainties about the clinical course,
stresses during parenting and caregiving efforts in the NICU prognosis, and quality of social support, a positive orienta-
contributes to potential posttraumatic stress disorder in tion is activated by validation or acknowledgment of parents’
parents of infants requiring intensive care.17,18 feelings and experiences. Validation then becomes a catalyst
The quality and orientation of the helping relationship in to a hope-empowerment process in which many crisis events,
neonatal therapy affect the coping style of parents as they try negative feelings, and insecurities are acknowledged in
to adapt to developmental and situational crises (see Figure a positive, supportive, nonjudgmental context in which
11-4). Although parents and neonatal therapists enter the decision-making power is shared.19 In contrast, a negative
orientation may be inadvertently facilitated by information NEONATAL COMPLICATIONS ASSOCIATED

overloading without exploration and validation of parents’ WITH ADVERSE OUTCOMES
feelings, experiences, and learning styles. This may lead to Improvements in neonatal intensive care over the last 30 years
magnified uncertainty, fear, and powerlessness with the have led to the increased survival of preterm and term infants.
misperception of excessive complexity in the proposed Specific obstetric advances include establishment of special-
neuromotor intervention activities. ized tertiary care centers, earlier identification of high-risk
In a hope-empowerment framework, parent participation pregnancies, improvements in prenatal diagnosis, and medica-
in neuromotor intervention allows sharing of power and tions used to stabilize maternal medical conditions and
responsibility and promotes continuous, mutual setting and enhance fetal well-being. Respiratory compromise in preterm
revision of goals with reality grounding. Adaptive power can infants has significantly decreased as a result of (1) maternal
be generated by helping parents stabilize and focus energy betamethasone administration to promote fetal lung maturity;
and plans and by encouraging active participation in inter- (2) availability of commercial surfactant to improve pulmo-
vention and advocacy activities.19 Exploring external power nary function; and (3) advances in ventilator design and
sources (e.g., Parent to Parent USA or other parent-to- capability, enhancing management of respiratory distress with
parent support groups20) early in the therapeutic relationship significantly diminished pulmonary dysfunction. In addition,
may help parents focus and mobilize.20-23 improvements in continuous monitoring of vital signs, radio-
Hope and empowerment are interactive processes. They logical imaging techniques, delivery of medications, and
are influenced by existential philosophy: the hope to adapt maintenance of thermal stability have aided earlier identifica-
to what is and the hope to later find peace of mind and mean- tion of neonatal problems and enhanced improvements in care.
ing for the situation, regardless of the infant’s outcome. In Increased survival is most evident in the extremely low–birth-
describing the effect of a prematurely born infant on the weight (ELBW) infant, that is, birth weight less than 1000 g.
parenting process, Mercer24 related that “hope seems to be For infants born at 23 weeks of gestation, survival has in-
a motivational, emotional component that gives parents creased from approximately 0% to more than 50%, and for
energy to cope, to continue to work, and to strive for the best infants born at 26 weeks of gestation, survival has increased
outcome for a child.” She viewed the destruction of hope as from 25% to 85%.26 It is important to note that the incidence
contributing to the physical and emotional withdrawal of severe neurological injury has decreased over time in these
frequently observed in parents who attempt to protect them- extremely preterm infants. However, a significant number
selves from additional pain and disappointment and then of preterm infants will exhibit long-term neurological impair-
have difficulty reattaching to the infant. ment owing to increased survival.
Hope contributes to the resilience parents need to get The long-term effect of a neurological insult on the devel-
through the arduous 1- to 4-month NICU hospitalization oping brain depends on the timing of the injury, the gestational
period and then begin to face the future in their home and age of the infant, and the nature and duration of the insult.27
community with an infant at neurodevelopmental risk. During the first month of gestation, the neural tube is formed.
Groopman25 proposed that hope provides the courage to Neurological insult at this time leads to abnormal neural
confront obstacles and the capacity to surmount them. He tube development, specifically anencephaly, encephalocele, or
described the process of creating a middle ground where myelomeningocele. Neuronal proliferation is nearly complete
truth (of the circumstances) and hope reside together as one by 5 months of gestation. All neurons and glial cells originate
of the most important and complex aspects in the art of in the ventricular and subventricular zone (germinal matrix).
caregiving. Disorders of proliferation result in microcephaly, with either
In a hope-empowerment context, parental teaching decreased size or decreased number of proliferating neuronal
activities are carefully selected to contribute to pleasurable units or macrocephaly. Neuronal migration occurs at 3 to
interaction between infant and parent. Gradual participation 6 months of gestation, and neurons are guided by glial cells to
in infant care activities and therapeutic handling in the form neuronal columns. Subplate neurons, essential for correct
NICU provide experience and build confidence for continu- organization of the brain, are formed at this time. Insults
ation in the home environment. during this period of development result in marked disturbance
Conversely, if the parents’ learning styles, goals, priori- of neurological structure and function with aberrations noted
ties, values, time constraints, energy levels, and emotional in gyral formation (lissencephaly, schizencephaly, and polymi-
availability are not considered in the design of the develop- crogyria) and/or absence of the corpus callosum.
mental program, the parents may experience failure, loss of Organization, consisting of elaboration of subplate neurons,
self-esteem, powerlessness, immobilization, or dependency. orientation of cortical neurons, development of dendrites and
The neonatal therapist may recognize signs of learned help- axons, synaptogenesis and apoptosis, occurs from 5 months of
lessness in parents when they show nonattendance, noncom- gestation through several years after birth. Subplate neurons are
pliance, negative interactions with infant and staff, or a present from 22 to 35 weeks of gestation; they form connec-
hopeless outlook during bedside teaching sessions. tions between neurons and guide axons and dendritic projec-
New events in the infant’s health or developmental status tions to the appropriate targets. Subplate neurons are sensitive
may create new crises and destabilize the coping processes. to hypoxia and result in abnormal brain development and
In long-term follow-up many opportunities occur within the impaired long-term neurological outcome. Synaptogenesis be-
partnership to validate new fears and chronic uncertainties fore birth is experience independent. However, synaptic forma-
within a hopeful, positively oriented, helping relationship. tion and elimination via apoptosis (programmed cell death) are
The alleviation of hopelessness is a critical helping task in most active after birth and are thought to be experience depen-
health care. This model provides a conceptual framework dent. This process confers plasticity to the brain and is the basis
for sharing the gifts of hope and power with parents and of individuality. The presence and importance of subplate
caregivers. neurons as well as synaptogenesis coincide with the time that
preterm infants are in the NICU and make the preterm brain for IVH include asphyxia, fluid bolus infusion (especially of
especially vulnerable to perturbations such as hypoxia, medica- hypertonic solutions), anemia, and pain.29 Platelet and coagu-
tions, stress, and pain. lation disturbances have been implicated as risk factors for the
The final phase of brain organization is glial maturation development of IVH. IVH is rarely seen in infants with gesta-
to astrocytes and oligodendrocytes. Astrocytes help main- tional age greater than 32 weeks owing to the developmental
tain the blood-brain barrier, provide nutrient support, involution of vessels in the germinal matrix.
regulate neurotransmitter and potassium concentration, and Diagnosed by cranial ultrasound, IVH is graded in severity
assist in neuronal repair after injury. Oligodendrocytes pro- from 1 to 4,28 with grade 1 IVH being the most mild because
duce myelin, a protective fatty sheath that surrounds axons the hemorrhage is confined to the germinal matrix. In grade 2
(white matter) and facilitates nerve transmission. Myelination IVH, the hemorrhage extends into the ventricle (Figure 11-5).
starts in midgestation and continues through adulthood. Grade 3 IVH occurs when the hemorrhage fills more than 50%
Oligodendrocytes are especially sensitive to hypoxia and other of the ventricle and causes ventricular distention. Grade 4
insults. Disruption of normal myelination results in white mat- IVH, or periventricular hemorrhagic infarct (PVHI), is a com-
ter hypoplasia and periventricular leukomalacia (PVL) (see plication of IVH caused by venous congestion of the terminal
later discussion) leading to impaired motor function.27 veins that border the lateral ventricles leading to white matter
The most common neonatal problems associated with necrosis (see later).30 It is important to note that IVH may not
impaired neurological functioning and long-term develop- be apparent on cranial ultrasound in the first few days after
mental delay are listed in Table 11-1. In addition to descrip- birth. However, 90% of IVHs can be detected by day 4. In
tions of neonatal neurological conditions, a discussion is addition, the full extent of the hemorrhage may not be appreci-
provided on the impact on neonatal development of mater- ated for several days after the initial diagnosis of IVH is
nal medication, such as drugs of abuse and psychotropic made.28 The evolution of grades 3 and 4 IVH over 10 days is
medications. The importance of developmental follow-up shown in Figure 11-6, A and B.
for healthy “late” preterm infants born at 34 to 366⁄7 weeks Many researchers have investigated the relationship of
of gestation is also discussed in this section. IVH grades with severity of neurodevelopment delay. In
general, grades 1 and 2 IVH are not associated with a sig-
Intraventricular Hemorrhage nificant increase in developmental abnormalities but do not
Intraventricular hemorrhage (IVH) is the most common brain ensure normalcy. Infants with severe IVH (grade 3 and/or 4)
injury in preterm infants born under 32 weeks of gestation and have increased mortality and are at markedly increased risk
is a significant risk factor for the development of neurodevel- for developmental disabilities, specifically spastic hemiple-
opmental deficits. The incidence of IVH varies inversely with gia or diplegia affecting the lower extremities. As can
gestational age. Approximately 15% of infants born at 1000 g be seen in Figure 11-7, motor tracts innervating the lower
or less will have severe IVH. Although the incidence of severe extremities are in close proximity to the area of the germinal
IVH has decreased over time, an increased number of these matrix and the site of the origin of IVH leading to lower-
infants survive owing to improvements in clinical care and extremity spastic cerebral palsy (CP). However, abnormali-
technology, leading to an increase in the number of surviving ties visible on cranial ultrasound are not able to absolutely
preterm infants who are significantly affected by IVH. predict long-term outcome because the amount of cortex
IVH originates in the microcirculation or capillary network damaged and the neuronal tracts affected by IVH cannot be
of the germinal matrix.28 The germinal matrix is located adja- identified by ultrasound. In addition, ultrasound may not be
cent to the ventricle and is a well vascularized area owing to sensitive enough to identify PVL (see later).
the high metabolic demand from the rapidly proliferating neu-
ronal stem cells. Vessels in the germinal matrix are thin walled
and fragile, which predisposes them to rupture. In addition,
preterm infants have impaired autoregulation—that is, the
inability to maintain cerebral blood flow across a large range
of blood pressures. Thus during labor, delivery, and the
immediate postpartum transition period, changes in blood
pressure can lead to cerebral hypoperfusion and ischemia as
well as to hyperperfusion and vessel rupture. Alterations in
CO2 lead to either reduced cerebral blood flow from hypocar-
bia or increased flow from hypercarbia. Other risk factors
TABLE 11-1 n NEONATAL COMPLICATIONS

AFFECTING BRAIN DEVELOPMENT
AGE SPECIFIC INSULT

Preterm Intraventricular hemorrhage (IVH)
Periventricular hemorrhagic infarct (PVHI)
Posthemorrhagic ventricular dilatation (PHVD)
Periventricular leukomalacia (PVL)
Figure 11-5 n Grade 2 intraventricular hemorrhage is illustrated by the
Necrotizing enterocolitis (NEC)
arrow indicating the area of the germinal matrix bleed. The arrowhead
Term Hypoxic-ischemic encephalopathy (HIE)
points to blood layering in the posterior aspect of the lateral ventricle.
A3
Sagittal A1
Right Sagittal
Right
B1
Sagittal A2 A4 Sagittal
Left Left
B3
B2
A B
Sagittal Sagittal
Right Left
C1
C2
Figure 11-6 n A, Cranial ultrasound of right grade 3 intraventricu-

lar hemorrhage (IVH) and left grade 4 IVH (PVHI periventricular
hemorrhagic infarct) on day 3 of life. A1 and A2 are taken in the sagit-
tal plane with the arrowhead pointing to the area of PHVI. A3 and A4
are coronal views with the arrow pointing to midline shift caused by
the left PVHI. B, Progression of right grade 3 IVH and left PHVI on
day 13 of life. Note retraction of the clot with ventricular enlargement
in B1 and B2. There is marked expansion of PHVI (arrowheads) with
dissolution of brain parenchyma in both B2 and B3. C, Cystic periven-
tricular leukomalacia (cystic PVL) is present on cranial ultrasound in
C3
both right (C1 and C2) and left (C2 and C3) sides of the brain as
marked by arrows. Cysts in the same infant are more evident using C4
MRI imaging (C4 and C5).
C5
C
Periventricular Hemorrhagic Infarct infarction. The usual initial distribution of PVHI seen on
Grade 4 IVH was originally thought to be an extension of IVH cranial ultrasound is fan-shaped echodensities in the periven-
into the parenchyma but is actually a known complication of tricular location (see Figure 11-6, A2-4). Over time there is
IVH.30 PVHI is caused by venous compression of the terminal destruction of preoligodendrocytes and motor axons leading
veins that border the lateral ventricles leading to impaired to white matter necrosis and the development of porence-
venous drainage and congestion and eventually hemorrhagic phalic cyst. PVHI is usually unilateral (approximately 70%),
is necessary in infants with PHVD that does not resolve with

serial removal of CSF. Significant complications of VP
shunt include sepsis, specifically ventriculitis, or shunt mal-
function such as blockage or failure. These complications
necessitate shunt revisions, which further compromise these
fragile infants. Researchers from a consortium of 17 tertiary
NICUs recently published results on the neurodevelopmen-
tal outcome at 2 years of age of ELBW infants with grade
3 and 4 IVH who were born from 1993 to 2002.36 Infants
who required VP shunt placement had significantly worse
outcomes than infants with grade 3 or 4 IVH alone
(Table 11-2). Moreover, the number of infants who were
untestable (MDI or PDI = 49) because of severe neurodevel-
opmental handicap was significantly increased in the group
of infants who received a VP shunt. In addition, CP was
significantly more prevalent in infants who had a VP shunt
placed than in infants with only grade 3 or 4 IVH.
Recent retrospective studies from the Netherlands indi-
cated that earlier intervention when the ventricles are mod-
erately dilated significantly decreased the need for VP shunt
from 62% to 16% and trended to improve long-term devel-
Figure 11-7 n Schematic diagram of corticospinal tract fibers that opmental outcome with a decreased incidence of moderate
extend from the motor cortex through the periventricular region into the to severe handicap.32,37 Thus, halting the progression of
pyramid of the medulla. The lower motor neurons affected by intraven- PHVD and decreasing the need for VP shunt is likely
tricular hemorrhage and periventricular hemorrhagic infarct are located to improve long-term outcome in these infants. However,
in close proximity to the ventricle. Bulbar and upper motor neuron because PVHD can spontaneously resolve without interven-
tracts are affected in term infants with hypoxic-ischemic encepha- tion, identification of factors that can accurately predict
lopathy and are parasylvian in location. (From Volpe JJ: Hypoxic which infant will develop persistent PHVD and conse-
ischemic encephalopathy: neuropathology and pathogenesis. In Volpe quently require VP shunt placement is needed.
JJ: Neurology of the neonate, Philadelphia, 1995, WB Saunders.)
Periventricular Leukomalacia
PVL is nonhemorrhagic cellular necrosis of periventricular
and approximately three quarters of cases are associated with white matter in the arterial watershed area and is present
severe IVH [grade 3 and 4]. Infants with small, unilateral in less than 10% of preterm infants. Like IVH, PVL is
PVHI have no increased risk of developmental delays com- inversely related to gestational age. PVL is the most com-
pared with infants with grade 3 IVH. However, if the PVHI is mon ischemic injury to the preterm infant and results from
bilateral or if multiple porencephalic cysts are present, the risk lack of cerebral autoregulation leading to decreased blood
of severe motor impairment and CP is significantly increased. flow in the vulnerable watershed arterial vessels. Subplate
In addition, approximately 50% of infants with PVHI have neurons, critical for normal neuronal organization and inter-
visual field defects, probably secondary to damage to the ax- action, are destroyed in PVL, leading to decreased white
ons of nerves carrying information to the visual cortex.30 matter. In addition, preoligodendrocytes are exquisitely sen-
sitive to oxygen and glucose deprivation leading to markedly
Posthemorrhagic Ventricular Dilatation abnormal myelination.
Approximately 50% of infants with severe IVH will develop
posthemorrhagic ventricular dilatation (PHVD) from either
blockage of the normal flow of cerebrospinal fluid (CSF) or TABLE 11-2 n OUTCOME OF EXTREMELY
decreased absorption of CSF. Approximately 50% to 75% of LOW–BIRTH-WEIGHT INFANTS WITH GRADE 3
these infants will develop progressive PHVD, resulting in OR 4 INTRAVENTRICULAR HEMORRHAGE
need for treatment. The severity of ventricular dilatation can WITH OR WITHOUT VENTRICULOPERITONEAL
be measured via serial cranial ultrasound examinations.31,32 (VP) SHUNT
Severe ventricular dilatation is usually evident by 2 to
3 weeks after birth. Rapid increase in head circumference NO VP SHUNT VP SHUNT P
does not occur until approximately 4 weeks after birth.33 Post- MDI ,70 326/719 (45.3%) 146/214 (68.2%) ,.0001
hemorrhagic ventricular dilatation is treated by serial removal MDI 5 49 130/719 (18.1%) 87/214 (40.7%) ,.0001
of CSF by spinal tap, subgaleal shunt, or placement of an PDI ,70 263/711 (37.0%) 163/214 (76.2%) ,.0001
Ommaya reservoir. Removal of CSF has been shown to PDI 5 49 149/711 (21.0%) 113/214 (52.8%) ,.0001
decrease intracranial pressure and improve cerebral perfu- CP 217/767 (28.3%) 158/227 (69.6%) ,.0001
sion34 and also to increase cortical gray and white matter.35 In Modified from Adams-Chapman I, Hansen NI, Stoll BJ, et al: Neurodevel-
addition, there is indirect evidence that ventricular distention opmental outcome of extremely low birth weight infants with posthemor-
itself may cause secondary brain injury through stretching rhagic hydrocephalus requiring shunt insertion. Pediatrics 121:1167-1177,
and disruption of axons, gliosis, and loss of oligodendrocytes. 2008.
No consensus has been reached on the optimal manage- CP, Cerebral palsy; MDI, Mental Developmental Index; PDI, Psychomotor
ment of PHVD. Ventriculoperitoneal (VP) shunt placement Development Index.
PVL can be either cystic (see Figure 11-6, C) or global growth is associated with abnormal performance at 1 year and
and may be difficult to identify on radiological images. probably reflects significant white matter injury. Infants with
Cystic PVL results from the focal dissolution of cellular tis- surgically managed NEC have been shown to have signifi-
sue approximately 3 weeks after the insult and can be identi- cantly increased incidence of CP (24% versus 15%), deafness
fied on ultrasound if greater than 0.5 cm in diameter.27 (4.1% versus 1.5%), and blindness (4.1% versus 1%).39 Meta-
However, cysts visualized by cranial ultrasound may disap- analysis of seven studies investigating the impact of NEC on
pear over time owing to fibrosis and gliosis. Thus the inci- neurodevelopmental outcome showed that infants with surgi-
dence of cystic PVL is felt to be underestimated by cranial cally treated NEC have a statistically significant increase in
ultrasound examination. Global PVL results from diffuse cognitive, psychomotor, and neurodevelopmental impairment
white matter injury and myelin loss. This finding can be compared with age-matched preterm infants without NEC.40
subtle with moderate ventricular dilatation and/or a mild Impaired neurodevelopmental outcome in infants with NEC
increase in extraaxial fluid on cranial imaging. Infants with is further exacerbated by associated sepsis and the release of
severe PVL have marked ventricular dilatation, increased inflammatory cytokines and mediators in addition to hypoxia,
extraaxial fluid, and decreased head growth. all of which contribute to further insult to preoligodendrocytes,
MRI obtained at term is more sensitive in identifying leading to white matter injury.
white matter injury than cranial ultrasound and is predictive
of subsequent neurosensory impairment and cognitive delay Cerebellar Injury
present in up to 50% of extremely preterm infants.38 Newer The cerebellum is essential for gross and fine motor control,
techniques, such as diffusion tensor imaging (DTI), func- coordination, and motor sequencing and plays an important
tional connectivity MRI (fcMRI), and morphometry for role in attention and language.36 The clinical hallmark of
analysis of cortical folding are being investigated as early damage to the cerebellum is ataxia. However, recent
markers of impaired neurodevelopmental outcome. Diffu- advances in functional MRI (fMRI) have demonstrated that
sion tension imaging measures the restriction of water there are interactions between the cerebellum and nonmotor
diffusion in the myelin sheath surrounding axons and yields areas of the brain involved in language, attention, and men-
information at the microstructure level about axon caliber tal imagery. Cerebellar injury can also be noted early in
changes and aberrations in myelination. In addition, DTI neonatal development from cranial ultrasound of the poste-
allows for visualization of brain fiber tracks and neuronal rior fossa (mastoid view). The incidence of cerebellar injury
connectivity. In research, fMRI is used to investigate inter- may be as high as 20% in ELBW infants.41 Although the
action between areas of the brain at rest and during tasks by mechanism for damage is unknown, IVH is present in more
analyzing changes in blood flow. Morphometic analysis of than 75% of infants with cerebellar injury, implying similar
sequential MRI scans has been used to create maps of corti- risk factors for both IVH and cerebellar hemorrhage or the
cal folding with quantification of surface area and degree of possibility that IVH leads to cerebellar hemorrhage. The
gyral formation. White matter injury results in delayed majority of cerebellar lesions (70%) are unilateral.
myelination and altered cortical folding.38 Both fMRI and Preterm infants with isolated cerebellar hemorrhage ex-
morphometric analysis of cortical folding are currently avail- hibit significant neurological impairments: hypotonia (100%),
able only in research studies, not in clinical management. abnormal gait (40%), ophthalmological abnormalities (ap-
proximately 40%), and microcephaly (17%).42 Overall, preterm
Necrotizing Enterocolitis infants with cerebellar hemorrhage performed significantly
Necrotizing enterocolitis (NEC) is the most common neonatal lower on tests of gross and fine motor skills and have deficits in
intestinal disease, with an incidence of 10% in extremely pre- vision and expressive and receptive language. Infants with both
term infants. The hallmark of NEC is pneumatosis intestinalis. cerebellar injury and IVH have greater motor impairment than
NEC is initially treated medically with antibiotic therapy and infants with isolated cerebellar hemorrhage. Socially, infants
cessation of enteral feedings. Surgery for NEC refractory to with isolated cerebellar hemorrhage exhibit delayed communi-
medical treatment or for intestinal perforation occurs in up to cation skills, decreased social skills with more withdrawn
50% of cases and has increased mortality of 20% to 40% com- behavior, and impaired ability to attend to tasks. Thus, cerebel-
pared with infants who are able to be treated medically. The lar injury increases the risk for poor neurodevelopmental out-
cause of NEC is not established, but risk factors include pre- come in cognition, learning, and behavior in preterm infants.42
maturity, umbilical artery catheterization, asphyxia, congenital For long-term effects of cerebellar damage, refer to Chapter 21.
heart disease, blood transfusion, and enteral feedings. Several
viruses (adenovirus, enterovirus, and rotavirus) and bacteria Hypoxic-Ischemic Encephalopathy
have been implicated as causative agents for NEC. However, Perinatal asphyxia, the result of a hypoxic-ischemic (HI)
bacteremia may be a secondary finding because infants with insult, affects three to five per 1000 live births and leads to
NEC are frequently in a septic condition either at the time of hypoxic-ischemic encephalopathy (HIE) in 0.5 to one per
presentation of NEC or after intestinal perforation. 1000 live births. Impaired oxygen delivery to the fetus can
Complications of NEC include sepsis, wound infection, result from maternal hypotension, placental abruption, pla-
and stricture formation (10% to 35%) requiring repeated sur- cental insufficiency, cord prolapse, prolonged labor, and/or
gery. Growth of infants with NEC can be impaired due to traumatic delivery. Approximately 15% to 20% of infants
feeding intolerance, prolonged total parenteral nutrition (TPN), with HIE will die, and 25% of the surviving infants will
removal of significant amounts of intestine, and repeated sur- exhibit permanent neurological sequelae. Clinical findings
geries and infections. Persistence of weight at less than 10% will vary depending on the timing and duration of the HI
for age is correlated with poor neuromotor and neurodevelop- insult, preconditioning and fetal adaptive mechanisms,
mental outcome.39 Failure to achieve normalization of head comorbidities, and resuscitative efforts. Infants who are
intrauterine growth restricted are at increased risk of an HI a marked increase in signal in the subcortical and parasagittal
insult due to decreased nutrient reserves.43 white matter occurs as well as in the deep nuclear structures
It is important to note that the injury from an HI insult on DWI. MRI spectroscopy, localized to the basal ganglia or
is an evolving and progressive process that begins at the subcortical area, yields information about degree of second-
time of the insult and continues through the recovery period ary energy failure by analyzing for the depletion of the high-
(Figure 11-8). The HI insult causes decreased oxygen and energy compound N-acetylaspartate and the presence of
glucose delivery to the brain, causing a shift from aerobic to lactate.44,45 The degree of secondary energy failure as noted
anaerobic metabolism. This causes a decrease in adenosine on MRI spectroscopy is predictive of death and poor neuro-
triphosphate (ATP) production, leading to failure of the developmental outcome at 1 and 4 years of age.
membrane-bound Na1-K1-ATPase pump. Sodium enters the Classification of the clinical signs associated with HIE
neuronal cell, causing depolarization and release of excitatory is shown in Table 11-3.46 Infants with grade 1 HIE rarely
neurotransmitters, specifically glutamate. This initial phase have long-term sequelae. Infants with grade 2 or moderate
can last several hours and is marked by significant acidosis, HIE have abnormal tone and reflexes and decreased sponta-
depletion of high-energy compounds (energy failure), cellular neous activity, with seizures commonly present. Approxi-
swelling caused by entry of sodium and water, and cellular mately 10% of infants with moderate HIE will die and up
necrosis, causing spillage of intracellular contents into the to 30% will have neurodevelopmental delay. Infants with
extracellular space. The degree of neuronal necrosis is directly severe HIE (grade 3) exhibit minimal or no spontaneous
related to the duration and severity of the HI insult. During activity or reflexes. Clinically evident seizures are seldom
the subsequent reperfusion phase, free radical production in- present, but electrographically evident seizures are more
creases and activation of microglia from extruded intracellular common. Approximately 50% of these infants die, and
contents occurs, causing release of inflammatory mediators. A of the survivors, more than 60% to 80% are profoundly
second phase of energy failure ensues, but without acidosis. impaired. Long-term consequences of HIE include bulbar
Calcium enters the cell and the mitochondria, which then turns palsies with difficulties in sucking, swallowing, and facial
on the apoptotic pathway (programmed cell death). During movement. These infants have difficulty with secretions and
this second phase of energy failure, seizures are often present. may require tube feeding owing to inability to protect the
Activation of the apoptotic pathway accounts for the majority airway. Upper-extremity involvement is more prominent
of cellular death and is the target for treatment.27,28 The spe- than lower-extremity deficits because the damage to the
cific timing of the initiation of the reperfusion phase and the cerebral cortex is located in the parasagittal region (see
second phase of energy failure is unclear in the clinical setting Figure 11-7). The development of epilepsy occurs in about
because the actual timing of the HI insult is not well defined. 30% of infants with HIE. Mental retardation and difficulties
In animal studies, the latency between the first and second at school age occur frequently.
phases of energy failure is several hours. Mild hypothermia (33.5° C) from application of cooling
In term infants with HIE the cerebral damage is located in blankets or caps is becoming the standard of care for infants
the deep structures of the brain (basal ganglia, thalamus, and $ 36 weeks’ gestation who have an acute asphyxial event
posterior limb of the internal capsule) as well as the subcorti- and moderate or severe HIE.47 Hypothermia has been shown
cal and parasagittal white matter.44 Diffusion-weighted MRI to decrease cerebral metabolic demand and thus help pre-
(DWI) is a very early diagnostic and sensitive technique to serve high-energy compounds. Hypothermia also delays
identify damage after the HI insult. As shown in Figure 11-8, membrane depolarization and decreases neuronal excitotox-
icity. Free radical production and microglial activation are
decreased. Most important, the activation of the apoptotic
pathway is diminished. Transient side effects of hypother-
Hypoxic-Ischemic Encephalopathy (HIE)
mia, such as bradycardia, mild hypotension, thrombocytope-
nia, and persistent pulmonary hypertension, can be medically
Primary Insult
Reperfusion
treated and are usually not significant.48,49 A meta-analysis
Secondary
energy of published randomized studies comparing infants with
failure moderate and severe HIE treated with either hypothermia
Necrosis
or normothermia shows that hypothermic treatment signifi-
Apoptosis cantly decreases mortality and morbidity (Table 11-4).50
Hypothermia appears to more efficacious in ameliorating
Cerebral damage brain damage in infants with mild HIE than in infants with
morbidity and severe HIE. It is known that hypothermia is most effective
mortality
when administered before the onset of the second phase of
Figure 11-8 n Sequential events that occur after hypoxic- energy failure. Because the HI insult can occur before deliv-
ischemic (HI) insult. Initial event leads to cellular necrosis and ery, it is postulated that hypothermia should be initiated as
cerebral damage. Secondary energy failure and initiation of the quickly as possible after delivery to increase the likelihood
apoptotic pathway lead to the majority of injury after HI insult. that it will diminish the neuronal damage and improve neu-
Degree of secondary energy failure as measured by magnetic reso- rodevelopmental outcome.
nance imaging spectroscopy is highly correlated with neurodevel-
opmental outcome. (From Adams-Chapman I, Hansen NI, Stoll BJ, Maternal Medication
et al: Neurodevelopmental outcome of extremely low birth weight The impact of maternal medications on the developing fetal
infants with posthemorrhagic hydrocephalus requiring shunt inser- brain depends on the specific drug as well as on the timing
tion. Pediatrics 121:e1167–e1177, 2008.) and duration of the drug exposure. Whereas the insults
TABLE 11-3 n SARNET SCORING SCALE FOR ENCEPHALOPATHY
STAGE 1 (MILD) STAGE 2 (MODERATE) STAGE 3 (SEVERE)

Level of consciousness Hyperalert Lethargy, obtunded Stuporous
Neuromuscular signs
Muscle tone Normal Mild hypotonia Flaccid
Posture Mild distal flexion Distal flexion Decerebrate
Movement Spontaneous Decreased to little Noxious stimuli
Stretch reflexes Overactive Overactive Decreased or absent
Primitive reflexes
Suck Weak Weak or absent Absent gag
Moro Strong Weak or incomplete Absent
Autonomic function Sympathetic Parasympathetic Depressed
Dilated pupils Constricted pupils Pupils nonreactive
Tachycardia Bradycardia Variable heart rate
Copious secretions Apnea
Periodic breathing Loss of temp regulation
Seizures None Common Uncommon
Focal or multifocal
Modified from Sarnat HB, Sarnat MS: Neonatal encephalopathy following fetal distress. A clinical and electroencephalographic study. Arch Neurol
33:696-705, 1976.
TABLE 11-4 n EFFECT OF MODERATE HYPOTHERMIA ON NEUROLOGICAL OUTCOMES

AT 18 MONTHS COMPARED WITH CONTROLS
RISK RATIO NUMBER

(95% CONFIDENCE RISK DIFFERENCE NEEDED TO
INTERVAL [CI]) (95% CI) TREAT (95% CI) P VALUE
Death or severe disability* 0.81 (0.71-0.93) 20.11 (20.18-20.04) 9 (5-25) .002
Survival with normal outcome† 1.53 (1.22-1.93) 0.12 (0.06-0.18) 8 (5-17) ,.001
Mortality 0.78 (0.66-0.93) 20.07 (20.12-20.02) 14 (8-47) .005
Severe disability in survivors* 0.71 (0.56-0.91) 2 0.11 (20.20-20.03) 9 (5-30) .006
Cerebral palsy in survivors 0.69 (0.54-0.89) 20.12 (20.20-20.04) 8 (5-24) .004
Severe neuromotor delay in survivors‡ 0.73 (0.56-0.95) 20.10 (20.18-20.02) 10 (6-71) .02
Severe neurodevelopmental delay in survivors§ 0.71 (0.54-0.92) 20.11 (20.19-20.03) 9 (5-39) .01
Blindness in survivors 0.57 (0.33-0.96) 20.06 (20.11-0.00) 17 (9-232) .03
Deafness in survivors 0.76 (0.36-1.62) 20.01 (20.05-0.03) NA .47
From Edwards AD, Brocklehurst P, Gunn AJ, et al: Neurological outcomes at 18 months of age after moderate hypothermia for perinatal hypoxic ischaemic
encephalopathy: synthesis and meta-analysis of trial data. BMJ 340:c363, 2010.
*
Severe disability was defined in the CoolCap and TOBY trials as the presence of at least one of the following impairments: Mental Development Index score
of less than 70 (2 standard deviations below the standardized mean of 100) on the Bayley Scales of Infant Development; gross motor function classification
system level 3 to 5 (where the scale is from 1 to 5, with 1 being the mildest impairment); or bilateral cortical visual impairment with no useful vision.
The NICHD trial defined disability as a Mental Developmental Index score of 70 to 84 plus one or more of the following impairments: gross motor function
classification system level 2; hearing impairment with no amplification; or a persistent seizure disorder.
†
Survival with normal outcome was defined as survival without cerebral palsy and with a Mental Developmental Index score of more than 84, a Psychomotor
Developmental Index score of more than 84, and normal vision and hearing.
‡
Severe neuromotor delay was determined on the basis of a Psychomotor Developmental Index score of less than 70 in survivors.
§
Severe neurodevelopmental delay was determined on the basis of a Mental Developmental Index score of less than 70 in survivors.
discussed previously cause predominantly cellular necrosis of specific proteins that are produced. This topic is too exten-
and apoptosis, medications given to the fetus and preterm sive to be covered here and has been previously reviewed.51,52
infant cause alterations in the structure and function of This section will focus on heroin, methadone, cocaine, meth-
genetic material as well as activation of the apoptotic path- amphetamine, and selective serotonin reuptake inhibitors
way. The hypothesis that factors acting early in life have a (SSRIs) used in the treatment of maternal depression.
long-lasting impact on development is called the Barker
hypothesis or the fetal origins of adult disease. It is proposed Cocaine
that the biological value of this reprogramming is to prepare It is difficult to ascertain the exact frequency of cocaine
the fetus for maximal adaptation through methylation and use during pregnancy, but reports indicate that 1% to 45%
deacetylation of histones, thereby determining the quantity of females have used cocaine during their pregnancy.28
Cocaine is extracted from the leaves of the coca plant and Opioid use during pregnancy has been associated with
can be smoked, inhaled, or injected into the bloodstream. tubal pregnancies, premature rupture of membranes, uterine
Cocaine induces an intense and immediate euphoric state irritability, preterm labor, and preeclampsia. Infants exposed
and can be very addictive. Unlike with opioids, physical to opioids in utero are intrauterine growth retarded at birth,
dependence does not occur, but severe and intense cravings but methadone has a less severe impact on fetal growth.
last for several months and can recur for years after cessa- Infants exposed to opioids in utero are noted to have a
tion of cocaine use. decreased incidence of respiratory distress related to en-
In the adult the actions of cocaine are mediated through hanced surfactant production56 and decreased hyperbilirubi-
several different neurotransmitter pathways in the brain. nemia as a result of induction of the enzyme glucuronyl
Cocaine may cause hypertension, tachycardia, and periph- transferase used in the metabolism of bilirubin.
eral and coronary artery vasoconstriction via activation of Withdrawal from narcotics occurs 2 to 3 days after
adrenergic pathways. The sense of euphoria is mediated delivery, but signs can be evident as long as 2 weeks after
through dopamine pathways. Alterations in sleep-wake delivery. Methadone withdrawal usually occurs later than
cycles are caused by the blocking of serotonin uptake. withdrawal from morphine or heroin related to its long half-
During pregnancy, cocaine causes decreased blood flow to life. Infants in withdrawal (neonatal abstinence syndrome
the kidneys and is implicated in preterm labor, uterine [NAS]) exhibit gastrointestinal symptoms of vomiting and
irritability, premature rupture of membranes, and placen- watery stools; neurological signs such as tremors; hyperto-
tal abruption. nicity; high-pitched and incessant cry; hyperalert state; and
Cocaine negatively affects neuronal proliferation, migra- sweating and fever. Infants with NAS have decreased ability
tion, growth, and connectivity, which distorts neuronal corti- to nipple feed despite excessive sucking on a pacifier.
cal architecture. However, the effects of intrauterine exposure Seizures can be present in 2% to 11% of infants with NAS.
to cocaine are difficult to determine because cocaine use is Two commonly used scoring methods for severity of NAS
frequently associated with abuse of other illicit drugs, ciga- are the Lipsitz57 and the Finnegan58 scales. The Lipsitz scale
rettes, and alcohol. Other confounding variables include poor has 11 components that are scored from 0 to 3, with any
nutrition and limited prenatal care. In a large prospective score over 4 necessitating treatment.57 The Finnegan scale is
blinded study, more infants exposed to cocaine in utero were a more comprehensive assessment, with more than 30 ele-
delivered prematurely and exhibited decreased weight, length, ments, and treatment is recommended if the score is greater
and head circumference compared with matched controls.53 than 8.58 A quiet, dimly lighted environment, decreased au-
However, cocaine exposure did not affect the incidence of ditory stimulation, and swaddling or holding have been used
congenital abnormalities. to decrease neonatal irritability and pharmacotherapy. About
The vasoconstrictive properties of cocaine increase the 30% to 80% of in utero opioid-exposed infants will require
risk for HI injury and middle cerebral artery stroke. Neo- medical treatment for NAS with morphine and either cloni-
nates with prenatal cocaine exposure demonstrate tremors, dine or phenobarbital. The goal of treatment is to decrease
hypertonia, irritability, and poor feeding ability. Cocaine- irritability, improve nippling efforts, and decrease vomiting
exposed infants have abnormal sleep patterns and are at a and diarrhea.
threefold to sevenfold increased risk of sudden infant death Infants exposed to opioids in utero continue to demon-
syndrome (SIDS). No difference was found in developmen- strate tremulousness, hypertonicity, irritability, and increased
tal testing54 between cocaine-exposed infants and matched crying episodes. In addition, they are less able to interact
controls, but the tests did not effectively evaluate arousal, with people, demonstrate decreased age-appropriate free
emotional control, and social interaction.55 In utero cocaine play, and have delayed fine motor coordination. The inci-
exposure has been linked to increased incidence of behav- dence of apnea and SIDS is increased in opioid-exposed
ioral problems and special education referrals in school- infants. An appropriate and nurturing home environment
aged children. On fMRI, differences in the right frontal is essential after discharge from the hospital to maximize
cortex and caudate nucleus are evident and indicate abnor- neurodevelopmental outcome.59,60
malities in regulation of attention and cognitive abilities
referred to as executive function. Selective Serotonin Reuptake Inhibitors
SSRI medications such as fluoxetine (Prozac, Fontex, Sero-
Opioids mex, Seronil), sertraline (Zoloft, Lustral, Serlain, Asenta),
The opioids are used less frequently than cocaine during paroxetine (Paxil, Seroxat, Sereupin, Paroxat), fluvoxamine
pregnancy, as less than 5% of pregnant woman test positive (Luvox, Favoxil), escitalopram (Lexapro, Cipralex, Esertia),
for opioids. Morphine, a naturally occurring opiate, and and citalopram (Celexa, Seropram, Citox, Cital) are com-
heroin, a synthetic opioid, readily cross the placenta and are monly prescribed to treat depression and anxiety disorders.
highly addictive. Because heroin can be injected intrave- The SSRI drugs inhibit serotonin reuptake, potentiating
nously, there is an increased risk for infection, especially serotonergic neurotransmitter signaling. At least 600,000
endocarditis, hepatitis, and human immunodeficiency virus infants are born yearly to mothers who have a major depres-
(HIV) infection. Methadone, a synthetic opioid, is the stan- sive disorder during their pregnancy.61 Medical therapy is
dard for treatment of opioid dependence and has a signifi- the most common form of treatment for depression during
cantly longer half-life than heroin and morphine. Over the pregnancy. Approximately 6% of pregnant woman use
past several years, a trend for increasing rather than decreas- SSRIs during pregnancy, and almost 40% of depressed
ing methadone daily dose during pregnancy has helped to women have been reported to use antidepressants at some
decrease maternal illicit drug abuse without increasing the time during pregnancy.62 The serotonergic system is present
incidence of withdrawal symptoms in the infant. early in gestation and is important in brain development.
Perturbations in this system are associated with alterations with infants born at term gestation. In addition, the use of
in somatosensory processing and emotional responses. Medicaid, insufficient medical care, and maternal tobacco
The SSRI medications readily cross the placenta and are use were higher in the mothers of late preterm infants. These
linked to an increased risk of spontaneous abortion but not multiple factors, as well as the home environment, are sig-
an increased incidence of malformations.61 A recently pub- nificant risk factors in determining the effects of late preterm
lished meta-analysis found that maternal depression was delivery on long-term outcome. Regardless of the specific
significantly associated with an increased incidence of pre- insult, late preterm infants are at increased risk of neurode-
term labor and neonatal birth weight of less than 2500 g but velopmental disabilities and should receive timely develop-
not intrauterine growth retardation of the fetus.63 Unfortu- mental follow-up to identify potential underachievement
nately, this study was unable to evaluate the effect of SSRI and behavioral problems.
therapy on these outcomes. Infants exposed to SSRIs in the
third trimester have symptoms similar to withdrawal from CLINICAL MANAGEMENT: NEONATAL
opioid exposure (irritability, tremors, jitteriness, agitation, PERIOD
and difficulty sleeping). Neonatal feeding difficulties are Pediatric therapists with preceptor, subspecialty training in
common, and seizures and abnormal posturing are occasion- neonatology and infant therapy approaches can expand neo-
ally present. These symptoms are transient, appearing 2 to natal services by creating clinical protocols and pathways
4 days after birth and disappearing by the second week of designed to optimize the development and interaction of
life.64 It is difficult to identify any specific adverse neurode- neonates and parents. The therapeutic partnership between
velopmental outcomes in infants exposed prenatally to parents and neonatal therapists during developmental inter-
SSRIs from published studies because of the variability in vention in the NICU sets the stage for parental competency
the specific SSRI taken, the duration and timing of SSRI in caregiving and compliance with follow-up in the outpa-
use, and the confounding factors of maternal depression and tient period. General aims of NICU clinical management of
the use of multiple medications.65 infants at risk for neurological dysfunction, developmental
delay, or musculoskeletal complications are to (1) promote
Late Preterm Birth posture and movement appropriate to gestational age and
Preterm births have increased over the last 10 years and now medical stability; (2) support symmetry and biomechanical
constitute about 13% of all births. Late preterm infants— alignment of extremities, neck, and trunk while multiple
that is, infants born at 34 to 366⁄7 weeks’ gestation—make up infusion lines and respiratory equipment are required;
approximately 70% of preterm births.66 Many factors are (3) decrease potential skull and extremity musculoskeletal
implicated in the early delivery of late preterm infants, deformities and acquired joint-muscle contractures; (4) fos-
including preterm labor, preeclampsia, premature rupture of ter infant-parent attachment and interaction; (5) modulate
membranes, sepsis, and multiple gestation pregnancies. The sensory stimulation in the infant’s NICU environment to
late preterm infant is at increased risk of respiratory distress promote behavioral organization and physiological stability;
from insufficient surfactant production, transient tachypnea (6) provide consultation or direct intervention for neonatal
of the newborn from decreased pulmonary water absorption, feeding dysfunction and oral-motor deficits; (7) enhance
persistent pulmonary hypertension, and complications of parents’ caregiving skills (feeding, dressing, bathing, posi-
mechanical ventilation (pneumothorax). Hospital stay is tioning of infant for sleep, interaction and play, and trans-
prolonged in the late preterm infant compared with the portation); and (8) prepare for hospital discharge and
infant born at term gestation owing to the increased integration into home and community environments.
difficulty with oral feeding, need for phototherapy for
hyperbilirubinemia, and continuation of antibiotic therapy Educational Requirements for Therapists
for suspected sepsis. Examination of and intervention for neonates are advanced-
Recent evidence has supported the concept that even level, not entry-level, clinical competencies. Neonatology is
healthy late preterm infants are at higher risk for neurode- a recognized subspecialty within the specialty areas of pedi-
velopmental delay compared with infants at term gestation. atric physical therapy70 and pediatric occupational therapy.71
The late preterm infant’s brain is vulnerable to injury No amount of literature review, self-study, or experience
because a significant portion of brain development and with other pediatric populations can substitute for compe-
maturation occurs during the last 2 months of pregnancy.67 tency-based, clinical training with a preceptor in an NICU.
Recent studies have shown that late preterm infants tested The potential for causing harm to medically fragile infants
lower in reading skills in kindergarten and first grade, but during well intentioned intervention is enormous.72-74 The
not in math skills, than infants born at term gestation.68 ongoing clinical decisions made by neonatal therapists in
However, kindergarten and first-grade teachers rated late evaluating and managing physiological and musculoskeletal
preterm infants as not as competent as term infants in math risks while handling small (2 or 3 lb), potentially unstable
and reading ability. Significantly more late preterm infants infants in the NICU should not be a trial-and-error experi-
required special education in kindergarten and first grade ence at the infant’s expense. Therapists with adult-oriented
compared with control infants. A trend toward increased training and even those with general pediatric clinical train-
enrollment in special education in the third and fourth grade ing (excluding neonatal) are not qualified for neonatal prac-
was reported.68 Late preterm infants were considered at in- tice without a supervised clinical practicum (2 to 6 months).
creased risk for (1) developmental delay at 3 and 4 years of The NICU is not an appropriate practice area for physical
age, (2) retention in kindergarten, and (3) referral for special therapy assistants, occupational therapy assistants, or
education.69 However, maternal age and education were student therapists on affiliations for reasons outlined by
significantly decreased in late preterm infants compared Sweeney and colleagues8: “handling of vulnerable infants in
the NICU requires ongoing examination, interpretation, and include managing caseloads of hospitalized children on
multiple adjustments of procedures, interventions, and physiological monitoring equipment, external feeding lines,
sequences to minimize risk for infants who are physiologi- and supplemental oxygen or ventilators. Participating in
cally, behaviorally, and motorically unstable or potentially discharge planning and in outpatient follow-up of high-risk
unstable.” The physical or occupational therapy assistant neonates are other options for providing exposure to exami-
and student therapist are not prepared, even with supervi- nation, intervention, and family issues when the infants and
sion, to “provide moment-to-moment examination and eval- parents are more stable. This clinical experience and a
uation of the infant and have the ability to modify or stop competency-based, precepted practicum in the NICU offer
preplanned interventions when the infant’s behavior, motor, the best preparation for appropriate, accountable, and ethical
or physiological organization begins to move outside the practice in neonatal therapy.76-78 In-depth study of perinatal
limits of stability with handling or feeding.”8 Appropriate and neonatal medicine and related obstetrical, neonatal
nonhandling experiences for physical therapist or occupa- nursing, high-risk parenting, and neonatal therapy literature
tional therapist students in the NICU are delineated by is recommended before pediatric therapy clinicians begin to
Rapport and colleagues,75 with a wide range of observational participate on the intensive care nursery team.
learning experiences with a preceptor recommended in this
specialized practice environment. Refer to Box 11-1 for ap- Indications for Referral
propriate nonhandling experiences for entry-level students. Research efforts in recent years have been directed toward
Delineation of advanced-level roles, competencies, and determining which neonates will have adverse neurodevel-
knowledge for the physical therapist75-77 and the occupa- opmental outcomes. Specific prenatal, perinatal, and neona-
tional therapist71 in the NICU setting have been described tal conditions associated with an increased likelihood of
separately by national task forces from the American Physi- long-term neuromotor disability have been identified as risk
cal Therapy Association and the American Occupational factors. However, the predictive value of these risk factors is
Therapy Association. These practice guidelines provide a compromised by the absence of uniform or consistent defi-
structure for assessing competence of individual therapists nitions, differences in the study samples and follow-up
working in NICU settings and offer a framework for design- procedures, and lack of standard measures of neurodevelop-
ing clinical paths for specific neonatal therapy services. mental outcome. In addition, ongoing changes in obstetrical
A gradual, sequential entry to neonatal practice is and neonatal procedures limit the applicability of findings
advised by building clinical experience with infants born at from longitudinal studies of infants born in earlier eras of
term gestation as well as with physiologically fragile older NICU care.
infants and children and their parents. The experience may Tjossem’s79 categories of biological, established, and
social risk combined with risk factors for adverse neurode-
velopmental outcome80 provide a framework for categoriz-
BOX 11-1n NEONATAL INTENSIVE CARE UNIT
ing indicators for neonatal therapy referral. An overview of
(NICU) OBSERVATIONAL EXPERIENCES FOR developmental risk categories and risk factors for neonatal
ENTRY-LEVEL STUDENTS75 therapy referral is listed in Box 11-2 to assist clinicians in
developing a referral mechanism for a clinical protocol
n Reviewing neonatal literature and neonatal therapy clinical based on risk categories.
practice guidelines71,75-77 before site visit to NICU
n “Shadowing” neonatal nurses to observe: Biological Risk
n Neonatal equipment (refer to Table 11-6) Biological risk refers to neurodevelopmental risk attribut-
n Caregiving routines able to medical or physiological conditions in the prenatal,
n Teaching styles with parents and grandparents perinatal, or neonatal period.79-81 Biological risks include
n Feeding procedures and equipment placental abnormalities, labor and delivery complications,
n Unique culture of the NICU compared with adult prenatal infection, and teratogenic factors. Examples of bio-
intensive care units logical risk factors include asphyxia, neonatal seizures,
n Skin-to-skin holding by parent prenatal exposure to drugs or alcohol, and the brain lesions
n Environmental adaptations (light, sound, clustered previously described. Birth weight is a strong predictor of
handling) outcome; in general, lower birth weight is associated with
n “Shadowing” neonatal therapist to observe: greater risk of adverse developmental outcomes.82,83
n Chart reviews Respiratory disease is generally considered an important
n Interdisciplinary rounds risk factor for motor and cognitive disability in infants born
n Discharge planning conferences preterm (Table 11-5).84 Although the presence of respiratory
n Behavioral and physiological baseline examinations disease alone does not appear to be predictive of neurodevel-
n Examination and intervention procedures adapted opmental outcome, severity of disease does appear to be
for medically stable infants at varying gestational related to long-term outcome.82 Infants with chronic lung
ages, acuity levels, and behavioral organization disease or bronchopulmonary dysplasia have been found to
n Parental teaching be at increased risk for CP and other neurodevelopmental
n Collaboration with neonatal nurses for positioning, abnormalities compared with preterm infants without bron-
feeding, and parent instruction chopulmonary dysplasia.85,86 Prolonged mechanical ventila-
n Observing and participating with neonatal therapist in tion and duration of supplemental oxygen were associated
NICU Follow-up Clinic with increased risk of neurodevelopmental disability.87
Administration of surfactant in the neonatal period has
BOX 11-2 DEVELOPMENTAL RISK

n
reduced the incidence and severity of respiratory disease in
INDICATORS FOR NEONATAL THERAPY very low–birth-weight infants but has not been associated
REFERRAL with a decline in neurodevelopmental disability.85
BIOLOGICAL RISK Established Risk

Birth weight of 1500 g or less Established risk is the risk for neurodevelopmental deficits
Gestational age of 32 weeks or less associated with a diagnosis that is clearly established in the
Small for gestational age (less than 10th percentile for neonatal period. Included in this category are congenital
weight) malformations, chromosomal abnormalities, central ner-
Prenatal exposure to drugs or alcohol vous system disorders, and metabolic diseases with known
Ventilator requirement for 36 hours or more developmental sequelae.
Intracranial hemorrhage: grades 3 or 4
Periventricular leukomalacia Environmental and Social Risk
Muscle tone abnormalities (hypotonia, hypertonia, asymmetry Environmental and social risk involves developmental risk
of tone or movement) related to competency in parenting roles and factors in
Recurrent neonatal seizures (three or more) family dynamics. Such risk may be heightened by prolonged
Feeding dysfunction hospitalization of infants with the following characteristics:
Symptomatic TORCH infections (toxoplasmosis, rubella, (1) suboptimal levels of stimulation and interaction (over-
cytomegalovirus infection, herpesvirus type 2 infection) stimulation or deprivation) in the NICU environment, (2) inad-
Meningitis equate infant-parent attachment, (3) insufficient educational
Asphyxia with Apgar score less than 4 at 5 minutes preparation of parents for caregiving roles, (4) meager finan-
Multiple birth cial resources of parents, and (5) limited or absent family
ESTABLISHED RISK support to assist in taking care of and nurturing the infant in
Hydrocephalus the home environment.
Microcephaly It is common for neonates born preterm to have a com-
Chromosomal abnormalities bination of risk factors from more than one major category.
Musculoskeletal abnormalities (congenitally dislocated hips, For example, an infant born prematurely to a single mother
limb deficiencies, arthrogryposis, joint contractures, in a drug treatment program for heroin use during preg-
congenital torticollis) nancy is considered to be at both biological and environ-
Brachial plexus injuries (Erb palsy, Klumpke paralysis) mental risk.
Myelodysplasia
Congenital myopathies and myotonic dystrophy Pain, Gestational Age, and Neurological
Inborn errors of metabolism Examination
Human immunodeficiency virus infection Multiple neonatal neurological and neurobehavioral exami-
Down syndrome nations have been developed to assess the integrity and
ENVIRONMENTAL AND SOCIAL RISK
maturation of the nervous system88-91 and to describe new-
High social risk (single parent, parental age younger than
born behavior.9,89 Most of these tests offer information on
17 years, poor-quality infant-parent attachment)
the quality of motor performance, attention, and interaction.
Maternal drug or alcohol abuse
Because these assessments are based on gestational age, an
Behavioral state abnormalities (lethargy, excessive irritability,
accurate calculation of gestational age is necessary at the
behavioral state lability)
time of the testing.92,93
TABLE 11-5 n FACTORS CONTRIBUTING TO PULMONARY DYSFUNCTION IN PRETERM NEONATES
Anatomical Capillary beds not well developed before 26 weeks of gestation

Type II alveolar cells and surfactant production not mature until 35 weeks of gestation
Elastic properties of lung not well developed
Lung space decreased by relative size of the heart and abdominal distention
Type I, high-oxidative fibers compose only 10% to 20% of diaphragm muscle
Highly vascular subependymal germinal matrix not resorbed until 35 weeks of gestation, increasing infant’s vulnerability
to hemorrhage
Lack of fatty insulation and high surface area/body weight ratio
Physiological Increased pulmonary vascular resistance leading to right-to-left shunting
Decreased lung compliance
Diaphragmatic fatigue; respiratory failure
Decreased or absent cough and gag reflexes; apnea
Hypothermia and increased oxygen consumption
Modified from Crane L: Physical therapy for the neonate with respiratory disease. In Irwin S, Tecklin JS, editors: Cardiopulmonary physical therapy, ed 2,
St Louis, 1990, Mosby.
Pain Assessment between the N-PASS and the PIPP assessments during
Despite immature myelinization, premature infants defi- routine heelstick in infants younger than 1 month old
nitely perceive pain and retain the memory of painful experi- born at 23 to 42 weeks’ gestation.107
ences. Skin receptors are developed by 14 to 16 weeks’ Indicators of pain summarized across the instruments
gestation. In addition, the density of pain receptors in the include the following categories: (1) physiological (heart
skin of neonates at 28 weeks of gestation is considered rate, oxygen saturation, breathing pattern), (2) behavioral
similar to and even exceeds adult density during maturation (eye squeeze, brow bulge, facial grimace, behavioral state
from birth to 2 years of age.94-96 Blackburn97 explained that including crying, sleeplessness), and (3) motor (tone and
although pain transmission in neonates occurs mainly movement in extremities).
through the slower, unmyelinated C fibers, the shorter dis-
tance in neonates that impulses travel to reach the brain Clinical Assessment of Gestational Age
compensates for the slower rate of transmission and creates in the Newborn Infant
substantial pain reception. Early pain experiences may cre- A method for clinical assessment of gestational age in the
ate later increased sensitivity to pain and vulnerability to newborn infant was developed by Dubowitz and colleagues92
stress disorders.98-100 If neonatal therapy assessment or inter- from data derived from a total of 167 preterm and term
vention procedures immediately follow a noxious procedure infants (28 to 42 weeks’ gestation) tested within 5 days
in the NICU, handling techniques may need to be modified of birth. The tool focuses on criteria for calculation of
or therapy session rescheduled to avoid contributing to a gestational age from a composite of 10 neurological and
cascade of aversive experiences for the infant. 11 external (physical) characteristics.
Psychometric data and clinical use of the pain tools are This test rates criteria on a 4-point scale; it is commonly
described for infants as early as 28 weeks of gestation. administered by nurses or physicians in the newborn nurs-
Many elements in the pain assessments101 have been identi- ery. The accuracy (95% confidence limit) of the gestational
fied by Als (the Neonatal Individualized Developmental age score is determined within a variation of 62 weeks on
Care and Assessment Program [NIDCAP]) as signs of any single examination. This measurement error can be
excessive stimulation and stress in the preterm infant. decreased to approximately 61.4 weeks when two separate
Specific extremity movements, such as hand to face, ele- examinations are performed. From the analyses of multiple
vated leg extension, salute, lateral extension of arms, finger tests on 70 of the 167 infants, the age score was equally reli-
splay, and fisting, have been proposed as indicators of stress able in the first 24 hours of age as during the next 4 days of
and/or pain.102 life. The behavioral state of the infant during the examina-
In addition to practice guidelines on pain assessment tion is not considered a significant variable in testing.
developed primarily by neonatal nurses, numerous instru- Calculation of gestational age is an important adjunct to
ments are available to assess pain in infants. Pain scale data all other neonatal assessment tools. It guides practitioners
are integrated into NICU nursing assessments and can be a in interpreting neurological and behavioral findings relative
valuable adjunct to the neonatal therapist’s baseline and to the expected performance of neonates at various gesta-
posttherapy observations. tional ages. Additional guidelines on gestational differences
n The Premature Infant Pain Profile (PIPP)103 assigns in neurological, physical, and neuromuscular maturation
points for changes in three facial expressions (brow can be found in the work of French pediatric neurologist
bulge, eye squeeze, and nasolabial fold), heart rate, and Amiel-Tison.88,89,108
oxygen saturation. Gestational age and pre-procedural
behavioral state are included in the assessment. The Newborn Maturity Rating—Ballard Score
maximal PIPP score is 21; the higher the score, the Ballard and colleagues109-111 designed a simplified modifica-
greater the pain. A score of 0 to 6 points indicates mini- tion of the Dubowitz gestational age tool. It has been widely
mal or no pain, whereas a score of 12 or more indicates adopted because of the time efficiency (3 to 4 minutes ver-
moderate to severe pain.103 sus 10 to 15 minutes) and the elimination of active tone
n The Face, Legs, Activity, Cry, and Consolability items, which are difficult to evaluate reliably in physiologi-
Behavioral tool (FLACC) uses grades of 0 to 2 for cally unstable newborns. The Ballard instrument involves
facial expression, leg activity, general activity, cry only six physical and six neurological criteria, with a 0 to
nature, and ability to be consoled and has been used in 5 scale and a maturity rating. It is designed to be used for
pediatric and adult settings. This test is capable of as- neonates (20 to 44 weeks gestation) from birth through
sessing pain in normal as well as cognitively impaired 3 days of age and has demonstrated concurrent validity with
children, thus giving it a high degree of versatility and the Dubowitz gestational age calculation tool. The gesta-
usefulness.104 Change in FLACC score has been used tional age of the infant is based on the obstetrical dating
to demonstrate that the use of sucrose and a pacifier criteria unless the clinical assessment of the infant deviates
during venipuncture is more effective in consoling more than 2 weeks from the obstetrical calculation.
infants younger than 3 months of age than infants
older than 3 months of age.105 Neurological Examination of the Full-Term Infant
n The Neonatal Pain, Agitation, and Sedation Scale The Neurological Examination of the Full-Term Infant was
(N-PASS) uses five indicators: (1) cry and irritability, designed by Prechtl112 to identify abnormal neurological
(2) behavioral state, (3) facial expression, (4) extrem- signs in the newborn period. The examination was devel-
ity movement and tone, and (5) vital signs. As with the oped from an investigation of more than 1350 newborns and
PIPP scale, additional points are added for decreasing was standardized on infants born at the gestational age of
gestational age.106 There was good correlation 38 to 42 weeks. If the test is used in premature infants who
neuromotor responses within a behavioral state context. The

30- to 45-minute examination consists of observing, elicit-
ing, and scoring 28 biobehavioral items on a 9-point scale
and 18 reflex items on a 4-point scale. This is an interactive
test and assesses the infant’s ability to recover from stimuli
and return to an alert state. The reflex items are derived
from the neurological examination protocol of Prechtl and
Beintema.114
The scale was designed to assess newborn behavior in
healthy 3-day-old term (40 weeks of gestation) white infants
whose mothers had minimal sedative medication during an
uncomplicated labor and delivery. Use of this examination
with infants born preterm requires modification of the
examination procedure to the environmental constraints of
an intensive care nursery and interpretation of findings rela-
tive to the gestational age and medical condition of the
infant. For preterm infants approaching term gestation (min-
imum of 36 weeks of gestation), nine supplementary behav-
ioral items are offered. Many of these items were developed
by Als9 for use with preterm and physiologically stressed
infants (see discussion of the APIB, later). In the manual,113
methods of adapting the Neonatal Behavioral Assessment
Scale (NBAS) for preterm neonates with accompanying
case scenarios are described to illustrate use of the findings
to enhance parent-infant interaction and guide developmen-
tal interventions.
Six behavioral state categories are outlined in the NBAS:
deep sleep, light sleep, drowsiness or semidozing, quiet
alert, active alert, and crying. Behavioral state prerequisites
are provided for each biobehavioral and reflex item to
Figure 11-9 n Flow diagram illustrating decision steps in the reduce the state-related variables in testing. During the as-
neurological examination process. sessment the examiner systematically maneuvers the infant
from the sleep states to crying and back to the alert states to
evaluate physiological, organizational, motor, and interac-
have reached an age of 38 to 42 weeks of gestation, lower re- tive capabilities during stimulation and physical handling.
sistance to passive movements (lower tone) may be expected. The scoring is based on the infant’s best performance, with
Delay of testing until a minimum of 3 days of age is advised flexibility allowed in the order of testing, repetition of items
to maximize the stability of behavioral states and neuromotor encouraged, and scheduling of the assessment midway
responses for improved reliability and validity of results. between feedings to give the infant every advantage to
The pattern of examination includes periods of both demonstrate the best possible responses.
observation and examination. A 10-minute screening test Four dimensions of newborn behavior are analyzed in
is offered to determine if the full 30-minute examination Brazelton’s NBAS: interactive ability, motor behavior,
of posture, tone, reflexes, and spontaneous movement is behavioral state organization, and physiological organiza-
required. Although specific requirements for examiner train- tion. Interactive ability describes the infant’s response to
ing are not addressed, Prechtl offers a flow diagram (Figure visual and auditory stimuli, consolability from the crying
11-9) to assist clinicians with organizing the neurological state with intervention by the examiner, and ability to main-
examination process. Significant findings from the examina- tain alertness and respond to social or environmental stimuli.
tion are summarized in the following categories: (1) quality Motor behavior refers to the ability to modulate muscle
of posture, spontaneous movement, and muscle tone (con- tone and motor control for the performance of integrated
sistency and resistance to passive movement); (2) presence motor skills, such as the hand-to-mouth maneuver, pull-to-
of involuntary or pathological movements (clonus, tremor, sit maneuver, and defensive reaction (e.g., removal of cloth
athetoid postures or movements); (3) behavioral state from face). In the assessment of behavioral state organiza-
changes and quality of cry; and (4) threshold or intensity of tion, the infant’s ability to organize behavioral states when
responses to stimulation. (Because of the transient pattern of stimulated and the ability to shut out irritating environmen-
neurological signs and rapid changes in the developing ner- tal stimuli when sleeping are analyzed. Physiological orga-
vous system, Prechtl advised repeated examinations to nization is evaluated by observing the infant’s ability to
monitor neurological status.) manage physiological stress (changes of skin color, fre-
quency of tremulous movement in the chin and extremities,
Neonatal Behavioral Assessment Scale number of startle reactions during the assessment). For
To document individual behavioral and motor differences in analysis, the information is divided into seven clusters: ha-
infants at term gestation to 2 months of age, Brazelton and bituation, orientation, motor, range of state, regulation of
Nugent113 developed a neonatal behavior scale to assess state, autonomic stability, and reflexes. The cluster systems
are highly useful for clinical interpretation and for data the behavioral profile of the infant and allows the practitioner
analysis in clinical research. Performance profiles of worri- to provide parents with individualized and unique informa-
some or deficient interactive-motor and organizational tion about their infant. This behavioral information promotes
behavior are identified by clusters of behavior associated positive parent-infant interaction and also a positive partner-
with potential developmental risk.115 ship between parents and practitioners.
Definite strengths of the NBAS are the well-defined Certification in administering, interpreting, and scoring
indicators of autonomic stress, analysis of coping abilities the 18-item NBO assessment is arranged through the
of high-risk infants experiencing external stimuli and han- Brazelton Institute in a 2-consecutive-day format. The train-
dling, and quality of infant-examiner interaction. These ing encompasses the following observation categories:
features generate specific findings to assist therapists in (1) habituation to external light and sound; (2) muscle tone
grading the intensity of assessment and treatment within and motor activity level; (3) behavioral self-regulation (cry-
each infant’s physiological and behavioral tolerance and ing and consolability); and (4) visual, auditory, and social-
in guiding the development of parental teaching strategies interactive abilities.119,120
to address the individual behavioral styles of infants. The
NBAS has proved to be more sensitive to the detection Neurological Assessment of the Preterm
of mild neurological dysfunction in the newborn period and Full-Term Newborn Infant
than have classic neurological examinations that omit the The Neurological Assessment of the Preterm and Full-Term
behavioral dimensions. This assessment is not predictive Newborn Infant is a streamlined neurological and neurobe-
but gives a good analysis of the infant’s strengths and havioral assessment designed by Dubowitz and colleagues122
weaknesses. Improved performance from repeat examina- to provide both a systematic, quickly administered newborn
tions over time is a better predictor of the infant’s ability examination applicable to infants born preterm or at term
and potential. gestation and a longer infant examination for children to
Participation of the parent in the newborn assessment 24 months of age. A distinct advantage of this tool is the
may yield long-term positive effects on infant-parent inter- minimal training or experience required by the examiner and
action and later on cognitive and fine motor development. the ease of adapting it to the infant and the environment. The
Widmayer and Field116reported significantly better face-to- adaptability of the test and use of the scoring form with stick
face interaction and fine motor-adaptive skills at 4 months of figure diagrams have made it useful for implementation in
age and higher mental development scores at 12 months of developing countries where English is not widely spoken.
age when teenage mothers of preterm infants (mean gesta- The test includes the six behavioral state categories of the
tional age at birth, 35.1 weeks) were given demonstrations NBAS and seven orientation and behavior items scored on a
of the NBAS. These demonstrations were scheduled when 5-point grading scale and sequenced according to the inten-
the premature infants had reached an age equivalence of sity of response. The orientation and behavior items consist of
37 weeks of gestation. the following categories: (1) auditory and visual orientation
Nugent117,118developed parental teaching guidelines for responses; (2) quality and duration of alertness; (3) irritability
using the NBAS as an intervention for infants and their (the frequency of crying to aversive stimuli during reflex test-
families. Published by the March of Dimes birth defects ing and handling throughout the examination); (4) consolabil-
foundation, the guidelines offer strategies for interpreting ity (the ability after crying to reach a calm state independently
each item according to its adaptive and developmental sig- or with intervention by the examiner); (5) cry (quality and
nificance, descriptions of the expected developmental course pitch variations); and (6) eye appearance (absent, transient, or
of the behavior (item) over several months, and recommen- persistent appearance of sunset sign, strabismus, nystagmus,
dations for caregiving according to the infant’s response to or roving eye movements).
the items. The 15 items that assess movement and tone and the six
A three-step examiner training involving self-study, prac- reflex items evolved from clinical trials on 50 term infants
tice, and certification phases is coordinated through the Brazelton using the clinical assessment of gestational age by Dubowitz
Institute, Children’s Hospital, Boston, Massachusetts.119,120 and colleagues,92 the neurological examination of the new-
Wilhelm115 recommended NBAS training for clinicians begin- born by Parmelee and Michaelis,123 and the neurological
ning to develop competence in examining at-risk infants. She examination of the full-term newborn infant by Prechtl.112
explained that it provides a system for developing basic The examination format was then used during a 2-year
handling skills with healthy, term infants without concerns of period on more than 500 infants of varying gestational ages.
stressing medically fragile preterm infants during the training After 15 years the authors revised the assessment in the
period. Learning the NBAS in term infants before entering second edition by eliminating seven items, expanding the
NICU practice provides familiarity with similar testing and tone pattern section, and developing an optimality score.
scoring procedures for preterm infants.115 Reliability data are not reported, but modification of exami-
nation procedures occurred during the pilot phase that pro-
Newborn Behavioral Observations System moted objectivity in scoring and a high interrater reliability
The Newborn Behavioral Observations (NBO) system, among examiners, regardless of experience level.
developed from the pioneering work and philosophy of The examination protocol is available in two formats:
Brazelton, is an interactive, observational tool for use with (1) Hammersmith Short Neonatal Neurological Examination
infants and parents in hospital, clinic, and home settings.121 and (2) Hammersmith Infant Neurological Examination (age
The focus is on prematurely born infants and at-risk infants, range, 2 to 24 months). The examination forms are illustrated
with emphasis on cultural competence, family-centered care, with stick figures and can accommodate both baseline and
and infant development. The NBO system helps determine repeat assessments. For neonatal therapy examinations the
forms can be effectively combined with a narrative impres- infant depending on examiner experience and infant stabil-
sion, treatment goals, and plan of care. A numerical score for ity. Although the APIB may be an instrument of choice for
each item and a summary score are provided in the revised the clinical researcher, it is not usually practical (time effi-
edition of the test. The authors advised that the scoring sys- cient) for many neonatal clinicians with heavy caseloads
tem was primarily intended for the purpose of research and in managed-care environments. Extensive training and reli-
for numerical charting of progress with sequential examina- ability certification are required to safely administer and
tions. Because of the continued clinical emphasis on patterns accurately score and interpret the test for clinical practice or
of responses, selected parts of the protocol (without sum- research.
mary scoring) are appropriate for examining premature or
acutely ill infants on ventilators, in incubators, or attached to Neonatal Individualized Developmental Care
monitoring or infusion equipment. Scheduling of examina- and Assessment Program
tions is recommended two thirds of the way between infant Als11 and Als and colleagues11,129 developed NIDCAP to
feeding sessions. document the effects of the caregiving environment on
Evolution of neurological patterns in infants with IVH, the neurobehavioral stability of neonates. This naturalistic
PVL, and HIE is described in the test manual and correlated observation protocol includes continuous observation and
with brain imaging. Abnormal neonatal clinical signs as- documentation at 2-minute intervals of an infant’s behav-
sociated with long-term neurological sequelae were persis- ioral state and autonomic, motor, and attention signals, with
tent asymmetry, decreased lower-extremity movement, and simultaneous recording of vital signs and oxygen saturation.
increased tone. Infants with IVH had significantly higher Documentation occurs before, during, and after routine
incidence of abnormally tight popliteal angles, reduced caregiving procedures. The infant’s strengths, weaknesses,
mobility, decreased visual fixing and following, and roving and coping skills are identified. A narrative description of
eye movements. The authors cautioned that early signs of the infant’s responses to the stress of handling by the pri-
motor asymmetry in neonates with cerebral infarction may mary nurse and to auditory and visual stimuli in the NICU
be associated with normal outcome, but normal neonatal environment is provided to assist caregivers and parents in
neurological examinations after cerebral infarction do not identifying the infant’s behavioral cues and providing
exclude the possibility of later hemiplegia.124 appropriate interaction. Options are described in the care
Long-term follow-up data beyond 1 year have not been plans for reducing aversive environmental stimuli and mod-
reported with this examination. Dubowitz and colleagues125 ifying physical handling procedures. This clinical tool
reassessed 116 infants (27 to 34 weeks of gestation) at allows neonatal therapists to determine the infant’s readi-
1 year of age. Of 62 infants assessed as neurologically nor- ness for assessment and intervention by observing the base-
mal in the newborn period, 91% were also normal at 1 year line tolerance of the infant to routine nursing care before
of age. Of 39 infants assessed as neurologically abnormal in superimposing neonatal therapy procedures.130 Sequential
the newborn period, 35% were found to be normal at 1 year observations occur weekly or biweekly. Parental involve-
of age. According to Wilhelm,115 the predictive value of a ment is strongly encouraged and instrumental in facilitating
negative test result with this instrument was 92%, but the a smooth transition to home. Examiner training in the
predictive value of a positive test result was only 64%. NIDCAP may be coordinated through the National Training
Interpretations of evaluative findings from the Neuro- Center at Children’s Hospital Boston, Massachusetts, where
logical Assessment for Preterm and Full-Term Newborn priority is now given to training NICU teams rather than
Infants for neonatal therapy practice are comprehensively individuals.10
described in a case study format by Heriza126 and
Campbell.127 Dubowitz128 discussed the clinical signifi- NICU Network Neurobehavioral Scale
cance of neurological variations in infants and offered Lester and Tronick131 developed a tool for preterm and drug-
decision guidelines to clinicians on when to worry, reas- exposed infants from 30 weeks of gestation to 6 weeks post-
sure, or intervene with developmental referrals. term. The test includes items from the NBAS, APIB,
Finnegan abstinence scale, and other neurological assess-
Assessment of Preterm Infants’ Behavior ments and consists of 115 items in general categories of
Als9 designed the APIB to structure a comprehensive neurological and neuromotor integrity (tone, reflexes, and
observation of a preterm infant’s autonomic, adaptive, and posture), behavioral state and interaction (self-regulatory
interactive responses to graded handling and environmen- competence), and physiological stress abstinence signs
tal stimuli. It involves six maneuvers with increasing chal- (drug-exposed infants). This test is state dependent and
lenging and complex interactions with a highly structured gives a comprehensive and integrated picture of the infant
format. As previously described in the theoretical frame- that is not divided into clusters. More than half118 of the test
work section of this chapter, this assessment is derived items are infant observations, and 45 items require physical
from synactive theory and is focused on assessing the handling of the infant. Test-retest reliability of preterm in-
organization and balance of the infant’s physiological, fants indicated correlations of 0.30 to 0.44 at 34, 40, and
motor, behavioral state, attention and interaction, and self- 44 weeks of gestation. This test is useful for management
regulation subsystems. The APIB has testing sequences of drug-exposed infants but may have limited predictive
and a scoring format similar to those used in Brazelton’s value. Training and certification in administration and scor-
NBAS, with increased complexity and expansion for ing of the test are coordinated through Brookes Publishing
premature infants. Company and available in the United States and internation-
Administration and scoring of the APIB may require 2 to ally with use of videoconferencing for lectures and
3 hours per infant and often two or more sessions with the demonstrations.
Test of Infant Motor Performance that emerge during fetal life and continue until approxi-
Developed by Campbell and colleagues,132 the 42-item Test mately 16 weeks postterm, when goal-oriented and volun-
of Infant Motor Performance (TIMP) is focused on evaluat- tary movements appear.144 The quality of movement is
ing postural control, spontaneous movement, and head assessed through observation and scoring of videotaped
control for neonates at 32 weeks of gestation to 16 weeks spontaneous movement of an infant in supine position with-
postterm. Functional motor performance is assessed through out stimulation or handling.144 A distinct difference occurs
observation of infant movement and through responses to between the GMs in the preterm infant and those in the term
various body positions and to visual or auditory stimuli. and postterm infant. GMs in the term infant, and for the first
Psychometric qualities of the test include (1) construct 8 weeks, change in amplitude and speed, taking on a writh-
validity133 and ecological validity,134 (2) concurrent validity ing quality. The writhing movements gradually give way to
at 3 months of age with the Alberta Infant Motor Scale the fidgety movements, which are present in awake infants
(AIMS),135 and (3) predictive validity at 5 to 6 years of age between 9 and 16 to 20 weeks postterm. Fidgety movements
with the Bruininks-Oseretsky Test of Motor Proficiency136 are small, circular movements of small amplitude and vary-
and at 4 to 5 years of age with the Peabody Developmental ing speed involving the neck, trunk, and extremities.140
Motor Scales and Home Observation for Measurement of Other approaches to neurological assessment can be found
the Environment: Early Childhood.137 Training on test pro- in additional references.140,144,145
cedures is available through 2-day workshops or through a This neonatal and young infant assessment instrument
self-guided training method with a CD-ROM from the test has gained substantial attention in the past 20 years for its
developer.138 high reliability, sensitivity, and predictive validity. In a com-
prehensive review of the psychometric qualities of neuro-
Neurobehavioral Assessment of the Preterm Infant motor assessments for infants, the GM assessment was rated
The Neurobehavioral Assessment of the Preterm Infant among the tools with the highest reliability, averaging inter-
(NAPI) was developed by Korner as a developmental test to rater and intrarater correlation coefficient, or k, greater than
assess medically stable infants from 32 weeks to term gesta- 0.85.146 Multiple studies have corroborated the predictive
tion using a sequence of specific movements. This test validity and sensitivity of this method. The sensitivity is
focuses on tone, reflexes, movement, response to visual and lower during the preterm period and during the writhing
auditory stimulation, and observation of cry and state. This movements, improving during the fidgety movement period.
tool does not require a specific preassessment state as is Sensitivity as high as 95% has been reported.140,147
required by the previously mentioned tests, but starts with
the infant asleep. It does not take as long to administer (less Testing Variables
than 1⁄2 hour) than the previously described tests and is easy Neuromuscular and behavioral findings in the newborn
to analyze. The data are categorized into seven clusters and period may be influenced by several variables. Increased
compared with standardized scores. With repeated examina- reliability in examination results and in clinical impressions
tions over time, persistent deviations from the normative may occur when these variables are recognized. Medication
scores indicate that the infant is at risk for developmental may produce side effects of low muscle tone, drowsiness,
delays and is in need of close follow-up. In addition, the and lethargy. Such medications include anticonvulsants,
NAPI has been shown to be predictive of short-term and sedatives for diagnostic procedures (CT scan, electroen-
long-term neurodevelopmental outcomes.139 cephalography, electromyography), and medication for
postsurgical pain management. Intermittent subtle seizures
Qualitative Assessment of General Movements may produce changes in muscle tension and in the level of
The assessment tools reviewed so far in this chapter require responsiveness. Mild, ongoing seizures may occur in the
direct handling of the infant. Infants born preterm are par- neonate as lip smacking or sucking, staring or horizontal
ticularly vulnerable to developing physiological stress dur- gaze, apnea, and bradycardia. Stiffening of the extremities
ing the maneuvers required by most tools available for occurs in neonatal seizures more frequently than clonic
infant assessment. Instead, noninvasive, repeated longitudi- movement. Fatigue from medical and nursing procedures
nal observation and assessment are needed to accommodate can result in decreased tolerance to handling, decreased
the concurrent motor variability, immature nervous system, interaction, and magnified muscle tone abnormalities.
and physiological vulnerability of the young, preterm Fatigue may also result when neurodevelopmental assess-
infant.140 Based on the pioneering work of Prechtl examin- ment is scheduled immediately after laboratory (hemato-
ing the continuity of prenatal to postnatal fetal movement,141 logic) procedures, suctioning, ultrasonography, or respira-
this criterion-referenced test focuses on evaluating the qual- tory (chest percussion) therapy. Tremulous movement in the
ity of spontaneously generated movements in preterm, term, extremities may be linked to conditions of metabolic imbal-
and young infants until 16 weeks postterm. A wide reper- ance (hypomagnesemia, hypocalcemia, hypoglycemia), and
toire of endogenously generated spontaneous motility in the low muscle tone may be associated with hyperbilirubinemia,
fetus including isolated limb movements, stretches, hiccups, hypoglycemia, hypoxemia, and hypothermia.148,149
yawning, and breathing movements can be identified as
early as 9 weeks.141-143 Summary
General movements (GMs) are spontaneously generated Practitioners must be aware of the normative and validation
complex movements involving the trunk, limbs, and neck. data and of the predictive characteristics of the test(s)
They vary in speed and intensity with a gradual onset, administered to allow appropriate interpretation of the re-
increase in speed and intensity, and a gradual end. These sults. Specific clinical training with a preceptor is essential
movements are among the number of movement patterns to administer, score, and interpret neonatal assessment
instruments accurately; to establish interrater reliability; In this subspecialty area of pediatric practice, neonatal
and to plan treatment based on the evaluative findings. therapists are responsible for the prevention of physiological
Even low-risk, healthy preterm infants are vulnerable to jeopardy in LBW infants while providing developmental
becoming physiologically and behaviorally destabilized services in the NICU. Before examination, discussion with
during neurological assessment procedures.150-152 This risk the supervising neonatologist and clinical nurse are advised
is reduced with precepted, competency-based clinical regarding specific precautions and the safe range of vital
training in the NICU. signs for each infant. Medical update and identification of
new precautions before each intervention session are recom-
Intervention Planning mended because new events in the last few hours may not
Level of Stimulation have been recorded or fully analyzed at the time therapy is
The issue of safe and therapeutic levels of sensory and scheduled. The nurse should be invited to maintain ongoing
neuromotor intervention is a high priority in the design of surveillance of the infant’s medical stability and provide as-
developmental intervention programs for infants who have sistance in interpreting physiological and behavioral cues
been medically unstable. The concept of “infant stimula- during neonatal therapy activities in case physiological com-
tion,” introduced by early childhood educators in the 1980s plications occur. If medical complications develop during or
to describe general developmental stimulation programs for after therapy, immediate, comprehensive co-documentation
healthy infants, is highly inappropriate in an approach based of the incident with the clinical nurse and discussion with the
on concepts of dynamic systems, infant behavioral organiza- neonatology staff are essential to analyze the events, outline
tion, and individualized developmental care. related clinical teaching issues, and minimize legal jeopardy.
For intervention to be therapeutic in a special care nurs- Areas of particular concern during neonatal therapy
ery setting, the amount and type of touch and kinesthetic activities include potential incidence of fracture, dislocation,
stimulation must be customized to each infant’s physiolog- or joint effusion during the management of limited joint
ical tolerance, movement patterns, unique temperament, motion; skin breakdown or vascular compromise during
and level of responsiveness. Rather than needing more splinting or taping to reduce deformity; apnea or bradycar-
stimulation, many infants, especially those with hyperto- dia during therapeutic neuromotor handling with potential
nus or those with tremulous, disorganized movement, have deterioration to respiratory arrest; oxygen desaturation or
difficulty adapting to the routine levels of noise, light, regurgitation and aspiration during feeding assessment or
position changes, and handling in the nursery environment. oral-motor therapy; hypothermia from prolonged handling
General, nonindividualized stimulation can quickly mag- of the infant away from the neutral thermal environment of
nify abnormal postural tone and movement, increase the incubator or overhead radiant warmer; and propagation
behavioral state lability and irritability, and stress fragile of infection from inadequate compliance with infection con-
physiological homeostasis in preterm or chronically ill trol procedures in the nursery. Signs of overstimulation may
infants. Implementation of careful physiological monitor- include labored breathing with chest retractions, grunting,
ing and graded handling techniques are essential to prevent nostril flaring, color changes (skin mottling, paleness, gray-
compromise in patient safety and to facilitate development. blue cyanotic appearance), frequent startles, irritability or
Infant modulation, rather than stimulation, is the aim of drowsiness, sneezing, gaze aversion, bowel movement,
intervention. Techniques of sensory and neuromotor facili- and hiccups. Signals of overstimulation expressed through
tation and inhibition developed for caseloads of healthy infants’ motor systems are finger splay (extension and
infants and children are inappropriate for the developmen- abduction posturing), arm salute (shoulder flexion with el-
tal needs and expectations of an infant with physiological bow extension), and trunk arching away from stimulation.11
fragility or premature birth history (less than 37 weeks of Harrison and colleagues154 found that motor activity cues of
gestation). preterm infants were correlated with low oxygen saturation
and should be carefully monitored during caregiving proce-
Physiological and Musculoskeletal Risk dures to minimize physiological instability.
Management Even a baseline neurological examination, usually pre-
Many maturation-related anatomical and physiological sumed to be a benign clinical procedure, may be destabiliz-
factors predispose preterm infants to respiratory dysfunction ing to the newborn infant’s cardiovascular and behavioral
(see Table 11-5). For this reason many preterm neonates organization systems. The physiological and behavioral tol-
require the use of a wide range of respiratory equipment and erance of low-risk preterm and term neonates to evaluative
physiological monitors (Table 11-6). Pediatric therapists handling by a neonatal physical therapist was studied in
preparing to work in the NICU and those involved with 72 newborn subjects.151 During and after administration of
designing risk management plans are referred to the neona- the Neurological Assessment of the Preterm and Full-Term
tal nursing literature for evidence and perspectives on Newborn Infant, preterm subjects (30 to 35 weeks of gesta-
assessing and managing neonatal stressors during intervention) had significantly higher heart rates; greater increases in
tions in the NICU.153-155 Because infants born prematurely blood pressure; decreased peripheral oxygenation inferred
or experiencing critical illness communicate via subtle from mottled skin color; and higher frequencies of finger
behavioral cues, their understated language is “not easily splay, arm salute, hiccups, and yawns than in term subjects.
interpreted unless caregivers understand how infants’ ability Neonatal practitioners must examine the safety of even a
to respond to stress reflects their maturation and neurodevel- neurological examination and weigh the risks and antici-
opment.”156 Their behavioral cues are considered more sub- pated benefit of the procedure given the expected physiolog-
tle and more likely to be disregarded than those of infants ical and behavioral changes in low-risk, medically stable
born at term gestation. neonates.150,151
TABLE 11-6 n EQUIPMENT COMMONLY ENCOUNTERED IN THE NEONATAL INTENSIVE CARE

UNIT (NICU)
EQUIPMENT DESCRIPTION
Thermoregulation radiant
warmer Unit composed of mattress on an adjustable tabletop covered by a radiant heat source controlled manually
and by servocontrol mode. Unit has adjustable side panels.
Advantage: provides ready access to infant and increases area for equipment.
Disadvantage: leads to convective heat loss, increases insensible fluid loss, and encourages stimulation
(excessive).
Double-walled Isolette Enclosed unit of transparent material providing a heated and humidified environment with a servocontrol
incubator system of temperature monitoring.
Access to infant through side portholes or opening side of unit.
Advantage: barrier to tactile stimulation, decreased convective losses.
Disadvantage: more difficult to get to infant, does not decrease noise from NICU, radiant heat loss if
Isolette is single walled.
Thermal shield Clear acrylic dome or plastic wrap placed over the trunk and legs of an infant in an Isolette to reduce
radiant heat loss.
Respiratory assistance
Conventional pressure Delivers positive-pressure ventilation; pressure limited, with volume delivered dependent on the stiff-
ness of the lung.
Volume ventilator Delivers positive-pressure ventilation; volume limited, delivering same tidal volume with each breath,
potentially decreasing barotraumas, as most use minimal pressure required to deliver a set tidal
volume; common ventilator in use.
High frequency ventilator Ventilator that delivers short bursts of air at high rates of flow (240-600 breaths/min).
Jet Active inhalation with passive exhalation; requires conventional ventilator, noisy.
Oscillatory Piston driven; active inhalation and exhalation.
Continuous positive air- Nasal prongs of varying lengths provide CPAP and controlled oxygen delivery. Using bubble CPAP,
way pressure (CPAP) positive pressure is adjusted by altering the depth of the expiratory tubing, which is under liquid.
device CPAP prongs can also be connected to a mechanical ventilator to deliver adjustable pressure
and a breath rate if required.
Nasal cannula Specific concentration of oxygen is delivered via soft nasal cannula, usually less than 1 L/min.
High-flow nasal cannula delivers humidified oxygen at flows up to 6 L/min and a variable amount
of distending pressure to help with alveolar inflation.
Oxyhood Clear acrylic plastic hood fitting over the infant’s head to provide an environment for delivering con-
trolled oxygen and humidification delivery.
Monitors
Cardiac, respiratory One unit will display heart rate, respiratory rate, and blood pressure. High and low alarm limits can be set.
Oxygen saturation Measures peripheral oxygen saturation and pulse from a light sensor secured to the infant’s skin. Values
can be displayed on the monitor.
Transcutaneous Noninvasive method of monitoring partial pressure of O2 and CO2 from arterialized capillaries through
the skin through the use of a heated sensor.
Cerebral oxygenation Noninvasive method to measure regional oxygen saturation, usually cerebral and somatic, to ensure
adequate oxygen delivery.
Amplitude-integrated Continuous recording of cerebral electrical activity used to evaluate presence of seizures, baseline brain
electroencephalogra- activity, and brain maturation.
phy (aEEG)
Intravenous catheter Used to deliver intravenous fluids, intralipids, and medications at a specific rate and to assist in obtaining
blood for analysis. Specific catheters include arterial and venous umbilical lines, peripherally inserted central
catheters (PICCs), surgically placed central catheter (Broviac, Cook), and peripheral intravenous catheter.
Extracorporeal mem- Heart-lung-kidney machine used for term infants with severe respiratory or cardiac failure.
brane oxygenation
(ECMO)
High-Risk Profiles The first high-risk profile involves the irritable, hypertonic
Three general high-risk profiles are observed from a infant. These infants classically have a low tolerance level to
dynamic systems perspective. In these profiles movement handling and may frequently reach a state of overstimulation
abnormalities, related temperament or behavioral character- from routine nursing care, laboratory procedures, and the
istics, and interactional styles associated with motor status presence of respiratory and infusion equipment. They
are identified. may express discomfort when given quick changes in body
position by caregivers and when placed in any position for a hypertonic and irritable. Caregiving for intermittently hy-
prolonged time. Predominant extension patterns of posture pertonic, disorganized, irritable infants can be frustrating
and movement are associated with this category of infants. for parents unskilled in reading the infant’s cues, in imple-
Quality of movement may appear tremulous or disorganized, menting consolation and containment strategies, and in
with poor midline orientation and limited antigravity move- using pacing techniques during feeding. This profile of in-
ment into flexion as a result of the imbalance of increased fant motor and behavioral disorganization represents a large
proximal extensor tone. Visual tracking and feeding may proportion of infants in a typical neonatal therapy caseload.
be difficult because of extension posturing or the presence Although these profiles address the extremes in motor and
of distracting, disorganized upper-extremity movement. In behavioral interaction, they suggest a need for identifying dif-
addition, increased tone with related decreased mobility in ferent tolerance levels of handling for neonates with abnormal
oral musculature may complicate feeding behavior. Hyper- tone and movement even though long-term developmental
tonic infants frequently demonstrate poor self-quieting abili- goals may be similar. Few neonates will demonstrate all be-
ties and may require consistent intervention by caregivers to haviors described in the high-risk profile, but outpatient sur-
tolerate movement and position changes. These temperament veillance of neonates with worrisome or mildly abnormal
characteristics and the signs of neurological impairment premotor and interactive behavior is advised to monitor the course
viously discussed may place infants at considerable risk for of those behaviors and the developing styles of parenting.
child abuse or neglect as the stress and fatigue levels of par-
ents rise and as coping strategies wear thin during the Timing
demanding care required by irritable, hypertonic infants.7,157 The timing of neurodevelopmental examination and treat-
Hypertonic, irritable infants constituted large percentages of ment for infants with high-risk histories or diagnoses is
neonatal therapy caseloads in the 1970s through the 1990s, based on the medical stability of the infant and, in some
but advances in neonatal pulmonary management have now centers, gestational age. All therapy activities need to be
decreased the numbers of infants matching this neurobehav- synchronized with the intensive care nursery schedule so
ioral profile. that nursing care and medical procedures are not interrupted.
Conversely, the lethargic, hypotonic infant excessively Neonatal therapists should not interrupt infants in a quiet,
accommodates to the stimulation of the nursery environ- deep sleep state but instead wait approximately 15 minutes
ment and can be difficult to arouse to the awake states, even until the infant cycles into a light, active sleep or semiawake
for feeding. The crying state is reached infrequently, even state. Higher peripheral oxygen saturation has been corre-
with vigorous stimulation. The cry is characteristically lated with quiet rather than with active sleep in neonates.
weak, with low volume and short duration, and related to Preterm infants reportedly have a higher percentage of active
hypotonic trunk, intercostal, and neck accessory muscula- sleep periods in contrast to the higher percentage of quiet
ture and decreased respiratory capacity. These infants are sleep observed in term infants.158 Allowing the preterm infant
exceedingly comfortable in any position, and when held to maintain a deep, quiet sleep by not interrupting is a thera-
they easily mold themselves to the arms of the caregiver. peutic strategy for enhancing physiological stability.
Depression of normal neonatal movement patterns is com- Timing of parental teaching sessions is most effective
mon. To compensate for low muscle tone when in the supine when readiness to participate in the care of the infant is
position, some preterm infants appear to push into extension expressed. Some parents need time and support to work
against the surface of the mattress in search of stability. through the acute grief process related to the birth of an
Although potentially successful in generating a temporary imperfect child before participation in developmental activities
increase in neck and trunk tone, the extension posturing is accepted. Other parents find the neonatal therapy program to
from stabilizing against a surface in supine lying interferes be a way of contributing to the care of their infant that also
with midline and antigravity movement of the extremities. helps them cope with overwhelming fears, stresses, and grief.
Such infants dramatically respond to containment position-
ing in side-lying and prone positions. Drowsy behavior Treatment Strategies
limits these infants’ spontaneous approach to the environ- This section addresses components of treatment for enhanc-
ment and decreases their accessibility to selected interaction ing movement, minimizing contractures and deformity, pro-
by caregivers. Feeding behavior is commonly marked by moting feeding behaviors appropriate to corrected age, devel-
fatigue, difficulty remaining awake, weak sucking, and inco- oping social interaction behaviors, and fostering attachment
ordination or inadequate rhythm in the suck-swallow pro- to primary caregivers. Management approaches to body posi-
cess, with the need for supplementation of caloric intake by tioning, extremity taping, graded sensory and neuromotor
gavage (oral or nasogastric tube) feeding. The risk for sen- intervention, neonatal hydrotherapy, and oral-motor and feed-
sory deprivation and failure to thrive is high for hypotonic ing therapy are presented; parental teaching is discussed here.
infants because they infrequently seek interaction, place few Evidence-based practice recommendations for neonatal ther-
if any demands on caregivers, and remain somnolent. apy are outlined in Table 11-7 and Box 11-3. In managing an
The third high-risk profile is the disorganized infant with intensive care unit caseload, the constant physiological moni-
fluctuating tone and movement who is easily overstimulated toring; modification of techniques to adapt to the constraints
with routine handling but remains relatively passive when left of varying amounts of medical equipment; scheduling of
alone. Disorganized infants usually respond well to swaddling interventions to coincide with visits of the parents and peak
or containment when handled. When calm, these infants fre- responsiveness of the infants; and ongoing coordination and
quently demonstrate high-quality social interaction and effi- reevaluation of goals, plans, and follow-up recommendations
cient feeding with coordinated suck-swallow sequence. When with the nursery staff create many interesting challenges and
distracted and overstimulated, however, these infants appear demand a high degree of adaptability and creativity from the
TABLE 11-7 n EVIDENCE-BASED RECOMMENDATIONS FOR NEONATAL PHYSICAL THERAPY
LEVEL OF
TYPE RECOMMENDATIONS EVIDENCE REFERENCES
Prevention Collaborate with caregivers to reduce risk of skull deformity, torticollis, Level II Van Vlimmeren et al, 2007244
and extremity malalignment through diligent positioning for symmetry Level II Vaivre-Douret et al, 2004303
and neutral alignment Level II Monterosso et al, 2003169
Examination Conduct baseline observation to determine physiological and behavioral Level II Sweeney, 1986150
stability (readiness) for evaluative handling
Provide continuous physiological and behavioral monitoring during Level II Sweeney, 1989151
and after evaluative handling to determine adaptation to evaluative
handling and to signal the need for modification of pace and
sequence, given expected physiological changes, particularly
during neuromotor test procedures
Intervention Collaborate with caregivers to create a developmentally supportive Level I Symington and Pinelli, 2006304
environment with modulated stimulation from light, noise, and Level II Westrup et al, 2004305
handling Level I Peters et al, 2009306
Support body position and extremity movement—(1) supine: semiflexed, Level II Vaivre-Douret et al, 2004303
midline alignment using blanket for swaddling containment or “nest” of Level II Monterosso et al, 2003169
positioning rolls; (2) prone: vertical roll under thorax; horizontal roll Level II Short et al, 1996307
under hips Level II Ferrari et al, 2007166
In selected neonates with movement impairment or disorganization Level II Girolami and Campbell, 1994308
consider therapeutic handling carefully graded in intensity and paced
to facilitate head and trunk control, antigravity movement, and
midline orientation
Consider gradual exposure to multimodal stimuli for stable neonates Level I Symington and Pinelli, 2006304
approaching hospital discharge
Provide opportunities for independent oral exploration through Level I Pinelli and Symington, 2005197
positioning with hands to face, and for nonnutritive sucking to
improve state organization and readiness to feed
Determine readiness for and advancement of oral feeding trials using Level II Kirk, Alder, and King, 2007198
infant behavioral cues Level II McGrath and Medoff-Cooper,
2002309
Encourage parental involvement with feeding while providing interventions Level III Law-Morstatt et al, 2003206
for physiological stability (pacing and slowed flow rate) Level II Chang et al, 2007207
Consider hydrotherapy before feeding for stable infants with movement Level IV Sweeney, 2003191
impairment
Education Educate parents on behavioral cues and developmental status to mitigate Level II Kaaresen et al, 2006310
parental stress and improve parental mental health outcomes Level I Melnyk et al, 2006311
Implement multiple methods of instruction for parents and caregivers Level V Dusing, Murray, and Stern,
(demonstration, discussion, video, and written materials) 2008212
From Sweeney JK, Heriza CB, Blanchard Y, Dusing SC: Neonatal physical therapy. Part II: Practice frameworks and evidence-based practice guidelines. Ped
Phys Ther 22:2-16, 2010.
clinician. Willingness to change an established assessment Positioning

plan, treatment strategy, or therapy schedule to meet the A diligently administered positioning program can greatly
immediate needs of the infant, parents, or nursery staff is assist infants on mechanical ventilators, receiving hood oxy-
paramount. For some infants with prolonged periods of only gen, or in incubators to simulate the flexed, midline postures
borderline stability with handling, a discharge examination of the neurologically intact term newborn swaddled in a bas-
with recommendations for follow-up care may be the best sinet. Preterm infants characteristically demonstrate low
practice. Productivity standards of billable hours used for postural tone, with the amount of hypotonia varying with
other caseloads of stable pediatric or adult clients in the hos- gestational age. Infants born prematurely do not have the
pital are not appropriate for the NICU setting and necessitate neurological maturity or the prolonged positional advantage
negotiation and reinterpretation with rehabilitation or therapy of the intrauterine environment to assist in the development
department managers to protect both the infant and the neo- of flexion. They are instead placed unexpectedly against
natal therapist. Tolley159 reported an expected mean produc- gravity and presented with a dual challenge of compensating
tivity of 5.0 billable hours (range 4 to 6.5 hours) for hospital- for maturation-related hypotonia and adapting to ventilatory
based pediatric physical therapists among hospitals surveyed and infusion equipment that frequently reinforces extension
across 32 states and the District of Columbia.93 of the neck, trunk, and extremities.
Shaping of the musculoskeletal system occurs during

BOX 11-3 n HIERARCHY OF EVIDENCE each body position experienced by neonates in the NICU.
A variety of positional deformities in the extremities and
LEVEL I skull can result from inattention to alignment. In Table 11-8,
Randomized controlled trials (RCTs) or systematic reviews common neonatal positional deformities, musculoskeletal
of RCTs consequences, and functional limitations are outlined. Sup-
LEVEL II porting the skeletal integrity of infants born prematurely is
Small RCTs, cohort studies, or systematic reviews of cohort challenging in the midst of numerous equipment obstacles,
studies restricted physical handling because of physiological insta-
LEVEL III bility, and limited spontaneous movement. Infants with
Case-control studies or systematic reviews of case-control gastroesophageal reflux are frequently positioned on wedges
studies that make symmetrical, midline postures difficult to main-
tain. Skull flattening may continue to evolve after NICU
LEVEL IV discharge from overuse of infant seats, reflux wedges, and
Case series (no control group) limited prone play experiences. Plagiocephaly (asymmetri-
LEVEL V cal occipital flattening) and a secondary torticollis may
Opinion of experts or authorities emerge when a strong head turn preference remains and
parents do not vary the direction of head turn for sleeping
Data from Oxford Centre for Evidence-Based Medicine, www.cebm.net, and infant seat use.
adapted with permission from Sweeney JK, Heriza CB, Blanchard Y, Retracted shoulder posture (scapular adduction with
Dusing S: Neonatal physical therapy. Part II: practice frameworks and shoulder elevation and external rotation) may accompany
evidence-based guidelines. Pediatr Phys Ther 22:13, 2010. excessive neck and trunk extension posture in preterm
infants. This abnormal posture can interfere with later reach-
Imbalance of excessive extension may occur in preterm ing, shoulder stability in the prone position, and rolling dur-
infants with prolonged mechanical ventilation who appear to ing the first 18 months of life.162,163 Excessive tibial
gain postural stability in the nonfluid extrauterine environ- torsion and out-toeing gait were reported in preterm infants
ment by leaning into or stabilizing against a firm mattress at 3 to 8 years of age and traced to prolonged “frog leg”
while in the supine position. De Groot160 explained the pos- (excessive abduction and external rotation with foot ever-
tural behavior of preterm infants as an imbalance between sion) in the NICU.164,165
low passive muscle tone and active muscle power. She theo- Goals of neonatal positioning procedures include the
rized that because preterm neonates have prolonged periods following:
of immobility (often in the supine position), exaggerated ac- n Optimize alignment toward neutral neck-trunk posi-
tive muscle power may be observed in the extensor muscula- tion, semiflexed, midline extremity posture, and
ture, particularly in the trunk and hips. This imbalance of neutral foot position
extension is viewed as nonoptimal muscle power regulation n Support posture and alignment within “containment
that may negatively influence postural stability, coordinated boundaries” of rolls, swaddling blanket, or other posi-
movement, and later hand and perceptual skills.160 tioning aids; avoid creating a barrier to spontaneous
Some neonates, especially those born at less than movement, and allow space for controlled extremity
30 weeks of gestation, may attempt to posturally stabilize by movement
hyperextending the neck in supine or side-lying positions n Create positions that promote alert states for enhanced
to compensate for maturation-related hypotonia.161 Neck short-duration interaction and sleep states that pro-
hyperextension posturing, without a balance of movement mote comfort and physiological stability
into flexion, may trigger later development of a host of n Offer positions that allow controlled, individualized
related abnormal postural and mobility patterns to compen- exposure to proprioceptive, tactile, visual, or auditory
sate for inadequate proximal stability.160 In some infants, stimuli while monitoring signs of behavioral and
excessive postural stabilizing into neck hyperextension may physiological stress from potential overstimulation
contribute to sequential blocking of mobility in the shoulder, The use of blanket or cloth diaper rolls or customized
pelvis, and hip regions. The potential components of this foam inserts in a neonatal positioning program may modify
high-risk, hypertonic postural profile appear in Box 11-4. increasing imbalance of extension in selected preterm or
chronically ill infants and promote movement and postural
stability from positions of flexion. After the infant is facili-
BOX 11-4n POTENTIAL COMPONENTS tated into a flexed posture in the side-lying position, poste-
OF HYPERTONIC POSTURAL PROFILE rior rolls behind the head, trunk, and thighs provide a
surface against which the infant can posturally stabilize
Hyperextended neck while a flexed midline posture is maintained (Figure 11-10).
Elevated shoulders with adducted scapulae An additional anterior roll between the extremities and the
Decreased midline arm movement (hand to mouth) use of a pacifier may promote further midline stabilization
Excessively extended trunk in flexion (Figure 11-11). Small neonates can be maintained
Immobile pelvis (anterior tilt) in a flexed, symmetrical posture in a circular nest formed
Infrequent antigravity movement of legs from a long blanket roll. Cloth buntings with circumferential
Weight bearing on toes in supported standing body straps and a foot roll (Figure 11-12) provide position-
ing support and containment of extremity movement. Ferrari
TABLE 11-8 n MUSCULOSKELETAL MALALIGNMENT AND FUNCTIONAL LIMITATIONS IN NEONATES
POSITIONAL
DEFORMITY CONSEQUENCES FUNCTIONAL LIMITATIONS
Plagiocephaly Unilateral, flat occipital region; head turn Limited visual orientation from asymmetrical head position;
preference; high risk for torticollis delayed midline head control
Scaphocephaly Bilateral, flat parietal and temporal regions Difficulty developing active midline head control in supine
position from narrowing of occipital region
Hyperextended Shortened neck extensor muscles; overstretched Interferes with head centering and midline arm movement in
neck and retracted neck flexor muscles; excessive cervical lordosis; supine position; interferes with head control in prone and
shoulders shortened scapular adductor muscles sitting positions; limits downward visual gaze
“Frog” legs Shortened hip abductor muscles and iliotibial Interferes with movement transitions into and out of
bands; increased external tibial torsion sitting and prone positions; interferes with hip stability
in four-point crawling; prolonged wide-based gait with
excessive out-toeing
Everted feet Overstretched ankle invertor muscles; altered Pronated foot position on standing; retained, immature
foot alignment from muscle imbalance foot-flat gait with potential delay in development of
heel-to-toe gait pattern from excessive pronation
Adapted from Sweeney JK, Gutierrez T: The dynamic continuum of motor and musculoskeletal development: implications for neonatal care and discharge
teaching. In Kenner C, McGrath JM, editors: Developmental care of newborns and infants, ed 2, Glenview, Ill., 2010, National Association of Neonatal Nurses.
Figure 11-12 n Use of cloth bunting with circumferential straps,

interior foot roll, lateral rolls, and sheepskin to promote body con-
Figure 11-10 n Positioning with diaper rolls to reduce extension tainment in prone flexion.
posturing.
and colleagues166 found increased frequency of extremity

movements across midline and fewer stiff postures when
neonates (25 to 31 weeks of gestation) were positioned
in supine position in a circular nest compared with supine
position without a nest.
Endotracheal tube placement frequently contributes to
the neck hyperextension posture in infants who require
mechanical ventilation (Figure 11-13). This iatrogenic com-
ponent can be avoided by repositioning the ventilator hoses
to allow enough mobility for slightly tucked chin and
partially flexed trunk posture. For neurologically impaired
infants with severe pulmonary disease necessitating pro-
longed ventilatory support, inattention to the alignment of
the neck and shoulders may lead to the development of a
contracture in the neck extensor muscles (Figure 11-14).
During the hospital stay, infants on ventilators (Figure
11-15) are now routinely positioned prone to enhance
Figure 11-11 n Pacifier promotes flexion and long roll allows extremity and trunk flexion in the prone position, improve
anterior and posterior containment of flexed side-lying position. oxygenation, and decrease irritability.167,168 Monterosso and
Figure 11-15 n Infant with endotracheal tube and ventilator

positioned prone in nest with straps. (Reprinted from Hunter JG:
Neonatal intensive care unit. In Case-Smith J, O’Brien JC: Occu-
pational therapy for children, ed 6, St Louis, 2010, Mosby.)
Figure 11-13 n Neck hyperextension posture magnified by the
position of the endotracheal tube.
cotton sheets,170 concern regarding the inhalation of micro-

fibers from sheepskin has limited widespread use unless the
sheepskin is covered by a thin blanket and used only to
midchest level.171
After the infant has been moved from intensive care to
intermediate care, transition to a standard mattress without
positioning aids is recommended to allow time for adapta-
tion to the type of mattress likely to be used at home. Infants
are also transitioned to the supine sleeping position during
the week before hospital discharge to reduce the risk of sud-
den infant death associated with the prone sleeping position
and other factors.171
The neonatal therapist provides consultation on body
alignment of infants in car seats when the LBW infant
has not passed the peripheral oxygen saturation test in
the car seat, which is usually conducted by the neonatal
nurse before discharge. Some infants require the use of
a car bed with body harness when they are unable to toler-
ate the semiupright position of a car seat without oxygen
desaturation.
Preliminary evidence has been reported to support neona-
tal positioning programs emphasizing postures of extremi-
ties in flexion and head in midline.166,172 Clinicians are
referred to the work of Hunter173,174 for detailed positioning
techniques in the NICU. Continued research efforts are
needed to measure effects of positioning and the risk-benefit
Figure 11-14 n Clinical presentation of contracture in neck exten- effects of other neonatal therapy interventions to guide
sor muscles related to hyperextension posture during prolonged future directions of neonatal practice.
mechanical ventilation.
Extremity Taping
The presence of perinatal elasticity encourages early man-
colleagues169 reported that using a small vertical roll along agement of congenital musculoskeletal deformities in the
the torso (sternum to pubis) decreased scapular retraction in neonatal period (birth to 28 days of age). A temporary liga-
the prone position. Although placing infants on a sheepskin mentous laxity is presumed to be present in the neonate
surface (see Figure 11-12) offers increased tactile input and because of transplacental transfer of relaxin and estrogen
has been correlated with increased weight gain in LBW in- from the mother. In addition to the influence of maternal hor-
fants compared with a matched group of infants on standard mones, the rapid growth of the neonate can foster correction
Figure 11-16 n Infant with lumbar meningomyelocele demon-

strating marked varus foot deformities before taping.
of malalignment if the deforming forces are expeditiously

managed. This peak period of hyperelasticity offers pediatric
therapists with advanced orthopedic expertise many opportu-
nities to manage congenital joint deformities.175
Intermittent taping of foot deformities (Figure 11-16) has
been more adaptable to the nursery setting than either casts
or splints and is more effective in achieving mobility than
range-of-motion exercises. Access to the heel for drawing
blood, inspection of skin and determination of vascular sta-
tus, and placement of intravenous lines can be accomplished
with the tape in place or by temporary removal of the tape
as needed. Therapists without a sound knowledge of arthro- B
kinematic principles and techniques should not attempt the
taping procedure, which involves articulation of the joint(s) Figure 11-17 n Significant correction in alignment of varus foot
into a corrected position before taping. Other components of deformities in neonate with a lumbar meningomyelocele. A, Lateral
the taping process include application of an external skin stirrup with open heel taping procedure. B, Moderate correction.
protection solution under the tape, application of an adhe-
sive removal solution when removing the tape, observance
of skin condition and vascular tolerance, development of a
taping schedule beginning with 1 hour and increasing functional position of slight extension. As muscle function
by 1-hour intervals as tolerated, and clinical teaching with returns, the taping is used intermittently to reduce fatigue
selected neonatal nurses for continuation of the taping if and overstretching of the emerging, but still weak, wrist
needed on night shifts and weekends. Infants with congeni- extensor musculature.
tal foot deformities required shorter periods of casting in
the outpatient period after taping of the extremity (Figure Soft Hand-Wrist Splint
11-17) was implemented during the inpatient phase. Taping Instead of using rigid, thermoplastic materials or taping, soft
is not appropriate for medically fragile infants on minimal foam straps with Velcro closures provide an alternative
handling protocols or for infants younger than 30 to method of support for wrist drop or hand-wrist malalign-
32 weeks of gestation because of potential epidermal strip- ment (Figure 11-20). The splinting material is illustrated in
ping from tape removal or vascular compromise from Figure 11-21 with a small notch for the thumb on the longer
inadvertent, excessive compression by either the tape or the strap placed across the palm and the shorter strap for the
underwrap layer. wrist band (longer strap attaches to posterior wrist band by
The availability of thin, self-adherent foam material now Velcro). The wearing time is increased by 1-hour intervals to
allows taping on an underwrap (bandage) layer rather than a 3-hour total wearing time and is often synchronized with
on the infant’s skin (Figure 11-18). Although this method nurse caregiving schedules (approximately 3-hour intervals)
creates a definite advantage in skin protection, it may cover for alternate on-off application. Collaboration with neonatal
the calcaneal region for blood drawing. Compromise in nurses on wearing schedule, skin and vascular tolerance,
alignment may occur if the underwrap layer is applied alignment, and parental teaching is critical for successful
loosely; conversely, restriction in circulation may be integration of soft splints into the care plan.
observed by edema or purple-blue color changes in the toes
if the underwrap is excessively tight around the foot or ankle. Therapeutic Handling
Infants with wrist drop from radial nerve compression Use of tactile, vestibular, proprioceptive, visual, and audi-
related to intravenous line infiltration also benefit from the tory stimuli to facilitate infant development has been
use of taping (Figure 11-19). The wrist is supported in a reported and reviewed by many authors.176-184 Selection and
A B
Figure 11-18 n Taping of varus foot deformity. A, Thin foam layer. B, Silk tape in lateral stirrup over foam layer.
B C
Figure 11-19 n Management of wrist drop in medically fragile neonate. A, Wrist drop before taping. B, Taping procedure. C, One week
after taping.
The typical early movement experiences include hand-to-

mouth movement, scapular abduction and adduction,
anterior and posterior pelvic tilt, free movement of the
extremities against gravity, and momentary holding of the
head in midline.127,185
Behavioral state and some movement abnormalities can
be modified by creative swaddling and gentle weight shifts
and nesting in the caregiver’s lap. Swaddling the infant in a
blanket with flexed, midline extremity position appears
to promote flexor tone, increase hand-to-mouth awareness,
inhibit jittery or disorganized movement, and elicit quiet,
alert behavior. These effects can also be accomplished in
skin-to-skin holding of infants against the parent’s chest, a
procedure now commonly adopted in NICUs in North
America.186,187 Application of neonatal therapy techniques
must be contingent on both the infant’s readiness for interac-
Figure 11-20 n Soft wrist extension splint. tion and the need for a recovery break in interaction because
of sensory overload. Teaching parents and caregivers to read
and respond to the infant’s motor cues for interaction, feed-
ing, change of body position, and rest breaks is a critical
quality-of-life component of the infant’s NICU therapy
program.
A semi-inverted supine flexion position (Figure 11-22) with
preterm neonates should be used with caution. This position
may be used with older infants (6 months old) to facilitate
elongation of neck extensor muscles and decrease the neck
hyperextension posture, but with neonates the position may
compromise breathing from positional compression of the
chest and from potential airway occlusion associated with
maximal flexion of the neck. The use of cardiorespiratory
and oxygen saturation monitors during therapeutic handling
activities is recommended for objective measurement of phys-
iological tolerance. Although the peripheral oxygen saturation
values from monitors may be intermittently unreliable because
of motion artifacts from either the infant’s spontaneous move-
ment or the therapist’s handling of the infant, reliable readings
Figure 11-21 n Soft splint materials. The long strap is used of oxygen saturation may be taken approximately 1 minute
across the palm with the notch for the thumb. The short strap wraps after the infant’s body is not moved.
around the wrist to stabilize the long strap on the dorsum of the Easily overstimulated preterm infants may not tolerate
hand. multimodal sensory stimulation but may instead respond to
a single sensory stimulus.79,181 Implementation of a position-
ing program, oral-motor therapy, and environmental modifi-
application of the sensory or neuromotor treatment options cations and reinforcement of developmental activities with
in neonatal therapy must occur with judicious attention to
the prevention of sensory overload and related physiological
consequences. Decision making on the type, intensity, dura-
tion, frequency, and sequencing of intervention within the
context of infant physiological and behavioral stability can
be learned only in a mentored clinical practicum in the
NICU setting. The current general guidance on intervention
is more observation, less handling, protection from bright
lights and loud conversation, and readiness for handling
determined on the basis of behavioral and physiological
cues of the infant.11,177,183,184
Primary aims of therapeutic handling include assisting
the newborn to achieve maximal interaction with parents
and caregivers and facilitating the experience of postural
and movement patterns appropriate to the infant’s adjusted
gestational age. Helping infants reach and maintain the
quiet, alert behavioral state and age-appropriate postural Figure 11-22 n Potential respiratory compromise to the infant
tone appears to enhance opportunities for visual and audi- from neck extensor muscle elongation in excessively flexed position
tory interaction and for antigravity movement experiences. while supine.
parents can be instituted only in collaboration with the shifts After medical clearance and individualized criteria for
of bedside nurses who are in charge of the infant’s 24-hour the maximum acceptable limits of heart rate, blood pressure,
day in the NICU. Collaboration with nurses is a major com- and color changes during hydrotherapy have been received
ponent of precepted neonatal therapy training and requires from the neonatal staff, the baseline heart rate and blood
integration into and valuing of the unique culture of pressure values are recorded and pretreatment posture and
the NICU.130 Part of NICU culture is the unique ecology of behavioral states are observed. The undressed infant is
environmental light and sound modifications, medical pro- swaddled and moved into a semiflexed, supine position. The
cedures, equipment, and caregiving patterns. Observing blood pressure cuff is placed around the distal tibial region
and analyzing the effects of the environment on an infant’s to continuously measure heart rate and blood pressure at
behavior, physiological stability, postural control, and feed- 2-minute intervals during the 10-minute water immersion
ing function are critical elements to establish a prehandling period. After being lifted into the water, the swaddled infant
baseline status before each neonatal therapy contact.130 is given a short period of quiet holding in the water without
body movement or auditory stimulation to allow behavioral
Neonatal Hydrotherapy adaptation to the fluid environment (Figure 11-24). A sec-
Modified for use in an intensive care nursery setting, the ond caregiver (e.g., nurse or parent) is recruited to stabilize
traditional physical therapy modality of hydrotherapy has the infant’s head and shoulder girdle region while the neo-
been adapted and implemented into neonatal therapy natal therapist provides support at the pelvis (Figure 11-25).
programs in some NICUs. Neonatal hydrotherapy was con- Within the loosened boundaries of the swaddling blanket,
ceptualized in 1980 at Madigan Army Medical Center in the movement techniques involve midline positioning of the
Tacoma, Washington, and results of a pilot study of physi- head and slow, graded movement incorporating slight flex-
ological effects were first reported in 1983.188 ion and rotation of the trunk, followed (if tolerated) by pro-
Indications for referral of medically stable infants to the gression distally to the pelvic girdle region and finally to the
hydrotherapy component of the neonatal therapy program shoulder girdle region. After guided trunk extensor flexion
include (1) muscle tone abnormalities (hypertonus or hypo- with partially dissociated movement at the shoulder or pel-
tonus) affecting the quality and quantity of spontaneous vic girdle, most infants will demonstrate active extremity
movement and contributing to the imbalance of extension movement in the water and the swaddling blanket is
in posture and movement (Figure 11-23); (2) limitation of adjusted (or removed) to allow more movement or more
motion in the extremities related to muscular or connective stability depending on the response of the infant. The
tissue factors; and (3) behavioral state abnormalities of improved range and smoothness of spontaneous extremity
marked irritability during graded neuromotor handling or, movement is facilitated by the buoyancy and surface tension
conversely, excessive drowsiness during handling that limits of the water. Movement experiences in the supine, side-
social interaction with caregivers and lethargy that contrib- lying, and prone positions are offered as tolerated. If the
utes to feeding dysfunction. movement therapy becomes stressful, with agitation or cry-
Infants are considered medically stable for aquatic inter- ing by the infant, body movement is stopped immediately,
vention when ventilatory equipment and intravenous lines are and the infant is either consoled or removed from the water
discontinued and when temperature instability and apnea or and held with warmed towels. Compromise in hemody-
bradycardia are resolved. A standard plastic bassinet serves as namic stability (increased heart rate, increased blood
the hydrotherapy tub, and the water temperature is prepared at
37.8° C to 38.3° C (100° F to 101° F). An overhead radiant
heater is used to decrease temperature loss and enhance ther-
moregulation in the undressed infant. Agitation of the water is
not included in the hydrotherapy protocol in the NICU.
Figure 11-24 n Swaddled infant is supported in neonatal hydro-

therapy tub by neonatal physical therapist and neonatal nurse. The
Figure 11-23 n Adjustment to water immersion before introduc- blanket is gradually loosened to encourage spontaneous, midrange
tion of guided movement during neonatal hydrotherapy. movement of the extremities.
hydrotherapy compared with the rest condition. Mean

daily weight gain after hydrotherapy was significantly
higher (P , .026) than after the rest condition. All infants
consumed 100% of the required feeding volume after the
10-minute hydrotherapy session, indicating that potential
overstimulation or fatigue did not occur. On a short-term
basis, weight gain was enhanced.191
Therapeutic bathing techniques are incorporated into the
parental teaching program to foster early parental participa-
tion in child care and in specific neonatal therapy activities
during the inpatient period to prepare for carryover into the
home environment. This early pleasurable involvement of
parent and child in hydrotherapy and therapeutic bathing
may provide a strong base for future participation in aquat-
ics as a family leisure sports activity and, if needed, as an
adjunct to an outpatient therapy program.
When oriented to treatment goals and trained in specific
hydrotherapy techniques for individual infants, the nursing
Figure 11-25 n Parents being trained in hydrotherapy tech- staff can effectively carry on the hydrotherapy program
niques for later therapeutic bathing at home. established by the neonatal therapist. This release of the
neonatal therapist’s role to nurses allows additional use of
hydrotherapy on evening and night shifts and continued
teaching and supervision of parents during evening and
pressure, decreased respiratory rate) and decrease in arterial weekend visits (see Figure 11-25).
oxygen tension during crying have been well documented in An additional advantage of neonatal hydrotherapy is cost-
neonates.189 Careful monitoring of behavioral tolerance to effectiveness, with the use of equipment readily available
hydrotherapy (with avoidance of crying) is considered criti- in the newborn nursery and the short time (10 minutes)
cal for reducing physiological risk with hydrotherapy. required for therapeutic bathing. Hydrotherapy becomes
Multiple therapeutic benefits have been observed from labor efficient for the neonatal therapist when it is incorpo-
the selective use of 10-minute aquatic intervention sessions. rated into nursing care plans and conducted by nurses and
Improved postural tone with semiflexed posture is obtained parents, with the therapist assuming a supervisory role.
with less time and effort by the therapist and with higher Although many clinical benefits may be obtained by judi-
behavioral tolerance by the infant than when a similar thera- cious use of hydrotherapy in the newborn nursery, pilot
peutic handling approach is used without the medium of study data obtained on physiological changes in high-risk
water. Postural tone changes are frequently maintained for infants during hydrotherapy clearly indicate a physiological
2 to 3 hours when aquatic intervention is followed by flexed, risk.188 This risk (7% increase in blood pressure and heart
midline body positioning in the side-lying or prone position rate in the pilot sample) must be carefully evaluated relative
on a water mattress or supported against rolls. Mild flexion to each infant’s general medical stability and baseline heart
contractures of knees and elbows and dynamic hip adduc- rate and blood pressure status before hydrotherapy can be
tion contractures can be safely and quickly reduced by included safely in a neonatal therapy program. In collabora-
gentle muscle elongation techniques in warm water. tion with the neonatology and nursing staff, the therapist
Enhancement of visual and auditory orientation responses must use established criteria for general medical stability
(e.g., visual fixing and tracking, auditory alerting, and local- and the maximal limits during hydrotherapy for blood pres-
ization to human voices), prolonged high-quality alertness, sure, heart rate, and acceptable color changes; this step is
and longer periods of social interaction with caregivers are essential for risk management. Physiological monitoring of
clinically observed during and after hydrotherapy sessions. mean blood pressure and heart rate with a neonatal vital
Improved sleep quality and behavioral organization were signs monitor during aquatic intervention is recommended.
documented in 12 preterm infants (less than 36 weeks The blood pressure cuff is a pneumatically driven device
of gestation) after a 10-minute hydrotherapy session in a that is not electronically connected to the infant and can be
NICU in Brazil.190 In this study the infants were used safely immersed in water. Because hypothermia is a recog-
as their own controls, and responses were measured by a nized risk with hydrotherapy, body temperature should be
Neonatal Facial Coding System scale and by sleep-wake routinely measured before and after the hydrotherapy ses-
cycles from an adapted NBAS. sion by using a thermometer with a digital display. A risk-
Feeding performance may improve when hydrotherapy benefit analysis of the potential physiological risk to each
is scheduled 30 minutes before feeding to prepare the infant and the expected therapeutic benefits is strongly
infant for arousal to the quiet, alert state and for flexed, advised before hydrotherapy techniques are incorporated
midline postural changes for optimal feeding. A sample of into a neonatal therapy program.
31 preterm infants received both a 10-minute hydrotherapy
session and a 10-minute rest period control condition Oral-Motor Therapy
(crossover design) before bottle feeding by a nurse blinded Feeding difficulties in preterm neonates may be related to
to the order of the treatment phase. Mean duration of neurological immaturity, depressed oral reflexes, prolonged
feeding was significantly decreased (P , .004) after use of an endotracheal tube for mechanical ventilation and
subsequent oral tactile hypersensitivity, or insufficient pos-

tural tone. Because behavioral state affects the quality of
feeding behavior, feeding performance may be significantly
improved by specific arousal or calming procedures before
feeding. Other variables influencing feeding may include
decreased tongue mobility, presence of tongue thrusting,
decreased lip seal on nipple, nasal regurgitation, tactile
hypersensitivity in the mouth, inefficient and uncoordinated
respiratory patterns, insufficient proximal stability from
hypotonic neck and trunk musculature, and hypertonic
posturing of the neck and trunk in extension.192,193
Three instruments for assessing oral-motor and feeding
behaviors in the nursery are the Neonatal Oral-Motor As-
sessment Scale (NOMAS),194 the Nursing Child Assessment
of Feeding Scale (NCAFS),195 and the Early Feeding Skills Figure 11-26 n Swaddled preterm infant fed in semiupright
(EFS) Assessment for preterm infants.196 The NOMAS is position with light support under chin.
used to evaluate the following oral-motor components dur-
ing sucking: rate, rhythmicity, jaw excursion, tongue con-
figuration, and tongue movement (timing, direction, and
range). Tongue and jaw components are analyzed during For some infants, oral intake by bottle may be improved
nutritive and nonnutritive sucking activity. Cutoff scores by individualizing nipple selection according to contour,
were derived from a pilot study with the instrument: a com- length, hole size, texture, and compression resistance. Wolf
bined score of 43 to 47 indicated “some oral-motor disorga- and Glass193,200 advised evaluation of the flow rate of liquid
nization”; a score of 42 or less indicated oral-motor dys- from various types of nipples and analysis of the effects of
function.194 The absence of a category to evaluate breathing nipple size, shape, and consistency on an infant’s sucking
pattern, work of breathing or respiratory exertion, and proficiency. Feeding infants in the side-lying position may
physiological variables during feeding limits the use of this improve tongue position, particularly if marked tongue
instrument to low-risk, healthy neonates. retraction is present (Figure 11-27). The timing of move-
The NCAFS is used to analyze parent-infant interaction ment therapy or neonatal hydrotherapy 30 minutes before
during feeding. It provides a method for evaluating the feeding may improve performance by preparing postural
responsiveness of parents to infant cues, signs of distress, tone, facilitating oral musculature, and enhancing alertness.
and social interaction opportunities during the feeding pro- Infants with orofacial anomalies (e.g., cleft palate, hypo-
cess. In concurrent validity studies, NCAFS scores were plastic mandible) often respond to bottle feeding with a
positively correlated with findings of the Home Observation Haberman feeding system (Medela, McHenry, Illinois),
for Measurement of the Environment inventory at 8 months which allows control of the flow rate through a valve in the
(r 5 0.72) and at 12 months (r 5 0.79).195 bottle and manual compression of the nipple. The Haberman
The EFS Assessment is a 36-item observational mea- feeder is an ideal option for infants with large bilateral cleft
sure of oral feeding readiness, feeding skill, and feeding lip and palate because of the long, flexible nipple, which
recovery. The assessment tool includes examination of allows formula to be released from manual compression of
physiological and behavioral stability, behavioral feeding the nipple by caregivers instead of requiring negative pres-
readiness cues, oral-motor coordination and endurance, sure for suction by the baby (Figure 11-28). Placement of
coordination of breathing and swallowing, and postfeeding the nipple on the middle of the tongue is advised regardless
alertness, energy level, and physiological state. Prelimi-
nary content validity and intrarater and interrater reliability
procedures were described as “stable and acceptable” (cor-
relation data not reported) with predictive, concurrent, and
construct validity testing in process.196 This tool is specifi-
cally designed for feeding examinations in the NICU envi-
ronment and with expanded psychometric testing will be a
relevant instrument in managing neonates with feeding
impairment.
General strategies during feeding may include semi-
flexed, upright positioning with light support under the chin
(Figure 11-26). Techniques such as tactile facilitation of the
facial muscles, use of a pacifier during gavage feedings,
light manual support to the jaw or lip, and thickening of
formula are frequent components of oral-motor therapy pro-
grams.193,197 Scheduling of feeding based on the infant’s
readiness (behavioral cues of hunger and alertness) is
usually implemented by neonatal nurses and shown to be
effective in helping infants advance the frequency of oral Figure 11-27 n Infant fed in side-lying position to improve
(rather than gavage) feedings.198,199 tongue position.
than that reported by parents of infants who are gaining

weight and feeding satisfactorily. Oral-motor dysfunction
has been reported as an early functional deficit in infants at
high risk for later neuromotor sequelae208; early support for
parents coping with a challenging feeding situation builds
competence in caregiving and also commitment to continu-
ity in outpatient developmental monitoring.
Parental Support
Grief Process
Strong, continuous support is essential to help parents
through perhaps the most frightening crisis in their adult
lives—the potential death or disability of their infant.209,210
Although touching and holding infants contribute to infant-
parent attachment,211 parents may initially establish emo-
tional and physical distance from the infant “s” as they cope
Figure 11-28 n Infant with cleft palate fed with specialized with the knowledge that the infant may die. During this time
nipple (Haberman Feeding System). of anticipatory grief, peer group support from other parents
of prematurely born children can be of immeasurable value.
Actively listening to the parents’ feelings and concerns and
providing support without judgment through their episodes
of the location of the palate or lip defect.201 The feeding of detachment and anger are critical. Although long-range
performance of infants with severe cleft palate deformities plans include participation of parents in all aspects of the
may be improved by a dental obturator. This custom- developmental program, the timing and amount of initial
fabricated prosthesis is inserted before feeding to cover the teaching must be individualized to the levels of stress and
defect in the palate. acute grief present.
The increasing popularity of breast feeding has encour- When an infant dies, the neonatal therapist begins the
aged the development of breast-feeding aids (e.g., Lact-Aid, important work of closure. This work includes attending
JJ Avery, Denver, Colorado). These devices allow supple- memorial or funeral services to support the family, writing a
mentation of oral intake during breast feeding through note expressing sympathy to the family, and initiating a
a small tube that goes to the mouth from a sterilized bag personal closure process. Neonatal therapists are advised to
containing infant formula. find a senior nurse mentor to guide them through the closure
Expected outcomes of oral-motor intervention supported process of identifying and dealing with feelings of loss
by clinical research include (1) increased number of nutritive regarding the infant and family. Finding meaning and value
sucks after perioral stimulation,202,203 (2) increased volume of in the process of caregiving rather than solely in functional
fluid ingested during nipple feedings,204 (3) decreased outcomes is an important task in the work of closure and in
number of gavage feedings and earlier bottle feeding,203,205 preventing professional burnout.
(4) accelerated weight gain,205 and (5) earlier hospital dis-
charge.205 Research evidence has emerged on the benefits of Parent/Caregiver Teaching
interventions to assist pacing to allow breathing pauses206 Components of the parental/caregiver teaching process may
and to provide slower flow rate with specialized nipples207 include (1) discussion of the program goals and services in
for preterm infants during the process of learning to the NICU; (2) orientation to the interdisciplinary follow-up
bottle feed. plan after discharge; (3) guidelines for recognizing and
Monitoring the infant’s physiological tolerance, breath- understanding the infant’s temperament, stress, and stability
ing pattern, and work of breathing is critical during oral- cues; (4) methods of creating a calm environment with pro-
motor examination, intervention, and feeding trials.193 Heart tection from aversive light and sound; and (5) specific
rate values may be monitored from either the cardiorespiro- instructions on selected corrected-age–appropriate develop-
graph or with peripheral oxygen saturation from a pulse mental activities or therapeutic handling techniques. When
oximeter. Color changes, diminished tone in facial muscles, used in conjunction with verbal instructions and demonstra-
and behavioral stress cues (e.g., restlessness, trunk arching) tions, a packet of written guidelines and pictures individual-
must be carefully monitored to allow appropriate response ized to the infant’s needs may improve parents’ overall skills
to early signs of fatigue, overexertion, and potential airway and understanding of the program.
difficulty. Regurgitation with aspiration of milk or formula Occasionally, when geographical distance prevents par-
into the lungs may occur during feeding trials, with compli- ticipation by parents in the neonatal therapy program, the
cations of pneumonia, cardiopulmonary arrest, and associ- infant’s individualized developmental plan may be mailed
ated asphyxia. Because of these risks, feeding trials should and later reviewed at discharge or during outpatient follow-
not be attempted by neonatal therapists untrained in manag- up. During times of separation, telephone contact with par-
ing the respiratory and general physiological monitoring ents helps foster attachment to the infant and explain the
components of neonatal feeding.200 purpose and content of the home developmental intervention
Success during feeding activities enhances parent-infant program as well as providing opportunity to discuss the
interaction and perceived competency in parenting. Parents critical need for follow-up. Parents need this ongoing dia-
of infants with feeding dysfunction describe higher stress logue to make the infant seem real to them and to allow
communication of their fears and concerns during the caregivers in care and nurturing of at-risk infants; and
separation. (3) assess the developmental progress of infants to ensure
Teaching strategies are most effective when they are that neuromotor impairments and delays in motor develop-
adapted to the learning style of the parents. This adaptation ment can be identified and intervention initiated as early as
may involve more demonstrations and an increased opportu- possible. Issues of assessment, intervention, and develop-
nity for supervised practice for some parents, particularly mental profiles of the high-risk infant after discharge from
those with reading or language difficulties that limit use of a the NICU are discussed in this section.
written instructional packet. Parents have shown preference
for combined educational methods including demonstration, Medical Management
video, and written materials rather than one single The routine medical care of preterm infants after discharge
method.212,213 Cultural caregiving practices of the family may be provided by a pediatrician, family practitioner, or
may necessitate elimination of common procedures such as health professional. Infants at neurodevelopmental risk are
use of pacifiers for nonnutritive sucking or hand-to-mouth frequently followed by a number of additional professionals,
engagement. including neurologists, ophthalmologists, cardiac or pulmo-
With consultation from and in collaboration with the nary specialists, nutritionists, public health nurses, physical
neonatal therapist, neonatal nurses can incorporate recom- and occupational therapists, and infant educators. Commu-
mendations to support skeletal and motor development into nication among these specialists is often minimal, especially
their routine discharge teaching activities. General consider- when they are located at different facilities, and access to
ations for discharge teaching by nurses may include the providers may be restricted by policies of varied hospital or
following214: managed-care systems. The parent or caregiver is often con-
n Varying the direction of head turn for sleeping in the fronted with conflicting opinions, demands, and expecta-
supine position to prevent plagiocephaly tions of the family and the infant. The follow-up clinic can
n Placing the head in midline with lateral rolls extend- play a valuable role in this situation by providing case
ing along the side of the head and trunk for car seats management to assist caregivers in coordinating necessary
and swings services, verify that all needs of the infant are being met, and
n Limiting the use of infant seats and encouraging the help parents set realistic goals and priorities for themselves
use of prone play on the floor with a roll under the and their child.
arms and upper chest to assist in head lifting and
weight bearing on the arms Family Support
n Highlighting the importance of the prone play position The stress that a vulnerable, premature, or at-risk infant
for strengthening the neck, trunk, and arm muscula- brings to a family is well documented. Grief, anger, and
ture to prepare for sitting and rolling depression are common reactions to the trauma and anxiety
n Reinforcing the value of interdisciplinary follow- of an unanticipated premature birth.209,215-217 The caregivers
up for musculoskeletal and neurodevelopmental of high-risk infants are required to become knowledgeable
monitoring about complex medical terminology and equipment. At dis-
n Recommending expedient follow-up if parents notice charge, they often become responsible for the administration
signs of head flattening, persistent lateral head tilt, of multiple medications of varying dosages, cardiopulmo-
strong asymmetrical head turn preference, or asym- nary resuscitation procedures and equipment, and compli-
metrical arm use cated feeding schedules requiring daily measurement and
In the neonatal period, the quality of infant-parent attach- recording of nutritional intake and output. In addition, fami-
ment and comfort level and proficiency in routine caregiving lies are often faced with an unexpected, large financial
and therapeutic handling set the stage for later parenting obligation to the hospital and the confusion of dealing with
styles. Helping parents find and appreciate a positive aspect different billing agencies and funding sources.
of the neonate’s motor or other developmental behaviors These stresses and demands are even more overwhelm-
gives them a spark of hope from which emotional energy ing for parents who are young, are single, or do not speak
can be generated to help them through the marathon of the English. In contrast to the 1980s, a greater proportion of
NICU experience. Empowering parents early in their parent- at-risk infants now seen in follow-up clinics are living with
ing experience with the infant is crucial. In the life of the caregivers other than their biological mothers. These care-
child, the effects of parent empowerment will last far longer givers may include other relatives, such as single fathers,
than neonatal movement therapy and positioning strategies. grandparents, aunts and uncles, foster care providers, or
preadoptive parents. At the same time the changing demo-
CLINICAL MANAGEMENT: OUTPATIENT graphics of American society are reflected in the increasing
FOLLOW-UP PERIOD ethnic diversity of preterm infants. Whether they are recent
immigrants to the United States, seasonal workers, or resi-
Purpose of Outpatient Follow-up dents of an ethnic neighborhood, parents from minority
for the at-Risk Infant ethnic groups are frequently overwhelmed by the complexi-
Systematic follow-up of the at-risk infant after discharge ties and procedures of a large medical institution. To serve
from the NICU is an essential component of the clinical this population adequately, a follow-up clinic team should
management of high-risk infants. The purpose of this have access to interpreters and include social workers who
follow-up is threefold: (1) monitor and manage ongoing are knowledgeable about community resources outside the
medical issues, such as respiratory problems and feeding predominant culture. Cultural competence, defined as per-
difficulties; (2) provide support and guidance to parents and forming “one’s professional work in a way that is congruent
with the behavior and expectations that members of a dis- follow-up clinic program have been shown to have advanced
tinctive culture recognize as appropriate among themselves,” performance on cognitive measures and to receive more in-
is an essential prerequisite for professionals working in a tervention services compared with unmonitored infants.233,234
high-risk infant follow-up clinic.218 For the physical thera- A confirmed or tentative diagnosis can direct the family
pist conducting an evaluation, cultural competence includes toward intervention services, financial resources, and social
familiarity with differing cultural norms regarding personal supports. Systematic follow-up and recognition of develop-
interaction, child-rearing practices, and family dynamics. mental problems in a high-risk infant play a major role in
Preterm or at-risk infants may be irritable, hypersensitive supporting the relationship between an infant and the care-
to stimulation, less responsive to the affective interactions of giver. The behavioral interaction of an infant with a develop-
adults, and more irregular in sleeping and feeding schedules mental disability is often different from that of a typically
compared with the term infant.219 The demands that such an developing infant, evoking negative maternal responses of
infant place on caregivers can be extremely stressful, espe- anxiety, frustration, or withdrawal.24,235 Diagnosis of a neu-
cially when other siblings in the home, financial concerns, rodevelopmental disability often facilitates dialogue about
and sleep deprivation are present. Although these stresses parental concerns and can assist caregivers in their process
may resolve as the infant’s schedule and temperament of accepting the disability and adjusting their expectations
become more stable, some studies raise concerns about their for the infant.
long-term impact on the parent-infant relationship and the
infant’s social and affective development.220,221 Follow-up Clinic Examination and Evaluation
The pediatric therapist in the follow-up clinic must be Processes
sensitive to these parent or caregiver stresses and concerns. It is widely recognized that preterm infants are at risk for
Because social work and nursing services may not be rou- neurodevelopmental and musculoskeletal impairments that
tinely available, the therapist, within the context of the may lead to functional limitations.6,236,237 Early assessment
examination, needs to be alert to cues in the behavior of the plays an important role for discharge teaching, follow-up
infant or caregiver that may indicate problems in the home. planning, and identifying infants at the highest risk for
Thoughtful questions regarding daily routines, feeding developmental impairments so they can be appropriately
patterns, the sleep schedule of the caregiver as well as the referred for early intervention.
infant, the caregiver’s impression of the infant’s tempera- Although developmental assessment in the neonatal
ment, and the availability of supportive resources can prompt period is useful, it has been shown to have low predictive
a discussion of concerns that may not be readily communi- value for later outcome. The neonatal period and the first 2 to
cated to a pediatrician or other professionals involved in the 3 months of life are characterized by variability in infant
child’s care. behavior and motor skills as well as instability of postural
organization and control.9,10 Longitudinal studies with
Examination of Neurodevelopmental Status sequential examinations indicate that neonatal examinations
Because preterm infants are at increased risk for neurode- are less accurate in long-term prediction of neurodevelop-
velopmental disabilities, close follow-up is necessary mental outcome than examinations administered to older
during the first 6 to 8 years of life. Compared with term infants.3-5 Ongoing repeated assessments to monitor develop-
infants, the incidence of CP is greater in infants born pre- mental outcome are necessary to ensure early identification
term, and the rate of CP increases with decreasing birth of potential functional activity limitations in infants.6,146,238
weight levels.82,222-225 CP is one of several major neuro- Pediatric therapists are in a unique position as consultants to
logical conditions that are sequelae of prematurity; others the multidisciplinary team to become involved in the process
include mental retardation, hydrocephalus, sensorineural of identification of infants at risk, care coordination, and
hearing loss, visual impairment, and seizure disorder. follow-up planning.239 Critical examination periods and signs
When examined as a group, these major disabling condi- of neuromotor or musculoskeletal abnormality indicating a
tions occur more frequently in LBW infants, and the inci- need for comprehensive examination by a pediatric physical
dence increases as the birth weight and gestational age of therapist are described in the next sections.
the infant decrease.222,226-228
Preterm infants are also at increased risk for more Months 2 to 4
subtle neurodevelopmental disabilities, including visual- The American Academy of Pediatrics has established guide-
motor dysfunction, speech and language deficits, reading lines for the follow-up of high-risk infants80 and has defined
and math problems, balance and coordination impairment, indicators for follow-up care.240 No specific timelines or
and behavioral disorders such as attention deficit and recommended schedules for follow-up visits are available,
hyperactivity.83,228-232 Longitudinal studies indicate that by but general agreement exists that the lower the gestational
school age approximately half of all infants with LBW age, the higher the risk for neurodevelopmental impair-
will have educational and learning deficits compared with ments. Although most early neonatal assessments have poor
a reported rate of 24% in the general population.227,228 predictive value, it is recommended that infants at highest
Overall 10% to 30% of preterm infants are estimated to risk be followed closely to ensure early identification and
eventually have “major” disabilities and another 40% to referral.6
have “minor” disabilities.227,228 Important changes in postural control, movement, and
A primary objective of developmental follow-up in behavioral organization occur at 2 to 4 months. Head
at-risk infants is the early identification of neurodevelop- control and balance reactions are emerging, and func-
mental disabilities and the expedient referral to therapeutic tional skills are present with orientation around the mid-
intervention services. Preterm infants who participate in a line.241 This is also the time when the transition from GMs
to goal-directed movement begins to take place (Figure early indicator of atypical development. Learning to transi-
11-29).242 The pediatric therapist can perform an early tion out of supine position and crawling are typically
developmental follow-up evaluation to monitor changes achieved at 10 to 12 months and by 12 to 14 months most
in posture and movement, development of head control, infants have achieved the ability to walk independently.241,242
midline orientation, visual skills and provide parental By 12 months corrected or chronological age, infants dem-
education. A marked increase in the prevalence of posi- onstrate a wide repertoire of behaviors in other domains
tional plagiocephaly (also known in the literature as including cognitive and language intertwined with motor
nonsynostotic plagiocephaly, plagiocephaly without syn- development. Multidisciplinary evaluation at 12 months
ostosis, or flat head syndrome)243,244 has occurred since is recommended for infants at risk, to create a more compre-
1992 when the American Academy of Pediatrics released hensive developmental profile.80
a position paper recommending that all infants be
positioned for sleeping on the back or side to minimize Months 18 to 24
risk of SIDS.245,246 Two to 4 months postterm is a critical With the foundation for gross motor skills well established by
window of time for identifying positional preferences 18 months, identifying deficits in the fine motor, cognitive,
in infants and providing parental education to prevent social adaptive, and language domains that might interfere
plagiocephaly. with school performance becomes the main focus of assess-
ment during this period. High prevalence of positive screen-
Months 6 to 8 ing for autism in infants born preterm has been described in
For most LBW infants, medical concerns have resolved at the recent literature.247 These data suggest that while the focus
this age and caregivers are raising questions about devel- of most follow-up programs for high-risk infants is on motor
opmental expectations. This is a period of great variability and cognitive abilities, evidence now supports the inclusion of
in the development of goal-directed behaviors and attain- screening tools to identify early signs of social and behavioral
ment of motor milestones that are dependent on postural dysfunction. Recent evidence also points to a high prevalence
control. General agreement exists that most typically of cerebellar damage or dysgenesis in preterm infants. Cere-
developing infants achieve independent sitting in this time bellar hemorrhage represents a high risk for cognitive and
frame.241 Definitive predictions on long-term prognosis motor delays in these infants.42,248 D’Amore and colleagues
are difficult for a preterm infant at this age. Tools measur- reported findings that assessment at 2 years of age can
ing specific milestones have low clinical value owing to reliably identify developmental impairments.249
variability in the acquisition of motor skills and postural Comprehensive assessment at 24 months is recom-
control.242 A developmental assessment with emphasis mended, including language, fine motor, adaptive, and cog-
on postural control at 6 to 8 months’ adjusted age (e.g., nitive skills. Most multidisciplinary follow-up programs
Alberta Infant Motor Scale) can document an infant’s stop at this time owing to cost and high rate of attrition.80
current level of performance and provide a baseline for Growing evidence is reported of higher rates of educational
subsequent evaluations. and behavioral challenges becoming apparent at school age
among children with ELBW. Therefore, ongoing follow-up
Months 10 to 14 beyond 24 months is desirable.249
During the first year of life, infants gain increasing levels
of postural control and express neurological integrity and Age Correction
capabilities through movement and exploration. Lack of Premature infants are scheduled for evaluations in the
variability and variety in movement strategies may be an follow-up clinic according to their corrected age (age
adjusted for weeks of prematurity). The issue of whether to
adjust for prematurity when assessing cognitive or motor
development is an ongoing question. Several researchers
have demonstrated that if chronological or unadjusted age is
used for standardized testing, the premature infant who is
developing appropriately will have a low developmental
quotient and test scores indicative of motor delay.250-254 If
age is adjusted for prematurity, the performance of the pre-
mature infants is comparable to that of term infants at 1 year.
Although some investigators caution that adjustment for
prematurity tends to result in overcorrection, particularly for
infants born at less than 33 weeks’ gestation,255 the general
consensus is that infants born prematurely should be evalu-
ated according to their corrected age.255,256
The decision regarding correction for gestational age in a
follow-up clinic should be based on the objectives and test-
ing protocol for that clinic. Consideration should be given to
the following factors: (1) the testing instruments used, with
attention to the competencies evaluated by the tool and
the number of preterm infants in the normative sample, and
Figure 11-29 n Goal-directed reaching and symmetrical lower- (2) the overall purpose of the evaluation and whether the
extremity alignment in 4-month-old born at term gestation. emphasis is on screening or diagnosis
outcome who were correctly identified as “normal” when

Neuromotor Assessment of the at-Risk Infant examined as infants. The children with normal developmental
Neuromotor Assessment Tools: Purpose and outcome who were inaccurately classified as “abnormal” by
Clinical Use the infant test are referred to as false positives; that is, they
Evaluation of the at-risk infant with a quantitative, standard were falsely determined by the test to be positive for the
assessment tool serves two major purposes in a follow-up developmental problem. On the other hand, children who
clinic: were classified as “normal” when tested as infants but subse-
1. Documentation of the infant’s motor status relative to quently are diagnosed with CP are described as false nega-
developmental norms or relative to the infant’s perfor- tives because the test falsely indicated that they were negative
mance on previous examinations. The information is for the developmental problem.
used to determine the child’s developmental progress, The positive predictive value of a test refers to the accu-
rate of change, or extent of motor delay. Achievement racy of the infant test in its classification of infants as
of this objective is determined by the scope and focus “abnormal.” For example, of the infants who were identified
of the assessment tools used in the evaluation. as “abnormal” or “suspect” by the infant test, the proportion
2. Identification of a neuromotor impairment to initiate of infants who are actually diagnosed with CP represents the
appropriate intervention services. Early identification positive predictive value. The negative predictive value
is not simply a task of detecting signs of neuromotor refers to the accuracy of the infant test in its classification of
deviation. The challenge is to identify those infants infants as “normal.” It is calculated as the proportion of
who are most likely to have an abnormal neurodevel- infants categorized as “normal” who actually have normal
opmental outcome. Achievement of this objective is developmental outcome. To the therapist in a follow-up
determined by the predictive validity of the assess- clinic, it may appear that the positive and negative predictive
ment tools that are used. values of a given test would be most useful because
they indicate the probability of a given outcome for an infant
Evaluation of Predictive Validity of Infant being tested. However, predictive value is not a stable mea-
Assessment Tools sure of the predictive validity of a test because the predictive
A primary goal of the neuromotor evaluation is prediction of values vary according to the prevalence of the developmen-
the long-term developmental outcome of the child on the tal problem within a group or population of infants. When a
basis of a clinical examination of the infant. The ability to condition is common, the positive predictive value will be
achieve this goal accurately depends on the clinical experi- relatively high. When the outcome of interest is rare, the
ence and expertise of the therapist as well as the predictive positive predictive value will be relatively low. Further
accuracy or validity of the assessment tool used. The predic- discussion about test evaluation and measurement can be
tive validity of a test is defined by sensitivity and specificity found in a number of excellent resources available to pedi-
and positive and negative predictive values (Table 11-9).257 atric therapists.257,258
The sensitivity of a test evaluates how sensitive the test is
in its ability to identify a defined developmental problem, Examples of Infant Assessment Tools
such as CP. In the testing situation described here, sensitivity In addition to the tools previously described for the assess-
is calculated as the proportion of children with abnormal neu- ment of the neonate, a number of testing instruments have
rodevelopmental outcome who were correctly identified as been designed for evaluation of the infant. The choice of
“abnormal” when examined as infants. Specificity refers to instrument for a particular clinical situation depends on the
how specific the test is in identifying only the defined devel- emphasis and purpose of the clinic as well as on the profes-
opmental problem and not overdiagnosing by identifying sional disciplines that are represented in the follow-up team.
children who do not have the problem. Specificity is calcu- Neurological findings may be the most useful indicator of
lated as the proportion of children with normal developmental impairment in the neonate or young infant because this is a
TABLE 11-9 n PREDICTIVE VALIDITY OF AN INFANT ASSESSMENT TOOL
OUTCOME (AT SPECIFIED AGE IN CHILDHOOD)

INFANT TEST NORMAL ABNORMAL
a b a1b5
No risk Correct nonreferrals Incorrect nonreferrals (false negatives) Total nonreferrals
c d c1d5
Risk Incorrect referrals (false-positives) Correct referrals Total referrals
a 1 c 5 Total normal at outcome.
b 1 d 5 Total abnormal at outcome.
Sensitivity 5 d/b, d 3 100 5 Percentage of abnormal children who were correctly identified as “no-risk” by the infant test.
Specificity 5 a/a, c 3 100 5 Percentage of normal children who were correctly identified as “risk” by the infant test.
Positive predictive value* 5 d/c, d 3 100 5 Percentage of infants identified as “risk” by the infant test who had abnormal outcome.
Negative predictive value* 5 a/a, b 3 100 5 Percentage of infants identified as “no risk” by the infant test who had normal outcome.
*Positive and negative predictive values will vary according to the prevalence of the abnormal outcome within the study population.
time when behavioral responses are influenced by the The cognitive scale of the BSID-III assesses sensorimo-
infant’s affective state and when motor skills are rudimen- tor development, object manipulation and relatedness,
tary. As the infant matures, neuromotor integrity manifests concept formation, memory, and simple problem solving.
with the acquisition of motor skills. For infants older than The expressive and receptive language subscales examine
3 months, longitudinal researchers indicated that observed verbal comprehension, vocabulary, babbling, utterances, and
neuromotor abnormalities were predictive of later CP only gesturing. The fine motor subscale examines grasping,
when accompanied by delay in one or more developmental motor planning, speed, and visual motor activities, and the
milestones.3,259 A brief review of the infant assessment tools gross motor subscale includes sitting, locomotion, standing,
commonly used in follow-up clinics is presented in the and balance. Social-emotional and adaptive behaviors are
following sections. tested using the parental report scales. Administration times
Bayley Scales of Infant Development. The Bayley vary depending on the age of the child but can range from
Scales of Infant Development (BSID) were first published approximately 50 minutes for children aged 12 months or
in 1969 in a format used extensively in clinical and younger to 90 minutes for children aged 13 months and
research settings throughout the United States. The older. The child’s chronological age (adjusted for prematu-
BSID-II,260 a revised version of the BSID, was published rity as needed) gives the examiner a starting point, desig-
in 1993. The goals of the revision process included updat- nated by a letter A through Q. The rules for establishing
ing the normative data, extending the upper age level of the basal and ceiling levels are the same for the cognitive,
test from 30 to 42 months, and adding more relevant test language, and motor scales. The child must pass three
items and materials. The revised test was standardized on consecutive items in order to establish a ceiling, and the test
1700 young children representing a distribution of race, is discontinued once the child fails five consecutive items.
gender, geographical region, and level of parental educa- The BSID-III has expanded basal and ceiling levels with
tion as an indicator of socioeconomic status. In addition, standardized scores ranging from 40 to 160. The mean for
approximately 370 children with various clinical diagno- the standardized composite score for the cognitive, lan-
ses, including autism, Down syndrome, developmental guage, and motor skills is 100 with standard deviation of 15.
delay, preterm birth, and prenatal exposure to drugs, were The language and motor skills also yield scaled scores with
tested with the BSID-II. Test scores from these children a mean of 10 and standard deviation of 3. In addition,
were not included in the normative data and are intended percentile rank, age equivalents, and growth scores can be
to provide a baseline of performance for children with derived.264,265
these diagnostic conditions.260 The BSID-III was standardized on a normative sample of
The expanded age range and updated normative data 1700 children from the ages of 16 days to 43 months 15 days
offered by the BSID-II enhanced its overall use as an assess- living in the United States in 2004. Stratification was based
ment tool. However, several areas of weakness have been on age, gender, parental education level, ethnic background,
identified in using the BSID-II, particularly with preterm and geographical area. Norms for the social-emotional and
infants.261-263 Unlike the protocol of the original test, the adaptive behavior scales were derived from smaller groups
administration of items and the scoring procedures for the (456 and 1350 children, respectively) but the same stratifica-
BSID-II are based on item sets. The appropriate item set for tion pattern was followed.264 There are a total of 91 items
an individual child is usually determined according to the in the new cognitive scale including 72 items from the
child’s chronological age, but the examiner is told to “select BSID-II. Many items from the former cognitive scale were
the item set that you feel is closest to the child’s current level removed, modified, or moved to other subscales such as
of functioning based on other information you might language and fine motor scales. The fine and gross motor
have.”260 The option to begin testing at different item sets, scales contain a total of 66 items including 18 new items.
which can yield different raw scores for the same infant, The parental report scales are a new addition to assess
introduces a level of variability in administration procedures social-emotional and adaptive behaviors.264,266
and test results that is inconsistent with the purpose of a The psychometric attributes of the BSID-III are as strong
standardized test.263 This problem is magnified for preterm as those of the earlier editions and are thoroughly described
infants because it places even greater importance on the in the technical manual.266,267 Reliability coefficients for the
decision of whether to test the infant according to chrono- subscales and composite scores range from 0.86 to 0.93,
logical or corrected age.260 with similar or higher coefficients obtained when the reli-
The third edition of the BSID (BSID-III), published in ability was examined testing special groups. Test-retest reli-
2006, is the most recent update.264 The goals for developing ability was examined in a sample of 197 children tested on
the current edition included updating the normative data, two separate occasions with an average interval of 6 days.
fulfilling the requirements set by the Individuals with Reliability coefficients ranged from .67 to .94, with an aver-
Disabilities Education Improvement Act of 2004, strength- age correlation score of .80. The technical manual describes
ening psychometric measures, updating testing materials, in great detail the convergent and divergent validity, illus-
simplifying test administration, and improving the test’s trating the correlation between the BSID-III and other
clinical utility.238,265 The BSID-III is a comprehensive relevant testing tools.264,266
assessment for children aged 1 to 42 months to be adminis- The BSID-III is still relatively new, and many questions
tered by experienced and trained professionals. This edition remain regarding its potential limitations and utility for
comprises five different subscales—cognitive, expressive, clinical application. Administration of the full test is a
and receptive communication; gross and fine motor develop- lengthy procedure, and the composite scores are reported
ment; and parental report scales to assess social-emotional to be higher than those of the BSID-II.267 Anderson and
development and adaptive behaviors. colleagues268 recently examined the ability of the BSID-III
to detect developmental delay in 2-year-old extremely pre- Four hundred and seven assessments were completed on
term children and those born at term with normal birth typically developing children aged 2 to 24 months in the
weights. The study participants were former preterm greater Denver, Colorado area. The gestational ages of the
infants born at less than 28 weeks or weighting less than children at birth were 37 to 42 weeks, and the majority were
1000 g (n 5 221). Two hundred and twenty healthy Caucasian (77%).
full-term infants with normal birth weight were randomly Construct validity of the MAC was established using
selected as a control group. Developmental assessment Rasch analysis with the 407 assessments. In brief, 34 of the
was conducted at 2 years (age corrected for prematurity) 37 super items fit the model based on fit statistics (infit and
using the cognitive, language, and motor scales of the outfit mean square error values of 0.5 to 1.7). Unidimension-
BSID-III. The social-emotional and adaptive behavior ality of the MAC was also achieved: Rasch principal com-
scales were not used. The authors observed a serious over- ponent analysis showed that the model explained 90.4 % of
estimation in the developmental progress of this sample. the variance. The Rasch analysis also indicated that the
Questions were raised regarding the sensitivity of this test MAC has excellent person and item reliabilities. The person
to detect developmental delay in children. More research is reliability index was .98, indicating that the person ability
needed to examine this test’s sensitivity and the interpreta- ordering would be stable if these children were assessed on
tion of test results, especially for high-risk or premature another evaluation tool with the same construct as “the mo-
infants. Continued use of the BSID-III should be con- tor super items” of the MAC. Cronbach’s alpha was .97,
ducted with caution in the absence of more data to estab- implying that the MAC super items were internally consis-
lish the sensitivity of this tool.266 tent with little redundancy. The item reliability index was
Alberta Infant Motor Scale. The AIMS269 was 1.00, indicating that the difficulty hierarchy of these motor
designed to evaluate gross motor function in infants from super items would be stable if another group of children
birth to independent walking, or birth through 18 months. with the same traits and sample size were tested.272
The stated purposes of the AIMS are (1) to identify infants
who are delayed or deviant in motor development and (2) to High-Risk Clinical Signs
evaluate motor maturation over time. The AIMS is described Longitudinal studies of LBW infants have been used to
as an “observational assessment” that requires minimal identify specific clinical signs or conditions that are most
handling of the infant by the examiner. The test includes predictive of abnormal neurodevelopmental outcome, such
58 items, organized by the infant’s position, designed to as CP. The conclusions among studies are inconsistent
evaluate three aspects of motor performance: weight bear- because of the lack of standard criteria for the risk variables,
ing, posture, and antigravity movements. The normative demographic and clinical variation in the study samples,
sample consisted of 2200 infants born in Alberta, Canada. and use of different outcome measures. Results from these
Raw scores obtained on the AIMS can be converted to studies are summarized in Table 11-10.
percentile ranks for comparison with motor performance of
the normative sample. Test-retest and interrater reliabilities, Neonatal Period
established on normally developing infants, ranged from During the neonatal period through 1 to 2 months after term
0.95 to 0.98 depending on the age of the child. The AIMS (40 weeks of gestation), clinical signs suggestive of neuro-
reportedly had high agreement with the Motor Scale of the motor abnormality include stiff, jerky movements or a pau-
BSID and the Gross Motor Scale of the Peabody Develop- city of movement. Prechtl and colleagues112 developed an
mental Motor Scales (PDGMS) (r 5 0.93 and r 5 0.98, reassessment technique based on the recognition of GMs that
spectively).5 An evaluation of concurrent validity between occur at specific times during maturation. Abnormal GMs
the AIMS and the Movement Assessment of Infants (MAI) are characterized as movements with “reduced complexity
at 4 and 8 months demonstrated acceptable agreement and a reduced variation. They lack fluency and frequently
(r 5 0.70 and r 5 0.84, respectively).270 have an abrupt onset with all parts of the body moving
The Movement Assessment of Children. The Move- synchronously.”114 Persistence of these movements is con-
ment Assessment of Children (MAC) assesses functional sidered to be predictive of CP or cognitive impairment.273
gross motor and fine motor skills of children from age
2 months to 24 months.271 The motor assessment is com- Infancy
posed of three sections including head control, upper At 4 months of age, hypertonicity of the trunk or extremities
extremities and hands, and pelvis and lower extremities. In is recognized as a high-risk clinical sign.89,259,274 Neck
addition, there are four assessment sections (general obser- extensor hypertonicity has been reported to be highly pre-
vations, special senses, primitive reactions, and muscle dictive of CP.3 This finding correlates with neck hyperexten-
tone) that contribute to the interpretation of MAC findings sion and shoulder retraction associated with the tonic laby-
for any one child. These four sections assist therapists in rinthine reflex in the supine position, which has been
making a therapy diagnosis, thus focusing the therapist’s identified in other studies as a high-risk sign.275 Although
selection of treatment modalities. The MAC, on average, neck hypertonicity was the single item most predictive of
has five functional test items per month over 23 months CP in one study, the majority of infants (60%) who exhib-
of development. It is anticipated that this number of ited this clinical sign did not subsequently develop CP.3
items will allow for accuracy in evaluative and discrimina- The predictive value of primitive reflexes has been exten-
tive measures, leading to effective clinical judgments. sively debated. Reflexes and neurological signs, such as the
The MAC can be completed in less than 30 minutes asymmetrical tonic neck reflex (ATNR) and tremulousness,
(20 minutes for some children), and it takes 5 minutes to have been correlated with CP in some studies3,276 but not in
update during reevaluation. others.275 Of the four sections in the MAI, primitive reflexes
TABLE 11-10 n MOTOR IMPAIRMENT “RED FLAGS” DURING NEONATAL INTENSIVE CARE UNIT
FOLLOW-UP
2 months* Persistent asymmetrical head position; risk for plagiocephaly and torticollis
Absent midline orientation even when visual stimulation is present
Jerky or stiff movements of extremities
Excessive neck or trunk hyperextension in supine position
4 months* Poor midline head control in supine position
Difficulty engaging hands at midline and in reaching for dangling toy
Persistent fisting of hands
Difficulty lifting head and supporting weight on arms in prone position
Trunk hypertonicity or hypotonicity
Resistance to passive movement in extremities
Persistent, dominant asymmetrical tonic neck reflex (“fencing” position of arms)
Stiffly extended or “scissored” legs with weight bearing on toes in supported standing
8 months* Inability to sit and roll independently
Inability to transfer objects between hands
Persistent asymmetry of extremities with differences in muscle tone and motor skill
Hypertonicity of trunk or extremities
12 months* Inability to pull to stand, four-point crawl, walk around furniture
Movement between basic positions
Persistent asymmetry of control in extremities
Reprinted with permission from Sweeney JK, Gutierrez T: The dynamic continuum of motor and musculoskeletal development: implications for neonatal care
and discharge teaching. In Kenner C, McGrath JM, editors: Developmental care of newborns and infants, ed 2, Glenview, IL, 2010, National Association of
Neonatal Nurses.
*Ages corrected for prematurity.
were found to be the least predictive of later outcome.259,277 kicking movement compared with typically developing
The positive support reflex, characterized by stiff extension LBW infants.279 Abnormal patterns of kicking, including
of the lower extremities when the infant is held in supported simultaneous flexion and extension of the hips and knees,
standing, is frequently cited as a high-risk sign, but this were associated with subsequent CP.280 Abnormalities of
posture is seen in both term and LBW infants and has not kicking described by Prechtl as “cramped-synchronized,”
been consistently associated with adverse sequelae.254,259,275 that is, limited in variety and characterized by “rigid move-
Persistent primitive reflex activity and asymmetry have been ment with all limbs and the trunk contracting and relaxing
identified as early signs of athetoid CP, more common in almost simultaneously,” were observed in 3-month-old
infants born at term.278 In Figure 11-30 a dominant ATNR infants who were subsequently diagnosed with CP.281
posture is demonstrated by a 4-month-old infant with athe- In addition to qualitative differences in motor function,
toid CP. Immature automatic reactions of balance and equi- delayed acquisition of motor milestones is an important
librium at 4 months, including head righting and the Landau indicator of neuromotor impairment. Several investigations
reaction, have been found to be a significant predictor of of the predictive validity of the MAI found volitional move-
abnormal neurological outcome.275 ment (gross and fine motor skills) to be the most predictive
Comparing an infant’s spontaneous, active movements MAI category at 4 and 8 months.259,277 This finding is sup-
with reflex or passive responses is important in determining ported by other studies in which delayed developmental
risk for neurodevelopmental disability. Systematic observa- milestones were significant predictors of later CP (Figure
tion of kicking activity in LBW infants indicated that infants 11-31).3,282 In particular, delay in achieving upright, gross
with neurological impairment demonstrated less alternate motor milestones, such as sitting without support, creeping
on hands to knees, and pulling to stand, was found to be
useful in identifying infants with neuromotor impairment.282
Challenges to Prediction of
Neurodevelopmental Outcome
Accurate prediction of neurodevelopmental outcome of LBW
infants on the basis of standard neuromotor tests is particularly
challenging because of several complicating factors.
Impact of Medical Status on Test Performance

Infants with LBW often exhibit motor delay or neuromotor
deviations because of their health or medical status, not
because of neurological impairment. Two primary examples
Figure 11-30 n Dominant asymmetrical tonic neck reflex in are residual influences from habitual positioning in the
4-month-old infant with athetoid cerebral palsy. NICU and chronic medical conditions.
Prolonged Motor Delay from Chronic Medical

Conditions. Infants with chronic lung disease typically
exhibit low muscle tone, delayed gross motor function, and
immature balance reactions. Motor skills are often delayed
as long as the infant’s pulmonary capacity is compromised,
but the rate of developmental progress typically accelerates
when the respiratory condition resolves.233,284 Intervention
for infants with persistent respiratory disease includes pro-
viding reassurance and support to the caregivers who are
dealing with the demands and stresses of parenting a medi-
A cally fragile child. Caregivers are advised to avoid aggres-
sive physical activity and excessive sensory stimulation that
could cause fatigue or tax the infant’s limited respiratory
capacity. At the same time, the child should be given oppor-
tunities to develop skills in nonmotor tasks. Adaptive posi-
tioning techniques reduce energy expenditure and fatigue
while enabling the infant to be supported in age-appropriate
postures (e.g., prone or upright sitting) for developing hand
B function, vestibular responses, and social skills.
Figure 11-31 n A, Typically developing term infant at 4 months Transient Dystonia
of age demonstrating ability to bring hands to midline and elevate During the first year of life up to 60% of all LBW infants, as
legs with flexion and abduction of hips and dorsiflexion of ankles. well as a number of term infants, exhibit abnormal neuro-
B, Infant diagnosed with cerebral palsy at 5 months of age; note logical signs that subsequently resolve without evidence of
inability to bring hands to midline because of shoulder retraction, major neurodevelopmental sequelae.285-290 This phenome-
extension and adduction of hips with limited movement into non is referred to as “transient dystonia.” The clinical
flexion, and plantarflexion of ankles. characteristics of transient dystonia described most fre-
quently are summarized in Table 11-11.
The presence of these findings on clinical examination
poses a challenge to the physical therapist because they are
Variations in Posture and Movement Caused by often indistinguishable from clinical signs considered to
Residual Influences from Time in the Neonatal Inten- represent early CP. From longitudinal studies researchers
sive Care Unit. Although current NICU positioning and have suggested that infants with transient neuromotor abnor-
handling procedures are increasingly sensitive to the devel- malities may be at increased risk for long-term neurodevel-
opmental needs of the neonate, the application of life- opmental problems. Infants who demonstrated abnormal
sustaining interventions (e.g., mechanical ventilation) neurological findings during the first 12 months, although
assumes priority in clinical management. On follow-up considered to be developmentally normal at 1 year of age,
evaluation, these infants are typically delayed in reaching reportedly had a higher incidence of mental, motor, and
skills and in achieving antigravity postures. behavioral deficits in preschool and at school age.88,287-289
LBW infants frequently exhibit asymmetry that may be However, a definite relationship between transient abnor-
related to intrauterine position or prolonged positioning in malities in infancy and long-term developmental outcome
the NICU necessitated by surgical or medical intervention. has not been confirmed in other studies.287,290,291
On outpatient follow-up examinations, this asymmetry may
appear as visual orientation to one side of the body, more
mature upper-extremity skill on the same side, and asym- TABLE 11-11 n CLINICAL CHARACTERISTICS
metry of primitive reflex activity. Physical deviations, such OF TRANSIENT DYSTONIA
as tightness of neck musculature on the preferred side and
relative weakness of the opposing muscles, or skull deformi- PERIOD CLINICAL CHARACTERISTICS
ties (plagiocephaly) may also be present.251,283 Asymmetri-
Neonatal period Neck extensor hypertonia
cal motor function resulting from intrauterine or NICU
Hypotonia
positioning can usually be distinguished from early spastic
Irritability; lethargy
hemiplegia or other hemisyndromes by clinical examination
Age 4 months Increased muscle tone in extremities
and by review of the infant’s medical history.251,259 Posi-
Truncal hypotonia
tional asymmetry is generally not associated with differ-
Scapular adduction, shoulder retraction
ences in muscle tone between the two sides of the body or
Persistent reflexes: asymmetrical tonic neck
with neuromotor abnormalities, such as fisting of the hand
reflex; positive support reflex
on the less-active side. Caregivers are advised to promote
Asymmetry
symmetrical posture through their physical handling of the
Age 6-8 months Increased muscle tone in lower extremities
infant, placement of the infant in relation to toys, social
Truncal hypotonia; minimal trunk rotation
activity in the room, and use of cushions or rolls to maintain
Immature postural reactions
midline positioning for the infant’s head and proximal
Immaturity of fine motor skills
musculature.
Abnormal neuromotor signs, even if they appear to be asymmetry is usually not observed in passive tone or reflex
transient, should not be considered as clinically insignifi- activity.251,283 One group of investigators concluded that
cant. These signs may indicate a child who is at risk for “these findings convey an important clinical message: if
subtle neuromotor problems that will not be functionally motor asymmetries are only restricted to the facet of active
evident until school age. Furthermore, neuromotor devia- muscle power, then they are unlikely to be of central origin
tions, although transient, may interfere with the infant’s and as such should not be seen as a sign of neurological
ability to form attachments with caregivers. The infant who impairment. In short, they constitute a typical feature of
arches back into extension instead of cuddling, has poor the post-term development of relatively healthy preterm
head control and difficulty establishing eye contact, or stiff- infants.”251 For most premature infants, these early varia-
ens when held may contribute to feelings of frustration, tions in movement and posture eventually resolve. However,
inadequacy, or resentment in caregivers. Instructing in in the first months of life neuromotor deviations may influ-
handling techniques to minimize these postures, as well as ence the infant’s performance on a standard assessment of
informing caregivers that these behaviors reflect neurologi- motor function or neurological status.
cal instability commonly seen in LBW infants, are often
valuable interventions during this transient period. NEUROMOTOR INTERVENTION
Differences between Preterm and Term Infant Levels of Intervention
Neuromotor Function Therapeutic intervention for the high-risk infant in the
Even when not compromised by chronic illness or neuro- outpatient phase after discharge from the NICU occurs at
logical impairment, the motor development of preterm LBW multiple levels. Type and intensity of intervention depend on
infants differs from that of typical term infants. Compared (1) the needs of the infant and family, (2) the structure and
with term infants, healthy preterm infants demonstrate vari- organization of the follow-up clinic, and (3) the availability
ations in passive and active muscle tone and initially have of resources in a particular clinical and geographical setting.
greater joint mobility, such as increased popliteal angles and
low muscle tone in the trunk.283,292 In the older infant, Assessment as Intervention
increased extremity tone is often present, particularly in the The clinical assessment of an infant is a unique opportunity
hips and ankles.253,283 Comparison studies have frequently for intervention on behalf of the infant and family. For the
noted that preterm infants tend to exhibit more neck hyper- full potential of this interaction to be realized, parents or
extension and scapular adduction and fewer antigravity caregivers must be informed and involved participants in
movements in the supine position (Figure 11-32).* the assessment process, not passive observers. The focus of
Primitive reflexes such as the ATNR, Moro reflex, and intervention in this context is on parent or caregiver support
positive support reflex persist longer in preterm infants, even with two primary components: education and positive
when assessed at corrected age.160,254,294 Gross and fine mo- reinforcement for parenting skills.
tor skills are frequently delayed in preterm infants, espe-
cially activities requiring active flexion, such as (1) bringing Education
hands to midline and feet to hands, (2) trunk stability The educational component of intervention includes
required for head control and upright sitting, and (3) trunk enabling the parents of an at-risk infant to recognize their
rotation for rolling and transitional movements.283,293 child’s unique capabilities and strengths as well as his or her
Preterm infants exhibit more asymmetry in active move- ability to respond to and influence the surrounding environ-
ment compared with infants born at term gestation, but ment. Caregivers learn about their infant’s individual
responses to stimuli—for example, what causes their child
to attend to a stimulus and what elicits stress reactions. Edu-
*References 163, 254, 274, 283, 289, 293. cation of parents includes describing typical characteristics
and common developmental patterns of the LBW or medi-
cally fragile infant that may differ from expectations that are
based on observations or published descriptions of healthy,
full-term infants. Parents of at-risk infants are informed
about the appropriate sequence and pace of development for
their child so they will be realistic in their expectations and
interpretation of the child’s progress. This anticipatory guid-
ance enables parents to prepare for and maximize learning
opportunities.
Reinforcement for Parenting Skills

During the follow-up examination, opportunities to provide
positive reinforcement to caregivers need to be emphasized.
Parents of a high-risk infant who responds inconsistently to
affective cues should particularly be given positive feedback
and affirmation for their investment of emotion and energy.295
Figure 11-32 n Healthy preterm infant at 4 months of corrected They should be reassured that they are providing appropriate
age demonstrating neck hyperextension, scapular adduction and and beneficial parenting and reminded that the infant’s behav-
shoulder retraction, and limited antigravity movement into flexion. ioral responses reflect neurobehavioral immaturity or instability
rather than an unpleasant personality or negative affective

feelings toward the caregiver.
Instruction in Home Management

A critical component of intervention is instruction in spe-
cific activities and handling techniques for home manage-
ment. The recommendations are made on the basis of the
therapist’s knowledge of the infant’s medical and neurologi-
cal history, current health status, and findings from the neu-
rodevelopmental assessment. The overall purpose may be to
maximize a healthy child’s growth potential or to promote
developmental progress in an infant who demonstrates delay
or neuromotor abnormality. In either case, the parent or
caregiver must have a clear understanding of the purpose of
the activity, what motor behavior it is intended to facilitate
or counteract, the underlying neurodevelopmental process
that the activity will support, and the desired response on the
Figure 11-33 n Toys suspended directly in front of infant to
part of the infant. This enables the parent to participate more
encourage symmetrical reaching and midline orientation of head.
creatively in the process of intervention by adapting and
modifying the recommendations according to the infant’s
responses and progress of the infant at home.
Although neuromotor handling recommendations are
specific to the individual child, some intervention activities Prone Positioning
are applicable to many preterm infants. Active play time in the prone position with weight bearing
on the arms is beneficial for the development of neck and
Activities to Counteract Shoulder Retraction trunk postural and shoulder girdle stability. The prone posi-
Up to 50% of LBW infants reportedly demonstrate shoulder tion also counteracts extension posturing tendencies because
retraction.163 This posture may inhibit the infant’s ability to the influence of the tonic labyrinthine reflex in the prone
bring hands to midline and often results in delayed achieve- position contributes to extremity flexion. However, parents
ment of upper-extremity skills and rolling. To overcome of vulnerable premature infants are often hesitant to place
shoulder retraction, play activities and carrying techniques their infants in the prone position for play. Many preterm
that bring shoulders forward and hands to midline are infants demonstrate a low tolerance for prone positioning,
encouraged. particularly if they have relatively large heads and are visu-
ally attentive. Duration of the prone play position can be
Reaching gradually increased; visually stimulating objects, including
Most premature infants are immature in reaching skills, mirrors, musical toys, and the faces of siblings or caregivers,
reducing their ability to interact with the environment. can be placed on the floor in front of the infant to encourage
Activities to counteract shoulder retraction will promote acceptance of the prone position. A roll or wedge positioned
reaching, but the infants should also be provided with other under the infant’s axillae and upper chest will facilitate the
opportunities to practice this skill. Infants who are ready to ability to push up in prone, particularly for the infant with
initiate reaching at 3 to 4 months of age often have only a low muscle tone. An infant who is apprehensive or stressed
visually stimulating mobile suspended beyond their reach when placed on the stomach may tolerate prone lying on
in the crib. Caregivers are advised to hang toys within the the caregiver’s chest, where reassuring eye contact can be
child’s reach in the crib, playpen, infant seat, or other suit- maintained.
able places. Objects that are suspended, rather than handed
to or placed in front of the infant, are preferred to promote Head Balance
the development of directed reach and grasp as well as Balance activities to develop active head control are frequently
shoulder stability (Figure 11-33). Commercially available, recommended for preterm infants. Tilting responses are usu-
relatively inexpensive activity gyms that stand upright on ally achieved most effectively with the infant in the parent’s
the floor are highly recommended for infants at neurode- lap. Instruction to the caregivers often includes demonstration
velopmental risk. and practice using a doll before attempts with the infant.
Emphasis is placed on the importance of (1) adequate trunk
Head Centering and Symmetrical Orientation support; (2) movement through small ranges; (3) slow, graded
Midline positioning of the head with symmetrical align- motion; (4) desired head-righting response; and (5) sensitivity
ment of the trunk and extremities is encouraged to coun- to indications of stress or fatigue.
teract the residual effects of asymmetrical positioning in
utero or during hospitalization. Midline orientation will Limited Use of Infant Jumper or Baby Walker
reduce the influence of the ATNR and promote symmetri- For infants with increased lower-extremity tone or a ten-
cal function of the right and left sides of the body. Asym- dency for toe-standing, the use of baby walkers and jumpers
metry that is not caused by neurological dysfunction tends is discouraged because of potential increased stiffness and
to resolve when positioning and environmental influences extension posturing of the legs.296 Mobile baby walkers are
are modified. associated with a high risk of injury, including serious
trauma such as burns, drowning, and severe head injuries clinical paths that include standardized examination instru-
resulting from falls down stairs.297-300 However, baby walk- ments, comprehensive risk-management plans, long-term
ers are usually enjoyable for infants and may provide care- follow-up strategies, and systematic documentation of
givers with some needed moments of respite in stressed outcome. Ongoing analyses of the physiological risk–
households. When recommending that time in a baby walker therapeutic benefit relationship of neuromotor and neurobe-
or jumper be restricted, the therapist should help the caregiv- havioral treatment for chronically ill and preterm infants
ers find alternative methods of positioning and amusement must guide the NICU intervention process. The quality of
for the infant. Parents are often reluctant to discard a baby collaboration between therapists and neonatal nurses largely
walker, believing that it promotes early ambulation and is determines the success of neonatal therapy implementation
beneficial for infants. Informing caregivers of the hazards of in the 24-hour care environment of the nursery.
infant walkers and research findings indicating walker use Pediatric therapists working in neonatal units are encour-
may delay the acquisition of gross and fine motor skills aged to participate in follow-up clinics for NICU graduates
enhances the likelihood of their cooperation to eliminate to identify and analyze the development of movement dys-
walkers and jumpers.297,298,301,302 The lower-extremity tone function and behavioral sequelae that may, in the future,
and movement effects of semisitting activity centers that be minimized or prevented with creative neonatal treatment
allow supported standing and some lateral steps have not approaches. The important preventive aspect of neonatal
been documented. treatment must be guided by careful analyses of neurodevel-
opmental and functional outcomes in the first year of life.
SUMMARY The preterm or medically fragile infant is at increased risk
This chapter on the NICU management and follow-up of for major and minor neurodevelopmental problems that may
at-risk neonates and infants has presented three theoretical manifest in infancy or not became evident until childhood.
models for NICU practice, reviewed neonatal neuropatho- Prenatal and perinatal risk factors may identify infants who
logical conditions related to movement disorders, and have a greater likelihood of neurological complications, but
described expanded professional services for at-risk neo- the relation between single factors and outcome is neither
nates and infants in a relatively new subspecialty within direct nor consistent. Abnormal neurological signs in the first
pediatric practice. Pediatric therapists participating in inten- year are also not reliably predictive of abnormal outcome.
sive care nursery and follow-up teams in the care of Attempts to identify factors that definitively indicate signifi-
high-risk neonates and their parents are involved in an cant brain injury are complicated by changing NICU tech-
advanced-level practice area that requires heightened re- nology, management procedures, environmental variables,
sponsibility for accountability and for precepted clinical and variability among and within individual infants.
training (beyond general pediatric specialization) in neona- In deciding whether and when an infant requires regular
tology and infant therapy techniques. Practice guidelines intervention, consideration must be given both to the poten-
for the NICU from national task forces representing the tial for abnormalities to resolve during the first year and to
American Physical Therapy Association and American the time span that may elapse before definitive evidence of
Occupational Therapy Association indicate roles, proficien- CP emerges. The pediatric therapist’s long-term clinical
cies, and knowledge for neonatal therapy and designate the management of the at-risk infant is guided by the develop-
NICU as a restricted area of practice to therapy assistants, mental course of the individual infant over time, including
aides, and entry-level students on affiliation. behavioral and cognitive growth as well as neuromotor
Inherent to this subspecialty practice is the challenge progress, considered within the context of the priorities and
to design comprehensive neonatal therapy protocols and values of the family.
CASE STUDY 11-1 n HIGH-RISK INFANT A

Infant A was born prematurely at 29 weeks of gestation with a including the ATNR, Moro reflex, and tonic labyrinthine reflex
birth weight of 940 g. Her neonatal course was complicated by influence in the supine position. Head balance was immature,
idiopathic respiratory distress syndrome, which was treated but emerging righting reactions were noted. When the infant
with surfactant. was observed in the supine position her posture was extended,
She was first evaluated in the high-risk infant follow-up but she was beginning to bring her hands to midline. In prone,
clinic at 4 months’ corrected age (6 months’ chronological she had started to push up on elbows but posture was immature
age). Her mother stated that the infant had several respiratory and unstable. Her parents were given recommendations for
illnesses after discharge from the NICU. She reported that her handling to include holding and carrying positions with shoul-
infant felt “tense” compared with her older child born at term ders forward to inhibit retraction, frequent play in the prone
and seemed to be “a little behind” in overall development. position, and increased opportunities for reaching in supine.
Performance on the BSID-II generated a Mental Development When the infant returned at 8 months’ corrected age, her
Index (MDI) of 94 and a Psychomotor Development Index mother reported that progress had been made in the areas of
(PDI) of 82. On the MAI this infant had a total risk score of 7. rolling, talking, and sitting. She indicated that the body
She had mildly increased lower-extremity muscle tone evident “stiffness” was less evident, but the infant still did not like to
in mild resistance to passive range of motion of her hips play on her “tummy” and instead preferred to use the baby
and ankles. She demonstrated persistent primitive reflexes, walker. BSID-II scores at this time were MDI of 101 and PDI
CASE STUDY 11-1 n HIGH-RISK INFANT A—cont’d

of 81. The MAI total risk score was 9. Increased muscle tone When seen at 12 months’ corrected age, the infant had
observed previously was less evident and she had full hip an MDI of 108 and a PDI of 88. Although not yet walking
mobility but some resistance to passive ankle movement. Mus- independently, she was cruising with good weight shift and
cle tone of the trunk was mildly hypotonic, but age-appropriate balance. She was able to creep reciprocally on hands and knees
antigravity movements were demonstrated in all positions. and pulled to stand. She picked up a pellet with an inferior pin-
Primitive reflexes were no longer evident except for the posi- cer grasp. No deviations of muscle tone or reflex development
tive support reflex, characterized by toe-standing tendency were observed during examination by the developmental pedia-
during weight bearing. Balance reactions were present but trician. The infant was developing normally and will return for
immature in some areas, including head righting into flexion follow-up at 2 years of age. Her parents were advised to call
and protective extension reactions. In volitional skills, the if she was not walking within 2 months or if they had any
infant was now sitting independently for up to 30 seconds, concerns regarding her pattern of independent walking.
could roll from supine to prone, and could pivot sideways in In the management of this child who demonstrated abnor-
the prone position. She could pick up a block with either hand mal signs, the primary responsibilities of the pediatric therapist
and transfer objects. Immaturity was observed in sitting bal- were ongoing assessment and parental guidance and teaching.
ance, inability to move out of sitting, and failure to move for- Although initial concerns about this infant’s muscle tone and
ward on the floor (i.e., low two-point crawl). She attempted to reflex deviations were present, diagnosing her with a particular
pick up a pellet but was unable to do so. Her parents were condition would have been inappropriate. When followed up
advised to discontinue using the baby walker and to maximize over time, the abnormalities resolved and proved to be tran-
play time on the floor. Because the infant reportedly enjoyed sient. This child should continue to be followed up in the
watching her 4-year-old sibling, the therapist recommended high-risk infant clinic because she remains at risk for other
that he play on the floor beside her. Her mother was also neurodevelopmental problems that may not become evident
advised to provide the infant with tiny bits of food (e.g., until school age.
Cheerios) to practice fine motor dexterity.
CASE STUDY 11-2 n HIGH-RISK INFANT B

Infant B was born prematurely at 29 weeks of gestation with a hips. On the right side, hip extension was accompanied by knee
birth weight of 1200 g. The neonatal course was complicated extension and plantarflexion of the ankle. She was not yet
by idiopathic respiratory distress syndrome and persistent reaching for objects, and her hands were frequently fisted, par-
apnea and bradycardia. Cranial ultrasonography revealed a ticularly on the right. Her parents were assisted with handling
left subependymal hemorrhage with ventriculomegaly and skills to reduce shoulder retraction and extension posturing and
left-sided PVL. to facilitate symmetry in movements and posture.
She was first seen in the high-risk infant follow-up clinic at When the family returned for a follow-up visit at 6 months
4 months’ corrected age (6 months and 17 days’ chronological they reported that their daughter was making good progress,
age). The parents stated that they had no specific concerns but she continued to prefer use of her left hand in spite of their
regarding their daughter’s development. On the BSID-II the efforts to encourage use of the right hand. At this evaluation,
infant received an MDI of 96 and a PDI of 87. On the MAI she Bayley Scale scores were an MDI of 94 and a PDI of 83; the
received a total risk score of 14. Muscle tone was normal at rest MAI total risk score was 13. The infant had made the following
but increased when she was active or agitated. Tone in the lower developmental progress: (1) rolling from supine to prone (over
extremities was mildly increased with restricted passive move- the right side only), (2) beginning sitting balance, and (3) reach-
ment in the hip adductor and gastrosoleus muscles bilaterally. ing out and grasping objects. She showed a preference for and
In the supine position she was frequently in an extended posture greater skill and dexterity with her left hand. Occasional fisting
and brought her hands to midline only once during the examina- was still observed on the right hand. She transferred objects
tion. In prone she was able to push up and elevate her head only from right to left. Muscle tone continued to be increased in
while kicking actively. In the prone suspended position, she the lower extremities, with restricted passive mobility of the
showed good postural elevation but movements were stiff. gastrosoleus muscles bilaterally. Toe clawing was observed on
Persistent primitive reflexes included the tonic labyrinthine the right with minimal spontaneous dorsiflexion observed on
reflex in supine, ATNR, neonatal positive support reflex, and bi- this side. Primitive reflexes were integrated except for persistent
lateral ankle clonus. Plantar grasp with toe curling was observed neonatal positive support and ATNR to the right. Automatic
on the right. Righting and equilibrium reactions were emerging. reactions were improved, but balance responses were asymmet-
She showed a mature Landau reflex with full extension in prone rical, with equilibrium reactions and protective extension reac-
suspension, which is atypical for her age. In volitional move- tions delayed on the right. Although the developmental progress
ment, mild asymmetry was evident because she had difficulty was encouraging, the persistent asymmetry remained a major
bringing her right arm forward when prone and brought her left concern. The infant was referred to a developmental interven-
arm to midline more frequently. Her kicking pattern when su- tion program with the recommendation that she receive
pine was low (close to the surface), and she did not elevate her consistent pediatric therapy in her home at least once a week.
Continued
CASE STUDY 11-2 n HIGH-RISK INFANT B—cont’d

The infant was seen in the follow-up clinic at 12 months’ with mild right hemiplegic CP. It was recommended that she
corrected age (14 months’ chronological age). On the BSID continue in the intervention therapy program and return for
the MDI was 95 and the PDI was 82. She now was creeping reevaluation at 2 years of age.
reciprocally on hands and knees. When she pulled to stand In the management of this child, the role of the pediatric
she consistently brought the left foot up first. She cruised therapist was assessment of neurodevelopmental status and
holding onto furniture with a tendency to stand on her toes referral to therapy when it became evident that the abnormali-
on the right. She picked up cubes with either hand but ties of muscle tone were persisting and interfering with devel-
showed partial palmar grasp on the right. She picked up a opmental progress. Of note, this child’s MDI scores were in
pellet with an inferior pincer grasp on the left but scooped it the normal range at both the 4- and 8-month examinations.
into the palm of the right hand. Muscle tone continued to be Because the BSID-II does not require infants to perform tasks
mildly increased in the lower extremities with Achilles ten- with both hands, a normal score can be obtained by using just
don tightness, particularly on the right. She sat independently one side of the body. This child should continue to be followed
with a mildly flexed thoracic spine. When moving into and up in the high-risk infant clinic after 2 years of age to provide
out of sitting, she lacked full trunk rotation, and weight was periodic reassessment and guidance to the family as they con-
predominately over the left hip. Language development was front questions of school placement and program planning for
considered appropriate for her age. The infant was diagnosed their child.
Acknowledgement accompanies this textbook. This online service will,

We appreciate significant contributions to the case studies when available, provide a link for the reader to a Medline
by Marcia Williams, PT, MPH, PhD. abstract for the article cited. There are 311 cited refer-
ences and other general references for this chapter, with
References the majority of those articles being evidence-based
To enhance this text and add value for the reader, all ref- citations.
erences are included on the companion Evolve site that
CHAPTER 12 Management of Clinical Problems of Children
with Cerebral Palsy*
CLAUDIA R. SENESAC, PT, PhD, PCS

cerebral palsy After reading this chapter the student or therapist will be able to:
direct intervention 1. Identify the parameters of the diagnosis of cerebral palsy including motor, family, and
family psychosocial components.
indirect intervention 2. Analyze the multifaceted aspects of the clinical problem and appreciate a multifaceted
postural and movement compensation approach to evaluation and treatment.
research 3. Analyze treatment strategies and their application to clinical problems.
spasticity 4. Identify and critique current research for the pediatric client with cerebral palsy.
treatment strategies 5. Identify the therapist’s role in the treatment of the child with cerebral palsy, with family
involvement, in different settings, and with other health professionals.
OVERVIEW British physician Little identified the condition on the basis

of observable characteristics of movement and posture, or—
Historical Perspective in other words—the external features of the condition, so the
Cerebral palsy is a misnomer at best. Little1 suggested the initial efforts at remediation fell to orthopedists such as
name in the mid-1800s, but there is still no established direct Deaver and Phelps.3,5,6 Deaver placed importance on exter-
relationship between the identifiable state of the brain and the nal bracing that was periodically reduced in the hope that the
distortions in posture and movement control that we are able child would take over control of increasing parts of his or
to observe in the individual.2,3 The condition is not always her own body.5 Phelps used bracing and surgery and was a
evident at birth, although the work of Prechtl4 statistically significant force in obtaining schooling for these children in
supports the possibility of a link between the quality of spon- the United States.6 He pointed out that they did not belong
taneous movements in the first months of life and later diffi- in academic classes with children diagnosed as retarded or
culties in coordinated movement expression. In only a small mentally handicapped and that children with cerebral palsy
number of children has a specific lesion been identified that should be exposed to a traditional academic curriculum.
corresponds to the observed motor responses of the child, In his Children’s Rehabilitation Institute in Reisterstown,
and this elite group includes children with porencephaly and Maryland, he also advocated restriction of a more functional
other early developmental malformations of the brain. limb to encourage use of the one less used, particularly in
Whether there is a biochemical element in the brain of a child work with the upper extremities.
that distorts the actual motor learning process has not been In the 1950s and 1960s there simultaneously emerged
established. There is a shocking variability in the age at new theories of neuromotor behavior that redefined the
which intervention is initiated for individual children and a clinical characteristics of cerebral palsy and permitted cli-
wide variety of programs that do not necessarily take into nicians to orient their intervention strategies to the princi-
account the current information available from clinical stud- ples of motor development and motor learning. Kabat in
ies on efficient motor development and brain function. This conjunction with Knott introduced proprioceptive neuro-
confusion has led us astray in understanding the process of muscular facilitation (PNF), which was applied to children
movement and postural distortion that characterizes children with movement disorders and to adults with a history of
who carry the label of “cerebral palsy.” trauma.7 The use of diagonal patterns of movement in this
Historically, the evolution of diagnosis and treatment approach changed the customary postures of the child and
intervention or management is clear and relates to the recog- introduced more functional movement patterns in logical
nition of the special needs of this minority of society. The learning sequences. Physical and occupational therapist
Margaret Rood added the more specific sensory compo-
nents of ice and light quick brushing of the skin surface to
*
This chapter is dedicated to Christine Nelson—master clinician, true guide the desired motor response.8 She spoke of the need to
friend, and mentor. Her gifts as a clinician and artistic eclectic approach to focus attention on both “heavy work” and “light work” dur-
the treatment of children were unmatched and often seemed beyond what
one could comprehend. She ever changed those she touched and enlight-
ing the early development of movement skills. These terms
ened all those she taught. Christine will be forever missed and yet her gift referred to the central body moving over limb support and
of touch will be everlasting and live on in her patients, students, and friends. limb movement with central stability. Bobath was working
317
in London at this same time and observed the need to have palsy is a heterogeneous collection of clinical syndromes,
a dynamic interaction between stability and mobility, after not a disease or pathological or etiological entity.10 Little
finding that inhibition of the reflexive movements was not described cerebral palsy as “a persistent disorder of move-
sufficient to change the functional outcome of the child ment and posture appearing early in life and due to a devel-
with cerebral palsy.9 They pointed out that the areas of the opmental nonprogressive disorder of the brain.”3 Current
child’s body that appeared to be spastic changed when the definitions have reiterated that atypical execution of move-
body was placed in a different relationship to gravity. This ment and interference with postural mechanisms are the key
observation held up for reexamination the prevailing view characteristics of this nonprogressive disorder affecting the
of the time, namely, that spasticity existed in a tendon or developing brain.10,11
muscle, a specific structure. Cerebral palsy affects the total development of the child.
Cerebral palsy was identified in the mid-1900s as an The primary disorder is of motor execution, but common
incident that occurred shortly before, during, or shortly after associated dysfunctions include sensory deficits (hearing or
the birth of the infant. Early intervention was recommended. vision); epilepsy; learning disabilities; cognitive deficits;
This time line was extended to cover the first 2 years of life, emotional, social, and behavioral problems; and speech and
which included early cases of meningitis, encephalitis, near- language disorders. The degree of severity varies greatly
drowning accidents, and so forth. Although the clinicians from mild to moderate to severe.10-12
mentioned tried to define cerebral palsy as a “disorder of
posture and movement control,” many of the children also Diagnostic Categorization of the
had learning problems and inadequate general brain devel- Characteristics of Cerebral Palsy
opment. There was a general agreement on categories In general, a diagnosis of cerebral palsy suggests that the
according to movement characteristics that included spastic- individual has a lesion within the motor control system with
ity, athetosis, flaccidity, ataxia, and rigidity. Categorization a residual disorder of posture and movement control. Varying
according to the part of the body affected was added to iden- degrees of associated components are seen with this disorder
tify hemiplegia, quadriplegia, diplegia, and even monople- that further define the category that a child may fall into:
gia, affecting one limb, and triplegia, affecting three limbs. severity of motor abnormalities, anatomical and magnetic
It was noted that some children moved from one category to resonance imaging findings, extent of associated impair-
another as they matured, and therapists began to be aware ments, and the timing of the neurological injury. In addition,
that a child with high tone could have some low tone under- the labeling process often identifies the parts of the body that
neath when spasticity was inhibited. Fluctuating tone could are primarily involved. Diplegia, hemiplegia, and quadriple-
be confused with ataxia, and the precise intervention strat- gia, respectively, indicate that the lower extremities, one side
egy might be elusive. of the body, or all four extremities are affected. This can
The birth process is complex at many different levels. be misleading to the therapist who is working with infants
Sequential hormonal changes alert both the fetus and the because these children often change their clinical signs and
mother that it is time for a separation. The infant moves into symptoms and their respective disabilities. The disorder is
position for exiting the uterus through the birth canal while not progressive, but the presentation of involvement of body
the mother’s body prepares to participate in the work (labor) segments may manifest itself differently as the child grows
of the expulsion. When all goes smoothly, the head of the and his or her structure and tonal distribution changes against
infant is molded by the passage through the birth canal, and gravity.
the membranous-like cranial plates return to their balanced The clinician must be aware that the categorization
alignment and functional motion. of cerebral palsy is based on descriptions of observable
When the birth process is prolonged for any of many rea- characteristics; thus, it is a symptomatic description. The
sons, the physiological timing of these changes is interrupted. hypertonus of spasticity prevents a smooth exchange between
Unique combinations of pressure may make it difficult for mobility and stability of the body. Constriction of respiratory
the membranous structures to maintain their structural align- adaptability occurs with poor trunk control. Incrementation
ment. That lack of structural alignment may persist long after of postural tone occurs with an increase in the speed of even
birth and affect future movement and development. Rapid passive movement, and clonus may occur in response
changes of pressure, with minor misalignments of the head to sudden passive movement. Although diagnostic terms
and body during the birth process, result in sufficient trauma reflect the distribution of excessive postural tone, the entire
to affect the nervous system and the delicate fascia and in a body must be considered to be involved. Spasticity, by nature,
small percentage of infants to affect the expression of spon- involves reduced quantity of movement, which makes its
taneous movements. In the majority of healthy infants born distribution easier to identify. Recruitment of the corticomo-
at term the spontaneous movements seem to assist in the tor neuron pool is affected in the presence of spasticity, and
activation of the central body and the limbs so that physio- therefore timing issues result in the poor grading of agonists
logical changes in the fascia are sufficient to permit a typical and antagonists.13,14 There is also a risk of reduction in the
expression of developmental movement responses after birth. range of limb movements over time when therapy does not
Body movement and respiration are coordinated with the include active adaptation in end ranges and organization
infant’s physiological rhythms in this initial adaptation to of postural transitions.15 This category (spasticity) has the
the world of gravity. With complications of the pregnancy or highest occurrence of cases of cerebral palsy.16 There are
the birth process, these spontaneous movements that are so several spastic types of cerebral palsy that require clarification.
easily made by the healthy infant become laborious and Spastic diplegia implies that the lower extremities are more
sometimes impossible, affecting motor actions, postural involved than the upper extremities but could manifest with
mechanisms, and the basic physiological rhythms. Cerebral varying degrees of hand function, and often the involvement
CHAPTER 12 n Management of Clinical Problems of Children with Cerebral Palsy 319
is asymmetrical.14 Hemiplegia displays involvement of follow may also be applied in such cases. As with cerebral
one side of the body and can manifest itself with the arm palsy, early positioning and handling after trauma may deter
involved more than the leg or the leg involved as much as later problems.
or more than the arm.10 Quadriplegia, as the term implies,
involves the entire body.10 EVALUATIVE ANALYSIS OF THE INDIVIDUAL
Dyskinetic syndromes, which include athetosis and dys- CHILD
tonic types of cerebral palsy, are characterized by involun-
tary movements. The term dyskinetic is commonly used Initial Observations and Assessment
with children who lack posture and axial and trunk coacti- Examination of the individual child begins with careful
vation. The excessive peripheral movement of the limbs observation of the interaction between parents and the
occurs without central coactivation. Dystonic types of cere- child, including parental handling of the child that occurs
bral palsy are dominated by tension, and athetosis usually spontaneously. Some additional insight can be gained
has a hypotonic base or underlying tone. Dyskinetic syn- about the relationship between parent and child by observ-
dromes may occur with greater involvement in particular ing how the child is handled both physically and emotion-
extremities, although the condition most often interferes ally. Does the child receive and respond to verbal reassur-
with postural stability as a whole. When pathological or ance from the parent in the therapy situation? Are
primitive reflexes are used to accomplish movement, there immediate bribes offered to the child? Does parent eye
is a difficulty with midline orientation. Dyskinetic distribu- contact increase the child’s confidence in responding?
tion of postural tone is changeable in force and velocity, Does family communication convey the idea of negativity
particularly during attempted movement by the individual. in the therapy situation or a difficult experience that will
Midrange control is limited if present at all, and frequently soon come to an end? The family orientation will affect the
end ranges of motion are used to accomplish a motor task.10 response of the child while working with the therapist.
For these reasons, these children have a reduced risk for Making connections with the child and family is a critical
contractures over time. component to a successful relationship that forms with
Hypotonicity is another category of cerebral palsy, but it ongoing treatment.
may also mask undiagnosed degenerative conditions (see The therapist working as part of a team may have the
Chapter 13). Recent reports suggest that “pure hypotonia” is advantage of a social worker or psychologist who will relate
not an attribute of cerebral palsy, and further testing to rule to the problems and motivations of the parents. Parental
out other causation may be indicated.16 responses toward the disabled child arise from the parents’
Hypotonia in a young infant may also be a precursor of uncertainty, fear, concern for the future, disappointment,
a dyskinetic syndrome. Often, athetoid movements or spas- distress, and other typical reactions to this unforeseeable
ticity are not noticed until the infant is attempting antigrav- life experience. The therapist will observe positive changes
ity postures, although there may be some disorganization in parental orientation to the child as the parents are edu-
apparent to the careful observer. Generalized hypotonia cated as to what can be done to help the child move for-
often masks some specific areas of deep muscle tension ward. They may be further assisted by opportunities to
with accompanying local immobility. interact with well-adjusted parents of older children with a
True ataxia is a cerebellar disorder that is seen more diagnosis of cerebral palsy. Assisting families to make con-
frequently as a sequela of tumor removal (see Chapters 21 nections with other families and children in the community
and 25) than as a problem occurring from birth. Ataxic provides them a supportive network of people who share
syndromes are more commonly found in term infants. This similar experiences.
type of cerebral palsy is a diagnosis of exclusion. In a small A problem-based approach to the assessment and man-
number of patients there is congenital hypoplasia of the agement of the child with cerebral palsy includes the family
cerebellum. Most of these children are hypotonic at birth as key members of the team.17 While observing the child, the
and display delays in motor acquisition and language experienced therapist will want to periodically elicit from
skills.10 Recruitment and timing issues remain problems in the parents their view of the problem. By listening carefully,
this population. Trajectory of the limbs, speed, distance, the therapist will also be able to discern the emotional
power, and precision are frequently documented as prob- impressions that have surrounded previous experiences with
lems in this category. Midline is often achieved, but control professionals. Sometimes what is not said is more important
of midrange movements of the extremities and control of than what is verbally offered immediately. Listening carefully
trunk postural reactions are affected. and clarifying facts are more important than overwhelming
These classifications, even when accurately applied, give the parents with excessive information and suppositions
the therapist only a general idea of the treatment problem during early contacts. Observation of the family response to
and must be supplemented by a specific analysis of posture information will keep the therapist on track in developing
and movement control during task performance, an inter- a positive relationship with parents that deepens over time.
view for home care information, and assessment of treat- The therapist’s role is often as interpreter of medical infor-
ment responses (see Chapters 7 and 8). The therapist is then mation as parents attempt to make some sense of their child’s
ready to establish treatment priorities for the individual diagnosis.
child. The next general step is to observe, in as much detail
Many of the characteristics described in the preceding para- as possible, the spontaneous movement of the child when
graphs also apply to children who have had closed head trau- separated from the parent (Figure 12-1). Is the child very
mas or brain infections. Further information can be obtained in passive? Does he or she react to the supporting surface
Chapters 24 and 26. Some of the treatment suggestions that (Figure 12-2)? Are there atypical patterns of movement to
Figure 12-3 n Emotional reactions are also translated into stronger

spastic reactions influencing respiratory adaptation (see Chapter 5 on
the limbic system).
Figure 12-1 n Typical infants accumulate a multitude of experi- environment with a doctor’s office, and the chance to estab-
ences as they move smoothly in their environments. lish rapport is lost. In some instances it is preferable to have
the parent gently remove some of the child’s clothing
or even to leave the child dressed during the first therapy
session. Gaining the trust of the child and parent is crucial
during the first few sessions.
Examination of the child’s status is more likely to be
adequate if the therapist follows the child’s lead when pos-
sible. Notes can be organized later to conform to a specific
format. It is often possible to jot down essential information
while observing the child moving spontaneously or while
the parent is holding the child. Reactions to the supporting
surface will differ in these circumstances. After the session,
the therapist may dictate the salient information into a tape
recorder, or a videotape or digital tape can be made to
Figure 12-2 n Lack of support surface contact demonstrates dif- capture the interactions and movement patterns. Attention
ficulty conforming to and activating off of the supporting surface. should be given to the typical movements of the child and to
those postures that the child spontaneously attempts to con-
trol. Building a treatment plan will be based on the strengths
of the child noted in these first encounters. Eye alignment is
important; the correspondence between visual and postural
reach a toy? Are clearly typical responses occurring with activity relates directly to the quality of movement control.
specific interference by reflexive synergies or total patterns It is important to note the interaction between the two sides
of movement? Does the child rely heavily on visual com- of the body. In noting atypical reactions and compensatory
munication? Do the eyes focus on a presented object, or movement patterns, the therapist must also indicate the
does the postural abnormality increase with an effort to position of the body with respect to the supporting surface.
focus the eyes? Does the child lead or follow hand activity? There is a tendency to compile more pertinent data by learn-
Does an effort to move result only in an increase of postural ing to cluster observations and relating one to the other.
tone with abnormal distribution? Does respiration adapt to Children are vibrant beings. Their choices of position tell us
new postural adaptations (Figure 12-3)? Is the child able to something about their habits and how comfortable they are
speak as well while standing as while sitting? in this situation. To be the slave of a preformulated sequence
This type of observation is valuable because movement destroys the decision-making initiative appropriate to the
patterns directly reflect the state of the central nervous sys- situation at hand. This is true for the therapist as well as for
tem and can generally be seen while the parent is still han- the child. Although it is important to see the child in every
dling the child.18 Once the child is on the mat or treatment position, making smooth transitions from one to another
table, outer clothing can be removed to observe interactions will ensure that the child is secure and give the therapist a
of limbs and trunk. Movement responses of the child can more accurate assessment of the child’s abilities. Noting the
gradually be influenced directly by the therapist. Many “preferred” position or movement strategy can provide
disabled children associate immediate undressing in a new information about the ability to conform to a support surface,
initiation of movement, muscle tightness, muscle tone distri- Reactions to Placement in a Position
bution, and movement variety in the child’s repertoire. If the child totally avoids certain postures during spontane-
Standardized assessments are often used by facilities to ous activity, these are likely to be the more important posi-
document the developmental level of the functioning of a tions for the therapist to evaluate. Observing how the child
child with disability and to justify treatment. The Gross conforms to the support surface and how much contact there
Motor Function Measure (GMFM) was developed to as- is with the surface will provide information about the ability
sess children with cerebral palsy and has good reliability to initiate movement from the surface. Support surface con-
and validity for children aged 5 months to 16 years.19,20 The tact is essential for weight bearing and weight shifting
Gross Motor Function Classification System (GMFCS), to occur; both are critical for movement. Placement of
developed in 1997, is often used in conjunction with the the child in the previously avoided position will permit the
GMFM.21,22 The GMFCS has five levels of classification therapist to feel the resistance that prevents successful con-
for gross motor function, emphasizing movement initiation trol by the child.29 As mentioned previously, this may be held
related to sitting, walking, and mode of mobility. Descrip- for the end of the assessment. The parent should play an
tors of motor function span an age range of 2 to 18 years, active role in the assessment whenever possible. Continued
reflecting environmental and personal factors. The Pediat- dialog with the parents reveals factors such as the frequency
ric Evaluation and Disability Inventory (PEDI) assesses of a poor sitting alignment at home or a habitual aversion to
children aged 6 months to 7.5 years in three domains: the prone position. Sitting close to the television set or tilt-
social, self-care, and mobility.23 The Functional Indepen- ing the head when looking at books should also be noted so
dence Measure for Children was developed as a test that functional vision skills can be related to other therapy
of disability in children aged 6 months to 12 years. This interventions.31 These contributions by the parents establish
assessment covers self-care, sphincter control, mobility, the importance of good observation and the need for parents
locomotion, communication, and social cognition.24-26 This and the therapist to work cooperatively. Therapists of differ-
tool has been used to track outcomes over time. Although ent specialties need to initiate continuing communication to
several instruments have been developed that meet psycho- coordinate therapy objectives.
metric criteria to document function in children with dis- According to the guide for typical development, infants
abilities, the GMFM and the PEDI are thought to be the should be able to maintain the posture in which they are
most responsive to change in this population of children placed before they acquire the ability to move into that posi-
because of their good reliability and validity.27,28 Often the tion alone.32,33 The problems presented by cerebral palsy
decision to use an instrument to assess development will occur to some extent as a reaction to the field of gravity in
be left up to the clinician or facility. To date, there is no one which the child moves.32 Visual perceptions of spatial rela-
tool that will cover all the categories necessary to docu- tionships motivate and determine movement patterns while
ment change in a child with cerebral palsy, so the clinician the child must react at a somatic level to the support surface.
will need to rely on observational skills to describe quality It is helpful, therefore, to attempt placement of the infant
of movement and response to changes in position in space or child into developmentally or functionally appropriate
and handling. postures that are not assumed spontaneously (Figure 12-4).
Each child will differ in the ability to separate from her or Resistance to placement indicates an increase in tone, a
his parents. Spontaneity of movement, interest in toys, gen- structural problem, or an inability to adapt to the constella-
eral activity level, and communication skills will also vary tion of sensory inputs for that alignment. A movement that
from child to child.29 Responding to the specific needs of the resists control by the therapist will be even less possible for
child enables the therapist to set priorities more effectively. If the child. What appears to be a passive posture may hide
fatigue is likely to be a factor, it is important first to evaluate rapid increases in hypertonicity when movement is initiated
those reactions that present themselves spontaneously, fol- or instability of a proximal joint when weight bearing is
lowed by direct handling to determine the child’s response
and potential for more typical movements. Movements or
abilities for which there is a major interference from spas-
ticity, reflexive responses, or poor balance may be better
checked at the termination of the assessment so that the
child remains in a cooperative mood as long as possible.
Information regarding favorite sleeping positions, self-care
independence, and chair supports used at home can be
requested as the session comes to a close.
Clinical reasoning involves taking information from the
assessment, including observations, results from standardized
tools, family input, and the therapist’s handling of the child to
formulate a treatment plan. Placing this information into a
framework that makes sense to the therapist, the physician,
other health professionals, and the family will assist in goal
writing. The International Classification of Functioning,
Disability and Health (ICF)30 is well known in the field
of health care and allows one to see the overall interaction of
the person with his or her environment and activities in the Figure 12-4 n Baby treatment must be dynamic and precisely
presence of the health condition.30 oriented to individual needs.
initiated. A child may have learned to avoid excitation of the smoothly established. Strabismus dysfunctions commonly
unwanted reactions and may fix the body position to avoid coexist with cerebral palsy and may cause the child to receive
the alignment that cannot be controlled. Another child may a double image of environmental objects. Judgments about
enjoy the sensory experience of accelerated changes in pos- space are related to a three-dimensional perception of the sur-
tural tone and deliberately set them off as a means of receiv- rounding environment, which requires coordinated use of the
ing the resulting stimulation to his or her system. two eyes. Conservative management of eye alignment prob-
lems is done with the use of lenses and prisms by the experi-
VISUAL-MOTOR ASSESSMENT enced optometrist, which permits the therapist to work for
It is the visual-motor aspect of performance function that is basic head control by the child before any irreversible changes
of primary concern to the therapist because spatial judgments are made to the eye muscles. Eye movement differentiates
are needed to control movement of the body in an upright from head movement in much the same way that the hand
alignment. The infant who is able to stand and walk along differentiates from general arm movement, corresponding to
a support and then seems unable to let go of the support is general maturation of the central system.
often found to have functional vision interferences. The child Because the visual system is first a motor system, chil-
with cerebral palsy most often demonstrates significant neu- dren with cerebral palsy most often have difficulty separat-
romotor delay in the developmental process, which often ing eye movement from head movement and controlled
results in the inadequate establishment of matching of inputs convergence for focal changes. When their posture is sup-
from the postural and visual systems (Figure 12-5). Visual- ported, eye movement can proceed to evolve in accuracy and
motor learning experiences are filled with compensatory complexity. With inadequate alignment of the head in rela-
responses from both systems. Vision plays an important role tion to the base of support, the visual system accumulates
in early motor development for learning about, manipulating, distortions and inconsistent input, which leads to the forma-
and exploring the environment. Therefore vision requires tion of an inadequate perceptual base for later motor learn-
attention during the assessment of motor abilities. (Refer to ing (see Chapters 4 and 28). Even after improvement in the
Chapter 28.) control of posture and movement, the visual system contin-
The visual system in its development has many parallels ues to adapt to the previous faulty visual-motor learning,
with the postural system.34 Binocular control and freedom of resulting in perceptual confusion and inefficient organiza-
movement are necessary for the system to function properly. tion of body movement in space. The therapist who is work-
Ambient visual processing must be integrated with central ing for improved motor control may notice that such a child
visual processing to take in information that relates to position reacts with adequate postural adaptations when facing the
in space and to focus on a particular target. A simple screen- therapist or a support and that the movement quality seems
ing examination may check acuity at 20 feet on the E chart to disintegrate when the child faces an open space. This
and declare vision to be normal. An ophthalmological exami- immediately jeopardizes the ability of the child to use her or
nation is needed to determine the health of the eye structures, his new responses after leaving the therapy environment.
particularly in the case of infants born preterm. Equally Visual orientation to the environment will dictate alignment
important is a functional vision examination given by a against gravity, and the reverse is also true; poor alignment
behavioral or developmental optometrist to reveal the level of against gravity will affect visual orientation to the environ-
efficiency that the two eyes have achieved in working together ment. Movement, postural stability, and muscle activation
and whether the ability to focus in far and near ranges is are closely related to vision.35
Padula, a behavioral optometrist specializing in neuro-
optometric rehabilitation, has described a posttrauma vision
syndrome in adults with acquired central dysfunction and
has applied this information to children with cerebral palsy.36
A perceptual distortion in the perceived midline of the body,
known as visual midline shift syndrome, is corrected with
the use of prescribed prism lenses, which then permits the
child to step into the perceived space with more confidence
(Figure 12-6). The observant therapist will begin to notice
that the sudden increase in neuromuscular tension in a child
taking steps in a walker is often accompanied by closing
of the eyes. This seems to be a momentary inability of the
central processing system to integrate the information arriv-
ing from different sources. With the use of prism correction,
the child experiences the body as more coherent with visual-
spatial perceptions. By incorporating an understanding of
visual observations into intervention strategies, physical and
occupational therapists are able to note compensatory adap-
tations by the complementary systems and use them to their
advantage in effective treatment intervention.
Some children who walk on their forefeet or even on their
toes and who have made little if any permanent gait change
Figure 12-5 n Touching the target integrates the new visual after the use of inhibitory casting or orthotics also fall
perception with the motor response. into the population described previously. With prisms that
influenced. When the child’s body does not respond in a

smooth way, the child begins to learn and perfect the uncoor-
dinated reaction. Repetition of inadequate ranges of move-
ment and limited variability of movement patterns begin to
establish the atypical appearance of posture in the child with
cerebral palsy.
The quality of a body posture or position in space deter-
mines the quality of the movement that is expressed. Lack
of head control, poor midline organization, and deficient
trunk strength begin the process of compensation. From a
distorted starting position the movement initiated is one that
is restricted (Figure 12-7). The lack of central “core” stabil-
ity in the body restricts the full mobility of a limb. This
limitation over time is increased by fascial and muscular
restrictions on smooth coordinated muscle action. The child
continues to learn the atypical responses because the move-
ment patterns tend to be reinforced by either accomplish-
ment or reinforcement of some kind from the environment.
Compensatory movement patterns evolve because of neces-
sity rather than any feedback as to efficiency or functional
smoothness.
Habitual movement patterns are established on the basis
of frequency of use, so the child with cerebral palsy tends to
repeat the atypical responses that have been learned. In the
Figure 12-6 n This 3-year-old girl with diplegia takes her weight therapy situation the child has the opportunity to learn new
evenly over two feet with the help of prism lenses to shift her per- combinations of input to create the basis for a more stable
ception of space while engaged in a motor task. postural control. Careful analysis of the postural adjust-
ments and movement patterns of the child with cerebral
palsy is crucial to initiate effective intervention strategies.
correct the perception of forward space, the child places the There are many factors to be considered in the context of the
entire foot in contact with the support. Such prism lenses are continuing developmental changes in the child, which makes
used during therapy handling as a perceptual learning expe- a simple solution impossible.
rience for the child, with the optometrist and the therapist Active therapy intervention allows the sensorimotor
coordinating their efforts. Hand-coordination activities also learning of the child to be modified so that some part of the
require timing of reach and grasp that is based on feed-for- compensatory response becomes unnecessary and the move-
ward input from the visual system.35 In some cases the ment becomes more typical (Figure 12-8). This relative
therapist observes the visual system to overfocus in the mo-
ment that the child loses control of his or her postural stabil-
ity. This suggests that the visual system may be attempting
to compensate for the inadequacy of the postural control,
much in the same way that we all adjust our head position
to see better. Understanding the nature of the continuing
dynamic interaction between these two functional subsys-
tems of the central nervous system and attending to the
needs of visual-postural orientation will increase the suc-
cessful evolution of clients with cerebral palsy.
POSTURE AND MOVEMENT

COMPENSATIONS
Compensatory patterns of movement arise from the motiva-
tion of the child to move in spite of various restrictions on the
expression of that movement. Components in the develop-
mental process that drive a person to right the head with the
horizon and the body against gravity are met with interfer-
ence from the central nervous system. Visual impressions of
the environment motivate movement, and the infant attempts
to influence nearby objects or confirm visual impressions by
reaching into the environment and touching. As visual aware-
ness enlarges to include more distant targets, the infant is
motivated to move toward the object or person seen. With
poor balance between flexion and extension and poor grad-
ing of agonist with antagonist, the resultant movements are Figure 12-7 n Compensatory postures restrict movement initiation.
Another body of work explains more about the direct link

between food intake and muscle efficiency for high perfor-
mance and normal function. The need for water is para-
mount for healthy fascia. In children with cerebral palsy and
related disorders there is often from the beginning a diffi-
culty in the smooth automatic sucking needed for nutritive
intake. Uncoordinated patterns of mandibular and tongue
motion persist when not addressed in early and precise inter-
vention strategies. Even the digestive process is affected
negatively by inadequate chewing, a higher-than-typical
percentage of food allergies, and less-than-efficient physio-
logical functions.45
It is likely that brain dysfunction in some of these
children extends to the hypothalamus, thus influencing the
entire digestive process. Duncan and colleagues46 have
documented the risk of osteopenia in nonambulatory chil-
dren with cerebral palsy. This retrospective study showed
that fewer than 75% of the calories needed were adminis-
tered to 95% of the children with gastrostomy tubes.
Nutrients were also deficient. This may explain part of the
poor physical response level of such children. Sonis and
Figure 12-8 n The experience of coming to stand over the more colleagues47 looked specifically at energy expenditure
affected side activates diagonal patterns of postural adjustment. in children and adolescents with spastic quadriplegia in
relation to food intake. They found dietary intake to be
markedly overreported for this population and determined
approximation of what is expected in a typical response may that nutrition-related growth failure was likely related to
occur in the area of initiation, timing, strength, or ability to inadequate energy intake. Reflux is also common in in-
sustain an antigravity alignment. As movement expression fants with developmental problems. In some infants reflux
and postural stability are better established, the compensa- subsides as the physical stress is reduced in the tissues
tory patterns are used less often, and new motor learning bordering the upper thoracic and cervical spine, but it can
occurs on a base of closer-to-normal experience. be related to milk sensitivity or even susceptibility to
Compensatory processes have their positive aspects.37 environmental contaminants.
The independence finally achieved by the older child reflects To supplement nutritional intake in the child with cere-
her or his intelligence and motivation and the family’s atti- bral palsy, the individual child must be considered with re-
tude toward the child and the disability. The most debilitat- gard to age, size, activity level, and growth factors.48 Ideally,
ing handicap of cerebral palsy in an intelligent child may be blood, urine, or hair analyses would be done to determine
social or psychological when the child is not accepted by the nutrient imbalances, and supplementation with specific nu-
family and therefore cannot develop a positive self-image. trients would be guided by a specialist. Environmental
Compensatory movement patterns may permit greater inde- medicine has taken the lead in this type of work. The reha-
pendence, if and when they do not limit or block the active bilitative process places increased demands on the entire
learning of new motor strategies. system and requires fuel to set the stage for improved mus-
cle function. Protein, carbohydrates, and adequate hydration
are sources that build muscle and provide a foundation for
OTHER ASSESSMENT CONSIDERATIONS strengthening and the advancement of motor skills in popu-
Nutritional Aspects of Neuromotor Function lations without disability.49,50 A well-balanced diet will
Nutrition is viewed as providing an important biochemical provide the requisite energy for exercise. However, little
base for enhanced human performance. Williams,38 author of research has been done specifically on children with cere-
the classic reference Biochemical Individuality, was one of the bral palsy and appropriate levels of protein during exercise.
first to point out the existence of significant variability in the Therefore it is necessary to discuss these issues with the
need for specific individualized nutrients because of differ- family using caution unless specifically trained to do so. A
ences in assimilation and other factors. Physicians Crook and nutritional consultation is warranted when concerns arise in
Stevens,39 Smith,40 Pfeiffer,41 and Cott42 are a few of the lead- this area.
ers who have analyzed the link between nutrient intake and
behavioral differences in children and adults. Many of these Consideration of Supplemental Oxygen
references address issues of attention and learning. To have Oxygenation of muscle tissue is considered essential for
efficient function of the transmitters at the myoneural junction smooth movement control, and it is generally accepted that
and good health for the myelinated neurons of the nervous respiratory support increases automatically to permit faster or
system, a variety of trace elements must be present.41 Lack of stronger movement patterns in a typical subject. Therapists
dendritic proliferation is associated with malnutrition regard- often note that children with cerebral palsy resist moving into
less of the cause.43 The ambulatory child with cerebral palsy new ranges of movement and that respiratory adaptation does
will need to be considered for a new level of energy expendi- not occur automatically. Supporting the child in the novel
ture to avoid short stature and poor nutritional status.44 posture until a respiratory adaptation is noted results in
acceptance of the new experience. Oxygen needs increase in

children during growth spurts or when mastering more verti-
cal postural alignments. Increased oxygen is also required for
sustained activity such as continuous walking.
Shintani and colleagues51 performed a careful study of
233 children with cerebral palsy to determine the presence
of obstructive sleep apnea. In 10 children with cerebral
palsy who were received at the hospital for treatment of
severe obstructive sleep apnea, these authors determined
that adenoidal or tonsillar hypertrophy were noted in only
four children and that the main cause of sleep apnea in the
other six children was pharyngeal collapse at the lingual
base. Fukumizu and Kohyama52 looked at central respira-
tory pauses, sighs, and gross body movement during sleep
in 19 healthy children, ages 3 months to 7 years. Central
pauses occurred more often during non–rapid-eye-movement A B
sleep and increased with age. Developmental differences
need further study. Figure 12-9 n The two sides of the body (A and B) often respond
Decreased oxygen levels have been associated with im- very differently to the same task, and therapy must be adapted
paired cognitive and physical performance in the litera- accordingly.
ture.53 In the presence of inadequate peripheral oxygen satu-
ration, low levels of oxygen can be administered during the
night. This practice has been used with selected low-tone Case Management and Direct Intervention
and athetoid children for improved energy during the day Simple documentation of observed changes in a child over a
during growth changes, but formal study is needed on a series of regular clinic visits is still too common for many
larger group of children with cerebral palsy. Better oxygen- children with cerebral palsy. Regular appointments, with
ation of the tissues can also result in increased food intake periodic assignment of a new piece of apparatus, do not
and consequently improved energy levels. constitute active treatment. Although physical intervention
in the form of direct handling of the child is considered a
conservative treatment by most physicians, relatively few
ROLES OF THE THERAPIST
children receive sufficient physical treatment at an early
Role of the Therapist in Direct Intervention age.56,57 Therapists need to demonstrate their unique prepa-
The primary role of the therapist is in direct treatment ration and describe their interventions in ordinary language
or physical handling of the child in situations that offer so that families as well as other health care professionals
opportunities for new motor learning. This should precede understand the importance of specific treatment versus gen-
and accompany the making of recommendations to par- eral programs of early stimulation that are designed for
ents, teachers, and others handling the child. Positioning neurologically intact infants.
for home and home handling recommendations should The prognosis for change in cerebral palsy is too often
always be tried first by the therapist during a treatment based on records of case management rather than on the
session. As noted for the initial assessment, many inter- effect of direct and dynamic treatment by a well-prepared
ventions will cause a reaction unique to the particular therapist. Bobath32,58 documented accurately the develop-
youngster.29,54 It is the role of the therapist to analyze the mental sequence expected in the presence of spasticity or
nature of the response that is accompanied by adaptation athetosis. Her book consolidates some observations of older
inadequacies, to analyze the movement problems, and clients that help professionals understand the uninterrupted
to choose the most effective intervention (Figure 12-9). It effects of the cerebral palsy condition. In any institution one
will then be possible for other persons to manage play acti can observe the tightly adducted and internally rotated legs,
vities and supervise independent functioning that reinforce the shoulder retraction with flexion of the arms, and the
treatment goals.55 chronic shortening of the neck so common as the long-term
The therapist working with these children becomes an effects of cerebral palsy. The long-term influence of atheto-
important and trusted resource to the family. At times the sis results in compensatory stiffness or limited movement
therapist who has had the more consistent contact with the patterns to create a semblance of the missing postural stability
child becomes the facilitator of better communication be- while a limited number of movement patterns with limited
tween the parents and medical or health care professionals. degrees of freedom are used to function (Figure 12-10).
The child who starts early and continues with the same Within the clinical community there is increasing evi-
therapist may make of this person a confidant and share dence that soft tissue restrictions further limit spontaneous
concerns that are difficult or uncomfortable for the child to movement in children with cerebral palsy. The fact that these
explain to parents. It is a challenge for the therapist who fol- fascial restrictions are often found in infants suggests that
lows the same child for an extended time to come up with they originate early rather than as a gradual result of limited
appropriate goals and new activities to continue positive ranges in movement. Because of the tendency of fascial tis-
change. Part of direct intervention is to recognize when the sue to change in response to any physical trauma or strong
amount of therapy can be reduced and replaced with recre- biochemical change, some of these characteristics might be
ational activities with peers. originating with traumatic birth experiences, and they would
whether the same principles that are considered important in

healthy individuals apply to people with disability. However,
it makes sense that practice would influence the use of any
new or relearned skill.
An occupational therapist, Josephine Moore, stressed
Bach-y-Rita’s66 works to emphasize some important points
for therapists regarding the concept of increasing functional
demands on the central system and the importance of the
neck structures in developmental movement sequences.
Children with spasticity often have a lack of developmental
elongation of the neck, whereas children with athetoid or
dystonic movement lack neck stability and consistent pos-
tural activation. Tone changes often originate with changes
in the delicate postural interrelationship between head and
body or with ambient visual processing.
By appreciating the abundance of polysynaptic neurons
and parallel processing in the central nervous system, the
therapist will become more optimistic regarding his or her
role as facilitator and feedback organizer to guide new
movement. Restak,67 in his book The New Brain, confirms
the continual reorganization of the brain in response to new
input. Several animal and human studies on neuroplasticity
have confirmed that the brain reorganizes after an injury and
that this reorganization is shaped by rehabilitation and
motor skill learning.68-71 In the child with cerebral palsy, the
therapist looks for subtle changes in the child’s response
to determine newly integrated sensorimotor learning. For
Figure 12-10 n Attempted movement activates atypical patterns example, excessive emphasis on extensor responses in the
and restrictions; restrictions are revealed with limited degrees of prone posture for the older child can jeopardize the quality
freedom available for function. of neck elongation in sitting, so it is essential to work on
the components necessary for control of the new posture
desired.
be exacerbated by daily use of limited patterns of movement. Therapy intervention is far from innocuous when it is
Tissue restrictions can also occur with immobilization or responsibly applied. A truly eclectic treatment approach
general infectious processes.59-61 Soft tissue begins to change comes with clinical experience and personal consideration
its physiological structure with the application of gentle sus- of observations of the functional problems presented
tained pressure, so it serves as documentation that there are by the complex issue of cerebral palsy at different ages.
changes caused by the therapist’s simple hand contact. Some Priorities in intervention strategies have a practical aspect,
of the sensory information in the form of tapping or holding and new developments in our knowledge lead us forward
or application of pressure affects fascial meridians and mus- in clinical applications. The intricacies of typical develop-
cle alignments.62 A therapist must be prepared to defend his ment offer many new clues for new effective interventions.
or her approach with a solid foundation, theory, and objective With high-quality treatment intervention the need for
outcomes. Objective documentation is important when deal- direct therapy service as a crucial aspect of case manage-
ing with any population but essential for demonstrating ment for these children is confirmed. Clinical findings in
therapeutic change. individual case studies need to become part of the profes-
Applying specific soft tissue treatment techniques to any sional literature to strengthen the efficacy of intervention
person with a neuromotor disorder creates the need for in this population.
immediate follow-up with practice of new skills using this
improved range of motion. Creating excessive tissue mobil- Special Needs of Infants
ity in a given area of the body can destroy the delicate The direct treatment of infants deserves special mention
patterns of coordination that permit synergic function in the because there are significant differences in intervention
person with cerebral palsy, so functional activation of the strategies for the infant and the older child. Aside from the
body after each specific mobilization is strongly recom- delicate situation of the new parents, the infant is less likely
mended to integrate the tissue change. Well documented in to have a diagnosis and presents a mixture of typical and
the literature on current motor control and motor learning is atypical characteristics. It is essential that the clinician have
the need to practice, practice, practice.63 Practice time is a strong foundation in the nuances of typical developmental
related to skill performance; the amount and type of practice movement and early postural control.4,72 Soft tissue issues
are determined by the stage of learning that an individual is must be addressed in detail. Direct intervention can be
in and the type of task to be learned63-65 (see Chapter 4 on offered as a means of enhancing development and overcom-
motor control and learning). Interestingly, most of what we ing the effects of a difficult or preterm birth. It will be im-
know about motor control and motor learning is based on portant, however, to pursue a diagnosis for the infant who
individuals who are “typicals,” and it is yet to be determined reaches 8 or 9 months of age and continues to need therapy
because third-party payers often require a diagnosis beyond often results in improved balance in standing. Any freedom
developmental delay or prematurity. gained in upper body control results in more efficient bal-
Infants with early restrictions in motor control should be ance in the upright posture.
followed until they are walking independently, even if they Inhibiting or stopping the movement of one part of a
no longer need weekly therapy. Infant responses can change movement range or even one limb must be done in a way
rapidly as the therapist organizes the components of move- that permits the child to activate the body in a functional
ment control. Soft tissue restrictions should be treated ini- way. The child who lies in the supine position with extreme
tially to have more success with facilitated movement re- pushing back against the surface is rarely seen when therapy
sponses. Careful observation is essential because all but the intervention has started early. The therapist initially elimi-
severely involved infant will change considerably between nates the supine position entirely but would incorporate into
visits. The therapist should invest some time in training the the treatment plan the activation of balanced flexion and
parents to become skilled observers while appreciating the extension in sitting with the ability to vary pelvic tilt for
small gains made by their infant. Physiotherapist Mary functional play and reaching (Figure 12-12). The child
Quinton73 has written specific intervention strategies for might later be reintroduced to a supine position with pos-
babies (Figure 12-11). Infant massage is important to im- tural transitions that support balanced control of the body
prove the bonding of mother and child and to improve with more differentiated movement.
physiological measures.74,75 One of the primary considerations for the child with spas-
Referral to other health care professionals is essential in ticity is adequate respiratory support for movement. Mobility
the presence of possible allergies, new neurological signs, of the thoracic cage and the midtrunk must be combined with
visual or auditory alterations, and persistent reflux or nutri- trunk rotation during basic postural transitions (Figure 12-13).
tional issues. There is always the possibility of convulsions Consideration of age-appropriate movement velocity will
when some brain dysfunction is present, and neurological guide the therapist in choosing activities that challenge better
evaluation should be recommended if this is a concern. respiratory adaptability and prepare for speech breathing
to support vocalization. The therapist will find it helpful
ORIENTATION TO TREATMENT STRATEGIES to hum or sing or even make silly sounds that encourage
The child whose movement is bound within the limitations sound production by the child during therapy. Movement
of hypertonicity suffers first of all from a paucity of move- of the child’s body changes respiratory demands and fre-
ment experience. Because early attempts to move have quently results in spontaneous sound production during ther-
resulted in the expression of limited synergistic postural apy. Assessing the ability to sustain a breath to speak is easily
patterns, the child often experiences the body as heavy or done during a therapy session by counting the letters in the
awkward and loses incentive to attempt movement. The alphabet that can be said with one breath. This should be
therapist will want to focus on the child’s ability to sustain done with the child supine and in an upright position because
postural control in the trunk. Central “core” stability to sup- trunk control required while sustaining a breath changes with
port directed arm movement or weight shifts for stepping the posture attained against gravity. Describing the chest
have not developed, so they need to be addressed during shape and movement of the thorax observed can serve to
therapy intervention. Improved upper extremity control assist the therapist in problem solving and prioritizing the
opens the possibility for new learning of more coordinated treatment plan.
tasks. Specific work on hand preparation for reach and In some children respiratory patterns remain immature
grasp follows use of the arm for directed movement and and superficial, which may be related to the causative factors
of the impairment. A lack of postural control limits even the
physiological shaping of the rib cage itself because the ribs
do not have an opportunity to change their angle at the spine.
A B
Figure 12-12 n A, Strong asymmetry and atypical tone in the
Figure 12-11 n Mary Quinton, British physiotherapist, is widely supine position. B, Simple seating can inhibit strong asymmetry
recognized as the originator of effective infant intervention. and make function a possibility.
time and a range of movement prepared beyond the essential

range for the functional goal. The therapist is addressing
a system that is deficient in its ability to receive, perceive,
and use the available input. This makes careful analysis
and functional orientation of the sensory input essential. If
the microcosm of experience given the child during a ther-
apy session is no more intense than an equal amount of
time in her or his living environment, the therapist has failed
to use this unique opportunity to deliver a meaningful mes-
sage to encourage the learning of new motor behavior.
Although therapists cannot provide during a treatment ses-
sion every experience necessary for all movement scenarios,
the therapist should provide the component parts necessary
for motor skills to be transferred or generalized to other
activities.
The therapist working with the child with cerebral palsy
constantly monitors the quality of the child’s motor response.
These continuing observations guide the manipulation of
the environment and the assistance given the child to move
toward a functional goal. Is the body tolerating the posi-
tion? Does the child adapt to the supporting surface and
use the supporting surface contact for movement initiation
(Figure 12-14)? Is the movement of a limb graded and with-
out unwanted associated reactions in other parts of the body?
By analyzing the answers to such questions, the therapist is
guided to an appropriate sequence of the therapy session and
is enabled to set functional treatment goals and realistically
change prognoses.
The therapist makes constant judgments as to the child’s
Figure 12-13 n Rotational patterns combined with transitional responses during therapy, challenging the child’s system
movements can be used to mobilize the thoracic cage. while ensuring success and moving toward improved con-
trol. By using specific intervention strategies, the therapist
works to introduce new somatosensory and motor learning.
The therapist must give careful support to sustain the transi- The therapist may introduce a slight modification of the
tional posture of the older child during transient respiratory child’s response, such as an elongation of a limb as it is
change. An active respiratory adaptation will increase the vari- being moved. At other times the therapist augments sensory
ability of postural adaptation. Improved respiratory adaptation information that helps direct a movement. Weight bearing
will improve trunk tone, just as dynamic trunk alignment over the feet may be simulated with the young child’s foot
facilitates better respiration. against the therapist’s hand and pressure given through the
Weight bearing changes postural tone. The trunk can be knee. Visual-motor experiences can be altered with the
helped to experience weight bearing in a variety of align- child’s use of prism lenses, prescribed by an optometrist for
ments by using inflated balls or rolls that offer a contoured use during therapy.
surface. The threshold of the original response is gradually To be meaningful, sensory input must be contextual and
altered so that the child begins to learn the new sensations meaningful to the individual who is receiving it. Multiple
and can follow guided postural transitions. When there are sensory systems are simultaneously activated by most thera-
distinct differences between the two sides of the body, atten- peutic input, and a variety of sights and sounds may be
tion must be given to lateral weight shifts in sitting and available in the immediate environment. Memory, previous
standing. Changes near the vertical midline of the body
seem to represent the more difficult input for the compro-
mised system to integrate. It may be necessary to assist
sustained weight over one side and then the other to initiate
the change. It is important to assist the shoulders to align
with the hips and that the visual orientation of the individual
brings the head to a correct alignment. Young children need
special help with segmental rotation of the trunk in the verti-
cal alignment so that the weight-bearing side is relatively
forward with dynamic balance of flexion and extension
influences.
Children and adolescents with cerebral palsy often require
a more intense or prolonged sensory cue for a desired move-
ment response to be obtained. Weight bearing against the Figure 12-14 n This child has little contact with the supporting
surface may need to be sustained for a prolonged period of surface, resulting in poor movement initiation.
learning, and cognition are activated during the therapy level to integrate more basic abilities. The assisted self-
interaction. The therapist makes a continuous reassessment dressing process is an effective way to introduce and inte-
of the child’s experiential needs compared with the current grate new movement and sensorimotor learning while using
input provided. When the therapist works with the child in a established movement skills. To sit well, the child needs
more upright alignment during at least part of the session, practice moving over the base of support and coming in and
the central nervous system is alert and more receptive to the out of sitting, and control of coming to stand from sitting.
incoming information. To walk well, the child may need to practice running to
The developmental meaning attached to the sensation of allow practice in changing rate, direction, range, and bal-
typical movement is complex and starts with the ability to ance. Sitting is made more dynamic by using a gymnastic
process contrasting stimuli. While several parts of the body ball as a seat. Transitional adaptations of posture may
are stable, another is moving. Stability of the proximal body be elaborated during therapy sessions to include more
permits a limb to extend forcefully or to be maintained complex alignments. Specific techniques are reviewed in
in space. Each new level of developmental dissociation of Chapter 9.
movement increases the complexity of central nervous sys- With the child dominated by athetoid movement, the
tem processing. The process of self-feeding illustrates how therapist’s role relates primarily to organization and grad-
internal and external stimuli impinge simultaneously on the ing of seemingly erratic movement responses and establish-
central nervous system. The process of guiding a full spoon ing function around midline. These children have the ability
toward the mouth initially engages the child’s attention. The to balance, but their balance reactions are often extreme in
arm is lifted at the shoulder to bring the fragrant food odor range and velocity. Their movements are rarely in the mid-
to the level of the mouth before elbow flexion takes the line, asymmetrical, and frequently dominated by primitive
spoon to the face (Figure 12-15). Between 2 and 6 years of reflexes, with poor midrange control of the trunk and ex-
age the self-feeding pattern is modified and the elbow moves tremities. Cognitively they are eager to participate and usu-
down beside the body. Now the motor aspect of the task has ally are responsive to working on specific goals that relate
become procedural and more efficient, permitting the child to functional success. By working to improve central con-
to participate in social exchanges with the family at the same trol, the therapist gradually introduces taking of body
time that she or he manages independent self-feeding. The weight over the limbs, with assistance to grade the postural
complexity of the task increases with the secondary task of control of the central body. By working closely with a
social exchange. behavioral optometrist the therapist can use visual input
A solid understanding of typical developmental sequences to improve the child’s balance reactions. In these children
is essential for the clinician providing direct treatment inter- the therapist may note that disruption of eye alignment or
vention.18,76 Early responses of the typical infant change focusing results in a momentary disorganization of postural
from a self-orientation to an environmental orientation as control (see Chapter 28).
new developmental competence emerges. More sophisti- Movement control must become procedural so that it
cated balance in independent sitting occurs as the ability is not interrupted by every environmental distraction. This
to pull to standing at a support begins to develop. Such is more likely to happen when balanced activity of the
knowledge of developmental details supports the therapist in visual, vestibular, and proprioceptive systems has been
introducing postural activities at a higher developmental achieved. Independent ambulation becomes practical when
the individual is able to think of something else at the same
time. The therapist begins this process by carrying on a con-
versation with the child to engage the cognitive attention so
that the motor act becomes more automatic. The concept of
graded stress is discussed in Chapters 5 and 6.
Direct intervention for the hemiplegic child takes into
account the obvious difference in postural tone between one
side of the body and the other. Treatment for children that
addresses itself only to the more affected side of the body
will not prove to be effective. The critical therapeutic experi-
ence seems to be that of integration of the two sides of the
body and the establishment of midline (Figure 12-16). The
child with hemiplegia differs from the adult stroke patient in
that the adult had a clearly established midline and integra-
tion of both sides of the body by learning to cross midline
before the stroke episode. The child with hemiplegia has not
had that experience and will need emphasis on this during
intervention. The integration of both sides of the body be-
gins early for the typical infant, with lateral weight shifts in
a variety of developmental patterns, and leads to postural
organization that permits later reaching for a toy while the
body weight is supported with the opposite side of the body.
Figure 12-15 n Maintaining the child’s elbow in this high posi- The child with a contrast in the sensorimotor function of the
tion initially permits forearm pronation and activates the shoulder two sides of the body needs to experience developmental
in the typical developmental pattern for improved motor learning. patterns that include rotation within the longitudinal body
Figure 12-17 n Bilateral arm activity with visual regard that

Figure 12-16 n This boy with hemiplegia tries to move a chair corresponds to hand motion incorporated into therapy.
by orienting only his more active side to the task and bearing
weight only briefly on the more affected side.
body weight simultaneously, as do the two feet. Although the
more affected hand may not develop sensation adequate for
axis and lateral flexion of the trunk, the more affected side skilled activity, an important treatment goal is sufficient
forward. The more affected side needs the experience shoulder mobility to move the arm across the body midline
of supporting the body and the experience of initiating and to assume a relaxed alignment during ambulation. Early
movement. treatment increases the possibility that the more affected hand
Development of hand use first focuses on bilateral arm will be used as an assisting or helping hand. There are some
activity while keeping the affected hand well within the children who have such severe sensory loss that active use is
functional visual field (Figure 12-17). The infant or young minimal, although considerable relaxation can be achieved.
child works primarily in sitting until dynamic trunk flexion The greater the discrepancy between the sensorimotor
is activated. Pelvic mobility is essential to activate the nec- experience of the one side of the body and the other, the more
essary trunk responses. The therapist may find that true lat- tendency the system seems to have to reject one of the mes-
eral flexion of the more affected side of the body is fully as sages. This can lead to distortions in verticality and is a major
difficult for some children as the initial active elongation of interference in bilateral integration. Functional vision evalu-
that side. There tends to be a high incidence of soft tissue ation is important to avoid the midline shift problem that will
restrictions in the shoulder and neck of the affected side. distort postural control. As body weight is shifted to the more
Children with hemiplegia have difficulty in sustaining a bal- intact side, flexor withdrawal patterns of the limbs increase
anced posture against the influence of gravity, and some in frequency and strength in some children. These postural
begin to struggle to do everything with the less affected side. reactions are often associated with lack of full weight bearing
This characteristic contrast in function may contribute to on the more affected side. The presence of a lateral visual
the development of seemingly hyperactive behavior that midline shift or some visual field loss may increase the
is related to the inability of the central nervous system avoidance of bearing weight on the more affected side.72 One
to resolve contrasting incoming information. Hyperkinetic important therapy goal is the achievement of graded weight
responses in one side of the body may compensate for rela- shift through the pelvis during ambulation (Figure 12-18).
tive inactivity in the opposite side. Leg length discrepancy, Treatment strategies must incorporate a wide variety
scoliosis, pelvic obliquity, and shortening between the ribs of more basic developmental alignments in which pelvic
and pelvis may develop. One goal of treatment is to bring weight shift is a factor. The choice of prone, moving from
these divergent response levels closer together so that the sitting to four-point support, or a simple weight shift while
child can experience more comfortable postural change and sitting on a bench will depend on the movement characteris-
adapt to later school demands. tics observed by the therapist during the evaluative session.
The limbs of the hemiplegic child will change in postural Diagonal adaptations are useful in the redistribution of
tone as the trunk reactions are brought under active control tone for upright function. Careful attention must be given to
and lateral weight shifts more clearly to the more affected pelvic alignment and mobility because the pelvis has a ten-
side. The two hands need the experience of sustaining the dency to be rotated posteriorly on the more affected side in
Figure 12-18 n The use of poles was introduced by the Bobaths as a way to achieve graded weight
shift for increasingly complex postural adjustments in standing and walking.
children who have not had good early therapy. This can child’s motor output may remain limited; changes in posi-
cause increased hip flexion and incomplete hip extension at tioning and opportunities for the child to have other sensory
terminal stance later if the child begins to walk with the and visual experiences will often serve to motivate the child
more affected side held posteriorly, a characteristic that may and contribute to motor learning. Home handling needs to
be observed during analysis of leg position in gait. Dynamic include a variety of positions during each day for seating and
foot supports will facilitate a more functional weight shift play. Consistency in these practices is essential for the child
when the child is not in the treatment session. The goal of with low tone to progress.
functional movement is best reached through a wide variety The process of undressing and dressing can be a dynamic
of weight-bearing postures, from the obvious developmental part of the treatment program for any child (Figure 12-19).
alignments to horizontal protective responses or reaching
above the shoulders in sitting and standing to incorporate
practical and commonly used adaptations.
The child with low muscle tone is perhaps the greatest
challenge for both therapist and parent. Adequate develop-
mental stimulation is difficult unless positioning can be
varied. Placing the child in a more upright alignment, al-
though it is achieved with complete support initially, seems
to aid the incrementation of postural control. To prepare the
low-tone body for function, it is helpful to review the articu-
lations for possible soft tissue restrictions. However, equally
important is not to take away muscle tightness that is provid-
ing a form of stability for the child without the ability to give
him or her another form of stability for functional use. The
neck and shoulder girdle are particularly vulnerable. Strong
proprioceptive input while accurate postural alignment
is ensured is an important part of the treatment session. A
direct push-pull motion of the limbs, which is gentle traction
alternated with approximation as described by Bobath,77
also assists in maintaining antigravity positions and creates
postural variance in the practice of antigravity postural reac-
tions. Positioning at home may include a high table that
supports the arms, allows for increased trunk extension in
good alignment, and permits voluntary horizontal arm mo-
tion. The therapist must be cautious of the tendency to fixate
in response to trunk instability and initial hypotonicity. This
seemingly hypertonic response, which can be distributed in
the deeper musculature, contributes to limited adaptability
rather than differentiated postural control. It is difficult to Figure 12-19 n With assistance, this boy with right hemiplegia
ramp up the corticomotor neuron pool even though the is helped to improve his self-esteem by exploring dressing.
Diagonal patterns of movement that are incorporated into RESEARCH

the removal of socks and shoes assist in the organization of Pediatric clinicians are faced with selecting treatments that are
midline orientation. Weight shifts and changes in stability- efficacious for children with cerebral palsy. Increasing pres-
mobility distribution occur throughout the dressing process. sure on clinicians to establish that treatments are effective in
Concepts of direction and spatial orientation are applied to improving functional abilities is often dictated by third-party
the relationship of body parts and clothing. Directional vo- payers. In the past there was little research on pediatric treat-
cabulary terms and names of clothing and body parts are ment approaches and protocols that withstood the rigors of
learned with this experience. A bench is useful because it scientific investigation. Today, several methods that warrant
permits the adult to sit behind the child who is just begin- mentioning are beginning to undergo systematic investigation.
ning to participate actively. The older child with difficult Constraint-induced movement therapy (CIMT) was devel-
balance reactions can use the bench in a straddle-sit align- oped from basic science experiments on deafferenated mon-
ment (Figure 12-20). Aside from the physical and perceptual keys to overcome learned nonuse of the upper extremity.78,79
benefits, this achievement of dressing independently is one This forced use approach was adapted for adult patients after
that offers the child a feeling of pride and independence. It a stroke; the affected upper extremity is forced to participate
is also a very practical preparation for the future when it is in activities and the less involved upper extremity is con-
introduced in keeping with individual developmental and strained. Practice is intense, with 6 hours of mass practice
emotional needs (Figure 12-21). for a 2-week period.80-82 This protocol has been quite success-
ful, with significant improvement in upper extremity move-
ment and use of the affected limb. CIMT is very popular and
is now beginning to appear in the clinical setting. The pediat-
ric client has also demonstrated a favorable response to this
treatment protocol in several single-case studies.83-87 In a
clinical randomized trial by Taub and colleagues,88 children
with hemiplegia or brain injury receiving CIMT for 21 con-
secutive days, 6 hours a day, demonstrated significant im-
provement in the amount of use, quality of movement, and
spontaneous use of the affected upper extremity. These results
were sustained at 6-month follow-up. Several studies from
around the world are beginning to show up in the literature with
varied methods. Most of these studies are promising; however,
further investigation is necessary to establish the critical
threshold, adequate dosage, and selection process for subjects
who will benefit the most.89-93 Clinicians are beginning to adapt
these studies to their therapeutic settings and modify the proto-
cols for clinical use and reimbursement potential.
Treadmill training has also been used in children with ce-
rebral palsy.94-101 The treadmill has been instrumental in reha-
bilitation for many years with a variety of purposes. This
treatment began with animal studies on spinalized cats and
rats and their responses to training on a treadmill in the recov-
ery of a walking pattern.102-105 Today this treatment is used
Figure 12-20 n Straddle-sitting on a bench gives this diplegic boy in many populations, including those with spinal cord injury,
postural stability while he concentrates on the buttoning process. traumatic brain injury, Parkinson disease, stroke, and cerebral
palsy.106 The use of the treadmill in children with cerebral
palsy has shown promising results: improvement in gait pat-
tern, increased walking speed, decreased coactivation in
lower extremity musculature, overall gross motor skill im-
provement, and improved and stabilized energy expendi-
ture.94-101 Treadmill training interventions have taken on many
different faces, including use of a regular treadmill, commer-
cial equipment, and high-tech body-weight support systems
(Figures 12-22 and 12-23). Commercial equipment is now
available to assist with supporting the body weight of indi-
viduals who otherwise would not be candidates for such a
treatment. Clinical adaptations have also been incorporated to
accommodate smaller bodies on a treadmill, which allows
the therapist to assist from behind or from the side. Setting
goals for this treatment must be precise, with an understand-
ing of the purpose intended for its use: improving endurance,
changing the parameters of the gait pattern, strengthening, and
Figure 12-21 n Organization of clothing within reach is essen- gait training. Each goal must be based on a sound theoretical
tial for success in independent dressing. foundation, which is now available from the literature.
Figure 12-22 n This young girl is practicing walking on the

treadmill without holding on and incorporating arm swing to
improve her stride length. Figure 12-23 n This young girl, who is an independent ambula-
tor with a rollator walker, is participating in partial body weight–
supported locomotor training to improve her gait pattern.
Strength training was never thought possible in the pres-
ence of spasticity in children with cerebral palsy. Several
studies have now shown that strength can improve in chil- colleagues117 and Moreau and colleagues118 studied muscle
dren with spastic cerebral palsy.107-110 Circuit training was architecture as a predictor of maximum strength and its re-
used in an afterschool training program for 4 weeks, two lationship to activity levels in cerebral palsy.118 They found
times a week for 1 hour of intense group training, resulting that ultrasound measures of the vastus lateralis muscle
in improved strength and functional performance.107 Strength thickness adjusted for age and the GMFCS level were cor-
was maintained in this group of individuals at an 8-week related and predictive of maximum torque in children with
follow-up posttest. Dodd and colleagues108 set up a home- and without cerebral palsy. A variety of methods for strength
based lower extremity strengthening program in a random- training can be incorporated into a clinical treatment setting
ized clinical trial with 21 individuals with cerebral palsy. All or home exercise program with anticipated improvement in
subjects in the treatment group had improved lower extrem- muscle strength, which may also result in improved func-
ity strength that was maintained at follow-up periods of tional status for children with cerebral palsy. Strength train-
6 and 12 weeks. In a study focused on biofeedback and ing encompasses free weights, aerobic workouts, stretch
strength training to improve dorsiflexion and range of motion, bands or tubing, and machines that address resistive exercise
Toner and co-workers110 demonstrated significant changes (Figure 12-24).
in active range of motion and dorsiflexion strength.110 Many adjuvant therapies that are popular in combination
Strength training in the presence of spasticity is also docu- with other treatments are available to individuals with cere-
mented in adults as beneficial not only for conditioning, bral palsy. Electrical stimulation (ES) has been used in a
improved range of motion, and psychological well-being, variety of ways with children with cerebral palsy. In several
but in some cases reduction of spasticity.111-114 Strength case reports by Carmick,119-121 neuromuscular ES used in
training in children and adolescents with cerebral palsy has conjunction with task-oriented practice was found to im-
continued with investigations into the intensity, type of con- prove sensory awareness, strength, gait parameters, and
tractions to practice, and dosage.115,116 Interestingly, a sys- passive and active range of motion. Neuromuscular ES was
tematic review of common physical therapy interventions used as an adjunct therapy with upper and lower extremity
in school-aged children found that strength training dem- practice protocols.119-122 ES has been used in conjunction
onstrated significant improvements in selected muscle with 6 weeks of intensive therapy to improve sitting posture
groups but no meaningful change in function. Martin and and trunk control for children with spastic cerebral palsy.
treatments, to learn new movement possibilities. BtxA has a

temporary effect, so the critical element is its combination
with other treatment modalities. Park demonstrated improved
range of motion and decreased spasticity in ambulatory
children with cerebral palsy when BtxA was used in combi-
nation with serial casting.135 It has also been used as an
alternative to control the progression of hip dislocation and
hip pain in children with cerebral palsy.136
Joint and trunk taping and strapping have been used to
provide sensory input and alignment for posture, balance,
and strengthening. To date, there is no evidence that these
adjunctive treatments actually provide these benefits, but
there is also no evidence that they do not. Further objec-
tive investigation will be necessary to address the issues
presented by these additives to therapy. Both are consid-
ered noninvasive with few side effects other than those
associated with adhesive allergy to tape and autonomic
Figure 12-24 n Using free weights to strengthen the upper ex- nervous system responses such as sweating and overheat-
tremities while stabilizing the trunk while seated on an incline ing. Several different types of tape (athletic, Leukotape P
bolster. Patella Tape [Notoden, The Netherlands]; Kinesio Tape
[Kinesio USA Corporation]) and strapping devices are
available commercially.
Radiographic studies confirmed the statistical significance Soft tissue mobilization is a method of stretching tight
in decreasing the kyphotic angle of the spine and sitting structures that have become restricted from overuse, spastic-
score on the GMFM.123 Two recent studies documented an ity, deformities, muscle shortening, surgeries, trauma, and
improved gait pattern and reduced spasticity in 27 children poor nutrition. This type of stretching has strong roots in
with spastic diplegic cerebral palsy after use of transcuta- osteopathic medicine but is not limited to that area of exper-
neous electrical nerve stimulation on selected lower tise. Over the years, research investigating the cellular and
extremity musculature for 15 minutes, three times a day, for tissue changes that occur with immobilization has revealed
1 week.124,125 Neuromuscular ES was used successfully in some interesting and shocking alterations in the muscle and
combination with dynamic splinting to improve upper ex- collagen fibers. With immobilization, slow muscle fibers
tremity range of motion at the elbow and wrist in 6 children show greater atrophy than fast fibers do.59 There is atrophy,
with cerebral palsy.126 ES has been in the therapy arena for a decrease in peak torque, an increase in fatigue resistance,
many years, and its selective use with children who have loss of strength, and reduced central activation when the
cerebral palsy may supplement regular therapy sessions plantar flexors of the ankle are immobilized.137,138 In a recent
with enhanced results. (Therapeutic subthreshold ES at me- study of children with severe spasticity, muscle biopsies
ridian points is discussed in the section on electroacupunc- were performed on the vastus lateralis to determine collagen
ture treatments and in Chapter 39.) accumulation in the spastic muscle. An increased accumula-
Botulinum toxin A (BtxA) has been used to assist in tion of collagen I fibers in the endomysium of the muscle
decreasing spasticity because it provides a permissive condi- was noted, with thickening and decreased muscle fiber con-
tion that improves and increases range of motion and prac- tent in the more severe cases.139 This study, in combination
tice of new motor patterns without interference from in- with what we understand about healthy muscle, reinforces
creased muscle tone. Gait velocity, stride length, range of the need to keep the muscles flexible and active to help pre-
motion, and decreased spasticity were noted to be signifi- vent this accumulation of collagen. Soft tissue mobilization
cant in 33 subjects with cerebral palsy after local injections and deep tissue stretching are methods that can improve the
of BtxA.127 Several studies have shown a decrease in spas- ability of the tissue to lengthen and fold, allowing for a more
ticity, successful treatment of foot deformities, and im- efficient activation of the muscle fibers, thus optimizing the
proved gait parameters with BtxA.128-131 In a study by Galli formation of typical synergies during practice of motor skills.
and colleagues,132 dynamic dorsiflexion improved during As mentioned earlier, children with cerebral palsy often
stance and swing phases of gait, with significant improve- have visual difficulties that are not acuity problems and that
ment in foot placement at initial contact. BtxA has been are not correctable with a standard lens. Vision therapy has
used with varying results as an adjunct treatment for the demonstrated good results when emphasis is placed on ocular
management of upper extremity spasticity.133 Lukban and motility and accommodation.140 When children were given
colleagues134 reviewed six randomized clinical trials with a intense visuo-oculomotor training, improvement was noted
total of 115 children receiving BtxA in the upper extremi- in visuo-oculomotor control.141 Improvement in the child’s
ties. Five of the six trials demonstrated a reduction in spas- ability to execute smooth pursuit precision and maximum
ticity that was time limited, and four of the six trials docu- velocity, improvement of saccadic movement precision and
mented improved hand function. Four systematic reviews stability, and shortening of the saccadic reaction time were
concluded that there simply was not enough evidence to significant after training.141 Although many clinicians are
support or refute the effectiveness of BtxA for upper extrem- not experts in the area of vision, vision is an important part
ity use. It allows for the reduction of muscle tone and opens of every therapy session. Incorporating vision as an integral
a window of practice opportunity, in combination with other part of a therapy session will not only improve the child’s
orientation in space but will address his or her ability to scan ered moderate when used on a select group of individuals
the environment while learning to move through space. with cerebral palsy.146-149 Other, more recent spasticity man-
Many therapies become popular by purporting to be a agement programs are less invasive and are often considered
“fix” for a particular problem associated with cerebral palsy. before use of this invasive procedure.
The clinician accepts responsibility for making sound judg- In 1968 a posterior rhizotomy surgical intervention was
ments concerning treatment and outcomes for children with developed, with some success reported in reducing spastic-
cerebral palsy. Not all the treatments used in therapy will be ity.151,152 It remained for Peacock and Arens153 to apply the
investigated rigorously in a scientific manner. However, procedure more selectively and functionally and to bring it
when a treatment approach is presented as advantageous for to the United States from South Africa. On the basis of their
many diagnoses and conditions, with claims of success be- experience, Peacock and colleagues insisted on daily neuro-
yond what is reasonable for those conditions, it is your duty developmental (Bobath) treatment for at least 1 year after
to proceed with caution. Always stop and think what theory the surgical intervention. Electromyographic testing before
and frame of reference the approach will fit best. Does this and during the surgery is used to determine which posterior
“new” therapy make sense with the knowledge you have of nerve rootlets are creating the spasticity in the lower ex-
anatomy, physiology, neurology, and motor learning? As tremities.154 The foundations for success are accurate selec-
clinicians we will always be tempted to try new approaches tion of the child, an experienced surgeon, and careful analy-
before the scientific community has investigated them thor- sis of therapy goals.
oughly. Clinicians, because they are creative and innovative, A more recent improvement in the selective dorsal rhi-
have advanced our professions. It is essential to advance zotomy (SDR) procedure was developed by Lazareff and
patient care with treatments that are safe and do no harm. colleagues,155 who enter a limited number of levels rather
Every environment affords research opportunities that con- than five levels of the spinal column and prefer to work
tribute to the treatment of children with cerebral palsy. close to the cauda equina, according to the technique of
Single-case reports and single-case studies are the beginning Fasano.156 Several studies have documented improvement
of this process and, although descriptive in nature and with in function and strength and reduction of spasticity out-
limited generalization, provide evidence for new therapeutic comes as far out as 3 to 5 years.151,157 In a more recent
approaches and further systematic investigation. longitudinal study by Nordmark and co-workers,158 it was
found that SDR was safe and effective in reducing spastic-
MEDICAL INFLUENCES ON TREATMENT ity without major complications. When combined with
Because the problems of cerebral palsy are so varied, the physical therapy and careful selection of candidates for the
condition lends itself to diverse interventions, some of which procedure, the functional outcomes over a period of 5 years
have a longer life than others. Management of spasticity has were lasting. Trost and colleagues159 reported on differ-
always been an area of great concern and interest, and over ences between preoperative and postoperative measures:
the years several treatments have been offered to control this the Ashworth scale for spasticity, the Gillette Gait Index,
positive sign. Various medications have been used to control oxygen cost for gait efficiency, and the Gillette Functional
spasticity; baclofen, diazepam, and dantrolene remain the Assessment Questionnaire for functional mobility. All out-
three most commonly used pharmacological agents in the come measures demonstrated improvement for the 136
treatment of spastic hypertonia.142 (See Chapter 36 for addi- subjects as a whole. Careful selection of the appropriate
tional information.) The baclofen pump has been used in candidate for this surgery followed by intense therapy inter-
children with excessive spasticity. This pump is implanted in vention is essential for the success of the procedure and for
the lower abdomen with a catheter leading to the intrathecal optimizing motor outcomes.
space for the administration of the drug. This treatment for A new approach to controlling spasticity is percutaneous
spasticity has been effective for some types of cerebral palsy radiofrequency lesions of dorsal root ganglion (RF-DRG), a
but led to complications in some patients with mixed cerebral noninvasive procedure that has been reported in the litera-
palsy, low body weight, younger age, gastrostomy tubes, and ture recently. Vles and colleagues160 performed a pilot study
nonambulatory status.143-145 of 17 patients with a diagnosis of cerebral palsy. They
The cerebellar implant so popular in the late 1970s reported that this new treatment is promising for reducing
offered the possibility of regulating tone by supplementing spasticity and improving function in children with cerebral
cerebellar inhibition.146-149 As time passed, the procedure palsy. Further investigation into this treatment is necessary
was used less often, and patients had difficulty getting re- to assess its effectiveness.
pairs or replacement parts for the implant. The procedure Alcohol (phenol) blocks and the use of botoxin (botulinum
that largely replaced the cerebellar implant was the place- toxin) (BtxA) have been used locally to affect a change in the
ment of four electrodes in the cervical area to offer more individual muscle or motor point injected.142,161-167 Both or-
control over postural tone.150 These had the advantage of thopedists and neurologists have taken an interest in the use
being adjustable so that the individual or a family member of botoxin to block selected muscle responses for a temporary
could make daily choices as to the optimal tone distribution. period. BtxA has been reported to have fewer side effects than
In some cases early success gave way to disappointment as the phenol blocks and is now considered the drug of choice
the system adapted to the inputs. In some cases the child or for this type of procedure.130 These conservative interventions
adolescent had to make a decision whether movement or serve to delay surgery until the child is more capable of
speech was more important on a given day. Therapy was responding to postsurgical therapy programs. Botulinum toxin
always recommended after the procedure, although the injection combined with serial casting has been shown to
nature of the specific program was left to the family to improve range of motion, muscle tone, and dynamic spastic-
decide. The success of the cerebellar stimulator is consid- ity in ambulatory children with cerebral palsy.135 Therapists
should be involved in the decision-making process of deter- EQUIPMENT

mining interventions for reduction of spasticity because they Equipment recommendations must take into account the
often are the most familiar with the child’s movement strate- physical space in the home and the amount of direct treat-
gies and the postural changes that occur in muscle tone as the ment available to the child (Figure 12-26). Young children in
child moves against gravity. particular can often use normal seating with slight adapta-
Orthopedic surgical intervention continues to be effec- tions. This not only is more socially and financially accept-
tive in cerebral palsy when there are tendon contractures or able but also permits changes as required by the child’s
specific structural limitations that are not accompanied by developmental progress. The portability of supportive seats or
excessive levels of spasticity.168,169 In any surgery the out- standers encourages the family to take the apparatus along
come is much improved by close coordination between for weekend outings or visits to relatives. Chair designs
therapist and surgeon, with a functional orientation toward should place children at an age-appropriate level in their
goal setting for the child. Early standing after surgery and environments. This permits a better quality of visual explo-
use of dynamic footplates inside the casts and orthotics af- ration and facilitates social exchange with siblings and visit-
ter cast removal will generally improve functional out- ing peers. When planning the amount of physical support
comes. Bony surgeries that offer better joint stability are needed by the child, the therapist considers varying the
usually planned for the termination of growth. The orthope- structural control in relation to activity (Figure 12-27). The
dist is also able to guide conservative positioning measures child who is merely watching the play of others or a televi-
to prevent hip problems resulting from spasticity while di- sion presentation may successfully control trunk and head
rect treatment intervention continues. Bracing of the trunk, balance independently with minimal support. However, con-
which is sometimes warranted for scoliosis or kyphosis, is centration on hand skills or self-feeding may necessitate
prescribed by the orthopedist. Surgical intervention for trunk control assistance via a chair insert to avoid the child’s
spinal deformities is determined by the physician, with use of compensatory reactions. As postural reactions be-
consideration of the child’s age, condition, and health and come more integrated and hence more automatic, support
the degree of curvature. should be diminished. There are now several options avail-
Children with cerebral palsy differ in their ability to able to the consumer on the internet. Families and therapists
relax completely during sleep, and a small number of these alike can search online for commercially available products
children can benefit from inhibitive casting or night brac- and alternatives to commercially available products.
ing. More often this type of positioning is used during For the more severely disabled child, equipment should
therapy sessions and independent ambulation to combine be easily and completely washable. Mothers should be able
control with weight bearing.170 The orthopedist should
participate in any plan for prolonged immobilization or
temporary casting that will be used on a 24-hour schedule,
such as serial casting to improve range of motion.171,172 A
variety of lower-extremity bracing is available, and its
selection is dependent on the segment to control and the
outcomes sought in positioning or dynamic action, as in
ambulation (Figure 12-25).
Figure 12-25 n A supportive shoe with footplates inside for this Figure 12-26 n An upright stander is easily incorporated into
low-tone child facilitates more typical trunk reactions and permits the home environment, providing the child with an upright posi-
use of the hands for play. tion, stimulation, and an opportunity to participate in activities.
Figure 12-27 n Use of a simple cut-out space in 3-inch foam gives this 1-year-old child security
while requiring more active trunk adaptation during play.
to place special seating inserts into wheelchairs or travel

chairs with one hand while holding the child. Wheelchairs
should be ordered with consideration for family needs and
the child’s environment. The most costly does not always
offer the best solution. Control straps and seating should
be adaptable and allow for future change on the basis of
growth and improved function. The severely limited child
needs seating changes at least once every hour during the
day to prevent pressure and provide environment changes
as in typical development. Pleasing color, good-quality
upholstery, and professional finishing are important not
only for the child but also for family members, who are
accepting the equipment as part of their personal living
environment.
As prices rise and the applicability of insurance changes,
the therapist must consider cost-effectiveness more carefully.
Parents are often desperate to do everything possible for the
child and tend to be very susceptible to high-powered adver-
tising and reassuring sales personnel. By providing a list of
essential equipment features, the therapist will aid the par-
ents in becoming informed consumers. Perusal of several
catalogs permits some comparison of quality and prices.
Adaptive equipment fairs are often open to parents and
therapists and are great opportunities to actually try the
equipment without the burden of the cost. Therapists can
forge a relationship with a representative of a medical supply
company and then access equipment on loan to try with their
patients for short periods of time. Once the appropriate
equipment has been purchased, periodic review of equipment
used by the child can serve to encourage the family to pass
along to someone else equipment items that are no longer Figure 12-28 n Balance equipment allows for increased com-
needed. Investment in expensive equipment also has the plexity in the therapy program with the refinement of balance
hidden effect of influencing both parent and therapist to reactions.
continue its use well beyond its effectiveness as a dynamic
supplement to treatment. For this reason more than any other,
large investments must be thoroughly researched with regard adjuncts to a home exercise program and should be consid-
to their long-term applicability for the child. ered, when appropriate, for a particular individual. Many of
Adaptive equipment is not the only type of equipment the commercially available items found at a sporting goods
that the therapist must consider for clients. Conditioning and store can be well adapted to fit the needs of the higher func-
strengthening, now recognized as beneficial to children and tioning person with cerebral palsy. With more emphasis
adults with cerebral palsy, open the door for equipment that on working out, sports equipment can even be found in dis-
lends itself to these parameters.107-114 Exercise bikes, tread- count stores. Offering to adapt equipment for your clients
mills (see Figure 12-22), light weights (see Figure 12-24), can encourage and increase compliance with exercises given
and balance equipment (Figure 12-28) are all valuable for home exercise programs.
ALTERNATIVE THERAPIES In the mid-1980s Pape186 published a case series involv-

Hyperbaric oxygen treatment is gaining popularity as a ing five children with cerebral palsy receiving therapeutic
treatment for cerebral palsy in children. Clinics are begin- (subthreshold) ES. Reported from this case series was that
ning to spring up worldwide for this type of therapy. overnight use of low-intensity ES in combination with stan-
Research on this treatment has been inconclusive, and scien- dard therapy demonstrated significant improvement in gross
tific evidence for its efficacy is lacking.173,174 Clinicians motor, balance, and locomotor skills as measured by the
should keep abreast of the latest developments in the treat- Peabody Developmental Motor Scales in children with mild
ment of cerebral palsy and inform families about the pros cerebral palsy.186 Results reported were based on observa-
and cons of designer treatments. Currently the Undersea tions by individuals who were not blinded to the purpose of
and Hyperbaric Medicine Society (UHM) states that there is the study, and therefore the research design was biased. In a
not enough evidence to recommend the use of hyperbaric follow-up study Steinbok and colleagues187 followed chil-
oxygen therapy for cerebral palsy.175,176 A systematic review dren who had undergone selected dorsal rhizotomy and re-
by McDonagh and colleagues177 found that there was no ceived therapeutic ES for 1 year. Although this study re-
significant difference between pressurized air versus room ported improvement in gross motor function, no other
air pressure and that the subjects in both groups made measures were changed: range of motion, spasticity, or
gains. This topic remains controversial even within the strength. Two well-controlled clinical trials investigating
UHM. Several articles and editorials have been written with this method of threshold electrical stimulation (TES) deliv-
the overwhelming conclusion that randomized clinical trials ery concluded that no objective effects on motor or ambula-
should be carried out to determine the effectiveness of this tory function or clinical benefit for children and youths with
treatment. cerebral palsy were detected.188,189 In two separate random-
The Adeli suit is a compression garment with bungee ized placebo-controlled clinical trials using TES, the results
cord–like elastic cords attached to the suit, along the trunk indicated no significant clinical benefit observed when com-
and extremities. This suit, once referred to as the therapeu- pared with neuromuscular ES.188,190 (Refer to Chapter 9 for
tic space suit or treatment-loading suit, was developed for additional discussion.) Parents often report changes with
Russian cosmonauts to counteract the adverse effect of this therapy, but the objective measures do not demonstrate
long-term zero gravity on the skeletal muscles.178 There are significant results. Clinicians in the field have reported that
a few studies that suggest that there are benefits for children overnight use of subthreshold ES builds muscle bulk, but
with cerebral palsy when they receive therapy in the unless combined with active participation of the muscula-
suit.175,178-181 The suit allows for controlled practice of ture, overall improvement in movement may not be seen.
activities with resistance, and assistance when necessary; This therapy, for children with cerebral palsy, requires a
the cords are attached in a variety of ways to give different special stimulation unit that is subthreshold and capable of
types of practice repetitions. As is prescribed by the propo- being used at night and has a shutoff setting that is activated
nents of this approach, intense practice is warranted to if the electrodes become disconnected from the child. The
reap the benefits. This therapy is expensive, and the suit, if unit is costly and further requires a clinician who is specially
bought to use at home, is also very expensive. Rosenbaum175 trained in its use to evaluate and periodically update the
has suggested that the benefits of this therapy vary with the treatment protocol. Significant benefits for this type of ES
type and severity of the involvement, the child’s ability to have not been documented.
participate in controlled movement, and the tolerance to The Peto method or “conductive education” (CE), devel-
wearing the suit for extended periods of time. In Australia oped by Andres Peto in Budapest after World War II, has
the Upsuit, described by Blair et al as being made of a been used in children with disabilities.191 This approach is
stretchy material, has been investigated for use with chil- an educational versus medical model, as the name implies,
dren with cerebral palsy.182 Similar results have been noted and the focus is on the many aspects of child develop-
by Chauvel et al that the type of cerebral palsy and the per- ment.192 It is based on practice and repetition combined with
son’s ability to participate in movement forecast the poten- verbal guidance of a trainer (conductor) and self-verbalization
tial outcomes.183 The Upsuit was also noted to compromise by the child as he or she performs the task or activity.175
lung capacity, which interfered with the child’s ability to Most of the claims that promote its value have come from
participate.182 As clinicians, it is important for us to stay the Peto Institute in Budapest, Hungary. The institute has
abreast of the latest developments and to provide families been very selective in choosing candidates for this approach
with reasonable alternatives. Although intense practice is on the basis of the child’s potential for independent mobil-
well known in the literature as being beneficial for motor ity and function with a good overall prognosis.175,191,193
learning, the addition of the suit brings in a component that Research into this method of delivery of services to children
“appears” to provide stability and graded control during with cerebral palsy has been sparse outside the Peto
practice. Caution when describing this approach to families Institute’s studies. In two studies in Australia, CE was found
is warranted because some evidence suggests that it may to have encouraging results in developmental changes.194,195
not be appropriate for every child with cerebral palsy. The In a randomized controlled study with children assigned
long-term effects and measurable outcomes after the use of to a CE group or a neurodevelopmental intervention group,
the Adeli suit and the Upsuit are not documented in the no significant differences were noted in the two groups.198
literature.175,184,185 The following questions must be asked: This approach advocates intense practice of all skills, motor
What happens to the motor abilities of the child when the and educational, with emphasis on self-doing regardless of
suit is removed? Has the suit actually prevented the devel- whether a compensatory pattern is used. Various pieces of
opment of postural antigravity coactivation of the trunk and equipment are used to facilitate independent skills: wooden
proximal axial joints? slatted beds to allow the child to pull and maneuver, and
wooden ladders mounted on the wall to assist with dressing systems and central nervous system processing will influence
and transitions. The literature on CE does stress the concept the choice of treatment techniques. Direct intervention will
of intense practice for motor learning. Similarly, promoting have more depth and specificity to improve the child’s control
practice in the CE environment must transfer to home and of posture and movement while the therapist appreciates the
community for carryover to everyday life situations.63 To complex interaction of developmental factors in cerebral
fully optimize the outcomes of CE, a commitment from palsy. On the basis of individual experience, each therapist
the family is essential, as is the case in most therapeutic develops a personal philosophy of treatment that incorporates
approaches. Further investigation into this approach is new research findings and evolving perceptions of the prob-
warranted before this philosophy is fully supported. For a lem of central nervous system dysfunction. Without a philo-
selected group of individuals, CE research results seem sophical or theoretical orientation for decision making, the
hopeful, although transfer to unpracticed tasks and different therapist may succumb to following each promising treatment
environments may be minimal.197-199 idea that is learned without having a clear image of the poten-
The Feldenkrais method was developed by an Israeli tial benefits for the specific client. “Commercial” programs
engineer, Moshe Feldenkrais, while looking for a solution may benefit the child whose needs match the program objec-
to his own knee problem. (Refer to Chapter 39 for additional tives. An “individualized” program adapts to the needs of the
discussion.) He started analyzing body alignment for more particular child and is shaped by the response of that child
efficient movement.200,201 This form of body work has been during therapy. Without an internalized treatment goal toward
used to help dancers, gymnasts, and other skilled persons which independent techniques are applied, the result may re-
improve their performance. Some therapists have under- main ineffective and unconvincing. The therapist in a direct
taken the long training necessary to understand typical treatment situation must develop a concise visualization of
movement in more detail and to improve the movement what is to be achieved in each session with the individual
coordination of their clients with neuromotor challenges. child based on a sound foundation. The repetition and practice
There is no published research on this method, but several that are so critical for learning must often be carried out at
books and training courses exist that promote its use. In a home, and the therapist becomes responsible for family in-
review article by Liptak184 on alternative interventions, the struction. Home exercise programs must be tailored for both
Feldenkrais method is described; however, no articles have the child and the family situation and must be in alignment
been published examining its use with children who have with the goals and expectations of the family. These programs
cerebral palsy. need to be practical, fun ideas for practice that can be incor-
Ida Rolf, trained in physics, had a son with some postural porated into the child’s home life with reasonable assurance
disorganization.202 She developed a structural approach, that the activities can and will be carried out regularly. Cre-
called the Rolfing technique, to improve body alignment; the ative therapeutic ideas for playtime, dressing, grooming,
technique uses specific release of deep soft tissue to restore mealtime, and relaxation time are best addressed in the home
effortless postural control against gravity.203 She was able to exercise program because these are everyday tasks that every
make positive changes in the movement patterns of many family encounters and the family is likely to be compliant.208
children with cerebral palsy, but she never claimed to treat the The time spent in therapy throughout the week cannot substi-
disorder itself. This approach requires special training in the tute for all the hours spent at home and school.
Rolfing technique, which is a type of deep tissue massage. Specialized therapy, like typical development, is potentially
Dr. William Sutherland,204 an osteopathic physician, a preparation for functional performance. Training in specific
developed direct treatment of the cranium, which is referred coordination skills may be necessary for the older child or
to commercially as cranial therapy or cranial sacral treat- adolescent and must begin with a thorough analysis of the
ment. (Refer to Chapter 39 for additional information.) This whole person who happens to demonstrate the effects of cere-
type of therapy is believed to have wide application to many bral palsy. Some children have learned self-care along with
disabilities and conditions.205 Today, there are persons trained brothers and sisters. Others have needed therapy guidance for
at many different levels, so the family of a child with cerebral each achievement. Intelligent children with strong motivation
palsy seeking this treatment will need to be certain that the may only need some assistance in avoiding use of atypical
practitioner is a professional and that she or he has experience reactions, whereas others have poor spatial orientation and
with small children.206 Cranial treatment is purported to re- minimal motivation to achieve independence. The therapist
store the physiological motion of the craniosacral system, most often needs to create a dialog with the individual who has
improving circulation of fluids to the brain as well as respira- the problem because parents are often fatigued and without
tory function, to which it is believed to be closely linked. energy to solve the issue of adolescent life skills. Perseverance
Research has been encouraged in this area to substantiate the is key to success with these individuals.
claims of this approach.
INVOLVING THE FAMILY
DEVELOPING A PERSONAL PHILOSOPHY To be successful, therapy for the child with cerebral palsy
OF TREATMENT includes active family participation. Variability of practice in
The practicing therapist continues to learn much about the different environments tends to promote more effective motor
nuances of typical human development (see Chapter 3).207 learning, and parents who learn to help their child early begin
The dynamic interaction of developmental movement compo- to understand the importance of their participation as well
nents becomes more significant as the therapist acquires as the nature of their child’s disability. Parents are in the pro-
greater clinical experience and recognizes developmental cess of healing their own self-image, which was so injured
change as a reflection of central nervous system maturation. when they learned of their child’s disability. They should not
Increasing knowledge of the functional nature of sensory be expected to become therapists per se but should learn to
observe small gains in treatment sessions that offer insight factors, poor or lack of insurance coverage, and lack of
into the child’s current strengths and weaknesses.17 qualified therapists may interfere with the delivery of direct
Parents need to adapt their expectations in keeping service. The therapist must then assume the role of teacher,
with the child’s continuing change and emotional maturity. counselor, or consultant. More often the new role emerges
Parenting a child with cerebral palsy is no easy task, and the as one in which the therapist tries to meet a combination
therapist will do well to develop respect for this demanding of needs and is frequently frustrated by lack of time, energy,
role. No one provides more for the child with cerebral palsy and community resources. The therapist may be a member
than the nurturing parent who guides the child to self-accep- of a community team that includes a psychologist, a social
tance of limitations without destroying personal initiative. worker, and a public health nurse. This sometimes creates
This is the child who most often becomes an independent more of a behavioral than a traditional medical orientation.
working adult (Figure 12-29). Therapists can also be primarily responsible to the public
The therapist must give serious thought to priorities in school systems, introducing therapeutic positioning to class-
home recommendations. Therapists must consider the size room teachers. For these types of situations the clinician will
of the family and whether there are siblings, outside em- find videotape a valuable adjunct to direct instruction. The
ployment of the mother and father, physical capabilities of individual child may be filmed with equipment, adequate
the child, general health status of the child, and psychologi- positions, or therapeutic procedures. Useful topic-oriented
cal acceptance of the problem within the family. The emo- videotapes are also available for professionals and families.
tional needs of some parents demand a period of less, rather Instruction of key personnel in these settings is critical to the
than more, direct involvement with the child. Other parents success of the therapist’s recommendations.
must be cautioned that repetition of an activity more times When children have no access to direct treatment, posi-
than recommended will not result in faster improvement. tioning is of paramount importance. The selected support is
This impression is sometimes gained from wide advertising used to avoid contractures, scoliosis, and permanent limita-
of commercial programs that offer the same activity se- tions in range of movement. Even the most severely limited
quence for every child and demand a large number of daily child should have a minimum of three positions that can be
repetitions. Both parent and therapist must appreciate the alternated during the day. In addition, the position selected
need for the central nervous system to have some time to should be as functional as possible for the individual child
integrate new sensorimotor experiences and to perfect to allow access to the child’s environment. In some cases
emerging control of postural adjustments. Excessive control this may mean encouraging eye contact. For another child,
of movement patterns and overprotection by an adult tends hand use becomes a possibility with proper trunk support.
to reduce the child’s initiation of postural change and Each program should be individualized to maximize poten-
decrease active sensorimotor learning. Health needs for tial for the child in that environment.
good nutrition and adequate rest must also be considered by Communication for the nonverbal client with cerebral
parents and professionals. The attitude of teachers in the first palsy must be an integral part of the therapy or school pro-
years is extremely important for the child with cerebral gram.209 A simple start may be made with pictures to per-
palsy. Advocating for a positive environment across the set- mit choices in food, clothing, and therapy activities. The
tings is advantageous for the child’s achievement. parents need encouragement to begin the process of letting
the child make some simple choices in food, clothing, or
ROLE OF THE THERAPIST IN INDIRECT preferred activities. Although computers have their place,
INTERVENTION the child should have the communication device with him
For many children with cerebral palsy, active treatment or her at all times. Language development in the young
is not available. Geographical isolation, socioeconomic child is enhanced by having this type of alternative com-
munication device available while articulation is still dif-
ficult. Use of head movement is a powerful influence on
muscle tone changes that may cause negative regression in
postural or visual control. Care and consideration should
be taken when evaluating the body part to access the de-
vice. Postural and visual control is essential toward the
goal of better function and communication. A solution that
was successful with one 9-year-old athetoid girl was mov-
ing the elbow back to a switch mounted on the vertical bar
of the wheelchair backrest to access her communication
device. Any activity that is repeated on a daily basis should
be examined in light of possible interference by atypical
patterns.
Affordable electronic systems with voice recording, por-
tability, and growth features are available. Communication,
which can be achieved by coordinating efforts with the
speech pathologist, can make the difference between pas-
sivity and active participation in the environment.209 Play
Figure 12-29 n Therapy goals must incorporate functional can be encouraged with the use of switch toys and touch-
activities that lead to personal independence if they are to be screen computers. Many new programs are being developed
pertinent for the older child and adolescent. for computers and electronic interactive books.
THERAPY IN THE COMMUNITY activities after mobilizing tissues that have been unused for
Therapists are often concerned with body functions and so many years (see Chapters 4 and 9). With current program
structure and neglect to address participation in real-life ac- directions, many older clients may not have had the oppor-
tivities such as school attendance, sports, employment, and tunity for direct treatment over time by a qualified therapist.
involvement in the community.210 Children with mild dys- For the minimally involved teenager, young adult, and adult
function as a result of cerebral palsy may be successfully with cerebral palsy the local gym offers an alternative to
incorporated into physical education (PE) classes if the direct therapy. General conditioning is very popular in com-
teacher is prepared to make some small adaptations. Teachers munity programs including weight training, endurance
generally appreciate the opportunity to discuss with the training, water exercises, yoga, and walking programs. A
therapist specific limitations of the child and those move- therapist can be consulted to establish an appropriate pro-
ments that should be encouraged. Taking the opportunity to gram for the individual who wants to work out at the gym
meet with the PE coach to establish adaptations and modifi- on equipment, attend special classes that are offered, or
cations or appropriate participation in activities is time well work out in the pool. When asked, it is the therapist’s area
spent for the child’s integration into the class. PE class is of expertise to help identify and make recommendations
often rewarding for the child and important in establishing regarding functional movement and activity participation for
peer relationships. For better success the child with func- adults with developmental disabilities such as cerebral palsy.
tional limitations can be incorporated into a class that follows (See the section in Chapter 35 on adults with developmental
the British form of movement education, which places much disabilities.) The movement toward a health orientation as
less emphasis on intragroup competition and encourages opposed to crisis intervention for illness will also affect
each child to progress at her or his own rate. services for children and adults with cerebral palsy. This
Classroom teachers who lack experience with children population does not have an illness or an active disease pro-
who have special needs are understandably reluctant to cess, and they strive to lead as normal a life as possible.
incorporate a child with movement limitations into the Many adults with neuromotor disabilities express their pref-
classroom until they know the child. A meeting with the erence to participate in the decisions that are made for them
therapist might be used to help the child demonstrate his or regarding their ultimate lifestyle and participation in the
her strengths, physical independence, and ability to par- community. The therapist who works with this population
ticipate in classroom activities. The child may often play should familiarize himself or herself with the patient’s living
an active role in the problem-solving process necessary for situation—family home, group home, or independent living—
a successful classroom experience. Children often have as well as the support system and work environment if
developed their own ways of managing the water fountain, applicable. These factors should be considered when estab-
the locker door, or personal care needs. Demonstrating lishing a viable program. Many opportunities for employment
these abilities reinforces strengths rather than limitations and volunteerism exist in communities for individuals with
and empowers the child to receive positive responses from disabilities. This may require that the therapist venture into
curious peers. the workplace to assess accessibility and modifications that
As programs that hire therapists move into the fields of can be done in that environment to make for successful inte-
prevention and early intervention, the therapist is dealing gration of the individual into the community. Optimal health
directly with a population that is not familiar with therapy for the adult with cerebral palsy has yet to be described, and
per se nor aware of the need for this intervention. The thera- much more data must be collected. However, it is an excit-
pist may discover a need to reorient previously accepted ing time as our society moves forward in its views and
concepts of general rehabilitation. Clarification of one’s own acknowledgment of disability (see Chapter 35).
ideas is essential to establish effective communication with
others. In some instances, active intervention to help the PSYCHOSOCIAL FACTORS IN CEREBRAL
child will precede the labeling or diagnostic process, and PALSY
referral to other specialists becomes part of the therapist’s We have defined cerebral palsy as a condition existing
responsibility. Philosophically, early therapy becomes an from the time of birth or infancy. The developing child has
enhancement of typical development rather than a remedial no memory of life in a different body. Movement limita-
process, and it is advocated in the natural environment by tions circumscribe the horizon of the child’s world unless
federal funding agencies for children 0 to 3 years of age. the family is able to provide enriching experiences. The
This implies introducing new concepts of quality in early development of both intelligence and personality relies
child development to the public. Day care programs are an heavily on developmental experiences and the opportunity
example of new early childhood settings that incorporate for self-expression.
children with impairments. The child with spastic diplegia or spastic quadriplegia
It is important to keep direct, active treatment available may be hesitant in making decisions or reaching out for a
for older children, adolescents, and adults who are moti- new opportunity because the world may seem overwhelm-
vated to change. Now that more effective procedures are ing and threatening. The child may find it easier to withdraw
available for changing some of the basic neurophysiological toward social isolation. Parents and professionals can help
movement characteristics observed in children with cerebral children, adolescents, and adults with cerebral palsy avoid
palsy, it is possible to achieve change with direct treatment these reactions by encouraging independence in thought and
of the older client. The adolescent often responds best to in physical tasks. Early choices can be made by the child
short-term, goal-oriented therapy programs that are patient regarding which clothes to wear or which task to do first.
centered. Motor learning concepts are better understood by Understanding the child’s limitations helps build successes
both the therapist and the client and can be incorporated in rather than failures. To function in spite of the constraints of
spasticity or other movement problems demands consider- for free play. Continuous demands are placed on children to
able effort on the part of the child. prove their intellectual potential in evaluations of various
Athetoid children, in contrast, have adapted to failures as types. Adults most often monitor their social interaction
a transient part of life. However disorganized their move- while they assume a dependent role. Nonetheless, these
ments, they repeatedly attempt tasks and eventually succeed. children’s social acceptance frequently rests on their skill in
Their social interactions reflect this life experience. Most interacting with persons in their environments. It is not fair
people will sooner or later succumb to the positive smiling to the child to evaluate the evolution of personality without
approach without analyzing the deeper communication considering these experiential factors.
offered by the child. These children are difficult for parents
to discipline and structure during their early years. Early DOCUMENTATION
treatment with concomitant guidance for young parents Developing a plan of care (POC) with objective measurable
ameliorates some of the problems by making the develop- goals is required for documentation of progress in intervention
mental expectations for the child more appropriate. The and reimbursement from third-party payers. (Refer to Chapter
words of professionals who are in contact with the parents 10 for additional recommendations.) Carefully extracting a
at the time of the diagnosis echo through time to influence child’s strengths and weaknesses from the assessment should
future decision making for the child. drive the POC. Using timed measures, distance, number of
Intelligent children with low tone demand that the world repetitions, standardized tools, and other measurable outcomes
be brought to them. Mentally limited children may fail to provides a source of encouragement for families and justifica-
receive sufficient stimulation for optimal development at tion for continued intervention.
their functional levels. Many of these children need visual Data collection is an important task in the treatment
or auditory evaluations and intervention, and some of them of cerebral palsy. Change occurs at variable rates, but it
need a special educational approach. Whatever the learning is important to document the cause and effect of change
potential of the child with cerebral palsy, it is not always whenever possible. Slides or videotapes are useful in
evident early in the child’s life. Parents find it difficult to recording functional comparisons over time. Digital video
know how to guide a child when they are not certain that now allows a specific analysis of movement sequences. A
an assigned task or calm explanation is understood by the motor drive unit or automatic advance on a 35-mm single-
child. lens reflex camera can record a sample of movement five
Parental guidance of the child with functional limitations or more times per second. Placing the subject against a
is also influenced by the adults’ adaptation to their offspring’s spaced grid in a specific alignment to perform a movement
problem. Parents need to resolve in their own way the emo- task allows for measurement of efficiency of movement.
tional impact of the child’s disability. Parents need time to These ideas may be applied to documentation of treatment
grieve the loss of dreams they had for their child, and each effectiveness or analyzed for an understanding of similar
person will approach this in his or her own way and time. movement problems in other clients. When attempting to
Each major milestone anticipated in a typical child’s life may document using photographs or filming, consistency in
bring on the grieving process again. Most parents feel inad- the environment is critical to the outcome and analysis.
equate, ignorant, and relatively helpless at being unable to Reliable comparisons made between one time point and
remedy the situation for their child. They need help in feeling another require the same testing environment, time of day,
good about themselves before they can effectively guide the and conditions.
child toward self-acceptance as an adequate human being. Methods of intervention or treatment are measurable for
Parents need guidance to provide themselves with opportuni- research and applicable to the functional problems presented
ties to rest and renew their energies. Therapists can be instru- by a diagnosis of cerebral palsy. Once a specific research
mental during this process by remaining nonjudgmental. question has been formulated, systematic recordings of
(Refer to Chapter 6 for additional information.) appropriate data can be gathered over time to accumulate
The therapist plays an important role in the psychosocial data for a viable study. There is value in longitudinal report-
development of children who receive regular treatment. The ing of a single case or a small group of individuals who have
child may perceive the therapist as a confidant, disciplinar- some characteristics in common because this aids our under-
ian, counselor, or friend at various stages of development. standing of what we need to prevent in the young child to
Some children accept the therapist as a member of their permit optimal function later. (See Chapters 8 and 10 for
extended family. This is natural, considering the extent to suggestions of impairment and disability measurements to
which therapists influence clients’ own self-awareness be used as objective measures for functional outcome stud-
through changes in their physical bodies. However, it also ies and record keeping.) Clinicians have a difficult time
places a personal responsibility on the therapist to be aware putting into words exactly what takes place during interven-
of the continuing interaction and its effect on the maturation, which further complicates research investigation into
tional process of the child. Long-term relationships with the efficacy of treatment. Descriptive analysis of treatment
patients and their families must remain professional for the is essential to document and begin to understand what a
therapist to be effective. therapist does during a therapy session. Understanding what
Any evaluation of personality characteristics in a dis- takes place in therapy will help identify questions that could
abled child must take into account the unnatural lifestyle be investigated more closely.
that is imposed by the need for therapy, medical appoint- The way in which therapists learn to view a problem
ments, limited environmental exploration, and hospitaliza- determines, to a large extent, the potential range of solutions
tion. The child is expected to separate from parents earlier available to them. Cerebral palsy is a complex of motor
than the average child and usually confronts many more and movement inabilities that cluster about the inadequacy
novel situations. There is little time or physical opportunity of central nervous system control, visual and soft tissue
restrictions, and the amazing ability of the human body to CASE STUDIES
compensate. Therapists need to look critically at develop- To understand the problems of children with cerebral palsy,
mental processes, qualities of movement, postural adjust- it is essential to follow some children over time to capture
ments, timing and limitations of movements, and the range the evolution of family problems. Functional treatment must
of dynamic functional movement. New areas of motor learn- change according to the developmental level, chronological
ing and systems and chaos theories offer the researcher age, and neuromotor responses of the child. Intervention
novel approaches to the challenge of cerebral palsy and the must be specific to the presenting problem of the moment
resultant disorder of posture control and movement learning. while the missing aspects of complete motor development
Environmental factors may have as much influence as spe- are considered. The case study comparison of two boys
cific central nervous system limitations. Early intervention illustrates the typical lack of clinical correlation between
should be analytical, specific, and based on a theoretical history and manifested characteristics of cerebral palsy.
foundation. Posture and movement control begins to change
with direct treatment. Analysis of the postural components References
and movement characteristics of children with cerebral To enhance this text and add value for the reader, all refer-
palsy will lead to meaningful research more quickly than ences are included on the companion Evolve site that accom-
will professional reliance on the traditional definitions of panies this textbook. This online service will, when avail-
the medical condition. Thorough documentation of therapy able, provide a link for the reader to a Medline abstract for
progress using objective measures is critical for develop- the article cited. There are 210 cited references and other
ment of more effective intervention strategies in the future general references for this chapter, with the majority of those
(see Chapter 10). articles being evidence-based citations.
CASE STUDY 12-1

A young woman was pregnant with her first child. She was At 3 years of age L. P. continued to have difficulty with
middle class, was well nourished, and had no identified risk control of her head position in space and was unable to initiate
factors. In the seventh month of pregnancy, her older sister died, postural changes with her head. The clinical picture was one
causing considerable emotional upheaval. The much-anticipated of low tone with athetoid movement. She could not speak,
infant was born, small for gestational age, at the correct date. communicated with looks and a few word approximations,
It was theorized that there had been inadequate intrauterine and hitched along the floor in a seated position with one
nourishment during the first 6 to 7 months. The child, L. P., supporting hand.
weighed 3 pounds, 8 ounces (1587 g) and was fed initially by By 5 years of age L. P. was still receiving therapy three
nasogastric tube. Her movements were quick, and eye move- times per week and could walk in a hesitating way with her
ment was very active for a newborn infant. She was seen for hands held. At this stage she was evaluated by a behavioral
therapy at 17 days of age, immediately after hospital discharge. optometrist and started vision therapy to prepare her to partici-
The initial therapy focus was on the practical task of pate in preschool activities. As a secondary benefit her balance
adequate nutritional intake so that the nasogastric tube could in walking improved markedly. L. P. began walking up and
be removed before scarring occurred from repeated passage of down 27 steps daily in her new home.
the tube. Swaddling was suggested to calm the infant and assist Now, at 7 years of age, L. P. can walk independently on
her organization of body movement. Simple handling was level surfaces. She has physical therapy once a week and vision
oriented toward moving the trunk over midline to let the head therapy once a week to maintain her control of posture and
follow and assisting the infant to assume age-appropriate movement. Her school performance is adequate to keep her
antigravity postures. with her age peers and she attends a regular school.
CASE STUDY 12-2

D. D. and E. D. were born within 6 months of each other at with prism lenses. At age 4 years D. D. was discovered to have
6 months 1 week of gestation. Both were first-born infants for a mild to moderate hearing loss; he still uses a hearing aid in
their respective mothers. D. D.’s mother was discovered to have a one ear. He speaks English and Spanish, as do his parents, and
double uterus when she had a miscarriage early in the pregnancy. he understands the French spoken to him by his grandparents.
E. D. had malnutrition during his intrauterine development. D. D. He functions in a regular school with his age peers and is a
started therapy just before he was 5 months old, and E. D. began well-adapted, active child.
therapy at almost 7 months old. At 6 years of age E. D. has moderately severe diplegia, with
At 6 years of age D. D. walks alone with very mild athe- some immaturity of hand use and trunk control. He speaks
totic “overflow.” He wears corrective lenses that were fit at English and Spanish well, although he demonstrates some emo-
2 years of age, and he returns for follow-up examinations with tional instability and difficulty in dealing with his disability. He
the behavioral optometrist once a year. Vision therapy was an is creative in storytelling and offers to tell original stories for
important adjunct to physical handling because it introduced other children in therapy. He is just beginning to walk with a
changes in spatial perception on the basis of specific sessions walker within interior environments and with low resistance.
CHAPTER 13 Genetic Disorders: A Pediatric Perspective
SANDRA G. BELLAMY, PT, MS, DPT, PCS, and EUNICE YU CHIU SHEN, PT, PhD, DPT, PCS

evaluation After reading this chapter the student or therapist will be able to:
functional skills 1. Describe the main types of genetic disorders and give examples of each type.
genetic disorders 2. Differentiate between genetic disorders diagnosed with clinical versus laboratory methods.
natural environments 3. Describe three modes of inheritance for single-gene disorders.
occupational therapist 4. Recognize key impairments that are common to many genetic conditions in pediatric
physical therapist clients.
5. Explain the physical or occupational therapist’s role in the recognition, referral, and
multidisciplinary management of genetic conditions in pediatric clients.
6. Identify resources and strategies for accessing information and increasing knowledge
about genetic disorders for use in clinical decision making.
7. Explain why it is important to include family members in the planning and development
of therapy programs for children with genetic disorders.
8. Describe and give examples of three types of assessment tools, and state the intended
purpose of each.
9. Describe the importance of developing therapy programs for children that are outcome
focused on functional skills in natural environments.
G enetic disorders in children can result in a wide variety of

movement impairments and disabilities. The resultant
impact of certain genetic conditions on the child may be evi-
be extensive, prolonged, and often inconclusive; therefore
pediatricians may refer children to occupational and physical
therapy before the nature of their condition is fully known.2,3
dent before or immediately after birth, whereas other condi- Although sometimes far removed from the hospitals and
tions may not be diagnosed until later in life when problems specialized centers that perform genetic testing and diagno-
manifest. In this chapter we discuss disorders of known ge- sis, the pediatric therapist is often able to contribute clinical
netic origin that physical and occupational therapists are evidence that will assist the diagnostic process.4-6 Further-
most likely to encounter in therapy programs for children. more, many genetic diseases and syndromes are increasingly
The Human Genome Project, completed in 2003,1 ex- survivable into adulthood; thus it is vital that physical and
panded knowledge about the genetic basis for disease and occupational therapists achieve competence in genetics and
congenital malformations. The impact of this project is just genomics in order to deliver care throughout the patient’s life
being realized, with new research into diagnostic techniques span.6,7 An overview of the general categories and subtypes
and treatment options for genetic disorders. Pediatric health of genetic disorders is presented first. Specific examples of
care professionals will be faced with questions from families each type are given, along with a brief description of key
who, in seeking diagnostic and prognostic information, are diagnostic features and issues commonly addressed with
accessing the wealth of information both in the lay scientific medical and therapeutic intervention. A summary of impair-
press and on the World Wide Web (Box 13-1 and Table 13-1). ments common to many pediatric genetic disorders is pre-
An accurate diagnosis of a specific genetic disorder sented in the second section. The third section includes a
(syndrome or disease) is necessary for a prognosis to be pro- discussion of the medical management of genetic disorders,
vided, for eligibility for therapy and education services to genetic counseling, and the ethical implications of genetic
be determined, and as a basis for genetic counseling for the screening and testing. The final section focuses on the physi-
child’s family.2 The diagnostic process for genetic disorders cal or occupational therapist’s role in the clinical manage-
includes a combination of clinical assessments by the physi- ment of children with genetic disorders. The therapist’s role
cian who collects the child’s medical history and a clinical and responsibilities in developing competence in recogni-
geneticist who may construct a family history or “pedigree” tion, referral, and clinical practice when working with
to recognize disorders with familiar inheritance patterns. patients and families affected by a genetic disorder are
Molecular studies may confirm a clinical diagnosis, differen- discussed. Evaluation procedures, treatment goals and objec-
tiate between diagnoses with similar clinical presentation, tives, and general treatment principles and strategies are
and identify the genetic cause of the disorder. Some genetic discussed from a family-centered perspective. A list of edu-
disorders are not easily identified, and laboratory testing can cational resources for clinicians and families is provided.
345
BOX 13-1 n RESOURCES

ON TOPICS IN AN OVERVIEW: CLINICAL DIAGNOSIS
GENETICS FOR CLINICIANS AND FAMILIES AND TYPES OF GENETIC DISORDERS WITH
REPRESENTATIVE CLINICAL EXAMPLES
TEXTBOOK Genetic disorders are typically divided into four categories:
Jorde LB, Carey JC, Bamshad MC, et al: Medical genetics, chromosomal, single-gene, mitochondrial, and multi-
ed 2, St Louis, 2000, Mosby. factorial. Chromosomal disorders arise when there is an
Review for clinicians of basic genetic science; helpful alteration in either the number or structure of chromosomes
illustrations. that exist in either autosomal or sex (X, Y) chromosomes.8
WORLD WIDE WEB Numerical or large structural chromosomal abnormalities
1. Understanding Genetics can be seen through a microscope; therefore a sample of
http://geneticalliance.org/sites/default/files/ksc_assets/ the patient’s peripheral blood can be used in detection of
pdfs/manual_all2.pdf disorders such as Down syndrome. When there is a suspi-
Free pdf-format primer on basics of human genetics for cion of a clinical spectrum associated with some of
health professionals and families. the known chromosomal microdeletions, translocations, or
2. Human Genome Project Information inversions, direct deoxyribonucleic acid (DNA) analysis
www.ornl.gov/sci/techresources/Human_Genome/ techniques such as fluorescence in situ hybridization (FISH)
home.shtml with use of specific sequence DNA probes can confirm a
Links to research; teacher and student tools; gene testing; specific suspected diagnosis. Indirect DNA analysis tech-
summary of current scientific knowledge in genomics; niques such as linkage analysis can be performed to confirm
and fundamentals of genetics including interactive single-gene disorders when the gene or genomic region
map of each human chromosome and associated disassociated with the disorder is unknown.9
eases and disorders. Of our 20,000 to 25,000 protein-coding genes,1 a single
3. Genetics Fact Sheets, Centre for Genetics Education gene may be responsible for approximately 6000 known
www.genetics.com/au/pdf/factsheets/fs01.pdf genetic traits. Approximately 4000 of these known traits are
Consumer-friendly pdf-format modules on basic genetics diseases or disorders.10 Single-gene disorders may be trans-
with simple illustrations for difficult-to-understand mitted through three different patterns: autosomal dominant,
scientific concepts. autosomal recessive, and sex linked. Dominant refers to the
4. Genetics Home Reference, National Library of Medicine case in which a mutated gene from one parent is sufficient to
(Bethesda, Md), 1993-2008 produce the disorder in offspring. Recessive refers to the case
http://ghr.nlm.nih.gov in which the disorder will not be expressed unless offspring
Consumer-friendly genetics glossary and encyclopedia inherited a mutated copy of that gene from both parents. It is
of genetic conditions. incorrect to say that a gene is recessive or dominant; rather
5. Family Village the trait, or disorder, is dominant or recessive.7
www.familyvillage.wisc.edu Inheritance is usually a term reserved for the transmis-
Website for children and adults with disabilities and sion of a previously recognized family trait to subsequent
their families, friends, and allies. offspring. However, many genetic disorders arise from new,
Covers various diagnoses, assistive technology, legal spontaneous mutations in a gamete, the single egg cell from
rights and legislation, special education, and leisure the mother or a sperm cell from the father. The remainder of
activities. the gametes from either parent are most likely normal. In
6. Making Sense of Your Genes: A Guide to Genetic this case their offspring will be the first in the family to
Counseling display the sporadic disorder, and the faulty gene can then
www.nsgc.org/client_files/GuidetoGeneticCounseling.pdf be passed onto subsequent generations. A disorder that
7. American College of Medical Genetics results from a single copy of a mutated gene is referred to
www.acmg.net as a dominant disorder, even if it is acquired by a spontane-
Information about educational courses in genetics for ous mutation. Not all literature sources will include sponta-
health care professionals. neous mutations in the description of inherited disorders.
8. Genetics and Public Policy Center It is important to understand how a disorder was
www.dnapolicy.org acquired, because the relative risks to other offspring for
Information on currently available genetics testing and the disorder vary according to mode of transmission. For
family planning. example, the risk of having another child with the same
9. National Newborn Screening and Genetics Resource genetic disorder that occurred as a result of a spontaneous
Center mutation is low. However, when one parent is affected by an
http://genes-r-us.uthscsa.edu/resources/newborn/ inherited dominant mutation, the risk of passing that faulty
00chapters.html gene onto each child is 50%.8
Lists what genetic screening tests are being performed in Most congenital malformations and many serious dis-
the 50 U.S. states. Gives definitions of various genetic eases that have an onset in childhood or adulthood are not
disorders. caused by single genes or chromosomal defects; these are
called multifactorial disorders.7,8
Mitochondrial disorders are caused by alterations in ma-
ternally inherited cytoplasmic mitochondrial DNA (mtDNA).
The clinical manifestations of mtDNA-related disorders are
extremely variable,11 and the occurrence is reportedly rare
CHAPTER 13 n Genetic Disorders: A Pediatric Perspective 347
TABLE 13-1 n ASSISTIVE TECHNOLOGY
RESOURCE WEBSITE INFORMATION

AbleData www.abledata.com Information about assistive technology (AT) products
and rehabilitation equipment
AccessIT www.washington.edu/accessit National Center on Accessible Information Technology
in Education
Alliance for Technology Access www.ataccess.org Public education, information, referral; network of
technology resources
Assistive Technology Industry www.atia.org/i4a/pages/index.cfm Information on products and services
Association
Assistive Technology Partners www.assistivetechnologypartners.com Information to assist persons with cognitive, sensory,
and/or physical disabilities
Assistive Technology Training http://atto.buffalo.edu AT applications that help students with disabilities
Online Project learn in elementary classrooms
Family Center on Technology www.fctd.info Provides guide to AT and transition planning
and Disability
National Public Website on Assistive www.assistivetech.net Features products by related functional area
Technology or disability, by activity, and by vendor
Protection and Advocacy for Assistive www.workworld.org/wwwebhelp/ Provides protection and advocacy services to help
Technology Program protection_and_advocacy_systems individuals with disabilities of all ages acquire, use,
_overview.htm and maintain AT services or devices; website identifies
each state’s program
Rehabtool.com www.rehabtool.com Information on AT products by categories
National Institute of Standards and http://standards.gov/standards_ Authoritative information and guidance on measurement
Technology (Standards.gov) gov/index.cfm and standards for all industry sectors
(5.0 per 100,000)8,12; however, collectively as a group of congenital anomalies, intellectual disability, or phenotypical
neuromuscular disorders, they account for substantial use changes that manifest later in life.8 Of the fetuses with
of health care resources.12 abnormal chromosomes that survive to term, about half
Currently there are over 1000 genetic tests available in have sex chromosome abnormalities and the other half have
the United States.1 Specific DNA testing may soon be able autosomal trisomies.8
to identify nearly all human genetic disorders. This not only The following section provides a brief overview of com-
allows for accurate and more complete diagnosis but should mon genetic disorders seen by physical and occupational
pave the way for the development of mechanisms for treat- therapists working with children.
ment, cure, and prevention of certain genetic conditions.4,5,8,9
Table 13-2 lists examples of specific disorders in categories Autosomal Trisomies
of the most common pattern of inheritance by which each Trisomy is the condition of a single extranuclear chromo-
occurs. some. Trisomies occur frequently among live births, usually
as a result of the failure of the parental chromosomes to
Chromosomal Disorders disjoin normally during meiosis. Trisomy can occur in auto-
Cytogenics is the study of chromosomal abnormalities. A somal or sex cells. Trisomies 21, 18, and 13 are the most
karyotype is prepared that displays the 46 chromosomes—22 frequently occurring trisomies; however, few children with
pairs of autosomes arranged according to length, and then trisomy 18 and 13 survive beyond 1 year of age.1
the two sex chromosomes that determine male or female Trisomy 21 (Down Syndrome). Trisomy 21 occurs in
sex. Modern methods of staining karyotypes enable analysis approximately one in every 740 live births,14 and its inci-
of the various numerical and structural abnormalities that dence is distributed equally between the sexes.10 The patho-
can occur. Most chromosomal abnormalities appear as nu- physiological features of Down syndrome are caused by an
merical abnormalities (aneuploidy) such as one missing overexpression of genes on human chromosome 21. Ninety-
chromosome (monosomy) or an additional chromosome, as five percent of individuals have an extra copy in all of their
in trisomy 21 (Down syndrome).8 Structural abnormalities body’s cells. The remaining 5% have the mosaic and trans-
occur in many forms. They include a missing or “extra location forms.15 In the United States the incidence of Down
portion” of a chromosome or a translocation error, which is syndrome increases with advanced maternal age.10 Detec-
an interchange of genetic material between nonhomologous tion of Down syndrome is possible with various prenatal
chromosomes. The incidence of chromosomal abnormalities tests, and the diagnosis is confirmed by the presence of char-
among spontaneously aborted fetuses may be as high as acteristic physical features present in the infant at birth.16
60%.8,13 About one in 150 live-born infants have a detect- Down syndrome is the most common chromosomal cause of
able chromosomal abnormality; and in about half of these moderate to severe intellectual disability.15 The typical
cases the chromosomal abnormality is accompanied by phenotypical features observable from birth are hypotonia,
n PARTIAL LISTING OF PEDIATRIC

TABLE 13-2
GENETIC CONDITIONS
APPROXIMATE INCIDENCE
SYNDROME OR DISEASE (UNITED STATES)
CHROMOSOMAL ABNORMALITIES
Autosomal Trisomy
Trisomy 21 1:740
Trisomy 18 1:5000
Trisomy 13 1:16,000
Sex Chromosome Aneuploidy
Turner syndrome 1:2500 females
Klinefelter syndrome 1:500-1000 males
Partial Deletion
Prader-Willi syndrome 1:10,000-30,000
Angelman Syndrome 1:12,000-20,000
Cri-du-chat syndrome 1:20,000-50,000
SINGLE-GENE ABNORMALITIES
Autosomal Dominant
Neurofibromatosis type 1 1:3500
Tuberous sclerosis 1:5800
Osteogenesis imperfecta 6-7:100,000
Autosomal Recessive Figure 13-1 n Ten-month-old girl with Down syndrome.
Cystic fibrosis 1:2500-3500 Caucasians
(highest ethnic incidence)
Spinal muscle atrophy 1:6000-10,000 Alzheimer disease may account for higher mortality rates
Phenylketonuria 1:10,000-15,000 later in life.18
Hurler syndrome 1:100,000 Impairments of visual and sensory systems are also com-
Sex-Linked mon in individuals with Down syndrome. As many as 77%
Duchenne muscular dystrophy 1:3500 of children with Down syndrome have a refractive error
Fragile X syndrome 1:4000 males, 1:8000 females (myopia, hyperopia), astigmatism, or problems in accom-
Hemophilia A 1:4000-5000 males modation.19 Hearing losses that interfere with language
Rett syndrome 1:10,000-22,000 females development are reportedly present in 80% of children with
MULTIFACTORIAL ABNORMALITIES Down syndrome. In most cases the hearing loss is conduc-
Cleft lip with or without cleft 1:1000 tive; in up to 20% of cases the loss is sensorineural or
palate mixed.16,20 Obstructive sleep apnea has been reported to
Clubfoot (talipes equinovarus) 1:1000 exist frequently in young children21,22 and adults with Down
Spina bifida 7:10,000 syndrome.23 Craniofacial impairments such as a shortened
palate and midface hypoplasia, along with oral hypotonia,
MITOCHONDRIAL ABNORMALITIES
tongue thrusting, and poor lip closure, frequently result in
Mitochondrial myopathy Rare
feeding difficulties at birth.24 Bell and colleagues studied the
Kearns-Sayre disease Rare
prevalence of obesity in adults with Down syndrome and
reported it in 70% of male subjects and 95% of female sub-
jects.25 Children with Down syndrome also appear to have a
higher risk of being overweight or obese,26-28 which may be,
epicanthic folds, flat nasal bridge, upward slanting palpebral in part, a result of the retarded growth and endocrine and
fissures, small mouth, excessive skin at the nape of the neck, metabolic disorders associated with trisomy 21.28 In a small
and a single transverse palmar crease (Figure 13-1). population study of children with Down syndrome, Dyken
Information compiled by the Centers for Disease Control and co-workers29 reported that there was a high prevalence
and Prevention for years 1968 through 1997 indicates that of obstructive sleep apnea associated with a higher body
the median survival age of individuals with Down syndrome mass index.
is 49 years, compared with 1 year in 1968. Improvements in Children with Down syndrome may have musculoskele-
the median survival age were less in races other than white, tal anomalies such as metatarsus primus varus, pes planus,
although the reasons for this remain unclear.14 Half of all thoracolumbar scoliosis, and patellar instability and have an
children with Down syndrome have congenital heart de- increased risk for atlantoaxial dislocation,30-32 which has
fects.16 Congenital heart problems, respiratory infection, been observed through radiography in up to 10% to 30% of
and leukemia are the most common factors associated with individuals with this syndrome30,31 with and without neuro-
morbidity and mortality in childhood,17 whereas a possible logical compromise.33 There is some controversy in the
increased tendency for premature cellular aging and medical community as to the necessity and efficacy of
radiographic screening for the instability.31,32 Proponents of

radiographic screening argue that neurological symptoms
of atlantoaxial instability may often go undetected in this
population because symptoms are often masked by the
wide-based gait and motor dysfunction already associated
with the disorder. If the child is unable to verbalize com-
plaints or the child is uncooperative with physical and neu-
rological examinations, symptoms may be missed. There is
particular concern about cervical instability if these children
undergo surgical procedures requiring general anesthesia32
and participate in recreational sports such as the Special
Olympics.31 Symptomatic instability can result in spinal
cord compression leading to myelopathy with leg weakness,
decreased walking ability,33 spasticity, or incontinence. Al-
though reportedly rare, there have been cases where atlanto-
axial dislocation has resulted in quadriplegia.30
Several researchers have explored the neuropathology
associated with Down syndrome. Changes in brain shape,
size, weight, and function occur during prenatal and infant
development of babies with Down syndrome, with impor-
tant differences apparent by 6 months of age.34 The rela-
tively small size of the cerebellum and brain stem was
reported by Crome and Stern in the 1970s.35 Marin-Padilla36
studied the neuronal organization of the motor cortex of a
19-month-old child with Down syndrome and found various
structural abnormalities in the dendritic spines of the pyra-
midal neurons of the motor cortex. He suggested that these
structural differences may underlie the motor incoordination
and intellectual disability characteristic of individuals with
Down syndrome. Loesch-Mdzewska37 also found neurologi-
cal abnormalities of the corticospinal system (in addition
Figure 13-2 n Twenty-one–month-old male with mosaic form of
to reduced brain weight) in his neuropathological study of
trisomy 18. (Reprinted with permission of Tucker ME, Garringer
123 individuals with Down syndrome aged 3 to 62 years.
HJ, Weaver DD: Phenotypic spectrum of mosaic trisomy 18: two
Crome38 reported lesser brain weight in comparison with
new patients, a literature review, and counseling issues. Am J Med
normal persons. Finally, Benda39 noted a lack of myeliniza-
Genet A 143:505–517, 2007.)
tion of the nerve fibers in the precentral area, frontal lobe,
and cerebellum of infants with Down syndrome. As
McGraw40 has pointed out, the amount of myelin in the syndrome.45 Typical malformations affect the cardiovascular,
brain reflects the stage of developmental maturation. The gastrointestinal, urogenital, and skeletal systems. Infants
delayed myelinization characteristic of neonates and infants with trisomy 18 have low birth weight and small stature, with
with Down syndrome is thought to be a contributing factor a long narrow skull, low-set ears, flexion deformities of the
to the generalized hypotonicity and persistence of primitive fingers, and rocker-bottom feet. Muscle tone is initially
reflexes characteristic of this syndrome.41 hypotonic, but it becomes hypertonic in children with longer
Trisomy 18. Trisomy 18, or Edwards syndrome, is the than typical life span.45 The period of hypertonicity in the
second most common of the trisomic syndromes to occur early years may change to low tone and joint hyperextensi-
in term deliveries, although it is far less prevalent than bility by preschool and school age. Microcephaly, abnormal
Down syndrome. It occurs in one in 5000 newborns, and gyri, cerebellar anomalies, myelomeningocele, hydroceph-
approximately 80% of affected infants are female.42 As aly, and corpus callosum defects have been reported in
with Down syndrome, advanced maternal age is positively individuals with trisomy 18.46
correlated with trisomy 18. Most cases of Edwards Common skeletal malformations that may warrant
syndrome occur as random events during the formation of attention from the developmental physical or occupational
reproductive cells; fewer cases occur as errors in cell divi- therapist include scoliosis,46 limited hip abduction, flexion
sion during early fetal development; and inherited, translo- contractures of the fingers, rocker-bottom feet, and talipes
cation forms rarely occur.42 Only 10% of infants born with equinovarus.45 Infants with trisomy 18 may also have
trisomy 18 survive past the first year of life; female and feeding difficulties as a result of a poor suck.47 Profound
non-Caucasian children survive longest.43 The survival intellectual disability is another clinical factor that will
of girls averages 7 months; the survival of boys averages affect the developmental therapy programs for children
2 months.43 Individuals surviving past infancy most often with trisomy 18.46,47
have the mosaic form, and there is high variance in Trisomy 13. Trisomy 13, also commonly called Patau
phenotype (Figure 13-2).44 syndrome, is the least common of the three major autosomal
Individuals with trisomy 18 generally have far more seri- trisomies, with an incidence of one in 10,000 to 20,000 live
ous organic malformations than seen in those with Down births.8,42 As in the other trisomic syndromes, advanced
maternal age is correlated with the incidence of trisomy syndrome,57 with a fewer number of cardiovascular
13.48 Fewer than 10% of individuals with trisomy 13 survive malformations in individuals with the mosaic form59; 33%
past the first year of life42,43; girls and non-Caucasian infants to 60% of individuals with Turner syndrome have kidney
appear to survive longer.42,43 Individuals surviving past malformations.51 Hypertension is common even in the absence
infancy most often have the mosaic form, and there is high of cardiac or renal malformations.57,60
variance in phenotype.43 As with Edwards syndrome, most There are numerous incidences of skeletal anomalies,
cases of Patau syndrome occur as random events during the some of which may be significant enough to require the
formation of eggs and sperm, such as nondisjunction errors attention of a pediatric therapist. Included among these
during cell division.48 are hip dislocation, pes planus and pes equinovarus, dislo-
Trisomy 13 is characterized by microcephaly, deafness, cated patella,51 deformity of the medial tibial condyles,46
anophthalmia or microphthalmia, coloboma, and cleft lip idiopathic scoliosis,57 and deformities resulting from
and palate.48 As in trisomy 18, infants with trisomy 13 fre- osteoporosis.10,57
quently have serious cardiovascular and urogenital malfor- Sensory impairments include decrease in gustatory and
mations and typically have severe to profound intellectual olfactory sensitivity61,62 and deficits in spatial perception
disability.49 Skeletal deformities and anomalies include and orientation,61 and up to 90% of adult females have
flexion contractures of the fingers and polydactyly of the moderate sensorineural hearing loss. Recurrent ear infec-
hands and feet.10 Rocker-bottom feet also have been tions are common and may result in future conductive
reported, although less frequently than in individuals with hearing loss.60 Although the average intellect of individuals
trisomy 18. Reported central nervous system (CNS) malfor- with Turner syndrome is within normal limits, the inci-
mations include arhinencephalia, cerebellar anomalies, dence of intellectual disability is higher than in the general
defects of the corpus callosum, and hydrocephaly.50 population.45 Noonan syndrome, once thought to be a
variant of Turner syndrome, has several common clinical
Sex Chromosome Aneuploidy characteristics; however, advancements in genetics research
The human X chromosome is large, containing approxi- have shown that the syndromes have different genetic
mately 5% of a human’s nuclear DNA. The Y chromosome, causes.63,64
much smaller, contains few known genes.8 Females, with Klinefelter Syndrome. Klinefelter syndrome is an ex-
genotype XX, are mosaic for the X chromosome, meaning ample of aneuploidy with an excessive number of chromo-
that one copy of their X chromosome is inactive in a given somes that occurs in males. The most common type,
cell; some cell types will have a paternally derived active 47,XXY, is usually not clinically apparent until puberty,
chromosome, and others a maternally derived X chromo- when the testes fail to enlarge and gynecomastia occurs.65
some. Males, genotype XY, have only one copy of the Nearly 90% of males with Klinefelter syndrome possess a
X chromosome; therefore diseases caused by genes on the karyotype of 47,XXY, and the other 10% of patients are
X chromosome, called X-linked diseases (see section on variants.66 The incidence of Klinefelter syndrome (XXY) is
sex-linked disorders), can be devastating to males and less about one in 500 to 1000 males, and an estimated half of
severe in females.8 In the presence of abnormal numbers 47,XXY conceptions are spontaneously aborted.8 The extra
of sex chromosomes, neither male nor female individuals X chromosome(s) can be derived from either the mother or
will be phenotypically normal.8 Two of the most prevalent the father, with nearly equal occurrence.67 Advanced mater-
sex chromosome anomalies are Turner syndrome and nal age is widely accepted as a causal factor.8,66 FISH analy-
Klinefelter syndrome. sis of spermatozoa from fathers of boys with Klinefelter
Turner Syndrome. Turner syndrome affects females syndrome suggests that advanced paternal age increases the
with monosomy of the X chromosome. The syndrome, also frequency of aneuploid offspring.68-70
known as gonadal dysgenesis, occurs in one in 2500 live Most individuals with karyotype XXY have normal intel-
female births.51,52 Turner syndrome is the most common ligence, a somewhat passive personality, and a reduced
chromosomal anomaly among spontaneous abortions.53,54 libido. Eighty-five percent of individuals having the nonmo-
Most infants who survive to term have the mosaic form of saic karyotype are sterile. Individuals with the karyotypes
this syndrome, with a mix of cell karyotypes, 45,X and 48,XXXY and 49,XXXXY tend to display a more severe
46,XX. The SHOX gene, found on both the X and Y chro- clinical picture. Individuals with 48,XXXY usually have
mosomes, codes for proteins essential to skeletal develop- severe intellectual disability, with multiple congenital anom-
ment. Deficiency of the SHOX gene in females accounts for alies, including microcephaly, hypertelorism, strabismus,
most of the characteristic abnormalities of this disorder.52,55 and cleft palate.10,65 Skeletal anomalies include radioulnar
Three characteristic impairments of the syndrome are sexual synostosis, genu valgum, malformed cervical vertebrae, and
infantilism, a congenital webbed neck, and cubitus valgus.56 pes planus.10 A 2010 systematic review of literature71 on
Other clinical characteristics noted at birth include dorsal neurocognitive outcomes of persons with Klinefelter syn-
edema of hands and feet, hypertelorism, epicanthal folds, drome concluded that problems of delayed walking in chil-
ptosis of the upper eyelids, elongated ears, and shortening of dren and persistent deficits in fine and gross motor develop-
all the hand bones.51,57 Growth retardation is particularly ment, and problems in motor planning.71,72 Giedd and
noticeable after the age of 5 or 6 years, and sexual infantil- co-workers published the results of a case-control study
ism, characterized by primary amenorrhea, lack of breast examining brain magnetic resonance imaging (MRI) scans
development, and scanty pubic and axillary hair, is apparent of 42 males with Klinefelter syndrome and reported cortical
during the pubertal years. Ovarian development is severely thinning in the motor strip associated with impaired control
deficient, as is estrogen production.10,58 Congenital heart of the upper trunk, shoulders, and muscles involved in
disease is present in 20% to 30% of individuals with Turner speech production.73
Partial Deletion Disorders

Deletions are one example of mutations that cause changes
in the sequence of DNA in human cells. A sequence change
that affects a gene’s function can cause the final protein
product to be altered or not produced at all.
Cri-du-Chat Syndrome. Cri-du-chat syndrome, also
referred to as cat-cry syndrome, and 5p minus syndrome
results from a partial deletion of the short arm of chromo-
some 5. Example nomenclature for a female with this
syndrome is (46,XX,del[5p]). The incidence of the syn-
drome is estimated to be one case per 20,000 to 50,000 live
births.10,74 Although approximately 70% of individuals
with cri-du-chat syndrome are female, there is an unex-
plained higher prevalence of older males with this
disorder.75 Advanced parental age is not a causal factor. A
study completed in 1978 indicated that life expectancy
was 1 year for 90% of infants born with this disorder,76 but
now life expectancy is nearly normal with routine medical
care.77
Primary identifying characteristics at birth include a
definitive high-pitched catlike cry, microcephaly, evidence
of intrauterine growth retardation, and subsequent low birth
A B
weight.10,76,78 Abnormal laryngeal development accounts for
the characteristic cry, which is present in most individuals Figure 13-3 n Illustration of the effect of imprinting on chromo-
and disappears in the first few years of life.76 Other features some 15 deletions. A, Inheritance of the deletion from the father
of individuals with this syndrome include hypertelorism, produces Prader-Willi syndrome. B, Inheritance of the deletion
strabismus, “moon face,” and low-set ears.10,76,78 Associated from the mother produces Angelman syndrome. (From Jorde L,
musculoskeletal deformities include scoliosis, hip disloca- Carey J, Bamshad M, White R: Medical genetics, ed 3, St Louis,
tions, clubfeet, and hyperextensibility of fingers and toes. 2005, Mosby.)
Muscular hypotonicity is associated with this syndrome,
although cases with hypertonicity have also been noted.79
Severe respiratory and feeding problems have also been Characteristics of PWS in infancy include hypotonia,
reported.77 Postnatal growth retardation has been docu- poor feeding, lethargy, and hypogonadism.85,86 Develop-
mented, with the median near the 5th percentile of the mental milestones in the first 2 years of life are not acquired
normal growth curve.80 until approximately twice the normal age.87,88 Between
Although intellectual disability and physical deformities 1 and 4 years of age, hyperphagia is apparent and if uncon-
are more severe with larger deletions,74 there is evidence trolled will lead to morbid obesity and its associated health
that with early developmental intervention these children complications.86,87,89 Most individuals with PWS have mild
can develop language, functional ambulation, and self-care to moderate intellectual disability, although some individu-
skills.81,82 als have IQ scores within normal limits.90 Maladaptive
Prader-Willi Syndrome and Angelman Syndrome. behaviors such as temper tantrums, aggression, self-abuse,
Prader-Willi syndrome (PWS) and Angelman syndrome and emotional lability have been reported.91 As a result of
(AS) are discussed together because they result from a struc- extreme obesity, many individuals with PWS have impaired
tural or functional loss of the PWS and AS region of chro- breathing that can produce sleepiness, cyanosis, cor pulmo-
mosome 15 (15q11-13), which can occur by one of several nale, and heart failure.91 Scoliosis is common but does not
genetic mechanisms.83,84 PWS has an incidence of one in appear to be related to obesity.92
15,000 to 30,00083 and AS has an incidence of one in 12,000 Clinical diagnosis is confirmed by laboratory genetic
to 20,000.83,84 These two syndromes illustrate the effect of testing techniques including DNA-based methylation test-
genomic imprinting, which is the differential activation of ing, FISH probe, and pyrosequencing assays.86,93 Most cases
genes of the same chromosome and location, depending on of PWS are caused by random mutations in parental repro-
the sex of the parent of origin (Figure 13-3).8 ductive cells.87 Other cases may result from translocation
PWS results from a failure of expression of paternally errors.86,94 Parental studies are important in translocation
inherited genes in the PWS region of chromosome 15.83 cases because 20% of cases cited in the literature involved
Conversely, AS results when the maternal contribution in the familial rearrangements, which may significantly increase
15q11.2-q13 region is lost.84 OCA2 is a gene located within the risk of recurrence.95
the PWS and AS region of chromosome 15 that codes for Angelman syndrome, named after Dr. Harry Angelman,
the protein involved in melanin production. With loss of one who first described children with AS in 1965, is character-
copy of this gene, individuals with PWS or AS will have ized by developmental delay or intellectual disability, sei-
light hair and fair skin. In the rare case that both copies of zures, ataxia, progressive microcephaly, and severe speech
the gene are lost, these individuals may have a condition impairments. Tongue thrusting, drooling, and sucking and
called oculocutaneous albinism, type 2, which causes severe swallowing disorders occur in 20% to 80% of children.
vision problems.84 Individuals often display spontaneous bouts of laughter
accompanied by hand-flapping movements and a character- DNA of immature lymphoblasts is altered and they repro-
istic walking posture of arms overhead and flexed el- duce in abnormal numbers, crowding out the formation of
bows.8,84,96 Infants appear normal at birth, but severe devel- normal cells in the bone marrow.102,105 Sixty percent of cases
opmental delay becomes apparent by 6 to 12 months of age. of ALL occur in children, with the peak incidence in the first
More unique features of the disorder do not appear until 5 years of life. A rise in the incidence of ALL has been
after 1 year of age. Children with AS typically have structur- reported during major periods of industrialization world-
ally normal brains on MRI and computed tomography (CT) wide105,106 and is hypothesized to be associated with expo-
scans, but electroencephalogram (EEG) findings are often sure to radiation107 and other environmental teratogens108,109
abnormal, showing a characteristic pattern that may assist in the preconception, gestational, and postpregnancy
with diagnosis before other clinical symptoms emerge,84,97 periods.103,106
and molecular studies can also confirm the disorder before With advancements in medical treatment protocols for
all of the clinical criteria for this diagnosis are met.84 pediatric patients, 5-year survival rates have improved to
Most cases of AS occur as a result of mutations involving 80%.104 Children aged 1 to 9 years at diagnosis have a better
deletion or deficient function of the maternally inherited prognosis than infants, adolescents, or adults diagnosed with
UBE3A gene. This gene codes for an enzyme, ubiquitin ALL.103 There are numerous forms of translocation muta-
protein ligase, involved in the normal process of removing tions associated with ALL. Some translocation forms of
damaged or unnecessary proteins in healthy cells. In most of ALL do not respond well to combination chemotherapy
the body’s tissues except the brain, both copies (maternal treatment; an example is the translocation that occurs
and paternal) of the UBE3A gene are active. Only the mater- between chromosomes 9 and 22, known as the “Philadelphia
nal copy of the gene is normally active in the brain, so if this chromosome.”104,110 Other translocations that result in
copy is absent or deficient, the normal cellular housekeeping hyperdiploidy (more than 50 chromosomes), in particular
process breaks down.84 The risk of having another child with within chromosomes 4, 10, and 17, may confer a more
AS can vary from 1% to 50% depending on which of the six favorable outcome.111
known genetic mechanisms is responsible for the disorder. Frequently, diagnosis is made when a physician relates
the child’s history of a persistent viral respiratory infection
Translocation Disorders with other characteristic clinical signs and symptoms con-
Translocation errors have been identified in many childhood sistent with hematopoietic leukemia. The key symptoms of
hematologic cancers and sarcomas.98,99 Translocation errors ALL are pallor, poor appetite, lethargy, easy fatigue and
are also commonly seen in couples with infertility.100 Trans- bruising, fever, mucosal bleeding, and bone pain.99 A com-
location abnormalities occur when genetic material is plete blood count will show a shortage of all types of blood
exchanged and rearranged between two nonhomologous cells, including red, white, and platelets. Diagnosis is con-
chromosomes (those not in the same numbered pair). The firmed by the presence of lymphoblasts in bone marrow.
structural abnormality can result in the loss or gain of chro- Radiographs may be necessary to determine metastases, and
mosomal material (an unbalanced arrangement) or no loss cerebrospinal fluid will be examined because early involve-
or gain of material (a balanced arrangement). Unbalanced ment of the CNS has important prognostic implications.106
arrangements can produce serious disease or deformity Cytogenetic studies will be performed to aid in selection of
in individuals or their offspring. Carriers of balanced treatment protocols and prognosis.104
arrangements—estimated at one in 500 individuals—often Referral to physical and occupational therapists is made
have a normal phenotype, but their offspring may have an for other common problems such as muscle cramps, muscle
abnormal phenotype.8 There are two basic types of translo- weakness, impaired gross and fine motor performance, de-
cations: reciprocal translocation and robertsonian transloca- creased energy expenditure, osteopenia, and osteoporosis.112
tion. Reciprocal translocations occur when two different
chromosomes break and the genetic material is mutually Single-Gene Disorders
exchanged. A robertsonian translocation occurs when there The previous section described genetic disorders that occur
is a break in a portion of two different chromosomes, with because of chromosomal abnormalities involving more than
the longest remaining portions of both chromosomes form- one specific gene. Other genetic disorders commonly seen
ing a single chromosome. The shorter portions that broke among children in a therapy setting include those that result
away usually do not contain vital genetic information; from specific gene defects. The inheritance patterns of
therefore the individual may be phenotypically normal.8 An single-gene traits were described by Gregor Mendel in the
example notation of a reciprocal translocation is 46,XY,t(7;9) nineteenth century. These patterns, autosomal dominant,
(q36;q34). This individual is male with a normal number of autosomal recessive, and sex linked, are discussed sepa-
chromosomes but with a translocation of genetic material on rately, and specific examples of syndromes or disorders
chromosomes 7 and 9; “q” refers to the short arm of these associated with each type are presented.
chromosomes, and the numbers “36” and “34” refer to the
location. Autosomal Dominant Disorders
Translocations occur in children seen in therapy settings, Mutations on one of the 22 numbered pairs of autosomes
including about 3% to 5% of children with Down syn- may result in isolated anomalies that occur in otherwise
drome,10 and translocations are found in 40% of all cases of normal individuals, such as extra digits or short fingers.
acute lymphoblastic leukemia (ALL).101 Each child of a parent with an autosomal dominant trait has
Acute Lymphoblastic Leukemia. ALL accounts for a 50:50 chance of inheriting that trait.8 Other autosomal
one fourth of all childhood cancers, and it is the most com- dominant disorders include syndromes characterized by
mon type of childhood cancer.102-104 ALL occurs when the profound musculoskeletal and neurological impairments
that may require intervention from a physical or an occupa- is sufficient to cause the disorder, it is still commonly clas-
tional therapist. Three examples of autosomal dominant sified as an autosomal dominant condition. There are fewer
disorders are osteogenesis imperfecta (OI), tuberous sclero- cases of autosomal recessive inheritance.8
sis, and neurofibromatosis (NFM). Prevention of fractures is an important goal in working
Osteogenesis Imperfecta. OI is a spectrum of with individuals with OI, but fear of handling and overpro-
diseases that results from deficits in collagen synthesis tection by caregivers may limit a child’s optimal functional
associated with single-gene defects, most commonly of independence. Caregiver education in careful handling and
COL1A1 and COL1A2, located on chromosomes 17 and 7, positioning should begin in the patient’s early infancy, and
respectively.10,113 OI is characterized by brittle bones result- training in the use of protective orthoses and assistive
ing from impaired quality, quantity, and geometry of bone devices is appropriate from the period of crawling through
material and hyperextensible ligaments.114,115 Deafness, re- ambulation.115,120,121 Aquatic therapy can be a valuable treat-
sulting from otosclerosis, is found in 35% of individuals by ment strategy for children with OI.120,121
the third decade of life.45 New knowledge about this disease Tuberous Sclerosis Complex. Tuberous sclerosis
from molecular genetic studies and bone histomorphometry complex (TSC) is characterized by a triad of impairments:
has expanded the classification subtypes of OI into types I seizures, intellectual disability, and sebaceous adenomas;
through VII.113,116 These classifications are helpful in deter- however, there is wide variability in expression, with some
mining prognosis and management, although there is a individuals displaying skin lesions only.122 Infants are fre-
continuum of severity of clinical features and much overlap quently normal in appearance at birth, but 70% of those who
in the features among the different classifications.116 Types go on to show the complete triad of symptoms display sei-
I, IV, V, and VI occur in the autosomal dominant pattern; zures during the first year of life. Although tuberous sclero-
whereas type VII occurs as a recessive trait, and types II and sis is inherited as an autosomal dominant trait, 86% of cases
III can occur as either dominant or recessive traits.113 OI occur as spontaneous mutations, with older paternal age
types V and VI account for only 5% of cases, and type VII a contributing factor. TSC affects both sexes equally, with a
has been found to date only in a Native Canadian popula- frequency of one in 5800 births.123 Mutations in the TSCI
tion.116 This section will compare and contrast only types I and TSC2 genes are known to cause tuberous sclerosis.10
through IV. The normal function of these genes is to regulate cell
The overall incidence of OI is one in 10,000 live births in growth; if these genes are defective, cellular overgrowth and
the United States, with types I and IV accounting for almost noncancerous tumor formation can occur.123 Tumor forma-
95% of all patients with OI.113 Ninety percent of dominant tion in the CNS is responsible for most of the morbidity and
forms of OI can be confirmed by DNA analysis.117 Type I is mortality with TSC,123 followed by renal disease associated
the least severe form, followed by types IV and III, with type with formation of benign angiomyolipomas.122 Diagnostic
II being the most severe. criteria for TSC have been established, and the determina-
Type I is characterized by blue sclera, mild to moderate tion can be made clinically; results of genetic testing are
bone fragility, joint hyperextensibility, and hearing loss in currently viewed as corroborative.122 Hypopigmented mac-
young adulthood.117 There are no significant deformities; ules are often the initial finding. These lesions vary in num-
individuals with this type may not sustain fracture until amber and are small and ovoid. Larger lesions, known as leaf
bulatory, and incidence of fractures decreases with age.117 spots, may have jagged edges.123 Sebaceous adenomas first
Type IV OI is characterized by more severe bone fragility appear at age 4 to 5 years, with early individual brown, yel-
and joint hyperextensibility than is type I. Bowing of long low, or red lesions of firm consistency in the nose and upper
bones, scoliosis, dentinogenesis imperfecta, and short stat- lips. These isolated lesions may later coalesce to form a
ure are common.114,116,118 characteristic butterfly pattern on the cheeks. Known also as
Children with type IV OI are often ambulatory but may hamartomas (tumor-like nodules of superfluous tissue), the
require splinting or crutches.114 skin lesions are present in 83% of individuals with tuberous
Children with type III OI have severe bone fragility and sclerosis.45
osteoporosis; often there are fractures in utero. Type III occurs Delayed development is another characteristic during
primarily in autosomal dominant inheritance in North infancy,124 particularly in the achievement of motor and
Americans and Europeans.116 The less frequent, autosomal speech milestones. Cerebral cortical tubers are present in
recessive form of OI, type III is characterized by progressive over 80% of patients and account for cognitive disability
skeletal deformity, scoliosis, triangular facies, large skull, including autism.122 Ultimately, 93% of individuals who are
normal cognitive ability, short stature, and limited ambulatory severely affected will have seizures, usually of the myo-
ability.114,116,119 The long bones of the lower extremities are clonic type, in early life, progressing in later life to grand
most susceptible to fractures, particularly between the ages mal seizures. Seizure development is the result of formation
of 2 to 3 years and 10 to 15 years,45 with the frequency of of nodular lesions in the cerebral cortex and white matter.45
fractures diminishing with age. Intramedullary rods inserted Tumors are also found in the walls of the ventricles. Neuro-
in the tibia or femur may minimize recurrent fractures.36 cytological examination reveals a decreased number of
Type II, the most severe form, is most often lethal before neurons and an increased number of glial cells and enlarged
or shortly after birth, although there are a few cases of chil- nerve cells with abnormally shaped cell bodies.10 Surgical
dren living to 3 years.116,119 Infants with type II OI have excision of seizure-producing tumors has been successful in
multiple fractures, often in utero, and underdeveloped some cases.122
lungs and thorax; therefore many die from respiratory com- Other associated impairments include retinal tumors and
plications after birth. Most type II cases are the result of hemorrhages, glaucoma, and corneal opacities.123 Cyst for-
spontaneous mutations; because only one copy of the gene mation in the long bones and in the bones of the fingers and
toes contributes to osteoporosis. Cardiac and lung tissues be caused by altered expression of neurofibromin in the
are also affected by TSC, and these effects are included in brain and/or hyperintense lesions in the brain seen on
the major diagnostic criteria.122 MRI.131 These focal areas of high signal intensity on
Neurofibromatosis. There are two recognized forms of T2-weighted MRI, known as unidentified bright objects
NFM: neurofibromatosis 1 (NFM1) and neurofibromatosis (UBOs), are seen in 60% of children and young adults with
2 (NFM2).125-127 Neurofibromas, or connective tissue tumors NFM1. The lesions, commonly found in the basal ganglia,
of the nerve fiber fasciculus, impede the development and internal capsule, thalamus, cerebellum, and brain stem,128,132
growth of neural cell tissues126,127 and are the hallmark tend to disappear in adulthood and often do not cause other
feature of NFM1. Neurofibromas are noncancerous, and overt neurological symptoms.128 Fewer than 10% of indi-
malignant changes are rare in children128 but an increased viduals are mentally retarded, but about 30% to 60% of
risk of malignancy has been observed in adult patients with affected children have learning disabilities that are mild
NFM1 and is a major contributor to decreased life expec- and nonprogressive.128,133 Poorer social skills and differ-
tancy by approximately 15 years.129 Tumors typically in- ences in personality, behavior, and quality-of-life perception
crease in number with increasing age. About half of all cases have been reported in children with NFM1 compared with
of NFM are caused by sporadic mutation in parental germ children without the disorder.126
cells or during fetal development.125-127 Schwannomas are In older children and adolescents, pain, itching, and
the main tumor type of NFM2 and classically appear bilater- stinging can occur from cutaneous neurofibromas, and in
ally on the vestibular nerves.127,130 NFM1 is also known as approximately half of all patients, neurological motor defi-
von Recklinghausen disease. Compared with type II, type cits occur from plexiform neurofibromas when the growth
I is more common (one per 3000 births)10,126 and usually puts pressure on peripheral nerves, spinal nerve roots, and
identified in younger children. It is associated with muta- the spinal cord.131 One percent to 5% of children aged 0 to
tions in the NF1 gene, which produces a protein, neurofibro- 6 years develop symptoms associated with optic pathway
min, the complete function of which is not yet understood glioma.126,128 Neurofibromatous vasculopathy interferes
but which is suspected to be a tumor suppressor. Diagnostic with arterial and venous circulation in the brain.126,131,134
criteria for NFM1 include the presence of two of the Hydrocephalus occurs in some individuals.126,128 Hyperten-
following features: six or more café-au-lait spots, two or sion is common and may develop at any age,126 and cardio-
more fibromas, freckling in the axillary or inguinal region, vascular disease is a major cause of premature death.129,131,135
optic pathway glioma, two or more Lisch nodules, specific Headaches are a commonly reported symptom in children,
osseous lesions, and a first-degree relative with NFM1.126 adolescents, and adults.126,128,136,137
Infants usually appear normal at birth, but initial café-au-lait Scoliosis may develop in 10% of patients and is rapidly
spots appear by age 3 years in 95% of individuals progressive from ages 6 to 10 years, or it may manifest in a
(Figure 13-4).131 Cognitive impairment is the most common milder form without vertebral anomalies during adoles-
neurological complication of NFM1131 and is postulated to cence.126 Other skeletal deformities include pseudarthrosis
of the tibia and fibula, tibial bowing, craniofacial and verte-
bral dysplasia, rib fusion, and dislocation of the radius and
ulna.126 Differences in leg length126 also have been noted and
may contribute to scoliosis. NFM2 occurs less frequently
than type I (one in 25,000 to 40,000 births)10 and is caused
by a mutation in the gene encoding the protein neurofi
bromin 2, also called Merlin.10 Merlin is produced in the
nervous system, particularly in Schwann cells that surround
and insulate the nerve cells of the brain and spinal cord.
Although type II shares characteristics with type I, it is
commonly characterized by tumors of the eighth cranial
nerve (usually bilateral), meningiomas of the brain, and
schwannomas of the dorsal roots of the spinal cord.10 Con-
trary to first descriptions of NFM1 and NFM2, café-au-lait
spots are seldom a singular feature of NFM2127; rather,
signs and symptoms of tinnitus, hearing loss, and balance
dysfunction usually appear during adolescence or in the
person’s early 20s.125,127 Problems with visual acuity caused
by strabismus and refractive errors are common in young
children.138 NFM2 may be underrecognized in children up
to 10 years old because early hearing loss and tinnitus are
present in only 20% of cases and otherwise only singular
features of the condition are observed. Infants may have
cataracts, and children may demonstrate unilateral facial
paralysis, eye squinting, mononeuropathy (foot or hand
drop), meningioma, spinal tumor, or cutaneous tumor. It is
recommended that children of parents with NFM2 should
Figure 13-4 n Four-year-old boy with neurofibromatosis and be considered to be at 50% risk for NFM2 and screened
characteristic café-au-lait spots on trunk. from birth.130
Autosomal Recessive Disorders increased respiratory demand; (2) increased workload of

An unaffected carrier of a disease-causing trait is heterozy- respiration as a deforming force on the immature musculo-
gous for the abnormal gene (possessing one normal and one skeletal frame; and (3) resultant impaired motor strategies for
mutated copy of the gene). If both parents are unaffected postural control during physical activity. Patients were once
carriers of the gene, each of their offspring has a 25% risk widely cautioned to avoid overexertion and fatigue with
of exhibiting the disorder.8 Consanguinity involving close exercise, but as patients are living longer, more evidence
relatives increases the chance of passing on autosomal supports the benefits of regular, even vigorous exercise for
recessive traits.8 Certain types of limb defects, familial children and adults with CF. Guidelines for exercise fre-
microcephaly, and a variety of syndromes are passed on quency and intensity have been published by the Association
through autosomal recessive genes. Four examples of of Chartered Physiotherapists in Cystic Fibrosis.146
autosomal recessive disorders affecting children in therapy Hurler Syndrome (Mucopolysaccharidosis I, Severe
settings are presented in this section: cystic fibrosis (CF), Type). Hurler syndrome is an inborn error of metabolism
Hurler syndrome, phenylketonuria (PKU), and spinal mus- that results in abnormal storage of mucopolysaccharides in
cle atrophy (SMA). many different tissues of the body.97 The incidence is esti-
Cystic Fibrosis. CF is one of the most common autoso- mated to be one in 100,000 live births for the severe forms10
mal recessive disorders and is more common in Caucasians, and one in 500,000 for milder forms.147 IDUA is the only
affecting one in 2000 to 4000.10 The CF gene has been gene currently known to be associated with this multisystem
mapped to chromosome 7, and its protein product, CF disorder.147
transmembrane regulator (CFTR).8 CFTR is involved in the Infants born with Hurler syndrome are usually normal in
regulation of chloride channels of the bowel and lung, which appearance at birth, may have inguinal or umbilical her-
is dysfunctional in patients with CF. Although CF has mark- nias,147 and may have higher birth weights than their sib-
edly variable expression, the overall median survival time lings. Symptoms of this progressively deteriorating disease
has improved from about 6 years of age in the 1940s to an usually appear during the latter half of the first year of
average of 36 years of age in 2006.139 In addition to the life,147 with the full disease picture apparent by 2 to 3 years
phenotypical features of CF, diagnosis of CF is made when of age.10,147 Diagnosis is made by identification of deficiency
two or more disease-causing mutations exist on the CTFR in lysosomal enzymes.147,148 Premature death, usually from
gene.139 Newborn screening tests for CF are required in all cardiorespiratory failure, occurs within the first 10 years
states in the United States.140 of life.147
Fibrotic lesions of the pancreas cause pancreatic insuffi- Characteristic physical features are caused by storage of
ciency in the majority of patients, which leads to chronic glycosaminoglycans (GAGs)147 and include a large skull
malnutrition. Ten percent to 20% of newborn infants with with frontal bossing, heavy eyebrows, edematous eyelids,
CF also have intestinal tract involvement with a meconium corneal clouding, a small upturned nose with a flat nasal
ileus. The sweat glands are commonly affected; high levels bridge, thick lips, low-set ears, hirsutism, and gargoyle-like
of chloride found in the sweat is the basis for the sweat facial features. Growth retardation results in characteristic
chloride test used in diagnosis. The most serious impairment dwarfism.147 Some individuals with the physical characteris-
in CF is the obstruction of the lungs by thick mucus, which tics of Hurler syndrome have normal intelligence, but most
leads to chronic pulmonary obstruction, infection that have progressive and profound intellectual disability.10,147
destroys lung tissue, and eventual death from pulmonary Spastic paraparesis or paraplegia and ataxia have been
disease in 90% of individuals.8 observed in individuals with Hurler syndrome.8 Commonly
Improved survival rate in recent decades is a result of reported orthopedic deformities include flexion contractures
improved antibiotic management, aggressive chest physi- of the extremities, thoracolumbar kyphosis, genu valgum,
cal therapy, and pancreatic replacement therapy. Postural pes cavus, hip dislocation, and claw hands as a result of joint
drainage, percussion, vibration, and breathing exercises deformities.45 Defective ossification centers of the vertebral
are key components of the management program provided bodies results in spinal deformity, complications of nerve
by the therapist and caregivers.141 Modern and less labor- entrapment, atlanto-occipital instability, and restricted cervi-
intensive devices such as those that provide positive expi- cal range of motion.147 Conductive and sensorineural hear-
ratory pressure may not be as effective at clearing secre- ing loss is common.147 Delayed motor milestones have been
tions as conventional chest physiotherapy,141 but patient noted as early as 10 months of age,148 with severe disabili-
and caregiver compliance with a regular program may be ties occurring with increasing age. Adaptive equipment
improved with them.142 Attention to diet is important, and often is needed, and most children with Hurler syndrome
every attempt should be made to maintain a routine exer- become wheelchair users in their later years.148
cise program with a goal of helping the children be more Phenylketonuria. PKU is the result of one of the more
active to improve their respiratory status and to prevent common inborn errors of metabolism. Mutations of the PAH
secondary impairments of adolescence and adulthood such gene located on chromosome 12 cause a deficiency in the
as stress incontinence in young women caused by exces- production of phenylalanine hydroxylase.149 Without this
sive coughing,143,144 chronic back pain, and osteopenia and enzyme, there is no conversion of phenylalanine to tyrosine,
osteoporosis.145 resulting in an abnormally excessive accumulation of
Massery143 describes the relationship among respiration, phenylalanine in the blood and other body fluids.149,150 If
postural control, and secondary impairments that develop untreated, this metabolic error results in mental and growth
in individuals with CF in the musculoskeletal and neuromus- retardation, seizures, and pigment deficiency of hair and
cular systems. She addresses the threefold problem faced skin.151 PKU is most prevalent among individuals of north-
by individuals with CF: (1) lung dysfunction leading to ern European ancestry, with a frequency of one in 10,000 to
15,000 births in the United States.149 It is estimated that one to the greatly increased susceptibility to pulmonary infec-
of every 50 individuals is heterozygous for PKU.8 tion, which usually results in death before the age of
Children born with PKU are usually normal in appear- 2 years.10,154,155
ance, with microcephaly and delayed development becom- SMA II, otherwise known as intermediate or chronic
ing apparent toward the end of the first year. Parents usually infantile SMA, has an onset at age 6 to 18 months and is
become concerned with the child’s slow development during associated with delayed motor milestones.156 Seventy per-
the preschool years.151 If PKU is untreated, the affected cent of children diagnosed with SMA II are alive at 25 years
child may go on to develop hypertonicity (75%), hyperac- of age.153
tive reflexes (66%), hyperkinesis (50%), or tremors (30%),152 Children with SMA II can usually sit independently if
in addition to intellectual disability. IQ levels generally fall placed but never stand unsupported.154 Bulbar weakness
between 10 and 50, although there have been reported rare with swallowing difficulties, poor weight gain, and dia-
cases of untreated individuals with normal intelligence.151 phragmatic breathing are common.155 Finger trembling is
A simple blood plasma analysis, which is mandatory for almost always present.153,154 Joint contractures are present in
newborn infants in all 50 U.S. states,140 can detect the pres- most individuals. Kyphoscoliosis of severity to require brac-
ence of elevated phenylalanine levels in nearly 100% of ing and/or surgery often develops, but patients are at risk of
cases.150 This test is ideally performed when the infant is at postanesthesia complications.154 Respiratory failure is the
least 72 hours old. If elevated phenylalanine levels are major cause of morbidity and mortality. Nocturnal oxygen
found, the test is repeated, and further diagnostic procedures desaturation and hypoventilation occur before daytime
are performed. Placing the infant on a low phenylalanine hypercarbia and are early indications of need for ventilator
diet (low protein) can prevent the intellectual disability support.154
and other neurological sequelae characteristic of this disor- SMA III is characterized by onset of symptoms in child-
der.151 Follow-up management by an interdisciplinary team hood after 18 months.153 It is also known as juvenile SMA or
consisting of a nutritionist, psychologist, and appropriate Kugelberg-Welander syndrome.10 These individuals have a
medical personnel is advised in addition to the special diet. normal life span and usually attain independent ambulation
Individuals with poor compliance with the recommended and maintain it until the third or fourth decade of life.153
diet have a greater risk of osteopenia in adulthood.150 Lower extremities are often more severely affected than the
Spinal Muscle Atrophy. SMA (5q SMA) is character- arms. Strength is often not sufficient for stair climbing, and
ized by progressive muscle weakness because of degenera- balance problems are common.153 Muscle aches and joint
tion and loss of the anterior horn cells in the spinal cord and overuse symptoms are frequently reported.154
brain stem nuclei.153,154 Diagnosis of SMA is based on SMA IV typically has an onset at older than 10 years
molecular genetic testing for deletion of the SMN1 gene of age and is associated with a normal life expectancy and
(named for “survival of motor neuron 1”), location 5q13. no respiratory complications.154,156 Individuals maintain
Another gene, SMA2, can modify the course of SMA. Indi- ambulation during the adult years.154
viduals with multiple copies of SMA2 can have less severe Variants of SMA occur in individuals with similar pheno-
symptoms or symptoms that appear later in life as the num- types and clinical diagnostic features of electromyography
ber of copies of the SMN2 gene increases.155 The overall (EMG) that are not associated with deletion of SMN1.156
disease incidence of SMA is five in 100,000 live births.155 Genetic testing for SMN gene deletion achieves up to 95%
The clinical classifications of SMA are still evolv- sensitivity and nearly 100% specificity.154 For cases that re-
ing.153,154,156 At present, four subtypes (types I to IV) are main unclear, a clinical diagnosis may be accomplished
well accepted, and a fifth, type 0, is being explored. The through EMG and muscle biopsy, which reveal neurogenic
subtypes are based on age at symptom onset and expecta- atrophy. Key physical signs are common: symmetrical
tions for maximum physical function, the latter being more weakness in the more proximal musculature versus distal,
closely related to life expectancy.156 and lower extremity weakness that is greater than in the
SMA type 0 is characterized by extreme muscle weak- arms.154 Traditional strength measurements are not practical
ness apparent before 6 months of age that likely had a pre- for children with SMA. The Gross Motor Function Mea-
natal onset.153,154 Some infants have a prenatal history of sure157 has excellent reliability in studies of gross motor
decreased fetal movements during the third trimester.153 evaluation in this population.154,158 Consensus guidelines on
SMA I, otherwise known as Werdnig-Hoffmann disease pulmonary care including assessment, monitoring, and treat-
or acute infantile SMA,10 has an onset before 6 months of ment; feeding and swallowing, gastrointestinal dysfunction
age.153,156 Incidence is estimated to be one in 20,000 live and nutrition; and orthopedic management have been pub-
births.10 It is characterized clinically by severe hypotonicity, lished by the Standard of Care Committee for Spinal Muscle
generalized symmetrical muscle weakness, absent deep ten- Atrophy.154 Currently there are no efficacious drugs to
don reflexes, and markedly delayed motor development. effectively treat the symptoms of SMA.160,161
Intellect, sensation, and sphincter functioning, however, are
normal.153 Children usually cannot sit without support and Sex-Linked Disorders
have poor head control.156 They have a weak cry and cough The third mechanism for transmission of specific gene de-
and problems with swallowing, feeding, and handling oral fects is through sex-linked inheritance. In most sex-linked
secretions.154 The diaphragm is spared, but combined with disorders, the abnormal gene is carried on the X chromo-
weakness in intercostal muscles, infants exhibit paradoxical some. Female individuals carrying one abnormal gene usu-
breathing, abdominal protrusion, and a bell-shaped trunk ally do not display the trait because of the presence of a
with chest wall collapse.154 Overall, this pattern of chest normal copy on the other X chromosome. Each son born to
wall weakness and poor respiratory function contributes a carrier mother, however, has a 50:50 chance of inheriting
the abnormal gene and thus exhibiting the disorder. Each death from HIV, life expectancy for those with severe hemo-
daughter of a carrier mother has a 50:50 chance of becoming philia who receive adequate treatment is 63 years.163 Factor
a carrier of the trait.8 Four syndromes that result in disability replacement therapy is credited for increasing the ease and
are discussed in this section: hemophilia, fragile X syn- safety of vigorous exercise and sports participation for indi-
drome (FXS), Lesch-Nyhan syndrome (LNS), and Rett viduals.166 The benefits of regular exercise are the same as
syndrome (RS). for unaffected individuals and outweigh the risks in treated
Hemophilia. Hemophilia is a bleeding disorder caused persons.167 A 2002 pilot study by Tiktinsky and colleagues167
by a deficient clotting process. Affected individuals will found decreased episodes of bleeding in a population of
have hemorrhage into joints and muscles, easy bruising, and young adults with a long-term history of resistance training
prolonged bleeding from wounds. The term hemophilia that began in adolescence.
refers to hemophilia A (coagulation factor VIII deficiency) Fragile X Syndrome. FXS is the most common sex-
and hemophilia B or Christmas disease (coagulation factor linked inherited cause of intellectual disability, affecting one
IX deficiency). There are numerous other clotting diseases, in 4000 males and one in 8000 females.168 Males manifest a
and some that were once referred to as hemophilia are now more severe form than females. A fragile site on the long
genomically distinguished. For example, von Willebrand arm of an X chromosome is present, with breaks or gaps
disease has a distinctly different genetic basis from hemo- shown on chromosome analysis. A region of the X chromo-
philia; it follows an autosomal recessive or autosomal some, named FMR1, normally codes for proteins that may
dominant pattern and involves mutation of the von play a role in the development of synapses in the brain.
Willebrand factor (VWF) gene, located on chromosome 12. Mutations of this region are errors of trinucleotide repeats,
VWF plays a role in stabilizing blood coagulation factor in which the number of CGG triplets at this region is
VIII.162 Hemophilia A and B occur as X-linked recessive expanded, thereby making the gene segment unable to pro-
traits owing to mutations of genes F8 and F9, respectively, duce the necessary protein.168
both of which are located on the X chromosome.163,164 Developmental milestones are slightly delayed in af-
Hemophilia A is reported to affect one in 4000 to 5000 fected males.168 Eighty percent of males are reported to have
males worldwide.163 Hemophilia B is less common, affect- intellectual disability, with IQs of 30 to 50 being common
ing one in 20,000 males worldwide.164 Hemophilia can but ranging up to the mildly retarded to borderline range.168
affect females, though in milder form. The severity and Penetrance (the proportion of individuals with a mutation
frequency of bleeding in hemophilia A are inversely related that actually exhibit clinical symptoms) in the female is re-
to the amount of residual factor VIII (less than 1%, severe; ported to be only 30%.8 Other impairments include epilepsy,
2% to 5%, moderate; and 6% to 35%, mild).163 The propor- emotional lability, attention-deficit/hyperactivity disorder
tions of cases that are severe, moderate, and mild are about (ADHD), and clinical autistic disorder in 30% of males.168,169
50%, 10%, and 40%, respectively.165 The joints (ankles, Life span is normal for individuals with this condition.168
knees, hips, and elbows) are frequently affected, causing Lesch-Nyhan Syndrome. Also known as hereditary
swelling, pain, decreased function, and degenerative choreoathetosis,170 LNS leads to profound neurological de-
arthritis. Similarly, muscle hemorrhage can cause necrosis, terioration. First described in 1964 by Lesch and Nyhan,171
contractures, and neuropathy by entrapment. Hematuria and it is associated with a mutation in the HPRT1 gene on the X
intracranial hemorrhage, although uncommon, can occur chromosome. This gene codes for an enzyme, hypoxanthine
after even mild trauma. Bleeding from tongue or lip lacera- guanine phosphoribosyltransferase, which allows cells to
tions is often persistent.8 recycle purines, some of the building blocks of DNA and
Hemophilia is usually diagnosed during childhood, with ribonucleic acid (RNA).172 Without this gene’s normal
the most severe cases diagnosed in the first year of life: function, there is an overproduction of uric acid (hyperuri-
bleeding from minor mouth injuries and large “goose cemia),172 which accumulates in the body. High uric acid
eggs” from minor head bumps are the most frequent levels are thought to cause neurological damage.170,172
presenting signs in untreated children.163 Children are The prevalence of LNS is one in 380,000 individuals.172
especially vulnerable to bleeding episodes owing to the Females born to carrier mothers have a 25% chance of
nature of their physical activity combined with periods of inheriting the mutation. There are rare reports of females
rapid growth.163 demonstrating this syndrome as a result of X chromosome
Treatment includes guarding against trauma and replace- inactivation. Most female carriers are considered to be
ment with factor VIII derived from human plasma or recom- asymptomatic, but some may have symptoms of hyperurice-
binant techniques.8 In the late 1970s to mid 1980s it was mia in adulthood.172
estimated that half of the affected individuals in the LNS is detectable through amniocentesis, and genetic
United States contracted hepatitis B or C or human immu- counseling is advisable for parents who have already given
nodeficiency virus (HIV) infection when treated with birth to an affected son.173
donor-derived factor VIII. The initiation of donor blood The prenatal and perinatal course is typical for affected
screening and use of heat treatment of donor-derived factor individuals. Hypotonia and delayed motor skills are notice-
VIII has almost completely eliminated the threat of infec- able by age 3 to 6 months.172 Dystonia, choreoathetosis,
tion.8 Although replacement therapy is effective in most and opisthotonus indicative of extrapyramidal involvement
cases, 30% of treated individuals with hemophilia A and emerge during the first few years of life.172 Many children
3% of individuals with hemophilia B have neutralizing are initially diagnosed with athetoid cerebral palsy when
antibodies that decrease its effectiveness.163,164 Before treat- pyramidal signs such as spasticity, hyperreflexia, and abnor-
ment with clotting factor concentrates was available, the mal plantar reflexes emerge.172 Most children never walk. A
average life expectancy was 11 years163; currently, excluding hallmark of the disease is severe and frequent self-injurious
behaviors such as lip and finger biting, which emerge in individuals with RS function in the range of severe to
almost all affected children by their third birthday.172 profound intellectual disabilities.
Because of the extreme self-mutilation that characterizes Head circumference is normal at birth, and its increase
this disorder, it has been questioned whether these children may decelerate in early childhood, but microcephaly is not
have normal pain perception.174 Although these children a consistent feature of RS.181 Retarded growth and muscle
have the abnormal catecholamine metabolism seen in other wasting are observed in most girls, likely associated with
patients with congenital pain insensitivity,175 behaviors doc- poor food intake and gastrointestinal problems.181
umented in children with LNS suggest that they do sense Almost one fourth of girls with RS never develop inde-
pain, demonstrated by their apparent relief when they are pendent ambulation skills; otherwise the onset of walking is
restrained from hurting themselves. Children may actually usually delayed until about 19 months of age.186 Initially
request the restraining device176 even when the device may hypotonia may be evident, but with advancing age, spastic-
be one that would not physically prevent biting, such as a ity of the extremities develops.187 Increased muscle tone is
glove or bandaid.172 A reported survey of parents of children usually observed first in the lower extremities, with contin-
with LNS indicated that parents often find behavioral pro- ued greater involvement than in the upper extremities.
gramming techniques helpful in modifying aggression Peripheral vasomotor disturbances, especially in the lower
toward self or others.176 However, there is no consensus on limbs, are often noted.181
the best kind of behavioral treatments, as any reward, either Scoliosis, which is often severe enough to require sur-
positive or negative, may increase the frequency of self- gical correction, occurs in most girls by adolescence,
injury.177 Some parents have reported that they elected characterized by a long C-shaped thoracolumbar curve,
tooth extraction as a means to prevent biting. Other impair- kyphoscoliosis, and an early onset of posterior pelvic tilt
ments in children with LNS include severe dysarthria and and abducted shoulder girdles.186,188-192 Heel cord tighten-
dysphagia. Bilateral dislocation of the hips may occur as a ing, and hip instability have also been identified as
result of the spasticity.172 Growth retardation is also appar- areas of potential concern.188 Abnormal EEG and seizures
ent, as well as moderate to severe intellectual disability.10 occur in 70% of individuals with RS in the first 5 years of
Individuals may have gouty arthritis and kidney and bladder life. Cranial CT results are normal or show mild general-
stones. ized atrophy. Breathing dysfunction, including wake
Blood and urine levels of uric acid have been decreased apnea and intermittent hyperventilation,186 is also associ-
successfully through the administration of allopurinol, with ated with RS. Interventions reported in the literature have
a resultant decrease in kidney damage. With current man- focused on splinting,193 behavioral modification tech-
agement techniques, most individuals survive into their niques to teach self-feeding skills,194 aquatic therapy,195
second or third decade of life.172 occupational therapy,196 music therapy,197 physical ther-
Rett Syndrome. RS is inherited in an X-linked pattern apy,181,190,191,197 and the first two combined in a dual-
and it affects females almost exclusively, as it is most intervention approach.198
often lethal in boys before age 2 years. Males may inherit
RS with an extra X chromosome in many or all of the Mitochondrial DNA Disorders
body’s cells.178-181 The estimated incidence is one in In addition to the nuclear genome, humans have another set
15,000 to 20,000 females.10,182 It has been reported that of genetic information within their mitochondria. Nuclear
99% of all cases of RS are the result of sporadic muta- genes exist in pairs of one maternal and one paternal allele.
tions.181,183 Most cases of RS, called classic RS, are caused In contrast, there are hundreds or thousands of copies of
by mutations in the MECP2 gene, which is responsible for mtDNA in every cell. mtDNA is small, circular, and double
directing proteins critical for normal synaptic develop- stranded. It has been well studied and was mapped long
ment; however, it is unclear how these mutations lead to before the human nuclear genome. mtDNA contains
all the signs and symptoms of the syndrome.181,184 Several 37 genes responsible for normal function of the mitochon-
variants of RS exist; they have overlapping features with dria in all body cells.198a Humans inherit mtDNA maternally.
classic RS but may have a much milder or more severe mtDNA is highly susceptible to mutation, and the molecule
course.181 has limited ability to repair itself. Tissues that have a high
Classic RS is characterized by apparently normal demand for oxidative energy metabolism, such as brain and
development during the first 6 months of life, followed by muscle, appear to be most vulnerable to mtDNA muta-
a short period of developmental plateau, and then rapid tions.11 Normal and mutated versions of mtDNA can coexist
deterioration of language and motor skills typically occur- within a patient’s body, but when a certain critical number
ring at 6 to 18 months of age.181,185 Most girls survive into of mutations exist, the body’s tissues will show clinical
adulthood.181 The hallmark of the syndrome is that during signs of dysfunction. These disorders affect the metabolic
the period of regression, previously acquired purposeful functions of the mitochondria, such as the generation of the
hand skills are also lost and replaced by stereotypical body’s energy currency, adenosine triphosphate. Many pa-
hand movements. These nonspecific hand movements tients with point mutations of mtDNA exhibit symptoms in
have been described as hand wringing, clapping, waving, early childhood; these mutations may be the most frequent
or mouthing. Virtually all language ability is lost, al- cause of metabolic abnormality in children.11 The minimum
though some children may produce echolalic sounds and birth prevalence of childhood mitochondrial respiratory
learn simple manual signing. Evidence of minimal recep- chain disorders is reported to be 6.2 per 100,000.12,199-201
tive language skills may be observed. Autistic behaviors, Medical intervention for mitochondrial encephalomyopa-
inconsolable crying and screaming, and bruxism are thies cannot treat the underlying disease, but the value of
common features of individuals with RS.181 Almost all rehabilitative therapies has been reported.202,203 An example
of a childhood disorder that can result from an mtDNA genetic disorders that are most relevant for physical or
mutation is Leigh syndrome. occupational therapists.
Leigh Syndrome Hypertonicity

Leigh syndrome, or subacute necrotizing encephalomyo Abnormalities of tone may result from dysgenesis or injury
pathy, may also be transmitted by X-linked recessive and to developing motor pathways. Hypertonia is common to
autosomal recessive inheritance. Approximately 20% of all many motor disorders and is defined as “abnormally in-
cases of Leigh syndrome are caused by mitochondrial creased resistance to externally imposed movement about a
mutations.204 The discussion in this section will focus on joint.”214 Examples of externally imposed movement are
characteristics of mtDNA-associated Leigh syndrome. passive movement by the therapist or changes in ankle and
Leigh syndrome has an onset in infancy, typically at 3 to knee position resulting from ground reaction forces during
12 months of age. Initial features may be nonspecific, such ambulation. Children with hypertonus generally display stiff
as a failure to thrive and persistent vomiting.204,205 It is a or jerky movements that are limited in variety, speed, and
progressive disorder caused by lesions that can occur in the coordination. Controlled, voluntary movements tend to be
brain stem, thalamus, basal ganglia, cerebellum, and spinal limited to the middle ranges of a joint. Total patterns of
cord. Common clinical features include seizures, epilepsy, flexion or extension may dominate, with limited ability for
muscle weakness, peripheral neuropathy, speech and feed- selective joint movements. Motor development of children
ing difficulties, gastrointestinal and digestive problems, and with hypertonicity may be further complicated by the reten-
heart problems. Most affected children have hypotonia, tion of primitive reflexes, which can result in stereotyped
movement disorders such as chorea, and ataxia. Life expec- movements associated with sensory input.214
tancy is 2 to 3 years; death most often results from respira- Sanger and colleagues214 proposed a classification sys-
tory or cardiac failure.204 tem to objectively define and distinguish different types
of hypertonicity. Three general types of hypertonicity—
Multifactorial Disorders spasticity, dystonia, and rigidity—may occur alone or in
Multifactorial disorders are believed to be a result of the combination. Spasticity is a velocity-dependent, increased
combined effects of mutations in multiple genes and envi- resistance to muscle stretch that may occur above a given
ronmental factors.8 Environmental factors may be those that threshold of speed and/or joint angle and may depend on the
have an impact on a developing fetus, such as prenatal diet, direction of joint movement. Dystonia is also an involuntary
or those that have an impact on humans as we age, such as alteration in the pattern of muscle activation during volun-
cigarette smoking. Disorders in this category can result tary movement or maintenance of posture. The observable
in congenital malformations such as spina bifida and club- disorder is demonstrated by intermittent muscle contractions
foot. An in-depth discussion of spina bifida can be found in causing twisting or repetitive movement, postures, or both.214
Chapter 15. Management information on clubfoot can Dystonia may be triggered by attempts at voluntary move-
be found in pediatric textbooks that include orthopedic ment or to prevent the movement of a joint (e.g., to prevent
information.206-209 knee buckling in stance). The pattern and magnitude of the
Many diseases such as cancer can result when the envi- abnormal muscle activity may change with the child’s
ronment interacts with genetic variations that exist in all arousal, emotional state, and tactile contact.214 Rigidity is
humans.8 Scientists are exploring genetic contributions to a form of hypertonus in which the speed of movement
premature births.210,211 Premature birth is the leading cause and joint angle do not affect the movement quality. Stiffness
of infant mortality and morbidity,212,213 and it most likely is caused by diminished tissue length and extensibility
a result of multiple genetic and environmental determinants of muscles and connective tissue is not included in the
that tend to run in families.210,211 Premature infants are at recent definitions of hypertonus (but may exist alongside
higher risk of neurological, musculoskeletal, and respiratory hypertonicity).214,215
problems than term infants are. Management of infants with Children may learn to use stereotypical patterns of move-
low birth weight is discussed in Chapter 11 of this text. ment and hypertonus to achieve functional goals by activat-
ing the muscle synergies of a reflex without sensory feed-
BODY STRUCTURE AND FUNCTION back.216 If a goal of therapy is to facilitate functional
PROBLEMS COMMON TO MANY PEDIATRIC movement that is not dominated by persistent reflexes, it is
GENETIC DISORDERS critical to practice new motor patterns to accomplish the
Specific examples of genetic disorders in children were functional activity for which that reflex is being used. The
presented in the foregoing section. Although there are focus of therapy activities needs to be on active movement
many disorders that physical and occupational therapists of the child and not on passive inhibition techniques of
may see often, others are rare and may only be suspected abnormal reflexes for the sake of “normalization” of tone
based on clustered problems of body structure, function, and movement.216-219
and activity limitations. Decisions about which interven-
tions to implement and the expected outcomes will be Hypotonicity
largely influenced by the diagnosis of the specific disorder, In contrast to hypertonia, low tone or hypotonia is not
once attained. Many genetic conditions share in common clearly defined. The “floppy” infant or child is commonly
a short list of primary problems that will negatively affect characterized as having hypermobility of joints that lack
the child’s physical movement and daily activities and resistance to passive movement, with diminished antigravity
participation, both immediately and in the long term. movement and postural stability. Hypotonia may occur be-
Table 13-3 summarizes the problems common to many cause of central or peripheral nervous system dysfunction,
TABLE 13-3 n CHARACTERISTIC FEATURES OF SELECTED GENETIC CONDITIONS
NEUROMUSCULAR
SYSTEM CARDIO-
GENETIC TYPICAL AGE CRANIOFACIAL MUSCULOSKELETAL INVOLVEMENT PULMONARY
CONDITION AT DIAGNOSIS DYSMORPHISM INVOLVEMENT AND TONE INVOLVEMENT
Trisomy 21 Prenatal or Yes Joint laxity and Hypotonia Yes
infancy instability
Trisomy 18 Prenatal, neonatal Yes Yes Hypotonia Yes

Turner syndrome Infancy, Yes Short stature, hip Yes
adolescence; dislocation, scoliosis
girls
Klinefelter syndrome Adolescence; Yes Yes Varies with age
adulthood;
males
Cri-du-chat syndrome Infancy Yes Yes Hypotonia Yes
Prader-Willi Infancy Yes Scoliosis Hypotonia Secondary to
syndrome obesity
Angelman syndrome Infancy Microcephaly Hypotonia and
seizures, ataxia
Acute lymphoblastic Childhood Bone pain and muscle
leukemia cramps
Osteogenesis Varies with type Multiple fractures and Yes
imperfecta and severity muscle weakness
Tuberous sclerosis Infancy, early Yes, cyst formation Hypertonia; seizures Yes, cyst formation
complex childhood
Neurofibromatosis Infancy, childhood Yes
type 1
Cystic fibrosis Infancy Chest wall deformity, Yes

muscle and bone
pain
Hurler syndrome Infancy Yes Yes Yes Yes
Spinal muscle Infancy Yes Hypotonia Yes

atrophy type II
Hemophilia type A Childhood or Joint pain and
earlier if severe hemarthroses
Fragile X syndrome Childhood Yes Joint laxity Yes
Lesch-Nyhan Infancy Gouty arthritis Varies
syndrome
Rett syndrome Late infancy to Scoliosis Hypotonia, ataxia,
early childhood seizures
as is the case in newborns with PWS and toddlers with SMA of cases.221,222 First-line genetic testing is indicated in neo-
type II, respectively. Many genetic disorders are revealed in nates with hypotonia plus facial dysmorphism or signs of
newborns based on the common features of severe, global peripheral hypotonia (e.g., as seen in SMA).221
hypotonia and low Apgar scores.220 Retrospective studies of Martin and colleagues223 surveyed physical and occupa-
newborns report key features of absence of antigravity tional pediatric therapists and reported that the majority of
movements and decreased reflexes. The presence of fetal therapists do not use formal examination methods to quan-
hypokinesia and/or polyhydramnios is reported to be predic- tify hypotonia directly, but rather use measurements for
tive of neonatal hypotonia in many cases.221 In full-term various expressions of hypotonia, most often muscle strength
neonates with hypotonia, studies report that 30% to 60% of and developmental milestones. This study also confirmed
cases are associated with a genetic disorder. A clinical neu- that most therapists agree that children with hypotonicity
rological examination such as described by Dubowitz and have diminished postural control and thus tend to lean on
the use of dysmorphic data bases can identify the majority supports to maintain a position. Examples of this behavior
OTHER SYSTEM OTHER DISEASE SENSORY MOTOR COGNITIVE

INVOLVEMENT PROCESSES DYSFUNCTION DELAY DELAY HALLMARK FEATURE(S)
Alzheimer disease, Vision, hearing Yes Yes Facial features, simian crease
sleep apnea,
obesity
Yes Yes Yes Yes Life span , 1 year
Distal lymphedema; Hearing Webbed neck appearance and
reproductive system short stature
Endocrine and reproductive Yes Yes Intellectual disability; course of

system gonadal development
Vision “Cat cry”

Reproductive system Morbid obesity Vision Yes Yes Hypotonia, obesity
Yes Yes Happy demeanor, sleep disorders
Osteopenia, Lethargy, fever, respiratory

osteoporosis infections
Dentinogenesis Hearing (type I) Multiple fractures
Skin lesions, adenomas; renal Vision Yes Yes Sebaceous adenomas, seizures
disease
Café-au-lait spots and Vision Café-au-lait spots, neurofibromas
cutaneous neurofibromas;
vasculopathy
Gastrointestinal system; Osteoporosis, Lung and digestive dysfunction
urinary stress incontinence osteopenia
in females
Yes Vision, hearing Yes Yes Progressive craniofacial
abnormalities and
developmental deterioration
Yes Yes Progressive loss of peripheral
motor function
Skin bruising Bleeding, bruising, joint pain,
and loss of motion
Yes Yes Intellectual disability, autism
Urogenital system Yes Yes Yes Self-injurious behavior, gouty
arthritis
Yes Yes Yes Regressive developmental delay;
stereotypical, purposeless hand
movements
are locking out weight-bearing joints and assuming posi- global hypotonia at birth that resolves and does not cause
tions that provide a broad base of support to maximize their long-term functional impairment.224,225 Hypotonicity is a
stability (Figure 13-5). Although retention of primitive re- persistent problem in many children with developmental
flexes is less likely in children with hypotonia compared delay. Therapists may address hypotonia and problems of
with those with hypertonia, delays in the development of postural control with a variety of treatment modalities and
postural reactions are a major concern. Limited strength and techniques including aquatic therapy,226 hippotherapy,227
lack of endurance are often concerns with children who have and neurodevelopmental therapy.228
hypotonicity. Hypotonicity and joint laxity are often associ-
ated with motor delay; however, therapists should not Hyperextensible Joints
assume that hypotonia and joint laxity are absolutely pred- Hyperextensible joints are commonly observed in children
icative of persistent motor delay.224 For example, many with hypotonicity and are noted in many children with
premature infants, with or without a genetic disorder, have a variety of genetic disorders, representing different
Figure 13-6 n Excessive bilateral pronation and flat feet associ-

ated with hyperextensibility in 8-year-old boy with global hypotonia.
Figure 13-5 n Eight-year-old boy with hypotonia associated

with a chromosomal translocation error. Note the broad base of
support in this “W” sitting position.
underlying organ structure problems. Activities should be

modified to avoid undue stress to these joints and the
surrounding ligaments, tendons, and fascia. For example,
positions that allow the knee or elbow joints to lock into
extension should be modified so that weight bearing occurs
through more neutral alignment. Varying the placement of
toys and support surfaces, providing physical assistance, and
using adaptive equipment can help modify weight-bearing A B
forces to achieve more neutral alignment.229 For example, if
hyperextensibility of ligaments leads to excessive pronation Figure 13-7 n Adapted equipment to promote participation in
in stance (Figure 13-6), the use of ankle-foot orthoses may upright activities for children with trunk weakness and poor pos-
provide enough support to the structures to allow functional tural control. A, The corner chair provides this child with Rett
activities in standing (see the discussion of orthotics in syndrome the proper back support, and toys can be placed within
Chapter 34). For a child who stands with knee hyperexten- her reach on the tray. B, This particular gait trainer allows appropri-
sion, a vertical stander may allow that child to stand and ate patterns of weight-shifting to occur while providing her with
play at a water table with her or his classmates for extended stability in the pelvis and lower trunk.
periods with the knees in a more neutral position. Rather
than restricting a child’s repertoire of upright positions, it is
preferable to modify an activity or provide external support hypotonicity, habitual positioning may lead to soft tissue
to enable a child to participate fully (Figure 13-7).230 restrictions. For example, children with hypotonia often
adopt a constant position of wide abduction, external rota-
Contractures and Musculoskeletal Deformities tion, and flexion at the hips (“frog” or “reverse W” posi-
Skeletal anomalies and deformities are associated with tion)233; in these children, soft tissue contractures can
many genetic disorders. The therapist should be aware of develop at the hips and knees. Children whose hips are
factors that can contribute to the development of deformities maintained in a position of adduction, flexion, and internal
to prevent or minimize such problems. The physical or rotation are at risk for hip subluxation or dislocation.233
occupational therapist may work with orthopedists, prosthe- Deformation of the anterior chest wall and shoulder gir-
tists, and orthotists to detect and prevent the progression of dles may result from primary problems in the cardiopulmo-
a variety of conditions. nary system such as in children with CF. When coupled with
Conditions that cause hypertonicity or spasticity are well hypotonicity and poor postural control, the anterior chest
known to place children at risk for joint contracture.231 wall muscles tighten as a result of the long-term rounded,
Children with hemophilia are at great risk of joint contrac- internally rotated shoulders and protracted scapulae, in
tures associated with hemarthroses and intramuscular hem- which case therapeutic interventions should target improv-
orrhages.232 Spinal deformities, such as lumbar lordosis and ing chest wall and scapulothoracic mobility as well as
thoracic kyphosis and scoliosis, are also common concerns strengthening postural muscles (Figure 13-8).234-236 In gen-
in children with hypertonia or hypotonia. Although eral, contractures and deformities are a concern for most
joint contractures are less likely to occur in a child with children who display a limited variety of postures and
movements. Therapists should consider the nature of the

disorder that places the child at risk for contractures when
choosing treatment techniques; disorder-specific techniques
can be found in pediatric occupational and physical therapy
textbooks.206,237
Respiratory Problems
A genetic risk for respiratory distress in infancy has been
suggested by reports of family clusters.238 Furthermore,
comparison of short- and long-term respiratory function in
infants with respiratory distress syndrome suggests that if all
other factors of nutrition, previous mechanical ventilation,
and gestational development are comparable, genetic risk
may account for cases of chronic and potentially irreversible
respiratory failure.238
Respiratory problems are often observed in children with
limited mobility. If the mobility impairments are the result
of hypotonicity or hypertonicity, impaired respiration may
be a result of chest and skeletal deformities. Many infants
with genetic disorders are born prematurely and are more
susceptible to respiratory problems than infants born at full
term.210,211,233 Prolonged mechanical ventilation and other
medical procedures may increase the time neonates spend in
the supine position, thus increasing the risk of gravity-
induced deformity of the rib cage and inefficiency of the
respiratory musculature.234,239,240
A Some children may find it difficult to tolerate one posi-
tion for an extended time owing to respiratory difficulties.
For these children, frequent changes of position and use of
adapted positioning devices may be necessary. Premature
infants in the neonatal intensive care unit may benefit from
regular prone positioning to facilitate restorative sleep,240-242
improved arterial oxygen saturation,243 and improved respi-
ratory synchrony.244 Children with respiratory problems
may require mobilization techniques, deep breathing, chest
expansion exercises, and postural drainage. In the case of
children with CF, a comprehensive program of respiratory
care is the primary therapy goal.245
Developmental Delay
Genetic disorders that affect neuromuscular, somatosen-
sory, and cognitive function are frequently associated with
developmental delays in children. The genetic basis for
multisystem syndromes such as Down syndrome or LNS
can be identified by cytogenetic and molecular techniques.
Congenital malformations, hearing impairment, and men-
tal or growth retardation are examples of common compo-
nents of developmental delay that often have a genetic
basis.
Developmental delay is typified by the failure to meet
expected age-related milestones in one or more of five areas:
physical, social and emotional, intellectual, speech and lan-
guage, and adaptive life skills. Developmental milestones
B that are typically assessed in the first 5 years of life can be
Figure 13-8 n Postural changes after therapeutic intervention to found in Box 13-2.
improve mobility in chest wall and shoulder girdles and strengthen Physical and occupational therapists can observe the
postural muscles in a child with cystic fibrosis. (Before interven- interaction among each of the five areas of development in
tion on left, after intervention on right.) an infant or child. For example, a child with severe hypoto-
nia who has limited movement experiences will not develop
a well adapted sensory system. Children with problems
processing sensory information often withdraw from social
interaction through which they would otherwise find
opportunities to develop speech, language, and social skills. that it facilitates knowledgeable surveillance for potentially
Dynamical systems theory246 explains this relationship associated health problems. A delayed diagnosis can
among all of the developing components in a child; lan- preclude timely implementation of beneficial medical, ther-
guage does not develop independently of gross motor skills, apeutic, and educational services. Children who are identi-
and the ability to feed or dress oneself is as related to social, fied to be at risk for developmental delay may be referred
emotional, and intellectual development as it is to fine motor to early intervention programs. Examples of assessment
skills. techniques and interventions for children with develop
Suspicion of developmental delay often leads to physi- mental delay can be found in pediatric physical therapy
cian referral. An accurate medical diagnosis is important in textbooks.247,248
BOX 13-2 n DEVELOPMENTAL MILESTONES
BY 1 MONTH Does not speak at least 15 words by 18 months

Sucks poorly and feeds slowly Does not use two-word sentences by 2 years
Lower jaw trembles constantly even when infant is not crying Does not know the function of common objects (brush,
or excited telephone, spoon) by 15 months
Does not respond to loud sounds or bright light Does not imitate actions or words; does not follow simple
Does not focus on and follow a nearby object moving side to side instructions
Rarely moves BY END OF THIRD YEAR
Extremities seem loose and floppy or very stiff Frequent falling and difficulty with stairs
BY END OF THIRD MONTH Persistent drooling or unclear speech
No Moro reflex Inability to build a tower of more than four blocks
Does not notice own hands by 2 months Difficulty manipulating small objects
Does not grasp and hold objects Cannot copy a circle
Eyes cross most of the time or eyes do not track well together Cannot communicate in short phrases
Does not coo or babble No pretend play
BY END OF FOURTH MONTH Little interest in other children
Head flops back when infant is pulled up to sitting by his or Extreme difficulty separating from caregiver
her hands BY END OF FOURTH YEAR
Does not turn head to locate sounds Cannot throw a ball overhand
Does not bring object to mouth Cannot jump in place with both feet
Does not smile spontaneously Cannot ride a tricycle
Inconsolable at night Cannot grasp a crayon between thumb and fingers; cannot
BY END OF FIFTH MONTH scribble
Persistent tonic neck reflexes Resists dressing, sleeping, using the toilet
Cannot maintain head up when placed on stomach or in Does not use sentences of more than three words; does not
supported sitting position use “me” and “you” appropriately
Does not reach for objects Ignores other children or people outside the family
Does not roll in both directions Does not pretend in play; no interest in interactive games
Persistent poor self-control when angry or upset
BY END OF SEVENTH MONTH
Reaches with one hand only BY END OF FIFTH YEAR
Cannot sit with help by 6 months Does not engage in a variety of physical activities
Does not follow objects at a distance Has trouble eating, sleeping, using the toilet
Does not bear some weight on legs Cannot differentiate between fantasy and reality
Does not laugh; does not try to attract attention through actions Seems unusually passive or aloof with others
Refuses to cuddle; shows no affection for caregiver Cannot correctly give her or his first and last names
Does not use plurals or past tense when speaking
BY END OF TWELFTH MONTH
Does not talk about daily experiences
Does not creep on all fours
Does not understand two-part commands
Cannot stand when supported
Cannot brush teeth efficiently
Does not search for toy hidden while he or she watches
Cannot take off clothing
Says no single words (e.g., “mama” or “dada”)
Cannot wash and dry hands
Does not use gestures such as waving hand or shaking head;
Cannot build a tower of six to eight blocks
does not point to objects or pictures
Does not express a wide range of emotions
BY END OF SECOND YEAR Seems uncomfortable holding a crayon
Cannot walk by 18 months
Failure to develop heel-toe walking pattern after several
months of walking
Behavioral Phenotypes in Genetic Syndromes growths for the control of seizures in individuals with
Study into the cognitive and behavioral aspects of individu- tuberous sclerosis.122 Surgical interventions such as gastric
als with certain genetic syndromes has given rise to the term bypass, small intestinal bypass, and jaw wiring have been
behavioral phenotype. Certain clusters of behavior that attempted for weight control in children with PWS but have
characterize a given syndrome can aid in the early recogni- had limited success.83,90
tion and diagnosis of a syndrome and can guide intervention
choices. Example aspects of behavioral phenotypes include Pharmaceutics
social interaction, sleeping patterns, mood, attention, moti- Second-generation bisphosphonates can reduce fracture
vation, adaptive and maladaptive strategies, intellect, and frequency, improve bone quality, and improve outcomes
memory.249-253 after orthopedic surgery in children and lessen the severity
Down syndrome, PWS, AS, FXS, and LNS are examples of osteoporosis in adults.115 Antibiotics and pneumoeusta-
of genetic disorders discussed in this chapter with delineated chian tubes to lessen the frequency and severity of otitis
behavioral phenotypes.250,251 Compulsive overeating in media can reduce the incidence of hearing loss in individu-
children with PWS, sleep disturbances in children with AS, als with Down syndrome20 and Turner syndrome.57 The use
and self-injury in children with LNS are behavioral prob- of appetite-regulating drugs for individuals with PWS has
lems that can have significant negative impact on quality of had equivocal results. Reproductive hormone therapy can
life for children and their families. Although children with promote pubertal development in girls with Turner syn-
Down syndrome have fewer maladaptive behaviors than drome57,60 and boys with Klinefelter syndrome.67,254 Growth
most children with intellectual disabilities,249,251,253 they hormone has been shown to improve stature in girls with
have been shown to abandon challenging tasks sooner than Turner syndrome.57 The use of anticonvulsants is an impor-
other children at similar developmental levels in exchange tant part of seizure management for individuals with RS186,190
for peer social interaction.252 Furthermore, this strength of and tuberous sclerosis.123 Allopurinol has been used for
sociability in children with Down syndrome contributes to individuals with LNS to prevent urological complications,
the child’s learning through modeling and peer collaboration. although it has no effect on the progressive neurological
A knowledgeable, observant therapist can use peer groups to symptoms.172 The use of large, potentially toxic amounts of
motivate and model for a child with Down syndrome but vitamins and minerals (the orthomolecular hypothesis) has
should also recognize that the child may be distracted by been proposed for children with many different types of
other children and default to a social strategy and avoid the developmental disabilities. This approach has been rejected
task at hand.252 for children with Down syndrome on the basis of the results
of several investigations. In addition, supplementation of
MEDICAL MANAGEMENT AND GENETIC individual metabolites such as 5-hydroxytryptophan or pyri-
COUNSELING doxine for children with Down syndrome is ineffective.255
The physical or occupational therapist should have general Pharmacogenetics is a new field of scientific research
knowledge of both medical management of children with that helps provide a biochemical explanation for why some
genetic disorders and genetic counseling for family mem- patients respond well to a medication and others with the
bers. This information allows the therapist to answer the same condition being treated do not. “Personalized medi-
family’s general questions and to refer family members to cine” is the concept in which doctors may make decisions
the appropriate persons for more specific information. regarding which medications, dosages, and combinations
with other drugs to prescribe based on the analysis of
Medical Management selected genes in their patients.15
Early detection of genetic disorders has improved the health
and survival of individuals with certain genetic disorders Cell Therapy
such as PKU, hemophilia, and CF. Medical treatment for Hematopoietic stem cell transplantation and enzyme re-
the other disorders is not curative but rather palliative or placement therapy can increase survival in children with
directed at specific associated anomalies. Hurler syndrome.147 Gene therapy could potentially correct
defective genes responsible for disease, but there has not yet
Surgical Intervention been much success in clinical trials. The purpose of gene
The congenital heart defects present in many individuals therapy is to replace missing or mutated genes, change gene
with Down syndrome can in most instances be corrected by regulation, or enhance the “visibility” of disease genes to
cardiac surgery.20 Orthopedic surgery in the form of inser- improve the body’s immune response. Gene therapy trials
tion of intramedullary rods in the tibia or femur may mini- have been approved for use in humans only on somatic cells.
mize the recurrence of repeated fractures associated with A vector carries the gene product to the person’s cells; vec-
OI.115,253 Surgical correction of dystrophic scoliosis may be tors may be either an altered virus, stem cells, or lipo-
warranted in individuals with NFM,131 RS,186,189,191,192,197 or somes.256 Gene therapy is still only experimental and is
Werdnig-Hoffmann disease154 if the deformity is severe and moving along cautiously in the United States. In 1999 and
bracing is not successful. Radiographic screening for atlan- again in 2003, individuals died while participating in gene
toaxial instability in children with Down syndrome can be therapy trials.1
initiated beginning at age 2 years.16 If atlantoaxial instability The oversight of gene therapy falls under the U.S.
is excessive or results in a neurological deficit, a posterior Department of Health and Human Services, which oversees
fusion of the cervical vertebrae is recommended.33 Surgical agencies that in turn are responsible for establishing
removal of obstructive or malignant tumors is advisable in research protocols (National Institutes of Health [NIH]),
certain cases of NFM, as is removal of cerebral nodular evaluating investigational gene products (Food and Drug
Administration), monitoring ethics (Recombinant DNA Ad- medical diagnosis of the child’s disorder. In the case of a
visory Committee), and educating human subjects (Office suspected chromosome abnormality, this usually involves
for Human Research Protections).257 The clinical develop- determining the karyotype of the child and possibly the
ment of a gene product that could be widely dispensed must karyotypes of the parents. Other diagnostic procedures may
include four phases: phase 1 consists of regulatory approval include a medical examination, FISH, DNA studies, bio-
of the protocol and then human pharmacology focusing on chemical studies, muscle biopsy, and other laboratory tests.
safety and tolerability and pharmacokinetics; phase 2 exam- A pedigree or family tree is constructed of all known
ines the effectiveness in terms of dose and regimen and tar- relatives and ancestors of both parents.260 Pedigree informa-
get populations; phase 3 determines a basis for licensure and tion includes the age at death and cause of death of ances-
marketing of the product; and phase 4 establishes therapeu- tors, a history of stillbirths and spontaneous abortions, and a
tic use in a wider population.257 As of November 2011 the history of appearance of any other genetic defects or
U.S. Food and Drug Administration had not approved any unknown causes of intellectual disability. The country of
human gene therapy products for sale,212 but the United origin of ancestors is also important because certain genetic
States led all others in the numbers of initiated proto- defects, such as PKU, are far more prevalent in families of
cols.258,259 Approximately 600 gene therapy protocols have a particular ethnic origin. Once the defect has been identi-
been initiated in the United States, most in the area of cancer fied and a pedigree constructed, Novitski260 advises that
research.1 In the United States about 50 therapy protocols further information be obtained from one of the comprehen-
have focused on treating nine different single-gene disorders sive resource texts on genetic disorders. Informing family
including CF, Duchenne muscular dystrophy, hemophilia B, members about the characteristics of the disorder and its
and mucopolysaccharidosis.258 To date, a gene therapy pro- natural history may diminish fears of the unknown.
tocol is underway for Duchenne muscular dystrophy but not The third procedure in genetic counseling is to estimate
for any of the disorders described in this chapter. The inter- the risk of recurrence of the disorder.261 In specific gene
ested reader can obtain an up-to-date listing of current gene defects, the probability of recurrence is fairly straightfor-
therapy protocols from the NIH’s Genetic Modification ward, with a risk of 25% for autosomal recessive disorders
Clinical Research Information System (GeMCRIS) on the and a 50% risk for each male child in sex-linked disorders.
World Wide Web at www.gemcris.od.nih.gov/Contents/GC_ These percentages, however, do not hold true in cases of
HOME.asp. spontaneous mutations. In cases of chromosomal abnor-
In light of the limited medical treatment strategies avail- malities, such as Down syndrome, karyotyping is mandated
able for children with genetic disorders, the physical or oc- to determine whether the child has the translocation type of
cupational therapist must be concerned with maximizing Down syndrome. In that case the risk of recurrence is much
the child’s developmental or functional potential within the greater than with a history of standard trisomy 21 Down
limitations imposed by the lack of possible cures and the syndrome.
prospect of the shortened life span that characterizes many Informing parents of the probability of recurrence is the
of these disorders. When deterioration of skills is expected, next procedure. Novitski260 points out the common misun-
therapy must be directed at maintaining current functioning derstanding that if a risk is one in four for a child to be
levels, minimizing decline, and minimizing caregiver sup- affected, as in an autosomal recessive disorder, many par-
port as much as possible. ents assume that if they have just given birth to a child with
the disorder, the next three children should be normal. It is
Genetic Counseling important to explain that each subsequent child faces a one
Developmental physical or occupational therapists must in four risk of inheriting the disorder regardless of how
have an understanding of the modes of inheritance of the many siblings with the disorder have already been born.
various genetic disorders and information about the services Estimating the risk of multifactorial disorders is a complex
that can be offered through genetic counseling. Although the process. Although these conditions tend to cluster in
physician has primary responsibility for informing the par- families, there is no clear-cut pedigree pattern. The risk of
ents of a child with a genetic disorder about the availability recurrence of a multifactorial disorder is typically low, but if
of genetic counseling, the close professional and personal a couple has had two children with the same condition,
relationships that therapists often develop with families may the recurrence risk is presumed higher, with either a high
prompt family members to seek this type of information genetic susceptibility or a chronic environmental insult
from the therapist. suspected.
Although a physical or occupational therapist cannot fill The fifth step in genetic counseling is for the parents to
the role of a qualified genetic counselor, it is important that decide on the course of action they will take for future preg-
therapists be aware of the availability and location of genetic nancies once the counselor has presented all available facts
counseling services so that they may be assured that parents to them.261 Some parents may choose not to have any more
of a child with a genetic disorder have this information. children; others may elect to undergo prenatal diagnostic
Most major university-affiliated medical centers provide procedures for subsequent pregnancies. These decisions rest
genetic counseling. entirely with the parents and may be influenced by their
individual religious or ethical preferences.
Process of Genetic Counseling Follow-up counseling and review of the most recent ad-
Six steps or procedures in genetic counseling were intro- vances in medical genetics are the final steps in the genetic
duced in the 1970s by Novitski; they included descriptions counseling procedure.260 Genetic counseling can play an
of various genetic tests and a clinical interview.260 The de- important role in opening channels of communication
sired outcome of genetic counseling is to make an accurate among parents, other family members, and their friends;
connecting parents and siblings to support groups; and help- hospitalization (usually the emergency department) than in
ing families to address their grief, sadness, or anger.262 The mothers of children with normal screening results.271 In the
effect of a child’s disability on the family may modify the case of a positive screening result, infants will typically
parents’ earlier decision to have or not to have more chil- undergo more definitive genetic testing.
dren. Recent medical advances may allow a more certain
prenatal diagnosis of specific genetic disorders.263,264 Genetic Testing in Infants and Children
Many genetic disorders can be diagnosed by clinical criteria
Early Detection of Genetic Conditions specific to that disorder. If a diagnosis cannot be made on
Diagnosis of many genetic disorders is made clinically, as in the basis of the patient’s clinical presentation, then genetic
observation of a congenital malformation; however, many testing may be warranted. There are currently about
serious conditions are not immediately apparent after birth. 900 genetic tests that can be offered by diagnostic laborato-
Detection of genetic conditions is performed through vari- ries; specific information can be found at www.genetests.
ous screening procedures, followed by specific diagnostic org. In the United States, the standards and methods of all
testing to confirm a suspected disorder. With technological laboratories performing clinical genetic tests are governed
advancements in genetics, these procedures have been ex- at the federal level.265
panded for the unborn and the newborn. Couples planning
to have children can be tested for specific genetic disorders Prenatal Testing
before conception or embryonic implantation.265 Health care Tests to diagnose a genetic disorder in a developing fetus
professionals and parents should be informed about both the can be placed into two broad categories: invasive and
positive and the negative aspects of using this new knowl- noninvasive procedures. Currently, in contrast to the most
edge and technology. The American College of Medical common invasive procedures, noninvasive methods typically
Genetics has published lists of the more common reasons cannot permit a definitive diagnosis, but they can be per-
for genetics referral as guidelines for health care providers formed with less risk to the fetus. Invasive procedures are
working with infants, children, or couples planning to have recommended in cases of high risk for a serious disorder,
children.266 when definitive diagnosis could lead to treatment, and to
allow parents to make decisions about the pregnancy.272 The
Newborn Screening ethical implications for prenatal testing are many. Parents
Routine newborn screening is required practice in the are often given information that requires a sophisticated
United States. Screening is performed on whole populations understanding of biology and medicine to fully understand
for common disorders. The purpose of screening is the early the implications and results of a diagnostic procedure. For
identification of infants who are affected by a certain condi- example, amniocentesis can detect many chromosomal
tion for which early treatment is warranted and available. Of abnormalities, but the functional outcome of some disorders
the 4 million newborn infants screened each year, approxi- can have great variety.273
mately 3000 have detectable disorders.267 Currently all Invasive Procedures. The most common prenatal diag-
50 states require screening for three disorders: PKU, con- nostic procedure is amniocentesis, which is used to detect
genital hypothyroidism, and galactosemia.140 Some popula- early genetic disorders in the fetus at 11 to 20 weeks’ gesta-
tions known to be at higher risk of certain disorders may be tion.272 This method involves inserting a long, slender nee-
screened automatically, or individuals may elect state- dle through the mother’s abdominal wall and into the pla-
specific screening.267 Most states screen for eight or fewer centa to extract a small amount of amniotic fluid.274
disorders.268 Tandem mass spectrometry (MS/MS) is a labo- Laboratory tests of amniotic fluid reveal all types of chro-
ratory technique that allows for the identification of several mosome abnormalities and a number of specific gene de-
metabolic disorders using a single analysis of a small blood fects, including LNS, and some disorders of multifactorial
sample drawn from the neonate. Many states use MS/MS for inheritance, such as neural tube defects. This procedure
newborn screening for various disorders and have expanded carries a risk of miscarriage of about 0.5% to 1.7%,275 and
their list of those that are mandated and those that are part the risk increases the earlier that it is performed.272
of limited pilot programs.269 Some genetic screening is per- Chorionic villus sampling involves extracting and exam-
formed primarily for research purposes when the disorder is ining a portion of the placental tissue. It has nearly a 99%
not preventable, for example, type I diabetes. Screening for detection rate for chromosomal abnormalities,272 and it can
type I diabetes is available in some states, and early reports be definitive earlier than amniocentesis; however, the risk of
are that 90% of parents consent to the test.270 severe limb defects (amniotic band syndrome) increases the
Benefits of newborn screening are earlier definitive earlier that it is performed. The miscarriage rate with this
diagnosis and medical intervention for the affected child. procedure is estimated to be 0.5%.272
Concerns about expanded newborn screening include hasty Noninvasive Procedures. Ultrasonographic examina-
medical decisions before conclusive evidence is available, tion of a fetus has been used to identify congenital malfor-
and parental stress because of a lag time between screening mations since 1956. It is currently offered to most women in
and definitive results. A study by Waisbren and colleagues271 the United States. It is currently believed that there is no
conducted on parents of children screened for biochemical inherent risk from this procedure. First-semester sonogra-
genetic disorders recognized that parents generally reported phy is performed mainly to confirm the gestational age, to
less stress the earlier a diagnosis could be made. However, identify multiple pregnancy, and to measure nuchal thick-
in the same study, in cases in which the test yielded a false- ness (NT). NT is a measure of the subcutaneous space be-
positive result, parents reported a higher stress index tween the skin and the cervical spine in the fetus; increased
and their children were twice as likely to experience NT is often associated with trisomies. Second-trimester
ultrasonography can detect problems in the quantity of statutes that contain confidentiality provisions, but these
amniotic fluid, large fetal structural defects, and certain provisions are often subject to exceptions.268
smaller defects associated with a genetic disorder. A defini- Traditionally in pediatric medicine, parents are presumed
tive diagnosis is not made on the basis of the presence of to be best suited to make the decision whether to pursue
small defects alone, but the findings are considered along genetic testing. Organizations such as the American Acad-
with the other risk factors present.276 Again, there are ethical emy of Pediatrics (AAP) have argued that parental auton-
questions about the risk to the parents (emotional stress and omy should not be absolute in cases of life-threatening situ-
uncertainty) versus the benefits of early detection. ations coupled with clear medical treatment benefit, but the
Tests of maternal serum screening done at about 15 to AAP supports efforts to make informed parental consent a
20 weeks’ gestation can detect chromosomal abnormali- standard in the United States. Furthermore, the AAP does
ties, but the accuracy depends on many factors, such as not support the broad use of carrier screening in children or
gestational age, maternal weight, ethnicity, multiple preg- adolescents or the position that newborn screening should
nancy, maternal type I diabetes, and maternal smoking.272 be used to identify carrier status in parents of newborns
Finally, it is possible to perform cytogenic analysis of fetal identified as having disorders through newborn testing.2 The
blood cells that can be isolated from a sample of the American Society of Human Genetics has recommended
mother’s blood, but this requires expensive equipment and that family members not be informed of misattributed pater-
expertise.272 nity revealed through testing for the purpose of screening for
disorders and that informed consent should include cautions
Assisted Reproductive Technology and regarding the unexpected finding of a different disease.280
Preimplantation Genetic Diagnosis Pediatricians and other health care professionals should
Couples who want to conceive often seek genetic counseling be prepared to equip families with the appropriate informa-
if one or more parents is aware of a familial genetic condition to use in the decision-making process about genetic
tion, if they are having difficulty conceiving, and commonly testing. From a medical standpoint, Ross and Moon281 pro-
in cases of advanced maternal or paternal age. More than pose a decision algorithm that weighs the risks and benefits
1 million babies have been born worldwide as a result of in of genetic testing. A decision to pursue genetic testing
vitro fertilization (IVF).277 IVF has enabled couples with would be advised if the child was symptomatic, had a sus-
fertility problems to conceive and more recently is used to pected genetic condition, or was from a high-risk family; if
diagnose a genetic disease or condition in an embryo when early diagnosis would decrease morbidity or mortality; and
it has differentiated into just eight cells.278 Chromosomal if the testing method was considered ethical and the testing
abnormalities are the most common detected abnormality, would lead to a beneficial treatment. Lastly, practitioners
and approximately 100 single-gene disorders have been and researchers should be prepared to educate families on
diagnosed.278 The ultimate purpose of preimplantation ge- the protections and limitations of the Genetic Information
netic diagnosis is to implant only mutation-free embryos Nondiscrimination Act of 2008 (GINA). This federal law,
into the mother’s uterus; however, infants conceived with which sets a nationwide level of protection for U.S. citizens,
assisted reproductive technology (ART) are two to four does not preempt state law, which usually provides broader
times more likely to have certain types of birth defects than safeguards. GINA prohibits health insurers from using the
children conceived naturally.277 The reasons for the in- results of predictive genetic testing done for an individual to
creased risk of birth defects is unknown, but it may be that determine policy rates for that individual or for persons in a
ART results more often in multiple births, which are at similar population; this includes information discovered in
higher risk regardless of use of ART.277 Intracytoplasmic the course of medical testing and research. However, it does
sperm injection (ICSI) is another form of ART used often not protect a person’s right to insurance for a genetic illness
in cases of paternal infertility. Male infertility caused by that is diagnosed. GINA prohibits insurers from requesting
azoospermia or oligozoospermia is associated with several or requiring that person undergo a genetic test. It prohibits
genetic factors such as paternal sex chromosome aneuploidy employers from requesting, requiring, or using a person’s
in the case of Klinefelter syndrome.70,279 Preimplantation genetic information in making employment decisions, in-
genetic testing is optional in the United States but recom- cluding information about the employee’s family’s genetic
mended in cases of family history and in men with non information. GINA does not apply to decisions about life,
obstructive azoospermia.279 disability, or long-term care insurance, nor does it apply to
members of the military.282
Ethical, Social, and Legal Issues in Genetics
Advancements in genetics have led to important ethical INTEGRATING GENETICS INFORMATION FOR
questions about testing and screening for genetic disorders PRACTICAL USE IN PEDIATRIC CLINICAL
during the course of a couple’s family planning and after the SETTINGS
birth of the child. Ethical debates about genetic testing are Therapists in all settings frequently find it challenging to
inevitable. The persistent ethical issue in newborn screening keep up with practice issues and the growing body of knowl-
surrounds mandatory or voluntary approaches taken by the edge and evidence in rehabilitative medicine. In clinical
states.2 All states require newborn screening, usually with- settings where most of a therapist’s day is spent in actual
out parental consent for the tests. Thirty-three states have hands-on treatment, the wealth of information that is avail-
newborn screening statutes or regulations that allow exemp- able may seem burdensome and practically inaccessible.
tions from screening for religious reasons, and 13 additional Patients and their families will present the therapist with
states have newborn screening statutes or regulations that many questions about medical interventions, diagnostic pro-
allow exemptions for any reason. The majority of states have cedures, and research. Although therapists know that a
working knowledge of all of these areas is important, often revealed that most professionals are not confident in their
time and access to resources are limited. education and working knowledge in the field of genetics.283
Pediatric therapists know the importance of collaboration Additional studies have indicated that there are not enough
with other professionals, including a type of collective genetic counselors271,281 to meet the growing needs of pa-
knowledge about the child and his or her diagnosis, impair- tients and families and that patients often express the most
ments, functional limitations, and quality-of-life issues iden- stress and dissatisfaction because their primary care physi-
tified by the family. A 1998 survey of individuals from six cian does not appear to be informed about their child’s dis-
different health professions, including physical therapists, order.271,284 See Case Study 13-1, Part 1.
CASE STUDY 13-1, PART 1 n THERAPISTS’ ROLE IN EARLY RECOGNITION

AND CLINICAL DIAGNOSIS OF GENETIC DISORDERS
This case study series portrays how basic knowledge and skills For the female patients discussed in this case, no remarkable
competence in genetics are essential for therapists in the deliv- information was revealed to indicate that a genetic disorder was
ery of services for pediatric patients with genetic disorders. suspect: Each of the girls was the first-born child and lived with
Two female patients with presenting signs of developmental her biological parents, in whom there was no previous family
delay received physical therapy services before and after history of a genetic disorder. This was the first pregnancy for the
receiving a definitive diagnosis. mothers of both girls. The mothers each became pregnant in their
SCREENING FOR GENETIC DISORDERS AT late 20s without the assistance of reproductive technology, and
INITIAL VISIT they received the recommended course of prenatal care without
Developmental delay is a common classification for infants and specialized prenatal testing. The course of the mothers’ pregnan-
young children entering into physical therapy services before a cies and deliveries were unremarkable, as was the neonatal period
definitive diagnosis of a particular genetic disorder has been made. for the children.
Family history and course of pregnancy are key areas for the phys- Both girls were Caucasian with blond hair, blue eyes, and
ical therapists to consider during ongoing assessment. The sensi- absence of notable dysmorphism. Figure 13-9, A and B shows
tive nature of this information requires that appropriate trust and 2 females. The first child was 16 months and the second was
rapport have been established between practitioner and family. 14 months before given a medical diagnosis.
MEDICAL RECORDS REVIEW
DYLEN: CONTRASTING DANIKA: CONTRASTING FEATURES OF BOTH

FEATURES FEATURES GIRLS
0-6 MONTHS OF AGE
Parental concerns Vomiting and abnormal eye Low muscle tone and slow motor
movements development.
Tests and measures Normal brain MRI scan and No tests
EEG at 3 months
Development Normal head control. Purposeful reach,
grasp, and object release.
Good eye contact and “happy”
disposition. Some babbling and
one-syllable sounds.
6-8 MONTHS OF AGE
Parental concerns Tongue thrusting and episodic Sleep disturbances Visual Both girls are slow to achieve
nystagmus; parents suspect disturbance suspected independent sitting and crawling.
seizures
Tests and measures
Development Pivots in prone position at Not yet sitting. Delayed trunk control. Both girls roll
8 months. over by 7 months.
Sits without support at 8 months.
EEG, Electroencephalogram; MRI, magnetic resonance imaging.
Pediatric therapists should recognize that marked developmen- factors: no dysmorphic features, healthy neonatal development,
tal delay and global hypotonia are two features common to absence of family history of genetic disorders, and no other
many genetic disorders. However, in the case of both girls, the major presenting risk factors (e.g., no prior loss of pregnancies,
urgency of a genetics referral was lessened by the following young maternal age, and normal course of pregnancy).
Family-Centered Service
Family-centered service is both a philosophy and an ap-
proach to service delivery that is considered to be a best
practice in early intervention and pediatric rehabilita-
tion.290-292 Children with genetic disorders have complex,
long-term needs that can be addressed by a family-centered
service delivery model. At the core of this model is the man-
ner in which therapists interact with the children and their
families—the therapists’ mindfulness, attentiveness, and
respectfulness, elements that are as important as the actual
interventions delivered.293,294 Therapists educated in the
family-centered approach are also able to understand the
impact of disability on a family as well as the value of
A B support systems such as family and community.262
Bailey and colleagues262 highlight the particular needs of
Figure 13-9 n A, Dylen at 16 months of age. B, Danika at families who have children diagnosed with genetic disorders
14 months of age. to have productive partnerships with health care providers.
Therapists should not be reluctant to learn about a rare con-
dition from parents, as many are not “passive recipients of
Basic Knowledge and Skills Competence information” but rather “co-producers” of what and how
for Physical and Occupational Therapists information available may be used in their child’s care.262,295
The National Coalition for Health Professional Education in Parents can be trusted to be a reliable source in the recogni-
Genetics (NCHPEG) is an organization of individuals from tion of their child’s condition and needs,296 but in some
approximately 120 health profession. They have proposed instances the therapist’s role may be to steer families to
basic competencies for all health care professionals.7 With a accurate information or assist with interpreting information
working knowledge about genetics, therapists can develop which they have discovered. For example, the term “untreat-
competence in eliciting and accessing genetic information able condition” may be misinterpreted by parents to mean
from subjective interviews with proper patient consent, can that there are no reasonable interventions that may benefit
learn how to protect patient privacy while making appropri- their child (see Case 13, Part 4).
ate recommendations to genetics professionals, and can The Relational Goal-Oriented Model (RGM) of service
understand the social and psychological implications of delivery links the “what” with a more in-depth consideration
genetic services.283,285 of the “how” (how service providers and organizations can
Professional education for physical therapists3 and occupa- optimize both the process and outcomes of service deliv-
tional therapists286 faces challenges to prepare practitioners ery).297 The role of the family in the child’s life and the impor-
who meet the minimum competencies set forth by NCHPEG.7 tance of the insights of parents into their child’s abilities and
Most physical therapists responding to the survey by Long and needs298 are crucial. Three important aspects of caregiving—
colleagues283 reported that they received most of their infor information exchange, respectful and supportive care, and
mation through nonscientific media and that they had limited partnership or enabling—are foundational to family-centered
or no education in genetics. Some of the barriers to implemen- care.299 Family-centered service recognizes that each family is
tation of genetics content in professional programs include unique, that the family is the constant in the child’s life, and
lack of faculty qualified to teach the content and time limita- that the family members are experts in the child’s abilities and
tions within a didactic program.3 Continuing education courses needs. The family works together with service providers to
for practicing clinicians are in short supply. Physical therapists make informed decisions about the services and supports the
have identified needs for continuing education in genetics to child and family receive. The strengths and needs of all family
include topics such as the role of genetics in common disor- members are considered in family-centered service.300 In the
ders such as cancer and heart disease, an overview of human interactional exchange between the child and family and the
genetics, what treatments were available, and how to direct therapists, understandings occur, commitments and decision
clients to information resources. Although occupational thera- are made, the child and family receive affirmation and support,
pists were not part of Long’s study, it is felt that colleagues and information is translated into meaningful, usable knowl-
would stress similar needs.283 edge through the process of communication.301,302 Developing
mutual trust and open communication among the child, the
Service Delivery for Children with Genetic family, and the physical and occupational therapists as well as
Disorders and Their Families other practitioners is at the core of clinical practice.
For therapists to be supportive of families they are working Therapists working with children need to recognize and
with, they must acknowledge the importance of family priori- acknowledge the multitude of tasks that all families work to
ties, respect the family’s cultural values and beliefs,287-289 in- accomplish. In addition to tasks specifically related to caring
clude families as integral team members, and promote and for a child with a disability, families must perform functions
deliver services that build on family and community resources. to address the economic, daily care, recreational, social, and
This section includes strategies for supporting families of educational and vocational needs of both individual mem-
children with genetic disorders, assessment strategies, con- bers and the family as a whole.
struction of therapeutic goals and objectives, and guiding As Turnbull and Turnbull303 have cautioned, each time
principles for pediatric interventions. professionals intervene with families and children, they can
potentially enhance or hinder the family’s ability to meet important to understand these differences and the intended
important family functions. For example, intervention that purpose for each type of assessment to ensure that evalua-
promotes a child’s social skills can be an important support tion tools are used appropriately. A list of tests and measures
to positive family functioning. On the other hand, interven- commonly used by pediatric physical therapists is summa-
tion that focuses on the child’s deficits can have a negative rized in Table 13-4.
impact on how the family perceives that child and the place
of the child in the family. The RGM emphasizes the impor- Discriminative Assessment
tance for therapists to join with parents to provide respon- A discriminative assessment is used to compare the ability
sive and flexible therapy services in accordance with chang- of an individual with the ability of members of a peer group
ing family needs and circumstances.304 or with a criterion selected by the test author.309 Such instru-
The Life Needs Model292,297 acknowledges the need ments provide information necessary to document children’s
for therapists to work collaboratively with service providers eligibility for special services but rarely provide information
in other disciplines to improve community participation useful for planning or evaluating therapy programs.310
and quality of life for children and youth with disabilities, Norm-referenced tests such as the Alberta Infant Motor
based on the expressed needs of the child and his or her Scale,311 the Bayley Scales of Infant Development (motor
family members. Assisting the family in identification of and mental scales),312 and the Peabody Developmental
a support group is often helpful for adjustment and continu- Motor Scales313 are examples of tests used with infants and
ing encouragement in coping with issues. Support groups young children to verify developmental delay or to assign
can be found at www.geneticalliance.org, a comprehensive age levels. The Test of Infant Motor Performance is used
website provided by the Alliance of Genetic Support to identify the risk of developmental delay in infants from
Groups. Family empowerment mediates relationships between 32 weeks postconception to 16 weeks after term.314 An ex-
family-centered care and improvements in children’s behav- ample of a norm-referenced assessment tool for older chil-
iors305 and directly affects families’ satisfaction with ser- dren is the Bruininks-Oseretsky Test of Motor Proficiency.315
vices for their children and their well-being.306 It may be possible to detect improved motor performance
by administering a developmental test used to identify chil-
Assessment Strategies dren who have motor delays. Such tests, however, usually
Knowledge of a child’s diagnosis can aid in the selection of cannot detect small increments of improvement because
appropriate assessment tools and can alert the therapist to any there are relatively few test items at each age level and de-
potential medical problems or contraindications associated velopmental gaps between items are often large. In assess-
with the specific syndrome that might affect the assessment ing whether intervention has been effective, the use of most
procedures (tests and measures). Therapists must be careful, discriminative tools does not examine a child’s performance
however, not to develop preconceived opinions about a child’s of functional activities in natural environments.310
capabilities on the basis of how other children with similar
diagnoses have performed. It is critical to remember that there Predictive Assessments
is wide behavioral and performance variability among chil- Predictive measures are used to classify individuals accord-
dren within each genetic disorder. For example, wide vari- ing to a set of established categories and to verify whether
ability in the achievement of developmental milestones has an individual has been classified correctly.309 Measures
been reported among children with Down syndrome.307 designed to predict future performance are often used to
The assessment process includes many components that detect early signs of motor impairment in infants who are at
in certain areas are specific to the practice of either physical risk for neuromotor dysfunction.310 Knowledge of develop-
or occupational therapy. For the physical therapist, use of mental milestones and the ability to identify typical and
the Guide to Physical Therapist Practice308 is recommended atypical movement at various ages is paramount to the
as a framework to identify appropriate tests and measures therapist’s competency in administering a structured assess-
for impairments or disabilities. For the occupational thera- ment such as those used to predict future disability in chil-
pist a useful reference is the assessment section of the text- dren. Prechtl and others316 have described how assessment
book Occupational Therapy for Children.237 of “general movements” in infants can be used to identify
Typically a therapist’s assessment begins with movement children with cerebral palsy.317 The Movement Assessment
observation and analysis followed by testing of the neuro- of Infants318 was designed to assess muscle tone, reflex de-
muscular status of the child, such as primitive reflexes, au- velopment, automatic reactions, and volitional movement
tomatic reactions, and muscle tone. For children with ortho- and has value in predicting future neurodevelopmental prob-
pedic involvement, assessment of muscle strength, joint lems in high-risk infants when administered during the first
range of motion, joint play, and soft tissue mobility is also year of life.319,320 The Test of Infant Motor Performance314
important. An assessment of the child’s developmental level and the Alberta Infant Motor Scale311 are other instruments
and functional ability should be completed. Such assess- commonly used to predict poor motor outcomes.
ments can be used to discriminate between typical and
delayed development, to identify the constraints interfering Evaluative Assessments
with the achievement of functional skills, and to guide the An evaluative measure is used to document change within
development of treatment goals and strategies. Most devel- an individual over time or change occurring as the result of
opmental assessment tools fall into one of the following intervention.309 Helping Babies Learn321 is a curriculum-
categories: (1) discriminative, (2) predictive, and (3) evalua- referenced test that provides information about a child’s
tive measures.309 Each of these three types of developmental developmental progress relative to a prespecified curriculum
assessment tools yields a different type of information. It is sequence.
TABLE 13-4 n TESTS AND MEASURES COMMONLY USED IN PEDIATRIC PHYSICAL THERAPY
TESTS AND MEASURES AGE RANGE PURPOSE

311
Alberta Infant Motor Scale (AIMS) Birth to 18 Identifies motor delays and measures changes in motor performance
months over time
Batelle Developmental Inventory359 Birth to 8 years Identifies developmental level and monitors changes over time
Canadian Occupational Performance Any age Identifies changes in parent’s perception or child’s self-perception of
Measure (COPM)360 performance over time
Bayley Scales of Infant Development, 1 to 42 months Identifies developmental delay in gross motor, fine motor, and cognitive
ed 2 (BSID-II)312,361 domains; monitors progress over time
Berg Balance Scale362 5 years and older Performance-based measure of balance during specific movement tasks
Bruininks-Oseretsky Test of Motor 4.5 to 14.5 years Identifies motor abilities and can be used for program planning; moni-
Proficiency (BOTMP)315 tors change over long periods of time for child with mild disabilities
Childhood Health Assessment Any age Measures quality of life from patient’s or parents’ perspective
Questionnaire (CHAQ)363
Child Health Questionnaire364 2 months to Measures quality of life from patient’s or parents’ perspective
15 years
Denver Developmental Screening Test II365 2 weeks to Screening tool for developmental delay
6.5 years
Early Intervention Developmental Birth to 3 years Measures development of gross and fine motor, language, perception,
Profile366 social, and self-care skills
Energy Expenditure Index (EEI)367 3 years and older Measures endurance level for activity; monitors changes over time
Functional Independence Measure 7 years and older Measures changes in mobility and activities of daily living skills; used
(FIM)323 for program evaluation and rehabilitation outcomes assessment
Functional Independence Measure for 6 months to Measures changes in mobility and activities of daily living skills; used
Children (WeeFIM II)324 7 years for program evaluation and rehabilitation outcomes assessment
Functional Reach Test (FRT)368 4 years and older Measures anticipatory standing balance during reach
Goal Attainment Scale369 Any age Individualized goal-setting format to detect small, patient-relevant
changes over time
Gross Motor Function Measure 5 months to Measures change in gross motor function over time
(GMFM)157 16 years
Harris Infant Neuromotor Test (HINT)370 Birth to 12 months Screening tool to detect early signs of cognitive and neuromotor delay
Health Utilities Index Mark 3371 Any age Measures child’s functional health status; computes cardinal utility value
that represents health-related quality of life
Modified Ashworth Scale (MAS)372 4 years and older Qualitative measurement of spasticity through resistance to passive joint
movement
Modified Tardieu Scale373,374 4 years and older Qualitative measurement of spasticity through resistance to passive joint
movement
Movement Assessment of Infants (MAI)318 Birth to 12 months Identifies motor abilities or dysfunction
Peabody Developmental Motor Scales Birth to 5 years Identifies gross and fine motor delays; used to monitor progress
(PDMS-2)313
Pediatric Clinical Test of Sensory 4 to 10 years Measures sensory system contributions to standing balance and postural
Integration for Balance (P-CTSIB)375 control
Pediatric Evaluation of Disability 6 months to Measures self-care and mobility performance in the home and commu-
Inventory (PEDI)325 7.5 years nity; monitors progress over time
Pediatric Outcomes Data Collection 0 to 19 years Assesses overall health, pain, quality-of-life components, and participa-
Instruments (PODCI)376 tion in ADLs (and sports in older children)
Pediatric Quality of Life Inventory 2 to 18 years Parent report and/or child self-report measuring health-related quality of
(PedsQL)377 life (HRQOL) in healthy children, adolescents, and those with acute
and chronic health conditions
School Function Assessment (SFA)326 Kindergarten to Measures function in the school environment
6th grade
Sensory Integration and Praxis Test378 4 to 9 years Measures sensory systems’ contribution to balance and motor coordination
Sensory Profile379 3 to 10 years Determines which sensory processes contribute to child’s performance
of activities of daily living
6-Minute Walk Test380 5 years and older Measures walking endurance; monitors progress over time
Test of Infant Motor Performance 32 weeks to Provides early identification of motor delay; assesses posture for early
(TIMP)314 4 months skills acquisition
Test of Sensory Function in Infants381 4 to 18 months Identifies sensory processing dysfunction and those at risk for develop-
mental delay or learning problems
Timed Up and Go (TUG)382 4 years and older Performance-based measure of anticipatory standing balance, gait, and
motor function
Toddler and Infant Motor Evaluation 4 months to Identifies children with mild to severe motor problems; measures
(TIME)383 3.5 years sensory development; monitors progress over time
ADLs, Activities of daily living.
To determine whether a child’s ability to perform mean- areas of self-care, sphincter control, mobility, locomotion,
ingful skills in everyday environments has improved, a communication, and social cognition.323 Seven levels of
functional assessment should be used. Functional assess- functional dependence ranging from needing total assistance
ments focus on the accomplishment of specific daily activi- to complete independence are used to determine an indi-
ties rather than on the achievement of developmental mile- vidual’s status. An adaptation of the FIM places greater
stones. Emphasis is placed on the end result in terms of emphasis on functional gains as opposed to the level of care.
the achievement of a functional task, although the form or The WeeFIM324 has been developed for use with children
quality of the movement should never be ignored by the through the age of 6 years.
therapist. Assistance in the form of people or devices is The Pediatric Evaluation of Disability Inventory (PEDI)
incorporated into the assessment of progress, with the mea- is a functional assessment that focuses on the domains of
surement of progress focusing on the achievement of inde- self-care, mobility, and social cognition.325 The PEDI incor-
pendence.322 Qualitative aspects of movement that have im- porates three measurement scales: (1) the capability to
portant functional implications, such as accuracy, speed, perform selected functional skills, (2) the level of caregiver
endurance, and adaptability, are also considered. assistance that is required, and (3) identification of environ-
Functional assessments can be used to screen, diagnose, mental modifications or equipment needed to perform a
or describe functional deficits and to determine the re- particular activity. The PEDI has been standardized and
sources needed to allow the child to function optimally in normed and is intended for use with children whose abili-
specific environments (e.g., school, home). Another use of ties are in the range of a typical 6-month-old to 7-year-old
functional assessments is to evaluate the nature of the prob- child.
lem and the specific task requirements limiting function to The final example of a functional assessment is the
develop educational plans and teaching strategies.322 A final School Function Assessment (SFA).326 The SFA is designed
use of functional assessments is to examine and monitor for to measure a student’s performance in accomplishing func-
changes in functional status. Such assessments can be used tional tasks in the school environment. It is composed of
for program evaluation and for determining the cost- three sections that focus on (1) the student’s participation in
effectiveness of services or programs. (See Chapter 8 for major school activities, (2) the task supports needed by the
additional information regarding evaluation tools.) student for participation, and (3) the student’s activity per-
The Functional Independence Measure (FIM) is an ex- formance. The SFA is standardized and was conceptually
ample of a functional assessment. The FIM assesses the developed to be reflective of the functional requirements of
effectiveness of therapy on functional dependence in the a student in elementary school. See Case Study 13-1, Part 2.
CASE STUDY 13-1, PART 2 n ASSESSMENT STRATEGIES

DYLEN: CONTRASTING DANIKA: CONTRASTING
FEATURES FEATURES FEATURES OF BOTH GIRLS
9-13 MONTHS OF AGE
Tests and Both girls have abnormal EEG findings
measures Alberta Infant Motor Scale score: below
5th percentile
PEDI: Composite independence in func-
tional skills less than 12% with total
caregiver assistance
Development Regression in motor skills: sitting, roll- Sits alone at 11 months Dylen demonstrates skill regression;
ing, and fine motor Danika slowly attains motor skills.
Babbling vocalization
Emergence of truncal and extremity
ataxia
Other objective Cold feet with reddened appearance; Emergence of hand- Both girls have small head
findings normal BP and HR flapping behaviors circumference.
BP, Blood pressure; EEG, electroencephalogram; HR, heart rate; PEDI, Pediatric Evaluation of Disability Inventory.
Another role of the therapist is to use assessment instruments motor, language, and self-help skills persisted, and ultimately
that will help establish a baseline of motor and self-help skills both girls and their families were referred for genetic evalua-
and to monitor for progress or regression; the Alberta Infant tion. Both girls received 1 to 2 hours of physical therapy weekly
Motor Scale and the PEDI, respectively, were initially used for in their natural environment. The Peabody Developmental
these purposes. Lastly, the results of these tests and outcomes Motor Scales were added to monitor progress, guide goal devel-
of intervention are interpreted and conveyed to the family and opment, and justify the need for continued early intervention
the child’s pediatricians. In the case of both girls, delays in services.
Family-Driven Goals and Objectives revisions in 1990 (IDEA),335-337 1991 (PL 102-119), 1997
(PL 105-17), and 2004 (PL 108-446, Individuals with
Therapy Goal Orientation Disabilities Education Improvement Act of 2004),338
Goal orientation is a second fundamental feature of effective physical and occupational therapists working in public
service. The earlier section on relation-based practice school settings are required to establish long-term annual
described how relationships among child, family, and thera- goals and short-term therapy objectives within the frame-
pists are fundamental in providing effective intervention. work of each child’s educational needs. The document
Goal orientation encompasses both joint goal setting by that defines a child’s educational needs, including therapy
(1) parents, caregivers, and families and (2) therapists and services, from preschool to twelfth grade is the IEP. Simi-
other service providers327 and the pursuit of meaningful lar requirements are in effect for infants to preschool-age
child-, parent-, and family-selected goals.328 Goals of par- children, documented in the individualized family service
ents and families are to create a supportive environment for plan (IFSP). An IFSP must be written after a multidisci-
their child, provide opportunities for growth and belonging, plinary assessment of the strengths and needs of the child
and assist their child to live as adaptive and independent a has been completed. This assessment must include a
life as possible.297 Family-centered care incorporates trust- family-directed assessment of the supports and services
ing relationships in which the therapist demonstrates respect necessary to enhance the family’s capacity to meet the
for the family’s values, beliefs, and goals rather than impos- needs of their child with a disability.337,338 A comparative
ing a plan of care on the child and family that aims to correct table of the components of an IEP and IFSP is found in
“deficiencies.” Table 13-5.
After a child’s strengths and needs have been evaluated
and the family’s objectives identified, therapy goals and Functional Objectives
objectives can be developed. In the past, establishment of The development of behaviorally written, measurable ther-
these goals has primarily been the responsibility of profes- apy objectives is crucial for monitoring the effects of inter-
sionals and often did not incorporate the needs and desires vention in a child with a genetic disorder. Many of the
of the family. More recently, however, professionals have clinical symptoms listed in the descriptions of genetic disor-
recognized the value of having families guide the process of ders described earlier in the chapter may be monitored
establishing intervention goals and objectives.329,330 This through systematic, periodic, data-keeping procedures. One
shift toward collaborative goal setting and family-centered example is the monitoring of functional hand skills in girls
care has occurred largely as a result of the belief that fami- with RS (see Case Study 13-1, Part 2). Periodic vital capac-
lies should determine their vision of the future for their ity measures for a child with OI or a child with Werdnig-
children and that professionals should act as consultants and Hoffmann disease can reflect progress toward a goal of
resources to assist families in achieving that vision. The maintaining respiratory function.
stress that caregivers experience with the everyday care of a Typically, in the past, therapy objectives focused on a
child can reduce compliance with a home therapeutic pro- child’s deficits. For example, delays in achieving motor
gram,331 which further supports the notion that parents milestones are often used to identify gaps in development,
should be jointly involved with therapists to determine goals and therapy objectives are written and programs established
and the means by which to attain identified outcomes.332 to address these deficits. When the child meets an objective,
When parents and families contribute to the planning pro- new deficits are identified and new objectives are developed.
cess, they are more likely to believe in goals that are set and A different model for goal development that is consistent
to play a role in ensuring that relevant strategies are imple- with a family-centered intervention philosophy is the “top-
mented.297 They gain a sense of control over their child’s down” approach, described by Campbell339 and later by
services, supports, and resources that contributes to their McEwen340 and Effgren.208 In this model, the child and fam-
personal and family’s well-being.333 For children living in ily identify a desired functional outcome that is the driving
the United States, these goals are developed within the factor for the therapeutic intervention plan. An example
context of individualized service plans. of this approach is seen in goal attainment scales. Goal
attainment scaling (GAS) is an individualized, criterion-
Individualized Service Plans referenced measure of small, clinically important changes in
In the United States, the Individuals with Disabilities a child’s functional performance over time.341,342 Similar to
Education Act (IDEA) requires public schools to develop an behavioral objectives, GAS requires (1) identification of
Individualized Education Plan (IEP) for every student with observable goals, (2) reproducibility of conditions under
disability. An IEP is designed to meet the unique educational which performance is measured, (3) measurable criteria for
needs of a student with disability as defined by federal success, and (4) a time frame for goal achievement. In con-
regulation 34 CFR 300.320.334 Under IDEA 2004, a free trast to behavioral objectives, however, GAS identifies five
appropriate public education (FAPE) is provided that is possible outcomes with accompanying score values. By us-
individualized to a specific student with a disability and ing five possible levels of attainment, it can be determined
that emphasizes special education and related services to whether a child has made progress despite not having
prepare the student for further education, employment, and achieved the expected outcome or whether progress has
independent living [20 U.S.C. 1400 et seq, 20 U.S.C. 1400 exceeded the expected outcome. Case Study 13-1, Part 3 is
© (5)(A)(i)]. an example of use of a goal attainment scale to assess a par-
Beginning with the enactment of United States Public ent and child functional objective of sitting up on the floor
Law 94-142 in 1975335 and several important legislative to play with toys.
CASE STUDY 13-1, PART 3 n FAMILY-DIRECTED GOALS AND OBJECTIVES

Both girls had poor postural control with muscular weakness toward the goal depicted in this goal attainment scale; how-
and hypotonia that limited the activity of sitting. The parents ever, Dylen did not, and in fact demonstrated regression.
of both girls expressed a desire that their daughters would be Dylen’s poorer outcome was indicative of the diagnosis of
able to sit up and play with toys on the family-room floor. The Rett syndrome (RS) that was revealed later. The family’s goals
table illustrates the use of GAS for the goal of seated play for Dylen were revised, and the GAS was still a suitable
with toys. In the course of care, Danika made good progress framework.
ATTAINMENT LEVEL IN TIME CRITERION-REFERENCED GOALS WITHIN EACH RANGE

SCORE FRAME (INSERT) ______ (TIME FRAME: 3 MONTHS )
22 Much less than expected outcome (Child name) ring-sits on the floor supported on one or two hands plus 25% physical
with therapy assistance for balance support and guided reach and grasp of toys placed near her lap.
21 Less than expected outcome with (Child name) ring-sits on the floor with standby assistance and uses both hands to
therapy play with toys placed near her lap.
0 Expected outcome with therapy (Child name) ring-sits on the floor with supervision, reaches and retrieves toys placed
in front of her at arm’s length away.
11 Greater than expected outcome (Child name) sits on floor independently and plays with toys placed at arm’s length in
with therapy front and to sides of her.
12 Much greater than expected out- (Child name) sits on floor independently and retrieves toys placed slightly out of
come with therapy reach in front, to sides, and 45 degrees behind her and returns to upright for play.
TABLE 13-5n COMPARISON OF REQUIRED COMPONENTS OF THE INDIVIDUALIZED EDUCATION

PLAN (IEP) AND INDIVIDUALIZED FAMILY SERVICE PLAN (IFSP)
IFSP IEP
IDEA PART C IDEA PART B
CONTENT (34CFR303.344) (34CFR300.320 THROUGH 300.324)
Information about A statement of child’s present levels of physical, A statement on child’s present levels of academic and
child’s status cognitive, communication, social or emotional, functional performance
and adaptive development (physical development
includes vision, hearing, and health status)
Family information Statement of family’s resources, priorities, and con- Information regarding parent’s concerns can be docu-
cerns related to enhancing the child’s development mented in the present information about the child’s status
Outcomes Statement of measurable outcomes expected to be Statement of measurable annual goals, including
achieved for child and family and the criteria, proce- academic and functional goals
dures, and timelines used to determine the degree of Include a description of how child’s progress toward
progress toward outcomes and whether modification annual goals will be measured and process to report
or revisions of outcomes or services were necessary child’s progress to parents
Services Statement of specific Early Intervention (EI) Statement of specific special education and related ser-
services necessary to meet child’s needs; include vices to be provided, modifications, and supplementary
frequency, intensity, method of service delivery, aids to be provided to child
location of services and natural environments
Schedule of services Projected dates for initiation of services, anticipated Projected date for beginning and ending date of service;
duration any modification needed; frequency, location, and dura-
tion of services
Service coordinator Identification of the service coordinator No comparable requirement
Transition plan Procedures and steps for transition from EI to Procedures needed for postsecondary goals related to
preschool services under Part B training, education, employment, and, where appropri-
Establish transition plan: 90 days to 9 months before ate, independent living skills
third birthday To be in effect when child turns 16 yr of age
Transfer of rights n/a Must include statement that child has been informed
about reaching the age of majority
Other Explanation of any time the child will not participate
along with nondisabled children
IDEA, Individuals with Disabilities Education Act.
Rather than focusing on a child’s deficits, such outcome- pruning process of the developing brain and frequently used
focused objectives provide a more positive and supportive movement patterns will be reinforced228,343,351; therefore ap-
context for therapy and at the same time address the family’s proaches of this nature may be beneficial for infants and
needs and priorities. This approach to developing therapy very young children with movement disorders.
goals and objectives in ways that support positive family Learning and performance of an activity seldom require
functioning is also an important aspect of delivering therapy just one component of function (e.g., mobility, language,
services to children and their families. cognition); therefore it should be understood and expected
that improvements in one domain may indirectly, but sig-
General Intervention Principles nificantly, have a positive impact on another. For example,
Several general treatment principles guide the delivery of Damiano351 stresses the benefits of a lifetime of regular
therapy services to children with genetic disorders and movement activity, with or without adaptations, on the over-
are detailed in this section. Special considerations for all development of children. Regardless of treatment tech-
treatment of a child with a specific genetic condition may niques, it is a widely accepted principle that children learn
be found in the preceding section. The reader is also new skills best when they are taught and practiced within the
referred to Chapter 9 for information on interventions context in which they will be used.352
in neurological rehabilitation.
Delivery of Services in Natural Environments
Focus on Activities and Participation The term “natural environment” refers to places and settings
The goal of any therapeutic program for children should be to in which infants and children typically spend their day.337
improve the quality and quantity of their participation in soci- The movement toward integrating therapy into classroom
ety. Achievement of basic motor skills such as sitting, stand- settings is one example of providing services in a natural
ing, and walking is an outcome that can be measured with environment.353-355 In an integrated model of service deliv-
commonly used clinical tools, but whether or not children ery, therapists work in the classroom with teachers, rather
actually apply new skills on a regular basis (participation) is than removing students to an isolated therapy room to pro-
more difficult to capture objectively.343 Therapists need to vide services. Therapists work closely with the teacher to
possess knowledge, skills, and tools if they are to assess and establish common goals for the student and to devise pro-
treat children in all domains defined by the World Health grams that will allow therapeutic activities to be interwoven
Organization (see Case Study 13-1, Part 1). With the purpose into a variety of activities throughout the day in a natural
of improving participation in the end, pediatric therapists manner.
employ a variety of intervention strategies to increase oppor- Another example of providing therapy in a natural envi-
tunities for children to achieve independence and enjoyment ronment is providing home-based services for infants and
of activities at home and school and in the community young children. Home-based programs are “normal” op-
Many of the classic therapeutic approaches for children tions for young children because the natural environment for
with neurological disorders incorporate techniques targeting most infants and toddlers is the home—either their own or
impairments of body structure and function, such as abnor- that of a day care provider.353-355 For children who are
mal muscle tone or joint alignment, to improve movement medically fragile, it is the preferred option for therapy.356,357
quality.228 Motor learning science and task-oriented models For other families, transportation to a center-based program
of neurological rehabilitation are based on the rationale that may be difficult because of the expense or length of travel
control of movement arises from appropriate practice required.
of skills within the context of functional activities and en-
riched environments.343-346 Intervention, therefore, is aimed Incorporating Therapy Activities into Daily
at teaching motor problem solving (adaptability to varied Routines
contexts),347 developing effective compensations that are Therapists need to work collaboratively with families to
maximally efficient, and providing practice of new motor develop activities that incorporate therapeutic activities into
skills in functional situations. Rather than teaching individu- the family’s daily routine (e.g., during play, dressing, bath-
als to perform movement patterns in a controlled therapy ing, meals). Rather than practicing narrowly defined tasks in
setting, this approach focuses on the learning that must take a controlled clinic environment, therapy activities should be
place for an individual to function independently of a thera- interwoven into a variety of activities throughout the day in
pist’s guidance.343,345 Environmental adaptations can take a natural manner. Practicing skills in the context of daily
many forms and include assistive technology that aids in the routines allows the child to learn to adapt to the real-life
attainment of functional outcomes such as independence in contingencies that arise during a functional task.345 In addi-
self-help skills, communication, and mobility.348 For exam- tion, activities become more meaningful to both the child
ple, children with Down syndrome commonly have hypoto- and the family (Figure 13-10).
nia, joint laxity, and delayed walking. Orthotics such as
supramalleolar orthoses may be used to improve underlying Use of Assistive Technology Devices
joint and postural instability,349 and treadmill training has The Assistive Technology Act of 2004 defines an assistive
been shown to diminish delays in walking.350 technology device as any item, piece of equipment, or
Modern neurophysiologic approaches use hands-on product system, whether acquired commercially, modified,
physical guidance with the child during movement practice or customized, that is used to increase, maintain, or improve
of functional skills and activities. The inhibition of certain functional capabilities of individuals with disabilities
movements and facilitation of others are based on the ratio- (29 U.S.C. Sec 2202[2]).358 Information for clinicians and
nale that less used movements will be eliminated in the families can be found in Table 13-1.
As noted previously, an important aspect of providing greater variety of movement patterns. Such devices can be
developmental therapy services is the use of assistive tech- constructed from readily available materials or obtained
nology devices to maximize a child’s functional abilities, commercially. The developmental physical or occupational
level of independence, and inclusion in school and commu- therapist works with the family and other team members to
nity activities with peers. Examples of assistive technology select, construct, or order assistive devices and to assist care-
include mobility devices, augmentative communication de- givers in the use of the devices.
vices, and adapted computer keyboards. Assistive technology Case Example 13-1, Part 4 demonstrates how these gen-
also includes adaptive devices such as splints, bath chairs, eral treatment principles are applied to a particular child
prone standers, and other positioning equipment that can be receiving therapy services. The case example also shows
used to provide optimal body alignment and minimize the how the family’s priorities and needs are considered and
risk for contractures or deformities while encouraging a supported in the planning and delivery of services.
CASE STUDY 13-1, PART 4 n FOCUS ON ACTIVITIES AND PARTICIPATION

IN MEANINGFUL ENVIRONMENTS AND ROUTINES
Both families were referred by their respective physicians During the period immediately after their child’s diagnosis,
for genetic counseling and evaluation on the suspicion of the parents in each family shared many questions and new-
AS based on cluster of developmental delay, features of blue found knowledge with the treating therapist. Both families
eyes and blonde hair, head circumference, and abnormal accessed local and national family support groups and were
EEG findings. Danika received an earlier referral to a encouraged to learn of activities that their children would likely
geneticist at 14 months of age; subsequent test results enjoy. The partnership between the therapists and the families
confirmed the diagnosis of AS by the time she was was strengthened by clarifying that “untreatable condition”
16 months old. The course of medical diagnosis for Dylen means that neither condition can be remedied by medical inter-
was confounded by her presenting signs of frequent, intense vention and by focusing on the families’ values and priorities.
vomiting coinciding with suspicion of seizure activity. Both girls had limited postural endurance in sitting and
Dylen did not receive genetic testing until she was standing that was anticipated to persist for several months.
30 months of age. In the cases of both girls, their Adaptive equipment for these activities was employed for both
documented response to therapy and course of motor girls to allow them to participate in functional upright activities
development were weighed by their respective physicians (see Figure 13-7). Danika enjoyed aquatic activities and tricy-
in their course of care and referral for genetics evaluation. cling more than she tolerated hippotherapy. Dylen was found to
AS was ruled out first for Dylen, and subsequent testing enjoy movement activities more when music was employed
confirmed a diagnosis of RS. during treatment.
DYLEN: CONTRASTING DANIKA: CONTRASTING

FEATURES FEATURES FEATURES OF BOTH GIRLS
14-36 MONTHS OF AGE
Diagnosis; body organ or Rett syndrome; gastroesophageal Angelman syndrome; sleep Global developmental delay,
structure and function reflux disorder seizures, hypotonia
Activities, limitations, Motor skills plateaued at Ambulatory with assistive Attends special education
and development 9 months’ chronological age, devices preschool
followed by period of skills Persistent absence of expressive
regression to 6 months’ language but learns to respond
developmental level to her name
Expresses pleasure with giggles
and subtle, jerky motions
Uses raking motion to pick up
small foods
Physical therapy Movement-oriented activities with Aquatic therapy Postural strengthening,
interventions music sensorimotor activities
Assistive and adaptive Adaptive seating provides best Walks indoors and outdoors Gait trainer
equipment opportunities to interact with with a gait trainer (see Figure Adaptive tricycle
others in learning environment 13-7, B) and shorter distances Bilateral ankle-foot orthoses
with a conventional posterior
four-wheeled walker
Participation of child and Support group events: walk,
family ride, stroll
SUMMARY
This chapter has addressed several chromosomal abnormali-
ties and specific gene defects that are most likely to be seen
in children in a typical developmental therapy setting. The
inclusion of family members in all aspects of therapy has
been stressed, along with the need to consider family goals,
priorities, and resources in the development and implemen-
tation of therapy services. The importance of developing
functional goals and delivering services in natural environ-
ments has also been emphasized. Finally, many diseases or
conditions have a genetic component that must be consid-
ered in the course of medical management. Physical and
occupational therapists should expand their working knowl-
edge of genetics to appropriately refer patients for genetic
services. Readers are encouraged to consult Box 13-1 for a
list of resources about genetic disorders, education, testing,
and interventions not described in this chapter.
Figure 13-10 n This child with Angelman syndrome enjoys
riding her adaptive tricycle to the park with her family. References
To enhance this text and add value for the reader, all refer-
companies this textbook. This online service will, when
available, provide a link for the reader to a Medline abstract
for the article cited. There are 383 cited references and other
general references for this chapter, with the majority of
those articles being evidence-based citations..
CHAPTER 14 Learning Disabilities and Developmental
Coordination Disorder
STACEY E. SZKLUT, MS, OTR/L, and DARBI BREATH PHILIBERT, MHS, OTR/L

developmental coordination disorder After reading this chapter the student or therapist will be able to:
learning disabilities 1. Be aware of characteristics that typically identify a child with learning disabilities.
life span disability 2. Become familiar with accepted definitions and terminology used in the field of learning
model of disablement disabilities.
motor learning 3. Investigate the proposed causes of learning disabilities.
neurodevelopmental treatment 4. Understand the clinical presentation of subgroups within the learning-disabled
nonverbal learning disabilities population.
praxis 5. Become familiar with members of the specialist team and service provision types for
sensory integration children with learning disabilities.
verbal learning impairments 6. Recognize the characteristics of the child with developmental coordination disorder.
7. Identify areas of evaluation to assess motor deficits effectively in the child with a
learning disability.
8. Become familiar with theoretical development and intervention techniques applicable to
children with learning disabilities and motor deficits.
9. Understand the lifelong ramifications for the individual with learning disabilities.
AN OVERVIEW OF LEARNING DISABILITIES

difficulties may be isolated (e.g., academic, motor, or
Clinical Presentation behavioral), combined (e.g., academic and motor), or global
Learning disabilities are not a singular disorder but a group (academic, motor, and behavioral).7 In addition to verbal
of varied and often multidimensional disorders.1 Difficulties and nonverbal disabilities, specific motor impairments also
in learning may manifest themselves in various combina- can be present and affect academic achievement or daily
tions of impairments in language, memory, visual-spatial life tasks.8,9
organization, motor function, and the control of attention
and impulses.2,3 The characteristics of a child with a learning Definition
disability are often diverse and complex. Each child presents The heterogeneity of persons with learning disabilities has
a different composite of system problems/impairments, made consensus on a single definition difficult. Many disci-
functional deficits, preventing participation in activities and plines describe learning disabilities according to their own
societal limitations. frames of reference. Medical professionals tend to relate the
The most commonly recognized performance difficul- deficit to its cause, particularly to cerebral dysfunction.
ties in learning are associated with academic success. Terms historically used include brain injured,10 minimal
Fletcher and colleagues4 argued that learning disabilities brain dysfunction,2 and psychoneurological disorder,11 all
should be characterized as “unexpected” because the child implying a neurological cause for the deviation in develop-
is not learning up to expectations despite adequate instruc- ment. Educational professionals, however, prefer to describe
tion. Typically the areas of deficits are observed in verbal the child’s difficulties in behavioral or functional terms.
learning, including difficulties with reading, the acquisition Educators view children with learning disabilities as “chil-
of spoken and written language, and arithmetic. Impair- dren who fail to learn despite an apparently normal capacity
ments in nonverbal learning are equally important and more for learning.”12 Current terminology within the academic
recently recognized. The three primary areas affected by environment includes reading disorder, mathematics disor-
nonverbal learning disorders include visual-spatial organi- der, disorder of written expression, and intellectual disabili-
zation, social-emotional development, and sensorimotor ties (formerly called mental retardation).13,14 The lack of
performance.5 Accompanying behavioral manifestations consensus for one accepted definition continues to affect
may include problems with self-regulatory behaviors, such consistency in diagnosis, research, and intervention for per-
as lack of attention, hyperactivity, and poor impulse con- sons with learning disabilities.
trol. Difficulties in social perception and social interactions After multiple revisions, the National Joint Committee
may also be observed.5,6 These learning and behavioral on Learning Disabilities (NJCLD), which represents
379
several professional organizations, proposed the following This description does not specifically address cause but
definition: does highlight psychological processes versus neurologi-
Learning disability is a general term [for a condition] that: cal impairments. The primary disability focus is on lan-
n Is intrinsic to the individual . . . [the term] refers to a guage, which may exclude difficulties in learning that
heterogeneous group. (Each individual with learning involve nonverbal reasoning. This definition does not men-
disabilities presents with a unique profile of strengths tion regulatory, reasoning, and social perception difficul-
and weakness.) ties that may contribute to understanding the student’s
n Results in significant difficulties in the acquisition and complete profile. On a foundational level this definition
use of listening, speaking, reading, writing, reasoning, formed the basis for creating academic programs and de-
or mathematical abilities. (These difficulties are evi- lineating appropriate services for children with learning
dent when appropriate levels of effort by the student disabilities.
do not result in expected performance, even when IDEA mandates that all children will have free and appro-
provided with effective instruction.) priate education and authorizes aid for special education and
n Is presumed to be due to central nervous system educationally relevant services for children with disabilities.
dysfunction and may occur across the life span. IDEA influences how children with learning disabilities are
(They persist throughout life and may change in identified and classified. The 1997 amendments of IDEA, by
their presentation and severity at different stages promoting the early identification and provision of services,
of life.) redirected the focus of special education services by adding
n May occur concomitantly with other impairments provisions that would enable children with disabilities to
or other diagnoses. (For example, difficulties in self make greater progress and achieve higher levels of functional
regulation and social interaction may exist separately performance.16
or result from the learning disability. Individuals with The IDEA 2004 amendments eliminate a previous re-
attention-deficit disorders, emotional disturbances or quirement that students must exhibit a severe discrepancy
intellectual disabilities may experience learning diffi- between intellectual ability and achievement for eligibility.
culties but these diagnoses do not cause or consti- This “severe discrepancy” policy often mandated that chil-
tute them.) dren would have to experience failure for several years to
n Is not due to extrinsic factors. (Such as insufficient or demonstrate the requisite degree of discrepancy.17 The cur-
inappropriate instruction, or cultural differences.)15 rent goal is to identify ways of serving students more quickly
This definition identifies a proposed cause but does not and efficiently once they begin to show signs of difficulty.17
provide a clear exclusion statement regarding what learning Congress also indicated specifically that (1) IQ tests could
disabilities may not result from. A positive component of not be required for the identification of students for special
this definition is the lifelong nature of the condition. Also, education in the learning disabilities category, and (2) states
by including the behavioral manifestations of regulatory and had to allow districts to implement identification models that
social difficulties, a more complete picture of functional used Response to Instruction (RTI).18 The RTI models sug-
problems for the individual with learning disabilities is pre- gest that the learning difficulty may be intrinsic to the child,
sented. This could assist in the creation of more comprehen- inherent in the instruction, or a combination of both. The
sive and life-spanning programs of service and ultimately models propose systematically altering the quality of instruc-
help in the recognition and remediation of functional and tion and repeatedly measuring the child’s response to that
societal limitations. instruction. Inferences can then be made about the child’s
The definition used in educational settings was initially deficits contributing to learning difficulties.19
passed in Public Law 94-142 and later incorporated into IDEA 2004 also limits the schools from finding a stu-
the Individuals with Disabilities Education Act (IDEA) dent eligible for special education services if the learning
(Section 602.26). problems are determined to be caused by a lack of appro-
Children with learning disabilities are defined by IDEA priate instruction. The law now encourages schools to
as follows: use scientific, research-based interventions to maximize a
n Individuals with a disorder in one or more of the basic student’s opportunity for success in the general education
psychological processes involved in understanding or setting (least restrictive environment [LRE]) before being
using spoken or written language. (This emphasizes placed in special education. IDEA encourages educators
the receptive and expressive difficulties a student may to stress the importance of identifying individual differ-
demonstrate.) ences and patterns of ability within each child and adjust
n Those who are experiencing difficulties in the ability the educational methods accordingly. Academic achieve-
to listen, think, speak, read, write, spell, or do mathe- ment relies heavily on the effectiveness of the teacher and
matical calculations. (These highlight the academic the instructional techniques. Studies indicate that learning
difficulties the student may experience.) disabilities do not fall evenly across racial and ethnic
n Those who may have conditions such as perceptual groups, with a higher incidence of special education
disabilities, brain injury, minimal brain dysfunction, services needed for black, non-Hispanic children.20 The
dyslexia, and developmental aphasia. No Child Left Behind Act challenges states and school
n Those who have a learning problem that does not result districts to become more accountable for improving edu-
from other disabilities such as motor deficits, emotional cational standards by intensifying their efforts to close
disturbances, or environmental, cultural, or economic the achievement gap between underachieving students and
differences. their peers.
CHAPTER 14 n Learning Disabilities and Developmental Coordination Disorder 381
Classifications The second dimension, impairment, includes the organ

The two most widely used classification systems are those and system dysfunction that potentially has a negative effect
of the American Psychiatric Association (Diagnostic and on functional performance. Children with learning disabili-
Statistical Manual of Mental Disorders [DSM])21 and the ties may demonstrate impaired balance, endurance, and
World Health Organization (WHO) (International Classifi- coordination of movements. Impairments that occur in one
cation of Diseases [ICD]).22 Educational professionals pre- or more systems may lead to functional limitations, the third
fer the DSM classification for its academic relevance. A dimension. The challenge for the clinician is to treat impair-
variety of specific academically related disorders are out- ments within the context of daily functional performance
lined in the DSM. The latest edition, DSM-IV-TR, classifies because impairments do not always result in functional
learning disabilities under “disorders usually first diagnosed limitation.
in infancy, childhood, or adolescence.” It subclassifies disor- Functional limitations involve whole-body functions that
ders into the following categories: are typically assessed but may or may not receive remedia-
Learning Disorders tion.24 For a child with learning disabilities this may include
n Reading disorder poor hand function in the performance of manipulation activi-
n Mathematics disorder ties involved in dressing and handwriting. When persistent
n Disorder of written expression functional limitations are not remediable and cannot be ade-
Motor Skills quately compensated for with assistive technology or other
n Developmental coordination disorder supports, disabilities in daily life occur. The child then fails to
Communication Disorders be an active participant in life roles, such as activities of daily
n Expressive language disorder living and school tasks. Emotional difficulties, such as depres-
n Mixed receptive-expressive disorder sion and decreased self-esteem, which may result from learn-
n Phonological disorder ing difficulties, can ultimately impair social interactions.
n Stuttering Community and environmental barriers, called societal
The classification system commonly used by therapists is limitations, also can lead to restriction in social participa-
the ICD. The ICD codes are state mandated diagnostic codes tion. An example of structural or attitudinal barriers that
used for billing and information purposes. In the recently re- prevent optimal participation in society is a child who
vised ICD-10 the category “specific delays in development,” cannot use playground equipment because of lack of acces-
which included “other specific learning difficulties,” was sibility. The ultimate goal for the clinician is to facilitate
changed to “disorders of psychological development.” The functional abilities and performance as well as provide nec-
term “learning” is no longer part of this classification. This essary supports so the child can become an active participant
updated classification is as follows: in society.
Disorders of Psychological Development The Model of Disablement proposes that the environ-
n Including specific developmental disorders (SDD) of ment, purpose, and level of participation should all be con-
speech and language (including acquired aphasia with sidered when evaluating performance. Determination of the
epilepsy) presence, severity, or kind of disability should be made
n SDD of scholastic skills on the basis of a combination of these factors. Within this
n SDD of motor function framework, a clinician does not assume that a handicap
n Pervasive developmental disorder exists because of an impairment but rather considers levels
of functional and societal abilities. This allows the therapist
Model of Disablement to determine intervention needs based on functional perfor-
Beyond classifying learning disabilities as a diagnosis, mance in relevant environments rather than being driven
the National Center for Medical Rehabilitation Research purely by diagnosis. A 9-year-old child with learning dis-
(NCMRR)23 and WHO have integrated related approaches abilities, for example, might have impairments in motor
to classify functional performance. This conceptual ap- components of muscle strength and balance. Although these
proach, the Model of Disablement, describes the multiple impairments can be identified on assessment, the Model of
dimensions of disability and identifies various internal and Disablement suggests that a disability does not exist unless
external factors that affect the way a disability manifests. these deficits affect functional performance (e.g., ascending
The purpose of this model is to shift classification of a dis- and descending stairs) and limit societal participation (e.g.,
ability to include assessment of functional performance and child cannot leave house independently to go to school or
societal participation as opposed to solely identifying com- play). The identified impairments, based on assessment in
ponent deficit areas. an academic setting, would have to affect participation
Five dimensions are outlined in the Model of Disable- within the educational environment (be educationally rele-
ment. They include pathophysiology, impairments, func- vant) to warrant intervention.
tional limitations, disabilities, and societal limitations.
Pathophysiology refers to the underlying disease or injury Incidence and Prevalence
processes at the tissue or cellular level. Proposed caus- Current data indicate that 15 million children nationwide
ative factors related to learning disabilities at this level have been diagnosed with some kind of learning disabil-
include brain damage, biochemical abnormalities, genetics, ity.25 According to a 2007 report to Congress on the imple-
and metabolic disorders. The challenge for interventionists mentation of IDEA, nearly 2.6 million students aged 6 to
is to recognize the signs and symptoms that confirm the 21 years are receiving special education services for spe-
diagnosis.24 cific learning disabilities. As of 2007, this represents 44%
of students with disabilities nationwide.25 Children with The right hemisphere processes input in a more holistic
specific learning disabilities represent the highest inci- manner, grasping the overall organization or the “gestalt” of
dence (number of new cases identified in a given period) a pattern.37,38 This type of organization is advantageous
among 13 disability categories, representing 44% of the for spatial processing and visual perception. Functionally,
total population of children receiving special education. the right hemisphere synthesizes nonverbal stimuli, such as
Overall, the estimated prevalence (total number of cases in environmental sounds and voice intonation, recognizes and
a population at a given time) of learning disabilities is ap- interprets facial expressions, and contributes to mathemati-
proximately 15% of the U.S. population, which translates cal reasoning and judgment. Over time these differences in
to one out of seven people.9 In children under age 18 years, left and right brain processing have become accepted and
8% to 10% of the population have some type of learning are commonly labels of cognitive style (i.e., left-brained
disability.26 Boys are more likely than girls to be identified versus right-brained learner).
as having a learning disability. According to Child Trends, A strict left-right dichotomy is oversimplified because it
10% of boys and 6% of girls aged 3 to 17 years had a learn- does not take into account many aspects of functional brain
ing disability in 2004.27 organization.37,39 Both hemispheres must work together for
a variety of specific academic outcomes such as reading and
Perspectives on the Causes of Learning mathematical concepts. In addition to the communication
Disabilities that occurs between the hemispheres via the corpus callo-
Learning disability is a diverse diagnosis with varied mani- sum, essential communication within the hemispheres is
festations; therefore searching for a single cause would be also present. Intrahemispheric communication is critical for
inadequate. Historically, researchers have studied causative developing higher level cognitive functions such as memory,
factors including (1) brain damage or dysfunction caused by language, visual-spatial perception, and praxis.40 Research
birth injury, perinatal anoxia, head injury, fetal malnutrition, suggests that children with learning disabilities show differ-
encephalitis, and lead poisoning; (2) allergies; (3) biochem- ent patterns of cerebral organization than normal children.37,39
ical abnormalities or metabolic disorders; (4) genetics; However, brain plasticity is the basis for designing and
(5) maturational lag; and (6) environmental factors, such as implementing a variety of intervention techniques aimed at
neglect and abuse, a disorganized home, and inadequate improving processing.
stimulation.28-30
Current sources agree that possible causes of learning Subgroups
disabilities can include problems with pregnancy and birth In early attempts to classify learning disabilities, Denckla
(e.g., drug and alcohol use, low birth weight, anoxia, and and Rudel41 determined that approximately 30% of the
premature or prolonged labor), and incidents occurring after 190 children they assessed by neurological examination
birth (e.g., head injuries, nutritional deprivation, and expo- could be classified into three recognizable subgroups. The
sure to toxic substances such as lead).31-34 Genetic and other 70% exhibited an unclassifiable mixture of signs.
hereditary links also have been observed, with learning dif- Of the 30%, the first subgroup was classified as children
ficulties often seen across generations within families.34 The having a specific language disability. These children, who
emotional and social environment have also been considered were failing reading and spelling, showed a pattern of
as a contributing factor to learning disabilities.14 inadequacy in repetition, sequencing, memory, language,
Children with learning disabilities frequently display a motor, and other tasks, all of which require rote function-
composite of neuropsychological symptoms that interfere ing. The second group had a specific visual-spatial disabil-
with the ability to store, process, or produce information. ity. These children had average performance in reading and
These symptoms typically include disorders of speech, spelling with delayed arithmetic, writing, and copying skills.
spatial orientation, perception, motor coordination, and The children in this subgroup all had social and/or emo-
activity level. Researchers have attempted to identify areas tional difficulties. The third group manifested a dyscontrol
of the brain that may be responsible for these functional syndrome. These children had decreased motor and impulse
limitations. Tools being used include empirical measures control, were behaviorally immature, and were average in
of physiological function such as electroencephalography, language and perceptual functioning.
event-related potentials (ERPs), brain electrical activity Grouping children with learning disabilities based on
mapping (BEAM), regional cerebral blood flow (rCBF), patterns of academic strengths and weakness is as important
positron emission tomography (PET), and functional mag- as grouping them based on neuropsychological or cognitive
netic resonance imaging (fMRI). These measures expand measures. With an academic classification the heterogeneity
the understanding of brain functioning but are best used of learning disabilities can be more clearly recognized and
in conjunction with data on functional and behavioral learning modalities can be adjusted to the individual child.
manifestations. A child with a specific reading difficulty, for example, could
Research findings on brain structure have documented be experiencing deficits in word recognition, fluency, or
that certain functions are specialized within each hemi- comprehension. Through identification of the specific areas
sphere and this specialization is optimal for efficient of weakness in reading, intervention can be individualized
learning.35,36 The left hemisphere processes information in to improve academic performance.4
a sequential, linear fashion and is more proficient at ana- Based on historical and current trends the following
lyzing details. Academically, this hemisphere is responsi- general subgroups will be explored: verbal learning
ble for recognizing words and comprehending material impairments, nonverbal learning disabilities (NVLDs),
read, performing mathematical calculations, and processing motor coordination deficits, and social and emotional
and producing language. challenges.
Verbal Learning Impairments parent with the disorder.50 Neuroanatomical abnormali-

Verbal learning impairments typically include dyslexia, ties, atypical brain symmetry, and disruptions in neural
dyscalculia, and dysgraphia. Harris13 classifies these deficits processing have been observed in children with reading
in functional terms, with dyslexia including disorders of disorders.14,18,48,51 Anatomically, the measurements that
reading and spelling, dyscalculia denoting a mathematics best discriminate between children with and without dys-
disorder, and dysgraphia describing a disorder of written lexia are the right anterior lobe of the cerebellum and the
expression. These learning disorders may occur individually area involving the inferior frontal gyrus of both hemi-
or concurrently. Each of these verbal learning impairments spheres.18 Dynamic investigations using functional brain
will significantly influence academic performance. imagining techniques (PET, fMRI, and the newer ultrafast
Dyslexia (Developmental Reading Disorder). Dys- echo planar imaging [EPI]) are providing significant infor-
lexia is a learning impairment in which the ability to read mation on brain functioning during cognitive tasks such as
with accuracy and comprehension is substantially less than reading and picture naming.14,48
expected for age, intelligence, and education and that impairs Reading skills consist of a combination of visually
academic achievement or daily living.21 The International perceiving whole words and phonetically decoding let-
Dyslexia Association adopted the following definition in ters, morphemes, and words.52 Individuals with reading
2002: “Dyslexia is a specific learning disability that is disorders exhibit brain activation patterns that provide
neurological in origin. It is characterized by difficulties evidence of an imperfectly functioning system for seg-
with accurate and/or fluent word recognition and by poor menting words into phonological (language) parts and
spelling and decoding abilities. These difficulties typically linking the visual representations of letters to the sounds
result from a deficit in the phonological component of lan- they represent.47 These disruptions of the posterior read-
guage that is often unexpected in relation to other cognitive ing system result in increased reliance on ancillary sys-
abilities and the provision of effective classroom instruc- tems during reading tasks, including the frontal lobe
tion. Secondary consequences may include problems in and right hemisphere posterior circuitry. This suggests
reading comprehension and reduced reading experience that the child with dyslexia may be compensating for
that can impede the growth of vocabulary and background poor phonological skills with other perceptual processes,
knowledge.”42 helping to explain why individuals with dyslexia can de-
Characteristics of dyslexia include the following43,44: velop reading skills, although they often remain slow and
n Difficulty learning to recognize written words nonautomatic.48
n An inability to sound out the pronunciation of an Dyscalculia (Mathematics Disorder). Dyscalculia
unfamiliar word is a learning impairment in which mathematical ability is
n Seeing letters or words in reverse (b for d or saw for substantially less than expected for age, intelligence, and
was)—although seeing words or letters in reverse is education and that impairs academic achievement or daily
common for children younger than 8 who do not have living.21 Difficulties occur with comprehending a variety of
dyslexia, children with dyslexia will continue to see math concepts, including number quantities, money, time,
reversals past that age and measurement. This disorder also involves difficulties
n Difficulty comprehending rapid instructions or following with computations and problem solving of specific math
more than one command at a time functions, which affects the ability to understand, remem-
n Problems remembering the sequence of things, such ber, or manipulate numbers or number facts.18 This hetero-
as learning the order of the alphabet or spelling geneous disorder may involve both intrinsic and extrinsic
n Difficulty distinguishing between similar sounds in factors.53 Intrinsic factors are hypothesized to include defi-
words; mixing up sounds in multisyllable words cits in visual-spatial skill, quantitative reasoning, sequenc-
(auditory discrimination) (e.g., aminal for animal, ing, memory, or intelligence. Extrinsic factors can be a
bisghetti for spaghetti) combination of poor instruction in the mastery of prerequi-
n Slow or inaccurate reading, with difficulty reading site skills as well as attitude, interest, and confidence in the
out loud subject.
n Difficulty rhyming Characteristics of dyscalculia include the following54:
Dyslexia is the most common learning disorder, affecting n Confusing numbers and math symbols (1, 2, 3, 4)
as many as 80% of individuals identified as learning dis- n Inconsistent ability in addition, subtraction, multipli-
abled.45 Prevalence rates range from 10% to 15% of the cation, and division
school-aged population,46 with the highest noted estimate n Problems sequencing numbers, or transposing them
of 17.4%.47 Historically, dyslexia was considered more when repeated
common in boys than in girls, but data indicate an equal n Difficulty with abstract concepts of time and direction
distribution between the sexes.48 Boys are more likely to act n Poor mental math ability
out as a result of having a reading difficulty and are therefore n Difficulty with money, budgeting, balancing check-
more likely to be identified early. Girls, on the other hand, are books, and financial thinking (e.g., checking change
more likely to try to “hide” their difficulty, becoming quiet or estimating the cost of items in a shopping
and reserved.18 basket)
Causes of dyslexia can be both genetic and neurobio- n Problem reading analog clocks
logical.14,18,42 Genetic causation has been linked to chro- n Trouble keeping score during games and playing
mosomes 1, 2, 3, 6, 11, 13, 15, and 18.49 There is a strong games with flexible rules of scoring such as poker
inheritability of the genetic links for dyslexia. Statistics Prevalence of dyscalculia is 5% to 6% in the school-
suggest that 30% to 50% of children with dyslexia have a aged population, with a nearly equal male-to-female
ratio.14,55 Geary56 concludes that individuals with arithme- Limited data are available on the prevalence of dys-
tic disabilities currently appear to constitute at least two graphia. Although 10% to 30% of school-aged children
subgroups: those with only mathematic disorders and struggle with handwriting, we cannot assume they have
those with concomitant reading disorders and/or attention- been diagnosed with dysgraphia.60 Difficulties in written
deficit disorder. expression are frequently underidentified and can be masked
Although there is evidence that this disorder is familial by reading disorders or considered to be attributable to poor
and heritable, much less research on its cause is provided motivation. Studies have suggested that dysgraphia may be
than on the causes of most other learning disorders. Dyscal- as common as reading disorders and may occur in 3% to 4%
culia shares genetic influences with reading and language of the population.13,58
measures. The association between dyslexia and dyscalculia Dysgraphia has been suggested to be a neurological
seems to be largely genetically mediated.14,55 Other risk fac- processing disorder that seldom occurs in isolation
tors for development of dyscalculia include prematurity and and can result from a number of other dysfunctions,
low birth weight. In addition, environmental deprivation, including attention deficit, auditory or visual processing
poor teaching, classroom diversity, and untested curricula weakness, and sequencing problems.14,61 The complex
have been linked to cause.55 nature of written expression makes finding the cause dif-
The neurological cause of dyscalculia was initially hypoth- ficult. Writing involves integration of spatial and linguis-
esized to be right hemisphere dysfunction because of the strong tic functions, planning, memory, and motor output. This
relation of visual-spatial skills to numerical computation.57 suggests involvement of both the left and right hemi-
Additional research supports the involvement of both hemispheres for skill in decoding, spelling, formulating and
spheres because mathematics computation involves a complex sequencing ideas, and producing work in correct spatial
relation of spatial problem solving, sequential analysis, lan- orientation, all coupled with rules of punctuation and
guage processing, and memory.55 Specifically involved are capitalization.
portions of the parietal and frontal lobes.14 In an effort to
compensate, individuals with dyscalculia can recruit alternate Nonverbal Learning Disability
brain areas, but this substitution often results in inefficient NVLDs (or NLDs) are considered by some to be a neuro-
cognitive functioning.55 psychological disability. Although this condition has been
Dysgraphia (Disorder of Written Expression). identified for more than 30 years, it has not yet been in-
Dysgraphia is a learning impairment in which writing cluded as a diagnostic category in the DSM.62 The pioneer
ability is substantially less than expected for age, intelli- in the field, Dr. Byron P. Rourke, first identified in 1985
gence, and education that impairs academic achievement this separate and distinct learning disability. In 1995
or daily living.21 The DSM, fourth edition (DSM-IV) di- he defined nonverbal disability as “a dysfunction of the
agnosis of “disorder of written expression” depends on brain’s right hemisphere—that part of the brain which
recognition of “writing skills substantially below those processes nonverbal, performance-based information, in-
expected given the person’s chronological age, measured cluding visual-spatial, intuitive, organizational and eva
intelligence, and age appropriate education” that “signifi- luative processing functions.”63 Nonverbal learning dis
cantly interferes with academic achievement or activities orders affect both academic performance and social
of daily living that require composition of written texts.”21 interactions in children. Three primary areas affected
Children with dysgraphia have specific difficulties in the by NVLDs include visual-spatial organization, sensory-
ability to write, regardless of the ability to read. This motor integration, and social-emotional development. The
may include problems using words appropriately, putting social and emotional difficulties for individuals with non-
thoughts into words, or mastering the mechanics of writ- verbal learning disorders are paramount, leading some
ing. Classifications of dysgraphia can include penmanship- researchers to label this a social-emotional learning
related aspects of writing (e.g., motor control and execu- disability.13,64 NVLDs are generally identified by a distinct
tion), linguistic aspects of writing (e.g., spelling and pattern of strengths and deficits, with excellent verbal and
composing), or a combination.58 This heterogeneous disorder rote memory skills and poorly developed sensory-motor
is frequently found in combination with other academic, and graphomotor ability, executive functioning, and social
learning, and attention disorders.13,18 interactions.13,65,66
Characteristics of dysgraphia include the following59: Characteristics of NVLDs include the following14,62,67:
n Poor legibility: irregular letter size and shapes, poor n Higher verbal IQ compared with performance (non-
spacing verbal) on the Wechsler Intelligence Scale for Children
n Mixing uppercase and lowercase letters; unfinished (WISC)
letters n Develops speech, language, and reading skills early
n Spelling difficulties n Strong vocabulary and spelling
n Fatigues quickly or complains of pain when writing n Ability to memorize and repeat a massive amount of
n Decreased or increased speed of copying or writing information provided it is in spoken form
n Needs to say words out loud while writing n Learns better and faster through hearing information
n Struggles with organizing thoughts on paper rather than seeing it
n Difficulty writing grammatically correct sentences n Difficulties with constructional and spatial planning
and organized paragraphs tasks
n Large gap between knowledge base and ability to n Fine and gross motor difficulties affecting printing
express ideas in writing and cursive writing, physical coordination, and
n Awkward pencil grip balance
n May exhibit limited facial expression, flat affect, un- Social and Emotional Challenges
changing voice intonation, and robotic speech Behavioral patterns or disorders associated with learning
n Poor interpretation of emotional responses made by disabilities include frustration, anxiety, depression, attention
others deficits, conduct problems, and global behavior problems.
n Trouble reading and understanding facial expressions, Ames69 stressed that no single behavior pattern is prevalent
gestures, and voice intonations in children with learning disabilities. Children with learning
n Nuances of spoken language, such as hidden meanings, disabilities not only struggle in the classroom, but experi-
figures of speech, jokes, and metaphors are interpreted ence difficulties in the social arena as well.70 Issues in learn-
on a concrete level ing and related behaviors affect one another in a complex
n Struggles with conversation skills, dealing with new manner, leaving us to wonder which is the cause and which
situations, and changing performance in response to is the symptom.
interactional cues Frustration, deflated self-esteem, and other social and
n Difficulties in problem solving and understanding emotional difficulties tend to emerge when instruction does
cause-effect relationships not match learning styles.71 This frustration mounts as the
n Poor awareness of social space child notices classmates surpassing them, and this often re-
n Can be intrusive and disruptive sults in exasperation with trying to keep up. The pressure
NVLDs make up 5% to 10% of all individuals with then becomes for the child to “try harder,” when ironically
learning disabilities.68 NVLD is frequently overlooked most do not understand just how hard the child is trying. The
in the educational arena because children with this disor- dissatisfaction in not meeting the teacher’s expectations is
der are highly verbal and develop an extensive vocabulary often overshadowed by the inability to succeed in personal
at a young age. Well-developed memory for rote verbal goals and a lack of self-worth. This can result in the devel-
information positively influences early academic learning opment of internal perfectionism to deal with the lack of
of reading and spelling. Yet these students will have dif- competence, with the belief of the child that he or she should
ficulty performing in situations where adaptability and not make mistakes.72
speed are necessary, and their written output will be Anxiety is another response that may occur with persis-
slow and laborious.65 Nonverbal learning disorders are tent difficulties in understanding and successfully com-
therefore challenging to identify at younger ages but pleting schoolwork. This occurs when the child feels
become progressively more apparent and debilitating out of control and lacks the ability to plan and execute
by adolescence and adulthood. The challenges in early strategies for success.71 The mismatch between ability,
identification, the absence from the DSM-IV, and the expectations, and outcomes can cause frustration, disap-
different views held by psychological and educational pointment, and stress, triggering a range of emotions and
disciplines often result in lack of awareness of, accurate behaviors that interfere with everyday functioning in mul-
diagnosis of, and appropriate service provision for these tiple environments.71
students. Other emotional difficulties are noted in attention.
Little is known about possible genetic or environmental When a lesson is taught in a manner that is too complex,
causes of NVLD. There are no family, twin, adoption, segrega- the child may become inattentive. Attention problems can
tion, or linkage studies available.14 Pennington14 proposes that influence behavior, often relating to difficulties with im-
both Turner syndrome and fragile X syndrome in females pulse control, restlessness, and irritability, affecting learn-
appear to be possible genetic causes of NVLD. Similarities ing and peer interactions. These issues frequently coincide
include deficits in executive functions, increased difficulties with frustration, anger, and resentment, which may mani-
in math versus reading and spelling, functional structural lan- fest as a conduct problem (e.g., verbal and nonverbal
guage but impaired pragmatic language, and social anxiety aggression, destructiveness, and significant difficulties in-
and shyness.14 Differential diagnosis is essential because teracting with peers). Children with learning disabilities
NVLD can occur in conjunction with dyscalculia, attention often become discouraged and fearful, are less motivated,
deficit, adjustment disorder, anxiety and depression, emotional and develop negative and defensive attitudes. These pat-
disturbances, and obsessive-compulsive tendencies. terns of behavior can worsen with age, contributing to
juvenile deliquency.3 Low self-esteem and depression
Motor Coordination Deficits are common during school years and tend to escalate
Children with learning disabilities may or may not manifest around age 10 years.73
motor coordination problems. Conversely, some children Poor academic progress, additional prompting needed
have motor and coordination problems but do not experi- from teachers, and negative attention for disruptive behav-
ence learning difficulties. Children with motor deficits iors can cause children with learning disabilities to perceive
typically have difficulty acquiring age-appropriate motor themselves as being “different.”74 Lack of success in school
skills and move in an awkward and clumsy manner. Diffi- experiences can influence the development of positive self-
culties in daily functional tasks and performance areas perception and can have powerfully negative effects on self-
(e.g., school and leisure skills) are common. Motor deficits esteem.71 A self-defeating cycle may be established: the
can result from a wide variety of neurological, physiologi- child experiences learning problems, school and home
cal, developmental, and environmental factors. These im- environments become increasingly tense, and disruptive
pairments can manifest in diverse ways depending on the behaviors become more pronounced. These responses, in
severity of the disorder and the areas of motor and social turn, further affect the child’s ability to learn. Lack of suc-
performance affected. This will be discussed at length in cess generates more failure until the child anticipates defeat
the next section. in almost every situation.
Physical educators, adaptive physical educators, physi-

Assessment and Intervention
cal therapists (PTs), occupational therapists (OTs), and
Specialists speech therapists also may be involved in the assessment
Evaluation and intervention for children with learning of motor deficits and related areas. Overlap in the areas
disabilities should involve an interdisciplinary team owing assessed may occur. The unique training of each pro
to the varied nature of presenting problems. Most chil- fessional influences both the selection of tests and the
dren with learning disabilities are seen by a group of qualitative aspects of assessment on the basis of observa-
professionals, the makeup of which depends on the pur- tions of a child’s performance. Although the evaluations
pose, location, philosophical orientation, or availability may appear similar, differences among professions are
of resources of a particular program. Box 14-1 lists the apparent in orientation and rationale when interpreting
different professionals and specialists who might partici- dysfunction.
pate in assessment or remediation of children with learn- Planning an assessment protocol can prevent unneces-
ing disabilities. The types of professionals are grouped sary duplication of testing and provide comprehensive
into the four categories of education, medicine and nurs- information related to the referral concerns. The assess-
ing, psychology, and special services; they have been ment is driven by the referral concerns and the functional
listed only once, although some professions could be difficulties the child is experiencing. Communication of
categorized in multiple ways. information between professionals and the parents will
Therapists should be familiar with the roles of the vari- generate a comprehensive picture of the child’s areas of
ous medical specialists and of primary care physicians. strength and weakness, necessary for effective intervention
Psychologists have two distinct and often separate roles in planning.
the care of children with learning disorders. The first role
is in identification of learning strengths and weaknesses. Coordinating Multiple Interventions
Psychological testing is often essential in the recognition As the number of disciplines involved in the assessment
of specific learning problems and may be done by clinical and therapeutic management of children with learning
psychologists, school psychologists, or clinical neuropsy- disabilities has steadily increased, communication for
chologists who specialize in diagnosis of learning disor- effective programming has become more challenging.
ders. The second role of psychologists is to provide mental Despite the benefits of specific skills brought to the case
health services and support systems to address academic, by each professional, the huge variety of well-meaning
social-emotional, and behavioral issues. Counseling and recommendations can result in service delivery overkill.
behavior management can also be provided by a psychia- Case Study 14-1 provides an example of the negative im-
trist, behavioral specialist, or social worker. School adjust- pact of overabundant specialized intervention on the child
ment or guidance counselors offer support and advice on and family. In this case, if all the interventionists had com-
specific academic difficulties, social conflicts, and affec- municated, a more realistic and effective plan could have
tive issues. been developed.
BOX 14-1 n TYPES OF SPECIALISTS WORKING WITH CHILDREN WITH LEARNING DISABILITIES
EDUCATION Ophthalmologist or optometrist

Classroom teacher Otologist or ear, nose, and throat (ENT) specialist
Special educator PSYCHOLOGY
Guidance counselor Clinical psychologist
Learning disability specialist Neuropsychologist
Educational diagnostician School psychologist
Reading specialist Child psychologist
Physical educator
SPECIAL SERVICES
Adaptive physical educator
Occupational therapist
MEDICINE AND NURSING Physical therapist
Family physician Speech and language pathologist
Pediatrician Audiologist
Pediatric neurologist Vision specialist
Psychiatrist Social worker
School nurse Recreational therapist
Biochemist Music therapist
Geneticist Vocational education specialist
Endocrinologist
Nutritionist
CASE STUDY 14-1 n MATT

Matt is an 8-year-old boy who was referred for clinic-based because he was bright yet aware of his learning disability
physical therapy intervention for 1 hour per week for reme- and frustrated by it. Thus Matt also saw a psychotherapist on
diation of severe motor coordination and planning problems a weekly basis. The psychotherapist recommended participa-
that accompanied his learning disability. In addition to tion in weekly group sessions, in addition to Matt’s individ-
Matt’s weekly treatment sessions, suggestions were made to ual sessions, to help improve peer relationships. Thus Matt’s
his mother for a home program to be accomplished three therapists had developed a 12-hour-a-day program for him
times a week for 15 to 30 minutes each time. Meanwhile, and his family. It was no wonder that Matt had difficulty in
Matt also received other services. Although he was main- developing peer relationships; he never had time. Matt’s
streamed into a regular classroom in accordance with the schedule also affected interaction in his own family. His
special education law, he was seen by the resource teacher mother believed that being a “therapist” to Matt interfered
on a daily basis and by the adaptive physical education with her role as his mother. She felt unable to carry out the
teacher twice a week to meet his specialized needs. The home program and felt guilty for not doing it.
classroom teacher told Matt’s mother that Matt must read What became apparent with Matt’s case was that although
at least one book a night because he needed additional each professional involved with him made an important contri-
reading practice. A reading tutor came to Matt’s house on bution to evaluation and intervention, the massive input, to
Saturday mornings. Ocular motor problems were identified, some extent, had a detrimental effect on Matt and his family.
so he was evaluated by an optometrist, who recommended Coordinating interventions and providing additional support at
weekly visits plus ocular exercises for 30 minutes a day. home can create a drain on the family and limit time for family
Matt developed secondary emotional problems, partly activities and extracurricular participation.
Effective coordination of intervention services presents a should be determined after assessing the specific needs of
dilemma because no single discipline is specifically trained the child. If services in a regular classroom, coupled with
for that role.75 Kenny and Burka75 stress the need for a person supplemental aids and services, do not meet the needs of the
to act as a coordinator for the management and integration of child, an alternate environment should be considered. The
the interventions received by the child. Unfortunately, this first adaptation might be to have the child participate for
role does not exist; therefore the parent must assume this the majority of the day in the regular classroom and leave for
responsibility. special instruction for part of the day. In some educational
settings, children with learning disabilities are given full-
School-Based Service Delivery Models time instruction in a special classroom with a small group of
The model of service delivery for each individual child should other children with learning disabilities. A special education
be developed to facilitate the student’s ability to be successful teacher or a learning disability specialist is in charge of the
in the learning environment. A continuum of services exists to classroom. The most specialized environment would be a
enable interventionists to be responsive to all children’s private school only for children with learning disabilities.
needs. The continuum includes consultation, integrated or
supervised therapy, and direct service.76 Unfortunately, a lack LEARNING DISABILITIES AND MOTOR
of available resources can influence what type and frequency DEFICITS OR DEVELOPMENTAL
of services are provided. In creating a plan that truly addresses COORDINATION DISORDER
the issues hindering a child’s learning within the academic Approximately half of children with learning disabilities
setting, the team must work together to fabricate relevant and have motor coordination problems.78 Motor deficits are often
inclusive goals. the most overt sign of difficulty for the child with learning
IDEA currently requires that all children in special educa- disabilities. Lowered academic achievement within any or all
tion be educated in the least restrictive environment. The law areas of learning (reading, spelling, writing) is also seen in
requires that students with disabilities be educated to the children with developmental coordination disorder (DCD).8,21
extent appropriate with their peers, within the inclusion class- A study by Jongmans and colleagues78 indicates that children
room. Removing the child from the classroom for special with concomitant perceptual-motor and learning problems
education and intervention is discouraged unless it is abso- are more severely affected in motor difficulties than those
lutely necessary for the student to learn effectively. Although with only DCD or who are only learning disabled. At times,
the model of inclusion can be effective for many children, extreme discrepancy in competence over a range of motor
it requires members of the team to work closely together skills exists, with strengths in some motor areas and signifi-
with the regular education teacher. This collaborative effort cant weaknesses in others. Presentation of difficulties may
ensures an understanding of the child’s special learning needs change over time depending on developmental maturation,
and incorporation of therapeutic procedures into the regular environmental demands, and interventions received.
classroom to facilitate the best learning environment. An International Consensus Meeting on Children and
Bricker77 contends that adhering strictly to this model can Clumsiness was held in 1994 with expert educators, kinesi-
be detrimental to certain students, and each case must be ologists, OTs, PTs, psychologists, and parents. These experts
looked at individually. The least restrictive environment discussed a common name to identify “clumsy” children
with movement, coordination, and motor planning difficul- Clinical Presentation

ties. The term developmental coordination disorder (DCD), DCD is a childhood disorder characterized by poor coordi-
as first described in DSM-III,21 was identified to distinguish nation and clumsiness. Typically, there is no easily identi
these children from those with severe motor impairments fiable neurological disorder accompanying this lack of
(such as those with cerebral palsy or paraplegia) and children motor skills required for everyday life.80 Characteristics
with normal motor movements. A child with DCD often can be seen in developmental areas such as gross motor,
exhibits difficulty with motoric academic tasks such as hand- fine motor, visual motor, self-care, and social-emotional
writing and gym class, self-care skills such as dressing and areas. Children tend to develop at a slower rate and require
using utensils, and leisure activities including playground more effort and practice to accomplish age-level tasks. The
games and social interactions.79 salient features are coordination difficulties that include
decreased anticipation, speed, reaction time, and quality
Definition and grading of movement.81,82 These children often have
As described in the DSM-IV-TR21 as one of the motor skill difficulties analyzing the task demands of an activity, in-
disorders, DCD has the following criteria: terpreting cues from the environment, using knowledge
n A marked impairment in the development of motor of past performance, and transferring and generalizing
coordination (criterion A) skills.83
n An impairment that significantly interferes with Coordination difficulties are most apparent when com-
academic achievement or activities of daily living plex motor activities are attempted. Physical education class
(criterion B) often presents major problems. For example, a 9-year-old
n The presence of coordination difficulties that are not the boy described his motor problems as follows: “When the
result of a general medical condition (e.g., cerebral palsy, gym teacher tells us to do something, I understand exactly
hemiplegia, or muscular dystrophy); the criteria for per- what he means. I even know how to do it, I think. But
vasive developmental disorder are not met (criterion C) my body never seems to do the job.”84 Case Study 14-2
n If mental retardation is present, the motor difficulties are describes the motor difficulties frequently encountered in
in excess of those usually associated with it (criterion D) children with DCD.
CASE STUDY 14-2 n PAUL

The following is a mother’s description of her child, Paul, run. That was where his handicaps were first being noticed,
who had motor coordination problems and learning disabilities: more by other teachers and professionals than by my husband
“I think when Paul was first born I tried to ignore the problem. and myself.
Paul is a child who never climbed or ran or drew pictures the “When we had to make the decision as to whether to put
way other kids did. But until he went to nursery school, Paul into kindergarten or hold him back, we were frustrated
I didn’t pay much attention to it. Maybe I didn’t want to pay because Paul was very bright and very alert. He has always
attention to it. Maybe I knew it was there and I didn’t want to known everything that was going on in the world.
know about it. I’m not sure. But Paul was always a verbal child “Now, the kids Paul knows and the kids who know Paul
and a creative and imaginative child. He and I had something know that he can’t do motor tasks and they’ll come over and
special because I used to enjoy that kind of creative imagina- play rocket ships with him. But there will come a time, as the
tive play. We used to have our own world of various fantasies, kids are getting older, that they won’t want to do this.”
heroes, and places. Paul’s mother, who also had learning and motor difficulties,
“Paul sat up at about 7 months; he crawled and crept on described her own disability as follows:
time. He didn’t learn to walk until he was about 15 months “The hardest course for me was gym. I was unfortunate
old. He walked cautiously, holding on and not letting go. He enough to have the same gym teacher throughout high school.
walked late, but he talked early. He said his first clear word, The teacher always used to think I was a lazy kid, that I just
“cat,” at 6 months. He knew what a cat was and could relate never wanted to try to do the exercises. Although I tried, I
to it. My husband and I were so enthusiastic about his sounds. couldn’t do the stunts and tumbling for anything. The other
In those days they said that if you stimulated your child and girls would do a somersault and I would still do it like a
talked to him and got him ready to talk, he could read early. 4-year-old. I’d just about get over.
I was concerned that Paul would be able to talk and have a “I took dance a couple of times. I never could figure out
marvelous vocabulary and read because I had a reading as a kid why I couldn’t point my toes. The teacher would
disability and a spelling disability. say, ‘Point your toes,’ and it never made any sense to me.
“When Paul was 4 years old and in nursery school, at I always curled my toes up. Only when somebody sat down
my first conference the teacher said, ‘Look out the window, with me and actually showed me did I know that that was
Mrs. B.—see Paul sitting at the bottom? All the other kids are how you were supposed to point your toes. With other kids,
climbing on top of the jungle gym.’ And then she showed me they just did what the teacher did. Nobody had to stop and
some art work. Paul couldn’t cut, he couldn’t paste, he tell them. I was the klutzy kid. I never could do the nice
couldn’t do any of it. We could definitely, at the age of 3 or leaps across the floor. But I would try. After two or three
4, see his problems. He was bright, but he couldn’t cut, paste, sessions my mother stopped giving me lessons. She was
or draw, he couldn’t climb, and he really didn’t know how to probably embarrassed.
CASE STUDY 14-2 n PAUL—cont’d

“As a girl, it wasn’t as traumatic not being athletic. As I got learning-disabled kids that you see: my clothes were not put
older, the need for a woman to be athletic tended to decrease, together properly, my shoelaces were untied, and my hair was
whereas for a boy, the need to be athletic and competitive never quite combed properly.
tends to increase. I foresee this as one of the major problems “It was very difficult for me to learn how to put on makeup
for Paul. and use a hairdryer. It would take many hours of trying to learn.
“Most of my life my friendships have always relied on For a long time, my fingernails were cut very short because
other people. I met most of my friends through other friends I didn’t know how to file them. It is still very hard for me to put
because I’ve gone along to things. I think it goes back to being on eye makeup and look in the mirror and try to figure it out.
teased as a child about the things I couldn’t do or the way I I still don’t feel as though I am completely put together. And
looked. If you looked at me, I probably looked like a lot of the I put a lot of effort and energy into looking good.”
Development of gross and fine motor skills, coupled with n Poor anticipation (do not use knowledge of past per-
the child’s ability to master body movements, enhances feel- formance to prepare)
ings of self-esteem and confidence. Through persistence in n Notably different quality of running and ball skills
mastering the varied challenges of motor exploration the from typical peers
child builds self-reliance. The frustrations and accomplish- n Difficulty learning bilateral tasks such as riding a
ments enhance confidence and the ability to take risks. bicycle, catching a ball, and jumping rope
By engaging in group activities children develop essential n Possible hesitance with and avoidance of new or com-
social skills, including how to compromise, work as a team, plex motor tasks (e.g., playground equipment, gym
and deal with conflicts and different personality styles. class)
Poor motor coordination often results in significant social n Possibly poor safety awareness
and emotional consequences. When a child is poorly coordi- n Inability to smoothly turn and position body when
nated she or he is often teased and shunned from group play. going up ladder to a slide or to get into a chair
This may lead to anxiety and avoidance of participation n Possible sedentary activity level; may prefer to engage
in games, as children frequently judge themselves to be in solitary play
both physically and socially less competent.85 Anxiety may n Tendency to not play games by the rules
be more prevalent in adolescence, most notably in boys.86 n Often, avoidance of team sports such as T-ball and
Because they are often unsuccessful in group participation, soccer
difficulties with navigating the changing demands of coop- Fine motor characteristics of DCD include the following:
erative play and negotiating with others and reluctance to n Diminished wrist and hand strength
advocate for themselves often result. Boys with learning and n Maladaptive or immature grasp patterns
motor coordination problems have been found to demon- n Possible use of excess or not enough pressure
strate significantly less effective coping strategies in all n Poor refinement of small motor movements with
domains of functioning compared with a normative sample.87 hands (qualitatively, the child looks like he or she is
Feelings of incompetence, depression, or frustration are wearing a pair of gloves when trying to manipulate
common and can be lifelong problems.88,89 The impact of small objects)
motor coordination difficulties on social behavior is exempli- n Often dropping or breaking of items
fied by this statement from a child with learning disabilities n Delayed dressing skills (buttons, zippers, fasteners,
and motor deficits: shoelaces)
n Trouble with eating utensils (scooping, piercing)
“They always pick me last. This morning they were all fighting n Difficulty with tool use (e.g., scissors, pencils, stapler,
over which team had to have me. One guy was shouting about it. hole punches)
He said it wasn’t fair because his team had me twice last week.
n Writing that is laborious and often illegible
Another kid said they would only take me if his team could be
n Impaired drawing ability characterized by poor motor
spotted four runs. Later, on the bus, they were all making fun of
me, calling me a “fag” and a “spaz.” There are a few good kids, I control, with wobbly lines, inaccurate junctures, and
mean kids who aren’t mean, but they don’t want to play with me. difficulty coloring within the lines.
I guess it could hurt their reputation.”84 n Decreased ability with pasting, gluing, manipulating
stickers and other art materials
Gross motor characteristics of DCD include the following: n Difficulty with constructive, manipulative play (e.g.,
n Diminished core strength and postural control block building, Tinkertoys, Legos)
n Delayed balance reactions n Often the presence of associated articulation deficits,
n Often falling, tripping, and bumping into things; possibly because of the fine motor nature demanded
acquiring more than the usual number of bruises for articulation
n Motor movements that are performed at a slower rate Visual motor characteristics of DCD include the following:
despite practice and repetition82 n Difficulty with visually guided motor actions (i.e.,
n Motor milestones that may be achieved in the later eye-hand and eye-foot coordination)
range of normal development n Hesitancy or decreased safety on stairs
n Trouble with timing needed for kicking, hitting, and An estimated 5% to 10% of children aged 5 to 11 years
catching ball had DCD.21 Boys diagnosed with DCD outnumber girls by
n Difficulty with hopscotch and four squares two to one. This difference may reflect higher referral rates
n Poor judgment of spatial relationships (knowing where for boys as a result of increased behavioral difficulties of
the body is in space) boys with motor incoordination.95
n Delayed development of prepositional and directional
concepts Perspectives on the Causes of Developmental
n Difficulty with spatial planning tasks such as puzzles, Coordination Disorder
building models, and constructional toys There is no single explanation for the cause of DCD. Neuro-
n Handwriting that is often labored, with spacing and logical dysfunction, physiological factors, genetic predisposi-
sizing problems evident; letters may be irregular, tion, and prenatal and perinatal birth factors have been pro-
illegible, and poorly organized on the page posed to explain the basis of DCD.91,96 It is recognized that
Self-care characteristics of DCD include the following: DCD is heritable and is genetically distinct from ADHD,
n Slowness to develop independence in activities of although the comorbidity rate is up to 50%.14 Comorbidity
daily living is high with other diagnoses, including autism spectrum
n Overreliance on parents to help with self-care skills disorders,80 as well as a variety of developmental learning
n Clothes that are often on backward or crooked problems such as math disability, reading disability, specific
n Struggles with cutting fingernails, putting on makeup, language disabilities, spelling and writing disabilities, and
tying necktie, using a hair dryer so on. Correlation has also been noted between preterm
n Difficulty blowing nose with tissue, putting on Band-Aid infancy and low birth weight with characteristics of DCD. The
n Trouble putting toothpaste on toothbrush heterogeneity of DCD makes finding a unitary cause difficult.
n Messy eater, spills often, does not recognize food Children with DCD present wide variability in both locus of
on face specific problems and functional disabilities. Further compli-
n Difficulty pouring from a container, opening lunch box, cating an understanding of the cause is that the intervention for
unwrapping sandwich, opening containers, peeling fruit the child with DCD is driven by competing treatments.97
n Trouble packing a bag, backpack, or suitcase Few studies have been conducted to look at brain
n Difficulty sequencing daily routines images in children with DCD, with no particular patterns
Social and emotional characteristics of DCD include the of abnormality observed.91 Hadders-Algra98 has suggested
following: that DCD is a result of damage at the cellular level in the
n Often emotionally immature neurotransmitter and receptor systems, rather than a spe-
n May exhibit behavioral difficulties such as acting out cific region of the brain. Resulting coordination difficulties
or becoming class clown can be from a combination of one or more impairments in
n May be more introverted and anxious proprioception, motor programming, timing, or sequencing
n Can appear fiercely competitive, hating to lose, com- of muscle activity.
plaining that rules are unfair Possible physiological origins of motor coordination
n Can be self-deprecating, calls self “stupid” deficits have highlighted multisensory processing. Ayres,99
n Often easily frustrated in her theory of sensory integration, suggested that the inte-
n May experience depression and feelings of incompetence gration among sensory systems is imperative for refined
n Has difficulty making and maintaining friendships, motor performance in children. She proposed that normal
plays alone development depends on intrasensory integration, particu-
n Has feelings of low self-worth, poor self-esteem90 larly from the somatosensory and vestibular systems. Lane100
n Perceived by others as lazy, overprotected, or immature91 outlines the role of vision, combined with vestibular and
n Adolescents may have fewer social pasttimes and proprioceptive inputs, as a foundation to motor perfor-
hobbies than peers mance. In combination, these systems sustain postural tone
and equilibrium, provide awareness and coordination of
Prevalence head movements, and stabilize the eyes during movement in
Estimating the prevalence of children with DCD is challeng- space.
ing. Great variety exists in the clinical presentation, with More recently, Piek and Dyck101 found support for the cor-
some children exhibiting motor deficits in all areas and others relations between DCD and deficits in kinesthetic perception,
having only isolated concerns. Among professionals there is visual-spatial processing, and multisensory integration.101 In
a lack of clarity on the definition and diagnostic criteria.91 general, it is thought that reduced rates of processing informa-
Overlap of symptoms associated with other conditions such tion and deficits in handling spatial information may underlie
as attention-deficit/hyperactivity disorder (ADHD), autism the deficits in motor control.80 Obviously more work is
spectrum disorders, perceptual-motor problems, and speech needed on the cause of DCD.
and language impairments further complicates differential
diagnosis.14,80,92 In addition, there is no single test or screen- Subtypes of Developmental Coordination
ing measure that can be used to confidently identify the Disorder
problem.93 Other factors influencing prevalence rates include Various approaches have been used to investigate subtypes
the criteria used to delineate a child with DCD from a typical of DCD, including classification by underlying causes, clini-
peer, differences in terminology, types and methods of test- cal and descriptive approaches, and statistical clustering.94
ing, reliability of the tests used, and heterogeneity of the test Initial attempts at classifying subtypes within DCD support
sample.79,93,94 the heterogeneity of this group of children.102 Work by
Dewey and Kaplan103 suggests that children with DCD may provides an overview of standardized tests available for the
be classified into subgroups based on distinctions in motor assessment of motor dysfunction in children with learning
planning and motor execution deficits. They identified three disabilities. Uses and limitations of the individual tests and
subgroups: children who exhibited deficits in motor execution test batteries are listed.
alone, those whose primary deficits were in motor planning, Identification of subtle motor difficulties is critical and
and children who exhibited a generalized impairment in challenging. These subtle motor difficulties initially can be
both areas. undetected, leading to unrealistic expectations of age-level
Macnab, Miller, and Polatajko104 identified five differ- motor performance. The child’s difficulty with skilled, pur-
ent profiles of children with DCD. They used measures of poseful manipulative tasks or with finely tuned balance
kinesthetic acuity, gross motor skill, static balance, visual activities may not be readily apparent in the classroom or
perception, and visual motor integration. Two distinct groups may be perceived as lack of effort. Children with DCD may
emerged, with children exhibiting generalized visual deficits be able to perform certain motor tasks with a level of
and generalized dysfunction in all areas. Generalized gross strength, flexibility, and coordination that is qualitatively
motor deficits did not emerge as a distinct subgroup, as average but must use increased effort and cognitive control
the third group demonstrated a discrepancy between static for sustained success.
balance and complex gross motor tasks, and the fourth Levels of performance in gross and fine motor testing may
group had poor performance on running but performed fall in the borderline range. Careful observations are of para-
well on kinesthetic acuity. Other groups included children mount importance, because the child’s deficits are often
with deficits in visual motor and fine motor problems. These qualitative rather than quantitative. A child might have age-
results suggest that a subtype based on motor execution or appropriate balance on testing but lack ability in weight shift-
planning problems alone may be too general. ing and making quick directional changes, which affects
the ability to participate in extracurricular activities such
Assessment of Motor Impairments as soccer or baseball. When assessing children with subtle
A variety of professionals may be involved in a comprehen- motor deficits, it is important to realize that many evaluation
sive assessment of motor deficits. Pediatric OTs and PTs are tools have been developed for children with moderate to
often the core team assessing functional motor concerns. severe neurological impairments.
Areas assessed by pediatric OTs and PTs often overlap, Children with DCD do not exhibit obvious evidence of
so communication is essential to ensure that testing is not neuropathological disease (i.e., “hard” neurological signs
replicated. Ideally, performance will be evaluated in multi- such as a cerebral lesion). Subtle abnormalities of the central
ple environments and include components of skill, functional nervous system are frequently noted by the presence of
performance areas, and social and societal participation. “soft” neurological signs. Deficits associated with soft neu-
Specific recommendations should include activities to en- rological signs include abnormal movements and reflexes,
hance performance in the environments in which the child sensory deficits, and coordination difficulties. Evaluation of
functions on a daily basis. soft neurological signs is typically part of an examination by
Clinical judgment of the therapist is important in de- a pediatric neurologist, although therapists can assess these
signing an assessment protocol and synthesizing informa- areas in conjunction with standardized testing. Box 14-2
tion to create a complete profile of the child. A variety of lists soft neurological signs frequently used to assess this
standardized and nonstandardized evaluation tools should population.
accompany structured clinical observations and caretaker Researchers suggest that a high percentage of children
interviews. Observations of the child can yield more readily with learning disabilities exhibit certain soft neurological
usable information than a standardized score,105 enabling signs. An early study reported that 75% of 2300 children with
the therapist to view the child in natural routines, self- positive total “neurological soft sign” ratings had the symp-
directed activities, and unstructured play. The interview tom of poor coordination.106 More recently, 169 children aged
process is essential to gather information about the child’s 8 to 13 years were assessed for a relation between soft neuro-
interactions and participation. This process paints a verbal logical signs and cognitive functioning, motor skills, and
picture of the child to help us to understand levels of func- behavior. Those children with a high index for soft neuro-
tioning and participation in a variety of environments. logical signs were found to have significantly worse scores in
Other crucial information obtained is how the child’s dif- each domain.107 The relationship between neurological soft
ficulties are affecting the ability to parent or teach the signs and DCD is difficult to validate without more current
student.105 systematic research; however, they are indicators that inter-
Before choosing an evaluation tool the therapist should vention may be needed.108
be aware of the intended purpose of this measure. Tools used In general, a composite of soft neurological signs is more
to assess children with DCD are used for distinct purposes: predictive of dysfunction than single signs. Children without
identify impairments, describe severity of impairments, or notable motor difficulties can frequently exhibit one or more
explore activity or participation limitations.83 The choice soft signs; therefore identification of a single sign must be
of evaluations may also be determined by the setting, frame interpreted cautiously. Neurological signs involving com-
of reference of the therapist, and functional concerns of plex processes were found to be the most predictive. The
the child. A therapist should be familiar with all aspects clinician needs to be familiar with typical developmental
of test administration and scoring for evaluation tools and patterns, as certain soft neurological signs such as motor
should comply with the training requirements described in overflow, right-left confusion, visual tracking difficulties,
the test manual. Test construction, reliability, and validity and articulatory substitution are expected at younger ages
for assessing DCD should be considered. Appendix 14-A and mature in quality over time.
BOX 14-2n COMMON SOFT NEUROLOGICAL SIGNS USED IN ASSESSMENT OF CHILDREN

WITH LEARNING DISABILITIES AND MOTOR DEFICITS
MINOR NEUROLOGICAL INDICATORS Postural-motor measures
Left-right discrimination Muscle tone
Finger agnosia Schilder’s arm extension posture
Visual tracking Standing with eyes closed (Romberg test)
Extinction of simultaneous stimuli Walking a line
Choreiform movement Tandem walking (forward and backward)
Tremor Hopping, jumping, skipping
Exaggerated associated movements Ball throw and catch
Reflex asymmetries Imitation of tongue movements
COORDINATION Pencil and paper tasks
Finger-to-nose touching Fine motor tasks (stringing beads, building block towers)
Sequential thumb-finger touching SENSORY INDICATORS
Diadochokinesia Graphesthesia
Heel to shin Stereognosis
Slow controlled motions Localization of touch input
Assessment measures of soft neurological signs vary considerably for children with learning disabilities, both in what signs are included in the assessment
and how they are grouped. This list represents a compilation of possible soft neurological signs.
Compiling a complete picture of motor deficits in chil- Manual muscle testing can provide detailed information
dren with learning disabilities involves assessing the follow- about impairment in strength of individual muscles but is
ing complex skills: (1) postural control and gross motor not regularly used in assessing children with learning dis-
performance, (2) fine motor and visual motor performance, abilities, unless concerns of a possible degenerative disease
(3) sensory integration and sensory processing, (4) praxis exist. More appropriately, strength should be assessed by
and motor planning, and (5) physical fitness. Each of these the child’s functional ability to move against gravity during
interrelated functions is described in this chapter as an area activities. Within developmental assessments, the therapist
of clinical assessment. is observing range of motion against gravity in skills such
as reaching, climbing, throwing, and kicking. The therapist
Postural Control and Gross Motor Performance also can have the child hold positions against gravity to
Muscle Tone and Strength. Low muscle tone and assess strength and endurance (e.g., prone extension and
poor joint stability have been identified as characteristic of supine flexion).
some children with learning disabilities. On observation, the Early Postural Reflexes. Early reflexes are essential for
child with low tone may look “floppy” and may have an the development of normal patterns of motor development.
open-mouth posture, lordotic back, sagging belly, and knees These reflexes facilitate movement patterns that become
positioned closely together. Muscles may be poorly defined integrated into purposeful motions. If they are not fully inte-
and feel “mushy” or soft on palpation, and joints may be grated, qualitative differences in muscle tone, postural
hyperextensible. A common method for assessing muscle asymmetries, transitional movement patterns, bilateral coor-
tone and proximal joint stability involves placing the child dination, and smooth timing and sequencing of motor tasks
in a quadruped position and observing the ability to main- may be observed. Residual reactions (e.g., asymmetrical
tain the position without locking of the elbows, winging of tonic neck reflex [ATNR] and symmetrical tonic neck reflex
the scapula, or sagging (lordosis) of the trunk. The therapist [STNR]) that might be noted in children with DCD are gen-
can determine joint stability by asking the child to “freeze erally subtle and most often are seen in stressful, nonauto-
like a statue.” The therapist then provides intermittent matic tasks. McPhillips and Sheehy looked at incidence of
pushes to the trunk, assessing the child’s ability to remain in primitive reflex patterns and motor coordination difficulties
a static position. in children with reading disorders.109 The group with the
Children with low tone may develop patterns of compen- lowest reading scores had a significantly higher rate of
sation called fixing patterns. These patterns often include ATNR and motor impairments when compared with good
elevated and internally rotated shoulders, internally rotated readers. Assessment for persistence of primitive reflex pat-
hips, and pronated feet. The child compensates for low tone terns in children with learning disabilities should emphasize
by using the stable joint positions and holding himself impact on functional aspects of performance.
or herself stiffly for increased stability. These patterns may The effect of lack of integration can be observed during
resemble those of children with slightly increased tone. tasks such as writing at a table or gross motor activities such
Careful observation and palpation of muscles will help to as using ball skills and jumping rope. Persistence of these
differentiate fixing patterns from increased muscle tone. primitive reflexes may be seen in the child’s inability to sit
Judgments of muscle tone are primarily made through clini- straight forward at the table. The ATNR influence might be
cal observations and felt in a hands-on assessment. observed by a sideways position at the table with the arm on
the face side used in extension. During ball games the child with feet in tandem with eyes closed, and (3) standing
may have diminished ability to throw with directional con- on one leg with eyes open and eyes closed. The Bruininks-
trol because head movements will influence extension of Oseretsky Test of Motor Proficiency, second edition
the face-side arm. If the STNR is not fully integrated the (BOT-2)111 and the Sensory Integration and Praxis Tests112
child is often unable to flex the legs while sitting at a desk have comprehensive balance subtests (see Appendix 14-A).
looking down at his or her work (neck flexion). Although Postural Control. Postural control is dependent on
residual reflex involvement may affect performance of these muscle tone, strength, and endurance of the trunk muscula-
tasks, many other components are involved that require ture, as well as automatic postural reactions required to
consideration. maintain a dynamic upright position. A child has adequate
Righting, Equilibrium, and Balance. Righting and postural control when he or she can maintain upright posi-
equilibrium are dynamic reactions essential for the develop- tions, shift weight in all directions, rotate, and move
ment of upright posture and smooth transitional movements. smoothly between positions. These areas are often deficient
Righting reactions help maintain the head in an upright in children with DCD, affecting both gross and fine motor
alignment during movement in all directions. Equilibrium performance.
reactions occur in response to a change in body position or The child may fatigue quickly and fall often during gross
surface support to maintain body alignment. In simpler motor play. Other body parts may be used for additional
terms, equilibrium reactions get us into a position, and righting support because of weak postural musculature—for exam-
reactions keep us in that position. Together these reactions ple, placing the head on the ground when crawling up an
provide continuous automatic adjustments that maintain the incline or sticking out the tongue when climbing or pumping
center of gravity over the base of support and keep the head a swing. In sitting, a child with diminished postural control
in an upright position. will fatigue quickly, either leaning on his or her hands for
Righting and equilibrium reactions are best assessed on additional support or moving frequently in and out of the
an unsteady surface such as a tilt board or large therapy ball. chair. These compensations affect the child’s ability to per-
These reactions occur in all developmental positions, and form fine motor tasks or maintain attention for cognitive
complete assessment will consider a range of positions dur- learning because so much effort is exerted on sitting up.
ing functional performance in gross and fine motor activi- Observing the effects of fatigue is important because both
ties. To test equilibrium, the child’s center of gravity is sitting and standing postures may deteriorate over the course
quickly tipped off balance. The equilibrium response is one of a day. Generally the problem stems from motor program-
of phasic extension and abduction of the downhill limbs for ming problems versus muscle power.
protection and of flexion of the uphill body side for realign- Gross Motor Skills. Gross motor coordination refers to
ment. In daily actions, most of the righting reactions are motor behaviors related to posture and locomotion, from early
subtle and occur continuously to relatively small changes in developmental milestones to finely tuned balance. Children
body position. Subtle shifts of the support surface can be with learning disabilities and DCD may attain reasonably
made to assess the child’s ability to maintain the head and high degrees of motor skill in specific activities. Motor ac-
trunk in a continuous upright position. Righting and equilib- complishments frequently remain highly specific to particular
rium reactions are the basis for functional balance and pos- motor sequences or tasks and do not necessarily generalize to
tural control. other activities, regardless of their similarities. When varia-
To balance effectively, we use visual information (about tion in the motor response is required, the response often
the body and external environment), proprioceptive infor- becomes inaccurate and disorganized.
mation (about limb and body position), and vestibular infor- Although children with DCD can sit, stand, and walk
mation (about head position and movement), in order to with apparent ease, they may be awkward or slow in rolling,
initiate an appropriate corrective response.110 Balance reac- transitioning to standing, running, hopping, and climbing.
tions occur as a response to changes in the center of gravity Skilled tasks such as skipping may be accomplished with
that stimulate the vestibular receptors (utricles and semicir- increased effort, decreased sequencing and endurance, and
cular canals). This stimulation causes muscles to activate, associated movements.
allowing balance to be maintained in static and dynamic Evaluation of gross motor skills should include both novel
activities (e.g., sitting in a chair, walking, standing on a bus). motor activities and age-appropriate skills. The child, for
When the vestibular system works in conjunction with example, can be asked to imitate a hopping sequence or
vision and information from the muscles (proprioception), maneuver around a variety of obstacles. Skills that have been
balance is easier and more refined. Considering the impact accomplished can be varied slightly (e.g., hopping over a
of these sensory systems working together is important dur- small box). Age-appropriate social participation tasks, such
ing assessment. The therapist should test the child’s balance as tag and dodge ball, can be observed for qualitative difficul-
with the child’s eyes open and closed and observe differ- ties in timing and spatial body awareness. Developmentally
ences in ease and quality of performance. Standing with earlier skills also should be observed to assess the quality
eyes closed relies more on vestibular and proprioceptive of performance. BOT-2111 and the Peabody Developmental
input, and difficulty may indicate that the child depends Motor Scales113 are examples of tools for standardized assess-
heavily on the visual system for balance. To further assess ment of gross motor skills (see Appendix 14-A).
this sensory interaction, balance should also be observed on
steady and unsteady surfaces (e.g., dense foam or a tilt Fine Motor and Visual Motor Performance
board) with and without visual orientation. Traditional tests Fine Motor Skills. Fine motor coordination involves
of balance include (1) the Romberg position—standing with motor behavior such as discrete finger movements, manipu-
feet together and eyes closed, (2) Mann position—standing lation, and eye-hand coordination. A child with DCD often
demonstrates multiple fine motor concerns. Areas of diffi- the Test of Visual Motor Skills,116 and the Spatial Awareness
culty typically include grasp and manipulation of small ob- Skills Program (SASP).117 The production of handwritten
jects and dexterous hand skills, such as buttoning or putting work can be assessed by using the Evaluation Tool of Children’s
coins into a vending machine. Assessment should include Handwriting (ETCH),118 the Test of Handwriting Skills (THS),119
both standardized assessments and structured observations and Handwriting Without Tears: The Print Tool.120 Hand-
of functional performance. writing and drawing samples provide important information
A complete fine motor assessment should include obser- regarding functional abilities in written production.
vations of proximal trunk control to distal finger movements.
Trunk control and shoulder stability affect the accuracy and Sensory Integration and Sensory Processing
control of reaching patterns and create a stable base from Ayres99 originally defined sensory integration as “the ability
which both hands can be used to perform bilateral skills. The to organize sensory information for use.” Information is
assessment of distal control considers wrist stability, devel- received through the senses and organized throughout the
opment of hand arches, and separation of the two sides of the nervous system to help us participate effectively in social,
hand, all providing a foundation for the control of distal motor, and academic learning. Integration of sensory input
movement. Qualitative observations of distal fingertip con- underlies basic functions such as arousal state, attention,
trol include finger motions to move objects into and out of regulation, and postural and ocular control. Skills such as
the palm of the hand and rotate an object within the hand. eye-hand coordination, bilateral coordination, projecting body
Although standardized assessments such as BOT-2111 movements in space (projected action sequences), motor
and the Peabody Developmental Motor Scales113 have fine planning, and skilled motor execution are end products
motor sections, they do not adequately measure manipula- of efficient sensory processing. More recently, it has been
tive components described previously. Combining qualita- proposed that the term sensory processing be used for the
tive observations during a variety of fine motor tasks with assessment and diagnosis of sensory challenges impairing
knowledge of typical development is important. Soft neuro- daily routines.121
logical signs, including diadochokinesia (rapid alternation The process begins with registration or recognition of
of forearm supination and pronation), sequential thumb-to- incoming sensory input (“What is it?”). The incoming in-
finger touching, and stereognosis (identifying objects and formation is quickly scanned for relevance in a process
shapes without visual input) can provide further qualitative called sensory modulation (“Is it important?”). Sensory
information. modulation determines the appropriate action for a situation
Eye-Hand Coordination, Visual Motor Integration. and regulates arousal. Our system needs to respond strongly
Eye-hand coordination is the ability to use the eyes and and quickly if our hand moves near a hot stove, but should
hands together to guide reaching, grasping, and release of not respond as strongly if we are unexpectedly bumped.
objects. This can include larger motions such as catching Discrimination of sensory input involves discerning subtle
and throwing a ball to more refined tasks such as putting differences in sensation to learn about the qualities of ob-
pennies in a bank or buttoning a shirt. Coordination of the jects and refine body movements within space. When we are
eyes and the feet (eye-foot coordination) is important for receiving clear information from our sensory receptors we
skills such as ascending and descending stairs and kicking a can understand and label what is happening (e.g., sour-
ball. Children with DCD often exhibit difficulties in one or sweet, hot-cold, soft-firm, heavy-light, up-down, fast-slow).
more of these areas. Qualitatively, they demonstrate poor Efficient sensory registration, modulation, and discrimina-
coordination in the timing and sequencing of their actions. tion result in organized social and motor behavior.
Evaluations such as BOT-2,111 Movement Assessment Battery Children with DCD often experience sensory processing
for Children, second edition (Movement ABC-2),114 and the difficulties. Diminished registration of sensation can result
Motor Accuracy Test of the Sensory Integration and Praxis in poor body and environmental awareness, low arousal
Tests112 have subtests that assess eye-hand coordination levels, and delayed postural reactions and motor coordina-
in a standardized way. Supplemental clinical observations tion. These children may be sedentary or seek out strong
include the assessment of ball skills, fine motor tasks such sensation. Sensory modulation difficulties manifest primar-
as stringing beads and building block towers, and written ily in emotional and behavioral responses. Behaviors often
accuracy tasks of drawing or coloring within a boundary. include oversensitivity, with aversive or exaggerated re-
Visual motor tasks involve the ability to reproduce sponses to sensation. These children struggle to remain
shapes, figures, or other visual stimuli in written form. This regulated during typical daily events, and may avoid uncom-
skill is multidimensional, involving perceiving a visual im- fortable sensations, demonstrate behavioral disorganization,
age, remembering it, and integrating it to a written response. seek strong, potentially unsafe input, become aggressive, or
Visual motor integration is the foundational skill needed for have tantrums. They often have difficulty performing in situ-
handwriting. In addition, handwriting involves combination ations involving integration of multiple inputs (e.g., cafete-
of fine motor control, motor planning, and sensory feedback ria, gym class, playground, team sports). Sensitivity to
to be accurate and legible. Children who have difficulties movement input can also cause the child to avoid play-
with handwriting commonly produce sloppy work with ground equipment or become nauseated during car rides.
incorrect letter formations or reversals, inconsistent size Delayed sensory discrimination typically results in poor
and height of letters, variable slant, and irregular spacing body awareness that may underlie qualitative motor difficul-
between words and letters. ties observed in children with DCD. They often exhibit
Assessment of visual motor skills can be completed through poor motor coordination and planning, deficient safety
standardized measures such as the Developmental Test of awareness, and poor grading of force, as well as timing and
Visual Motor Integration (BEERY VMI, Fifth Revision),115 sequencing difficulties. These children may avoid complex
motor challenges, team sports, and playground activities. task is not successful, and awkward motor execution. Poor
Discrimination difficulties can also affect the acquisition planning abilities can lead to the child being adult depen-
of prepositional concepts (up, down, left, right, in front dent, hesitant, or resistant to trying new activities. At times,
of, behind, next to). The child who has difficulty discrimi- children with dyspraxia also may exhibit poor anticipation
nating information from the body typically exhibits deficits of their actions. They can quickly engage in play with the
in skilled actions involving balance, timing movements in equipment but demonstrate little regard for safety (e.g.,
space, bilateral and eye-hand coordination, fine motor con- kicking a large ball across the room where other children are
trol, and handwriting. playing). Movements are often performed with an excessive
Clinical observation of a child’s responses to a variety of expenditure of energy and with inaccurate judgment of the
sensory inputs and the ability to organize multiple inputs required force, tempo, and amplitude.129 Such children typi-
provides essential information regarding the integration of cally require more practice and repetition to master more
sensory input. Sensory modulation dysfunction is not easily complex, sequential movements. Frequently, children with
identified with standardized, skill-based measures because planning problems recognize the differences between their
of its physiological basis. Caregiver questionnaires, such performance and that of other children the same age, which
as the Sensory Processing Measure122,123 and the Sensory significantly affects their self-esteem.
Profile124,125 can provide valuable information on modula- Manifestations of poor motor planning ability are appar-
tion and regulation. ent in many daily tasks. Dressing is often difficult. Children
Gross and fine motor tasks that involve postural and are not able to plan where or how to move their limbs to put
ocular responses, bilateral motor coordination, planning, on clothes. Problems are often demonstrated in constructive
and sequencing reflect efficient sensory processing. Soft manipulatory play, such as building with toys, cutting, and
neurological signs, coupled with observations of play (e.g., pasting. Similarly, learning how to use utensils, such as a
playground, gym class, recess) can provide qualitative infor- knife, fork, pencil, or scissors, is difficult. The child with
mation on sensory discrimination and planning. The Clinical dyspraxia often also has problems with handwriting.
Observations of Motor and Postural Skills (COMPS) assess- Standardized assessments of praxis include the tests of
ment tool126 is a set of six standardized clinical observations Postural Praxis, Sequencing Praxis, Praxis on Verbal Com-
and soft signs that can be useful in identifying motor deficit mand, Oral Praxis, Constructional Praxis, and Design Copy
with a postural component. The Sensory Integration and of the Sensory Integration and Praxis Tests.112 The First-
Praxis Tests,112 the Miller Assessment for Preschoolers,127 STEP130 is a preschool screening tool with a section assess-
and the Toddler and Infant Motor Evaluation (TIME)128 are ing motor planning abilities. Clinical observations can add
used most commonly to assess various aspects of sensory valuable information regarding the child’s ability to see the
integration function. Other tests, such as BOT-2111 and the potential for action, organize and sequence motor actions for
Movement ABC-2,114 can provide qualitative observations success, and anticipate the outcome of an action.
in addition to quantitative measures of motor skill.
Physical Fitness
Praxis and Motor Planning Physical fitness involves a person’s ability to perform
Praxis involves the ability to plan and carry out a new physical activities that require aerobic fitness, endurance,
or unusual action when adequate cognitive and motor skills strength, or flexibility. Factors influencing fitness include
are present. The components of praxis include ideation or motor competency, frequency of exercise, physical health,
generating an idea of how one might act in the environment, and genetically inherited ability. Physical fitness can en-
planning or organizing a program of action, and execution compass health-related and skill-related fitness.131 Cardio-
of the action sequence. Motor planning involves the same respiratory endurance, muscular strength and endurance,
components relative to a novel motor task. flexibility, and body composition are components of
Children with praxis difficulties, or dyspraxia, may health-related fitness and important to monitor. Agility,
exhibit a paucity of ideas. The child may enter a room speed, and power are the skill-related fitness components
filled with toys or equipment and have limited capacity and are needed for the acquisition of motor skills and
to experiment and play. Other children with dyspraxia sports and recreational activities.132
may move from one activity to the next without generating Children with DCD often have performance difficulties
effective plans for participating in or completing tasks. in games and athletic activities. They are often less active
Lack of variation and adaptation in play can be another than typical peers and withdraw from physical activity. As
indication of planning problems. Observations of typically a result, the level of physical fitness, strength, muscular en-
developing children show continuous modifications in durance, flexibility, and cardiorespiratory endurance may be
play, with spontaneous adaptations to motor sequences, poorly developed. Hands and Larkin131 found that body
making explorations varied and increasingly successful. mass index (BMI) in children with motor difficulties was
Children with dyspraxia often have difficulties in situations higher than in a control group of typical peers.131 The per-
characterized by changing demands, such as unstructured centage of overweight and obese 10- to 12-year-olds was
group play. Transitions also may be difficult because they found to be significantly higher in a DCD group than in
involve the creation or adaptation of a plan. Frustration and typically developing peers.133 This increased weight may
difficulties with peer interactions frequently are part of the further increase their movement difficulties.
composite. In a study of 52 children aged 5 to 8 years with DCD,
Observations of motor planning deficits may include Hands and Larkin131 revealed significantly lower scores on
trouble figuring out new motor activities, disorganized ap- tests for cardiorespiratory endurance, flexibility, abdominal
proaches, resistance or inability to vary performance when a strength, speed, and power than the age- and gender-matched
controls. Another study of 261 children aged 4 to 12 years Interpreting test data, integrating findings, identifying
found similar disparities for children with DCD, with poor functional limitations, and creating goals is a complex pro-
performance in fitness tests, with the exception of flexibility.133 cess. Initial impressions of the child’s areas of difficulty may
These disparities in fitness were found to increase with age result in the recommendation for further examination before
between the two groups.133 outlining refined goals relevant to functional performance.
One task of the PT is to differentiate between poor Collecting additional assessment information may involve
physical fitness, resulting from low motor activity as op- observations in other environments or during functional
posed to problems of low muscle tone, joint limitations, daily tasks, and/or formal testing. The end product is the
decreased strength, and reduced endurance. The low motor creation of statements that delineate the type and quality of
activity is a neurologic sign and leads to a developmental lag behavior desired as a result of remediation. In other words,
or deviation in motor function. Collaboration among the the therapist must set treatment goals to be achieved through
physical educator, the adaptive physical educator, and the intervention.
PT is critical. The President’s Challenge is a physical fitness Setting goals for the child with learning disabilities
test administered twice a year in schools across the country. with motor deficits must be done by considering a variety of
Children complete five events that assess their level of factors:
physical fitness in strength, speed, endurance, and flexibil- 1. Referral information and age of the child
ity. The test was founded in 1956 by Dwight Eisenhower to 2. Medical, developmental, and sensory processing history
encourage American children to be healthy and active, after 3. Parents’ and teachers’ perception of the child’s strengths
a study indicating that American youths are less physically and concerns about functional impairments
fit than European children.134 Standardized assessments 4. Educational information
of gross motor functioning, such as BOT-2,111 that assess a. Major difficulties experienced in school
strength, speed, and endurance can provide information b. How motor problems are interfering with the
related to a child’s fitness level. child’s daily participation
c. Current services being received
5. Child’s peer relationships, play and leisure activities,
INTERVENTION FOR THE CHILD WITH
and self-esteem
LEARNING DISABILITIES AND MOTOR
6. Therapists’ observations and assessment of the child
DEFICITS OR DEVELOPMENTAL
through informal and formal evaluation, both standard-
COORDINATION DISORDER
ized and nonstandardized
Creating an Intervention Plan 7. Functional expectations and abilities at home and
Using the information gathered throughout the assessment school
process, the therapist synthesizes areas of strength and Goals for the child should be stated in terms of long-
weakness to develop an intervention plan. If impairments, term and short-term objectives. Goal setting ideally in-
activity limitations, and participation restrictions exist that volves establishing specific, measurable, attainable, real-
affect the child’s successful performance, intervention may istic, and time-targeted objectives. Short-term objectives
be warranted. Children with DCD, for example, might dem- are generally composed of three parts: (1) the behavioral
onstrate impairments in coordination or balance that under- statement is what will be accomplished by the child;
lie activity limitations in catching or throwing a ball, which (2) the condition statement provides details regarding how
create participation restrictions in playing baseball with the skill or behavior will be accomplished; and (3) the
peers.135 Determining the child’s functional difficulties and performance statement denotes how the skill or behavior
identifying the severity of the impairment will be important will be measured for success. The most important consid-
to justify service and guide the service delivery model and eration is ensuring that the goals and objectives chosen are
type of intervention. For children with DCD who have relevant to the child’s functional daily performance and
greater impairment and activity limitations, individualized are meaningful to the team, including the family, working
treatment may be more beneficial, whereas those with less with the child. Case Study 14-3 provides an example of
involvement may thrive with group intervention.136 functional objectives.
CASE STUDY 14-3 n JONATHAN

Jonathan was a 6-year-old referred for an occupational therapy climbing the jungle gym and coming down the slide at the neigh-
evaluation by his parents and teacher because of concerns borhood playground. Jonathan tended to play on the outskirts of
regarding motor skill development. Assessment results revealed groups. When he did attempt to interact he became angry because
several areas of impairment, including poor discrimination of his the children would not play the game by his rules. At home,
body position and movement in space, diminished postural Jonathan often was frustrated by tasks of daily living such as
control and balance reactions, motor planning deficits, delayed putting on his coat, snapping his pants, and tying his shoes. His
eye-hand coordination, qualitative fine motor deficits, and delayed mother reported that Jonathan frequently called himself “stupid”
visual-motor integration affecting his handwriting. Jonathan’s when he could not independently complete self-care skills.
mother reported that he was clumsy and seemed to bump into When determining appropriate behavioral objectives for
things constantly. Of greater concern was that Jonathan seemed Jonathan, looking at the areas of functional relevance such as
fearful of activities that his peers found pleasurable, such as pleasure and safety in gross motor play, peer interactions, and
CASE STUDY 14-3 n JONATHAN—cont’d

independence in age-appropriate activities of daily living is To address greater success in peer interactions:
critical. These areas of concern for Jonathan were consistent 1. Jonathan will participate in a structured game, following
with those of his parents. His parents wanted him to feel more the rules, for 10 minutes.
competent and less frustrated in play, at home, and at school. 2. Jonathan will play outside with the children in the
Jonathan’s goal was to “not be so stupid that kids won’t play neighborhood without conflict for at least 1 hour.
with me.” The OT believed that through remediation of sensory Although impairment level objectives could have been writ-
discrimination and motor deficits Jonathan could develop ten to address the same areas, they would have been of limited
improved motor competence and planning abilities. This would relevance to Jonathan and the team working with him. Balance
lead to greater success in peer interactions and improved and postural control also could be addressed by an objective
feelings of self-confidence. Based on these common desires stating that Jonathan would stand on one foot for 10 seconds.
the following goals and objectives were made. The functional implications of this objective would not have
One of the long-term goals was Improve Jonathan’s plan- been clear, and Jonathan and his parents would be without an
ning and coordination abilities to increase his confidence and outcome measure that was measurable and meaningful to
success in gross motor activities. Jonathan was interested in them. Thus it would have negated the effects of working as a
learning to ride a bicycle without training wheels, and his par- cohesive team toward a common goal.
ents were hopeful that he could become more confident at the If the OT or PT is working as a member of a team within
neighborhood playground. These behavioral objectives would the school, behavioral objectives will have implications
measure the development of improved proficiency in discrimi- for the child’s performance in the school environment.
nation of his body in space, postural control balance reactions, Within the school system, statements of goals and specific
and motor planning. The following objectives were written: objectives are included in the Individualized Educational
1. Jonathan will independently climb the ladder and come Plan (IEP). In Jonathan’s case, specific objectives that were
down the slide without exhibiting fear, bumping into meaningful to the classroom situation included the ability
other children, or falling. to sit in the chair to complete written assignments for
2. Jonathan will develop the ability to ride his bicycle without 15 minutes and increase accuracy of letter formation, size,
training wheels in straight lines and will learn to turn and spacing on written assignments. Other areas related
corners. (Note: Successfully riding the bicycle becomes to gross and fine motor skills and peer interactions also
the performance measure of behavior in this objective.) were influential to Jonathan’s success at school. Specific
To address improvement in independence for self-care: objectives written pertaining to school would have
1. Jonathan will put on his coat independently in correct functional outcome measures chosen from tasks within
orientation and successfully zip it four out of five times. the school environment such as gym class, playground
2. Jonathan will successfully tie his shoes without assistance interactions, and classroom expectations.
in a timely manner.
Models of Intervention and child (i.e., practicing getting on and off bike, balancing on
Improvements in motor deficits can be achieved through a still bike, gliding, braking, steering, pedaling) can allow suc-
variety of models of intervention, both indirect and direct. cess at each step.
Indirect intervention involves working with key people in Within a school setting, environmental adaptations might
the child’s life to help them facilitate the child’s delineated include changing the height or position of the desk or
goals. An indirect model can occur through consultation, decreasing extraneous visual and auditory distracters. Task
specialized instruction, and coaching. Direct intervention accommodations could include using a special grip to
involves the therapist working directly with the child on facilitate more refined pencil grasp or allowing more time
specific goal areas or skills. for written work. In these instances the teacher would be
Mild deficits, subtly affecting participation in activities, responsible for carrying out the program and determining its
may be addressed through a consultative (indirect) approach. effectiveness. Communication between the therapist and
This model of service provision incorporates the use of teacher encourages problem solving and changing action
another team member’s expertise to be responsible for the plans over time. Kemmis and Dunn138 demonstrated positive
outcome of the child.76,137 The therapist may suggest environ- outcomes on a variety of functional classroom goals when
mental or task adaptations to facilitate more successful par- an OT and teacher met weekly throughout the school year in
ticipation. Consultation with parents would be appropriate for what they called a “collaborative consultation approach.”
the goal of riding a bicycle without training wheels. Using this model they achieved 63% (134 of 213) of their
Parents may not understand the complexity of the task and outlined goals. This collaborative effort supports the shared
may be focused only on the end product, which can cause responsibility for identifying the problem or weakness of the
frustration for all involved. To facilitate confidence and child, creating possible solutions, implementing the inter-
success, the therapist might recommend environmental modi- vention as the solution, and altering the plan as necessary for
fications such as beginning on an open stretch of grass or dirt, increased effectiveness.76
with no other people around to decrease the child’s anxiety. Another model of indirect therapy involves teaching
Breaking the task down into incremental steps for the parent members of the team to implement treatment strategies.
Specialized instruction or coaching allows the therapist to may be an appropriate focus of intervention for children
support a child within the natural environment by working with DCD.
with care providers who are with the child every day. The Typically, skill-based interventions are used to address
aim is to educate key people in the child’s life to coach activity limitations. These interventions emphasize the de-
the child within the context of teachable moments.139 velopment of specific skills, rather than underlying compo-
These moments, when a child is interested and working nents alone. This is referred to as a “top-down” approach,
on acquiring a new skill, occur throughout the day and in using cognitive strategies and problem solving. The thera-
many different environments.140 This allows for therapeutic pist, family, and child identify specific functional activities
consistency and repeated practice, thereby increasing the to work on. Breaking the activity into smaller, incremental
chances for skill acquisition within the context of daily steps can facilitate the ease of learning by encouraging suc-
routines. cess. Shoe tying is a good example of a skill best taught in
With this model, the therapist observes children and steps.
adults doing familiar routines and collaborates with the At times, skill-based intervention and practice occur out-
adults to enhance those routines in varied environments.141 side the context where the child will typically perform that
When implementing an intervention strategy, the therapist task. Certain skills may not be easily generalized and would
might teach another adult to guide specific, developmen- be best taught in the context where the child would do the
tally appropriate skill sets with the child, or problem solve skill, such as tooth brushing. Progress is seen more rapidly
to create strategies for greater success. Over time parents when a task-related behavior that is meaningful to the child
and other caregivers are empowered to look at a toy, is used. Eye-hand coordination tasks, for example, become
an activity, or an experience and find ways to adapt it to more meaningful within the context of a game of hot potato
increase successful involvement and skill development. or baseball. Barnhart95 suggests an integrated approach to
Successful coaching can enhance parent-child relationships facilitating development in the child with DCD, including
indirectly by helping parents to feel more comfortable both bottom-up, physiological interventions, and top-down,
and competent in their abilities to meet their child’s cognitive strategies.
needs.142 Recently, emphasis has shifted to models of treatment
Direct intervention involves designing individualized that highlight participation. Intervention focuses on increas-
treatment plans and carrying them out with the child indi- ing the child’s ability to take part in the typical activities
vidually or in a small group. This approach can focus on of childhood.83 These treatment methods assume that skill
developing the foundations that underlie motor perfor- acquisition emerges from interaction among the child, the
mance such as sensory processing, postural control, and task, and the environment.97 Intervention is contextually
motor planning. Specific skills, such as shoe tying or bike based, occurring in everyday situations and focusing on the
riding, can be practiced with the therapist as well, breaking activities and tasks inherent to that situation. Problem solving,
these tasks down into component skills. Through combin- preparatory activities, and skill training may be used
ing approaches, adapting methods over the course of ther- together to increase successful participation. This type of
apy, and responding to the changing needs of the child over approach may minimize the challenges of learning new
time, progress is achieved more effectively.97 Best practice skills for a child who cannot easily generalize learning to
dictates that direct therapy should always be provided in new situations.
conjunction with one of the other service models to ensure The intervention methods presented in this chapter
generalization of skills to natural settings.76 Without the use for remediation of motor deficits in the child with learning
of other models, therapists cannot be confident that changes disabilities include Ayres sensory integration; neurodevel-
observed in the isolated setting are affecting the child’s opmental treatment (NDT); motor learning approaches (e.g.,
overall performance. Cognitive Orientation to Daily Occupational Performance
[CO-OP])144; sensorimotor treatment techniques; motor skill
Intervention Approaches training approaches (e.g., Ecological Intervention145); and
According to the International Classification of Functioning, physical fitness. None are mutually exclusive, and each re-
Disability and Health (ICF),143 interventions should be quires a level of training and practice for competence as well
directed toward several distinct goals: as experience in normal development. Most therapists syn-
n To remediate impairment thesize information from different intervention techniques
n To reduce activity limitations and use an eclectic approach, pulling relevant pieces from a
n To improve participation variety of intervention modalities to best meet the needs of
Interventions focused on remediating impairments gen- each child.
erally target the improvement of processing abilities (e.g.,
visual, proprioceptive, and vestibular) or performance com- Ayres Sensory Integration
ponents (e.g., balance and strength). The tenet is that The sensory integration theory and treatment were devel-
by strengthening these foundational skills the child will oped by A. Jean Ayres,99,112 with concepts drawn from
develop greater success in appropriate activities and par- neurophysiology, neuropsychology, and development. Her
ticipation. This type of intervention is referred to as a purpose in theoretical development was to explain the ob-
“bottom-up” approach and is based on neuromaturational served relationship between difficulties organizing sensory
or hierarchical theories. Ayres’s sensory integration therapy input and deficits in academic and neuromotor “learning”
is an example of bottom-up intervention. Missiuna, Rivard, observed in some children with learning disabilities and
and Bartlett83 suggest that addressing secondary, prevent- motor deficits.146 The theory proposes that “learning is
able impairments, such as loss of strength and endurance, dependent on the ability of normal individuals to take in
sensory information derived from the environment and from performance including social, emotional, and motor develop-
movement of their bodies, to process and integrate these ment. Children with sensory processing difficulties will
sensory inputs within the central nervous system, and to use exhibit problems that limit their occupational performance in
this sensory information to plan and organize behavior.”146 a variety of environments.149
Ayres112 used “learning” in a broad sense to include the Vestibular, proprioceptive, and tactile sensory inputs
development of concepts, adaptive motor responses, and used in therapy are powerful and must be applied with cau-
behavioral change. tion. The autonomic and behavioral responses of the child
The goal of sensory integration intervention is to elicit must be monitored carefully. The therapist should be
responses that result in better organization of sensory input knowledgeable about sensory integration theory and inter-
for enhanced participation and generalization of functional vention before using these procedures. Monitoring behav-
skills. Sensory integration treatment is based on the belief ioral responses after the therapy session also is suggested
that active involvement in individually designed, meaningful through parent or teacher consultation. Intervention precau-
activities that are rich in sensory input will enhance the ner- tions are elaborated by Ayres,99 Koomar and Bundy,150 and
vous system’s organization and integration of sensation.147 Bundy.151
Active exploration and variation in the context of play re- Research on the Effects of Sensory Integration
sults in adaptive responses,148 which positively affect the Procedures. Sensory integration is an evolving theory,
child’s ability to participate in daily life activities. During based on developments in the fields of neuroscience, re-
intervention, sensory input is provided in a planned and search, and clinical practice.152 The current neuroscience
organized manner while eliciting progressively harder adap- literature supports the basic tenets of sensory integration
tive behavioral and motor responses. The therapist strives to including neuroplasticity, and positive changes in behavior
find activities that are motivating and tap the child’s inner and learning as a result of enriched environmental condi-
drive to encourage adaptation. “Evincing an adaptive behav- tions, dynamic participation in meaningful activities, and
ior promotes sensory integration, and, in turn, the ability to developmentally appropriate sensory motor experiences.147
produce an adaptive behavior reflects sensory integration.”99 Within the field of occupational therapy, sensory integration
Effective intervention requires melding the science of a neu- is the most extensively researched intervention procedure,
rophysiological theory with the art of “playing” with the with over 80 research studies that measure some aspect of
child. treatment effectiveness.153 Clinically, sensory integration
A sensory integration treatment session for a child with principles are estimated to be used by approximately 90% of
postural difficulties might involve having the child riding a American OTs working in the school system for children
swing pretending to be a fisherman while keeping a look- with learning disabilities and motor deficits.154 Despite over
out for whales that might bump his boat. This “pretend 35 years of theoretical development, research, and interven-
play” scenario taps the child’s motivation and inner drive tion practice, the value and effectiveness of this therapeutic
to be productive (fishing), while challenging him with a modality continues to be questioned and critiqued.155-158 The
potential “out of my control” situation (whales). The thera- complexity of sensory integration theory, the individualized
pist will adapt this activity in a variety of ways to maintain approaches that treatment warrants, and the difficulty find-
an appropriate level of challenge and adaptation (adaptive ing sensitive outcome measures create many challenges in
response). The type and amount of sensory input, postural designing appropriate and valid research studies.
demands, bilateral control, timing, and planning require- Clinicians using sensory integration procedures attest
ments are all considered and can be adapted to an easier or to the effectiveness of this treatment approach in making
harder level to maintain adaptation and learning. Sensory important functional changes. Testimonials from parents of
input can be controlled through the speed and direction children who have received occupational therapy with sen-
the boat moves and the amount of work the child must do sory integration procedures are frequently heard. In Cohn’s
with his arms to propel the boat and catch fish. Additional research, parents identified two important outcome mea-
sensory input can be provided through “rocky seas” and sures for intervention.159 The first included change in the
“whales crashing the side of the boat.” The boat can facili- child, such as improved self-regulation, perceived compe-
tate more or less postural adaptation by the amount of sup- tence, and social participation. The second was related to
port it provides and the speed of its movement. The child parents developing the ability to understand their child’s
can pull a rope to propel the swing, or the therapist can behavior in a new way and having their experiences vali-
provide the movement to decrease the bilateral coordina- dated to better support and advocate for the child.
tion and postural demands. A more demanding bilateral Accurate analysis of the efficacy of sensory integration is
response could include pulling a rope and catching a fish complicated by a wide variety of methodological design
simultaneously. Unexpected movements of the boat, fish, flaws in the available research. The majority of the studies
and whales will require greater timing and planning for include heterogeneous samples, small sample sizes, and
success. inconsistencies in the frequency, length, and duration of
For this intervention technique to be appropriate, the mo- treatment. Schaaf and Miller153 note that a major challenge
tor and planning difficulties observed in a child with learning in interpreting the existing research is related to the outcome
disabilities need to be a result of deficits in processing sen- measures used. Researchers have not consistently used a
sory information. Each child’s intervention plan should be theoretical base to explain how treatment techniques influ-
individualized based on the results of a comprehensive evalu- ence the outcomes chosen.153 In addition, the dependent
ation and responses to sensory input within therapy. Contri- variables measured were often not related to the expected
butions of sensory registration, modulation, and discrimina- outcomes of treatment, were too many in number, or were
tion should be considered for their impact on functional poor measures of change over time.153,156 Many studies
include outcome measures that are not sensitive to small originally designed for use with children with cerebral
increments of change or meaningful to parents as treatment palsy in whom the underlying problem was a lesion in the
priorities.159 central nervous system that produced abnormal muscle
Perhaps the most challenging aspect of developing tone and deficits in coordination of posture and movement,
strong research studies is the variability of sensory integra- affecting functional performance.168 The original frame-
tion intervention. Treatment is individualized and adapted work was based on hierarchical levels of reflex integration
frequently in response to the child’s changing needs and in the nervous system and the normal developmental se-
successes.160 Many of the studies that claim to be using quence. Abnormal postural responses were lower-level
sensory integration therapy do not adhere to the core theo- hierarchical reactions that did not integrate in a typical
retical principles, or they violate them.161 Developing stan- time frame (e.g., ATNR, STNR), thereby inhibiting the
dardized and replicable treatment is a major challenge for development of mature postural mechanisms and voluntary
future research studies. Researchers and clinicians have movements. The NDT approach emphasized specific ways
focused extensively on improving the quality of efficacy to inhibit abnormal reactions and facilitate more normal
research, resulting in the development of improved func- muscle tone and movement.171,172 The assumption was
tional outcome measures (goal attainment scaling)162 and that encouraging more normalized automatic movement
treatment fidelity (fidelity measure).161 Significant progress patterns would lead to functional carryover.172
has also been made in defining homogeneous subgroups for The original hierarchical “impairment-based” model of
analysis, describing replicable treatments, and choosing reflex integration has been replaced with a more dynamic
valid outcome measures.153 “interactive systems” model that emphasizes both internal
Schaaf and Miller153 note that diverse findings are and external factors of motor control. Currently, NDT
not surprising given the current level of research. The therapists view the execution of movement as a complex
knowledge base in sensory integration research is still in its interaction of the neural and body systems, organized by
infancy, with the need for substantial work to generate more the specific task requirements and constrained by physical
rigorous empirical data to support the efficacy of this inter- laws of the environment.170 The nervous system is viewed
vention approach.153 Increased emphasis on high-quality, as dynamic and adaptable, capable of initiating, anticipat-
randomized controlled studies is essential.163 ing, and controlling movements with ongoing sensory feed-
Approximately half of the research studies conducted to forward information and feedback.170,173 Many body factors
date show some positive effects, with sensory integration are recognized as contributing to dysfunctional movement
treatment being more effective than or equally as effective patterns, including abnormal muscle tone, primitive reflex
as other approaches used.153 In a recent systematic review patterns, delayed development of righting and equilibrium
of 27 research studies, May-Benson and Koomar164 con- reactions, specific muscle weakness, body biomechanics,
cluded that the synthesis of evidence indicates that sensory cardiovascular or respiratory weakness, lack of fitness, and
integration may result in a variety of positive outcomes. sensory, cognitive, or perceptual impairments.170,173,174 As
Specific areas identified included sensory-motor skills, NDT’s theoretical and clinical development progressed,
motor planning, social skills, attention, behavioral regula- there was acknowledgement that intervention had not auto-
tion, and reading and reading-related activities, as well as matically carried over into functional performance as had
functional outcomes as measured by individually designed been anticipated. As a result, treatment strategies began to
goal attainment scales (e.g., improved sleep patterns, in- shift, with preparation for specific functional tasks done
creased food repertoire, pumping a swing, and manipulat- in settings where children typically participate.175 The focus
ing fasteners). Positive gains in motor performance were on normalizing muscle tone and altering movement
found in 10 of 14 studies reviewed, with the implication patterns as a foundation for performance was replaced
that the gains were maintained after the cessation of treat- with emphasis on activity-related impairments and client-
ment. Arbesman and Lieberman149 identified that the posi- directed functional outcomes.170,172,173 Ecological, family-
tive development of motor skills as a result of sensory centered intervention was identified as essential to target
integration intervention was most consistently noted in key environments, activities, and functional outcomes.170
their review of 198 articles. Other recent, well designed This dynamic treatment approach now emphasizes active
studies have demonstrated positive effects on behavioral involvement in meaningful tasks to enhance independent
outcomes including significant gains in attention, cognitive participation in various environments. The goal of NDT
and social skills,163 and socialization165 and increased intervention is for the child to use more efficient movement
engagement, with decreased aggression.166 May-Benson strategies to complete life skills with greater success. Over
and Koomar164 suggest that given the current level of posi- the life span these strategies will minimize secondary im-
tive results, OTs can begin to use this information to sup- pairments that can create additional functional limitations
port the use of sensory integration treatment, particularly or disability.170,174
for sensory motor outcomes and client-centered functional NDT uses physical handling techniques directed toward
goals. developing the components of movement necessary for
functional motor performance. Movement components of
Neurodevelopmental Treatment postural alignment and stability, mobility skills, weight
NDT is a treatment technique formulated by Karel and bearing, weight shifting, and balance are all foundations
Berta Bobath167,168 to enhance the development of gross for smoothly executed movements in space.169 Assessment
motor skills, balance, quality of movement, hand skills, and analysis of posture and movement components are
and daily tasks such as mobility and self-care for individu- ongoing, using a problem-solving approach that identifies
als with movement disorders.169,170 These techniques were and builds on the child’s strengths and limitations.170
Therapists employ a combination of handling techniques the 21 studies they reviewed owing to unclear population
and encouragement of active movements targeted toward definitions, unclear treatment protocols and goals, and lack
the specific functional skills on which the child is work- of clarity regarding therapist skill levels.
ing.98 Feedback involves both tactile-proprioceptive Several current research studies have demonstrated posi-
(“hands-on”) and verbal cues, which are graded back tive changes in gross motor performance as a result of
or changed according to the needs and emerging skills of intensive NDT intervention.176,179,180 Arndt and colleagues179
the individual child.172 The therapist’s hands guide the used an operational definition of NDT based on trunk
reactions, with the child actively participating in problem coactivation for treating infants with posture and movement
solving and adapting performance. Practice of more effec- difficulties. After 10 hours of treatment over 15 days, the
tive postural reactions and reduction of abnormal move- infants who received the NDT protocol significantly im-
ment patterns are embedded into meaningful activities. A proved in gross motor function compared with infants in the
skilled therapist balances the quality of movement patterns control play group. These skills were maintained at a
with the importance of active involvement in learning new 3-week follow-up evaluation. Bar-Haim and colleagues176
motor tasks.173 At times, participation and independent used a randomized controlled trial for 24 children with
task completion are more important than qualitatively cerebral palsy, with 40 hours of treatment over a period of
normal movement patterns. 4 weeks. They compared intensive NDT treatment with the
Although NDT was developed for children with central use of the Adeli suit (AST), which stabilizes the trunk and
nervous system insults resulting in deficient postural and extremities of the wearer to help normalize motor actions.
movement control for daily skills, it lends its use to children Although there was no superiority noted between these two
with more minimal motor involvement. Of particular rele- intensive treatment modalities, both groups made signifi-
vance to the child with learning disabilities and DCD is cant gains in gross motor function that were sustained after
facilitation of improved righting and equilibrium responses, nine months. Tsorlakis and colleagues180 further assessed
automatic postural adjustments, and balance reactions. Han- the variable of intensity of services in their 16-week treat-
dling techniques can help develop improved qualitative ment study. The efficacy of NDT for children with spastic
control, as well as encouraging active problem solving and cerebral palsy was supported by this study, as both groups
task adaptation by the child. of children who received NDT intervention demonstrated
Research on the Effects of Neurodevelopmental statistically significant gains in gross motor function. In-
Treatment. NDT is an evolving theoretical and treatment creased intensity of services was also supported, as motor
approach, based on principles derived from research in gains were statistically greater for the group that received
neural plasticity, motor development, motor control, and intervention five times a week compared with two times a
motor learning.170 It is the most commonly used treatment week.
framework for children with cerebral palsy.176 Despite this, Brown and Burns177 completed the only systematic re-
relatively few studies are available on the efficacy of NDT view that was identified as high quality by colleagues.181
to date.172 Those that are available have not definitively They selected 17 studies to include on the basis of use of
shown NDT to be effective as a treatment modality or more NDT as the treatment modality, reported clinical outcomes,
valuable than other therapies.175,177 One of the major prob- and random group assignment. Their analysis did not pro-
lems confounding interpretation of the current state of re- vide definitive evidence that NDT is beneficial for children
search has been the significant change in theoretical devel- with neurological dysfunction. The authors suggest that
opment and clinical application over time. The revised available research did not reveal either efficacy or inefficacy
practice model of NDT is better reflected in the current of NDT as a treatment approach. Butler and Darrah175 sug-
research, which shows more promise.170,178 It has been suggest that absence of evidence on the effectiveness of NDT
gested by Bain172 that studies conducted before 2000, when should not be construed as proof that the treatment is not
NDT was defined with outdated operational definitions, effective, but certainly reflects areas in which more mean-
should not be considered as evidence that current practice ingful research is needed. Sharkey and colleagues178 suggest
is ineffective. that recognition of these limitations will encourage practi-
Methodological concerns in many of the available stud- tioners to implement “second-generation” research that is
ies make interpretation of efficacy more challenging. In a characterized by well designed studies that systematically
review of older treatment studies, Royeen and DeGangi169 evaluate operationally defined intervention techniques and
noted significant methodological problems that were at- determine what works for specific ages and diagnoses of
tributable to the lack of conclusive evidence regarding children.
NDT. These included poorly defined objective outcome
measures, overreliance on subjective clinical observations, Motor Learning Theories
and small sample sizes. In addition, sample populations Motor learning refers to the process of acquiring, expand-
varied greatly, including adults and children with cerebral ing, and improving skilled motor actions. The basic treat-
palsy and Down syndrome as well as high-risk infants. ment premise of motor learning theories is that improve-
More recently Sharkey and colleagues178 highlighted dif- ment in movement skills is elicited through appropriate
ficulties in developing well designed treatment studies, practice and timely feedback. Motor learning has taken
including the heterogeneity of children with cerebral palsy, place when a permanent change in the child’s ability to
both in functional limitations and goals, small sample respond to a movement problem or achieve a movement
sizes, and ineffectiveness of standardized outcome mea- goal has occurred, regardless of the environment.182 There-
sures to assess qualitative and functional changes. Butler fore therapists measure learning through tests that measure
and Darrah175 cautioned interpretation of efficacy from retention and transfer of skills.183
The closed-loop theory of Adams184 is recognized as the to intervention decisions. The types of tasks are gross
first comprehensive explanation for motor learning. Adams motor or fine motor; simple or complex; discrete, serial, or
believed that the central nervous system, based on sensory continuous; and environment changing or stationary.
feedback, controls the execution of movement. He proposed Gross and fine motor tasks are classified according to the
that once a movement has occurred, errors are detected and type of muscle groups required.189 Gross motor skills use
compared with existing “memory traces.” With practice, large muscles and tend to be fundamental skills (e.g.,
these memory traces become stronger and the accuracy of walking and running). Fine motor skills tend to require
the movement increases, thus emphasizing learning through greater control of small muscles and usually have to be
feedback. taught (e.g., handwriting, cutting). Task complexity refers
In 1975 Schmidt contributed the idea of “open loop” mo- to the level of difficulty and amount of feedback required.
tor learning, which emphasized the ability to produce rapid Simple tasks, such as reaching, require a decision fol-
action sequences in the absence of sensory feedback (e.g., lowed by a response. A complex task, such as cutting out
hitting a baseball). He proposed that new movements were a picture, requires continual monitoring and feedback
created from previously stored motor programs (schemas) until completion. Tasks can require simple single actions
of similar movements, as opposed to feedback from indi- or the coordination of sequential motions for completion.
vidual motor actions.185 Schemas comprise general rules for A single discrete movement has a clear beginning and
a specific group of actions that can be applied to a variety of ending, such as activating a button. Serial movements re-
situations.186 When a motor action occurs, the initial move- quire a series of distinct movements combined to achieve
ment conditions, parameters used, outcomes, and sensory the outcome, such as writing a sentence. Continuous move-
consequences of the action are stored in memory. With each ments, such as running, contain movements that are
goal-directed movement, specific parameters are used (e.g., repetitive. Tasks that are discrete or serial can be practiced
force needed to pour juice into a glass), and consequences in parts, but continuous tasks usually need to be practiced
occur (e.g., spillage or not). Repeated actions using different as a continuous segment.
parameters and creating different outcomes create data sets Environmental variations can greatly increase the com-
that help refine the motor program, reducing errors and plexity of the task, requiring higher levels of feed-forward
improving anticipation or feed-forward information.186 information and feedback. In an unstable or changing envi-
Schmidt’s185 schema theory contributed to current theories ronment, the child has to learn the movement and monitor
of motor learning principles regarding practice schedules the environment to adapt to changes—for example, running
and feedback about outcome of movements, known as on an uneven surface. The more predictable and stable the
knowledge of results. task and environments are, the easier it is to learn and repli-
Based on the knowledge of motor skill development in cate motor skills. Tooth brushing is an example of a task that
children with DCD, four key variables are important to con- generally occurs in a stationary environment. Home and
sider in targeted intervention. They include stage of the classrooms can be stable, in that many elements within these
learner, type of task, scheduling of practice, and type of settings are fixed and do not change. The size and shape
feedback. of chairs, location of toys on the floor, and movements
Three stages of the learner have been proposed187,188: the of other children are considered “variable features” within
cognitive stage, the associative stage, and the autonomous these stable environments. These variable features require
stage. As a child learns and develops new motor actions, he a greater amount of motor control because the child must
or she progresses through the various stages at different adjust movements and actions to the changing demands.
rates, depending on the complexity of the skill. The cogni- Therapists generally practice in stable environments and
tive stage is the initial phase of learning in which there is therefore must ensure that the children are able to func-
large variability as the child gets the general idea of the tion under varied circumstances encountered in daily life
movement.135 Awkward body postures are observed, errors situations.
are often made, and awareness of what needs to be improved Practice is believed to increase learning of a skill or
or changed does not exist. (Consider when a young child movement. Variations in practice can occur in the order
attempts to throw a ball; the throw is a gross movement, the tasks are performed, in the environment where the tasks are
projection of the ball varies, and the movement appears unpracticed, and by changing aspects of the task. Practice
coordinated.) As practice continues, the degree of accuracy schedules can be developed based on the practice techniques
increases, which is characteristic of the associative stage. (blocked or random), or how task learning is approached
Fewer errors are made and error information is used to cor- (component or whole task). Blocked practice means the task
rect the movement patterns. (As the child continues to throw is repeatedly rehearsed, sometimes focusing on one aspect
the ball, the ball may get closer to the target with improved of a technique or a specific motor sequence (e.g., hitting a
coordination observed.) During the autonomous stage, the golf ball off a tee with the same club). Repetitive, blocked
skill is performed fluently and automatically, without as practice often leads to improved immediate performance,
much effort or thought. Improvements in accuracy continue particularly in situations that are stable. Random practice
and errors are detected, with corrections made automati- involves performing a number of different tasks in varied
cally. (The child can now throw a ball at a target and hit the order or employing several different aspects of technique
target with coordinated, accurate movements, such as pitch- (e.g., hitting golf balls from a tee, sand trap, and rough with
ing; however, if a child were introduced to throwing a curve the appropriate club). Random practice encourages learners
ball, the stages would start over.) to compare and contrast strategies used in performing the
The type of task is a mechanism to classify motor skills task, which positively influences performance in changeable
in a dimensional fashion. Task components contribute environments.
If a task is discrete or contains multiple parts, breaking it When using the motor learning model of practice, the
down into components for blocked practice may be benefi- therapist should incorporate a variety of teaching techniques
cial. For success in changeable situations in which the task including verbal instructions, positioning, and handling, as
component is integrated into skilled action, whole-task prac- well as observational learning (demonstrations).183 The task
tice is essential—for example, practicing shooting basket- and environment should be structured with extrinsic and
balls, then practicing while moving or running toward the intrinsic feedback provided, using a practice schedule that is
basket. When generalization is the goal, practice sessions optimal for the type of task.192 Children with DCD benefit
can progress from stable (shooting from specific positions from experiential and guided learning when practice is per-
on the court) to a changeable environment (such as shooting formed so that each repetition of the action becomes a new
basketballs with a person trying to block the shot). Opportu- problem-solving experience. To test whether motor learning
nity and variety in practice appear to improve motor learn- has occurred, the therapist must create opportunities for
ing, particularly when skills are practiced in a random man- demonstration of retention (repeating what was learned in a
ner. Practice should therefore be varied and occur in multiple previous session), transfer (perform a different but closely
environments (e.g., home and school) to maximize motor related task), and generalization (perform a learned task in a
learning. new environment).
Different types of feedback also affect the process of One method of intervention based on the principles of
learning. Intrinsic feedback is received from any of the motor learning is the CO-OP, a frame of reference devel-
child’s internal sensory systems and is usually not per- oped as a treatment approach specifically for children with
ceived consciously unless external direction draws atten- DCD.144 In this cognitive-based approach, the therapist
tion to it (e.g., when a child performs a task with his or her focuses on the movement goal and facilitates the child’s
tongue sticking out). Extrinsic feedback is received from identification of the important aspects of the task, exam-
an outside source observing the results of an action and ines the child’s performance during the task, identifies
can be provided in the form of knowledge of performance where the child is having the most difficulty, and problem
(KP) or knowledge of results (KR). KP focuses on move- solves alternative solutions.192 Rather than using verbal
ments used to achieve the goal, whereas KR focuses on the instructions, this approach uses guided questions to help
outcome. the child discover the problems, generate solutions, and
Therapists tend to provide excessive feedback, espe- evaluate his or her attempts in a supportive environment.
cially when task performance is below what is expected. Furthermore, the therapist solicits verbal strategies from
Low frequency and fading feedback, progressively de- the child that can help guide the motor behavior, such as
creasing the rate at which feedback is provided, appear typical verbal cues that the therapist tends to provide dur-
to be most effective in facilitating learning.129 One pro- ing intervention.
posed reason is that with less feedback the individual can To benefit from the CO-OP approach, the child must have
more readily engage in the processes that enable learning sufficient cognitive and language ability to rate the level of
versus focusing on external cues. During intervention, his or her performance and satisfaction of self-identified
feedback should not be provided for every movement or goals using the Canadian Occupational Performance Mea-
task execution. It is more beneficial to offer children the sure (COPM).193 The basic objectives of this approach in-
opportunity to self-evaluate and correct their own perfor- clude skill acquisition, cognitive strategy development, and
mance. The therapist can provide feedback as necessary to generalization or transfer of skills into daily performance.
encourage successful task completion and reduce frustra- CO-OP is delivered over 12 one-on-one sessions, each last-
tion. Verbal feedback can be general—”Did that work?”— ing approximately 1 hour. The therapy process is divided
or specific—”Do you need to throw it harder or softer to into five phases: preparation, assessment, introduction, ac-
reach the target?” quisition, and consolidation. Children are taught to talk
Children with DCD often lack the skills required to themselves through performance issues using an approach
analyze task demands, interpret environmental cues, use of Goal-Plan-Do-Check. Domain-specific strategies are
knowledge of performance to alter movements, or adapt to used to enhance performance, with the purpose of helping
situational demands.190 They therefore do not interpret the child to see how he or she can set goals, plan actions,
and use sensory or performance feedback as well as chil- talk through doing, and check outcomes. Using this frame of
dren who are developing typically.183 Motor observations reference, therapists help the child acquire occupational
of the child with DCD often reveal clumsiness, difficulty performance skills using a metacognitive problem-solving
judging force, timing, and amplitude of motions, and process.144
deficits in anticipating the results of a motor action. Reac- Research on the CO-OP Model of Motor Learning.
tions, movements, and response times are typically Current beliefs regarding the nature of motor learning for
slower.191 With this in mind, the type of task and the children with DCD suggest that assessment of participation,
method of teaching should be considered when recom- versus impairments, should be used to determine change
mending participation in sports and leisure activities.135 over time.183 By increasing the child’s ability to participate
Children with DCD can become successful in repetitive in childhood activities, secondary deficits such as loss of
sports, such as swimming, skating, skiing, and bicycling. strength and endurance might be prevented. Relatively new
Ball-related sports, however, such as hockey, baseball, intervention strategies that employ contemporary motor
tennis, football, and basketball, tend to be more difficult learning principles emphasize the role of cognitive pro-
and frustrating owing to the high level of unpredictability cesses (top down) in development of specific skills. The
and frequent changes in the direction, force, speed, and CO-OP model uses this approach to help children achieve
distance of the movement.135 their functional goals.144
Research on the effectiveness of this approach to improve difficulty keeping up with the skilled activities in gym class
motor skills and functional performance is limited but shows such as rope jumping, components of these activities will be
promise. Polatajko and Cantin194 reviewed three articles encouraged, with emphasis on sequencing and timing. The
describing four studies and concluded that there was conver- therapist may use a heavier jump rope or wrist and ankle
gent evidence for the effectiveness of the CO-OP approach weights to provide more sensory information for improved
for children with DCD. task performance.
An exploratory study completed in 1994 by Wilcox as In sensorimotor intervention, tasks are chosen for
part of his graduate work was discussed by Polatajko and their innate sensory and motor components. The child
colleagues.195 This initial single case study included 10 chil- is directed to activities that encourage the use of the body
dren aged 7 to 12 years who were referred to occupational in space to complete a structured motor sequence. Activi-
therapy for motor problems. Using a global problem-solving ties incorporate sensory components such as movement
approach to intervention, this study sought to identify (vestibular), touch (tactile), and heavy work for the mus-
whether children with DCD could use these strategies to cles and joints (proprioception). Play interactions are
acquire skills of their choice, and, once learned, whether the considered important to encourage sensorimotor integra-
skills were maintained and performance in other areas en- tion within the context of meaningful interactions with
hanced. Children selected skills that were challenging and persons and objects.196 Children may propel themselves
meaningful for them, such as shuffling playing cards, apply- prone on a scooter board through an “obstacle maze”
ing nail polish, making a bed, and writing legibly. Each of while looking for matching shapes, for example. This
the 10 children made gains in the chosen activity, with 29 of activity provides tactile, proprioceptive, and vestibular
the 30 targeted skills showing improvement. sensory input and encourages the development of postural
A pilot study compared the CO-OP model to a traditional strength and endurance while addressing perceptual skill
treatment approach with a group of 20 children aged 7 to development.
12 years. Findings indicated that the CO-OP model of inter- Research on Sensorimotor Intervention. Sensori-
vention produced larger gains on client-selected goals. motor intervention is a widely accepted modality, used by
Improvements in self-ratings of performance and satisfac- 92% of school-based therapists as a foundation for improv-
tion were greater than in the comparative group. Although ing handwriting.199 The activities used and goals addressed
informal, the follow-up data suggested that children main- in treatment are extremely varied, as all functional motor
tained their acquired skills and applied strategies to other skills involve some level of sensory and motor organization.
motor goals. Activities can range from horseback riding to using a vibrat-
Limitations in making conclusions regarding the effec- ing pen when learning how to write letters. Owing to the
tiveness of this treatment are mandated by the small enormous variation in intervention strategies and outcome
number of research studies, primarily carried out by the measures, operationalizing treatment to make comparisons
same research group. Mandich and colleagues97 suggest between research studies can be difficult. In a recent system-
that larger studies with control groups are needed. Sug- atic review Polatajko and Cantin194 found five studies that
gestions for future research include identification of met their criteria for using sensorimotor intervention. In
the salient features of this treatment approach, as well as those five studies both the techniques used (e.g., therapeutic
determining the generalization and skill transfer to other riding, movement therapy, educational kinesiology) and
settings. the populations addressed (autism, sensory modulation dis-
order, DCD) varied greatly. This review suggested that
Sensorimotor Intervention evidence for the effectiveness of sensorimotor intervention
Sensorimotor activities provide the foundation for the devel- was “inconclusive,” with the heterogeneity of diagnoses
opment of play in children.196 The first level of play (i.e., and functional problems limiting the ability to interpret
sensorimotor) is pleasurable, intrinsically motivated activity efficacy.194
that involves the exploration of sensation and movement.196 Overall, relatively few studies have investigated the effi-
As children react with adaptive motor responses to the array cacy of sensorimotor integration. In an early comparison
of sensations from their bodies and the environment, central study, DeGangi and colleagues200 found that children pro-
nervous system organization occurs. The assumption that vided with structured sensorimotor therapy made greater
the organization of sensory and motor experiences is essen- gains in sensory integrative foundations, gross motor skills,
tial to effective motor performance is the premise of senso- and performance areas such as self-care than children who
rimotor intervention.197 Treatment encourages the child to engaged in child-centered activity. More recently, Chia and
actively engage in a variety of sensory-rich, motor-based Chua201 used sensorimotor intervention in a random con-
activities, to enhance functional motor performance.195 Evo- trolled study of 14 children with learning disabilities and
lution of sensorimotor intervention has not revolved around DCD. Intervention consisted of providing sensory stimuli
a single, unified theory but has incorporated a variety of and facilitating a normal motor response while remediating
theoretical foundations.198 impairments in posture and muscle weakness. Positive re-
The goals of sensorimotor intervention are outcome sults in neuromotor functioning were noted. In a second
based, with emphasis on the development of age-appropriate study, Inder and Sullivan202 used educational kinesiology
perceptual-motor and gross motor skills. The therapist techniques in four single-subject design experiments. Posi-
chooses activities that meet the child’s developmental levels, tive gains were documented in some aspects of sensory
promote sensory and motor foundations, and encourage organization and in an overall decrease in the number of
practice of appropriate motor skills. For the child having falls children had.
Four studies have explored the effects of sensorimotor who interact with the child on how to develop specific
remediation on handwriting. Those programs that used targeted skills. All caregivers are actively involved in goal
sensorimotor interventions over only a short period of time development and achievement. By having a variety of indi-
did not yield positive results. Sudsawad and colleagues203 viduals work together across daily life environments, children
compared the effects of kinesthetic-based intervention more quickly become skilled.145 This approach also develops
with handwriting practice with 45 first graders over a the caregivers’ ability to understand the demands of specific
4-week period. They found neither group to make signifi- tasks and to help facilitate the child’s performance in all
cant improvements in handwriting, and suggest that there settings.
is no support that kinesthetic training improves hand
writing legibility for this age. In 2006, Denton and col- Physical Fitness Training
leagues204 compared sensorimotor intervention with thera- As previously reviewed, children with DCD are at great risk
peutic practice in 38 school-age children with handwriting of low levels of physical fitness. The benefits of fitness and
difficulties over 5 weeks. These authors noted moderate physical activity in minimizing disease and maximizing
improvements in handwriting with therapeutic practice and overall wellness are well documented. Deleterious effects
a decline in ability in the sensorimotor group. They suggest resulting from DCD or factors associated with it include but
that although sensorimotor foundations did improve with are not limited to fatigue, hypoactivity, poor muscle strength
sensorimotor intervention, there is no indication that these and endurance, decreased flexibility, poor speed and agility,
foundations affect the development of handwriting. Their and diminished power.
findings suggest that structured therapeutic practice using Specific muscular training may be needed to undo the
motor learning principles has a much stronger impact on effects of reduced activity.207 Poor muscle strength, espe-
the development of handwriting. cially in the abdominal area, can lead to musculoskeletal
Two studies that investigated the combined effects of issues such as back pain because posture and pelvic align-
sensorimotor intervention and higher-level teaching strate- ment require adequate muscle strength. Children with DCD
gies did demonstrate a positive impact on handwriting.205,206 often require specific instruction to perform muscle strength-
Peterson and Nelson205 found that low socioeconomic first ening activities (e.g., sit-ups, push-ups) with appropriate
graders who received 20 sessions of occupational therapy form. Decreased flexibility and muscle tightness in the
combining sensorimotor, biomechanical, and teaching- lower extremities can contribute to difficulties in running,
learning strategies made significant gains over those receiv- jumping, and hopping. Flexibility can be encouraged with a
ing academic instruction alone. Weintraub and colleagues206 regimen of stretches specific to the areas of tightness. Gentle
compared a control group with two treatment conditions and regular stretching can be incorporated into warmups
(task-oriented approach versus combination of sensorimotor during sessions or physical activities.
and task orientation). Immediately after treatment, and at a Fitness can improve and be maintained when children
4-month follow-up, significant gains in handwriting were participate in regular, preferably daily, physical activity.
observed in both treatment groups compared with the con- These activities often require more structure and direction
trol group. The authors support the use of “higher-level” for children with movement difficulties. As therapists, our
teaching strategies to improve the skill of handwriting. overall goal should be to educate children about the value
and enjoyment of regular activity.207 Hands and Larkin207
Motor Skill Training suggest the following plan to ensure children with DCD
Motor skill training involves learning skills and subskills learn or rediscover the joy of movement:
functionally relevant to the child’s daily performance. Tasks n Educate children to understand and monitor their bodies’
are taught in a sequential manner by developmental ages or responses to exercising (e.g., heart rate increases when
by steps from simple to complex. Skill training can occur for they run).
a wide variety of gross, fine, and visual motor tasks, as well n Assist them in finding developmentally appropriate
as activities of daily living. An assortment of theoretical activities they will enjoy with some success.
models and techniques may be used based on the child’s n Encourage them to maintain a healthy, active lifestyle
impairment and activity and participation deficits. by encouraging participation in lifelong activities such
Motor skill training can involve both indirect and direct as swimming, cycling, golf, sailing, yoga, or weight
facilitation of specific motor tasks. Activities that include training.
balance, locomotion, body awareness, and hand-eye coordi- In sports and leisure activities, the emphasis should be on
nation can improve functional skills such as being able to sit participation and fitness rather than competition. Encourage
at a desk within the classroom and complete written work, activities that do not require constant adaptation, as children
as well as success in recess games such as basketball. Spe- with DCD tend to be more successful in sports that have a
cific skills such as dribbling and foul shooting can also be repetitive nature to the movements (e.g., swimming, running,
specifically taught and practiced. The goal is to provide a skating, skiing).135,207 Sports that have a high degree of spatial
great variety of motor activities at the child’s developmental challenge or unpredictability, such as baseball, hockey, foot-
motor level to promote motor generalizations for more suc- ball, and basketball, are less likely to be successful for children
cessful participation. with DCD.208 Activities that are taught through sequential
An example of this approach is Sugden and Henderson’s145 verbal guidance, such as karate, may be easier to learn.135
“ecological intervention,” which is a method of skill training PTs and OTs can have a positive impact on participation
for children with DCD. In this model the therapist, called a in fitness activities for children with DCD. A summary of
movement coach, provides instruction to many individuals key suggestions,83 including the following, can assist with
encouraging involvement in community sports and leisure Several attributes have been identified as predictors of
activities: success for adults with learning disabilities.212,215 A combi-
n Provide frequent encouragement and reward effort. nation of internal and external factors supports the individual
n Encourage participation, rather than competition; in the belief that he or she can take control, evaluate needs,
emphasize fun, fitness, and skill building. and develop appropriate coping strategies, while knowing
n Use a variety of teaching methods to demonstrate when to seek additional help.215 The ability to be proactive,
new skills (one-on-one instruction, verbal cues, set goals, and persevere is a key internal element to attaining
demonstration). success. Having an understanding of one’s learning disabil-
n Provide hand-over-hand instruction during the early ity, with recognition of strengths and limitations, affords
acquisition phase. more thoughtful choices in life roles. Self-esteem and confi-
n Break down skills into smaller, meaningful parts. dence are promoted with external emotional support and
n Keep the environment as predictable as possible. positive feedback from family, friends, teachers, work col-
n Modify or adapt equipment for safety (e.g., use foam leagues, and employers.
balls instead of hard balls). The persistent motor coordination difficulties attributed
n Focus on the enjoyment, not the product. to DCD further affect perceptions of competence89,216 and
n Encourage multiple roles in some activities (e.g., referee, successful accomplishment of daily life skills.217 Raskind
scorekeeper, time keeper). and colleagues212 found that physical status, including motor
n Recognize the child’s strengths, and reinforce social impairment, was an important variable in determining suc-
interaction. cess in adulthood. Slowness and variability in movement
Encouraging and facilitating participation in a healthy life- continue to be a pervasive feature, causing difficulties in
style can aid in ending the vicious cycle of withdrawal, dimin- tasks that require sequencing and dual-task performance,
ished opportunities for physical development, and decreased such as driving a car.217 Adults with DCD report higher levels
fitness and strength over time, a pattern very commonly seen of difficulty with motor-related tasks such as self-care and
in children with DCD.83 handwriting.218 Compared with adults with dyslexia, a
greater number of adults with DCD continue to live at home
LEARNING DISABILITIES AND with their parents, have fewer spare-time activities, and are
DEVELOPMENTAL COORDINATION more socially isolated.218,219
DISORDER ACROSS THE LIFE SPAN Learning disabilities with motor impairments appear to
Learning disabilities persist into adulthood and present life- have a persistent effect on a sense of competence and self-
long challenges. Continuing issues with attention, cognition, concept.8,219 Many individuals develop negative perceptions
emotional adjustment, and interpersonal skills can affect of themselves as they experience frustration and ineffective-
education, employment, family life, and daily routines. Ado- ness. They set lower aspirations, further reinforcing the
lescents and adults with learning disabilities frequently cycle of failure. A combination of ADHD and DCD was
struggle with the concentration and organization needed to found to be the most important predictor of poor psychoso-
effectively manage daily routines and finances, vocational cial functioning in early adulthood.220 Higher incidence of
education or training, job procurement and retention, and drug and alcohol abuse, affective disorders, crime convic-
finances.209,210 tion, and unemployment have been documented. Without
A recent longitudinal study211 demonstrated that IQ adequate support, adults with DCD have difficulties reach-
scores remain stable from childhood to adulthood, as do ing their potential. They may benefit from counseling about
deficit areas. Therefore, poor readers remain poor readers, their condition, vocational assessment and guidance to assist
poor spellers remain poor spellers, and delayed math skills with finding a suitable work environment, time management
persist. In addition, adults who had affective illness or mood and organizational strategies, workplace and academic accom-
disorders as children have a significant risk of recurrent epi- modations, and behavioral management.
sodes (e.g., depression, bipolar disorder). An early 20-year These cycles of ineptitude, frustration, and poor self-
longitudinal study of individuals with learning disabilities concept are highlighted in Case Study 14-2. Paul’s mother,
cited a rate of 42% for adult psychological disturbance (e.g., Mrs. B., was not diagnosed with learning disabilities until
depression, alcohol abuse, anxiety disorders), compared age 20 years. Nevertheless, she completed both bachelor’s
with 10% in the general population.212 and master’s degrees in counseling. Although the academic
More recently, Seo and colleagues213 found more opti- frustrations are no longer an issue, the learning disability
mistic results in their comparison of outcomes for individu- continues to interfere with her work and home performance.
als age 21 and 24, with and without documented learning Mrs. B. describes her organizational difficulties and identi-
disabilities. No significant differences were found in post- fies a continuous need to make lists to function in her job.
secondary school achievement or employment rates and She concentrates on not looking “clumsy” and is fearful she
earned income, although the 21-year-olds with learning will trip over things and look foolish. Learning and accom-
disabilities did receive significantly more public aid, such plishing tasks continue to require increased effort compared
as food stamps, social security, and unemployment. The with her peers. Thus even in adulthood the learning disability
learning-disabled group did not have increased incidence continues to present difficulty in functional performance.
of committing crimes or feeling victimized as young adults. A letter from a woman with learning disabilities, motor
Emotional health211 and strong social relationships214 coordination impairments, and sensory integration problems
are crucial for success; therefore children should be sup- is included in Box 14-3. She describes how her learning dis-
ported to develop healthy social connections and personal ability affects her current functioning and how it affected her
talents. when she was a child.
BOX 14-3 n A LETTER FROM AN ADULT WITH A LEARNING DISABILITY
I am 26 years old, a professional bassoonist with a master’s Not all teachers were so insensitive. My fourth grade
degree in music performance. My name is Wendy. Through teacher made every effort to let me go at my own pace,
Jane, an occupational therapist, I discovered when I was letting me read on a college level and do 2 years of math
24 years old that I had learning problems and sensory inte- on my own. Left to my own devices, I can learn and love
gration problems. to do so. My fifth grade teacher forced me to do math the
I invert letters and especially numbers. When people long way with steps. I just know the answer by looking at
speak English to me, I feel it’s a foreign language. There’s multiplication or division problems, even algebra prob-
translation lag time. When learning new things, I either lems, but to this day I cannot understand how one does it
understand intuitively or never. I can’t seem to go through in steps. If a teacher didn’t accept this, I was in for a year
step-by-step learning processes. of hell. I cried a lot in school, from frustration mostly, and
Physically, I’m extremely sensitive to motion. When I pretended to be sick a lot.
I was little, we moved every year. I spent the first 5 years I never had friends until college. I guess I was too dif-
of my life feeling sick. It seems that I feel everything more ferent to be acceptable. I grew up in a rigid, repressive,
strongly than most people. I have an extremely low thresh- religious community, which made it especially difficult to
old of pain, and even pleasure tends to overload me. If I am be accepted. My differences were labeled evil, or, at best,
touched unexpectedly it hurts, it’s so jarring. This causes a I was ignored. I left high school at age 16 for college,
lot of problems with interpersonal relationships. I can’t where at least I could structure what I wanted to learn. It’s
stand to have people close to me; it produces an adrenalin never been easy for me to make friends, although it’s better
reaction. now. Music circles tend to be a bit crazy so I fit in more
Motor activities are also a problem; my muscles don’t easily.
seem to remember past motions. Despite the many times My learning disabilities still are problems. My motor
I’ve walked down steps and through doors, I still have to and learning problems get in the way of my music, but my
think about how high to lift my foot and about planning coping mechanisms are strong. I deal better with my clum-
my movements. When eating, I have to think about chew- siness now. Just being diagnosed by Jane has made a big
ing or I bite my tongue or mouth. I don’t think other difference. To have things labeled, to be told and realize
people think about these things. I’m physically inept; that it’s not my fault, has given me a sense of peace. It’s
I can bump into the same table 10 times running. I’m also allowed me to turn from inward depression to outward
always bruised, and as a child people constantly labeled anger at those who labeled me stupid and clumsy. Just
me as clumsy. Physical education courses were hell as a being able to admit anger allows one to let it go.
child, especially gymnastics, where you are forced to Other than my testing and subsequent conversations
leave the ground and swing or walk on balance beams with Jane, I have not received treatment for my problems.
or uneven bars. I cannot begin to explain the terror or I believe that adults with my problems can be helped.
disorientation. I wish programs were available in all areas of the country.
Academically, I was labeled stupid or, more frequently, At age 26, I feel much better about myself than I did even
lazy. I was told that I was not trying. Actually, my IQ is at age 24. It’s a matter of growth and coping with major
high and my coping mechanisms are complex. If they only differences.
knew how hard I was trying. I was lucky because I taught The greatest advice I would give to educators and
myself to read at an early age. I would never have learned therapists working with problem children is to accept. Ac-
to read otherwise. Even so, my first grade teacher wouldn’t cept what they can do well; don’t make an issue of what
believe that I could read so far past my age. She called me they can’t do. We all have our strengths and weaknesses. If
a liar when I said that I had finished each “Dick and Jane” a child can’t do math, so what! Buy the child a calculator
book. I was forced to read each one 50 times before she and the child will do a lot better with it than with a label
would give me a new one. of stupidity following her through life.
SUMMARY in the child (i.e., a true learning disability) versus issues

Learning disabilities are heterogeneous and multidimen- from inadequate or ineffective instruction. Early identifica-
sional in their presentation. Research continues to work on tion of difficulties and systematic alteration of instruction
identifying the causes and the associated functional deficits methods are advocated to serve students more quickly and
of learning disabilities, as well as developing effective inter- efficiently. The challenge for the clinician is to recognize the
vention techniques. The variability of the group suggests a multitude of components that interact to impede functional
spectrum of neurological processing difficulties. As physi- abilities and social participation for the child with learning
ological measures of brain function improve, our theoretical disabilities.
understanding increases. Children with learning disabilities frequently manifest
Assessment and teaching methods continue to be refined motor coordination problems. These motor deficits may be
to identify underlying deficits and effective remediation subtle and difficult to identify in a neurological evaluation or
strategies. The current trend is to recognize issues inherent standardized testing. With better awareness and assessment
measures, more children are being identified and diagnosed including social and emotional functioning, self-care, edu-
with DCD. Many theoretical models have been developed in cation, vocation, and interpersonal relationships. Both in-
an attempt to explain the qualitative motor deficits observed trinsic factors (perseverance, insight, sense of control) and
in children with learning disabilities as well as provide con- extrinsic factors (emotional support, mentoring, positive
structs to develop intervention programs. Continued formal feedback) play a role in the ultimate success of the indi-
research and careful documentation of clinical outcomes are vidual with learning and motor challenges. Our role as a
needed to synthesize therapeutic approaches that best meet pediatric therapist is to provide the necessary extrinsic sup-
the individual needs of the child. port and effective intervention to alleviate the deleterious
Identification, advocacy, teaching, and remediation are effects of living with a disability.
important aspects of the OT’s and PT’s roles. The goal is to
formulate an intervention program that best addresses the References
underlying deficits in foundation skills and the functional To enhance this text and add value for the reader, all refer-
weaknesses in daily life tasks. The experienced interven- ences are included on the companion Evolve site that ac-
tionist will combine knowledge from many areas of theo- companies this textbook. This online service will, when
retical development and remediation to facilitate the best available, provide a link for the reader to a Medline abstract
performance in each child. for the article cited. There are 234 cited references and other
It is clear that learning disabilities and DCD both persist general references for this chapter, with the majority of
across the life span and can have a multitude of detrimental those articles being evidence-based citations.
effects. All areas of daily life performance can be affected,
APPENDIX 14-A n A Summary of Standardized Motor Tests

Bruininks-Oseretsky Test of Motor Proficiency, Construction and Reliability
Second Edition (BOT-2) (2005)111 The Bruininks-Oseretsky Test has been carefully standardized on
Authors 1520 subjects from 38 states, stratified across sex, ethnicity, socio-
Robert H. Bruininks, PhD and Brett D. Bruininks, PhD economic, and disability status. Sample sizes were larger in the
Source younger age ranges, as children develop more rapidly than adoles-
American Guidance Service, Inc., Circle Pines, MN 55014 cents. Three measures of reliability were determined for subtests,
Ages composites, short form, and complete form: internal consistency,
4 to 21 years test-retest, and interrater. In general all were high, with best reliabil-
Administration ity shown when the complete form was used.
Individual. Complete form: 45 minutes to 1 hour. Short form: Comment
15 to 20 minutes. The Bruininks-Oseretsky Test of Motor Proficiency–2 appears to
Equipment be one of the better standardized tests of motor performance. The
Test kit needed 1978 version has been one of the most widely used tests to assess
Description motor proficiency and has been used in research to identify motor
The Bruininks-Oseretsky Test of Motor Proficiency–2 is the most deficits in individuals with DCD.221,222 It offers various levels of
recent revision of the Oseretsky Tests of Motor Proficiency, first testing and screening with a comprehensive “complete form” that
published in Russia in 1923. The Bruininks-Oseretsky Test is de- provides the most reliable measure of overall motor proficiency.
signed to provide information on a wide range of motor skills and The short form can be used for screening, to determine if further
is sensitive enough to identify mild to moderate motor control evaluation is necessary. In addition, only those single subtests or
problems. In this revision the authors worked to improve the test composites that are relevant to the child’s areas of difficulty can be
item presentation, quality of the test kit, and functional relevance administered. When qualifying a student for special education, it is
of test content. They also expanded the norms through age 21 and suggested that this thorough assessment be used. In this revision
improved the test items for 4- to 5-year-olds. The BOT-2 yields the authors focused on including a variety of tasks that are engag-
standard scores, scaled scores, and percentile ranks, separately for ing and goal directed, assessing both quantitative and qualitative
males and females, and combined. Age equivalency scores are also aspects of motor performance. When testing children with motor
available. The test assesses motor functioning in eight areas: dysfunction, careful attention must be paid to qualitative perfor-
1. Fine Motor Precision: Functionally relevant drawing, paper mance on individual items. A child may demonstrate the ability
folding, and cutting item that focuses on assessment of pre- to complete a test item, but qualitatively it is accomplished with
cise finger control. These items are untimed and they focus increased effort, decreased refinement, and speed.
on qualitative refinement. Movement Assessment Battery for Children
2. Fine Motor Integration: Copying geometric shapes of in- (Movement ABC-2) (2007)114
creasing complexity as accurately as possible. These items Authors
are untimed, as they focus on precision. Sheila E. Henderson, David A. Sugden, and Anna L. Barnett
3. Manual Dexterity: Goal-directed activities that involve reach- Source
ing, grasping, and bimanual coordination with small objects Harcourt Assessment, Proctor House, 1 Proctor Street, London,
(pennies, pegboard, card sorting, and stringing beads). Em- WC1V6EU
phasis is placed on speed and accuracy (dexterity), and all Ages
items are timed. 3 to 16 years
4. Bilateral Coordination: Items that measure skill in sequential Administration
and simultaneous coordination of the upper and lower ex- Individual; 20 to 30 minutes
tremities. Includes both familiar tasks (e.g., jumping jacks Equipment
and finger pivots [“itsy-bitsy spider”]), as well as novel tasks Test kit required
(e.g., tapping feet and fingers with opposite sides of the body). Description
5. Balance: Static and dynamic balance items measure motor The Movement ABC-2 is a recent revision of the M-ABC, which
control for maintaining posture when standing, walking, and was originally developed from the Test of Motor Impairment
making transitional movements in space (e.g., reaching for (TOMI)–Henderson Revision. The Movement ABC-2 is a norm-
a plate on a shelf). Assesses three areas that affect balance: based assessment of gross and fine motor performance whose main
trunk stability, static and dynamic postural control, and use intent is to identify and describe children with movement difficul-
of visual cues. ties. The test was also designed for intervention planning, program
6. Running Speed and Agility: Four activities to assess speed and evaluation, and research. There are three components of the Move-
agility (shuttle run, hopping on one and both feet, stepping ment ABC-2: the standardized evaluation, a checklist of compe-
over balance beam). tency in specific daily motor behaviors, and a companion manual
7. Upper-Limb Coordination: Items that involve visual tracking for program planning (Ecological Approach to Intervention).145
and coordinated hand and arm movements (eye-hand coordi- The evaluation tool yields standard and percentile scores, with two
nation). Tasks include catching, throwing, and dribbling, determined cut-off points delineating definite movement difficulties
with one or both hands together. and children at risk. The age ranges have been expanded, and the
8. Strength: Assesses trunk and upper and lower extremity test is divided into three age brackets: children aged 3 to 6 years,
strength necessary for effective gross motor performance in 7 to 10 years, and 11 to 16 years. The test items vary in each age
daily activities. range, with increased complexity and skill required in the older age
Continued
APPENDIX 14-A n A Summary of Standardized Motor Tests—cont’d

bands. There are eight tasks assessing three areas of coordination: subtests that measure interrelated motor abilities that develop early
manual dexterity, ball skills, and static and dynamic balance. in life. The PDMS-2 can be used by OTs, PTs, diagnosticians, early
1. Manual Dexterity: Speed and dexterity with the preferred intervention specialists, adapted physical education teachers, psy-
hand. Includes two manipulative tasks such as coins in chologists, and others who are interested in examining the motor
a bank, stringing beads, pegs in a pegboard, threading abilities of young children. The PDMS was designed for use with
lace, and turning pegs and nuts and bolts, as well as a children who show delay or disability in fine and gross motor
written maze. skills. Test items are similar to those on other developmental
2. Ball Skills: Eye-hand coordination is assessed with two scales, but only motor items are included. Items are scored on a
beanbag and ball tasks emphasizing aiming at a target and 3-point scale: 0 for unsuccessful, 1 for partial, and 2 for successful
catching. performance. Age-equivalent scores, motor quotients, percentile
3. Static and Dynamic Balance: One task of static balance with rankings, and standard scores are provided. Scoring software for
eyes open, and two tasks of dynamic balance that emphasize the PDMS-2 is available to convert the PDMS-2 scores into stan-
spatial precision and control of momentum. dard scores, percentile ranks, and age equivalents and generate
Construction and Reliability composite quotients. The software also can be used to compare
The standardization sample included 1172 children in the United PDMS-2 subtest performances and composite performances to
Kingdom. A stratified sampling was done to ensure that representative identify intraindividual differences and provide a printed report of
proportions of age, gender, race and ethnicity, and parent educational the student information, including treatment goals and objectives.
level were included. Before the main study, testing was done around Subtests
the world and revealed no culture-specific problems. The researchers 1. Reflexes: This eight-item subtest measures a child’s ability to
consider this revision to be similar enough to the M-ABC that the automatically react to environmental events. Because reflexes
studies employed with that test remain relevant. Test-retest reliability typically become integrated by the time a child is 12 months
for consistency of individual item scores fell within a range deemed old, this subtest is given only to children from birth through
acceptable, from 0.64 to 0.86. The mean of 0.77 was achieved for the 11 months.
test as a whole. Interrater reliability was excellent, exceeding 0.95. 2. Stationary: This 30-item subtest measures a child’s ability to
Comment sustain control of his or her body within its center of gravity and
This revision is the culmination of a lengthy program of research retain equilibrium.
and development begun in 1966 by two groups of researchers. The 3. Locomotion: This 89-item subtest measures a child’s ability to
Movement ABC-2 offers some additional advantages: (1) the move from one place to another. The actions measured include
checklist helps teachers identify children with movement prob- crawling, walking, running, hopping, and jumping forward.
lems; (2) information is provided for a cognitive-motor approach 4. Object manipulation: This 24-item subtest measures a child’s
to intervention across environments. The test items are easy to ability to manipulate balls. Examples of the actions measured
administer and score. Although the manual provides a clear picture include catching, throwing, and kicking. Because these skills
of what the task looks like, there are no standardized verbal in- are not apparent until a child has reached the age of
structions given in this revision. Scoring is based on a traffic light 11 months, this subtest is given only to children aged 12 months
system, with the red zone (at or below 5th percentile) indicating and older.
significant movement difficulty, amber zone (6th to 15th percen- 5. Grasping: This 26-item subtest measures a child’s ability to use
tile) delineating children at risk, and the green zone (above 15th his or her hands. It begins with the ability to hold an object with
percentile) indicating the absence of movement difficulties. Per- one hand and progresses up to actions involving the controlled
centile scores are useful for parents and teachers because they are use of the fingers of both hands.
easily understood, but therapists should recognize that they do not 6. Visual-motor integration: This 72-item subtest measures a
form an equal interval scale, tending to cluster near the median of child’s ability to use his or her visual perceptual skills to per-
the normal curve. Therefore for subtest raw scores near the median, form complex eye-hand coordination tasks such as reaching and
a change of 1 point to the raw score could result in an increase of grasping for an object, building with blocks, and copying
8 percentile points, whereas at either end of the normal curve this designs.
might only translate to 2 percentile points. Composites
Peabody Developmental Motor Scales, Second 1. Gross Motor Quotient: This composite is a combination of
Edition (PDMS-2) (2000)113 results on the subtests that measure the use of the large muscle
Authors systems:
M. Rhonda Folio and Rebecca R. Fewell n Reflexes (birth to 11 months only)
Source n Stationary (all ages)
Pro-Ed, 8700 Shoal Creek Boulevard, Austin, TX 78757 n Locomotion (all ages)
Ages n Object manipulation (12 months and older)
Birth to 5 years 2. Fine Motor Quotient: This composite is a combination of
Administration results on the subtests that measure the use of the small muscle
40 to 60 minutes (test items may be scored by direct observation or systems:
by parent or teacher report) n Grasping (all ages)
Description n Visual-motor integration (all ages)
An early childhood motor development program that provides, in 3. Total Motor Quotient: This composite is formed by a combina-
one package, both in-depth assessment and training or remediation tion of results on the gross and fine motor subtests. Because of
of gross and fine motor skills. The assessment is composed of six this, it is the best estimate of overall motor abilities.

Construction and Reliability the clinical observations used by therapists have not had stan-
Reliability coefficients were computed for subgroups of the norma- dardized administration or objective scoring and they have not
tive sample (e.g., individuals with motor disabilities, African taken into account the child’s age and changes in abilities as the
Americans, Hispanic Americans, girls, and boys) as well as for the child matures. The authors felt it was important to objectify
entire normative sample. The normative sample consisted of 2003 these observations and relate performance to age, as neuromotor
persons residing in 46 states and was collected in the winter of maturation is very rapid in this age group. The test is designed
1997 and spring of 1998. Normative samples relative to geography, to measure cerebellar function, postural control (stability), and
sex, race, and other critical variables are therefore representative of motor coordination (mobility). Motor planning and sequencing
the current U.S. population. are not intended to be measured directly, and repetition of directions,
A test-retest reliability of 0.84 for the Gross Motor Scale and of therapist prompting, and item practice are meant to separate
0.73 for the Fine Motor Scale (0.89 Total Motor) was reported based motor performance from the cognitive aspects of planning. The
on a sample of 30 children from Austin, Texas, in the age range of test has six test items:
2 to 11 months. A second group of 30 children from Nacogdoches, 1. Slow Movements: Assesses the ability to move arms in a
Texas, ages 12 to 17 months, were tested with a test-retest reliability slow and symmetrical manner. Scoring is based on quality
of 0.93 for the Gross Motor Scale and 0.94 for the Fine Motor Scale of performance, speed, and symmetry.
(0.96 Total Motor). These values are of sufficient magnitude for a 2. Rapid Forearm Rotation: A test of diadochokinesis; score is
tester’s confidence in the test scores’ stability over a period of time. based on the number of forearm rotations accurately com-
The correlation coefficients between the PDMS and the PDMS-2 for pleted in 10 seconds.
criterion-prediction validity in the Gross and Fine Motor Quotients 3. Finger-Nose Touching: Measures proprioceptive mecha-
exceed 0.80, which supports the equivalency of the tests. nisms of motor control by switching midtask from eyes
The PDMS-2 scores were correlated with those of the Mullen open to eyes closed. Scoring is based on accuracy, fluidity of
Scales of Early Learning: AGS Edition (MSEL:A) when both tests motion, and force of touch.
were administered on the same day to 29 children, aged 2 months to 4. Prone Extension Posture: The child holds a position of
66 months, in Evansville, Indiana. The relations of the PDMS-2 and extension against gravity. Scoring is based on the duration
MSEL:A demonstrated that Gross and Fine Motor Quotients exceed of ability to hold position, quality of extension, and effort.
0.80, high enough to support the equivalency of the tests. When the 5. Asymmetrical Tonic Neck Reflex: The degree of inhibition
concurrent validity of the age equivalent and standard scores of the of this reflex is looked at by measuring elbow flexion with
Bayley Scales of Infant Development II (BSID-II) Motor Scale and manual head turn in a quadruped position. This test is also
PDMS-2 were calculated, the standard scores show poor agreement useful for identifying poor postural stability through obser-
and had low concurrent validity, particularly the BSID-II Motor vations including needing a wide base of support to sustain
Scale and the PDMS-2 Locomotion Subscale.223 The differences in the position and locking elbows for stability.
the scores of these two tests warrant concern when using one test to 6. Supine Flexion Posture: The child holds a position of flexion
make clinical decisions for service eligibility. against gravity. Scoring is based on the duration of ability to
Comment hold position, quality of extension, and effort.
The PDMS-2 is primarily useful for children with mild to moderate Construction and Reliability
motor deficits, such as a child with learning disabilities or a child with Standardization of the test was done in two cities (Calgary and
developmental delay. The test does not discriminate among children Hamilton) on a sample of 123 children, 67 who demonstrated DCD
with moderate to severe motor disability because they fall far below and 56 with no known motor problem. Test-retest reliability, inter-
the standard scores given. The skill categories are unevenly distrib- rater reliability, internal consistency, and construct validity were
uted and have too few items at some age levels to be meaningful. completed with a sample of 132 children with and without DCD.
Despite the drawbacks, the PDMS-2 is probably the most valuable Test-retest reliability over 2 weeks was 0.98, and interrater reli-
motor scale test currently available for preschool children. ability for pediatric OTs was 0.87. The internal consistency was
Clinical Observations of Motor and Postural Skills, high, indicating that the test discriminates well between children
Second Edition (COMPS) (2000)126 with and without motor problems, although the sample size was
Authors small.
Brenda N. Wilson, MS, OT(C), Nancy Pollock, MSc, OT(C), Comment
Brenda Kaplan, PhD, and Mary Law, PhD, OT(C) The COMPS is a useful standardized tool for identifying subtle
Source motor problems in children and was designed for and tested on
Therapro Inc., 225 Arlington Street, Framingham, MA 01702 children with DCD. The COMPS was not designed for children
Ages with known neurological or neuromotor problems such as intel-
5 to 11 years lectual delay, cerebral palsy, or epilepsy. It can be used as a screen-
Administration ing tool for motor dysfunction, to assist in determining intervention
Individual; 15 to 20 minutes approaches that would be beneficial for the child, and possibly to
Equipment measure change over time. Children with motor performance prob-
Test kit required for ATNR measurement tools. Stopwatch and mat lems can score within normal limits on the COMPS, and children
needed for certain items. who demonstrate low scores may not be exhibiting functional dif-
Description ficulties. The association between these clinical observations and
The COMPS is a standardized screening tool using six clinical functional performance is not always clear; therefore it is essential
observations suggested by Ayres99,224 of “soft neurological signs” to gather information regarding the child’s current functioning
to identify motor problems with a postural component. Historically, from observation and interview.
Continued

Miller Assessment for Preschoolers (MAP) (1988)127 Source
Author Psychological Corporation, 555 Academic Court, San Antonio, TX
Lucy Jane Miller, PhD 78204-0952
Source Ages
Psychological Corporation, 555 Academic Court, San Antonio, TX 2 years, 9 months to 6 years, 2 months
78204-0952 Administration
Ages Individual; 15 minutes
2 years, 9 months to 5 years, 8 months Equipment
Administration Test kit
Individual; 20 to 30 minutes, including scoring Description
Equipment The FirstSTEP is a quick screening test for identifying develop-
The MAP Test Kit mental delays in all five areas defined by IDEA and mandated by
Description PL 99-457: cognition, communication, physical, social-emotional,
The MAP was designed to identify preschool children who exhibit and adaptive functioning. Twelve subtests assess cognitive, com-
mild to moderate developmental delays. The MAP is a developmen- munication, and motor domains. An optional Social-Emotional
tal assessment intended for use by educational and clinical personnel Scale includes 25 items from five areas (task confidence, coopera-
to identify children in need of further evaluation and remediation. It tive mood, temperament and emotionality, uncooperative antisocial
can also be used to provide a comprehensive, clinical framework that behavior, and attention communication difficulties) that are scored
would be helpful in defining a child’s strengths and weaknesses and on the basis of behaviors observed by the examiner during the test
that would indicate possible avenues of remediation. The test is session. The Adaptive Behavior Checklist is an optional measure
composed of 27 items and a series of structured observations. The completed by parent interview to assess the child’s self-help and
test items are divided into five performance indexes: adaptive living skills. The Parent/Teacher Scale provides additional
1. Foundations: Items generally found on standard neurological information about the child’s typical behavior.
examinations and sensory integrative and neurodevelopmen- Cognitive Domain
tal tests 1. Money Game (quantitative reasoning): The child is asked a se-
2. Coordination: Gross, fine, and oral motor abilities and ries of questions about coins, regarding quantity, amount, com-
articulation parisons, size, and numeration. This subtest requires cognitive
3. Verbal: Cognitive language abilities, including memory, understanding of simple arithmetic concepts.
sequencing, comprehension, association, following direc- 2. What’s Missing? Game (picture completion): The child is asked
tions, and expression to identify what is missing from the pictures of common objects
4. Nonverbal: Cognitive abilities such as visual figure-ground, or events by naming or pointing. This subtest measures visual
puzzles, memory, and sequencing figure-ground as well as gestalt closure abilities.
5. Complex Tasks: Tasks requiring an interaction of sensory, 3. Which Way? Game (visual position in space): The child is
motor, and cognitive abilities asked to look at a stimulus figure that is turned in a specific
Construction and Reliability direction. The child then selects the response figure that
The MAP has been well standardized on a random sample of 1200 matches. This subtest measures visual discrimination and the
preschool children. The sample was stratified by age, race, sex, size ability to perceive directionality visually.
of residence, community, and socioeconomic factors. Data were 4. Put Together Game (problem solving): The child is asked
collected nationwide in each of nine U.S. Census Bureau regions. to select the pieces that best fit a certain space. The subtest
Reported reliabilities are good. In a test-retest on 90 children, 81% requires abstract thinking.
of the children’s scores remained stable. The coefficient of internal Language Domain
consistency on the total sample was 0.798. Interrater reliability on 1. Listen Game (auditory discrimination): This two-part activity
40 children was reported as 0.98. requires the child to listen as the examiner names and points to
Comment three similar-sounding pictures. Then the child chooses the
The MAP was developed by an OT and provides information that is pictures that represent the words. The second part requires the
of particular relevance to therapists. It is carefully standardized and child to discriminate between words that are the same and
fills a need for early identification of learning and motor deficits in words that are different. This task taps phoneme discrimination
children. Several articles have been published supporting the validity and requires good auditory processing skills.
of this test as a screening instrument.225-228 Reviews of the MAP in 2. How Many Can You Say? Game (word retrieval): The child’s
the Ninth Mental Measurements Yearbook have described it as “the linguistic fluency and word-finding skills are measured by ask-
best available screening test for identifying preschool children with ing the child to count, recall animals, and recite rhyming
moderate preacademic problems”229 and “an extremely promising words.
instrument which should find wide use among clinical psychologists, 3. Finish Up Game (association): The child is asked to complete a
school psychologists, and OTs in assessing mild to moderate learn- phrase that is initiated by the examiner. The subtest requires the
ing disabilities in preschool children.”230 A more complete review of child to demonstrate an understanding of the association be-
this test is provided by King-Thomas and Hacker.231 tween concepts (e.g., big and little).
FirstSTEP (Screening Test for Evaluating 4. Copy Me Game (sentence and digit repetition): The child is
Preschoolers) (1993)130 asked to repeat a series of meaningful verbal stimuli and then a
Author series of numbers. This subtest measures verbal memory, gram-
Lucy J. Miller, PhD matical abilities, and verbal expression skills.

Motor Domain Source
1. Drawing Game (visual-motor integration): The child is pre- Pro-Ed, 8700 Shoal Creek Boulevard, Austin, TX 78757-6897
sented with paper and pencil tasks. This subtest requires the Website: www.proedinc.com
integration of fine motor and visual-perceptual abilities. Ages
2. Things with Strings Game (fine motor planning): The child is 5 to 12 years
asked to perform a series of motor movements with the upper Administration
extremities with a wooden cube and a string. These items tap Individual; untimed. Individual scales may be completed in 5 to 10
the ability to plan and execute a series of motor actions and minutes.
measure fine motor planning or praxis. Equipment
3. Statue Game (balance): The child is asked to assume a series of Record form; rating scale guides
increasingly more difficult positions that require the child to Description
balance with eyes open and vision occluded. The subtest taps The SFA is a judgment-based, criterion-referenced assessment to
the abilities needed to maintain equilibrium and screens for evaluate and monitor a student’s performance on functional tasks
proprioception, vestibular perception, and visual processing that support school participation. This measure was designed to
difficulties. facilitate collaborative program planning for students with a vari-
4. Jumping Game (gross motor planning): The child is asked ety of physical and cognitive disabilities. The SFA measures a
to imitate the examiner through a series of increasingly more student’s ability to perform activities within the school setting
difficult tasks that involve jumping in specific patterns. Gross that support participation in the academic and social aspects of an
motor and motor planning abilities are measured. elementary program (grades K-6). Functional skills such as mov-
Construction and Reliability ing around the school, using classroom materials, interacting
The FirstSTEP is norm referenced and was standardized on with peers, and caring for personal needs are included. The
1433 children. Norms are provided in 6-month intervals for each instrument is best completed by school professionals who are
of seven age groups. The standardization sample closely matches familiar with the student’s typical performance. Task items are
demographic characteristics provided by the U.S. Census Bureau. written in measurable, behavioral terms that can be used directly
Scores are reported in standard scores as well as a three-category, in the student’s IEP. Criterion cut-off scores are provided for use
color-coded risk status to indicate whether the child is functioning in determining eligibility for special services. The SFA contains
in the normal or delayed range. The FirstSTEP is a highly reliable three parts:
instrument. Overall test reliability (split half) is 0.90, with indi- 1. Participation: Rates the student’s involvement in six major
vidual domains ranging from 0.71 to 0.87. Test-retest reliability school activity settings: regular or special education class-
indicated a high degree of consistency in the classification of room, playground or recess, transportation, bathroom and
a child’s performance across two test sessions (90% agreement toileting activities, transitions to and from class, and meal-
for composite score; 85% to 93% for individual domain scores). time or snack time.
Results also indicated a high level of interrater agreement (r 5 0.94 2. Task Supports: Identifies and rates the assistance and adap-
on composite scores). tations currently provided to the student for both physical
Validity studies of the FirstSTEP indicate that the FirstSTEP and cognitive and behavioral tasks. Two types of task sup-
has good construct, content, and discriminant validity. Therefore it ports are examined separately: assistance (adult help) and
can effectively identify children with developmental delays. A adaptations (modifications to the environment or program,
study of 900 children demonstrated that children with delays per- such as specialized equipment or adapted materials).
form 1.5 to 2 standard deviations below the mean in all domains. 3. Activity Performance: Examines the student’s performance
The results of a concurrent validity study suggest that the motor of specific school-related functional activities. Physical
domain of the FirstSTEP measures constructs similar to those mea- Tasks include travel, maintaining and changing positions,
sured by the Bruininks-Oseretsky Test of Motor Proficiency and recreational movement, manipulation with movement, using
support the use of the motor domain of the FirstSTEP as an indica- materials, setup and cleanup, eating and drinking, hygiene,
tor of the child’s motor functioning. clothing management, going up and down stairs, written
Comment work, and computer and equipment use. Cognitive/Behavioral
The FirstSTEP is an easy-to-administer test that is highly effective as Tasks include functional communication, memory and under-
a screening instrument. The FirstSTEP was developed by the OT who standing, following social conventions, compliance with adult
also developed the MAP (the Miller Assessment for Preschoolers), directives and school rules, task behavior and completion,
and like the MAP the test provides information that is of particular positive interaction, behavior regulation, personal care aware-
relevance to therapists. Although individual items on the FirstSTEP ness, and safety.
differ from the MAP, many are derived from the MAP, and the test is Construction and Reliability
based on the same theoretical framework as the MAP. Area domains, A sample of 678 students in two groups participated in the stan-
social-emotional scales, and the adaptive behavior checklist from this dardization of SFA. One group included children with special
tool can also be used in conjunction with other tests to provide addi- needs (363 students). These students had a variety of disabilities,
tional information on the child’s areas of strengths and weakness. including motor impairment, communication impairment, emo-
A Spanish version, Primer Paso, is also available for use. tional or behavioral difficulties, and cognitive limitations. The
School Function Assessment (SFA) 1998232 second group included 315 students in regular education programs.
Authors Internal consistency was excellent at 0.92 to 0.98, indicating that
Wendy Coster, PhD, OTR/L, Theresa Deeney, EdD, Jane Haltiwanger, items in a scale relate to one another and measure the same con-
PhD, and Stephen Haley, PhD, PT struct. Test-retest reliability was 0.82 to 0.98.
Continued

Comment 9. Design Copying: Part I—The child copies a design by con-
This assessment is unique in its focus on activity and participation necting dots on a dot grid. Part II—The child copies a de-
levels, versus identifying impairments. It helps school personnel sign without the use of a dot grid; both process and product
recognize and ameliorate functional limitations that are affecting are scored.
successful school participation. The comprehensiveness of assess- 10. Constructional Praxis: Working with blocks, the child
ment and functional IEP-ready goals makes it a useful tool to attempts to duplicate two different block structures. In the
qualify and develop programs for children in need of special edu- first structure, the child observes the examiner building
cation services. Complete instructions are contained in the assess- the model; the second structure is preassembled.
ment booklet. Therefore the respondent does not need to refer to a 11. Postural Praxis: The child imitates unusual body positions
manual to complete items, easing data collection. The manual is demonstrated by the examiner.
then used to compute transformed scores and interpret results. 12. Oral Praxis: The child imitates movements of the tongue,
The Sensory Integration and Praxis Tests (SIPT) lips, and jaw demonstrated by the examiner.
(1989)112 13. Sequencing Praxis: The child imitates a series of simple
Author arm and hand movements demonstrated by the examiner.
A. Jean Ayres 14. Bilateral Motor Coordination: The child imitates a series
Source of bilateral arm and foot movements demonstrated by the
Western Psychological Services, 12031 Wilshire Boulevard, Los examiner.
Angeles, CA 90025 15. Standing and Walking Balance: This subtest consists of
Ages 15 items in which the child assumes various standing
4 years to 8 years, 11 months and walking postures.
Administration 16. Motor Accuracy: The child traces a printed, curved black
Individual; 2 hours; examiner certification required line with a red, nylon-tipped pen, first with the preferred
Equipment hand and then with the nonpreferred hand.
SIPT Test Kit 17. Postrotatory Nystagmus: The child is rotated first counter-
Description clockwise and then clockwise on a rotation board; the dura-
The SIPT is a major revision and restandardization of the South- tion of postrotatory nystagmus, a vestibulo-ocular reflex, is
ern California Sensory Integration Tests.239 Four new tests of observed.
praxis were added, five tests underwent major revisions, eight In addition to these 17 tests, a series of clinical observations
tests underwent minor revisions, and four tests were deleted. aids in interpreting the SIPT. These clinical observations include
The tests are designed to identify sensory integration and praxis the following:
deficits in children with learning disabilities. The 17 tests are n Eye dominance
described as follows: n Eye movements
1. Space Visualization: Select from two blocks the one that will n Muscle tone
fit into a form board. Mentally manipulating the forms is re- n Co-contraction
quired to arrive at the correct choice on the more difficult test n Postural background movements
items. n Postural security
2. Figure-Ground Perception: The child selects from six pictures n Equilibrium reactions and protective extension
the three that are superimposed or embedded with other forms n Schilder’s arm extension posture
on the test plates. n Supine flexion
3. Manual Form Perception: Part I—A geometric form is held n Prone extension
in the hand and the counterpart is selected from a visual n Asymmetrical tonic neck reflex
display. Part II—A geometric form is felt with one hand n Hyperactivity, distractibility
while its match is selected from several choices with the n Tactile defensiveness
other hand. n Ability to perform slow motions
4. Kinesthesia: With vision occluded, the child attempts to n Thumb-finger touching
place his or her finger on a point at which this finger had been n Diadochokinesis
placed previously by the examiner; a separate recording n Tongue-to-lip movements
sheet is provided for each child. n Hopping, jumping, skipping
5. Finger Identification: With hands screened from view, the Construction and Reliability
examiner touches the child’s finger, the shield is removed, The construction of the SIPT was based on a theoretical model
and the child then points to the finger touched. developed from observation of children with learning disabilities
6. Graphesthesia: The examiner uses his or her finger to draw and supported by factor analytical and cluster analysis studies. In-
a design on the back of the child’s hand without the child terpretation follows a clinical model based on patterns of scores
looking; the child then reproduces the design. rather than a poor score on any one test.
7. Localization of Tactile Stimuli: With vision occluded, the The SIPT was nationally standardized on 1997 children from
child touches the spot on his or her hand or arm that was across the United States and Canada. Sex, geographical location,
touched by the examiner with a specially designed pen. ethnicity, and type of community are represented in proportion to
8. Praxis on Verbal Command: The examiner verbally the 1980 U.S. Census. Test-retest reliability was evaluated in a
describes a series of body movements, and the child sample of 41 dysfunctional children and 10 normally functioning
executes them. children with ranges from moderate to high. As a group, the praxis

tests had the highest reliabilities. Interrater reliability is excellent, Comment
with most correlations between raters at 0.90 or higher. The VMI provides a quick and easy method to assess the develop-
Comment ment of a child’s ability to copy geometric forms. The uses
The SIPT is computer scored and interpreted, and a full eight-color are varied, including identification of significant difficulties with
profile (WPS Chronograph) is provided that summarizes major visual-motor integration and determination of an effective inter-
SIPT testing and statistical results in a clear manner. Initial validity vention program, as well as use as a research tool. For complete
studies of the SIPT indicate a good ability to discriminate between assessment of the child with learning disabilities and DCD, it is
normal and dysfunctional groups and across ages. The SIPT is the best used in conjunction with other tests of gross motor, fine motor,
most comprehensive assessment of sensory integration and praxis; visual perception, and eye-hand coordination. This culture-free,
however, it requires specialized training for administration and in- nonverbal assessment is suitable for children with diverse educa-
terpretation, and the test kit and scoring of protocols are expensive. tional, environmental, and language backgrounds. New to this
Beery-Buktenica Developmental Test of addition are teaching materials to promote visual-motor integration
Visual-Motor Integration (BEERY VMI), Fifth for children from birth through elementary school. These include
Revision (2010)115 My Book of Letters and Numbers; My Book of Shapes; a laminated
Authors wall chart of basic gross motor, fine motor, and visual developmen-
Keith E. Beery, PhD, Norman A. Buktenica, and Natasha A. Beery tal milestones; and a checklist for parents of over 200 developmen-
Source tal “stepping stones” to help parents observe skill development and
Multi-Health Systems, Inc., 3770 Victoria Park Ave,Toronto, Ontario, track progress.
Canada M2H 3M6 Email: [email protected]; website: Test of Visual-Motor Skills Revised (TVMS-R)
www.mhs.com (1995)116
Ages Author
2 to 18 years Morrison F. Gardner
Administration Source
Individual or group; 5 to 15 minutes Children’s Hospital of San Francisco, Publication Department
Equipment OPR-110, PO Box 3805, San Francisco, CA 94119
Protocol booklets (test forms), No. 2 pencil Ages
Description 3 to 13 years
The BEERY VMI was developed for early screening and interven- Administration
tion of visual-motor deficits. It tests the ability to integrate visual Individual or group; three to six children
and motor abilities by presenting 30 geometric forms of increasing Equipment
difficulty to copy. A booklet is provided; below the design is a Protocol booklet, No. 2 pencil
blank space in which the child replicates the form. A shorter format Description
including the first 21 items is best suited for children aged 2 to The TVMS-R consists of a series of 23 forms to be copied by the
7 years. Items are judged pass or fail on criteria given in the manual, child. Each form is on a separate page of the booklet. The booklet
and scores are reported in standard scores and percentiles. Age contains some forms commonly used in visual-motor tests (e.g.,
equivalents are also available. If the child has significant difficulty lines and circles), but many forms are unique to this test. Care was
completing the test or scores below the average range, this may be taken to avoid forms that resemble language symbols. The revision
indicative of a visual-motor integration delay. To further assess of the TVMS has updated norms, standardization, and scoring
where the difficulties lie, two additional tests are available to assess criteria.
visual perception and motor coordination separately. Two different scoring methods are now available. Modifica-
1. Visual Perception: Visual perception is assessed by limiting tions in scoring the TVMS-R include a classification system
the motor response to pointing. The child matches geometric to characterize errors in one of eight categories. The eight clas-
forms to a stimulus. Administration takes approximately sifications are closure, angles, intersecting and overlapping
3 minutes. lines, size of design, rotation or reversals, line length, overpen-
2. Motor Coordination: Motor accuracy is assessed on a task of etration or underpenetration, and modification of design. Scoring
drawing within a double-lined path. Administration takes of each design is completed by following a definitive criterion,
approximately 5 minutes. with errors and strengths identified. The examiner can identify
Construction and Reliability specific areas of strength and weakness in visual-motor integra-
The Beery VMI is based on a significant amount of research on tion on the basis of the number of errors and accuracies
visual-motor integration, coordination, and development. The test recorded. Standard scores, scaled scores, percentile ranks, and
has been normed five times between 1964 and 2003, with a total of stanines are available for both weaknesses and strengths. An
more than 11,000 children. For this revision the Visual Perception alternative scoring method (ASM) was also designed to allow a
and Motor tests were standardized on the same sample of 2512 straight point system designation for each form. The forms are
children. This test has an extensive range of age-specific norms, scored on a 0- to 3-point scale. A score of 0 indicates that the
with 600 children for the age range of 2 to 6 years. Test-retest reli- child is unable to copy the form with any degree of motor ac-
ability is high for the three test components, ranging from 0.90 to curacy. Scores of 1 and 2 indicate various visual-motor errors
0.92. Various studies of interrater reliability, internal consistency, for which criteria are both written and illustrated. A score of
and concurrent and construct validity are reported in the manual. 3 demonstrates precision in execution. Age equivalents, stan-
Comparisons to other tests assessing visual-motor integration dard scores, scaled scores, percentile ranks, and stanines are
supported the validity the Beery VMI. provided.
Continued

Construction and Reliability Construction and Reliability
The TVMS-R was administered to 1484 children in the San Fran- The SASP was field tested on a sample of 322 children aged 4 to
cisco Bay area aged 3 years to 13 years, 11 months. The overall 11 years attending two schools in Houston, Texas. These schools
sample was 51.9% male and 48.1% female. Cronbach’s coefficient were selected for their demographic diversity. The small sample
alpha was used to determine the internal consistency of the test. size was deemed appropriate given the nature of the test. The inter-
These reliability coefficients ranged from 0.72 to 0.84 over the age nal consistency reliability was examined using Cronbach’s coeffi-
ranges, with a value of 0.90 for the sample as a whole. Test-retest cient analysis, yielding an average score of 0.76, indicating overall
reliability was not reported in the manual, but the author noted the reliability of the SASP across ages. Interrater reliability was 0.96.
need for research in that area. Content description and content identification were found to be
Comment valid for the limited purpose of this assessment, indicating that
The TVMS-R is a companion test to the Test of Visual-Perceptual examiners can use the SASP test with confidence to determine
Skills (TVPS), which is a motor-free test of form perception. Using entry points for the SASP curriculum.
the tests together can determine whether the child’s form reproduc- Comment
tion reflects incorrect visual perception or whether the problem is Spatial awareness skills are considered to develop both hierarchically
in motor execution. The TVMS-R places greater expectations on and cyclically, with the child developing a more complex understand-
motor precision than do other visual-motor tests. For example, a ing of global and part relationships over time. By the age of 10 the
line must touch an intersecting line without crossing over it. There- foundational spatial skills assessed by the SASP are thought to be
fore this test should be used only when motor control and construc- fully developed. Therefore a maximum score is expected for children
tive abilities are important. aged 10 or older. Although the SASP was normed for children aged
Spatial Awareness Skills Program (SASP) 4 through 10, it is possible to use it with individuals above this age.
(1999)117 If an older child does not perform appropriately on testing, and aca-
Author demic achievement is consistent with this substandard score, then the
Jerome Rosner remedial program can be used to encourage spatial awareness skills.
Source The author cautions that the remedial program will not eliminate
Pro-Ed, 8700 Shoal Creek Boulevard, Austin, TX 78757 academic deficits but will provide strategies to assist in learning.
Ages Evaluation Tool of Children’s Handwriting (ETCH)
4 to 10 years (1995)118
Administration Author
Individual; 5 minutes to administer Susan J. Amundson
Equipment Source
Protocol booklet, test booklet, No. 2 pencil, and transparent scor- O.T. Kids, PO Box 1118, Homer, AK 99603
ing guide Ages
Description First through sixth grades (6 to 11 years)
The SASP test and curriculum was developed and revised from Administration
two earlier programs by the same author called Perceptual Skills Individual
Curriculum (PSC) and Preparation for Learning (PREP). These Equipment
programs focused on teaching analytical abilities to improve Protocol booklet, task sheets and wall charts, stopwatch, No. 2
visual and auditory perceptual skills that are important for aca- pencil
demic learning. The SASP test is designed to assess spatial Description
analysis and organizational skills through copying progressively The ETCH is designed to evaluate manuscript and cursive writing
harder spatial figures. Items are judged pass or fail on criteria for components of legibility and speed. Specific components of the
given in the manual and through use of a scoring transparency. child’s handwriting, including letter formation, spacing, size, and
Raw scores are converted to age equivalents and used to deter- alignment, are included for assessment. The following tasks are
mine the child’s placement level within the SASP curriculum. presented in order:
The assessment and curriculum are designed to address the visual 1. Lowercase alphabet letters (from memory)
motor deficits exhibited by children with often hard-to-explain 2. Uppercase alphabet letters (from memory)
learning problems. 3. Numeral writing (from memory)
Spatial awareness refers to the ability to recognize what one 4. Near point copying (visual model)
can see, as well as understand space and time concepts that 5. Far point copying (visual model)
cannot be seen. It is a critical precursor to elementary school 6. Dictation (verbal)
achievement as a foundation to spatial concepts such as decod- 7. Sentence composition (independent)
ing words and performing mathematical calculations. The SASP A quick reference card is included with standardized directions
curriculum teaches spatial analysis skills with activities that and timing criteria. Written and illustrated scoring criteria have
show children how to break patterns into their structural ele- been designed to assist the evaluator in determining the legibility
ments, paying attention to features such as absolute and relative of letters and numbers. The primary focus of scoring is whether the
qualities, position, and magnitude. This type of organization written material is readable.
enhances the ability to chunk information into larger units Construction and Reliability
for more efficient processing of information. Spatial reasoning Based on the efforts of numerous occupational therapy practitio-
forms the logic of coding systems used for spelling, reading, ners, students, and professors, the ETCH itself has evolved through
writing, and math. scientific investigation. Pilots of the ETCH were designed by using

adaptations of written tasks from existing tools. Three editions of achieve each score and provide information on typical develop-
the ETCH have been sampled by practitioners working in school mental performance and error types. Possible errors include over-
systems, with feedback given on the examiner’s manual, ease of extended or underextended lines, broken lines, overlapping or
administration and scoring, item selection, scoring procedures, and reworked lines, parts missing, distortion of shape, or omitted dots
face validity of the instrument. When the ETCH was published, it or line crossings. Additional scoring looks at the speed of perfor-
lacked normative and psychometric information. Since that time, mance as well as reversed letters, case substitutions, and touching
test reliability, validity, and normative data of the ETCH are being letters.
compiled through various research studies. Eight studies of hand- Construction and Reliability
writing speed are included for reference in the manual. The most The THS was standardized with children from various parts of the
recent examination of test-retest reliability studies234 identified United States. Approximately equal numbers of boys and girls
adequate stability of total word (0.95), letter (0.88), and number were tested, with representation for both right and left handedness.
(0.84) scores. Individual letter, word, and number subtests were The manuscript version was administered to 494 children with
not as stable and should be used cautiously in interpretation of a median age of 6 years, 11 months; 406 were right handed and
problems. Interrater reliability ranged from 0.63 to 0.94 for indi- 61 were left handed. The cursive version was administered to
vidual manuscript items and 0.64 to 0.97 for cursive. Overall, the 345 children with a median age of 9 years, 8 months; 309 were
total word reliability is more stable than task scores, ranging from righted handed and 36 were left handed. Norms were derived for
0.90 to 0.98. each of the 10 subtests as well as the additional scores. Cronbach’s
Comment alpha was used to calculate the internal consistency of the test at
The ETCH assesses functional writing skills that are relevant to each age level. These reliability coefficients ranged from 0.51 to
academic performance. The varied tasks allow the examiner to 0.78 for the manuscript version and 0.29 to 0.87 for the cursive
identify areas of strength and weakness in written performance, version. Two items on each version had low reliabilities (writing
including legibility components, speed, and composition models eight numbers and copying 10 letters), probably a result of the
(visual, verbal, and memory). Information received from the small number of items in the subtest. Scores on the manuscript
ETCH is qualitative at this point because of the lack of normative version correlated positively with scores on the TVMS-R, indicat-
samples. The author suggests that the ETCH be used in conjunc- ing that the handwriting skills being tested involve a visual-motor
tion with observations of the child’s writing activity in natural en- component.
vironments, such as classroom and home, as a determination of Comment
difficulties in functional written performance. This test was developed primarily as a means for various profes-
Test of Handwriting Skills (THS)119 sionals to measure children’s handwriting skills. The THS can be
Author administered by OTs, teachers, psychologists, resource specialists,
Morrison F. Gardner educational diagnosticians, learning specialists, and optometrists.
Source While gathering pertinent information for test development, the
Psychological and Educational Publications, Inc., PO Box 520, author arranged two conferences with teachers from kindergarten
Hydesville, CA, 95547 to fifth grade and obtained information from various professionals
Ages throughout the United States. In designing this test he recognized
5 through 11 years; manuscript norms 5 to 8 years, cursive norms that three main methods of handwriting were being taught
9 to 11 years (D’Nealian, Palmer, and Zaner-Bloser) and that children, in gen-
Administration eral, have better accuracy for copied letters and words versus dicta-
Individual or group administration; 15 to 20 minutes tion that involves holding symbols in memory.
Equipment This test is functional and relevant to academic performance. The
THS manual, manuscript or cursive test booklet, No. 2 pencil, and varied tasks and additional scoring allow the clinician to identify
stopwatch areas of strength and weakness in written performance. This can
Description provide the foundation for recommendations within the classroom
The purpose of the THS is to assess a child’s neurosensory integra- and/or guide intervention.
tion ability in handwriting, with focus on uppercase and lowercase The Print Tool120
letter formation as well as numbers. It is designed to assess Authors
strengths and weaknesses in the motoric aspects of handwriting, Jan Olsen, OTR and Emily Knapton, OTR/L
including legibility and speed. Varied aspects of written perfor- Source
mance are assessed, including the following: Handwriting Without Tears, 8001 Mac Arthur Boulevard, Cabin
1. Spontaneously writing, from memory, uppercase and lower- John, MD 20818
case letters of the alphabet in sequence Email: [email protected]; website: www.hwtears.com
2. Writing, from dictation, uppercase and lowercase letters of Ages
the alphabet out of alphabetical sequence 6 years old and older
3. Writing, from dictation, numbers out of numerical order Administration
4. Copying selected letters of the alphabet Individual; administration for handwriting sample takes 15 minutes,
5. Copying selected words scoring about 30 minutes
6. Copying selected sentences Equipment
7. Writing selected words from dictation Student booklet and pencil, or a writing sample can be collected
Scoring criteria are well delineated in the manual. Sample from school; evaluation score sheet; transparent measuring tool for
visual representations are given to illustrate the quality needed to accurate scoring; comprehensive scoring overview; handwriting
Continued

remediation plan forms to develop goals; and strategies for reme- 7. Control: Neatness and proportion of letters and numbers
diation 8. Spacing: Amount of space between letters in words and
Description between words in sentences
The Print Tool is a formal printing assessment to use in evidence- Construction and Reliability
based remediation programs. The Print Tool is used to evaluate The Print Tool has been recently released. Standardization has not
handwriting skills, plan intervention, and measure progress in stu- been completed; however, suggested age-related expectations are
dents experiencing handwriting difficulty. A student’s ability to print provided in the manual. Data collection is in process for standard-
dictated letters and numbers is assessed. Evaluation scores for each ization in the future.
specific skill area assessed help identify the student’s strengths and Continuing education is recommended to understand the
needs. The Print Tool provides general remediation suggestions and complete process of observing, scoring, and compiling a
specific strategies to be carried out with the author’s remedial Hand- remediation plan with The Print Tool. A Level 1 Certification
writing Without Tears curriculum. This tool can be used in all school is offered by the Handwriting Without Tears organization.
and therapy settings and with all curricula. Eight handwriting com- Both the assessment and the award-winning Handwriting
ponents for capitals, lowercase letters, and numbers are considered: Without Tears curriculum were developed based on the
1. Memory: remembering and writing dictated letters and authors’ many years of successful practice in evaluating and
numbers remediating handwriting problems. The Handwriting Without
2. Orientation: Facing letters and numbers in the correct direction Tears program is a structured remedial program geared
3. Placement: Putting letters and numbers on the baseline toward different age and skill levels. It is multisensory,
4. Size: How big or small a child chooses to write developmentally based, and easy to implement. Support is
5. Start: Where each letter or number begins provided via the Handwriting Without Tears website.
6. Sequence: Order and stroke direction of the letter or number
parts
CHAPTER 15 Spina Bifida: A Congenital Spinal Cord Injury
KRISTIN J. KROSSCHELL, PT, MA, PCS, and MARI JO PESAVENTO, PT, PCS

Chiari malformation After reading this chapter the student or therapist will be able to:
crouch-control ankle-foot orthosis 1. Identify the various types of spina bifida.
diastematomyelia 2. Recognize the incidence and etiology of spina bifida.
hydrocephalus 3. Identify the clinical manifestations of myelomeningocele, including neurological,
lipomeningocele orthopedic, and urological sequelae.
myelodysplasia 4. Comprehend medical management in the newborn period and beyond.
myelomeningocele 5. Determine physical and occupational therapy evaluations, including manual muscle
reciprocating gait orthosis testing, range of motion, sensory testing, reflex testing, developmental and functional
sacral agenesis and mobility assessments, and perceptual and cognitive evaluations.
spina bifida cystica 6. List the major physical and occupational therapy goals and appropriate therapeutic
spina bifida occulta management for each of the following stages: (a) before surgical closure of sac, (b) after
standing A-frame surgery during hospitalization, (c) preambulatory, (d) toddler through preschool age,
tethered spinal cord (e) primary school age through adolescence, and (f) transition to adulthood.
7. Identify psychological adjustment to congenital spinal cord injury.
A spinal cord injury is a complex disability. When a spinal

cord lesion exists from birth, additional complexity is
added. This congenital condition predisposes many areas of
brain and spinal cord tissue. In the normal embryo, neural
tube closure begins in the cervical region and proceeds
cranially and caudally. Closure is generally complete by the
the central nervous system (CNS) to not develop or function twenty-sixth day.
adequately. In addition, all areas of development (physical,
cognitive, and psychosocial) that depend heavily on central Types of Spina Bifida
functioning will likely be impaired. The clinician therefore Spina bifida involves a defect in the neural tube closure and
must be aware of the significant impact this neurological the overlying posterior vertebral arches. The extent of the
defect has on motor function as well as a variety of related defect may result in one of two types of spina bifida:
human capacities. occulta or cystica. Spina bifida occulta is characterized by
A developmental framework, the Guide to Physical a failure of one or more of the vertebral arches to meet and
Therapist Practice,1 and the International Classification of fuse in the third month of development. The spinal cord and
Functioning, Disability and Health (ICF) have been used to meninges are unharmed and remain within the vertebral
aid in understanding the sequential problems of the child canal (Figure 15-2, A). The bony defect is covered with skin
with spina bifida. The developmental model, however, that may be marked by a dimple, pigmentation, or patch of
must always stay in line with the functional model for hair.2 The common site for this defect is the lumbosacral
adult trauma because the problems of the congenitally area, and it is usually associated with no disturbance of
involved child grow quickly into limitations in functional neurological or musculoskeletal functioning. Spina bifida
activities and participation in life of the injured adult. With cystica results when the neural and overlying vertebral
concentration on the present but with an eye to the future, arches fail to close appropriately. Cystic protrusion of the
appropriate management goals can be achieved. meninges or the spinal cord and meninges is present
through the defective vertebral arches.
OVERVIEW OF CONGENITAL SPINAL CORD The milder form of spina bifida cystica, called meningo-
INJURY cele, involves protrusion of the meninges and cerebrospinal
A congenital spinal cord lesion occurs in utero and is pres- fluid (CSF) only into the cystic sac (see Figure 15-2, B). The
ent at the time of birth. Understanding how this malforma- spinal cord remains within the vertebral canal, but it may
tion develops requires an appreciation of normal nervous exhibit abnormalities.3 Clinical signs vary (according to
system maturation. The nervous system develops from a spinal cord anomalies) or may not be apparent. This is a
portion of embryonic ectoderm called the neural plate. relatively uncommon form of spina bifida cystica.
During gestation, the neural plate develops folds that begin A more severe form of spina bifida cystica, called myelo-
to close, forming the neural tube (Figure 15-1). The neural cele or myelocystocele, is present when the central canal of
tube differentiates into the CNS, which is composed of the spinal cord is dilated, producing a large, skin-covered
419
cyst. The neural tube appears to close normally but is

distended from the cystic swelling. The CSF may cease-
lessly expand the neural canal. Prompt medical attention is
mandatory. This form of spina bifida is also rare.4
The more common and severe form of the defect is known
as myelomeningocele, in which both spinal cord and meninges
are contained in the cystic sac (see Figure 15-2, C). Within the
sac the spinal cord and associated neural tissue show extensive
abnormalities. In incomplete closure of the neural tube (dysra-
phism), abnormal growth of the cord and a tortuous pathway
of neural elements make normal transmission of nervous
impulses abnormal. The result is a variable sensory and motor
impairment at the level of the lesion and below.2 In an open
myelomeningocele, nerve roots and spinal cord may be
exposed, with dura and skin evident at the margin of the lesion.
Exposure of the open neural tube to the amniotic fluid environ-
ment leads to neuroepithelial degeneration, with massive loss
of neural tissue by the end of pregnancy.5
Although spina bifida cystica can occur at any level of
the spinal cord, myelomeningoceles are most common in
the thoracic and lumbosacral regions. Myelomeningocele
occurs in 94% of the cases of spina bifida cystica, and two
thirds of open lesions involve the thoracolumbar junction.2
Figure 15-1 n Neural tube forming. (From Stark GD: Spina The terms spina bifida, myelodysplasia, and myelomeningocele
bifida: problems and management, London, 1977, Blackwell are frequently used interchangeably.
Scientific.) Other forms of spinal dysraphism include diastematomy-
elia, lipomeningocele, and sacral agenesis. Diastematomyelia
is present in 30% to 40% of patients with myelomeningocele
and is secondary to partial or complete clefting of the spinal
cord.6 Lipomeningocele, another form of spina bifida cystica,
is usually caused by a vertebral defect associated with a
superficial fatty mass (lipoma or fatty tumor) that merges with
the lower level of the spinal cord. No associated hydrocepha-
lus is present, and neurological deficit is generally minimal;
however, problems with urinary control and motor control of
the lower extremities may be noted.7 Neurological tissue inva-
sion may be caused by a tethered spinal cord; therefore early
lipoma resection is indicated for cosmesis and to minimize
neurological sequelae. Lumbosacral or sacral agenesis may
occur and is caused by an absence of the caudal part of the
spine and sacrum. Children with this form of dysraphism may
have narrow, flattened buttocks, weak gluteal muscles, and a
shortened intergluteal cleft. The normal lumbar lordosis is
absent, although the lower lumbar spine may be prominent.
Calf muscles may be atrophic or absent. The pelvic ring is
completed with either direct opposition of the iliac bones or
with interposition of the lumbar spine replacing the absent
sacrum. These children may have scoliosis, motor and sen-
sory loss, and visceral abnormalities including anal atresia,
fused kidneys, and congenital heart malformations. Manage-
ment is started early and is symptomatic for each system.8
Failure of fusion of the cranial end of the neural tube
results in a condition known as anencephaly. In this condi-
tion some brain tissue may be evident, but forebrain devel-
opment is usually absent.9 Sustained life is not possible
with this neural tube defect; therefore this condition is not
discussed further.
Figure 15-2 n Types of spina bifida. A, Spina bifida occulta. Incidence, Etiology, and Economic Impact
B, Meningocele. C, Myelomeningocele. (From McLone DG: Statistics about the incidence of spina bifida vary consider-
An introduction to spina bifida, Chicago, 1980, Northwestern ably in different parts of the world. Spina bifida and anen-
University.) cephaly, the most common forms of neural tube defects,
CHAPTER 15 n Spina Bifida: A Congenital Spinal Cord Injury 421
affect about 300,000 newborns each year worldwide.10 acid–preventable spina bifida and anencephaly cases.15
In the United States the incidence is currently 2.48 per Genetic considerations, such as an Rh blood type, a specific
10,000, down from approximately 7.23 per 10,000 births gene type (HLA-B27), an X-linked gene, and variations in
from 1974 through 1979 (before the folic acid mandate).11,12 the many folate pathway genes have been implicated, but
Current worldwide folic acid fortification programs have not conclusively.28,29 Malformations are attributed to abnor-
resulted in decreased incidence of spina bifida,13,14 with mal interaction of several regulating and modifying genes in
annual decreases of 6600 folic acid–preventable spina bifida early fetal development.30 Disturbance of any of the sequen-
and anencephaly births reported since 2006.15 There was tial events of embryonic neurulation produces neural tube
a 31% decline in spina bifida prevalence rates in the defects (NTDs), with the phenotype (i.e., spina bifida, anen-
immediate postfortification period (October 1998 through cephaly) varying depending on the region of the neural tube
December 1999).13 There was a continued decline in spina that remains exposed.5 Environmental factors combined
bifida prevalence rates from 1999 to 2004 of 10%.16 Studies with genetic predisposition appear to trigger the develop-
have also demonstrated that decline varied by ethnicity and ment of spina bifida, although definitive evidence is not
race from prefortification to optional fortification to manda- available to support this claim.31
tory fortification in the United States.16,17 Initially after The incidence of spina bifida has declined since the
fortification, the largest decline in prevalence was noted advent of amniocentesis and the use of ultrasonography for
in Hispanic and non-Hispanic white races or ethnicities. prenatal screening. The presence of significant levels of
Despite this initial decline, postfortification prevalence rates alpha fetoprotein in the amniotic fluid has led to the detec-
remain highest in infants born to Hispanic mothers, and less tion of large numbers of affected fetuses.32 Currently, mater-
in infants born to non-Hispanic white and non-Hispanic nal serum alpha-fetoprotein levels have been effective
black mothers.16 In addition to periconceptual folate supple- in detecting approximately 80% of neural tube defects.33
mentation, it is thought that incidence has decreased subse- Prenatal screening can be most effective when a combina-
quent to food fortification in several countries, decreased tion of serum levels, amniocentesis or amniography, and
exposure to environmental teratogens, and increased and ultrasonography is used.34-36 Although this screening is not
more accurate prenatal screening for fetal anomalies.10 yet performed routinely, it is suggested for those at risk for
Spina bifida is thought to be more common in females the defect. Knowledge of the defect allows for preparation
than in males, although some studies suggest no real sex for cesarean birth and immediate postnatal care. This
difference.3 A study of the association of race and sex with includes mobilization of the interdisciplinary team that will
different neurological levels of myelomeningocele found continue to care for the child. For parents who decide to
the proportions of whites and females to be significantly carry an involved fetus to term, adjustment to their child’s
higher in patients with thoracic-level spina bifida.4 A sig- disability can begin before birth, which includes mobilizing
nificant relation also has been noted between social class their own support system. Education from an integrated
and spina bifida: the lower the social class, the higher the team regarding what will follow after delivery and neurosur-
incidence.18,19 gical closure is imperative to aid families in decision making
A multifactorial genetic inheritance has been proposed and to allow families to assess and understand the child’s
as the cause of spina bifida, coupled with environmental disability and future care options.
factors, of which nutrition, including folic acid intake, are Other advances in the field of prenatal medicine that
key. Cytoplasmic factors, polygenic or oligogenic inheri- affect spina bifida management and outcome include in
tance, chromosomal aberrations, and environmental influ- utero treatment of hydrocephalus and in utero surgical repair
ences (e.g., teratogens) have all been considered as possible to close the myelomeningocele. This challenging surgical
causes.5,15 Genetic factors seem to influence the occurrence procedure is practiced in only a few specialty centers and so
of spina bifida. The chances of having a second affected far has been shown to offer palliation of the defect at best.37
child are between 1% and 2%, whereas in the general popu- Treatment such as this, in conjunction with prenatal diagno-
lation the percentage drops to one fifth of 1%.20,21 Although sis, has been shown to have a positive impact on the inci-
these factors are related to the incidence of spina bifida, the dence and severity of complications associated with spina
cause of this defect remains in question. Environmental bifida.38-45 Limitations of current postnatal treatment strate-
conditions, such as hyperthermia in the first weeks of preg- gies and considerations of prenatal treatment options con-
nancy, or dietary factors, such as eating canned meats or tinue to be explored. Ethics, timing of repair, and surgical
potatoes or drinking tea, have been implicated but not procedures are all being investigated. In addition, continued
substantiated.22,23 In addition, historically, nutritional defi- assessment of outcomes from those who have undergone
ciencies, such as of folic acid and vitamin A, have been presurgical management requires continued exploration.
implicated as a cause of primary neural tube defects.24-27 The Management of Myelomeningocele Study (MOMS)
Approximately 50% to 70% of neural tube defects can be was initiated in 2003 as a large randomized, clinical trial
prevented if a woman of childbearing age consumes suffi- designed to compare the two approaches to the treatment of
cient folic acid daily before conception and throughout the infants with spina bifida (prenatal or fetal surgery versus
first trimester of pregnancy. As a result of research findings postnatal surgery) to determine if one approach was better
in support of folic acid implementation, the U.S. Public than the other. The primary end point of this trial was
Health Service has mandated folic acid fortification since the need for a shunt at one year, and secondary end points
1998 as a public health strategy. Prenatal vitamins, espe- included neurologic function, cognitive outcome, and
cially folic acid, are recommended to discourage the condi- maternal morbidity after prenatal repair. This study had
tion’s development. Current fortification programs are pre- 112 patients enrolled in 2007 with a projected enrollment
venting about 22,000 cases, or 9% of the estimated folic of 200.46-49 The trial was stopped for efficacy of prenatal
surgery after enrollment of just 183 infants. Results demon- level of the lesion with a chart delineating the segmental
strated that prenatal surgery significantly reduced the need innervation of the lower limb muscles. Orthopedic deformities
for shunting and improved mental and motor function at may result from the unopposed action of muscles above the
30 months. Reduced incidence of hindbrain herniation at level of the lesion. This unopposed pull commonly leads to hip
12 months and successful ambulation by 30 months were flexion, knee extension, and ankle dorsiflexion contractures.
also reported. While prenatal surgery was associated with When the spinal cord remains intact below the level of the
improved function and reduced need for shunting, maternal lesion, the effect is an area of flaccid paralysis immediately
and fetal risks, including preterm delivery and uterine below the lesion and possible hyperactive spinal reflexes
dehiscense at delivery were reported.49a distal to that area. This condition is quite similar to the neu-
In 1996 the lifetime cost to society per affected person rological state of the severed cord seen in traumatic injury.
with spina bifida was estimated to be $635,000.50,51 More This second type of neurological involvement again results in
recent estimates have not been reported; however, with an orthopedic deformities, depending on the level of the lesion,
economy in flux it is likely that this value underassesses the spasticity present, and the muscle groups involved.
costs to society today. In addition to medical management Orthopedic Deformities. The orthopedic problems
costs per child, there are additional costs that affect both the that occur with myelomeningocele may be the result of
family and society across the life span that are variable and (1) the imbalance between muscle groups; (2) the effects of
often related to differential market forces and social welfare stress, posture, and gravity; and (3) associated congenital
policies.50 malformations. Decreased sensation and neurological com-
In 2007, Ouyang52 reported that average medical expen- plications also may lead to orthopedic abnormalities.53
ditures during the first year of life for those with spina bifida Besides the obvious malformation of vertebrae at the
during 2002 and 2003 averaged $50,000 (using MarketScan site of the lesion, hemivertebrae and deformities of other
2003 database). The majority of expenditures during infancy vertebral bodies and their corresponding ribs also may be
were from inpatient admissions secondary to surgeries being present.53,54 Lumbar kyphosis may be present as a result of
concentrated during this time period for those with spina the original deformity. In addition, as a result of the bifid
bifida. After infancy, average medical care expenditures dur- vertebral bodies, the misaligned pull of the extensor muscles
ing 2003 ranged from $15,000 to $16,000 per year among surrounding the deformity, as well as the unopposed flexor
different age groups of persons with spina bifida. Incremen- muscles, contributes further to the lumbar kyphosis. As the
tal expenditures associated with medical care were not child grows, the weight of the trunk in the upright position
stable, but decreased with increasing age, from $14,000 per also may be a contributing factor.54 Scoliosis may be present
year for children to $10,000 per year for adults 45 to at birth because of vertebral abnormalities or may become
64 years of age.52 evident as the child grows older. The incidence of scoliosis
is lower in low lumbar or sacral level deformities.54,55
Clinical Manifestations Scoliosis may also be neurogenic, secondary to weakness or
The most obvious clinical manifestation of myelomeningo- asymmetrical spasticity of paraspinal muscles, tethered cord
cele is the loss of sensory and motor functions in the lower syndrome (TCS), or hydromyelia.55 Lordosis or lordoscolio-
limbs. The extent of loss, while primarily dependent on the sis is often found in the adolescent and is usually associated
degree of the spinal cord abnormality, is secondarily depen- with hip flexion deformities and a large spinal defect.3,54
dent on a number of factors. These include the amount of Many of these trunk and postural deformities exist at birth
traction or stretch resulting from the abnormally tethered but are exacerbated by the effects of gravity as the child
spinal cord, the trauma to exposed neural tissue during grows. They can compromise vital functions (cardiac and
delivery, and postnatal damage resulting from drying or respiratory) and therefore should be closely monitored by
infection of the neural plate.2 Specific clinical impairments the therapist and the family.
that commonly lead to functional limitations for the child As has been alluded to previously, the type and extent of
with spina bifida are addressed in this section. deformity in the lower extremities depend on the muscles
that are active or inactive. In total flaccid paralysis, in utero
Sensory Impairment deformities may be present at birth, resulting from passive
Children with spina bifida have impaired sensation below the positioning within the womb. Equinovarus (clubfoot) and
level of the lesion. The loss often does not match exactly the “rocker-bottom” deformity are two of the most common
level of the lesion and needs to be carefully assessed. Sen- foot abnormalities. Knee flexion and extension contractures
sory loss includes kinesthetic, proprioceptive, and somato- also may be present at birth. Other common deformities are
sensory information. Because of this, children will often hip flexion, adduction, and internal rotation, usually leading
have to rely heavily on vision and other sensory systems to to a subluxed or dislocated hip. Although many of these
substitute for this loss. problems may be present at birth, preventing positional
deformity (such as the frog-leg position), which may result
Musculoskeletal Impairment from improper positioning of flaccid extremities, is of the
Weakness and Paralysis. Determining neurological utmost importance. Orthopedic care varies throughout the
involvement is not as straightforward as assumed. At birth, course of the child’s life. Changes in clinical orthopedic
two main types of motor dysfunction in the lower extremi- management have evolved to establish evidence-based
ties have been identified. The first type involves a complete interventions.56
loss of function below the level of the lesion, resulting in a Osteoporosis. Because the paralyzed limbs of the child
flaccid paralysis, loss of sensation, and absent reflexes. The with spina bifida have increased amounts of unmineralized
extent of involvement can be determined by comparing the osteoid tissue, they are prone to fractures, particularly after
periods of immobilization.57,58 Early mobilization and Hydromyelia. Twenty percent to 80% of patients with
weight bearing can aid in decreasing osteoporosis.54,59 myelomeningocele have hydromyelia.6,70,71 Hydromyelia
Fortunately, these fractures heal quickly with appropriate signifies dilation of the center canal of the spinal cord as
medical management. hydrocephalus signifies dilation of the ventricles of the
brain. The area of hydromyelia may be focal, multiple, or
Neurological Impairment diffuse, extending throughout the spinal cord. The hydromy-
Hydrocephalus. Hydrocephalus develops in 80% to elia may be a consequence of untreated or inadequately
90% of children with myelomeningocele.21,60 Hydrocepha- treated hydrocephalus with resultant transmission of CSF
lus results from a blockage of the normal flow of CSF through the obex into the central canal, with distention a
between the ventricles and spinal canal. The most obvious result of increased hydrostatic pressure from above.6 The
effect of the buildup of CSF is abnormal increase in head increased collection of fluid may cause pressure necrosis of
size, which may be present at birth because of the great com- the spinal cord, leading to muscle weakness and scoliosis.
pliance of the cranial sutures in the fetus, or it may develop Common symptoms of hydromyelia include rapidly pro-
postnatally.61 Other signs of hydrocephalus include bulging gressive scoliosis, upper-extremity weakness, spasticity, and
fontanels and irritability. Internally, a concomitant dilation of ascending motor loss in the lower extremities.6,72 Aggressive
the lateral ventricles and thinning of the cerebral white mat- treatment of hydromyelia at the onset of clinical signs
ter are usually present. Without reduction of the buildup of of increasing scoliosis is mandatory and may lead to
CSF, increased brain damage and death may result. improvement in or stabilization of the curve in 80% of cases.
Chiari Malformation. Patients with myelomeningocele Surgical interventions may include revision of a CSF shunt,
have a 99% chance of having an associated Chiari II malfor- posterior cervical decompression, or a central canal to
mation.6 Cardinal features of the Chiari II malformation pleural cavity shunt with a flushing device.6,67
include myelomeningocele in the thoracolumbar spine, Tethered Cord. Tethered spinal cord is defined as a
venting of the intracranial CSF through the central canal, pathological fixation of the spinal cord in an abnormal caudal
hypoplasia of the posterior fossa, herniation of the hindbrain location (Figure 15-3). This fixation produces mechanical
into the cervical spinal canal, and compressive damage to stretch, distortion, and ischemia with daily activities, growth,
cranial nerves. This malformation is a congenital anomaly and development.73 Ischemic injury from traction of the conus
of the hindbrain that involves herniation of the medulla and directly correlates with degree of oxidative metabolism and
at times the pons, fourth ventricle, and inferior aspect of the degree of neurologic compromise. In addition to ischemic
cerebellum into the upper cervical canal. The herniation injury, traction of the conus by the filum may also mechani-
usually occurs between C1 and C4 but may extend down cally alter the neuronal membranes, resulting in altered elec-
to T1.6,62,63 In those with Chiari II malformations and spina trical activity.74-78 The presence of tethered cord syndrome
bifida there is a significant reduction in cerebellar volume, (TCS) should be suspected in any patient with abnormal neu-
and within the cerebellum the anterior lobe is enlarged and rulation (including patients with myelomeningocele, lipom-
the posterior lobe is reduced.64 Not all Chiari II malforma- eningocele, dermal sinus, diastematomyelia, myelocystocele,
tions are symptomatic. As a result of a symptomatic Chiari tight filum terminale, and lumbosacral agenesis). Presenting
malformation, problems with respiratory and bulbar func- symptoms may include decreased strength (often asymmetri-
tion may be evident in the child with spina bifida.2 Paralysis cal), development of lower-extremity spasticity, back pain at
of the vocal cords occurs in a small percentage of patients the site of sac closure, early development of or increasing
and is associated with respiratory stridor. Apneic episodes degree of scoliosis (especially in the low lumbar or sacral
also may be evident, although their direct cause remains in level),79,80 or change in urological function.68,81-83 Approximately
question. Children with spina bifida also may exhibit diffi- 10% to 30% of children will develop TCS after repair of
culty in swallowing and have an abnormal gag reflex.2 Prob- a myelomeningocele. Because essentially all children with
lems with aspiration, weakness and cry, and upper-extremity repaired myelomeningocele will have a tethered spinal cord,
weakness also may be present in children with a symptom- as demonstrated on magnetic resonance imaging (MRI), the
atic Chiari II malformation.65,66 Thus, depending on the
orthopedic deformities present and the neurological involve-
ment, severe respiratory involvement is possible in the af-
fected child. These symptoms may be caused by significant
compression of the hindbrain structures or dysplasia of pos- Cysts
terior fossa contents, which can also occur in patients with
Chiari II malformation.6,67 This complex hindbrain malfor- Tension
mation is a common cause of death in children with myelo- on cord
meningocele despite surgical intervention and aggressive Scar
medical management.68
Association Pathways. Diffusion tensor tractography
studies of association pathways in children with spina bifida
have revealed characteristics of abnormal development, Normal Tethered
impairment in myelination, and abnormalities in intrinsic
axonal characteristics and extraaxonal or extracellular space.
These changes in diffusion metrics observed in children with Figure 15-3 n Tethered cord in myelodysplasia. (From Staheli LT:
spina bifida are suggestive of abnormal white matter develop- Practice of pediatric orthopedics, Philadelphia, 2001, Lippincott
ment and persistent degeneration with increased age.69 Williams & Wilkins.)
diagnosis of TCS is made based on clinical criteria. The six The impairment of intellectual and perceptual abilities
common clinical presentations of TCS are increased weak- has been linked to damage to the white matter caused by
ness (55%), worsening gait (54%), scoliosis (51%), pain ventricular enlargement.2 This damage to association tracts,
(32%), orthopedic deformity (11%), and urological dysfunc- particularly in the frontal, occipital, and parietal areas, could
tion (6%).84 This clinical spectrum may be primarily associ- account for the often severe perceptual-cognitive deficits noted
ated with these dysraphic lesions or may be caused by spinal in the child with spina bifida.69,104 Lesser involvement of the
surgical procedures.73 The cord may be tethered by scar tissue temporal areas may account for the preservation of speech,
or by an inclusion epidermoid or lipoma at the repair site.6 whereas the semantics of speech, which depends on associa-
The primary goal of surgery is to detach the spinal cord where tion areas, is impaired. The “cocktail party speech” of children
it is adherent to the thecal sac, relieving the stretch on the with spina bifida can be deceptive because they generally
terminal portion of the cord. Surgery to untether the spinal use well-constructed sentences and precocious vocabulary.
cord (tethered cord release [TCR]) is performed to prevent A closer look, however, reveals a repetitive, inappropriate,
further loss of muscle function, decrease the spasticity, help and often meaningless use of language not associated
control the scoliosis,80,85 or relieve back pain.86,87 with higher intellectual functioning. Research on learning dif-
The effectiveness of a TCR may be demonstrated by an ficulties in children with spina bifida and hydrocephalus
increase in muscle function, relief of back pain, and stabili- suggests that many of these children experience difficulties.
zation or reversal of scoliosis.80,85,87 It has been reported that Tasks and skills affected include memory, reasoning,
scoliosis response to untethering and progression of scolio- math, handwriting, organization, problem solving, attention,
sis after untethering vary with location of tethering80,87 as sensory integration, auditory processing, visual perception,
well as Risser grade88 and Cobb angle.89 Those with Risser and sequencing.101-103
grade 3 to 5 and Cobb angle less than 40 degrees are less
likely to experience curve progression after untethering. Integumentary Impairment
Those with Risser grades 0 to 2 and Cobb angle greater than Latex allergy and sensitivity have been noted with increasing
40 degrees are at higher risk of recurrence.74,89 Spasticity, frequency in children with myelomeningocele, with frequent
however, is not always alleviated in all patients.90 Selective reports of intraoperative anaphylaxis.105-109 These children
posterior rhizotomy has been advocated for patients whose have also been reported to have a higher than expected
persistent or progressive spastic status after tethered prevalence of atopic disease.110 A 1991 Food and Drug
cord repair continues to interfere with their mobility and Administration Medical Bulletin estimated that 18% to 40% of
functional independence.68,70 patients with spina bifida demonstrate latex sensitivity,105,111
Bowel and Bladder Dysfunction. Because of the with others reporting an incidence of 20% to 67%.112,113
usual involvement of the sacral plexus, the child with spina Within latex is 2% to 3% of a residual-free protein material
bifida commonly deals with some form of bowel and blad- that is thought to be the antigenic agent.107 Frequent exposure
der dysfunction. Besides various forms of incontinence, to this material results in the development of the immuno-
incomplete emptying of the bladder remains a constant con- globulin E antibody. Children with spina bifida are more likely
cern because infection of the urinary tract and possible kid- to develop the immunoglobulin E sensitivity because of
ney damage may result.91 Regulation of bowel evacuation repeated parental or mucosal exposure to the latex antigen.114
must be established so that neither constipation nor Because of the risk of an anaphylactic reaction, exposure to
diarrhea occurs. Negative social aspects of incontinence can any latex-containing products such as rubber gloves, therapy
be minimized by instituting intervention that emphasizes balls, pacifiers, spandex, dental dams, elastic or rubber
patient and family education and a regular, consistently bands, balloons, adhesive bandages, or exercise bands should
timed, reflex-triggered bowel evacuation.92 be avoided. Latex-free gloves, therapy balls, treatment
Cognitive Impairment and Learning Issues. The last mats, and exercise bands are now widely available and should
major clinical manifestation resulting from the neurological be considered for standard use in all clinics treating children
involvement of myelomeningocele is impaired intellectual with spina bifida. Spina bifida, even in the absence of multiple
function. Although children with spina bifida without hydro- surgical interventions, may be an independent risk factor for
cephalus may have normal intellectual potential, children with latex sensitivity. Foods reported to be highly associated with
hydrocephalus, particularly those who have shunt infections, latex allergy include avocado, banana, chestnut, and kiwi.115
are likely to have below-average intelligence.93-95 These chil- Latex-free precautions from birth are more effective in pre-
dren often demonstrate learning disabilities and poor academic venting latex sensitization than are similar precautions insti-
achievement.96 Even those with a normal IQ show moderate to tuted later in life.115-117 Latex sensitization decreased from
severe visual-motor perceptual deficits.97 The inability to coor- 26.7% to 4.5% in children treated in a latex-free environment
dinate eye and hand movements affects learning and may from birth.117
interfere with activities of daily living (ADLs), such as button- The presence of paralysis and lack of sensation on the
ing a shirt or opening a lunchbox.98 Difficulties with spatial skin places the child with spina bifida at major risk for pres-
relations, body image, and development of hand dominance sure sores and decreased skin integrity. Various types of skin
may also be evident.2,98 Children with myelomeningocele breakdown have occurred in 85% to 95% of all children
demonstrate poorer hand function than age-matched peers. with spina bifida by the time they reach young adulthood.118
This decreased hand function appears to be caused by cerebel- Common areas at risk for pressure sores include the lower
lar and cervical cord abnormalities rather than hydrocephalus back, kyphotic or scoliotic prominences, heels, feet, toes,
or a cortical pathological condition (see Chapter 21).99 and perineum. A pressure sore may result from excessive
Prenatal studies have shown that the CNS as a whole is skin pressure that can cause reduced capillary flow, tissue
abnormally developed in fetuses with myelomeningocele.100-103 anoxia, and eventual skin necrosis. Excessive pressure may
manifest itself early as reactive hyperemia, a blister, and Neurosurgical Management

later as an open sore or overt necrosis. Chronic, untreated Since the early 1960s the presence of a myelomeningocele
sores may lead to osteomyelitis and eventual sepsis.110 has been treated as a life-threatening situation, and sac clo-
Pressure sores often result in loss of time from school and sure most often takes place within the first 24 to 48 hours of
work and can lead to financial hardship from medical treat- life.2,127 Recent advances in treatment have led to investiga-
ment and hospitalizations. These negative consequences can tional treatment in utero to repair the defect before birth.38
largely be prevented with attention to education and instruc- The aim of either surgery is to replace the nervous tissue
tion of the child and family. The goal of such education is to into the vertebral canal, cover the spinal defect, and achieve
foster an understanding of the causes of skin breakdown and a watertight sac closure.128 This early management has
the necessary meticulous attention to skin care that must be decreased the possibility of infection and further injury to
carried out on a regular basis. the exposed neural cord.24,128,129
Progressive hydrocephalus may be evident at birth in
Growth and Nutrition a small percentage of children born with myelomeningo-
Nutritional intake and weight gain and loss have been found to cele. A greater majority, however, have hydrocephalus 5 to
be problematic in children with myelomeningocele. Early on, 10 days after the back lesion has been closed.128,130-132 With
infants with spina bifida may have feeding issues as a result the advent of computed tomography (CT), early diagnosis of
of an impaired gag reflex, swallowing difficulties, and a high hydrocephalus can be made in the newborn without the need
incidence of aspiration.2,66 Altered oral-motor function has for clinical examination.
been attributed to the Chiari II malformation.119 These impair- Although clinical signs are not always definitive, hydro-
ments may lead to nutritional issues and delayed growth and cephalus may be suspected if (1) the fontanels become full,
weight gain. Speech, physical, and occupational therapists as a bulging, or tense; (2) the head circumference increases rap-
team are often needed to address these issues. idly; (3) a separation of the coronal and sagittal sutures is
Conversely, obesity can be a significant issue for children palpable; (4) the infant’s eyes appear to look downward
with spina bifida. This problem is complex and multifacto- only, with the cornea prominent over the iris (“sunsetting
rial.120 Mobility limitations and decreased energy expendi- sign”); and (5) the infant becomes irritable or lethargic and
ture result in lower physical activity levels. In addition, has a high-pitched cry, persistent vomiting, difficult feeding,
decreased lower limb mass diminishes the ability to burn or seizures (Table 15-1).21,61,133
calories, which leads to weight gain. Decreased caloric If the results of CT confirm hydrocephalus, a ventricular
intake as well as a lifelong engagement in rewarding and shunt is indicated. This procedure involves diverting the
physically challenging physical activities are both necessary excess CSF from the ventricles to some site for absorption.
to enhance weight control and control obesity. In general, two types of procedures—the ventriculoatrial
Children with myelomeningocele are short in stature. (VA) and ventriculoperitoneal (VP) shunt—are currently
Growth in these children may be influenced by growth- used, the latter being the most common (Figure 15-4). The
retarding factors as a result of a neurological deficit such as shunt apparatus is constructed from Silastic tubing and con-
tethered cord.121 Endocrine disorders and growth hormone sists of three parts: a proximal catheter, a distal catheter, and
deficiency have also been found to contribute to short stature
in this population.122 As a result of complex CNS anomalies
(midline defects, hydrocephalus, Arnold-Chiari malforma-
n SIGNS AND SYMPTOMS
TABLE 15-1
tion), these children are at risk for hypothalamopituitary
dysfunction leading to growth hormone deficiency.123,124 OF SHUNT MALFUNCTION
Treatment with recombinant human growth hormone has
Infants Bulging fontanel
proven successful in fostering growth acceleration in these
Swelling along the shunt tract
children.123,125,126
Prominent veins on scalp
Psychosocial Issues Downward eye deviation (“sunsetting”)
Vomiting or change in appetite
Considering all the clinical manifestations resulting from
Irritability or drowsiness
this congenital neurological defect, social and emotional
Seizures
difficulties will arise for these children and their families.
High-pitched cry
These will be considered as appropriate when discussing
Toddler Headache
the stages of recovery and rehabilitation from birth through
Vomiting or change in appetite
adolescence.
Lethargy or irritability
The preceding discussion concerning the clinical prob-
Swelling along the shunt tract
lems of the child with spina bifida is intended to inform, not
Seizures
overwhelm, the clinician. With a firm understanding of the
Onset of or increased strabismus
difficulties to be faced, evaluation and intervention can be
Older child All the above, plus:
more efficient and effective.
Deterioration in school performance
Medical Management Neck pain or pain over myelomeningocele site
Personality change
At or before birth, the myelomeningocele sac presents a
Decrease in sensory or motor functions
dynamic rather than a static disability. The residual neuro-
Incontinence that begins or worsens
logical damage will be contingent on the early medical
Onset of or increased spasticity
management that the fetus or newborn receives.
Figure 15-4 n A, Ventriculoatrial shunt. B, Ventriculoperitoneal shunt. (From Stark GD: Spina
bifida: problems and management, London, 1977, Blackwell Scientific.)
a one-way valve. As CSF is pumped from the ventricles and infections of the CNS. With the use of antibiotics,
toward its final destination, backflow is prevented by the shunting, and early sac closure, the survival rate has
valve system. In this manner intracranial pressure is con- increased from 20% to 85%.61,94,137
trolled, CSF is regulated, and hydrocephalus is prevented
from causing damage to brain structures. An alternate means Urological Management
of controlling hydrocephalus may be the use of endoscopic Initial newborn workup should include a urological assess-
third ventriculostomy (EVT). EVT is a procedure that, in ment. The urology team aims to preserve renal function and
selected patients with obstructive hydrocephalus, allows promote efficient bladder management. An early start to
egress of CSF from the ventricles to the subarachnoid space. therapy helps to preserve renal function for children with
This can decompress the ventricles and allow normal spina bifida.138 Initially, a renal and bladder ultrasound is
intracranial pressures and brain growth. This procedure is performed to assess those structures.100 Urodynamic testing
typically reserved for last resort.134 can be performed to determine any blockage in the lower
Unfortunately for children with spina bifida, their prob- urinary tract. Functioning of the bladder outlet and sphinc-
lems do not end after the back is surgically closed and a ters, as well as ureteric reflux, also can be evaluated. These
shunt is in place. Management strategies in the care of tests, plus clinical observations of voiding patterns, help
shunted hydrocephalus vary.135 Shunt complications occur the urologist classify the infant’s bladder function. If the
frequently and require an average of two revisions before bladder has neither sensory nor motor supply, a constant
age 10 years.60 The most common causes of complications flow of urine is present. In this case infection is rare because
are shunt obstruction and infection.2,136 Revising the blocked the bladder does not store urine and the sphincters are
end of the shunt can clear obstructions. Infections may be always open.139
handled by external ventricular drainage and courses of If no sensation but some involuntary muscle control of
antibiotic therapy followed by insertion of a new shunting the sphincter exists, the bladder will fill, but emptying will
system.2 The problem of separation of shunt components not occur properly. Overflow or stress incontinence results
has been largely overcome by the use of a one-piece in dribbling urine until the pressure is relieved. Because of
shunting system. The single-piece shunt decreases the com- constant residual urine, infection is a potential problem and
plications of shunting procedures. kidney damage may result.139 When some voluntary muscle
Prophylactic antibiotic therapy 6 to 12 hours before control but no sensation is present, the bladder will fill and
surgery and 1 to 2 days postoperatively is effective in con- empty automatically. The child can eventually be taught to
trolling infection for both sac repair and shunt insertion.71 empty the bladder at regular intervals to avoid unnecessary
This brief course of antibiotics has not led to resistant accidents.
organisms. The main cause of death in children with Regardless of the type of bladder functioning, urine
myelomeningocele remains increased intracranial pressure specimens are taken to check for infection, and blood
samples are taken to determine the kidney’s ability to filter deformities and need for surgical intervention. Important
the body’s fluids. On the basis of clinical findings, the urolo- management issues relevant to function that the physical
gist will suggest the appropriate intervention. therapist (PT) should be aware of may include hip disloca-
A program of clean intermittent catheterization (CIC) tion, knee valgus stress, scoliosis, foot deformities, frac-
done every 3 to 4 hours prevents infection and maintains the tures, osteoporosis, and postoperative management.
urological system.140-143 Parents are taught this method and Hip Dislocation. Hip dislocations may occur at any
can then begin to take on this aspect of their child’s care. At level of neurologic deficit.155 The goal of treatment for those
the age of 4 or 5 years, children with spina bifida can be with hip dislocation should be maximum function, not
taught CIC; thus they become independent in bladder care at radiographic realignment. The most important factor in
a young age. Achieving this form of independence adds to determining ability to walk is the level of neural involve-
the normal psychological development of these children. ment and not the status of the hip.153,156-159 A level pelvis and
Some children may require urinary diversion through the good hip range of motion (ROM) are more important than
abdominal wall (ileal conduit) or through the appendix hip relocation. In those with lower lumbar lesions and asym-
(Mitrofanoff principle appendicovesicotomy)144-146 or other, metry caused by contracture, treatment will be directed at
less common methods, such as intravesical transurethral blad- releasing the contracture and no attempts will be made to
der stimulation, to handle their urinary condition.140,147 Al- reduce the hip. Hip dislocations in those with sacral level
though CIC is not possible for all children with spina bifida, lesions should be considered as lever-arm dysfunction, and
it remains the method of choice for bladder management. surgical hip relocation is indicated.56,155,157,158 Immobiliza-
Bowel management and training programs should be tion after hip dislocation may lead to a frozen immobile
started early. Medications, enemas, and attention to fiber joint from an open reduction procedure, redislocation from
content in the diet are all of value in establishing a bowel a lack of significant dynamic forces available for joint stabil-
management program. The Malone antegrade continence ity around the hip joint, and an increased fracture risk.
enema (ACE) procedure is an important adjunct in the case Recently a questionnaire, the Spina Bifida Hips Question-
of adults and children with problems of fecal elimination in naire (SBHQ), to evaluate the ADLs that are important to
whom standard medical therapies have failed.148,149 children with spina bifida and dislocated hips and their
families has been developed and has demonstrated construct
Orthopedic Management validity as well as reliability.160
Orthopedic management of the newborn with a myelomenin- Knee Valgus Stress. Many children with spina bifida
gocele will generally concentrate on the feet and hips. Soft who walk have excessive trunk and pelvic movement, knee
tissue releases of the feet may take place during surgery for flexion contractures, and rotational malalignment that may
sac closure. Casting the feet (Figure 15-5) and performing lead to excessive knee valgus stress. The most common
early aggressive taping are also effective in the management deformities leading to this problem are rotational malalign-
of clubfoot deformities.150,151 Short-leg posterior splints ment of the femur and femoral anteversion in association
(ankle-foot orthoses [AFOs]) may be used to maintain range with excessive anterior tibial torsion. These deformities
and prevent foot deformities. should be addressed via surgical correction as excessive
The orthopedist also will evaluate the stability of the knee valgus stress can lead to knee pain and arthritis in adult
hips. In children with lower-level lesions, attempts to pre- life.56,159,161,162 In addition, the PT may need to reassess the
vent dislocation are made by using a hip abductor brace child’s gait pattern and use of assistive aids and bracing to
(Figure 15-6, A) or a total-body splint (Figure 15-6, B) for a minimize stress and maintain long-term joint viability for
few months after birth. With higher-level lesions, dislocated those with spina bifida over the life span.
hips are no longer treated because they do not appear to have Scoliosis. The prevalence of scoliosis in spina bifida is
an effect on later rehabilitation efforts.133,152-154 estimated to be as high as 50%. Increasing scoliosis can lead
Orthopedic management needs to be ongoing throughout to loss of trunk stability when curves are greater than
the child’s lifetime, with continued assessment of orthopedic 40 degrees and when associated pelvic obliquity becomes
25 degrees or more. Surgical intervention, often recom-
mended to prevent further progression, may improve or
further impair sitting balance, ambulation, and performance
of ADLs.163 Various authors have reported that although
surgery can improve curves by up to 50%, surgical morbid-
ity must be considered and complications may be as high as
40% to 50%. Functional benefits are largely unsubstantiated
owing to poorly constructed studies.164-166 Wai166 suggests
that spinal deformity may not affect overall physical func-
tion or self-perception. After surgical correction it may take
up to 18 months to appreciate functional improvement, and
walking may be difficult for those who were just exercise
ambulators before correction. Although surgical repair of
scoliosis does improve quality of life in patients with cere-
bral palsy and muscular dystrophy, this has not been demon-
strated in those with spina bifida.167 Interventions such as
Figure 15-5 n Plaster cast of the foot and ankle to reduce club- chair modifications to shift the trunk to improve balance in
foot deformities. the coronal plane and reduce pelvic obliquity and truncal
Figure 15-6 n A, Hip abductor brace. B, Total body splint.
asymmetry should be considered as a first option, before over stressed joints and to maximize reduction of the
surgical correction.163,167 patient’s pain and discomfort. Strengthening, particularly
Back Pain. Back pain needs to be efficaciously evalu- of the gluteal muscles, for those who are ambulatory may
ated in those with spina bifida who report back pain. Know- also be indicated. In addition, programs aimed at weight
ing when the patient experiences pain, what increases pain, reduction may be necessary to alleviate stress and pain to
what positions exacerbate pain, and what region of the body preserve long-term viability of tissues. In addition, for
is affected can help lead to appropriate referral, testing, and women the chest may cause tension on the upper back, and
management. Knowing if your patient has a shunt, spinal breast reduction has been advocated for some to relieve this
rods, and/or a Chiari malformation will also be important to tension.168-170
your assessment and management. Pain in the neck, shoul- Foot Deformity. The goal of treatment of the foot in
ders, and upper back with associated weakness and/or spina bifida should be a flexible and supple foot. An insen-
abnormal sensory findings should be evaluated by the treat- sate flail foot often becomes rigid over time, and foot man-
ing neurosurgeon to rule out shunt malfunction. Spinal rods agement can become complicated by pressure sores. Up to
that have broken or that are breaking through the skin may 95% of patients will use an orthosis, and a supple flail
also be a source of pain in this area. Pain not caused by rods, foot will be easier to manage over time. Surgeries that are
a shunt, Chiari issues, or a syrinx may have a mechanical extraarticular with avoidance of arthrodesis, as well as sim-
cause and could be a result of poor posture, tension, or ple tenotomies versus tendon releases and lengthenings,
weight gain. A patient who reports low back pain may have may best manage outcomes for bracing and ambulation.56
a symptomatic tethered cord if the patient is also reporting Equinovarus deformities may be managed with early and
changes in gait, increased tripping or falling, bladder changes, intensive taping in the newborn period, known as the French
and/or pain shooting down the legs. Manual muscle testing method,171,172 stretching and casting, and surgical interven-
(MMT) and urodynamic testing (refer to Chapter 29) are tion. The Ponsetti method, advocated by some, also has been
appropriate at this point and should be compared with base- reported to have positive outcomes; however, the significant
line testing findings. Mechanical low back pain may be a investment in time and commitment by the family for fre-
result of abnormal gait mechanics, asymmetrical strength, quent cast changes may affect the ability to carry out other
and use of older orthotics that no longer fit. Assessment of ADLs without disruption.155 In those with lipomas, foot
seating and support systems, including cushions, and gait deformity that may be acquired over time is best managed
mechanics and use of orthotics and ambulatory aids are in a similar manner. Maintaining a supple and plantigrade
mandatory to increase stability and redistribute balance foot with adequate muscle balance with use of soft tissue
correction through tendon lengthening, tendon transfer, and Manual Muscle Testing
plantar fascial release is recommended until 8 years of age. The first and most obvious request for evaluation may be to
After that time, deformities may become more rigid and determine the extent of motor paralysis. In the newborn,
may necessitate more bony procedures.173 testing may be done in the first 24 to 48 hours before the
Osteoporosis, Osteopenia, and Fracture. Osteoporo- back is surgically closed. In this case, care must be taken not
sis (thinning of the bone) and osteopenia (low bone mineral to injure the exposed neural tissue during testing. Prone and
density [BMD]) in the legs and spine have been described in side lying to either side are the most convenient and safe
children and teens with spina bifida. These conditions positions for evaluation during this time. Subsequent testing
increase the risk of fracture, increase the time for healing after is done soon after the back has been closed and as indicated
fracture, and may lead to back pain. A study by Valtonen and throughout childhood. The traditional form of MMT is not
colleagues in 2006 documented the occurrence of osteoporo- appropriate or possible for the infant or young child. Follow-
sis in adults with spina bifida. This condition often is not ing is a discussion of how muscle testing can and must be
recognized.174 Medical factors such as physical inactivity, adapted for this age group.
decreased vitamin D, diminished exposure to sunlight, uri- In evaluating the newborn, the importance of alertness is
nary diversion, renal insufficiency, hypercalciuria, medication paramount. A sleeping or drowsy infant will not respond
for epilepsy, and oral cortisone treatment for more than appropriately during the evaluation. The infant must be in
3 months increase the risk of osteoporosis.59,175,176 It can be the alert or crying state to elicit the appropriate movement
assumed that patients with meningomyelocele are at potential responses. Testing hungry or crying infants provides an
risk to develop osteoporosis at a younger age because of advantage because they are likely to demonstrate more
impaired walking ability and subsequent low physical loading spontaneous movements in these behavioral states.
of the lower limbs. Older age and higher levels have been The cumulative effect of a variety of sensory stimuli may
associated with increased numbers of fractures in spina be more effective in bringing the infant to alertness than
bifida.174 The optimal strategies for prevention and treatment using one stimulus in isolation. For example, the infant may
of osteoporosis in this population have not been established. be picked up and rocked vertically to allow maximum
Further research is required to see if the methods used to pre- stimulation to the vestibular system and to help bring the
vent and treat osteoporosis in individuals without spina bifida child to an alert state. In addition, the therapist may talk to
also work for teens and adults who have spina bifida. Consid- the child to help him or her fixate visually on the therapist’s
ering the effects of prolonged immobilization on indepen- face. Tactile stimuli above the level of the lesion further add
dence in daily activities and quality of life, there should be no to the child’s level of arousal, thus contributing to more
disagreement that all efforts are necessary to prevent these conclusive test results. In this way the CNS receives an
fractures. Furthermore, osteoporotic fracture may lead to a accumulation of information from a variety of sensory sys-
vicious cycle of immobilization, decreased bone density, and tems rather than relying on transmission from one system
repeated fractures.174 Annual incidence of fracture is 0.029% that may be weak or inefficient.
in adolescents and 0.018% in adults.177 Studies have shown As the child is aroused, spontaneous movements can be
promising results of regular functional electric stimulation– observed and muscle groups palpated. Additional methods
assisted training, but this is often nearly impossible to carry to stimulate movement may be necessary. For example, tick-
out in daily life.178 The effects of standing programs on bone ling the infant generally produces a variety of spontaneous
density are unclear.179,180 The prevention of fractures should movements in the upper and lower extremities. Passive
be among the major goals in the rehabilitation of people with positioning of children in adverse positions may stimulate
meningomyelocele. The assessment of BMD is worthwhile in them to move. For example, if the legs are held in marked
patients with risk factors for osteoporosis, because low BMD hip and knee flexion, the infant may attempt to use extensor
is a known risk factor for fractures.175 musculature to move out of that position. If the legs are held
Postoperative Management. Care should be taken to in adduction, the child may abduct to get free. Holding a
avoid postoperative complications such as skin breakdown limb in an antigravity position may elicit an automatic
and postimmobilization fractures in the postoperative “holding” response from a muscle group when spontaneous
period. To decrease the risk of nonunion and allow for early movements cannot be obtained in any other way.
mobilization and weight bearing, one should consider rigid In grading muscle strength, differentiation between spon-
internal fixation versus Kirschner wire fixation. After sur- taneous, voluntary movement and reflexive movement is
gery, immobilization in a custom-molded body splint rather important. After severing of a spinal cord, distal segments of
than a hip spica cast is preferred. Postoperative physical the cord may respond to stimuli in a reflexive manner. This
therapy should begin as soon as wounds are stable and heal- results from the preservation of the spinal reflex arc and is
ing is occurring. Therapy should focus on ROM (active and known as distal sparing. If distal sparing of the spinal cord
passive) and early weight bearing. Crawling should be is present, the muscles may respond to stimulation or
strictly forbidden for a minimum of 3 to 4 weeks postim- muscular stretch with reflexive, stereotypical movement
mobilization to reduce the risk of fracture.159 patterns. The quality of this reflexive movement will be dif-
ferent from that of spontaneous movement and must be
EVALUATIONS distinguished when testing for level of voluntary muscle
In attempting to evaluate the child with spina bifida, a number functioning.
of evaluations can be chosen, each designed to test specific yet Muscle strength is generally graded for groups of mus-
perhaps unrelated components of function. The following sec- cles and can be graded by using either a numerical (1 to 5)
tion discusses those test procedures or specific standardized or an alphabetical designation (Figure 15-7) or simply by
tests that would best define the complexity of the problem. noting presence or absence of muscular contraction by a
Figure 15-7 n Muscle examination form using alphabetical designation. (Courtesy Josefina
Briceno, PT, Children’s Memorial Hospital, Chicago.)
plus or a minus on the muscle test form. The last method

may be sufficient initially, but as the child matures a more
definitive muscle grade should be determined.
By use of a MMT form that lists the spinal segmental
level for each muscle group, an approximate level of lesion
can be determined from the test results (see Figure 15-7). >T10 Loss of trunk
Because the spinal cord is often damaged asymmetrically, control
L2 Hip abduction
MMT does not always accurately reflect the level of the and adduction lost
lesion. If reflex activity is also noted on the form, the pres-
ence of distal sparing of the spinal cord can be determined.
Muscle testing of the newborn gives the clinician an appre-
ciation of muscle function and possible potential for later
T12 Loss of hip
ambulation as well as an awareness of possible deforming flexion
forces. For example, if hip extensors or abductors are not
functioning, then the action of hip flexors and adductors
must be countered to prevent future deformities.
Muscle testing of the toddler or young child may require L4 Loss of knee
some of the techniques previously described. In addition, flexion and
ankle dorsiflexion
developmental positions can be used to assess muscle L3 Loss of knee
strength in an uncooperative youngster. For example, extension
strength of hip extensors and abductors can be assessed as a
child attempts to creep up steps or onto a low mat table.
With addition of resistance to movements, fairly accurate
muscle grades can be determined. To elicit hip flexor action
in sitting, if an interesting toy or object is placed on the
child’s ankle or between the toes, the child will often lift the S1 Loss of ankle L5 Loss of hip abduction
leg spontaneously to reach for it. Ingenuity and creativity plantarflexion and extension
are prerequisites for muscle testing in the young child. Reli- Figure 15-8 n Weakness related to level of spinal defect. (From
ability of MMT in children with spina bifida younger than Staheli LT: Practice of pediatric orthopedics, Philadelphia, 2001,
5 years is difficult but has been demonstrated in a clinic set- Lippincott Williams & Wilkins.)
ting where all therapists were trained in specific MMT tech-
nique to ascertain consistency in testing.181 By the age of
4 or 5 years, muscle grades can generally be determined by cognitive and language abilities to respond appropriately
traditional testing techniques, although the reliability of the to testing.
test results will increase with the age of the child.182 In most In the newborn, sensory testing can best be done if the child
clinics MMT is used to assess strength and changes in is in a quiet state. Beginning at the lowest level of sacral
strength over time. Reliability of MMT may be called into innervation, the skin is stroked with a pin or other sharp object
question when trying to assess meaningful detectable until a reaction to pain is noted. Although none of these meth-
changes in power against gravity. If that is the case, one can ods is fail-safe, they may be helpful in adapting a muscle test
use hand-held dynamometry (HHD) to test muscles with a to a newborn or young infant. Repeated evaluation may be
grade of 3 or greater. Excellent intertester reliability of HHD necessary to get an accurate picture of muscle function.
for children with spina bifida has been demonstrated.183 Because of dermatome innervation the pin is usually
Muscle testing is indicated before and after any surgical drawn from the anal area across the buttocks, down the pos-
procedure and at periodic intervals of 6 months to 1 year to terior thigh and leg, then to the anterior surface of the leg
detect any change in muscle function. Timely detection of and thigh, and finally across the abdominal muscles. Reac-
any loss in strength is critical, as the child may encounter tions to be noted are a facial grimace or cry, which indicates
increased weakness resulting from tethering of the spinal that the painful sensation has reached a cortical level. Care
cord or shunt malfunction as he or she grows. The level of must be taken to see that each sensory dermatome has been
innervation should not decrease throughout the life of the evaluated. Results can be recorded by shading in the derma-
child with spina bifida. In the growing child or adolescent, tomes where sensation is present (Figure 15-9).
an increasing weakness resulting from shunt malfunction, The therapist may be called on to evaluate the newborn
tethering of the spinal cord, or hydromyelia frequently can before surgical closure of the spinal meningocele. Although
be substantiated by a muscle test of the lower extremities. sensory and motor levels can be determined as previously
The MMT is also valuable in determining the motor level described, the infant’s general condition should be considered
so that potential future functional level can be determined in interpreting test findings. Any medication taken by the
(Figure 15-8). mother during labor and delivery may influence the neonate’s
performance and thus should be noted. In addition, the physi-
Sensory Testing ological disorganization normally seen in all infants during the
Sensory testing of the infant and young child is simplified first few days after birth may also affect testing.184 At best, this
to determine the level of sensation as accurately as possi- presurgical evaluation establishes a tentative baseline, but sig-
ble with a minimal amount of testing. Full sensory tests nificant changes in the infant’s neurological status in the first
are not possible until the child has acquired sufficient few weeks of life should not be surprising to the clinician.
Range-of-Motion Evaluation
A complete ROM evaluation of the lower extremities is
indicated for the newborn with spina bifida. The therapist
must be aware of normal physiological flexion that is great-
est at the hip and knees. In the normal newborn these appar-
ent “contractures” of up to 35 degrees are eliminated as the
child gains more control of extensor musculature and kicks
more frequently into extension.
In the child with spina bifida, contractures may be evident
at multiple joints at birth because of unopposed musculature
(Figure 15-11). Hip adduction should not be tested beyond the
neutral position to avoid dislocation of hips, which are often
unstable. Range should be done slowly and without excessive
Figure 15-9 n Lower-limb dermatomes. (From Brocklehurst G:
force to avoid fractures so often experienced in paralytic lower
Spina bifida for the clinician. Clin Dev Med 57:53, 1976.)
extremities. ROM should be checked with the same frequency
as MMT. Active ROM of the upper extremities can be assessed
by observation and handling the infant. A formal ROM evalu-
ation for the upper extremities is not usually indicated. A
In the young child from 2 to 7 years of age, light touch baseline ROM and tone assessment of the upper extremities
sensation and position sense can be tested in addition to pain should be completed.
sensation. Again, to elicit an appropriate response and
reliable test results, the ingenuity of the therapist will be Reflex Testing
required. Using games such as “Tell me when the puppet The purpose of reflex testing is twofold: to check for the
touches you” may be more effective for the young child than presence of normal reflex activity and to check for the inte-
traditional testing methods. Sensory dermatome mapping gration of primitive reflexes and the establishment of more
using the chart in Figure 15-9, or a similar form such as the mature reactions. In the newborn, for example, strong root-
WeeSTeP once the child with spina bifida gets older, can aid ing and sucking reflexes are expected. In the child with spina
in establishing sensory level as well as insensate areas that bifida, because of possible involvement of the CNS as previ-
may be at high risk of injury.185 ously described, these reflexes may be depressed or absent.
From age 7 years through adolescence, additional sensory Because these reflexes play an integral part in obtaining
tests of temperature and two-point discrimination may be nutrients for the infant, their value is obvious. On the other
added. Traditional methods are usually sufficient to ensure hand, primitive reflexes that persist past their expected span
reliable testing, but a more behavioral approach may be indi- also may indicate abnormality. For example, if the asym-
cated depending on the individual’s cognitive functioning. metrical tonic neck reflex persists past 4 months, it will limit
After testing, a survey of the sensory dermatome chart the infant’s ability to bring the hands to midline for visual
should indicate whether sensation is normal, absent, or and tactile exploration.
impaired. MMT and sensory testing (dermatomes) can assist As the primitive reflexes (initially needed for survival
in determining spinal level of function (Figure 15-10). and to experience movement) become integrated, they are
Root Urinogenital Bladder
Sphincter
L1 Hip Knee Ejaculation L1 tone
L2 Flexors Adductors Extensors L2

2, 3 and 2, 3, 4
internal
L3 rotators L3
Knee Invertors
Abductors
L4 2, 3, 4 L4
jerk 4 4, 5
4, 5
Extensors Flexors Dorsiflexors Extensors

L5 L5
5, 1 5, 1
External
Ankle Evertors Flexors 1, 2 Retention
S1 jerk rotators 5, 1 1, 2 S1
1
S2 Plantarflexors Intrinsics Erection S2

2, 3 Dribbling
Bladder incontinence
S3 (para- S3
sympathetic)
Figure 15-10 n Segmental nerve supply of the lower extremities. (From Stokes M: Physical
management in neurological rehabilitation, London, 2004, Elsevier.)
supporting surface? From the hands-knees position, can

reciprocal crawling be initiated without the lower extremi-
ties being held in wide abduction? Can the child pull to
stand easily by using trunk rotation? Assessing the quality of
the child’s abilities will assist the clinician in determining
where therapeutic measures should begin and what the goals
of such intervention will be. Standardized assessments may
provide the families with guidelines and a record of motor
skills over time (see Chapter 3).
There are no standardized functional or motor assess-
ments specific to those with spina bifida. Some assessments
look at development relative to standardized norms and may
guide the family and therapist in determining treatment
goals and challenges. This information may be invaluable in
determining bracing needs and other equipment needs
as well as timing of various interventions. If a standardized
Figure 15-11 n Infant with myelomeningocele with contrac- assessment were desired to use with the infant with spina
tures. (From Molnar GE, Alexander MA: Pediatric rehabilitation, bifida, the Alberta Infant Motor Scale (AIMS) might be
Philadelphia, 1999, Hanley & Belfus.) appropriate. The AIMS186 is designed to measure motor
development from birth to 18 months of age. It is a 58-item
observational test of infants in supine, prone, sitting, and
standing positions. Each item includes detailed descriptions
replaced by more mature and functional reactions. The of the weight-bearing surface, the infant’s posture, and anti-
righting and equilibrium reactions help the child attain the gravity movements expected of the infant in that position.
erect position and counteract changes in the center of grav- The AIMS requires minimal handling of the infant and can
ity. Because these reactions depend on an intact CNS as well be completed in 20 to 30 minutes. The test was normed on a
as a certain level of postural control, they may be delayed, cross-sectional sample of 2200 infants in Alberta, Canada.
incomplete, or absent in the child with spina bifida. For Interrater and test-retest reliability are high (0.95 to 0.99),
example, a child with a low thoracic spinal cord lesion may as is concurrent validity with the Peabody Developmental
show an incomplete equilibrium reaction in sitting. This Motor Scales (PDMS) (0.99) and the Bayley Scales of
may be caused by the lack of a stable postural base or by Infant Development (0.97). Predictive validity of the AIMS
lack of initiation of the reaction centrally. Both the neuro- appears to be fair.187 For the child with spina bifida the
logical and muscular components of these reactions must be AIMS could be used to assess current motor development
considered. Reflex testing for the child with spina bifida and track progress in motor development over time.
may not be as intensive as that for a child with cerebral The Milani-Comparetti Motor Development Screening
palsy. It may, however, provide a check on the progress of Test for Infants and Young Children may also be useful in
normal development and as such reflect the integrity of the assessing the functional level of the child with spina bifida.
CNS (see Chapters 3 and 16). This screening examination is designed to evaluate motor
development from birth to 2 years of age (Figure 15-12).188
Developmental and Functional Evaluations It requires no special equipment and can be administered in
Besides being aware of a child’s sensory and motor levels, 4 to 8 minutes. The test evaluates both spontaneous behavior
assessing the functional level is also important. Two impor- and evoked responses. Spontaneous behavior includes pos-
tant questions need to be asked: “Does the child show tural control of the head and body in various positions as well
normal components of posture and movement synergies?” as a sequence of active movement patterns. Primitive re-
and “What is the child’s level of function and mobility?” flexes, righting, and equilibrium reactions constitute the
Several developmental and functional evaluations can be evoked responses. The Milani-Comparetti test was normed
used with the child with spina bifida. The following are on a sample of 312 children from Omaha, Nebraska. Inter-
some suggestions for evaluation approaches or specifically rater reliability percent of agreement was 89% to 95%.
designed tests to assist in assessment of this area. Test-retest reliability percent agreement was 82% to 100%.
Initially, a developmental sequence may be used to assess Predictive validity of the test has not been well established.188
how a child is functioning. In each position used, both pos- The Milani-Comparetti test should assist the clinician in
ture and movement are evaluated. The goals in using this evaluating each child’s underlying postural mechanisms and
type of assessment are to determine what a child can and his or her ability to attain the erect position. The test manual
cannot do, the quality of the action, and what is limiting the provides information on special examination procedures and
child. The progression begins in the supine position, rolling scoring.
to prone, prone on elbows, prone on hands, up to sitting, on The Ages and Stages Questionnaire (ASQ) is a screening
hands and knees, kneeling, half-kneeling, standing, and assessment that assesses developmental and social-emotional
walking. Both the ability to attain and the ability to maintain delays during crucial early ages of life. This test is available
the positions should be assessed. in English and Spanish and can be completed in 10 to
The way in which a task is accomplished is as important 15 minutes.189 It was developed and validated on 15,138 children
to evaluate as the accomplishment itself. For example, in in all 50 states and several U.S. territories. The test-retest reli-
rolling, is head righting sufficient to keep the head off the ability (0.92), interrater reliability (0.93), validity (0.82 to 0.88),
Figure 15-12 n Milani-Comparetti Motor Development Screening Test revised score form.
sensitivity (0.86), and specificity (0.85) have been well docu- takes 45 to 60 minutes to complete or 20 to 30 minutes per
mented.189 This test provides parents and providers with a subtest. The two scales allow a comparison of the child’s
checklist to easily assess change over time. motor performance with a normative sample of children at
The PDMS-2 is another standardized assessment that various age levels. A stratified sample of 2003 children from
may prove helpful in evaluating a child with congenital spi- 46 states in the United States was used to develop PDMS-2
nal cord injury.190 The PDMS-2 was developed using item test norms. Test-retest and interrater reliability are high. Con-
response theory (IRT) and consists of six gross and fine tent, construct, and concurrent validity have been well estab-
motor subtests from birth through 6 years of age. The test lished. Although the child with activity limitations would not
be expected to succeed on many of the gross motor items at method for tracking change over time. The PEDI has had a
the later age levels, the scale still serves as a reminder rich tradition in helping to document functional develop-
of expected gross motor performance at each age. The fine ment, and new methods proposed for the next generation of
motor scale offers a chance to assess fine motor performance the PEDI include using item banks and computer adaptive
of children with congenital spinal cord injury. This area has testing. The computer adaptive testing feature and the
been frequently overlooked in children with myelomeningo- revised and expanded content of the new PEDI will enable
cele. Fine motor development, however, may be affected therapists to more efficiently assess children’s functioning to
because of congenital abnormalities in brain development a broader age group of children.194,195
associated with myelomeningocele or related to tethering of Another assessment of motor performance that may be
the spinal cord that can result in fine motor paresis. In addi- commonly used with the school-age child with spina bifida
tion, the PDMS-2 offers guidelines for administering the test is the School Function Assessment (SFA). The SFA is stan-
to children with various activity limitations.190 dardized and was conceptually developed to reflect the
The Bruininks-Oseretsky Test of Motor Proficiency, sec- functional abilities and needs of a student in elementary
ond edition (BOT-2) can be used to evaluate the higher school. The three areas assessed include the student’s
functioning ambulatory child with lower lumbar or sacral participation in school activities, task supports required by
level spina bifida.191 Fine manual control, manual coordina- the student for participation, and the student’s activity
tion, body coordination, and strength and agility subtests performance.196,197 It was designed to facilitate collaborative
can be used to assist in evaluating areas of fine motor con- program planning for students with a variety of disabling
trol, balance, and coordination difficulties. This test has conditions. The instrument is a judgment-based (question-
been standardized on a sample of 1520 subjects from age naire) assessment that is completed by one or more school
4 through 21 years.191 professionals who know the student well and have observed
The Movement Assessment Battery for Children, second his or her typical performance on the school-related tasks
edition (Movement ABC-2), can be used to identify children and activities being assessed. Items have been written in
who are significantly behind their peers in motor develop- measurable, behavioral terms that can be used directly in the
ment, assist in planning an intervention program in either a student’s Individualized Educational Plan (IEP).196
school or a clinical setting, and measure change as a result of
intervention or can serve as a measurement instrument in Gait Analysis
research involving motor development. This tool may be use- Formal computerized gait analysis was initially used to
ful to assess children with lower lumbar and sacral level evaluate children with cerebral palsy. Increasingly it is being
myelomeningocele, as well as children with lipomeningocele. used to evaluate children with meningomyelocele once they
The Movement ABC identifies and evaluates the movement have established a gait pattern to determine factors leading
problems that can determine a child’s participation and social to changes in gait, including changes in alignment, muscle
adjustment at home or school. The Movement ABC Check- length, muscle torque, and symmetry. The gait analysis may
list provides classroom assessment of movement difficulties, aid in decision making regarding orthotic and orthopedic
screening for “at risk” children (ages 5 to 12 years), and interventions. Whether it is useful to do formal gait analyses
systematic monitoring of treatment programs. It provides in all children with spina bifida remains to be determined.198
a comprehensive assessment for those identified as “at risk” Gait analyses have also been useful in establishing a data-
(3 to 16 years, 11 months), yielding both normative and base of trends in kinetics and kinematics for various levels
qualitative measures of movement competence, manual dex- of spina bifida.
terity, ball skills, and static and dynamic balance.192
Finally, the Pediatric Evaluation of Disability Inventory Perceptual and Cognitive Evaluations
(PEDI) is a comprehensive assessment of function in chil- When evaluating a child with spina bifida, some assessment
dren aged 6 months to 7 years.193 The PEDI measures both of perceptual and cognitive status is important to include.
capability and performance of functional activities in three The appropriate assessment depends largely on the age of
areas: self-care, mobility, and social function. Capability is the child. The assessment may be performed by the physical,
a measure of the functional skills for which the child has occupational, or speech therapist, depending on the setting.
demonstrated mastery. Functional performance is measured For the newborn from 3 to 30 days old, the Brazelton
by the level of caregiver assistance needed to accomplish a Neonatal Behavioral Assessment scale may be adapted to
task. A modifications scale provides a measure of environ- assess the infant’s organization in terms of physiological
mental modifications and equipment needed in daily func- response to stress, state control, motoric control, and social
tioning. The PEDI has been standardized on a normative interaction.184 Ideally the infant should be medically stable
sample of 412 children from New England. Some data from and free from CNS-depressant drugs before evaluation.
clinical samples (N 5 102) are also available. Interrater Generally this evaluation will occur after the back lesion has
reliability of the PEDI is high as demonstrated by high intra- been closed and a shunt has been positioned to relieve the
class correlation coefficients (ICCs 5 0.96 to 0.99). Concur- hydrocephalic condition.
rent validity of the PEDI with the WeeFIM (child’s version Although test results may not have prognostic value
of the Functional Independence Measure) was also high because of the plasticity of the nervous system at this young
(r 5 .80 to 0.97).193 The PEDI can be administered in age, they supply the clinician with information concerning
approximately 45 minutes by clinicians or educators famil- the current status of the child. This information can be con-
iar with the child or by structured interview of the parent. veyed to the infant’s caregivers—both medical personnel
The PEDI should provide a descriptive measure of the func- and parents—so that strengths can be appreciated and
tional level of the child with myelomeningocele as well as a weaknesses anticipated and handled appropriately. Helping
parents identify that their infant has his or her own unique MVPT-3 can be used with individuals from 4 to 70 years of
characteristics and assisting them in dealing with these char- age, and the TVPS-3 can be used with children from 4 to
acteristics does a great deal to strengthen already precarious 18 years of age. The TVPS-3 has two levels; the lower level
parent-infant bonding. tests children from ages 4 to 12 years, and the upper level
Repeated administration of the Brazelton Neonatal tests children from ages 12 years to 17 years, 11 months.
Behavioral Assessment scale in the first month of life may Both tests are easy and quick to administer (less than
help monitor the infant’s progress in organization and reflect 15 minutes) and, based on the examiner’s experience and
the curve of recovery. Although the manual for this behavioral training, interpretations can be made with prescription for
assessment is complete, proper administration, scoring, and remediation. The MVPT-3 was standardized on a nationally
interpretation require direct training with someone already representative sample. The test-retest reliability of the
proficient in using the scale.199 Excellent training videos for MVPT-3 was 0.81.206 Performance on the motor-free test
the Brazelton Neonatal Behavioral Assessment scale are has been shown to be independent of the degree of motor
available through the Brazelton Institute for purchase or involvement when compared with other tests of visual per-
through the local university’s learning resource centers.200 ception.206 The TVPS-3 was standardized on a nationally
A full developmental evaluation appropriate for the infant stratified sample of 2000 children across the United States.
and toddler with spina bifida is the Bayley Scales of Infant With a firm database provided by a thorough physical and
and Toddler Development, Third Edition (BSID-III).201 The occupational therapy evaluation with referrals to other pro-
Bayley Scales, consisting of a mental and motor scale and a fessionals as appropriate, a reasonable treatment plan can be
behavioral rating scale, can be used to test children from age developed and updated as necessary.
1 month to 42 months. The test provides information on
gross motor, fine motor, language, social-emotional, adap- TREATMENT PLANNING AND
tive, and cognitive development. REHABILITATION RELATED TO SIGNIFICANT
The BSID-III is well standardized and reliable and takes STAGES OF DEVELOPMENT
approximately 45 minutes to administer. It is not an easy test
to learn and initially requires supervision of an experienced Newborn to Toddler (Preambulatory Phase)
tester. This edition provides new normative data, extended Stage 1: Before Closure of Myelomeningocele—
age range, expanded content coverage, and improved psy- Early Newborn Period
chometric qualities. Physical therapy management of the infant in stage 1 is
The BSID-III provides the clinician with a broader view of limited by his or her medical condition (Table 15-2). Thera-
the child’s total development. The gross motor information pists are called on a regular basis in large tertiary care
from this developmental assessment will not be specific centers to carry out preoperative MMT to help to ascertain
enough for a therapist evaluating a child with spina bifida. functional motor level. Physicians (neurosurgeons and
The additional information on fine motor, language, personal- orthopedic surgeons) on the spina bifida care team rely on
social, and cognitive development, however, is sufficient and this assessment to guide their discussion with the families
will be important in planning a comprehensive intervention regarding care and prognosis. When carrying out the preop-
program.201 erative MMT, great care must be taken to avoid contaminat-
Various tests are available as screening tools to test ing an open sac, which is usually covered with a Telfa non-
visual-motor integration and perception. The Beery-Buktenica adherent dressing or a wet sterile dressing that must be kept
Developmental Test of Visual-Motor Integration, 6th Edition moist with a saline solution.
(Beery VMI) is an early screening tool to aid in diagnosis of
learning problems in children. It assesses integration of Stage 2: After Surgery, during Hospitalization,
visual perception and motor control of children from age and Transition to Home—Newborn through
2 years through 18 years. The test takes 10 to 15 minutes to Early Infancy
complete and requires the child to be able to copy designs. Therapeutic intervention after surgical back closure during
The Beery VMI is norm referenced and was standardized on stage 2 is often limited by the infant’s neurological and or-
a large sample of children chosen from throughout the thopedic status. A major goal during this stage is to prevent
United States. There is also an adult version that can be used contractures and maintain ROM while giving stimulation to
with individuals 19 to 100 years of age that facilitates iden- provide as normal an environment as possible. Traditional
tification of neurological and related problems in the adult.202 ROM exercises can be taught to nursing staff and family.
Children with spina bifida often exhibit upper-extremity They also can be carried out while the child is being held
weakness in addition to probable sensory dysfunction. As a at the adult’s shoulder or prone over the adult’s lap.
result, fine motor skills in children with spina bifida are These positions allow closeness between the caregiver and
often impeded by slowness and inadequate adjustment infant, thus encouraging maximal relaxation and interaction
of manipulative forces, and a non–motor-perceptual test is between them. ROM movements and positioning in prone or
often desired.203-205 The Motor-Free Visual Perception Test, side lying may be initiated to prevent or decrease contrac-
Third Edition (MVPT-3)206 and the Test of Visual Perceptual tures in the lower extremities. If clubfeet are present, soft
Skills, Non-Motor, Third Edition (TVPS-3)207 can be used tissue stretching may be indicated. Stretching begins distally
to determine the child’s visual perceptual processing skills on the soft tissue of the forefoot and proceeds proximally
on the basis of a non–motor assessment of these skills. Both toward the calcaneus. This is done to take advantage of
tests evaluate visual discrimination, visual memory, spatial the pliability of soft tissue structures and to minimize
relations, figure-ground, and visual closure. The TVPS-3 fixed deformity later. In addition, taping may be used to
also evaluates form constancy and sequential memory. The maintain optimal ROM and alignment between periods of
TABLE 15-2 n SUMMARY OF TREATMENT PLANNING AND REHABILITATION RELATED

TO SIGNIFICANT STAGES OF DEVELOPMENT
STAGE OF RECOVERY MAJOR PHYSICAL THERAPY GOALS PHYSICAL THERAPY MANAGEMENT

NEWBORN TO TODDLER (PREAMBULATORY PHASE)
Stage 1: before Determine functional motor level Preoperative manual muscle testing
surgical closure of
myelomeningocele—
newborn
Stage 2: after Confirm functional motor level Postoperative manual muscle testing
surgery, during Prevent contracture and deformity ROM exercises taught to hospital personnel and family
hospitalization— Encourage normal sensorimotor Positioning in prone and side lying
newborn to infant development Provide toys of various colors, textures, and shapes
Graded auditory and visual stimuli: music boxes, squeaky toys,
brightly colored objects
Therapeutic handling to encourage good head and trunk control
Stage 3: condition Confirm functional motor level Manual muscle testing once or twice per year
stabilized—infant to Encourage normal development sequence Work in sitting on head righting and equilibrium reactions
toddler Eye-hand coordination activities
Early weight bearing on lower extremities
Encourage prone progression
Weight shifting in standing frame
Comprehensive home program
TODDLER THROUGH ADOLESCENT (AMBULATORY PHASE)
Stage 4: toddler Confirm functional motor level Manual muscle testing once or twice per year
through preschool Begin ambulation Choose appropriate orthotic device
Continue development in cognitive and Gait training
psychosocial areas Development and strengthening of righting and equilibrium
Collaborate on goals with other team reactions
members Consider referral to EI program
Public preschool program
Continue home program
Open communication with other team members
Stage 5: primary school Confirm functional motor level Postoperative manual muscle testing
through adolescence Reevaluate ambulation potential Replace orthotic device as necessary
Maintain present level of functioning Wheelchair prescriptions as necessary
Prevent skin breakdown as child becomes Teach locomotion activities
more sedentary Maintain strength in trunk and extremities
Promote independence in self-care skills Teach skin care
Remediate any perceptual-motor problems Work with team members to teach dressing, feeding, hygiene, and
Provide appropriate adaptive devices bowel and bladder care
Promote self-esteem and social-sexual Provide program and activities for sensorimotor integration
adjustment Check for fit and proper use of adaptive devices
Collaborate with other team members in counseling efforts
EI, Early intervention; ROM, range of motion.
stretching.150 In treating the newborn after surgery, great When the child is not being handled, resting positions
care must be taken to avoid contaminating the surgical can be used to maintain ROM and enhance development.
dressing, which is usually covered with Xeroform Petrola- The prone position is the most advantageous because it pre-
tum Gauze (3% bismuth tribromophenate in a special petro- vents hip flexion contractures and encourages development
latum blend on fine mesh gauze). This dressing is nonadher- of extensor musculature as the child lifts his or her head.
ent and clings and conforms to all body contours. The Side lying, which allows the hands to come to midline and
Xeroform dressing is covered with Telfa. This postoperative generally encourages symmetrical posture, can be used for
dressing remains on for 2 weeks. alternate positioning. As much as possible, the supine posi-
Because of their medical conditions, hospitalized infants tion should be avoided because the child is most dominated
often experience early separation from their parents. Teach- by primitive reflexes and the effects of gravity in this posi-
ing the family to handle the child as described may enhance tion. For example, for the child with spina bifida with CNS
parent-infant bonding. Adequate bonding is essential for involvement, the effects of the tonic labyrinthine reflex com-
normal psychosocial development to occur. bined with paralytic lower extremities may make movement
from the supine position extremely difficult. Before initiat- activities should be introduced. Upper-extremity function-
ing activities in the supine position, the therapist should ing is often overlooked in the child with spina bifida, whose
obtain medical clearance. problems appear to be concentrated in the lower extremities.
A normal sensory experience should be presented to the However, most children with spina bifida show decreased
child in spite of the hospital setting. Toys of various colors, fine motor coordination, and this problem should be
textures, and shapes should be available. Musical mobiles addressed as developmentally appropriate. The normal in-
held low enough for the child to reach provide a variety of fant begins to reach and grasp by 6 months of age; therefore
sensory experiences. Stimuli such as squeaky toys or the the child with spina bifida must be given ample opportuni-
human face and voice can be used to encourage visual and ties to practice and perfect these same skills at an early age.
auditory tracking. Controlled stimulation relevant to the Because many children with spina bifida may be receiving
infant’s neurological state, rather than overstimulation, PT as their primary service through EI in these early
should be the rule. Depending on the age of the child, months, referral to and consultation with an OT at this age
appropriate learning situations must be presented to provide are highly recommended.
the child with as normal an environment as possible for Following a normal developmental sequence, the child
perceptual and cognitive growth. with spina bifida will usually begin some form of prone
A major therapeutic goal is to guide the child through the progression as trunk and upper-extremity stability improve.
developmental sequence, ultimately preparing him or her to This is a significant phase of development because it allows
assume the upright posture. In this immediate postsurgical for the development of a sensorimotor base as the child
stage, the primary emphasis should be on attaining good expands environmental horizons. During this phase of high
head and trunk control and eliciting appropriate righting mobility, insensate skin must be checked for injury fre-
reactions. For example, the child can be seated on the thera- quently and often must be protected by heavier clothing.
pist’s lap, facing the therapist, and alternately lowered This may help prevent any major skin breakdown, which
slowly backward and side to side. This action helps stimu- could significantly delay the rehabilitation process. For
late head righting and strengthen neck and abdominal some children with high-level lesions in whom prone mobil-
muscles. Weight shifting in various positions and through ity is not safe or practical for long distances, a Star Car
therapeutic handling is important to enhance development of (Tash) (Figure 15-13), the Ready Racer (Tumble Forms), or
early head and trunk control. Developmental handling may the PlasmaCar may be used. These provide the child with a
be limited by surgical interventions that limit mobility. means of exploring the environment safely but indepen-
This second stage ends as the child is discharged from the dently.
hospital. After discharge the child should be monitored Emphasis on head and trunk control and strengthening
closely by the spina bifida team, which may include a neu- exercises in a variety of sitting postures is quite important
rosurgeon, an orthopedist, a urologist, a nurse clinician, a in this early preambulatory phase. Development of ade-
PT, an occupational therapist (OT), an orthotist, and a social quate strength and motor control for trunk righting, equilib-
worker. Before discharge, a definitive home program as well rium reactions, and protective reactions will ultimately
as referral to the local Early Intervention (EI) program lead to improved sitting balance. Hands-free sitting with
should be given to the family because the child will most good balance is the optimal goal in this stage to allow for
likely require ongoing therapy, including both PT and OT.
Other professionals who may be involved in the child’s EI
program may include speech and language pathologists
(SLPs), developmental therapists (DTs), social workers, and
psychologists.
Stage 3: Condition Stabilized—Infant to Toddler
(Preambulatory)
In the third stage of rehabilitation, the major emphasis is on
preparing the child mentally and physically for upright
standing and mobility. In addition, routine MMT should be
performed every 6 to 12 months to reassess functional motor
level and to ascertain that no change in status has occurred.
Goals of preventing contractures and maintaining ROM will
remain throughout the child’s life. Unless this is done,
standing and ambulation become more difficult and often
impossible. If possible, prone positioning during play and
sleeping assists greatly in stretching tight musculature. Rest-
ing splints for the lower extremities or a total-body splint
can be used as necessary to position and maintain ROM and
alignment.
Developmental strategies should be aimed at facilitating
movement and motor control. Assuming that the child has
previously gained good head and trunk control, the next step
is development of sitting equilibrium reactions. As sitting
balance improves, fine motor and eye-hand coordination Figure 15-13 n Caster cart used for independent mobility.
independence and freedom in play skills. In addition, milestones. Providing appropriate facilitation of mobility
hands-free sitting is a necessary precursor to ambulation at a level similar to that of a child’s peers is important for
with lower-extremity bracing and often is the determinant psychosocial growth and development (Figure 15-15).
in deciding if a child will use a standing frame or will When the child attempts to pull to a standing position or
become a functional ambulator. would be expected to do so normally (at 10 to 12 months of
Early weight bearing is also of utmost importance, both age), the use of a standing device is indicated. Generally a
physiologically and psychologically. The upright position standing frame is the first orthosis chosen. This is a rela-
has beneficial effects on circulation and renal and bladder tively inexpensive tubular frame to which adjustable parts
functioning as well as on the promotion of bone growth and are attached (Figure 15-16). Because it is not custom made,
density.59,176,208,209 Psychologically, weight bearing in an it can be fitted fairly quickly, although adjustments may be
upright posture allows a normal view of the world and con- necessary to accommodate spinal deformities. This standing
tributes to more normal perceptual, cognitive, and emotional device offers support of the trunk, hips, and knees and leaves
growth. One way to achieve this weight bearing is in the hands free for other activities. Time spent in the standing
the kneeling position. This is developmentally appropriate frame should be increased gradually. This allows the child to
because children 8 to 10 months old frequently use kneeling adjust to the upright position in terms of muscle strength,
as a transition from all fours to standing. endurance, blood pressure, and pressure on skin surfaces.
Because young infants are frequently held in the standing After children have built up a tolerance for standing, they
position and bounced on their parents’ laps, this form of may be taught to move in the device by shifting their weight
weight bearing on the lower extremities is appropriate from from side to side. Initial shifting of weight onto one side of
birth onward. Failure to promote weight bearing in this man- the body is necessary to allow the other side to move for-
ner may deprive the child with spina bifida of the normal ward. This preliminary weight shift is also a prerequisite for
experience of standing at a very early age. When standing developing equilibrium reactions in the standing position
these children, however, care must be taken to see that the and thus will prepare the child for later ambulation. As the
lower extremities are in good alignment and that undue pres- child shifts weight, the trunk musculature on the weight-
sure is not exerted on them (Figure 15-14). In this way the bearing side should elongate and on the non–weight-bearing
risk of fractures is minimized and a normal weight-bearing side should shorten as muscle strength allows. This normal
experience is provided. reaction to weight shifting also includes righting of the
Also in this phase of preambulation, transitions from one head and should be closely monitored by the therapist for
position to another should be assessed and facilitated. completeness.
Teaching the child strategies for transitions will enhance his A therapy program must be designed to meet the indi-
or her optimal functional independence. Compensations vidual’s needs in each area. Age alone does not determine
may be taught to substitute for weakened musculature. In the appropriate therapeutic goals. Goals that are not suited
addition, adaptive equipment and mobility devices may be for the child’s cognitive and emotional needs, in addition to
recommended to enhance acquisition of age-appropriate physical needs, will not facilitate best outcomes. For exam-
ple, an 18-month-old may have the physical capabilities to
ambulate independently with crutches and braces. The child
may not, however, have the cognitive skills necessary to
learn a four-point gait or be ready emotionally to separate
from his or her mother for intensive therapy sessions. A
more realistic goal may be to let the child walk holding onto
furniture (cruising) while a wheeled walker for more
independent ambulation is slowly introduced. Another alter-
native to using a conventional walker is to encourage the
child to play with push toys such as grocery carts and baby
buggies.
During this preambulatory stage, therapy goals may be
accomplished through a comprehensive home program, with
frequent checks to note progress or problems and to change
the program accordingly. For the more involved child,
increased frequency of direct intervention may be indicated
to achieve optimal developmental progress.
The program often must be reevaluated and goals changed
if conditions such as shunt malfunctions or fractures occur.
The warning signs for shunt dysfunctions are generally
those previously described for suspected hydrocephalus. In
addition, swelling along the shunt site may indicate a mal-
function. Swelling and local heat or redness of a limb are the
usual signs of a fracture. The limb may also look mis-
aligned. Fever may accompany a fracture. As previously
mentioned, these fractures generally heal quickly with
proper medical intervention and minimally interrupt reha-
Figure 15-14 n Assisted standing with normal postural alignment. bilitation efforts.
Figure 15-15 n Adaptive devices can help the young child with spina bifida reach major
milestones at the same time as peers. (From Ratcliffe KT: Clinical pediatric physical therapy,
St Louis, 1998, Mosby.)
use of mental imagery and physical knowledge of their

environments expand. Concepts of size, number, color,
form, and space are all developing. Emotionally, most chil-
dren are becoming more independent and begin to break
away from the sheltered environment of the home. They are
now more interested in interacting with others and become
social beings to a greater extent.
All these changes in physical, cognitive, and emotional
development will be evident in the child with spina bifida,
although the degree depends on the extent of the functional
limitations and their effect on the child’s ability to participate
in life. The characteristics of normal development must be
understood so that cognitive, emotional, and motor behaviors
can be nurtured and enhanced in the child with spina bifida.
Goals for this as for any other stage must address physi-
cal, cognitive, and emotional development. The most obvi-
ous goal at this stage is to help the child who is already
standing to progress to an ambulatory status. Even the child
with a low thoracic lesion can usually manage some form of
ambulation.
Thus far, the child has learned to shift weight in the
standing frame. By rotating the trunk toward the weighted
side, the non–weight-bearing side can be shifted forward
(Figure 15-17). By reversing the weight shift, the opposite
side can be moved forward and a type of “pivoting forward”
Figure 15-16 n Standing frame. A, Anterior view. B, Lateral view.
progression can be accomplished. To maintain balance
while shifting, the child may initially use a two-wheeled
walker. The therapist may help initiate weight shift and
trunk rotation by alternately pulling the arms forward.210
Toddler through Adolescent (Ambulatory Once the child has gained this form of mobility, the type of
Phase) permanent bracing chosen will depend on the level of the
Stage 4: Toddler through Preschool lesion and a variety of other factors.
The fourth period in development marks the end of infancy The overall goal for ambulatory training is to promote
and the beginning of childhood. For the typically developing efficient, independent mobility with the least amount of
child who has developed a strong sensorimotor foundation, bracing while maintaining optimal joint integrity. Ambula-
physical development is marked by increased coordination tory potential as well as choice of bracing depends on many
and refinement of movement patterns. In addition, a great factors, including neurosegmental level of the lesion, motor
variety of motor skills will be achieved as the typically power at the neurosegmental level, extent and degree of
developing child learns to throw, catch, run, hop, and jump. orthopedic deformity, balance, age, height, weight, sex, mo-
This is also a period of great cognitive growth, as children’s tivation, spasticity, design and effectiveness of the orthosis,
minute compared with typically developing peers (14.4 ver-

sus 40.8 steps per minute) and a decrease in frequency of
alternating steps. In contrast to these interlimb coordination
differences, they reported that within-limb step parameters
in infants with spina bifida were quite similar to those in
children who were typically developing.
For thoracic and high-level lumbar lesions, a parapodium is
often chosen. The parapodium was developed by the Ontario
Crippled Children’s Centre in 1970 and is similar to the stand-
ing frame except that hinges at the hips and knees allow for
sitting and standing.210 It also can be adjusted for growth and
can accommodate orthopedic deformities. As with the stand-
ing frame, proper alignment of the parapodium is critical. The
therapist, in conjunction with the orthotist, should check
for correct standing alignment. The prevention of additional
orthopedic deformities, development of good muscular con-
trol, and normal body image depend on a well fitting orthosis.
After a pivoting gait has been learned with the parapodium,
a swing-to or swing-through gait can be attempted. By 4 to
5 years of age, a swing-through gait, with the child using
Lofstrand crutches, can usually be accomplished. Variations of
Figure 15-17 n Weight shift and forward rotation in standing the parapodium allow for easier locking and unlocking of hip
frame. and knee joints. A swivel or pivot walker also may be attached
to the footplate to allow for crutchless walking.
Another type of orthosis for the child with a thoracic or
effectiveness of PT intervention, environmental factors, high lumbar lesion is the Orthotic Research and Locomotion
upper-extremity strength and control, and cognitive level.163,211 Assessment Unit (ORLAU) swivel walker. It consists of
The best prognosis for ambulation is most often seen in the modular design similar to that of the standing frame, with a
child who is not shunted and has good cognition, good quad- chest strap and knee blocks attached to swiveling foot-
riceps power, no deformity, a stable neurological condition, plates.216 Rather than the whole base moving forward, as
and hands-free sitting balance. Factors that may limit poten- when weight is shifted in the parapodium, in the swivel
tial for ambulation include hydrocephalus, high-level lesions, walker each footplate is spring-loaded and is able to swivel
kyphosis or kyphoscoliosis, and unstable neurology. forward independently. This allows for independent balance
Developmental progression preceding ambulation in on one foot and therefore crutchless ambulation. The
spina bifida shows a high degree of variability.212 A study by ORLAU swivel walker is manufactured in the United
Bartonek in 2010212 confirmed that those with greater mus- Kingdom, and assembly kits may be ordered; however,
cle power of the lower limb muscles ambulated earlier and availability is limited (Appendix 15-A).217 Another parapo-
more frequently. Those engaged in physical therapy pro- dium in use is the Rochester parapodium. Separate hip and
grams aimed at achieving the specific goal of walking are knee joints allow a variety of free movement for sitting and
also likely to make earlier strides toward ambulation, bending down. The lower portion remains rigid and support-
although this has not been formally studied. Those who have ive when hips are flexed. Therefore a child can bend and
been enrolled in early treadmill body-weight support train- pick up objects from the floor, and the unlockable hip and
ing (BWST) programs as infants have demonstrated early knee joints will relock automatically on extension.218
stepping responsiveness, and it has been suggested that There is a perception that although children with myelo-
BWST training intervention could promote muscle strength- meningocele use orthoses effectively, very few continue
ening and take advantage of neural plasticity to promote ambulation into adulthood. Two studies of patients with
development of the neuromuscular patterns necessary to thoracic-level myelomeningocele219 demonstrated that the
support the onset of gait. There is also potential to improve ORLAU can be used effectively into the adult years. Both
bone density, cardiovascular function, and the integrity of studies noted a 58% to 59.4% compliance rate. Patients in
lower spinal sensorimotor function. Infants with spina bifida the studies who started using the ORLAU after 11 years of
demonstrate developmental delay as early as 3 months of age continued use for 3 to 24 years. Mazur and colleagues219
age, and BWST intervention during the first postnatal year noted that those with myelomeningocele who did not ambu-
has the potential to reduce this delay and promote earlier late had a fivefold increase in pressure sores and twice the
onset of gait in the population with spina bifida.213 Efficacy number of fractures compared who those who did ambulate.
of enhanced sensory inputs during treadmill stepping in Vigorous walking programs should be considered to assist
children with spina bifida has also been examined. Increas- long-term health.
ing friction by using Dycem matting and enhancing visual Both the parapodium and swivel walker have had some
flow by using a checkerboard pattern on the treadmill belt problems with instability, ease of application, and cosmesis.
both appear to be more effective than the standard black New designs attempt to correct these problems.220 Neverthe-
treadmill belt in eliciting stepping.214 Teulier and col- less, existing limitations in the parapodium and swivel walker,
leagues215 studied stepping responses in infants with particularly energy cost of walking, slow rate of locomotion,
spina bifida. They reported a decrease in number of steps per and cosmesis, have limited their use, primarily to the younger
child. These devices, however, remain an effective means of through use of hip flexor or lower abdominal muscle contrac-
preventing musculoskeletal deformities caused by long-term tion or through use of active or passive trunk extension. In a
sitting, wheelchair positioning, and general immobility. They study of 15 patients with lesions from T10-L3, use of the
also enhance social-emotional development gained from the RGO produced favorable results. It was used effectively by
upright position.216,221 Another option for the child with a 13 of the 15 patients. Initial use of the RGO was initiated at
higher-level lesion and good sitting balance is the reciprocat- 5 years, and eight of the 15 discontinued use at 10 years
ing gait orthosis (RGO). This brace consists of bilateral long- of age. During the period of use, four became community
leg braces with a pelvic band and thoracic extension, if neces- ambulators, nine were household ambulators, and two
sary. The hip joints are connected by a cable system that can remained nonfunctional (standing only). Average daily use
work in two ways: If the child has active hip flexors, he or she ranged from 6 hours for those ambulating in the community
can activate the cable system by shifting weight and flexing to 30 minutes for those who were nonfunctional ambulators.
the non–weight-bearing extremity. This brings the weight- Six of the 15 had no quadriceps power yet were able to func-
bearing extremity into relative extension in preparation for the tionally use the RGO for ambulation. Strong motivation and
next step. Without hip flexors, the child extends his or her realistic goals are important to successful use.224
trunk over one extremity, thus positioning it in relative exten- A more common means of maintaining the upright posi-
sion. By virtue of the cable system, the non–weight-bearing tion has been through the use of long- or short-leg braces.
extremity moves into flexion, thus initiating a step. Several Polypropylene braces and carbon-reinforced braces are con-
types of the RGO are in use, including the dual-cable LSU222 siderably lighter than metal bracing and therefore reduce the
and the horizontal-cable type.223 energy cost of walking for the child with spina bifida. They
Most recently the Isocentric Reciprocating Gait Orthosis allow close contact and can be slipped into the shoe rather
(I-RGO) (Center for Orthotics Design, Campbell, California) than worn externally, thus affording the patient a better-
has been used for children with high-level spina bifida. It has fitting, more cosmetic orthosis.
a more cosmetic and efficient design compared with the The type of orthosis chosen (long-leg, with or without
dual-cable LSU or horizontal-cable–type RGO. This cable- pelvic band, or short-leg) depends on the level of the myelo-
less brace has two to three times less friction and therefore is meningocele and the muscle power within that level (Table
more energy efficient. The brace stabilizes the hip, knee, and 15-3). Because lesions are frequently incomplete, muscle
ankle joints and balances the person, enabling him or her strength must be accurately assessed before bracing is pre-
to stand hands free without the use of crutches or a walker scribed. Independent sitting balance with hands free also is a
(Figure 15-18).223 Leg advancement for walking occurs prerequisite for use of long- or short-leg braces. Even children
with L3 to L4 lesions who demonstrate incomplete knee
extension may be able to use a short-leg brace with an anterior
shell rather than requiring long-leg bracing.225 This crouch-
control AFO (CCAFO) will prevent a crouching gait pattern
by improving knee extension during gait (Figure 15-19).226
Another alternative to a standard solid ankle AFO may be the
carbon fiber spring AFO. This brace provides dynamic assist,
supports the patient through the entire stance phase, and in-
creases the energy return during the third rocker phase of
push-off, simulating the natural push-off action.227 For chil-
dren demonstrating excessive knee valgus caused by hip
adduction, use of a Ferrari KAFO (FKAFO) may be consid-
ered.228 The PT must work in conjunction with the orthopedist
and orthotist to have each child fitted with the minimal
amount of bracing that allows for joint stability and a good
gait pattern (see Chapter 34).
Children with lower-level lesions (L5-S1) who use below-
knee bracing often develop the ability to or choose to ambu-
late without assistive devices. However, recent studies have
shown that crutch use may decrease excessive pelvic motion,
which results in reducing abnormal joint forces.162,229 Use
of crutches may prevent abnormal joint forces, maintain
joint integrity, and decrease the risk of additional orthopedic
complications.
Literature has suggested that crutch-assisted ambulation
may result in long-term pathology. In patients with higher
lumbar lesions (L3-L4) who use Lofstrand crutches, the
dynamics and kinematics of upper-extremity function were
explored during swing-through and four-point reciprocal
modes of gait. Although there were better joint kinematics
in the shoulder and other upper-extremity joints during
swing-through gait, kinetics were more problematic with
Figure 15-18 n Reciprocating gait orthosis. increased force and torque in shoulder and wrist joints in
TABLE 15-3 n COMMON GAIT PATTERNS AND LEVELS OF ASSISTANCE REQUIRED

IN MYELOMENINGOCELE
RECOMMENDED LEVEL
LEVEL OF LESION MUSCLE PERFORMANCE OF ASSISTANCE AND BRACING AMBULATORY PROGRESSION
T8-L1 and above Flaccid LEs with fair to poor Parapodium: ORLAU, Toronto,
trunk Rochester
Assistive devices often unnecessary
with ORLAU but may improve
function with Toronto or
Rochester braces.
L1-L2 Flaccid LEs with hip flexors Parapodium with progression to Begin ambulating with a walker, progress
present RGO to forearm crutches.
RGO, ambulating with hips locked. Four-point or swing-through gait.
L3-L4 Fair quadriceps with weak or HKAFOs may be used with severe Begin ambulating with a walker, progress
absent hamstrings lordosis because of weak or to forearm crutches.
absent gluteal musculature and Four-point gait.
decreased trunk control or to Begin ambulating with a walker and
control rotation and abduction progress to forearm crutches.
and adduction. In some rare cases the patient may
If quadriceps are less than fair progress to no assistive device at all
strength, KAFOs may be needed. depending on the gait pattern.
As the patient progresses he or she With increased use of trunk reversal the
may be cut down from KAFOs to patient should be returned to forearm
AFOs; AFOs may be used with or crutches to allow for a pattern that is
without twister cables. more cosmetic and energy efficient.
Four-point gait pattern.
L5 Good hip flexors and AFOs with or without twisters Forearm crutches or no assistive devices.
quadriceps; fair anterior depending on gluteal strength. Four-point gait.
tibialis; weak gluteus AFO is used to prevent a crouch
medius and maximus, toe gait pattern from weak
extensors and gastrocsoleus gastrocsoleus.
S1 Good hip flexors, quadriceps, AFO Generally no assistive device is used
gluteus medius, and toe unless decreased balance reactions or
extensors; weak gluteus excessive lateral trunk flexion is present.
maximus and gastrocsoleus
S2-S3 Good hip flexors, quadriceps, Often no bracing needed. Often no assistive devices needed.
gluteus medius and
maximus, and gastrocsoleus
AFO, Ankle-foot-orthosis; HKAFO, hip-knee-ankle-foot orthosis; KAFO, knee-ankle-foot orthosis; LE, lower extremity; RGO, reciprocating gait orthosis.
those using a swing-through gait. Whereas the swing- means of mobility should have elastic twisters prescribed to
through gait allows a potentially faster mode of ambulation, allow for ease of creeping and transitions. The older and
long-term use of this pattern may lead to increased upper- more active child will require heavy-duty torsion cables.
extremity pathology. Careful monitoring of all joints, Rotational stresses may eventually lead to onset of late
including upper-extremity joints, during gait reassessment degenerative changes around the knee. A tibial derotation
should be considered in order to deter and manage these osteotomy may be indicated to prevent these changes from
potential issues that may compromise overall joint integrity occurring.159,231,232
and function.230 For children with low lumbar or sacral lesions who have
The excessive femoral torsion present in all newborns at at least fair strength in their dorsiflexors and plantar flexors,
birth does not decrease with growth and development in the often a University of California Biomechanics Laboratory
child with spina bifida because of abnormal gait and activ- (UCBL) or polypropylene shoe insert to control foot posi-
ity.159 Children ambulating with AFOs often show excessive tion is the only bracing needed. These inserts fit snuggly
rotation at the knee because of the lack of functioning lateral inside the shoe and help control calcaneal and forefoot insta-
hamstrings. Rather than going to a higher level of bracing, a bilities. A supramalleolar orthosis (SMO) will also fit easily
twister cable can be added, which often decreases the rotary inside the shoe but will provide additional medial and lateral
component during gait.159 Twister cables can be heavy-duty support and stability that an insert would not provide. Even
torsion or more flexible elastic webbing, depending on func- though a child may be able to ambulate without an assistive
tion. Typically, the young child who is just beginning to pull device or bracing, consideration must be given to the
to stand and remains reliant on floor mobility as the primary stresses that occur at the joints that over time may lead to
Figure 15-19 n Crouch control ankle-foot orthosis.
orthopedic deformity. The greatest risk of joint instability

often occurs at the knee. Barefoot walking versus use of an Figure 15-20 n Balance and strengthening exercises done on a
AFO has shown increased instability, joint stress, and pain movable surface.
at the hip and knee as well as increased energy expendi-
ture.233-235 Even though children may be able to ambulate
without the use of crutches, comparison of gait kinetics and board require use of these muscles while providing an
kinematics of walking with crutches has shown a significant enjoyable exercise for the child. Participation in hippother-
decrease of valgus forces at the knee and better overall apy and aquatherapy programs at this age can also be ben-
alignment of the lower extremities.229 Treatment aimed eficial. These treatment approaches can be used to improve
at strengthening the gluteus medius and maximus to aid in muscle strength in general and the gait pattern when bracing
increasing pelvic stability and reducing kinematic compen- is reapplied. Regardless of the strengthening activities
sations can also be important in the management of patients chosen, the pediatric therapist has the special task of using
with lesions at this level to enhance their efficiency during creativity to involve the child in therapeutic play activities.
ambulation.236,237 The ideas for creative activities are limitless but essential for
Gait training, begun as the child first starts to stand, can combining therapy with age-appropriate cognitive abilities.
now continue in a more formalized manner. By using the Studies examining the effects of strength and endurance
appropriate orthosis and assistive devices (walker, crutches, training in those with spina bifida238-240 have demonstrated
or cane), each child must be helped to achieve the most enhanced functional outcomes. These studies included indi-
efficient and effective gait pattern possible (see Table 15-3). viduals in wheelchairs as well as ambulators. Interventions
As a part of gait training, the child should be taken out of the aimed at strengthening those with spina bifida, including
bracing and “challenged” so that righting and equilibrium electrical stimulation,241,242 behavioral treatment,243,244 and
reactions can be developed to their maximum. For example, motor skills training243 have been reported. The evidence
having a child maintain balance while sitting on a ball or supporting strengthening, using exercise, electrical stimula-
other movable surface (tilt board, trampoline) requires tion, and motor skills training was recently examined by
the participation of all available musculature, especially Dagenais and colleagues in a systemic review.245 This
abdominal and trunk extensor muscles (Figure 15-20). review synthesized six studies supporting interventions in
Strengthening available musculature is a primary objective strengthening.240-246 The level of evidence for the studies
in this phase of treatment. Slow gains in muscle strength was determined using the American Academy of Cerebral
are often the result of continued emphasis on strengthening Palsy and Developmental Medicine Levels of evidence.247
during physical therapy. In addition to trunk muscles, the Levels of evaluated studies were graded and noted to be II
gluteus medius, gluteus maximus, and quadriceps are often (smaller randomized controlled trials with wider confidence
targeted. Prone activities such as picking up toys while over intervals, n ,100), IV (case series or cohort study without
a Swiss ball or moving around while prone on a scooter concurrent control, case-control study) or V (case study).
The total number of subjects in all of the studies was 26, impedes their ability to anticipate changes in terrain and poses
with 50% of those subjects in the Andrade and colleagues246 a safety problem. Vision may be the most relevant system to
study. All six studies reported improvements in strength; allow them to scan and preplan for changes in their walking
however, only three noted statistically significant results. environment.
Although the evidence suggests the possibility of being able Gait training and muscle strengthening are not the only
to increase muscle strength using these modalities, in this consideration of the therapist. How cognitive and psychoso-
population one must interpret the results with caution owing cial development can be enhanced during this stage of the
to lack of rigor, small subject populations, and variability child’s development is also important. One appropriate solu-
across studies. tion is to place the child in a center-based EI program.
Spina bifida is a congenital-onset condition that requires Although these programs may vary in the services they pro-
intervention by the PT from infancy through adolescence. vide, most usually include age-appropriate play activities
Most of the impairments and functional deficits described and some type of parental counseling. In addition, many of-
throughout this chapter last throughout a lifetime. Health fer therapeutic intervention from physical, occupational, and
providers familiar with the complexity of the secondary speech therapists. This intervention may occur in groups
impairments should follow individuals with spina bifida on a or individually and typically occurs in the child’s natural
regular basis. The PT plays an important role in screening for environment.
potential problems and providing recommendations for In addition to the socialization that center-based EI pro-
maintenance of mobility and health-related fitness as well as grams provide for the child with myelomeningocele, they
promoting activity and participation for children with spina also teach the child age-appropriate ADLs, such as dressing
bifida. OTs within the school systems often provide interven- and undressing. At this age ADL skills are more appropri-
tions within the area of integration of perceptual function. ately taught in a group setting than individually. For many
However, additional research needs to be done to guide “best children the EI program, along with individualized therapy,
practice,” as there is little support for those interventions that is sufficient to enhance development in the physical, cogni-
expert clinicians deem beneficial in the habilitation of these tive, and psychosocial realms.
patients. Presently, when children reach age 3 years, public
Obesity affects ability for interaction and participation at school education becomes available to them. The pre-
multiple levels across the life span of those with spina bifida. school or early childhood (EC) program continues to offer
Obesity may affect independence in transfers and ambula- the same fundamental benefits as the EI program. It is the
tion, self-care and mobility, as well as personal social inter- role of the EI therapist to communicate the specific needs
actions at all ages. Often the child with spina bifida may be of each child entering the public school system. In this
the last to be asked to participate and/or the child’s inability way continuity in the child’s rehabilitation program is
may affect his or her ability or willingness to participate in preserved.
physical games and activities with peers. Equipment and The spina bifida team, usually headed by a pediatrician or
orthotic needs can also be complicated by increases in clinical nurse specialist, continues to follow the child closely
weight gain and obesity as the child ages. during this stage. The neurosurgeon checks shunt function-
Studies using dual-emission x-ray absorptiometry (DEXA) ing and performs revisions as necessary. The orthopedist
have shown that those with spina bifida have significantly de- supervises bracing efforts to prevent and correct deformities
creased lean body mass as compared with controls.248 Children in the spine and lower extremities. Well-child care and gen-
with spina bifida likely have lower metabolic rates secondary eral medical treatment are the responsibility of the pediatri-
to decreased muscle mass and decreased ability to burn calo- cian on the team. The urologist continues to monitor renal
ries. Those with higher-level lesions often have greater issues functioning while keeping the child dry and free of infec-
with obesity owing to decreased lower-extremity muscle activ- tion. At this stage the clinical nurse specialist will usually
ity, decreased physical activity, and decreased overall muscle teach bowel and bladder training to the child and family.
mass. Adult wheelchair users have been shown to require 1500 This clinician generally initiates this training according to
fewer calories per day, and overall nutritional intake should be age-appropriate developmental guidelines.
lower for those with spina bifida than their typically develop- Bladder training usually consists of transferring the job
ing peers.249-251 Interventions to address physical inactivity and of CIC from the parents to the child. Children as young as
obesity may include increased physical activity, regular exer- 3 years, but certainly by the age of 5 years, can learn CIC in
cise, behavioral modifications, nutrition education programs, a short period.254 Children may first practice on dolls with
and attention to weight control. Exercise programs targeting male and female genitalia. Next, using mirrors to understand
upper-extremity resistance combined with aerobic activities their own genital anatomy, they are able to accomplish the
such as swimming and arm-cycling using arm-ergometry can technique on themselves. CIC in conjunction with pharma-
be helpful in addressing obesity in those with spina bifida. cotherapy is useful in achieving continence in children with
Adolescents with spina bifida who used an upper-extremity spina bifida.140 Another method of bladder training recently
cycling program that was integrated with video gaming three being used in the United States is intravesical transurethral
times per week for 16 weeks improved oxygen uptake and bladder stimulation. This technique has allowed children
maximum work capability.248,252,253 with neurogenic bladder to rehabilitate their bladder
Sensory limitations may impede progress during early am- function so that they can detect bladder fullness and gener-
bulation training. Because of limited kinesthesia and proprio- ate effective detrusor contractions, leading to improved
ception, available sensory systems must be augmented and the continence.100,255
child taught to substitute with nonimpaired sensory systems. Bowel training can be achieved through proper diet,
Impaired kinesthesia in children with myelomeningocele regular evacuation times, and appropriate use of stool
softeners and suppositories.100 Constipation (and resulting behaviors should be promoted from very early on. Engage-
bypass diarrhea) can be prevented by proper habit training ment in family decision making and opportunities for active
and use of fiber supplements. Stool softeners (not laxa- problem solving have been linked to increased positive self-
tives and enemas) and suppositories should be used to esteem and ego development.259 During this time period,
keep the stools soft and help stimulate evacuation. Finally, children with spina bifida are at risk for developmental
toilet training, which amounts to scheduled toileting in delays in social functioning.258
time with the stool stimulants, usually achieves bowel During this period, they are building the skills of the
continence. Surgical procedures, such as the Malone ACE future, preparing them for adult work. This is a prime time
procedure,91 may be necessary when other interventions to introduce and teach new skills while fostering increased
have failed. The Malone ACE procedure, performed in autonomy and independence. Autonomy is difficult for the
conjunction with a Mitrofanoff procedure to gain urinary youngster with spina bifida. Motor skills impeding progress
continence, can help these patients attain a better quality toward autonomy vary for each child and are dependent on
of life. Consistency at each step along the way is the key the level of spina bifida as well as any cognitive impairment.
to successful bowel training. A therapist may be called on However, it has been observed that the motor skills hardest
to assist the parents and child in achieving independence to attain are those that involve motor planning and that the
in this ADL. process skills hardest to attain are related to adaptation of
Other members of the team, such as the psychologist, performance and initiation of new steps. Thus guidance to
social worker, and dietician, continue to function in their learn not only how to do things but how to get things done
appropriate roles, interacting with the child and family is important.260 Interventions targeting independence have
as necessary. PTs and OTs, as members of the team, must been embedded into several camp programs throughout the
collaborate with the efforts of other team members in the country geared specifically toward those with spina bifida.
creation of their treatment plans. O’Mahar and colleagues261 report on one such camp pro-
Mobility and bladder and bowel dysfunction in toddlers gram focused on campers 7 to 14 years of age in northern
and school-age children represent ongoing stressors for par- Illinois. Camp Ability emphasized individualized collabora-
ents of children with spina bifida. It has been noted by many tive (i.e., parent and camper) goal setting, group sessions
that spina bifida represents a considerable challenge to all with psychoeducation and cognitive tools, and goal monitor-
family members, particularly mothers. Family climate, paring by the camp counselor. Campers reported significant
ents’ partner relationships, and social support networks play gains in individual goals, management of spina bifida
a considerable role in balancing stress and psychological responsibilities, and independence. Medium effect sizes in
adjustment for parents. Awareness of available systems of goal attainment and progress from the start to the end of the
support for the patient and family as well as resources to camp session were noted.261 It appears that these camps may
which parents and their children can be referred for psycho- have significant benefit in addressing management of one’s
logical and social support as needed is important for all disability as well as independence in self-care skills. An
health care practitioners.256,257 added benefit is the social interaction and physical activity
that the camp participants engaged in while working toward
Stage 5: Primary School through Adolescence their goals.
The fifth stage of development is marked by less rapid As the energy cost of walking becomes too high, use of a
growth than earlier childhood but ends with a period of rapid wheelchair for locomotion often becomes appropriate. To a
physiological growth. Children in the 6- to 10-year age teenager whose emotional needs include a strong peer iden-
group are interested in a wider variety of physical activities tity, transitioning to a wheelchair may foster increased inde-
as they challenge their bodies to perform. The adolescent, pendence owing to improved ability to participate and
however, is going through a period of great sexual differen- engage with peers. Appropriate alternatives may be to delay
tiation as primary and secondary sexual characteristics the decision to use a wheelchair full time or limit ambulation
develop more fully. to short distances or to those places most important to the
Cognitively, children are able to solve problems in a child. Again, goals must be tailored to the child’s needs and
more sophisticated manner, although they revert to illogical encompass his or her whole being.
thinking with complex problems. As they reach adoles- In accordance with the child’s growth spurts, frequent
cence, they become capable of hypothetical reasoning and adjustment or reordering of bracing will be necessary. Con-
their thought processes approach those of adults. tinual reevaluation of orthotic needs may reveal that the level
Emotionally, the 6- to 10-year-old is in a period of rela- of bracing may decrease as the child grows and becomes
tive calm. Children are interested in schoolwork and are stronger; the opposite development is also a possibility.
eager to produce. This is a period during which they are Usually during this stage, if it has not occurred previ-
developing relationships outside of the family and beginning ously, the evaluation of future ambulation potential occurs.
to assume an identity and autonomy. Problem-solving and The child whose larger size and limited abilities make
decision-making skills are at the crux of this time period. ambulation more difficult each day frequently requests this
However, it is also challenging to promote independence evaluation. Strength does not increase in the same propor-
and minimize self-reliant behaviors. Social passivity may tion as body weight.127 Ambulation, although possible for
ensue as “learned helplessness” behaviors emerge. There- the young child, may be impossible for that same person as
fore professionals, including both PTs and OTs, should a young adult.
begin targeting independent function early, and before Although no guidelines include every patient, generally
adolescence.258 Relevant family education regarding this is- children with thoracic-level lesions are rarely ambulators by
sue should be inherent in all care plans, and independent the late teens.152,262,263 Those with upper lumbar lesions may
be household ambulators with long-leg bracing but will interaction activities. In conjunction with the nurse, PT,
require wheelchairs for quick and efficient mobility as and OT, self-care skills of dressing, eating, and food
adults. With low lumbar lesions, most adults can become preparation; general hygiene; and bowel and bladder care
community ambulators. Patients with sacral-level lesions can be addressed. Because the adolescent is so concerned
are usually able to ambulate freely within the community. with achieving independence, he or she is more likely to
Many require minimal bracing and ambulate without assis- comply with a regimen of strengthening exercises if shown
tive devices.152,159 It must be remembered that ambulatory how they relate to functional independence. A creative
status is not determined by level of the lesion alone. The therapist may, for example, incorporate trunk stability and
muscle power available; degree of orthopedic deformity; upper-extremity strengthening work in activities such as
age, height, and weight of the patient; and, of course, moti- making popcorn or getting ready for a dance. In addition,
vation are also determining factors.82,154,159,264 fostering social and recreational independence through
Because a large number of older children with spina bifida adaptive sports and fitness programs and leisure activities
will become wheelchair dependent, potential problems con- should not be overlooked. Participation in adaptive sports
nected with a sedentary existence must be explored. Skin can aid immensely in improving strength, endurance, and
care, always a concern for the child with spina bifida, self-esteem. Community adaptive recreational programs
becomes a priority for the constant sitter. Mirrors may be used may include T-ball, martial arts, swimming, tennis, basket-
for self-inspection of the skin twice daily. Well-constructed ball, skiing, bowling, and many other common sports and
foam, gel, or air-cell seat cushions are essential for distribut- leisure activities (Figures 15-21 through 15-23).
ing pressure evenly. Children should be taught frequent Locomotion activities should include all gait-related
weight shifting within the chair to relieve pressure areas. skills, such as falling down, getting up, and ambulation on
Clothing should not be constricting but should be heavy various terrains and stairs. Transfers of all types should also
enough to protect sensitive skin from wheelchair parts. Chil- be included in locomotion activities. Again, a creative thera-
dren must also be taught to avoid extremes of temperature and peutic program helps make achievement of skills more pal-
environmental hazards, such as radiators, sharp objects, and atable. For example, school-aged children may enjoy a
abrasive surfaces. The therapist must reinforce the importance competitive relay race situation in which each child falls,
of skin care to prevent setbacks in the rehabilitation process gets up, walks across the room, and sits down in a chair
that may result when skin breakdown develops. safely. This type of activity combines gait-training activities
Children with higher-level lesions may need spinal with group socialization and may meet a variety of goals
support to prevent deformities. A polyethylene body jacket (motor and psychosocial) at the same time.
or thoracolumbar-sacral orthosis (TLSO) can be used to Achievement of independence in ADLs for the child and
provide this support and, it is hoped, prevent the progression adult with spina bifida does not depend solely on the level of
of any paralytic deformities. Whatever type of device or paralysis. Also important are psychosocial and environmental
wheelchair padding is used, the therapist must check to see factors. Mean ages for the achievement of various ADL
that weight is distributed equally through both buttocks and activities have been developed and may assist the therapist in
that the spine is supported as needed. Part of the therapeutic establishing realistic therapeutic goals in this area.266
intervention is to provide strengthening exercises or activi-
ties to be done out of the supporting orthosis. This is neces-
sary to maintain existing trunk strength and to preserve the
child’s present level of function.
Generally, in late childhood or early adolescence, ortho-
pedic deformities that have been gradually developing
require surgical intervention. Progressive scoliosis or
kyphosis may require internal fixation when conservative
methods fail.265 Sectioning of tight or contracted muscles at
the hip and knee is often required.152 The iliopsoas, adduc-
tors, and hamstrings are frequently the offending muscles.
These surgeries, followed by strengthening exercises and
gait training, often add to the ambulatory life of the child
with spina bifida. For example, in a child who displays an
extreme lordotic posture, hip flexion contractures may be
present and surgical lengthening of the tight muscles may
be required to allow improved biomechanical alignment for
standing and balance. Strengthening of the hip extensors
and abdominals also helps prevent future muscle imbal-
ances that may lead to contractures and tightness. A postop-
erative therapeutic program might include periods of prone
lying to prevent future contractures and strengthening of
hip extensors and abdominals that were previously over-
stretched by the lordotic position.
Of primary importance during this stage is preparing
the child for independence in ADLs, which may be Figure 15-21 n Participation in wheelchair racing. (Courtesy
broken down into self-care, locomotion-related, and social Su Metzel.)
Also as previously discussed, children with spina bifida

have a general perceptual deficit that can manifest itself in a
variety of ways. First, the child may have difficulty recog-
nizing objects and the relations that they have to one
another. He or she may therefore perceive the world in a
distorted manner and have reactions that are unstable and
unpredictable. These perceptual difficulties will most likely
affect academic learning, and the child may associate failure
with the learning process. Difficulties in attaining indepen-
dence in ADLs are also linked to perceptual problems.
Finally, emotional disturbances may be attributed in part to
the perceptual difficulties of the child with spina bifida.98
Remedial programs, such as the Frostig Program for the
Development of Visual Perception, have been effective
in improving the visual perception of children with spina
bifida.98 Programs of this type are most effective when
remediation begins early—preferably at or before the time
the child enters school. Developmental optometry examina-
tion and remediation programs focusing on vision training
may also be of benefit.
Children with spina bifida may also have difficulties with
tasks requiring sensorimotor integration. Children requiring
programs for sensorimotor integration should be referred to
a therapist certified in this area. If one is not available, many
appropriate activities for sensorimotor integration may be
Figure 15-22 n Participation in wheelchair basketball. (Courtesy
adapted from Ayres267 or Montgomery and Richter.268
Su Metzel.)
Regardless of the school setting chosen for the child, the
therapist should be able to serve the classroom teacher as a
consultant. Advice on adaptive seating and therapeutic goals
appropriate for the classroom help ensure that the rehabilita-
tion process will continue in the classroom as well as
promote optimal conditions for learning.
When a child is moving from the preschool to the elemen-
tary school setting, the support of the therapeutic team is
essential and invaluable. The teacher’s expectations, as created
by the therapist, regarding the child’s special needs and abilities
often spell the difference between success and failure of com-
plete academic and psychosocial integration within the school
setting. Even though the child may no longer require direct
therapeutic intervention, periodic consultation, including site
classroom visits, is recommended to prevent minor problems
from developing into major ones. For example, bowel and blad-
der accidents can be avoided by scheduling regular times for
toileting. The teacher may be able to make minor adjustments
in the teaching schedule to accommodate this scheduling. Also,
full-control braces (from hip to ankles) may seem overwhelm-
ing to the layperson. If the teacher is shown how the braces lock
and unlock to allow the child to sit or stand to walk, he or she
may feel more at ease if ever called on to assist the child.
The psychological perspective of the child colors thera-
peutic goals in this stage. As the child nears adolescence,
these psychosocial aspects become of paramount impor-
tance. Although the therapist should not take on the role of
Figure 15-23 n Participation in adaptive tennis. (Courtesy the psychologist, collaborative efforts in the area of counsel-
Su Metzel.) ing will be necessary. Questions will arise many times dur-
ing physical and occupational therapy sessions, requiring
factual answers that the therapist can and should provide.
Often during this stage of rehabilitation the therapist is
asked to assist in assessing cognitive function. The perceptual TRANSITIONS: ADOLESCENCE
and cognitive evaluations previously discussed may be The consequences of the physical, medical, and cognitive
administered and the results interpreted for parents and school effects of spina bifida extend into young adulthood and
personnel. have an impact on quality of life.269 Adolescence is a
stormy emotional period. Adolescents remain in turmoil as to hospitals that are nine times higher than in the nondis-
they seek their identities through sexual, social, and voca- abled population. Adults with spina bifida have medical
tional activities. As their value systems develop, they feel expenditures that are three to six times greater than those of
less ambivalence between remaining as children and striv- adults without spina bifida.169
ing for independence. For the child with myelomeningo- Although there are few standard protocols to follow in
cele, adolescence is not an optimal time to introduce new the medical management of adults with spina bifida, the
skills leading toward self-care and independence. coordinated interdisciplinary approach shown to be effective
in the care of children and adolescents is not available
TRANSITION TO ADULTHOOD to most adults with spina bifida. A recent study from the
Eighty-five percent of infants born with spina bifida will Netherlands that reviewed life span issues of people with
reach their adult years.169 For the adolescent with spina childhood-onset disabilities reported that more than 50%
bifida the transition to adulthood includes a new set of requested more information on their specific medical condi-
expectations. Independent mobility expands to driving or tion and the consequences of this condition on adulthood
arranging public transportation and getting to the correct recreation.275 In a 2009 study from the University of Pittsburgh
destination in unfamiliar surroundings, including accessibil- that surveyed 179 adults from 19 to 64 years of age, 75%
ity to the community for leisure and recreation, continued could not name their primary care physician and had not
education, or job opportunities. Self-care includes perform- seen a medical professional in over a year.169
ing ADLs but expands to household management and finan- Dicianno and colleagues169 identified five key elements
cial responsibilities. Social relationships expand to a larger necessary for the successful transition from pediatric to
arena including long-term partnerships with friends and adult medical care. These included early preparation and
business contacts and encounters with equipment vendors, education of the individual and family, flexible timing of the
insurance professionals, and medical providers and may transition, introduction to the transition clinic, interested
include hiring, firing, and directing personal health care adult center providers, and a coordinated transfer of care
assistants. Recent information from the American Journal of approach among the individual, family, pediatric primary
Public Health indicates that 50% to 70% of adults with care providers, and adult specialists. The barriers included
spina bifida live with family or in an assisted-living arrange- child health care providers refusing to “let go,” reluctance to
ment.270 For some adults with spina bifida, living indepen- leave a family-centered care program, and adult care provid-
dently in our society is a difficult goal to reach. ers having limited knowledge about or interest in caring for
The individual with spina bifida continues to require these individuals. Finding a primary care physician or phys-
assistance with management of and resources related to iatrist who can assist with identifying a team of health care
medical, rehabilitative, and social-emotional needs through specialists for referrals as needed is a major concern for this
adulthood.169,270-272 Secondary impairments span a wide adult population. The Spina Bifida Association of America
range of domains, but management of secondary health con- publishes a health guide for adults living with spina bifida,
ditions is a priority in reducing mortality, deterioration of based on feedback from adults across the United States. The
general health, and further impairments through the adult Health Guide was sponsored by a grant from the National
years. Renal, respiratory, and cardiac complications have Center for Birth Defects and Developmental Disabilities and
been identified as frequent causes of death.169 Living with the Centers for Disease Control and Prevention.
the long-term consequences of spina bifida places increased Additional unmet needs reported by adults surveyed were
demands on the musculoskeletal system, and the effects of related to functional mobility, household management, and
aging can appear earlier than usual. Osteoporosis, increased active recreation. Being independent with regard to mobility
risk of fractures, risk of osteoarthritis, and muscular pain was the most important determinant in quality-of-life
from overuse of the upper extremities with use of crutches, surveys.16,275 A review of the literature of the past few years
longer-distance wheelchair propulsion over all terrains, has indicated high unemployment rates for people with dis-
increased transitions for self-care management and routines, abilities. For those with spina bifida, 47% of adults were in
and abnormal stresses placed on the knee from weak hip competitive employment, 15% were in sheltered or sup-
abductors and calf muscles can lead to degenerative changes ported employment, and 38% were unemployed or had
and joint pain. Obesity and weight gain resulting from a never been employed.276 Limited mobility accounts for only
more sedentary lifestyle and hypertension, heart disease, part of the high unemployment rate. Accessibility into
and diabetes are common problems with aging.273 Thinner public buildings is another factor that limits employment
and less elastic skin that is susceptible to breakdown, insuf- opportunities. Tight doorways, steep ramped entrances and
ficient pressure relief and poor tissue perfusion, inconti- exits, inefficient workstations, and unreliable public trans-
nence and perspiration, wound infections after surgical portation all play a role in a lower employment rate. Univer-
procedures, burns and bumps that occur to insensate limbs, sal design (broad-spectrum solutions that produce products
and long-term immobilization during hospitalizations have and environments that are usable and effective for every-
been major sources of decreased skin integrity.169,273,274 one), construction of newer buildings with attention to
Muscular strength, flexibility, balance, and endurance adjustable work tables for computers and equipment access
decrease during the aging process. Changes in the CNS for people with different body proportions, wider doorways,
affect memory, reaction time, and attention span. An lower counters, doors that open electrically, and bathroom
increased risk of depression and anxiety has been docu- modifications (for both manual and power wheelchair users)
mented in several studies that measure quality of life in the with Americans with Disabilities Act (ADA) specifications
adult with spina bifida.169,272,275 Secondary conditions in for building modifications will improve universal access for
adults with spina bifida have been linked to admission rates all people with and without disabilities. Modifications of
bathrooms that accommodate wheelchair and crutch users delayed social and sexual growth in these adolescents:
including different height grab bars and roll-in shower (1) severity of the mental handicap, (2) poor manual dexter-
arrangements, sloping landscape for entrances and exits, and ity, (3) lack of education, (4) overprotective parents, and
room modification with lower counters and closet access, to (5) limitations in health care personnel’s ability to address
name a few considerations, may enhance travel and leisure sexuality with physically disabled patients and their fami-
time and recreational opportunities. lies.279 Either the parents or the child may bring up questions
According to the American Journal of Public Health, about sexuality. Parents of children with spina bifida realize
most adults with spina bifida use some form of assistive the need to teach their children about sexuality, but they
technology (AT) that plays a significant role in increasing often feel inadequate about doing so and are reluctant to
independence at home and in the community.272 Thirty-five bring up questions to health care professionals.280 The thera-
percent use bracing, 23% use walking devices, and 57% to pist must be open, informed, and able to provide resources
65% use lightweight wheelchairs (both manual and power to both parents and children.
assisted). PTs and OTs have extensive knowledge in the Generally, the sexual capacity of the female with spina
field of rehabilitation. Mobility equipment needs change as bifida is near normal—that is, she has potential for a normal
people age. Therapists have expertise in adaption and modi- orgasmic response, is fertile, and can bear children.281 The
fication and can recommend solutions for decreased mobil- pregnancy, however, may be considered high risk, depend-
ity. Physical changes in the workplace and home to decrease ing on existing orthopedic abnormalities. Affected males are
excessive stress on joints while maintaining flexibility and frequently sterile and have small testicles and penises. Their
musculoskeletal alignment for efficiency without pain may potential for erection and ejaculation depends on the level of
also be required. Evaluating the individual needs of the cli- the lesion. In many cases psychological problems may be a
ent and locating and selecting the types of technology that primary cause of sexual failure. Sexuality is not merely a
may enhance the adult’s personal care management and process involving the genitalia; it also depends on a positive
improve efficiency in household tasks may make the differ- body image and a feeling of self-esteem that is nurtured
ence in helping the client have a more satisfying quality of from birth.282
life. Cell phones, computer access, and watch timers for
pressure relief and personal care routines can all assist PSYCHOSOCIAL ADJUSTMENT
memory and organizational skills.272 TO CONGENITAL CORD LESIONS
AFOs with carbon springs that store energy, crutch tips The previous sections on goal setting and rehabilitation of the
that can be changed to accommodate different surfaces (e.g., child with spina bifida covered birth through adolescence.
with spikes attached for snow and ice), forearm crutches After adolescence, rehabilitation can be handled in much the
with hand grips and forearm cuffs that distribute weight and same manner as an adult spinal cord injury. Keeping in mind
reduce joint stress to shoulders and wrists, powered add-on the global effects of spina bifida on the growing child as he or
devices for manual wheelchairs to reduce stress on painful she approaches adulthood is important, however.
shoulders, adjustable furniture, and wrist rests, footrests, Because of the congenital nature of spina bifida, psycho-
and arm supports to ensure correct posture and reduce cervi- logical adjustment is somewhat different from adjustment to a
cal and lumbar strain are examples of current and experi- traumatic spinal cord injury. The psychological adjustment to
mental AT that may promote greater independence. Specific this congenital disability must be considered from the perspec-
devices are supported by the individual needs assessment of tive of the parents, the family, and, of course, the child.283
the patient by the therapist and education in the device’s A longitudinal study concerning the psychological aspects
maintenance for appropriate use and durability. It is beyond of spina bifida showed that the parents go through a series of
the scope of this chapter to discuss specific equipment items. steps in the adjustment process. From birth to approximately
Spina bifida is a congenital-onset condition that requires 6 months of age, the parents experience shock and bewilder-
intervention by the therapists from infancy through adoles- ment. Information given during this time may be rejected or
cence. Most of the impairments and functional deficits misinterpreted. Health care professionals therefore must be
described throughout this chapter last throughout a lifetime. ready to repeat the same information to parents on several
Health providers familiar with the complexity of the second- occasions during the first few years of the rehabilitation pro-
ary impairments should follow individuals with spina bifida cess. The period of 6 to 18 months of the child’s life may be
on a regular basis. Both PTs and OTs play an important role the most stressful on parents. Frequent hospitalizations during
in screening for potential problems and providing recom- this time place increased pressure on the whole family.
mendations for maintenance of mobility and health-related Parents are now able to comprehend fully the implications of
fitness. their child’s functional limitations and inability to participate
Adolescents with spina bifida show great concern about in life. They begin to worry about the future and the impact of
self-esteem and social-sexual adjustment.277 These concerns the disability on the rest of the family structure. The period
appear directly related to efficient bowel and bladder from age 2 years through the preschool years is relatively
management.278 Strategies to cope with bowel and bladder peaceful. The parents are more concerned with toilet training,
difficulties, as previously outlined, combined with appropri- social acceptability, and general information on child rearing.
ate emotional support from family and medical personnel They seem less aware of their child’s cognitive limitations
help alleviate this concern. as he or she continues to develop into a relatively happy,
Although great advances in medical management of well-adjusted child.
children with myelomeningocele have occurred, a contrast- By the age of 6 years, children are becoming more aware
ing lack of improvement related to sexual function and of their limitations and parents are concerned about prob-
reproductive issues exists. Five factors have contributed to lems that may arise as their children enter elementary
school. The child’s psychological adjustment depends on the persuaded to let go. Children with spina bifida must develop
severity of the motor problems but primarily on the attitude responsibility and independence by being given the chance
of the parents and family and on the environmental condi- to interact and even compete with their peers. During ado-
tions to which he or she is exposed.284,285 lescence, concerns regarding independent living situations
Because of their disabilities, children with spina bifida and vocational placement must be addressed. With a founda-
are often denied small tasks or chores that promote a sense tion of strong support systems fostering emotional maturity,
of responsibility in the growing child. To promote emotional the future can be bright for the child with a congenital spinal
growth and psychological well-being, caregivers must be cord injury (Case Study 15-1).
CASE STUDY 15-1 n MICHAEL

This case study focuses on the physical therapy management model for case management within the scope of occupational
of Michael, a teenage boy with myelomeningocele, a congeni- therapy. Many of the examination tools and intervention strate-
tal spinal cord injury. Michael is now 16 years old and a gies may be the same, although the objective outcome may
sophomore in high school. focus on different expectations.
A spinal cord injury is a complex disability. When a spi- NEWBORN EPISODE OF CARE
nal cord lesion exists from birth, an additional level of com- Michael was a term baby delivered by planned cesarean section
plexity is added. This congenital condition predisposes the with a prenatal diagnosis of myelomeningocele. The diagnosis
CNS to have many areas that may not develop or function was made during the second trimester after fetal ultrasound
adequately. In addition, all areas of development—physical, evaluation and amniocentesis. The family met with the neuro-
cognitive, and psychosocial—that depend heavily on central surgeon before delivery. Michael was delivered at 38 weeks
functioning will likely be impaired. The clinician therefore of gestation and was transferred at 1 day of age to the local
must be aware of the significant impact this neurological children’s hospital for a planned surgical closure of his spina
defect has on motor function as well as a variety of related bifida. An orthopedic, urological, neurosurgical, and physical
human capacities. Management can best be organized by therapy assessment occurred at 1 day of age before back clo-
using the Guide to Physical Therapist Practice. The sure. Michael was the second child for this family. His mother
following case study uses the concepts in the Guide to was diagnosed with breast cancer at his birth. His sister is
discuss several episodes of care throughout Michael’s life. 2 years older and lives at home. Both parents are professional
The Guide to Physical Therapist Practice is designed to working parents; they have a nanny to assist with child care.
provide a framework for the PT to assist in client manage- Presurgical MMT showed the presence of hip and knee
ment.1 During the infant to adolescent period of life, Michael’s musculature (L2-L4). Three days after the back closure,
specific PT needs will change. Michael’s presentation of Michael required VP shunt insertion to control hydrocepha-
congenital spinal cord dysfunction may be best represented lus. Postoperative MMT findings at 7 days of age were
through the life span by preferred practice patterns, which may identical to preoperative results. Before discharge at 8 days
include the following1: of age, the parents were instructed in ROM exercises,
n 4F: Impaired joint mobility, motor function, muscle positioning, and developmental handling. Michael would be
performance, range of motion, and reflex integrity monitored in a myelomeningocele clinic twice a year for an
associated with spinal disorders MMT and functional assessment with a team of health care
n 5C: Impaired motor function and sensory integrity providers (see Table 15-4).
associated with nonprogressive disorders of the central EPISODE OF CARE: 6-MONTH FOLLOW-UP
nervous system—congenital origin or acquired in At the age of 6 months Michael was readmitted to the hospital
infancy or childhood because of a shunt malfunction that necessitated VP shunt revi-
n 6E: Impaired ventilation and respiration or gas exchange sion. After 2 weeks at home Michael returned to the clinic for
associated with ventilatory pump dysfunction or failure postoperative follow-up. No medical concerns were present,
n 7A: Primary prevention and risk reduction for integumentary but clinicians discovered that Michael had not yet begun to roll
disorders over. An evaluation with a standardized test was conducted.
Examination (evaluation) is a comprehensive screening and The AIMS was administered as well as additional tests of
specific testing process that leads to a diagnostic classification ROM, strength (by observation), reflexes, muscle tone, and
required before intervention and is performed for all clients. endurance to establish a developmental baseline. At this visit a
It consists of three components: client history, systems review, total-body night splint was fabricated to maintain hip alignment
and tests and measures. The PT may identify impairments, and prevent contractures of the lower extremities. Family
functional limitations and disability, and changes in physical education in latex precautions was provided. The need for
function or overall health status. The PT synthesizes the find- assistive and adaptive devices was also assessed.
ings to establish a working diagnosis (ICD-9 code 741.0). At this point, Michael had not received physical therapy
Results from the evaluation are established, and interventions because of the mother’s health issues. Because of this situation,
with anticipated outcomes are made.1 Refer to Tables 15-4 to physical therapy that could be provided at home was recom-
15-10 for a detailed synopsis of Michael’s episodes of physical mended. The family was given a referral to their local EI
therapy during his 16 years. The OT should go through a simi- system and a list of local pediatric physical therapy providers
lar process of screening and testing to develop a conceptual to obtain services (see Table 15-5).
Continued
CASE STUDY 15-1 n MICHAEL—cont’d

EPISODE OF CARE: EARLY INTERVENTION EPISODE OF CARE: PREADOLESCENCE
The family contacted their local EI program after the previous (7 TO 10 YEARS OF AGE)
clinic visit. An evaluation and assessment determined Michael Michael’s physical conditioning continued to progress, and from
was eligible for services. A variety of appropriate developmental ages 7 to 10 years he participated in adaptive sports and activi-
assessments were used to determine eligibility (see Table 15-6). ties in his community. Swimming had become a favorite activity.
At the individual family service plan meeting, physical therapy inter- He had been swimming to increase strength and endurance since
vention was determined to be the primary service at this time. he was a toddler at the suggestion of his PT. Because of his
Occupational, developmental, and speech therapies were not rec- activity level and independence in most functional activities,
ommended. Michael received weekly physical therapy from age routine physical therapy had been discontinued, with only bian-
6 months through 3 years. Therapy focused on developmental nual clinic visits remaining. Michael and his family continued
exercises to promote mobility, sitting and standing balance activi- intensive stretching and a prone positioning program that they
ties, strengthening available lower-extremity musculature, and had been performing since Michael was a toddler. At his 10-year
orthotic assessment and gait training. The EI PT instructed the clinic visit, the therapist administered formal MMT and ROM
family in activities to complement the weekly physical therapy assessment and also carried out the PEDI. Scaled scores on the
program. Michael began crawling at 1 year. At 10 months he used PEDI were used because he was above the age of normed stan-
a vertical stander to initiate an upright stand position and began dards for the test. An area of skin breakdown was noted on the
gait training at 15 months. Michael began walking with AFOs left malleolus and decreased weight bearing was noted during
and a forward walker at 30 months (see Table 15-6). gait. MMT and orthopedic examinations showed no changes.
EPISODE OF CARE: EARLY CHILDHOOD A brace check noted the AFO was too small and needed to be
At age 3 years, Michael transitioned into an EC program at his local replaced. The brace was replaced and the PT reviewed skin
school. Physical therapy was a related service that was included as care and brace fit with Michael and his family (see Table 15-9).
part of his IEP. The transition meeting from EI to EC programming EPISODE OF CARE: ADOLESCENCE (AGE 13 YEARS)
provided continuity of care unique to his needs. A global assessment At age 13 years Michael had gained a significant amount of
tool to assess his mobility and self-care skills was administered. weight that was limiting endurance for long-distance ambula-
This test, the PEDI, was administered by the PT. Physical therapy tion. Michael was referred to a nutritionist for counseling. The
management included ROM, strengthening, and gait training as it PT in conjunction with the physician discussed with Michael
affected his ability to function in the preschool classroom and sur- and his parents the possibility of using a wheelchair for long-
rounding play areas. The school he attended was environmentally distance travel. After much discussion and initial resistance
appropriate for his needs. It was one level, with wide doorways that from Michael, Michael and his family decided to try a wheel-
allowed the use of his walker and then progressive use of forearm chair. At age 16 years, Michael continues to be an independent
crutches with AFOs to advance his mobility skills. At the suggestion ambulator and uses a wheelchair for long distances only. He
of his PT in the outpatient myelomeningocele clinic, Michael be- continues to be monitored on an annual basis or as needed as
came involved in a toddler swimming program at the park district. problems arise. Michael’s physical therapy has encouraged and
A tricycle was adapted at the recommendation of the same therapist continued to motivate him to maintain his participation in
to assist with strengthening his lower extremities and to allow him sports activities during his teenage years (see Table 15-10).
to efficiently keep pace with his peers (see Table 15-7). EPISODE OF CARE: ADULTHOOD
Another framework that can organize the management of Michael has graduated from high school and has been attend-
Michael’s interventions is the ICF. The ICF identifies components ing college on a large campus the past few years as a graduate
of health and contextual factors that are important for achievement student majoring in history. He works part time as a tour guide
of desired outcomes.286 Relationships between components of at the Museum of Natural History. As he drives to work, he is
health and contextual factors change over time. An example is required to transfer to and from his car on a regular basis. He
given for one episode of care in EC (Figure 15-24). needs to disassemble his chair to get it in and out of his car
At age 5.5 years, an IEP meeting was conducted before efficiently. Increased frequency of transfers to and from the car,
Michael entered kindergarten. The family and school team as well as the need to disassemble and reassemble his chair on
decided that no resources or special adaptations were necessary a regular basis, has led to an increase in shoulder pain and dis-
to enhance his education. The school he was to attend was comfort. He continues to use his crutches for short distances
determined to be environmentally appropriate to meet his needs. (150 feet) at work using a swing-through gait. He uses an ultra-
EPISODE OF CARE: TETHERED CORD EPISODE light manual wheelchair for all other mobility throughout the
(AGE 6 YEARS) day. His wheelchair is equipped with carrying loops for his
At his semiannual visit to the myelomeningocele clinic, the par- crutches at the back of his chair. Michael returned to his pri-
ents mentioned that Michael was falling frequently while using mary care physician because of his new episode of pain. He
his forearm crutches and AFOs. Reddened areas at the left lateral was referred to PT to address his issues with pain. The PT has
calcaneus were noted. Routine MMT showed decreased strength evaluated his gait and recommended that to minimize stress on
in his left leg with an increase in muscle tone through his left his shoulders he should switch from a swing-through to a four-
leg. An MRI revealed a tethered spinal cord. A neurosurgical point gait pattern (Table 15-11). He has also been referred to
TCR was performed and his AFOs were revised. Two months OT for further assessment of efficient transfer techniques to
after TCR Michael was again independent in ambulation and and from the car. In addition, a referral to an AT team has been
had progressed to stair climbing with one rail. His next routine recommended to evaluate the need for assisted power wheels as
6-month MMT showed a return of strength to the level that well as an additional means of transportation to reduce stress
preceded his tethered cord episode (see Table 15-8). on his shoulders.
TABLE 15-4 n NEWBORN EPISODE OF CARE FOR CHILD WITH SPINA BIFIDA
PRACTICE SYSTEMS ANTICIPATED GOALS

PATTERNS TO REVIEW TESTS AND RESULTS INTERVENTIONS AND EXPECTED OUTCOMES
4F Musculoskeletal MMT: No innervation Collaborate and coordinate Joint integrity and mobility
7A Integumentary below L4 systems review evaluation results improves.
6E Cardiopulmonary Muscle length: WFL with health care team. Postural control and muscle
5C Neuromuscular ROM: WFL Document impairments, functional performance improves.
Cognition and Skin integrity: WFL limitations, and strengths. Sensory awareness develops
communication HR/RR/CE: WFL Prepare a plan of treatment. appropriately.
Reflexes: Absent Therapeutic exercises to improve Risk prevention: parents under-
DTRs below knee balance, muscle strength, and stand signs and symptoms of
(1) Suck/swallow mobility. VP shunt malfunction.
(1) Galant Family training: instruction in Caregivers understand importance
Muscle tone: Flaccid below ROM; developmental positioning of checking skin for irritation
L4; low in trunk; for function and enhancement of and breakdown.
UEs WFL performance; instruction regard- Cognition and language
Motor skills: On informal ing sensory impairment and skin develops appropriately.
testing turns head, kicks, integrity and areas of pressure; Improved infant and family
clears head in prone and instruction in signs of VP sense of well-being.
Observation: Decreased pain shunt malfunction. Stressors decrease.
in LEs
CE, Cardiorespiratory endurance; DTR, deep tendon reflex; HR, heart rate; LE, lower extremity; MMT, manual muscle testing; ROM, range of motion;
RR, respiratory rate; UE, upper extremity; VP, ventriculoperitoneal; WFL, within functional limits.
TABLE 15-5 n SIX-MONTH FOLLOW-UP EPISODE OF CARE FOR CHILD WITH SPINA BIFIDA

4F Musculoskeletal MMT: No innervation Revised shunt: neurosurgery. Joint integrity and mobility
7A Integumentary below L4 Collaborate and coordinate improve.
6E Cardiopulmonary Muscle length: WFL systems review evaluation results Motor control and muscle
5C Neuromuscular ROM: WFL with health care team. performance improve.
Cognition and Skin integrity: WFL Document impairments, functional Established in physical therapy
communication HR/RR/CE: WFL limitations, and strengths. program.
Reflexes: Absent Prepare a plan of treatment. Risk prevention: parents under-
DTRs below knee Therapeutic exercises to improve stand signs and symptoms of
(1) Suck/swallow balance and mobility. VP shunt malfunction and
(1) Galant Family training: review instruction importance of checking skin
Muscle tone: Flaccid below in ROM, positioning for for irritation and breakdown.
L4; low in trunk; function, signs of shunt Latex precautions understood.
UEs WFL malfunction, and latex Sensory awareness develops
Motor skills: AIMS precautions. appropriately.
completed; could not Refer to EI program. Cognition and communication
roll over, head righting Fabricate night splint for LE develop appropriately.
emerging in prone and alignment. Improved infant and family
supported sitting Transition to vertical stander at sense of well-being.
Observation: 10 months of age. Infant and family stressors
Decreased pain in LEs decrease.
VP shunt malfunction
AIMS, Alberta Infant Motor Scale; CE, cardiorespiratory endurance; DTR, deep tendon reflex; EI, Early Intervention; HR, heart rate; LE, lower extremity;
MMT, manual muscle testing; ROM, range of motion; RR, respiratory rate; UE, upper extremity; VP, ventriculoperitoneal; WFL, within functional limits.
TABLE 15-6 n EARLY INTERVENTION EPISODE OF CARE FOR CHILD WITH SPINA BIFIDA

4F Musculoskeletal MMT: No innervation Collaborate and coordinate Joint integrity and mobility
7A Integumentary below L4 systems review evaluation results improves.
6E Cardiopulmonary Muscle length: WFL with health care team. Postural control and muscle
5C Neuromuscular ROM: WFL Document impairments, functional performance improves.
Cognition and Skin integrity: WFL limitations, and strengths. Risk prevention: parents under-
Communication HR/RR/CE: WFL Prepare IFSP; case manager estab- stand developmental exercises,
Reflexes: Absent DTRs lished physical therapy as appropriate equipment use,
below knee primary service by collaboration and importance of checking
Emerging head and trunk with developmental, occupa- for skin irritation and
righting tional, and speech therapists. breakdown.
Muscle tone: Flaccid below Therapeutic exercises to improve Sensory awareness develops
L4 low in trunk; UEs WFL balance, mobility, and strength. appropriately.
Motor skills: BSID-2 Standing with vertical stander at Cognition and language develop
administered by PT with a 10 months, gait training with appropriately.
30% delay noted; fine vertical stander and walker at Improved infant and family
motor domains of PDMS-2 15 months, and progression to sense of well-being.
administered by OT with ambulation with AFOs and Infant and family stressors
no delays noted forward walker at 30 months. decrease.
Observation: Family training: instruction in ROM Developmental, occupational,
Decreased pain in LEs and developmental activities to and speech therapists will
Hawaii Early Learning improve performance; sensory reassess at 6-month intervals
Profile (HELP) stimulation and body awareness and intervene if needed.
administered; WFL activities; skin integrity; and
for age assessment of adaptive equipment
Oral-motor, language: WFL needs (vertical stander at
10 months, AFOs to position feet
in standing, adjustment of stroller
to provide leg support, and walker
for progression of gait).
AFOs, Ankle-foot orthoses; BSID-2, Bayley Scales of Infant Development, Second Edition; CE, cardiorespiratory endurance; DTR, deep tendon reflex; HR, heart
rate; IFSP, Individual Family Service Plan; LE, lower extremity; MMT, manual muscle testing; OT, occupational therapist; PDMS-2, Peabody Developmental
Motor Scales, second edition; PT, physical therapist; ROM, range of motion; RR, respiratory rate; UE, upper extremity; WFL, within functional limits.
TABLE 15-7 n EARLY CHILDHOOD EPISODE (3 TO 5 YEARS) OF CARE FOR CHILD WITH SPINA BIFIDA

4F Musculoskeletal MMT: No innervation below L4 Collaborate and coordinate Joint and skin integrity
7A Integumentary Muscle length: WFL systems review evaluation maintained.
6E Cardiopulmonary ROM: WFL results with health care team. Postural control and muscle
5C Neuromuscular Gait: ambulates with AFOs and Document impairments, performance improves.
Cognition and walker functional limitations, and Mobility improves.
communication Skin integrity: WFL strengths. Risk prevention: parents
HR/RR/CE: WFL Improve mobility in school understand progression with
Established baseline walking environment through crutches.
distance and time with therapeutic exercise and Parents understand latex
6-minute walk test progressive gait training precautions in community
Reflexes: Absent DTRs below with crutches. environment and home.
knee Family training: instruction in Sensory awareness develops
Muscle tone: Flaccid below L4; gait with crutches. appropriately.
low in trunk; UEs WFL Health and wellness: swimming Improved child and family
Motor skills: PEDI carried out and biking in community sense of well-being.
Independent walking with walker setting. Tricycle adapted. Child and family stressors
and AFOs Educate family and Michael decrease.
ADLs are age appropriate with regarding latex precautions.
assist
School Function Assessment
(SFA) completed at 5 years
of age
Cognition: WFL
PEDI social function
section: WFL
ADLs, Activities of daily living; AFOs, ankle-foot orthoses; CE, cardiorespiratory endurance; DTR, deep tendon reflex; HR, heart rate; MMT, manual muscle
testing; PEDI, Pediatric Evaluation of Disability Inventory; RR, respiratory rate; UE, upper extremity; WFL, within functional limits.
Health Condition
Myelomeningocele
Body functions and Activities Participation

structure Walking indoors Self-care skills
Skeletal alignment Walking outdoors Independence in home
Muscle performance Up/Down stairs Early childhood
Balance Riding tricycle educational program
Cognition Swimming program
Environmental Factors Personal

School environment Self-esteem
Classroom location Peer acceptance
Features of pool Family support system
Motivation to succeed
Figure 15-24 n Michael’s early childhood episode of care as illustrated by the International
Classification of Functioning, Disability and Health model.
TABLE 15-8 n TETHERED CORD EPISODE OF CARE FOR CHILD WITH SPINA BIFIDA (AGE 6 YEARS)

4F Musculoskeletal MMT: decreased strength left leg Collaborate and coordinate Joint integrity, strength, and
7A Integumentary Muscle length: WFL systems review evaluation mobility are restored.
6E Cardiopulmonary ROM: not WFL results with physician. Gait performance improves.
5C Neuromuscular Gait: changed; weakness left side Physician ordered MRI, Risk prevention: parents under-
Cognition and Skin integrity: redness lateral surgery for release of stand signs and symptoms of
communication border of calcaneus tethered spinal cord. tethered cord.
HR/RR/CE: 6-minute walk test Document impairments, Skin integrity is maintained.
exhibits decreased pace functional limitations, and Sensory awareness develops
Reflexes: strengths. appropriately.
Hyperreflexive DTRs below L3 Prepare a plan of treatment. Cognition and language skills
on left Increase therapeutic exercises, develop appropriately.
Absent DTRs below knee on neuromuscular rehabilitation, Improved child and family
right and gait training to restore sense of well-being.
Muscle tone: increased through balance and gait with Stressors decrease.
left leg, flaccid below L4 on crutches.
right, low in trunk Family training: instruction in
UEs WFL stretching for muscle length
Motor skills: falling more often and developmental activity
Decreased ability to stand and to progress, enhance
walk; weight shifted toward performance.
right Refer to orthotist for brace
Status: unchanged adjustments.
CE, Cardiorespiratory endurance; DTR, deep tendon reflex; HR, heart rate; MMT, manual muscle testing; MRI, magnetic resonance imaging; ROM, range of
motion; RR, respiratory rate; UE, upper extremity; WFL, within functional limits.
TABLE 15-9 n PREADOLESCENT EPISODE OF CARE FOR CHILD WITH SPINA BIFIDA (AGE 10 YEARS)

4F Musculoskeletal MMT: Back to baseline; no Collaborate and coordinate Postural control and muscle
7A Integumentary innervation below L4 systems review evaluation results performance are maintained.
6E Cardiopulmonary Muscle length: WFL with health care team. Joint integrity and mobility are
5C Neuromuscular ROM: WFL Document impairments, functional maintained.
Cognition and Skin integrity: WFL limitations, and strengths. Michael understands the
communication HR/RR/CE: WFL Discharge from formal PT. importance of checking skin
Reflexes: Absent DTRs Home program to continue and for irritation and breakdown
below knee include intensive stretching and and appropriate brace fit.
Balance and equilibrium prone activities. Michael becomes active in the
appropriate for L4 spinal Continue health and wellness community adapted sports
level programs, including swimming programs.
Muscle tone: Flaccid below and community recreational Improved child and family
L4, low in trunk; programs. sense of well-being.
UEs WFL Continue to educate Michael on Client satisfaction.
Motor skills: PEDI importance of skin integrity, latex
conducted; maximal sensitivity, and brace fit.
score of 100 achieved Referred to orthotist for brace
Measured self-care, replacement.
mobility, and social
function
Observation: WFL
CE, Cardiorespiratory endurance; DTR, deep tendon reflex; HR, heart rate; MMT, manual muscle testing; PEDI, Pediatric Evaluation of Disability Inventory;
PT, physical therapy; ROM, range of motion; RR, respiratory rate; UE, upper extremity; WFL, within functional limits.
TABLE 15-10 n ADOLESCENT EPISODE OF CARE FOR CHILD WITH SPINA BIFIDA (AGE 13 YEARS)

4F Musculoskeletal MMT: No innervation below L4 Collaborate and coordinate Joint integrity and walking
7A Integumentary Muscle length: WFL systems review evaluation results mobility are maintained.
6E Cardiopulmonary ROM: WFL with health care team. Risk prevention: Michael under-
5C Neuromuscular Skin integrity: WFL Document impairments, functional stands signs and symptoms of
Cognition and Anthropometric: Increase in limitations, and strengths. decubitus pressure areas.
communication height and weight Prepare a plan of treatment. Michael’s long-distance
HR/RR/CE: Increased HR Assistive equipment, wheelchair mobility increases
and decreased distance evaluation; to be used for long independence.
traveled in 6-minute walk distances. Social and recreational
test; increased fatigue Short-distance ambulation with opportunities increase.
during long-distance forearm crutches to continue at Improved client sense of
ambulation home and school. well-being.
Reflexes: Absent DTRs below Client education: weight shifts Stressors decrease.
knee every hour while using
Muscle tone: LEs no change; wheelchair for skin integrity.
UEs WFL Refer to nutritionist.
Motor skills: Decreased abil- Design a plan to incorporate
ity to keep pace with peers driver’s education through high
Observation: school as well as rehabilitation
Increased social abilities resources.
RR, respiratory rate; UE, upper extremity; WFL, within functional limits.
TABLE 15-11 n EPISODE OF CARE FOR ADULT WITH SPINA BIFIDA (AGE 25 YEARS)

4F Musculoskeletal MMT: No innervation Collaborate and coordinate Joint integrity and walking
7A Integumentary below L4 systems review evaluation results mobility are maintained.
6E Cardiopulmonary Muscle length: WFL with health care team. Risk prevention: Michael under-
5C Neuromuscular ROM: decreased in shoulder Document impairments, functional stands signs and symptoms of
Cognition and WFL LE limitations, and strengths. joint overuse.
communication Skin integrity: WFL Prepare a plan of treatment. Michael decreases stressful
Anthropometrics: Increase Assistive equipment, wheelchair walking and increases
in height and weight modification to decrease shoulder independence.
HR/RR/CE: Increased HR stress for long distances. Social and recreational
and decreased distance Short-distance gait training four- opportunities increase.
traveled in 6-minute walk point with forearm crutches to Improved client sense of
test; shoulder pain during continue home and community well-being.
short distance ambulation, ambulation. Stressors decrease.
fatigue with longer Incorporate flexibility and strength
distances training.
Reflexes: Absent DTRs Client education: reduce shoulder
below knee stress with efficient body
Muscle tone: LEs, no mechanics.
change; UEs WFL Refer to occupational therapy.
Motor skills: Decreased Design a plan to incorporate
ability to keep pace with efficient transfer of wheelchair
peers to car and rehabilitation
Observation: Painful right resources.
shoulder during walking
and transfer of wheelchair
into vehicle for transport
RR, respiratory rate; UE, upper extremity; WFL, within functional limits.
Acknowledgment
APPENDIX 15-A n ORLAU Swivel Walker
We dedicate this chapter to Jane W. Schneider and all the
Distributors
children who have taught us so much.
United States
References Mopac Ltd
To enhance this text and add value for the reader, all refer- 206 Chestnut Street
ences are included on the companion Evolve site that ac- Eau Claire, WI 54703
companies this textbook. This online service will, when (715) 832-1685
available, provide a link for the reader to a Medline abstract
for the article cited. There are 286 cited references and other United Kingdom
general references for this chapter, with the majority of J. Stallard, Technical Director
those articles being evidence-based citations. Oswestry Orthopaedic Hospital
Shropshire, SY107AG
UK
CHAPTER 16 Traumatic Spinal Cord Injury
MYRTICE B. ATRICE, PT, BS, SARAH A. MORRISON, PT, BS,
SHARI L. McDOWELL, PT, BS, PAULA M. ACKERMAN, MS, OTR/L,
TERESA A. FOY, OT, BS, and CANDY TEFERTILLER, DPT, ATP, NCS

American Spinal Injury Association (ASIA) After reading this chapter the student or therapist will be able to:
autonomic dysfunction 1. Describe the demographics, etiology, and mechanism of injury of spinal cord injury.
autonomic dysreflexia 2. Discuss the acute medical management of person with spinal cord injury.
body-weight–supported treadmill (BWST) 3. Describe the secondary complications of spinal cord injury, the appropriate
bulbocavernosus reflex interventions, and the impact of complications on the rehabilitation process.
complete lesion 4. Identify the basic components of the examination process.
deep vein thrombosis (DVT) 5. Identify patient problems based on the examination, establish appropriate goals,
discomplete and plan individualized treatment programs for patients with a spinal cord injury.
Functional Independence Measure (FIM) 6. Describe adaptive equipment available to increase function.
incomplete lesion 7. Discuss progression of each individual and the process of discharge planning
intermittent catheterization throughout the rehabilitation process.
locomotor training 8. Describe functional expectations for individuals with complete spinal cord injuries.
lower motor neuron 9. Identify equipment needs for a given spinal cord injury lesion.
mobile arm support (MAS) 10. Describe various aspects of activity-based therapies to promote recovery after spinal
neuroprosthetics cord injury.
offset feeder
orthostatic hypotension
paraplegia
pressure ulcer
pulmonary embolism (PE)
spinal cord injury (SCI)
spinal shock
tenodesis
tetraplegia
upper motor neuron
S pinal cord injury (SCI) is a catastrophic condition that,

depending on its severity, may cause dramatic changes
in a person’s life. SCI usually happens to active, indepen-
pathologist, dietician, assistive technologist, respiratory
care practitioner, psychologist, social worker, vocational
counselor, rehabilitation engineer, and chaplain.3-5 The
dent people who at one moment are in control of their lives most important element determining success in any reha-
and in the next moment are paralyzed, with loss of sensation bilitation program is the patient’s and family’s active
and loss of bodily functions, which can lead to dependence participation throughout the rehabilitation process.
on others for even the most basic needs. To reduce negative This chapter provides a general overview for the man-
impact, individuals with SCI need a well-coordinated, spe- agement of individuals with SCI throughout inpatient and
cialized rehabilitation program to assist them in maximizing postacute phases of the rehabilitation continuum. The in-
the development of skills necessary to live a satisfying and formation is intended to aid health care professionals in the
productive postinjury life.1,2 treatment of individuals with SCI by providing guidelines
A successful rehabilitation program requires a team of to maximize each individual’s return to their preinjury
health care professionals who work in unison to address lifestyle.
alterations in body function, increase the individual’s
independence in all daily activities, and return the indi- SPINAL CORD LESIONS
vidual to the highest level of community participation SCI occurs when the spinal cord is damaged as a result of
specific to that individual’s life situations. Minimally, the trauma, disease processes, vascular compromise, or con-
team should include a physician, case manager, occupa- genital neural tube defect. The clinical manifestations of
tional therapist, physical therapist, therapeutic recreation the injury vary depending on the extent and location of the
specialist, prosthetist or orthotist, nurse, speech-language damage to the spinal cord.
459
Tetraplegia standards, any sensation in the anal mucocutaneous junc-

Tetraplegia (preferred to quadriplegia) refers to impairment tion, or deep anal sensation, indicates that the lesion is
or loss of motor and/or sensory function as a result of dam- incomplete. If only sensation is preserved, the injury is
age to the cervical segments of the spinal cord. Function classified as AIS B. If motor function in key muscles
in the upper extremities, lower extremities, and trunk is is maintained to some degree, patients may achieve level C,
affected. It does not include brachial plexus lesions or injury D, or E classification. This testing will be reviewed further
to peripheral nerves outside the neural canal.6 in this chapter.6,12
Paraplegia DEMOGRAPHICS
Paraplegia refers to impairment or loss of motor or sensory The incidence of traumatic SCI in the United States is ap-
function as a result of damage to the thoracic, lumbar, or proximately 12,000 new cases per year.13 Approximately
sacral segments of the spinal cord. Depending on the level 3000 new cases of spinal cord impairment resulting from
of the damage, function may be impaired in the trunk and/or disease and congenital anomalies occur each year. The num-
lower extremities. This term is used to refer to cauda equina ber of people living in the United States today with SCI is
and conus medullaris injuries but not to lumbosacral plexus between 231,000 and 311,000.13 Fifty-three percent of trau-
lesions or injury to peripheral nerves, which are considered matic SCIs occur in persons aged 16 to 30 years. However,
outside of the central nervous system.6 the median age of the general population of the United States
has increased by 8 years since the mid 1970s, and the average
Complete, Discomplete, and Incomplete age of the SCI population has steadily increased. Since 2005,
Lesions the mean age at the time of injury is 40.2 years.13,14 Persons
In a complete lesion, sensory and motor function in the older than 60 years of age at injury have increased from 4.7%
lowest sacral segments (S4-S5) is absent postinjury.6 The before 1980 to 11.5% for injuries occurring since 2000. This
American Spinal Injury Association (ASIA) classification trend explains the increase in the median age during this
for this type of injury is ASIA Impairment Scale (AIS) A. same time period from 27.9 years to 35.3 years. Table 16-1
Complete injuries to the spinal cord are usually the result lists additional demographics.
of extensive trauma or disease and are often segmentally In 2005 the average length of inpatient stay was 50 days
associated with damage to the nerve roots in the interverte- (12 days in an acute-care facility and 38 days in rehabilita-
bral foramina.7 Function of the roots originating from the tion). The average yearly health care and living expenses
more cranial portion of the intact cord can be expected to
return within 6 months.7
Discomplete injury is a relatively new term in SCI TABLE 16-1 n SPINAL CORD INJURY
research and practice. It is defined as a lesion that is “clini- DEMOGRAPHICS
cally complete but which is accompanied by neurophysio-
logical evidence of residual brain influence on spinal cord Mean age at injury 40.2 years
function below the level of the lesion.”8 Studies of persons Most common age at injury 19.0 years
whose spinal cord injuries were considered complete under SEX
ASIA standards have shown that in a large percentage Male 80.9%
(84%) there was residual brain influence on the spinal cord Female 19.1%
below the level of the lesion.8,9 The current gold standard CAUSES OF INJURY
for testing, the AIS, is unable to detect this residual func- Motor vehicle accident 41.3%
tion, which suggests that AIS testing may be providing an Falls 27.3%
inaccurate picture of the patients’ neurological plasticity Violent acts 15.0%
and recovery potential. The Brain Motor Control Assess- Sports injuries 7.9%
ment (BMCA) is emerging as a desirable adjunct to the Other 8.5%
standard ASIA testing.9 In the BMCA, surface electromy-
ography (EMG) is used to quantify the motor unit activity NEUROLOGICAL CATEGORIES
of the lower extremities in response to a standard testing AT DISCHARGE
protocol including active and passive movement of the Incomplete tetraplegia 38.3%
lower extremities, reinforcement maneuvers (e.g., Jendrassik Complete paraplegia 22.9%
or Valsalva) performed above the level of injury, tendon Incomplete paraplegia 21.5%
taps and vibration, and elicitation and suppression of reflex Complete tetraplegia 16.9%
activity. The motor unit responses are quantified and com- No deficits 0.7%
pared with normative data to establish a voluntary response COMMON INJURY SITES64
index and a similarity index. In other words, the results of C5 14.9%
the BMCA help to determine how different the subjects’ C4 13.6%
motor responses are from those of persons with intact neu- C6 10.8%
rological systems.8-11 This testing requires specialty equip- T12 6.7%
ment but can easily be administered by physical therapists C7 5.3%
once they have received the appropriate training. Data from National Spinal Cord Injury Statistical Center: Spinal cord in-
With incomplete lesions there is detectable residual sen- jury: facts and figures at a glance, February 2010, Birmingham, AL, 2010,
sory or motor function below the neurological level and University of Alabama, National Spinal Cord Injury Statistical Center.
specifically in the lowest sacral segment. According to ASIA Available at www.uab.edu/NSCICSC.
CHAPTER 16 n Traumatic Spinal Cord Injury 461
vary according to severity of injury. In the first year, indi- damage to neural and other soft tissues as well as severe
viduals with high tetraplegia spend $829,843, whereas indi- hemorrhaging in the surrounding gray and white matter, re-
viduals with paraplegia spend an average of $303,220.13 sulting in immediate cell death.20,21 In the next few minutes
Today 87.7% of persons with SCI are discharged to a non- after the insult, injured nerve cells respond with trauma-
institutional residence. Life expectancies for patients with induced action potentials, which lead to increased levels of
SCI continue to increase but are still below the national intracellular sodium. The result of this influx is an increase
average of persons without SCI. Mortality rates are signifi- in osmotic pressure movement of water into the area.
cantly higher during the first year after injury, especially Edema generally develops in up to three levels above and
for severely injured persons. According to the National SCI below the original insult and leads to further tissue decon-
Database, the leading causes of death after an SCI are pneu- struction.19,21,22 Increased levels of extracellular potassium
monia, pulmonary emboli, and septicemia.13 and intracellular concentrations of calcium also result in an
Statistics suggest a high incidence of multiple trauma electrolyte imbalance that contributes to a toxic environ-
associated with a traumatic SCI (55.2%).15 The most comment.23-25 Abnormal concentrations of calcium within the
mon injuries are fractures (29.3%) and loss of conscious- damaged cells disrupt their functioning and cause break-
ness (28.2%).15 Traumatic pneumothorax or hemothorax down of protein and phospholipids, leading to demyelin-
are reported in 17.8% of persons with SCI. Traumatic head ation and destruction of the cell membrane.25 The cascade
injuries of sufficient severity to affect cognitive or emo- of these events consequentially contributes to a dysfunc-
tional functioning are reported in 11.5% of all cases.15 Skull tional nervous system.
and facial fractures, along with traumatic head injuries and During this acute phase, evidence of spinal shock may be
vertebral artery and esophageal disruptions, are common in present. Spinal shock occurs 30 to 60 minutes after spinal
cervical injuries.16 Limb fractures and intrathoracic injuries trauma and is characterized by flaccid paralysis and absence
(rib fractures and hemopneumothorax) are frequent in tho- of all spinal cord reflex activity below the level of the spinal
racic injuries, whereas intraabdominal injuries to the liver, cord lesion.26,27 This condition lasts for about 24 hours after
spleen, and kidneys are associated with lumbar and cauda injury, represents a generalized failure of circuitry in the
equina injuries.16 spinal neural network, and is thought to be directly related
to a conduction block resulting from leakage of potassium
SEQUELAE OF TRAUMATIC SPINAL CORD into the extracellular matrix.28 The completeness of the lesion
INJURY cannot be determined until spinal shock is resolved. The
As stated previously, most spinal cord injuries occur as a re- signs of spinal shock resolution are controversial; however,
sult of trauma, be it motor vehicle accidents, falls, violence, the return of reflexes may be a good indication.
or sports-related injury. The degree and type of forces that are The secondary phase of the injury occurs within the
exerted on the spine at the time of the trauma determine the course of minutes to weeks after the acute process and is
location and severity of damage to the spinal cord.17 Injuries characterized by the continuation of ischemic cellular death,
to the vertebral column can be classified biomechanically as electrolytic shifts, and edema. Extracellular concentrations
flexion or flexion-rotation injuries, hyperextension injuries, of glutamate and other excitatory amino acids reach concen-
and compression injuries.18 Penetrating injuries to the cord trations that are six to eight times greater than normal within
are usually the result of gunshot or knife wounds.18 the first 15 minutes after an injury.24 In addition, lipid per-
Spinal cord damage can also be caused by nontraumatic oxidation and free radical production also occur.29 Apoptosis
mechanisms. Circulatory compromise to the spinal cord (a secondary programmable cell death) occurs and involves
resulting in ischemia causes neurological damage at and reactive gliosis. There is also an important immune response
below the involved cord level. This can be caused by a that adds to the secondary damage that may be a result of
thrombus, swelling, compression, or vascular malforma- a damaged blood-brain barrier, microglial activation, and
tions and dysfunction. Degenerative bone diseases can increased local concentrations of cytokines and chemo-
cause compression of the spinal cord by creating a stenosis kines.30 The lesion enlarges from the initial core of cell
of the spinal canal and intervertebral foramina. Stenosis death, expanding from the perilesional region to a larger
can also result from the prolapse of the intervertebral disc region of cell loss.
into the neural canal. The encroachment of tumors or ab- In the chronic phase, which occurs over a period of days
scesses within the spinal cord, the spinal canal, or the to years, apoptosis continues both rostrally and caudally.
surrounding tissues can also lead to SCI. Congenital mal- Receptors and ion channels are altered, and with penetrating
formation of the spinal structures, as in spina bifida, can injuries scarring and tethering of the cord occurs. Conduc-
also compromise the spinal cord and its protective layers tion deficits persist owing to demyelination, and permanent
of connective tissue. Some of the more common diseases hyperexcitability develops with consequential chronic pain
and conditions that result in compromise of the spinal cord syndromes and spasticity in many SCI patients.26 Changes
include Guillain-Barré syndrome, transverse myelitis, amy- in neural circuits result from alterations in excitatory and
otrophic lateral sclerosis, and multiple sclerosis.12 inhibitory inputs, and axons may exhibit regenerative and
After the spinal cord has sustained damage, cellular sprouting responses but go no farther than 1 mm.24
events occur in response to the injury and are classified in Medical interventions are evolving to limit the impact of
three phases of progression: acute, secondary, and chronic the acute SCI and the subsequent progression that follows.
responses. The acute process begins on occurrence of an Growing interest in protection and repair of the injured ner-
injury and continues for 3 to 5 days.19 Abrupt necrosis or vous system has led to an improved understanding of the
cell death can result from both mechanical and ischemic pathophysiology associated with SCI and has resulted in
events. The impact of an SCI often causes direct mechanical the implementation of several therapeutic strategies that are
currently being investigated in phase 1 and 2 clinical trials. CLINICAL SYNDROMES

The effects of methylprednisolone sodium succinate, tiril- Some incomplete lesions have a distinct clinical picture
izad mesylate, monosialotetrahexosylganglioside, thyrotropin- with specific signs and symptoms. An understanding of the
releasing hormone, gacyclidine, naloxone, and nimodipine various syndromes can be helpful to the patient’s team in
have all been examined in randomized controlled trials planning the rehabilitation program. Figure 16-1 depicts
over the last few years. Although the primary outcomes the anatomy of the spinal cord.30,31 This basic anatomy of
in these studies did not demonstrate statistically significant the spinal cord can be referred to as the various syndromes
effects, a secondary analysis demonstrated that methylpred- are described.
nisolone sodium succinate given within 8 hours of injury
was associated with modest clinical benefits.17 Phase 2 trials Central Cord Syndrome
with monosialotetrahexosylganglioside and thyrotropin- Hyperextension injuries usually result in a central cord
releasing hormone also yielded some therapeutic benefits, syndrome.31 This injury causes bleeding into the central
but further studies need to be completed to determine effi- gray matter of the spinal cord, resulting in more impairment
cacy. Several current or planned studies exist to evaluate of function in the upper extremities than in the lower
the potential benefits of early surgical decompression and extremities.31 Most incomplete lesions result in this syn-
electrical field stimulation, neuroprotective strategies such drome, especially in elderly individuals when cervical
as riluzole and minocycline, the inactivation of myelin inhi- stenosis is present.30 Although the prognosis for functional
bition by blocking Nogo and Rho, and the transplantation recovery is good for individuals with central cord syn-
of various substrates into the injured spinal cord.17 Promis- drome, the pattern of recovery is such that intrinsic hand
ing clinical trials are also underway to minimize the second- function is the last thing to return. Approximately 77% of
ary phase of injury and to promote healing and neuronal clients with central cord syndrome will attain some level of
regeneration (Table 16-2). If medical interventions can ambulatory function, 53% bowel and bladder control, and
bridge the central lesion or limit the secondary progression, 42% hand function.12,32,33
the functional loss that follows SCI will be minimized and
chances of recovery improved. Anterior Spinal Artery Syndrome
Anterior spinal artery syndrome is usually caused by flex-
ion injuries in which bone or cartilage spicules compro-
mise the anterior spinal artery.31 Motor function and pain
TABLE 16-2 n PHASES OF INJURY TABLE and temperature sensation are lost bilaterally below the
injured segment.31 The prognosis is extremely poor for
return of bowel and bladder function, hand function, and
PHASE DESCRIPTION
ambulation.12,33
Acute Systemic hypotension and spinal shock
Hemorrhage Brown-Séquard Syndrome
Cell death from direct insult or ischemia Occasionally, as a result of penetrating injuries (gunshot or
Edema stab wounds), only one half of the spinal cord is damaged.
Vasospasm The Brown-Séquard syndrome is characterized by ipsilat-
Shifts in electrolytes eral loss of motor function and position sense and contralat-
Accumulation of neurotransmitters eral loss of pain sensation several levels below the lesion.31
Induced hypothermic treatment The prognosis for recovery is good. Nearly all clients attain
Secondary Continued cell death some level of ambulatory function, 80% regain hand func-
Continued edema tion, 100% have bladder control, and 80% have bowel
Continued shifts in electrolytes control.12,33
Free-radical production
Lipid peroxidation Posterior Cord Syndrome
Neutrophil and lymphocyte invasion and release Posterior cord syndrome is rare, resulting from compres-
of cytokines sion by tumor or infarction of the posterior spinal artery.
Apoptosis Clinically, proprioception, stereognosis, two-point discrim-
Calcium entry into cells ination, and vibration sense are lost below the level of the
Chronic Continued apoptosis radiating from site of injury lesion.31
Alteration of ion channels and receptors
Formation of fluid-filled cavity
Scarring of spinal cord by glial cells
Demyelination Posterior columns (position sense)
Regenerative processes, including sprouting by Lower limb }
neurons Trunk } Lateral pyramidal tract
Upper limb } (motor)
Altered neurocircuits
Lateral spinothalamic
Syringomyelia Lower limb } tract (pain and temperature,
Trunk } crosses from opposite side
Data from Hulsebosch CE: Recent advances in pathophysiology and treat- Upper limb } before ascending)
ment of spinal cord injury. Adv Physiol Educ 26:238–255, 2002; and
Anterior spinal artery
Sekhon LH, Fehlings MG: Epidemiology, demographics, and pathophysi-
ology of acute spinal cord injury. Spine 26(24 suppl):S2–S12, 2001. Figure 16-1 n Cross-sectional anatomy of the spinal cord.
Cauda Equina Syndrome

Damage to the cauda equina occurs with injuries at or be-
low the L1 vertebral level. This syndrome results in a lower
motor neuron lesion that is usually incomplete. This lesion
results in flaccid paralysis with no spinal reflex activity
present.12,26
Conus Medullaris Syndrome

Injury of the sacral cord and lumbar nerve roots within the
neural canal results in a clinical picture of lower-extremity
motor and sensory loss and areflexic bladder and bowel.12,31
MEDICAL MANAGEMENT
Short-term medical treatment includes anatomical realign-
ment and stabilization interventions and pharmacological
management to prevent further neurological trauma and Figure 16-2 n Gardner-Wells tongs. Reduction is accomplished
enhance neural recovery. through weights attached to the traction rope. (Courtesy Dr. H.
Herndon Murray, Assistant Medical Director, Shepherd Spinal
Surgical Stabilization Center, Atlanta, Georgia.)
One of the first interventions after acute traumatic SCI is to
stabilize the spine to prevent further cord or nerve root dam-
age. In the emergency department, diagnostic studies reveal
the severity of the spinal injury and the type and degree of After cervical surgical stabilization, a hard collar such as a
the instability. On the basis of these findings, the physician, Philadelphia collar (Figure 16-4) or sternal-occipital-mandibular
client, and family decide on treatment. Many options must immobilizer (SOMI) brace is used until solid bony fusion
be considered regarding the optimal operative strategy. Indi- has developed. The Aspen collar also provides this stability
cations for surgical intervention include, but are not limited (Figure 16-5). The solid bony fusion usually takes 6 to
to, signs of progressive neurological involvement, type and 8 weeks. Postoperatively, care must be taken to protect the
extent of bony lesions, and degree of spinal cord damage.34 bony fusion.
The following discussion describes nonsurgical and surgical When surgery is not indicated, or when more postoperative
interventions. stabilization is required, halo traction may be indicated. The
halo device restricts more movement in the upper cervical
Cervical Spine spine compared with the lower cervical spine.38 The halo trac-
At the scene of the accident, emergency medical profes- tion device consists of three parts: the ring, the uprights, and
sionals exercise extreme caution to immobilize the injured the jacket (Figure 16-6). The ring fits around the skull, just
patient and prevent excessive movement. If there is com- above the ears. It is held in place by four pins that are inserted
pression of neurological tissue, vertebral fracture, or dislo- into the skull. The uprights are attached to the ring and jacket
cation, reduction must occur to minimize ischemia and by bolts. The jacket is usually made of polypropylene and
edema formation.35 In the emergency department, reduc- lined with sheepskin. This equipment is left in place for 6 to
tion is accomplished by cervical traction with the goal of 12 weeks until bony healing is satisfactory.5 The advantage of
immediate and proper alignment of bone fragments and using the halo device is the ability to mobilize the client as
decompression of the spinal cord until further stabiliza- soon as the device has been applied without compromising
tion.34,36,37 The most widely used traction method is the spinal alignment. This allows the rehabilitation program to
Gardner-Wells tongs (Figure 16-2), which are inserted into commence more rapidly. It also allows for delayed decision
the skull. Weights are added at approximately 5 pounds making regarding the need for surgery.
of traction per level of injury to achieve reduction of the The disadvantage of the halo device is that pressure and
dislocation and to maintain alignment.36 friction from the vest or jacket may lead to altered skin in-
Precautions must be taken during therapy to prevent un- tegrity.7 Special attention must be given to ensure the skin
necessary movement at the injury site. The traction rope must remains intact. During more active phases of the rehabilita-
be kept in alignment with the long axis of the cervical spine, tion process, the halo device may slow functional progress
and the weights must be allowed to hang freely. Cervical because of added weight and interference with the middle
rotation must be prevented. In addition, continued traction to end range of upper-extremity movement. In a small per-
should be maintained at all times. centage of patients, there are complications of dysphagia
When surgical stabilization is indicated, common surgi- and temporomandibular joint dysfunctions associated with
cal protocols include posterior and anterior approaches. wearing the halo device.7
Figure 16-3 shows radiographs of a person who had an ante-
rior and lateral cervical fusion at C3-C4. Unstable compres- Thoracolumbar Spine
sion injuries are usually managed by a posterior procedure Internal fixation of the thoracolumbar region is necessary
except when there is a deficient anterior column. Anterior ap- when stability and distraction cannot be maintained by other
proaches are indicated for patients with evidence of residual means.39 Common thoracic stabilization procedures include
anterior spinal cord or nerve root compression and persistent transpedicular screws (Figure 16-7) and a hybrid type of
neurological deficits.34 instrumentation.
A B
Figure 16-3 n A, Radiograph of person who had an anterior cervical fusion at C3-C4. B, Lateral
radiologic view of anterior fusion C3-C4.
Figure 16-4 n Philadelphia collar. It is fabricated of polyethyl- Figure 16-5 n The Aspen collar (formerly known as the Newport
ene foam with rigid anterior and posterior plastic strips, it is easily collar) encircles the neck, is somewhat open, and provides cervical
applied via Velcro closures, and it limits flexion, extension, and motion restriction. It is rigid yet flexible at its edges to conform to
rotary movements of the cervical spine. each patient’s anatomy. Pads and shells are removable and washable.
Figure 16-7 n Radiograph of transpedicular screws. (Courtesy

Dr. H. Herndon Murray, Assistant Medical Director, Shepherd
Spinal Center, Atlanta, Georgia.)
(GM1). To date, two major pharmacological clinical trials have

Figure 16-6 n Halo vest. Basic components are the halo ring, been completed.
distraction rods, and jacket (jacket not pictured). The National Acute Spinal Cord Injury Study41 (NASCIS-2)
used high doses of methylprednisolone and showed signifi-
cant improvements in sensory and motor function 6 months
Postoperatively, an external trunk support may be neces- after injury.42 Young and Flamm43 showed that methylpred-
sary to limit excessive vertebral motion and to maintain nisolone enhanced the flow of blood to the injured spinal
proper thoracic and lumbar alignment.39 This may be achieved cord, preventing the typical decline in white matter, extracel-
by a custom thoracolumbosacral orthosis (Figure 16-8) or a lular calcium levels, and evoked potentials, thus preventing
Jewett brace (Figure 16-9). Initially the client’s activity may progressive posttraumatic ischemia.44-47 The dosage recom-
be limited to allow for a complete fusion to take place and to mended by the NASCIS-2 study is 30 mg/kg of methylpred-
minimize the possibility of rod displacement. All spinal limi- nisolone followed by an infusion of 5.4 mg/kg/hr for 23 hours.41
tations should be discussed with the surgeon postoperatively. The therapist must be aware of side effects that may occur
The goals of the operative procedures at any spinal level with such high doses of steroids, including gastric ulcers,
discussed are to reverse the deforming forces, to restore decreased wound-healing time, hypertension, cardiac arrhyth-
proper spinal alignment, and to stabilize the spine.40 All mias, and alteration in mental status.42
these procedures have advantages and disadvantages. The
surgeon, client, and family must be involved in the decision- THERAPEUTIC REHABILITATION
making process to select the most appropriate method CONTINUUM OF CARE
of treatment. This will allow the therapeutic rehabilitation Therapeutic rehabilitation can be effectively delivered
process to begin. beginning in an acute-care setting at the time of injury and
continuing on through a lifetime of care. Rehabilitation
Pharmacological Management Immediately teams may use one of three models: multidisciplinary,
after Traumatic Spinal Cord Injury interdisciplinary, and transdisciplinary.3 The standards set
Neurological damage from SCI may be a result of (1) physical forth by the Commission on Accreditation of Rehabilita-
disruption of axons traversing the injury site, or (2) as de- tion Facilities (CARF) suggest that the interdisciplinary
scribed earlier, cellular events that follow the primary injury. model of team structure is optimal in the rehabilitation
Investigators believe that secondary injuries to surrounding setting.31
tissues can be lessened by pharmacological agents, specifically The continuum of care may be divided into several phases
methylprednisolone and monosialotetrahexosylganglioside that include medical management (previously described),
Figure 16-9 n Jewett hyperextension brace. A single three-point

force system is provided by sternal pad, suprapubic pad, and thoraco-
lumbar pad. Forward flexion is restricted in the thoracolumbar area.
Figure 16-8 n Custom thoracolumbosacral orthosis. This molded

plastic body orthosis has a soft lining. It controls flexion, extension, As the acute phase progresses, out-of-bed activities are
and rotary movements until healing of the bone has occurred. tolerated for longer periods of time and the patient begins
to work toward specific long-term goals. In accordance
with Medicare guidelines for inpatient rehabilitation, the
inpatient rehabilitation, outpatient rehabilitation, and home client is able to participate in therapeutic programs a mini-
health. The continuum also includes returning the patient mum of 3 hours a day.48,49 The intensity of therapy may
into wellness programs and community reentry outreach continue to be limited according to unresolved medical
programs. The progression of a patient through the rehabili- issues.
tation process will vary greatly from one person to the next. As medical issues resolve and endurance improves, the
The patient may also move back and forth throughout the patient will progress to a higher and more active level of
continuum of care. participation. During inpatient rehabilitation, the patient
gains varying levels of independence in specific skills. The
Inpatient Rehabilitation patient may be taught advanced skills to perform activities
Inpatient rehabilitation begins during the critical and acute- of daily living (ADLs), transfers, and mobility. Commu-
care stages after an SCI. The primary emphasis of early nity outings may be scheduled to refine advanced skills,
rehabilitation is to lessen the adverse effects of neu- identify further needs, and foster community reintegration
rotrauma and immobilization. This focus may last from a and participation. In addition, the following will be com-
few days to several weeks, depending on the severity and pleted unless otherwise indicated by the rehabilitation
level of injury and other associated injuries. Although team: (1) family training, (2) home and school or work
therapeutic intensity may be limited, clients may begin evaluation, (3) delivery and fitting of discharge equipment,
participating in early therapy that should include, but (4) instruction in home management, (5) instruction in
should not be limited to, out-of-bed activities, gaining up- home exercise programs, (6) dependent passenger driving
right tolerance, range-of-motion (ROM) exercises, early evaluations, (7) assistive technology (AT) referrals for de-
strength training, and skin-management education. Goals vices to enable computer access and other electronic aids
during this phase should focus on prevention of secondary to daily living (EADLs), (8) referrals for continued ser-
complications and preparing the client for full rehabilita- vices, and (9) driving evaluation. Discharge planning
tion participation. The treatment team must begin dis- largely encompasses activities aimed at a smooth transition
charge planning and family training in this phase. back into the community whenever possible.
Outpatient Rehabilitation and Community history of current condition if applicable, medical and surgi-
Reentry cal history, family history, reported patient and family health
Discharge from an inpatient rehabilitation program marks status, and social habits.52 If the history suggests a loss of
only the beginning of the lifelong process of adjustment to consciousness or brain injury, the clinician should consider
changes in physical abilities, community reintegration, and the possibility of compromised cognition and should include
participation in life activities. Inpatient rehabilitation pro- tests and measures during the examination and assessment
vides an environment best suited for learning self-care appropriate to that impairment.
skills, yet “the implications of living in the community with
SCI can scarcely be anticipated accurately by the newly in- Systems Review
jured individual or the able-bodied staff.”50 Because of the The physiological and anatomical status should be reviewed
shortened lengths of hospitalization, services provided after for the cardiopulmonary, integumentary, musculoskeletal,
discharge are becoming increasingly important. A direct and neuromuscular systems. In addition, communication,
consequence of this shift results in outpatient treatment of affect, cognition, language, and learning style should be
patients who have more acuity, greater care needs, and fewer reviewed.51
skills attained in the inpatient rehabilitation program before
entry into the outpatient arena. Common outpatient therapy Tests and Measures
treatment programs have included advanced transfer train- Depending on the data generated during the history and
ing, advanced wheelchair mobility training, locomotor train- systems review, the clinician performs tests and measures to
ing, upgraded ADL training, and upgraded home exercise help identify impairments, activity limitations, and participa-
program instruction. This is a shift in the typical program tion restrictions and to establish the diagnosis and prognosis
structure because these skills were traditionally a part of the of each client. Tests and measures that are often used for
inpatient rehabilitation. persons with SCI are included in Box 16-1. For more detail
Services provided after inpatient discharge may include related to specific tools, refer to the Guide to Physical
day programs, single-service outpatient visits, wellness Therapist Practice.52
programs, and routine follow-up visits and services. The
“day program” concept has emerged to meet the demand
Neurological Examination
for more comprehensive rehabilitation services. The pri-
mary purpose of these services is to provide a coordinated American Spinal Cord Injury Association
effort for the client to return to full reintegration into the Examination
community. There is a variety of day program options for It is recommended that the international standards of ASIA
individuals, with each program offering various levels of be used for the specific neurological examination after an
care that range from two coordinated disciplines to services SCI.53 See Figure 16-10 for the ASIA motor and sensory
like those of an inpatient rehabilitation program. One com- examination form. Assessment of muscle performance al-
mon thread for virtually all day program settings is that the lows for specific diagnosis of the level and completeness of
clients are medically stable, do not require skilled nursing injury. The examination of muscle performance includes
services during the night, and need a coordinated approach each specific muscle and identifies substitutions from other
for two or more services with the focus on performance of muscles.
functional skills and on the transference of these skills into
the community.
EXAMINATION AND EVALUATION BOX 16-1 n TESTS AND MEASURES52

OF BODY FUNCTION AND STRUCTURE
Regardless of where the patient begins the rehabilitation Aerobic capacity and endurance
process, an examination is completed on admission. The Anthropometric characteristics
examination and evaluation will assist in establishing the Assistive and adaptive devices assessment
diagnosis and the prognosis of each patient as well as de- Community and work integration or reintegration
termining the appropriate therapeutic interventions. The Environmental home and work barriers examination
client and caregivers participate by reporting activity per- Gait, locomotion, and balance
formance and functional ability.51 Any pertinent additions Integumentary integrity
to the history stated by the client should be described. The Joint integrity and mobility
client’s statement of goals, problems, and concerns should Motor function
be included. The main areas of the examination are out- Muscle performance
lined here. Orthotic, protective, and supportive devices
Pain
History Posture
A review of the medical record is the first step toward the Range of motion
examination because it provides the background information Reflex integrity
and identifies medical precautions. The history should in- Self-care and home management
clude general demographics, social history, occupation or Sensory integrity
employment, pertinent growth and development, living envi- Ventilation, respiration, and circulation
ronment, history of current condition, functional status and Diagnosis of impairment and disabilities
activity level, completed tests and measures, medications,
STANDARD NEUROLOGICAL CLASSIFICATION OF SPINAL CORD INJURY

MOTOR LIGHT
TOUCH
PIN
PRICK SENSORY
R L KEY MUSCLES R L R L KEY SENSORY POINTS
C2 C2
0 = absent
C3 C3
1 = impaired
C4 C4
2 = normal
C5 Elbow flexors C5 NT = not testable C2
C6 Wrist extensors C6 C3
C7 Elbow extensors C7 C4
C8 Finger flexors (distal phalanx of middle finger) C8 C5

T2
T3
T2
C5
T1 Finger abductors (little finger) T1 T4
T5
T2 T2 T6
T7
T3 0 = total paralysis T3 T8
T1 T1
T4 1 = palpable or visible contraction T4 C6
T9
C6
T10
T5 2 = active movement, T5 T11
gravity eliminated
T6 T6 L1
T12
L1
3 = active movement, Palm Palm
T7 T7
against gravity
T8 4 = active movement, T8
T9 against some resistance T9 L2 L2
T10 5 = active movement, T10
C8
C8
T11 against full resistance T11
C6
C7
L3 L3
C7
T12 NT = not testable T12 Dorsum Dorsum
L1 L1
L2 Hip flexors L2 L4 L4
L3 Knee extensors L3 L5 L5
L4 Ankle dorsiflexors L4
L5 Long toe extensors L5
S1 Ankle plantar flexors S1 Key Sensory Points
S2 S2 S1 S1
S3 S3 S1
S4-5 Voluntary anal contraction (Yes/No) S4-5 Any anal sensation (Yes/No)
PIN PRICK SCORE
TOTALS + = MOTOR SCORE TOTALS { +
+ =
= LIGHT TOUCH SCORE
(max: 112)
(max: 112)
(MAXIMUM) (50) (50) (100) (MAXIMUM) (56) (56) (56) (56)
NEUROLOGICAL R L COMPLETE OR INCOMPLETE? ZONE OF PARTIAL R L

LEVEL SENSORY Incomplete = Any sensory or motor function in S4-S5 PRESERVATION SENSORY
The most caudal segment MOTOR Caudal extent of partially MOTOR
with normal function ASIA IMPAIRMENT SCALE innervated segments
This form may be copied freely but should not be altered without permission from the American Spinal Injury Association. 2000 Rev.
Figure 16-10 n American Spinal Injury Association motor and sensory evaluation form. (Courtesy
American Spinal Injury Association, Atlanta, Georgia.)
Along with the strength of each muscle, the presence, Another tool that is recognized as a primary outcome measure
absence, and location of muscle tone should be described. to assess functional recovery for the client with SCI is the
The Modified Ashworth Scale is a common tool used to de- Spinal Cord Injury Independence Measure III (SCIM III).56
scribe hypertonicity.54 The client’s sensation is described by This tool was specifically designed for the functional assess-
dermatome. The recommended tests include (1) sharp-dull ment of individuals with SCI. The SCIM III has been shown
discrimination or temperature sensitivity to test the lateral to be valid, reliable, and easily administered.57-59 Other tools,
spinothalamic tract, (2) light touch to test the anterior spino- such as the Quadriplegia Index of Function (QIF)60 and
thalamic tract, and (3) proprioception or vibration to test the the Craig Handicap Assessment and Reporting Technique
posterior columns of the spinal cord. Sensation is indicated (CHART),61 are options. Additional assessments for patients
as intact, impaired, or absent per dermatome. A dermatomal with SCI are described in Table 16-3.
map is helpful and recommended for ease of documentation.
GOAL SETTING FOR ACTIVITY
Functional Examination AND PARTICIPATION SKILLS
It is recommended that a complete functional assessment be Goal setting is a dynamic process that directly follows the
performed on initial examination and thereafter as appropri- examination. Each activity limitation identified should be
ate. Myriad tools exist to assess functional skills. Many insti- addressed with specific short- and long-term goals. The cli-
tutions develop functional assessments that address home, nician must interpret new information continuously, which
community, and institutional mobility and ADL functional leads to continuing reevaluation and revision of goals.62
skills. The Functional Independence Measure (FIM) is one Goals are always individualized and should be established
of the more commonly used tools that is currently applied in collaboration with the treatment team, the client, and the
for many impairment diagnostic groups, including SCI.55 caregiver, and with realistic consideration of anticipated
TABLE 16-3 n ASSESSMENT OF FUNCTION SUMMARY TABLE
OVERALL FUNCTIONAL ASSESSMENTS DESCRIPTION

SPINAL CORD INJURY (SCI) FUNCTIONAL ASSESSMENTS
Spinal Cord Injury Independence Measure (SCIM)239 Designed for the functional assessment of individuals with spinal cord
injury in the categories of self-care, respiratory, sphincter management,
and mobility skills
Quadriplegia Index of Function (QIF)60 Assesses function for individuals with tetraplegia in the categories
of transfers, grooming, bathing, feeding, dressing, wheelchair mobility,
bed activities, bowel, bladder, and knowledge of personal care
Capabilities of Upper Extremity (CUE) instrument240 Assesses the action of grasp, release, and reaching in individuals with
tetraplegia by measuring reaching and lifting, pulling and pushing, wrist
action, hand and finger actions, and bilateral action
WALKING FUNCTION ASSESSMENTS
Spinal Cord Injury Functional Ambulation Inventory Observational gait assessment that assesses gait, assistive device use, and
(SCI-FAI)241 walking mobility
Walking Index for Spinal Cord Injury (WISCI)242 An ordinal scale describing walking function that takes into consideration
level of independence, assistive device use, and lower-extremity orthotic use
Six-minute walk test243 An endurance walking test that measures the distance walked over a 6-min
period of time
Ten-meter walk test244 Measures walking speed by measuring how fast an individual walks a
distance of 10 m
Timed Up-and-Go Test244 Assesses standing, walking, turning, and sitting
WHEELCHAIR FUNCTION ASSESSMENTS
Wheelchair Circuit245 Assesses the performance of various wheelchair propulsion skills by
measuring ability, performance time, and physical strain for eight
standardized skills
Wheelchair Assessment Tool246 Measures the ability and time to perform six mobility and wheelchair skills
for individuals with paraplegia
Wheelchair Skills Test247 Assesses the ability to perform 50 separate skills in the areas of wheelchair
handling, transfers, maneuvering the wheelchair, and negotiating obstacles
Obstacle Course Assessment of Wheelchair User Assesses the wheelchair user’s performance in 10 difficult environmental
Performance248 situations
Wheelchair Users Functional Assessment (WUFA)249 A 13-item assessment of wheelchair skills in individuals who primarily
use a manual wheelchair for their mobility
Wheelchair Physical Functional Performance (WC-PFP)250 Assesses the ability to complete various tasks from the wheelchair by
measuring upper body strength, upper body flexibility, balance, coordina-
tion, and endurance
Functional Evaluation in a Wheelchair251 Assesses functional performance from a manual and/or power wheelchair
via a self-administered questionnaire
needs on return to the home environment. Factors to con- positioning, skin care management, transfers, transportation
sider in the goal-setting process include age, body type, and driving, wheelchair management, and wheelchair mo-
associated injuries, premorbid medical conditions, addi- bility. Refer to Table 16-4 for anticipated goals for each
tional orthopedic injury, cognitive ability, psychosocial issues, level of injury. Information presented in this table should be
spasticity, endurance, strength, ROM, funding sources, and recognized as general guidelines because variability exists.
motivation. These guidelines are most usefully applied to patients with
Long-term goals for the rehabilitation of patients with complete SCI. Goal setting for individuals with incomplete
SCI reflect functional outcomes and are based on the SCI is often more challenging, given the greater variability
strength of the remaining innervated or partially innervated of client presentations and the uncertainty of neurological
musculature. Short-term goals identify components that in- recovery. As with any patient, continual reevaluations pro-
terfere with functional ability and are designed to “address vide additional insight into functional limitations or progres-
these limiting factors while building component skills”7 of sion and potential and thereby direct the goal-setting pro-
the desired long-term goals.63 cess. In addition to specific functional goals and expectations,
Functionally based goals are established in the following family training; home, work, or school modifications; and
areas: bathing, bed mobility, bladder and bowel control, community reentry should be considered.
communication, environmental control and access, feeding, Rehabilitation teams may elect to hold a goal-setting or
dressing, gait, grooming, home management, ROM and interim conference for each patient, during which team
Text continues on page 475.
TABLE 16-4 n FUNCTIONAL EXPECTATIONS FOR COMPLETE SPINAL CORD INJURY LESIONS
ANTICIPATED EQUIPMENT
FUNCTIONAL COMPONENT OUTCOME POTENTIAL TO ACHIEVE OUTCOMES
C1-4
Sitting tolerance 80-90 degrees for 10-12 hours per day Power wheelchair with power tilt,
recline
Wheelchair cushion
Communication
Mouth stick writing Minimal assistance Mouth sticks and docking station
ECU Setup ECU
Page turning Minimal assistance to setup Book holder
Computer operation Minimal assistance to setup Computer
Call-system use Setup Call system or speaker phone
Cuff-leak speech (ventilator dependent) Up to 6 hours
Feeding Dependent, but verbalizes care
Grooming Dependent, but verbalizes care
Bathing Dependent, but verbalizes care Reclining shower chair
Dressing Dependent, but verbalizes care
Bowel management Dependent, but verbalizes care
Bladder management Dependent, but verbalizes care
Bed mobility
Rolling side to side Dependent, but verbalizes care Four-way adjustable hospital bed
to assist caregiver with task
Rolling
Supine, prone
Supine to and from sitting
Scooting
Leg management
Transfers
Bed Dependent, but verbalizes care Overhead lift system
Tub, toilet Hydraulic lift
Car Slings
Floor
Power wheelchair mobility
Smooth surfaces Modified independent Power wheelchair with power recline
or tilt system
Ramps Modified independent
Rough terrain Modified independent Lap tray
Curbs Dependent, but verbalizes Armrests, shoulder supports,
and lateral trunk supports
Manual wheelchair mobility
Smooth surfaces Dependent, but verbalizes Manual reclining or tilt wheelchair
with same options as power
wheelchair
Ramps
Rough terrain
Curbs
Stairs
Skin
Weight shift Modified independent with power wheelchair Recline or tilt wheelchair
Padding, positioning Dependent, but verbalizes Wheelchair cushion
Skin checks Dependent, but verbalizes Pillow splints, resting splints
Mirror
Community:ADL-
dependent passenger evaluation Dependent, but verbalizes Modified van
ROM exercises to scapula, upper extremity, Dependent, but verbalizes
lower extremity, and trunk
Exercise program Independent for respiratory and neck exercises Portable or bedside ventilator
(C1-3 only)
TABLE 16-4 n FUNCTIONAL EXPECTATIONS FOR COMPLETE SPINAL CORD INJURY LESIONS—cont’d
C5
Sitting tolerance 90 degrees for 10-12 hours per day Power recline or tilt wheelchair
Wheelchair cushion
Communication
Telephone use Modified independent Telephone adaptations
ECU Setup ECU
Page turning Setup Book holder, wrist support with cuff
Computer operation Supervision Computer
Writing, typing Setup Long Wanchik brace
Feeding Minimal assist to setup Mobile arm support or offset feeder
Adaptive ADL equipment
Grooming
Wash face Minimal assistance to setup Mobile arm support or offset feeder
Comb or brush hair Minimal assistance Wrist support with adapted cuff
Oral care Minimal assistance to setup Adaptive ADL equipment
Bathing Dependent, but verbalizes care Upright or tilt shower chair
Dressing Dependent, but verbalizes care
Bowel management Dependent, but verbalizes care
Bladder management Dependent, but verbalizes care Automatic leg bag emptier
Bed mobility
Rolling side to side Dependent to maximal assistance 4-way adjustable hospital bed to assist
caregiver with care
Rolling
Supine, prone
Scooting
Leg management
Transfers
Bed Dependent to maximal assistance for level Overhead or hydraulic lift and slings
transfers, verbalizes unlevel transfers
Tub, toilet Possible transfer board
Car
Floor
Power wheelchair mobility Recommended mode of locomotion Power wheelchair with power recline
or tilt system
Smooth surfaces Modified independent
Ramps Modified independent Recommend lap tray
Rough terrain Modified independent Armrests, shoulder supports, lateral
trunk supports
Curbs Dependent, but verbalizes
Smooth surfaces Dependent to minimal assistance for short dis- Upright or reclining wheelchair with
tances on smooth surface special back and trunk supports
Ramps Dependent, but verbalizes care
Rough terrain Dependent, but verbalizes care Consider manual wheelchair with
power assist pushrims
Curbs Dependent, but verbalizes care
Stairs Dependent, but verbalizes care
Skin
Weight shift Modified independent with power wheelchair Recline or tilt wheelchair
Maximal assistance to dependent with manual and wheelchair cushion
wheelchair
Padding, positioning Dependent, but verbalizes Pillow splints or resting splints
Skin checks Dependent, but verbalizes Mirror
Home management
Prepare snack Maximal to moderate assistance Wrist support with cuffs
Continued
Community ADL
Drive van Independent Highly adapted vehicle
Dependent passenger evaluation Dependent Modified van
ROM exercises to scapula, upper extremity, Dependent, but verbalizes
lower extremity, and trunk
Exercise program Airsplints or light cuff weights
Upper extremity and neck Minimal assistance E-stim unit
C6
Sitting tolerance 90 degrees for 10-12 hours per day
Communication
Telephone use Modified independent Adaptive ADL equipment
Page turning Tenodesis splint
Writing, typing, keyboard Short opponens splint
Feeding Modified independent Adaptive ADL equipment
Grooming Minimum assistance to modified independent Adaptive ADL equipment
Tenodesis splint
Bathing
Upper body Minimal to modified independent assistance Upright shower chair
Lower body Moderate assistance Various bathing equipment
Dressing
Upper body Modified independent Adaptive ADL equipment
Lower body (bed) Maximum to minimal assistance
Bowel management Maximum to modified independent Dil stick
Bladder management Male: moderate assistance to modified independent Tenodesis
Female: moderate assistance to dependent Adaptive ADL equipment
Bed mobility
Rolling side to side Independent to minimal assistance Four-way adjustable hospital bed
or regular bed with loops or straps;
or no equipment
Rolling
Supine, prone
Scooting
Leg management Minimum assistance to dependent
Transfers
Bed Minimal assistance Transfer board
Tub, toilet Moderate assistance
Car Maximal to moderate assistance
Floor Dependent, but verbalizes procedure
Power wheelchair mobility Recommended mode of locomotion Power upright wheelchair for weak
C6
Rough terrain Modified independent
Manual wheelchair mobility Ultralight upright wheelchair
(recommended as primary only
if scapulae grades are 3 or better)
Smooth surfaces Modified independent May need adaptations to facilitate
more efficient propulsion (i.e., push
pegs, plastic-coated handrims)
Rough terrain Moderate to minimal assistance
Curbs Dependent, but verbalizes procedure Consider manual wheelchair with
power assist pushrims
Stairs Dependent, but verbalizes procedure
Skin
Weight shift Modified independent Upright wheelchair with push handles
Pad, positioning Moderate to minimal assistance Mirror
Skin checks Moderate to minimal assistance
Home management
Light home management Minimal assistance Various adaptive ADL equipment
Heavy home management Dependent to moderate assistance
Community ADL
Driving vehicle Modified independent Modified vehicle
ROM exercises to scapula, upper extremity, Minimal assistance Leg lifter to assist with
lower extremity, and trunk lower-extremity ROM
Exercise program Minimal assistance Cuff weights
Air splints
E-stim unit
C7-8
Communication
Telephone use Modified independent Adaptive ADL equipment
Page turning
Writing, typing, keyboard
Feeding Modified independent Adaptive ADL equipment
Grooming Modified independent Adaptive ADL equipment
Bathing
Upper body Modified independent Upright shower chair
Lower body Modified independent Various bathing equipment
Dressing (upper and lower body) Modified independent for upper-body dressing Adaptive ADL equipment
In bed Minimal assistance to modified independent for
lower-body dressing
In wheelchair
Bowel management Modified independent Dil stick
Bladder management
Bed Male: modified independent Various bladder management
or adaptive ADL equipment
Wheelchair Female: moderate assistance to modified
independent
Male: modified independent
Bed mobility
Rolling side to side Modified independent Leg lifter
Rolling
Supine, prone
Scooting
Leg management
Transfers
Bed Modified independent Transfer board
Tub, toilet Modified independent May not need transfer board for even
surfaces
Car Minimal assistance for loading wheelchair
Floor Maximal assistance
Power wheelchair mobility
Smooth surfaces Modified independent Power upright wheelchair
Continued
Smooth surfaces Modified independent Upright wheelchair
Curbs Minimal to moderate assistance
Stairs Maximal assistance
Skin
Weight shift Modified independent Upright wheelchair with push handles
Pad, positioning Minimal assistance to modified independent
Skin checks Minimal assistance to modified independent Mirror
Home management
Light home management Modified independent Various ADL equipment
Heavy home management Moderate assistance
Community ADL
Driving vehicle Modified independent Modified vehicle
ROM exercises to scapula, upper extremity, Modified independent Leg lifter to assist with lower-
lower extremity, and trunk extremity ROM
Exercise program Modified independent Cuff weights or e-stim unit
PARAPLEGIA
Communication Independent
Feeding Independent
Grooming Independent
Bathing
Upper body Independent Upright tub chair
Lower body Modified independent Long-handled sponge and hand-held
shower hose
Dressing (upper and lower body) Adaptive ADL equipment
In bed Modified independent
In wheelchair Modified independent
Bowel management Modified independent Dil stick if positive bulbocavernous
reflex
Suppositories if negative
bulbocavernous reflex
Bladder management Modified independent
Bed mobility
Rolling side to side Modified independent
Rolling
Supine, prone
Scooting
Leg management
Transfers
Bed Modified independent May need a transfer board
Tub, toilet
Car
Floor
Upright wheelchair
Manual wheelchair mobility Upright wheelchair
Ramps
Rough terrain
Curbs Moderate assistance to modified independent
Stairs (three or four)
Ambulation Depends on level of injury
Smooth surfaces Modified independent for T12 injuries Appropriate orthotics and assistive
and below device(s)
Will vary with higher thoracic injuries
Ramps
Rough terrain
Curbs
Stairs
Skin
Weight shift Modified independent
Pad, positioning
Skin checks Mirror
Home management
Light home management Modified independent
Heavy home management Modified independent Various adaptive ADL equipment
Community ADL
Driving vehicle Modified independent Hand controls for vehicle
ROM exercises to left extremity and trunk Modified independent Leg lifter to assist with
lower-extremity ROM
Exercise program Modified independent Cuff weights, e-stim if any weakened
lower-extremity muscles
ADL, Activity of daily living; ECU, enviornmental control unit; ROM, range of motion.
members, including the client, have the opportunity to dis- especially at the sacrum, should be taken into account.
cuss the long-term goals that have been established. It may Recently, padded spine boards have become available and
be useful to request that the patient sign a statement ac- are recommended to reduce the risk of skin complications.
knowledging understanding of, and agreement to, all long- Preventive skin care begins with careful inspection. Soft
term goals. tissue areas over a bony prominence are at greatest risk for
acquiring a pressure sore.67 Key areas to evaluate include the
EARLY REHABILITATION AND sacrum, ischia, greater trochanters, heels, malleoli, knees,
COMPLICATION PREVENTION occiput, scapulae, elbows, and prominent spinous processes.
Early rehabilitation of the patient with SCI begins with pre- A turning schedule should be initiated immediately. Even
vention. Preventing secondary complications speeds entry if the patient has unstable fractures or is in traction, he or
into the rehabilitation phase and improves the possibility she can be turned and positioned with flat pillows using the
that the patient will become a productive member of society. logroll technique. Even small changes off the sacrum and
Table 16-5 describes an overview of the primary compli- coccyx are helpful. The patient’s position in bed should be
cations that can arise after an SCI. In this table, known initially established for turns to occur every 2 to 3 hours.66
causes and common management activities are reviewed. This interval can be gradually increased to 6 hours with
Tests and measures commonly used to determine the com- careful monitoring for evidence of skin compromise. A red-
plication and the recommended medical and/or therapeutic dened round area over the bone that does not disappear after
interventions are listed in the table. Although various reports 15 to 30 minutes is the hallmark start of a pressure sore, and
of incidences are published, the largest database is the action to avoid or minimize pressure in the area must be
Model Spinal Cord Injury Care Systems report.41,64 Because taken immediately to avoid progression. Turning positions
of their high incidence and potential effect on long-term include prone, supine, right and left side-lying, semiprone,
outcomes, the following complications require further dis- and semisupine positions.68,69 Secondary injuries such as
cussion: skin compromise, loss of ROM or joint contrac- fractures and the presence of vital equipment, such as venti-
tures, and respiratory compromise after SCI. lator tubing, chest tubes, and arterial lines, should be consid-
ered when choosing turning positions. The prone position is
Preventing and Managing Pressure Ulcers the safest position for maintaining skin integrity but may not
and Skin Compromise always be feasible.
After SCI and during the period of spinal shock, patients Pillows or rectangular foam pads may be used to bridge
are at greater risk for development of pressure ulcers.65,66 off the bony prominences and relieve potential pressure.
The use of backboards at the emergency scene and during This is especially helpful above the heels. Padding directly
radiographic procedures contributes to potential skin com- over a prominent area with a firm pillow or pad may only
promise; therefore, immediate concern for tissue death, increase pressure and should be avoided. Great care should
TABLE 16-5 n COMPLICATIONS AFTER SPINAL CORD INJURY
DIAGNOSTIC TESTS MEDICAL TREATMENT

COMPLICATION CAUSE AND MEASURES OR INTERVENTION THERAPEUTIC INTERVENTION
CARDIOPULMONARY
Pneumonia Bacterial or viral infec- Radiographic Antibiotics, bronchodilator Chest physical therapy: percussion,
Atelectasis tion, prolonged immo- studies, diagnostic therapy, therapeutic vibration, postural drainage,
bilization, prolonged bronchoscopy bronchoscopy, suctioning mobilization, inspiratory
artificial ventilation, breathing exercises
general anesthesia
Ventilatory failure Weakness or paralysis Pulmonary function Artificial ventilation and Airway and secretion management
of the inspiratory tests (PFTs), arterial supportive therapy, treatment as above, early mobili-
muscles, unchecked blood gases (ABGs), management of under- zation once stabilized, biofeed-
bronchospasm end-tidal CO2 monitor- lying cause (e.g., pneu- back to assist with ventilator
ing, pulse oximetry monia), oxygen therapy weaning as appropriate
Deep vein throm- Venous status, activation Doppler studies, leg Subcutaneous heparin3,252 Early mobilization and range of
bosis (DVT)* of blood coagulation, measurements, Prophylactic anticoagu- motion (ROM) for prevention,
pressure on immobi- extremity visual lation can decrease centripetal massage for prevention,
lized lower extremity, observation and incidence to 1.3%5 compression garments, education
and endothelial palpation, low-grade Vena cava filter for about smoking cessation, weight
damage65-67 fever of unknown failed anticoagulant loss, and exercise; avoid constrict-
origin prophylaxis ing garments and monitor overly
tight leg bag straps and pressure
garments (Paralyzed Veterans of
America DVT guidelines)
Pulmonary Dislodging of DVT Ventilation-perfusion Vena cava filter None
embolus lung scan, signs and Anticoagulation therapy
symptoms including
chest pain, breathless-
ness, apprehension,
fever, and cough
Orthostatic Vasodilation and decreased Monitor blood pressure Medications to increase Gradient compression garments:
hypotension venous return, loss with activity and blood pressure, fluids Ace wraps, abdominal binders,
of muscle pump action changes in position, in the presence of appropriate wheelchair selection
in dependent lower observation for signs hypovolemia to prevent rapid changes in posi-
extremities and trunk253 and symptoms tion early in rehabilitation
Apneic True origin unknown; be- Electrocardiogram Hyperventilation Remove noxious stimulus
bradycardia lieved to be caused by Heart rate
sympathetic disruption Respiratory rate
resulting in vagal domi-
nance in response to
a noxious stimulus
or hypoxia254
INTEGUMENTARY SYSTEM
Pressure ulcers Prolonged external skin Wound measurements, Nutritional support as Irrigation and hydrotherapy,
pressure exceeding staging classification, needed, surgical or dressing management,
the average arterial nutritional assessment71 enzymatic debride- electrotherapy71
or capillary pressure255 ment, surgical closure,
muscle flap, skin flap
or graft, antibiotics as
appropriate
Shearing Stretching and tearing See pressure ulcers See pressure ulcers Add protective padding during
of the blood vessels that functional activities, skill perfec-
pass between the layers tion, correct handling techniques
of the skin7
Moisture Excessive sweating See pressure ulcers See pressure ulcers, treat Protective barrier ointments and
below the level of in- possible urinary tract powders, establish effective bowel
jury, urinary and bowel infection, medications and bladder programs, educate for
incontinence, poor for bladder incontinence improved hygiene, and refine
hygiene activity of daily living (ADL) skills
TABLE 16-5 n COMPLICATIONS AFTER SPINAL CORD INJURY—cont’d

NEUROMUSCULAR
Spasticity Upper motor neuron Ashworth or Modified Antispastic pharmaco- Prolonged stretching; inhibitive
lesion73 Ashworth Scale logical agents: positioning or casting
Deep tendon reflex Baclofen pump baclofen, diazepam Cryotherapy, weight-bearing
spasticity scale insertion75 (Valium), dantrolene exercise, and aquatic therapy
evaluation Surgical intervention:
myelotomy, rhizotomy,
peripheral neurotomy73
Botox injection
Flaccidity Lower motor neuron Deep tendon reflexes None None for treating flaccidity; how-
lesion7,256 (would be absent) ever, secondary treatments that
Most often in injuries need to be considered include
at L1 level and below positioning to improve postural
support, education for skin pro-
tection, and bracing and splinting
to maintain joint integrity
Neurogenic Refer to bowel Positive bulbocaverno- Oral laxative, supposito- Establish comprehensive bowel
bowel† management sus reflex: indicates ries, and enemas program
reflexic bowel
Autonomic Triggering of an uncon- Sudden rise in systolic Catheterization of the Immediately position the client
dysreflexia trolled hyperactive blood pressure of bladder, irrigation of in upright position, identify and
response from the sym- 20-40 mm Hg above indwelling catheter, remove noxious stimuli, check
pathetic nervous system baseline254 pharmacological clothing and catheter tubing for
by a noxious stimulus7; Observation of signs management if systolic constriction, and perform bowel
noxious stimuli may and symptoms: blood pressure is program if fecal impaction is
include bowel or bladder Sweating above level greater than 150 mm Hg suspected
distention, urinary tract of injury Remove ingrown toenail
infection, ingrown toe- Goose bumps if present
nail, tight clothing, and Severe headache
pressure sore Flushing of skin from
vasodilation above
level of injury254
Ulcers, Venous status, activation Radiographic studies, Medications to increase Gradient compression garments;
gastrointestinal of blood diagnostic blood pressure Ace wrap
OTHER
Thermoregulation Interruption between Body temperature Cooling or warming Education about risk and proper
problems communication with blanket if extreme protection from elements; behav-
autonomic nervous sys- ior modification, education for
tem and hypothalamus proper hydration and appropriate
Lack of vasoconstriction clothing
and inability to shiver
or perspire254
Pain Radicular pain originating Pain scales, functional Immobilization and Restore ideal alignment and
from the injury,7,257,258 assessment,260 rest, pain medications, posture; thermal modalities and
kinematic or mechanical taxonomy injections for pain electromodalities; manual therapy,
pain, direct trauma, or antiinflammatory improve movement patterns
referred pain258,259 measures
Urinary tract Presence of excessive Urinalysis, urine culture Antibiotics Monitor fluid intake and educate
infections bacteria in urine and sensitivity, for proper technique during
temperature bladder care
Contractures Muscle imbalance around Goniometric Tendon release; Botox ROM functional use of extremity,
joint; prolonged immo- measurements injection for isolated casting or splinting, achieving
bilization, unchecked spasticity and maintaining optimal postural
spasticity, pain alignment
Continued
TABLE 16-5 n COMPLICATIONS AFTER SPINAL CORD INJURY—cont’d

Heterotopic Unknown Alkaline phosphatase Etidronate disodium Maintain available ROM; avoid
ossification levels (increase after (Didronel): use prophy- vigorous stretching during
(HO) 6 weeks)261,262; obser- lactically or during inflammatory stage; achieve
vation for sudden loss inflammatory stage and maintain optimal wheelchair
of ROM, local edema, Surgical resection positioning
heat, erythema,
nonseptic fever
Osteoporosis and Bone demineralization263 Bone scan None; calcium supple- Weight-bearing techniques: amount
joint changes ment for prevention and type unknown, specific to
degenerative spinal cord injury
Spinal deformities Muscle imbalance or Posture evaluation, If severe: surgical fixa- Restore postural alignment, avoid
weakness around spinal seating evaluation tion, thoracic orthosis repetitive asymmetrical activities,
column; poor postural control spasticity
support, asymmetrical
functional activities
Gastroduodenal Acute: disruption of Hematocrit and hemo- Surgical intervention; re- Establish effective bowel program,
ulcers, gastroin- central nervous system, globin; observation of store normal gastroin- establish high-fiber diet, provide
testinal bleeding abdominal trauma or gastrointestinal fluids testinal function education and stress management
stress response to neu-
roendocrine system264
Chronic: impairment of
autonomic nervous
system7
Metabolic, Impairment of autonomic Observe for fatigue, None known Education, exercise, and weight
endocrine nervous system malaise; undesirable control
weight gain62
*Consortium for Spinal Cord Medicine Clinical Practice Guidelines: Prevention of thromboembolism in spinal cord injury, Washington, D.C., February 1997,
Paralyzed Veterans of America.
†
Consortium for Spinal Cord Medicine Clinical Practice Guidelines: Neurogenic bowel management in adults with spinal cord injury, Washington, D.C., March 1998,
Paralyzed Veterans of America.
be taken for regular checks if this bridging technique is used have poor motor control and impaired balance and must be
in the trunk or buttocks region while the patient is in bed, carefully monitored to avoid injury.
owing to eventual shifting of the foam. Should skin compromise occur, the first intervention is
Keeping the head of the bed as low as tolerated mini- to identify and remove the source of the compromise.
mizes the risk for shearing and excessive sacral pressure. Modifications to the seating system or changing to a more
For patients who are not appropriate for rigorous turning pressure-reducing mattress system or cushion may be neces-
schedules (e.g., patients with unstabilized fractures), spe- sary. Examination and treatment will then need to focus on
cialty alternating pressure mattresses are available. Low air healing the wound and preventing other secondary compli-
loss, alternating pressure, or even air-fluidized mattresses cations that may occur as a result of potential immobility
are available for those who require the head of the bed to be and delayed physical rehabilitation. The reader is encour-
elevated more than 30 degrees for prolonged periods and aged to refer to Pressure Ulcer Treatment: Clinical Practice
also have other extenuating conditions such as respiratory Guideline, developed by the Agency for Health Care Research
distress, diabetes, and/or low prealbumin.70 and Quality, for examination tools, including the classifica-
While the patient is sitting, an appropriate pressure redis- tion of pressure ulcers.71,72
tribution (relief) cushion is recommended and a pressure Treatment interventions may include hydrotherapy, spe-
relief (weight shift) schedule is established and strictly cialty wound dressings, electro-modalities, and thermal mo-
enforced. dalities to increase circulation.71 Mechanical, autolytic, enzy-
Although pressure is one of the most prevalent causes matic, or surgical debridement may be necessary to obtain
of skin compromise, other forces may lead to problems, and maintain a viable wound bed. If the wound does not
including friction, shearing, excessive moisture or dryness, heal, surgical interventions with myocutaneous or muscle
infection, and bruising or bumping during activities. This flaps may be necessary for closure. Coordinated return-to-sit
is especially true of clients with SCI because of altered ther- programs or protocols after such medical interventions are
moregulation, changes in mobility, decreased or absent necessary to prevent opening of the surgical site. Such surgi-
sensation, and incontinence of bowel and bladder. In addi- cal procedures are costly and significantly delay functional
tion, as patients begin to learn functional skills, they may rehabilitation.
After closure and healing of the wound, education be- shortening of the flexor tendons. Some clinicians argue
comes a priority to maintain skin integrity. The client must that the client can develop a fixed flexion contracture
adhere to a more rigorous skin check program as rehabilita- of the proximal interphalangeal joints, interfering with
tion continues, giving special attention to the affected area. future surgical attempts to restore finger function.38 It is
Teaching patients to advocate for themselves and to problem recommended to promote tenodesis functioning via adap-
solve equipment and lifestyle issues that may affect their tive shortening while maintaining joint suppleness.
skin condition will reduce the recurrence rate. Alcohol, In the presence of weakened or paralyzed elbow exten-
tobacco, and drug use (both recreational and prescription) sors, shortening of the elbow flexors should be prevented
should be managed for long-term success. Prevention of because it will impair ADL function and transfer skills.7,69
skin compromise is critical and cannot be stressed enough Contracted elbow flexors or pronator muscles in a client
to health care providers, patients, and caregivers. with an SCI level of C6 can cost this client his or her inde-
pendence. Likewise, the rotator cuff and the other scapular
Prevention and Management of Joint muscles should be assessed for their length-tension relation-
Contractures ships and their ability to generate force. Normal length of
The development of a contracture may result in postural these muscles should be maintained. For example, achieving
misalignment or impede potential function. Daily ROM external rotation of the shoulder (active and passive) is criti-
exercises, proper positioning, and adequate spasticity con- cal for clients with low-level tetraplegia. Shortening of the
trol may help prevent contractures.66 Contracture preven- subscapularis and other structures can quickly result in a
tion includes the use of splints for proper joint alignment, decrease in motion, limiting bed mobility, transfers, feeding,
techniques such as weight bearing, ADLs, and functional and grooming skills. Patients with complete paraplegia who
exercises. Patients exhibiting spasticity may require more are candidates for ambulation require normal ROM in the
frequent ROM intervention.66,73 lower extremities. If the hip flexors or knee flexors are
allowed to shorten, achieving standing and ambulation goals
Adaptive Shortening or Adaptive Lengthening will be more difficult.
of Muscles The combination of lengthened hamstrings and tight
Although isolated joint ROM should be normal for all back extensor muscles provides stability for balance in the
patients, allowing adaptive shortening or adaptive lengthen- short- and long-sitting positions. This aids in the efficiency
ing of particular muscles is recommended to enhance the of transfers and bowel and bladder management. Balance
achievement of certain functional skills.69,74 Likewise, un- in long sitting assists with lower-extremity dressing and
wanted shortening or lengthening of muscles should be other ADLs. Hamstrings should be lengthened to allow 110
prevented. The following section reviews a few examples to 120 degrees of straight leg raising without overstretching
of these concepts as they relate to SCI. back extensor muscles.
Tenodesis is described as the passive shortening of the
two-joint finger flexors as the wrist is extended. This ac- Splinting to Prevent Joint Deformity
tion creates a grasp, which assists performance of ADLs Deformity prevention is the first goal of splinting.76 Patients
(Figure 16-11).69,75 A patient with mid to low tetraplegia with cervical spinal cord injuries may have lost normal neu-
may rely on adaptive shortening of these long finger flex- ral input to musculature in their wrists and hands. Other
ors to replace active grip.69 If the finger flexors are clients may have partial motor control, which may lead to
stretched across all joints during ROM exercises, the muscle imbalances and loss of ROM. In the absence or weak-
achievement of some functional goals may be limited. ness of elbow extensors, a bivalve cast or an elbow extension
ROM to the finger flexors should be applied only while the splint at night may be beneficial to prevent joint contractures.
wrist is in a neutral position. There is controversy over At the wrists, a volar wrist support is commonly used ini-
tially and may be progressed to a longer-term option of a
definitive wrist orthosis. Other splints often used for defor-
mity prevention of the hands include resting hand splints
with proper positioning to maintain the support of the wrist
and web space (Figure 16-12, A).77 Another hand-based
option is the intrinsic plus splint (Figure 16-12, B), which
places the metacarpophalangeal joints closer to 90 degrees
of flexion and decreases intrinsic hand muscle tightness.
Another goal of splinting in the SCI population is to
increase function. Patients with tetraplegia at the C5 level
rely on an orthosis to be independent with communication,
feeding, and hygiene. They must have joint stability and
support at the wrist and the hand to perform these skills.
The splint is often adapted with a utensil slot or cuff so
that the client can effectively perform the skills mentioned
previously.
Patients who are not strong enough to use their wrists
for tenodesis may require splinting to support their wrists
until they can perform wrist extension against gravity. Long
Figure 16-11 n Tenodesis grasp. opponens splints can be used to position the thumb for
A B
Figure 16-12 n A, Resting hand splint. B, Volar intrinsic plus splint maintains alignment of the
wrist and fingers to promote metacarpophalangeal flexion for tenodesis grasp.
function but support the weak wrist (Figure 16-13). Once and prefabricated splints. A well fitting, prefabricated splint
the wrist muscles strengthen, the long opponens splint can can be as effective as a custom-fabricated splint in certain
be cut down to a hand-based short opponens to maintain situations. Custom splints require additional resources and
proper web space and thumb positioning while maximizing clinician expertise. One way to minimize time spent in fab-
tenodesis. rication of splints is to use a good pattern and premade
As mentioned previously, clients with injuries at the C6 straps. Finally, educating the client on the splint-wearing
level can use their wrists for a tenodesis grasp.75,78,79 Critical schedule, skin checks, and splint care is important for pre-
components of the splint assessment for these clients are venting skin breakdown.
the positioning of the thumb, web space, and index finger
observed during the grasp. It is recommended that the Treatment for Joint Deformity
client’s hand be positioned with the thumb in a lateral pinch If a joint contracture occurs despite preventive measures,
position because this is the most commonly used prehension more aggressive treatments are necessary. This may include
pattern to pick up objects. Clients who are not splinted may more aggressive use of splinting, plaster or fiberglass cast-
not have the proper positioning to pick up objects because ing techniques, or botulinum toxin type A (Botox) injec-
their tenodesis is “too tight” or “too loose.” tions.81-83 When splinting is not effective, fabrication of
Clients with C8 to T1 injuries or clients who have incom- serial or bivalve casts may be indicated. The client with
plete injuries may have “clawing” or hyperextension of the minimal ROM limitations may require only one cast. Most
metacarpophalangeal joints. This is caused by finger exten- commonly, the client has a significant limitation and
sor musculature that is stronger than finger flexor muscula- requires serial casts, in which several casts are applied and
ture.75,80 To prevent this, a splint can be made to block the then removed over a period of weeks to increase extensibil-
metacarpophalangeal joints and promote weak intrinsic ity in the soft tissues surrounding the casted joint.84 The
muscle function. Depending on the extent of the imbalance, involved joint is placed at submaximal ROM.85 Once the
these splints can be used during function or worn only cast is removed, the joint should have an increase of approxi-
at night. Cost, time, material, and clinician experience are mately 7 degrees of ROM.85 This process continues until
important considerations when deciding between custom the deformity is minimized or resolved. The final cast is a
bivalve so that the cast can act as a positioning device that
can be easily removed. Casting contraindications are skin
compromise over the area to be casted, heterotopic ossifica-
tion, edema, decreased circulation, severe fluctuating tone,
and inconsistent monitoring systems. The elbow, wrist
and hand, and finger joints are the most common joints
casted for clients with SCI. Casting for most of these
clients may be the last resort to regain increased ROM
before a client can begin using feeding, grooming, or com-
munication skills. Long-arm casts are used when elbow
and wrist contractures must be managed simultaneously.
If evaluation of the upper extremity reveals a pronation or
supination contracture, a long-arm cast would also be the
cast of choice. Dropout casts are used with severe elbow
flexor or extensor contractures, but the patient should be in
a position in which gravity can assist. Wrist-hand and finger
casts are indicated for contractures that prevent distal upper-
extremity function. Most commonly, a client will have a
wrist flexion-extension contracture or have finger flexor-
extensor tone and will require a cast to use the tenodesis
or individual fingers for fine motor skills. Sometimes wrist
casts with finger shells or resting hand extensions on casts
Figure 16-13 n Long opponens splint with fabricated utensil holder. are needed to ensure that the hand, fingers, and web space
are maintained in a position of optimal function. Casting is The abdominals are the primary muscles used for forced
an expensive and labor-intensive treatment modality, but if expiration in such maneuvers as coughing or sneezing. The
indicated and used appropriately it can assist a client in re- latissimus dorsi, the teres major, and the clavicular portion
gaining lost joint ROM needed for increased independence of the pectoralis major are also active during forced expira-
and function. tion and cough in the client with tetraplegia.92 Alterations
Botox may be used in conjunction with casting. In a in the function of these muscles will have an impact on
study conducted by Corry and colleagues83 tone reduction the patient’s ability to clear secretions and produce loud
was evident when botulinum toxin type A was used; how- vocalization. Gravity plays a crucial role in the function
ever, ROM and functional improvement varied among of all ventilatory muscles.89 Neural input to the diaphragm
subjects. Pierson and co-workers82 found that, with careful increases in the upright position in persons with intact ner-
selection, subjects who received botulinum toxin type A vous systems. As one moves into an upright position, the
had significant improvements in active and passive ROM. resting position of the diaphragm drops as the abdominal
Research indicates that patients who have flexor spasticity contents fall.89 The diaphragm is effectively shortened,
without fixed contracture will benefit the most. which makes generating a strong contraction more difficult.
Surgical intervention may be recommended by an ortho- With intact abdominal musculature, however, a counter
pedic physician in severe cases of joint contracture.86 Some pressure is produced and adequate intraabdominal pressure
of the more commonly used surgical options include joint is maintained, allowing the diaphragm to perform work. If
manipulation under anesthesia, arthroscopic surgical re- weakness or paralysis of the abdominal wall is present, the
leases, open surgical releases, and rotational osteotomy. client may need a binder or corset to maintain the normal
pressure relationship.69,87,93-95 Unless the SCI has affected
Prevention and Management only the lowest sacral and lumbar areas, some degree of
of Respiratory Complications ventilatory impairment is present and should be addressed in
Early management must focus heavily on preventing pulmo- therapeutic sessions.
nary complications and maximizing pulmonary function so Many treatment techniques are available to address the
the patient may perform physical activities. The clinician myriad causes of ventilatory impairment. Decreased chest
should first determine which ventilatory muscles are im- wall mobility and the inability to clear secretions should
paired. The primary ventilatory muscles of inspiration are the always be addressed. Interventions may include inspiratory
diaphragm and the intercostals. The diaphragm is innervated muscle training, chest wall mobility exercises, and chest
by the phrenic nerve at C3 through C5. The intercostals are physical therapy.69,74,90,96,97
innervated by the intercostal nerves positioned between the
ribs. If the diaphragm is weak or paralyzed, its descent will Inspiratory Muscle Training
be lessened, reducing the patient’s ability to ventilate.87-90 Inspiratory muscle training may be used to train the dia-
Accessory muscles of ventilation are primarily located in phragm and the accessory muscles that are weakened by
the cervical region.91 The accessory muscles are used to aug- partial paralysis, disuse from prolonged artificial ventila-
ment ventilation when the demand for oxygen increases, as tion, or prolonged bed rest. In the presence of significant
during exercise. Accessory muscles may also be recruited to impairments, it is generally recommended that training be
generate an improved cough effort.66 The most commonly initiated in the supine or side-lying position and progressed
cited accessory muscles are the sternocleidomastoids, the sca- to the sitting position when tolerated. When training a mod-
lenes, the levatores scapulae, and the trapezius muscles.88,89 erately weak diaphragm, gentle pressure during inspiration
The erector spinae group may also assist by extending the may be used to facilitate the muscle (Figure 16-14). Acces-
spine, thus improving the potential depth of inspiration.89 sory muscle training may be facilitated with the client in the
A B
Figure 16-14 n Diaphragm facilitation. A, Hand placement and patient positioning to facilitate
the diaphragm and inhibit accessory muscle activity. B, Firm contact is maintained throughout inspi-
ration. The lower extremities are placed over a pillow in flexion to prevent stretching of the
abdominal wall.
supine position while a slight stretch is placed on these require some residual use of mechanical ventilation even
muscles.74 The stretch is accomplished by shoulder abduc- after maximal tolerance has been achieved so as not to over-
tion and external rotation, elbow extension, forearm supina- fatigue the phrenic nerve. Other researchers are considering
tion, and neutral alignment of the head and neck. A more also pacing intercostal muscles108 or using a combination
challenging position incorporates upper thoracic extension. of diaphragmatic and intercostal pacing. There is limited
The clinician’s hands are placed directly over the muscle evidence comparing the outcomes associated with these
to be facilitated. The patient is instructed to breathe into devices in isolation or in combination therapies.
the upper chest (Figure 16-15). As the treatment progresses,
the diaphragm may be inhibited for short training periods Glossopharyngeal Breathing
by applying pressure over the abdomen in an upward direc- Glossopharyngeal breathing is another way of increasing
tion. Care must be taken to avoid excessive pressure to vital capacity in the presence of weak inspiratory mus-
prevent occlusion of vital arteries. cles.90,94,97 Moving the jaw forward and upward in a circular
As the inspiratory muscles strengthen, resistive inspira- opening and closing manner traps air in the buccal cavity.
tory devices may be used. Inspiratory devices are relatively A series of swallowing-like maneuvers forces air into
inexpensive and most function similarly. Most devices have the lungs, increasing the vital capacity. This technique has
a one-way valve that closes when the patient inspires, forc- been reported to increase vital capacity by as much as 1 L.74
ing him or her to breathe either through a small aperture Although this technique is rarely used to sustain ventilation
or against a spring-loaded resistance. Although evidence for long periods of time,109 it may be used in emergency
fully supporting this intervention remains inconclusive,98 situations and to enhance cough function. The client with
some researchers have shown improvements in total lung high tetraplegia should attempt to master this skill.
capacity99 and improved endurance measures.100 The dia-
phragm may also be trained by using weights on the ab- Secretion Clearance
dominal wall with the client positioned supine. Derrickson Ventilatory impairment occurs when the client is unable to
and colleagues101 concluded that both inspiratory muscle clear secretions.87,110 Factors such as artificial ventilation
training devices and abdominal weights are effective in and general anesthesia hamper secretion mobilization. With
improving ventilatory mechanics. Muscle trainers, however, artificial ventilation, clients may require an artificial air-
appear to promote more of an endurance effect than the way.110,111 The presence of this airway in the trachea is an
use of abdominal weights. irritant, and the client subsequently produces more secre-
tions.87 A description of various types and parameters of
Diaphragm and Phrenic Nerve Pacing ventilation is beyond the scope of this chapter. Clinicians
When the primary inspiratory muscles are no longer voli- working with clients requiring artificial ventilation are
tionally active as a result of SCI, diaphragm or phrenic nerve referred to other publications.110,112
pacing may be used to cause the diaphragm to contract. Secretions are most commonly removed by tracheal suc-
These interventions are most commonly indicated when the tioning, unassisted coughing, or assisted coughing. Re-
lesion is at or above the C3 level.101-106 Electrical stimulation cently there has been a resurgence of previously used
may be applied directly or indirectly through a vein wall or technologies that provide rapidly alternating pressures
the skin or directly to the phrenic nerve via thoracotomy. through a mouthpiece or an endotracheal tube to remove
Transdiaphragmatic pacing, in which electrodes are placed secretions. This is commonly referred to as insufflation-
laparoscopically on the diaphragm, is also an option.107 exsufflation.113 To date, conclusive research determining
Transdiaphragmatic pacing is less invasive than direct phrenic which single technique or combination of techniques achieves
nerve pacing, may be implanted and initiated on an outpa- the best outcome is not available. Insufflation-exsufflation
tient basis, and may result in improved outcomes. Both may result in fewer complications and is reported to be more
of these procedures require a reconditioning program that comfortable to the client. Barriers to implementation of these
involves extensive caregiver and client training. Many clients techniques may include expense of the equipment and com-
petency barriers in that training is required. Postural drainage,
percussion or clapping, and shaking or vibration are used
to assist with moving secretions toward larger airways for
expectoration.17,69,79
Assisted coughing is typically used with people who are
unable to generate sufficient effort.97 The assistant places
both hands firmly on the abdominal wall. After a maximal
inspiratory effort, the patient coughs and the assistant sim-
ply supports the weakened wall. A gentle upward and in-
ward force may be used to increase the intraabdominal
pressure, yielding a more forceful cough (Figure 16-16).84,97
Excessive pressure over the xiphoid process should be
avoided to prevent severe injury.
Patients may learn independent coughing techniques. In
preparation for a cough, the patient positions an arm around
the push handle of the wheelchair, opening the chest wall to
enhance inspiratory effort. The other arm is raised over the
Figure 16-15 n Accessory muscle facilitation. Hand placement head and chest during inspiration. This procedure is fol-
and patient positioning. lowed by a breath hold, strong trunk flexion, and then a
A B
Figure 16-16 n Quad coughing. A, Hand placement for the Heimlich-like technique. B, Anterior
chest wall quad coughing. The inferior forearm supination promotes an upward and inward force
during the cough.
cough (Figure 16-17).97 Another technique for independent elevating footrests or tilt-in-space wheelchairs when they
coughing is accomplished by placing the forearms over the are first acclimating to the upright position.69,74,97
abdomen and delivering a manual thrust during cough. This The client is transferred initially to a reclining or tilt-
technique is more difficult and may not provide an inspira- ing back position and progressed to an upright position
tory advantage. as signs and symptoms of medical stability allow. The
client should be monitored for evidence of orthostatic
Early Mobilization hypotension. Dizziness or lightheadedness is most com-
Getting the patient upright as soon as possible promotes mon. Ringing in the ears and visual changes also may
self-mobility and should be planned carefully. An appropri- occur. Changes in mental function may indicate more
ate seating system for pressure relief and support should be serious hypotension, and the client should be reclined
chosen. Most patients require a reclining wheelchair with immediately. Assessing blood pressure before and during
Figure 16-17 n Self-produced quad coughing. A, Full inspiratory position. B, Expiratory or cough
position.
address special considerations for functional progression

related to SCI.
Optimal neck, shoulder, and upper-extremity strength
and ROM are important factors to consider in order to
maximize functional outcomes. Neck musculature is typi-
cally painful and restricted in cervical injuries, especially
after surgical procedures. Most clients will have a cervical
orthosis in place postoperatively to prevent rotation and
flexion and extension. Cervical spine mobility may be so
limited that correcting a forward head posture is the first
goal. Soft tissue massage, manual therapy, and other mo-
dalities may be beneficial. When cleared by the physician,
the client can begin more aggressive neck exercises.
Key muscle groups in the shoulder to consider are the
scapular stabilizers and movers, which allow for humeral
Figure 16-18 n Abdominal binder. Correct placement is over flexion, adduction and abduction, shoulder internal and
the anterior-superior iliac spine and at the level of lower rib cage. external rotation, and scapular movements. Clients with
Custom corsets may be used if an elastic binder does not provide high cervical injuries have the potential for development of
adequate support to enhance vital capacity. tight upper trapezius muscles. Upper trapezius inhibitory or
scapular taping to relax the tight muscles and facilitate the
weak scapular musculature is often beneficial. In the injury
activities provides an objective measurement of the client’s levels above C7, the scapular musculature may not be fully
status. innervated, and thus positioning in the proper alignment
Because of paralysis, the abdominal wall may not support and strengthening the innervated musculature are essential.
the internal organs and viscera. In these cases an abdominal The clinician will use findings from manual muscle testing
binder or corset should be applied to all clients with lesions and the goniometric examination to determine the appropri-
above T12 to assist in venous return74,95,97; as discussed, this ate stretching and strengthening programs. Patients may
will enhance ventilatory function. If the client has a history need to begin with gravity-eliminated exercises using air
of vascular insufficiency or prolonged bed rest, wrapping the splints, bilateral slings, skateboards, and functional electrical
lower extremities with elastic bandages while applying the stimulation (FES).
greatest pressure distally may be beneficial.
Abdominal binders and corsets are fitted so that the top Activities of Daily Living and Instrumental
of the corset lies just over the lower two ribs.95 The bottom Activities of Daily Living
portion is placed over the anterior iliac spine and iliac crest ADLs include skills such as communication, feeding, groom-
(Figure 16-18). The corset or binder should be adjusted ing, bathing, dressing, bladder and bowel management,
slightly more tightly at the bottom to assist in elevating the home management, and community reentry. Instrumental
abdominal contents.69,74,97 Properly fitting the abdominal activities of daily living (IADLs) encompass multistep ac-
binder is essential. If it is placed too high or allowed to ride tivities to care for self and others,114 such as parenting,
up, ventilation may be impaired by restriction of chest wall household management, and financial management. Depend-
excursion. If placed too low, it will not provide the neces- ing on the level and severity of the SCI, patients will achieve
sary abdominal support. varying levels of independence. Most of the ADL areas dis-
The client can be transferred initially with a manual or cussed in this section will address skill levels with a complete
mechanical lift. Lift systems may be advantageous because injury. Activities should be graded differently for an incom-
they allow total control of the client and give the assistant plete injury after completion of ASIA and manual muscle
more time to ensure that monitoring devices, lines, or tubes testing. For purposes of this discussion we will use terms
attached to the client remain intact. Lift systems may be used in the FIM.
freestanding hydraulic lifts or electronic devices or may be Patients with high-level tetraplegia (C1 to C4) will be
mounted on the ceiling. dependent in most ADLs as well as IADLs but will be able
Once the client is out of bed, a weight shift or pressure to verbalize how to safely perform all skills. Patients with
relief schedule is immediately established. Initially, weight low-level tetraplegia (C5 to C8) may achieve some level of
shifts are performed at 30-minute intervals and modified ac- independence, but this will vary according to the amount of
cording to skin tolerance.67 A timer may be issued to ensure intact musculature and the patient’s body shape and weight,
reminders for weight shifts. This is particularly important if age, and motivation level. The ability of these patients to
the client has cognitive deficits. The skin is inspected thor- achieve maximum independence in all areas of ADLs may
oughly before and immediately after out-of-bed activities. be accomplished only through the use of appropriate ortho-
Total sitting time is progressed according to tolerance. ses or adaptive equipment. See Table 16-4 for functional
expectations and Table 16-6 for orthotic indications.
REHABILITATION: ACHIEVING FUNCTIONAL Patients with injuries at the C5 or C6 level are especially
OUTCOMES challenging in this area of rehabilitation. These patients
Once secondary complications are managed and the client must have biceps function and adequate elbow ROM before
is able to tolerate out-of-bed activities, more aggressive any ADL goals can be achieved. To achieve these goals,
functional training begins. The following information will patients also need to work toward supporting their body
TABLE 16-6 n UPPER-EXTREMITY ORTHOTICS
SPLINT LEVEL OF SPINAL CORD INJURY RATIONALE

DYNAMIC ORTHOTICS
Mobile arm support Weak C5 Function
(ball-bearing Incomplete injuries Assists in reaching in horizontal and vertical planes
feeder) Also indicated with shoulder weakness Increases functional ROM and strength
(internal-external rotator muscle Independence with feeding and hygiene after setup
grades 22 to 3/5; bicep-supinator Provides support to allow correct movement patterns
muscle grades 22/5)
Overhead rod and Weak C5 Function
sling Incomplete injuries Increases functional ROM and strength
Also indicated with shoulder weakness Independence with wheelchair driving after setup
(internal-external rotator muscle Independence with feeding and hygiene after setup
grades 3 to 31/5; bicep/supinator Provides support to allow correct movement patterns
muscle grades 3/5)
STATIC SPLINTS, CASTS, AND ORTHOTICS
Resting hand splint C1-C7 Position
Prevent joint deformity
Preserves web space
Preserves balance with intrinsic and extrinsic musculature
Intrinsic plus splint C1-C7 Position
Same as resting hand splint but places finger MP joint in more flexion
Long term, allows better tenodesis alignment of first digit and thumb
Elbow extension C5-C6 Position
splints, bivalve cast Prevents elbow contracture from muscle imbalance and/or hypertonicity
Rolyan TAP splint C5-C6 Position
(prefabricated) Provides constant low stretch
Use with muscle imbalance and/or mild hypertonicity
Dorsal wrist support C5 Function (e.g., slot for utensils) and position
splints Prevents severe wrist drop and ulnar deviation
If positioning is needed long term, may consider permanent splint
fabricated by orthotist
Long opponens C5 Position and function
splint Can be dorsal or volar
Prevents wrist drop and ulnar deviation
Preserves web space and supports thumb, reducing subluxation
Slot may be fabricated for function
Wrist cock-up splint C5 Position and function
Incomplete injuries Supports wrist in slight extension
Allows finger movement for incomplete injuries
Short opponens C6-C7 Position and function
splint Supports thumb to prevent subluxation
Improves tenodesis and prehension
Tenodesis brace or C6-C7 Function
splint Enhances natural tenodesis in either tip-pinch or lateral pinch
May consider permanent splint fabricated by orthotist
MP block splint C8-T1 Position
Prevents “claw hand” or hyperextension of the MP joints
Protects weak intrinsic musculature
MP, Metacarpophalangeal; ROM, range of motion.
weight with simultaneous extension of the shoulder, elbow, wheelchair positioning with lap trays, armrests, wedges, or
and wrist, otherwise known as propping (see Figure 16-35, lateral trunk supports is important to maximize function for
A to C). Elbow positioning devices such as pillow splints, persons with C5 or C6 injuries.
casts, or resting splints enhance alignment. Other orthotics Patients with a C7 or C8 level of injury generally will not
to consider for maximizing function include definitive wrist prove to be as challenging for the rehabilitation therapist.
supports and mobile arm supports (MASs)115 or short op- With the presence of triceps, the ADL skills are easier to
ponens splints if the patient has wrist extension. Appropriate achieve. Most patients, given the right body type, will be
able to achieve these goals with only minimal assistance that would affect feeding, but they may have weak wrist
from a caregiver. function. Some of the dorsal wrist supports have a cuff built
Patients with paraplegia usually achieve total indepen- in that can be functional. The patient with no finger func-
dence with communication, feeding, and grooming. These tion can use a wrist-driven tenodesis brace for managing
patients may need adaptive equipment to perform some objects or to hold a feeding utensil (Figure 16-21). A uni-
of these IADL and ADL skills; however, they should be able versal cuff can be worn on the hand to hold feeding utensils
to be performed without assistance from another person. (Figure 16-22, A). The patient with weak finger function
Endurance is a major concern for the patient’s independence can use built-up handles on the utensils. There are also
while performing ADLs. Some skills require a considerable commercially available and esthetically pleasing utensils
amount of time and effort. If endurance becomes a factor, such as those in Figure 16-22, B. Cutting can be difficult for
patients should choose to perform some activities while patients without finger function.
receiving assistance for other skills that are too challenging
or time-consuming. Grooming
The basic components of grooming are washing the face,
Feeding combing or brushing the hair, performing oral care, shav-
Patients with C1 to C4 tetraplegia are dependent in feeding ing, and applying makeup. More advanced grooming ac-
but can verbalize this skill. Patients with C5 SCI with weak tivities may include nail care, donning and doffing of
shoulders and biceps musculature require a dynamic ortho- contact lenses, or other hygiene tasks specific to the indi-
sis to support the upper extremity during feeding. The most vidual. Individuals with C1 to C4 tetraplegia are dependent
common orthoses used are the MAS115,116 (Figure 16-19) but can verbalize these skills. Patients with C5 injuries
and the offset feeder (Figure 16-20). Patients with low- perform these skills with some assistance but may require
level tetraplegia may not have weakness in the shoulder orthotic devices, such as an MAS or offset feeder for
shoulder support and a splint for wrist support. Patients
with low-level tetraplegia may need cuffs or built-up
grips on razors, brushes, and toothpaste to be independent
(Figure 16-23). A proper bathroom setup for optimal wheel-
chair positioning is important for all patients. Patients with
tetraplegia often rely on the support of the elbows as an
assist, so sink height should be considered. The proper
positioning and adaptive equipment will be the difference
between independence and dependence in these skills
(Figures 16-24 and 16-25).
Bathing
Bathing includes washing and rinsing the upper and lower
extremities and the trunk. Patients with C1 to C4 tetraplegia
are dependent in bathing but are instructed to verbalize this
skill. Patients with C5 injury can range from requiring maxi-
mal assistance to being dependent in bathing. Patients with
low-level tetraplegia bathe with moderate assistance to total
independence with use of adaptive devices. Patients with
Figure 16-19 n Mobile arm support (MAS) used during feeding. paraplegia are typically independent in bathing but may need
Figure 16-21 n Tenodesis braces have varying grasps. The larg-

Figure 16-20 n Offset feeder orthosis. est grasp position allows the client to hold a soft drink can.
A B
Figure 16-22 n A,Universal cuff used for feeding. B, Dining with Dignity is one commercially
available type of flatware for individuals with impaired grip.
adaptive devices. After examination of the patient’s upper-

extremity strength, balance, spasticity, body type, endurance,
and home accessibility, the therapy team can determine
the appropriate bathing equipment and setup for the patient
(Figure 16-26). Patients with limited upper-extremity and
trunk strength may need straps to assist with trunk support
and adaptive cuffs to control the hand-held shower head.
Basic bathing safety should be taught to all patients. Bathing
safety includes checking the water temperature with a
known area of intact sensation, skin checks before and after
bathing, and skin protection during the transfers. These pre-
cautions are necessary to prevent burns and skin breakdown
during the bathing process.
Figure 16-23 n Simple razor adaptation that helps turn razor on
and off with a grosser motor movement. Dressing
Dressing includes dressing and undressing the upper and
lower extremities with clothing that fits the patient’s premor-
bid lifestyle. Patients with C1 to C5 tetraplegia are dependent,
Figure 16-24 n A, A client with a C5 spinal cord injury is able to brush his teeth with use of a
cuff, adapted long straw, and proper wheelchair positioning at the sink. B, Client with C6 spinal cord
injury uses bilateral tenodesis to support toothpaste while holding a toothbrush in his mouth.
Figure 16-27 n Client with low-level tetraplegia maintains bal-

ance while performing lower-extremity dressing in bed.
Figure 16-25 n Sink height can be important in assisting this

client with C6 spinal cord injury to brush his hair.
Figure 16-26 n Bathroom setup with shower or commode chair Figure 16-28 n Early practice when dressing in the wheelchair
and hand-held shower head. may involve leaning on a surface to assist with this skill.
but they can verbalize safe techniques to perform all the dress- cleanup of self. Water or video urodynamic studies are per-
ing skills. Independence in this skill for patients with low formed to determine the patient’s bladder status and the
tetraplegic and paraplegic injuries may depend on where most optimal bladder training program. Patients often enter
the skill is performed (e.g., mat, bed, or wheelchair). Patients the rehabilitation program with an indwelling catheter as
with low-level tetraplegia can perform upper body dressing their bladder management program. The indwelling catheter
and undressing independently with equipment such as a but- should be removed as soon as possible because it puts the
ton hook, hook and loop fasteners (Velcro), or adapted loops. patient at risk for chronic urinary tract infections.117
Lower-body dressing is usually performed in bed (Figure 16-27) On the basis of injury level, patients have either a reflex-
versus the wheelchair because of endurance, strength, and ive bladder (upper motor neuron lesions) or an areflexive
body type issues. Patients with paraplegia are expected to bladder (lower motor neuron lesions).69 The reflexive blad-
dress with total independence in the bed, but they may need der reflexively empties when the bladder is full. The thera-
equipment such as a leg lifter or a long-handled shoehorn for peutic goals for managing the reflexive bladder include
dressing in the wheelchair (Figure 16-28). This should be low-pressure voiding and low residual urine volumes. The
encouraged, if possible, for independence in the community. nonreflexive bladder will not empty reflexively and needs
to be manually emptied at regular intervals. The goals for
Bladder Management managing the areflexive bladder include establishing a regu-
Bladder management includes determining and performing lar emptying schedule and continence between emptying.
the bladder program, clothing management, body position- Management of an areflexive bladder includes performance
ing, setup and cleanup of equipment, disposal of urine, and of intermittent catheterizations.
Patients with C1 to C5 tetraplegia are typically dependent process, and in this situation the rectum should be emptied
in their bladder programs. An automatic leg-bag emptier can before suppository insertion.119 If the established bowel
assist with just the elimination component of the bladder program is not followed consistently, involuntary bowel
skill; however, the patient will still be dependent in all of the movements or impaction may occur.
other components of bladder management. Male patients Bowel management training must begin as soon as the
with injuries at C6 level and below may be able to complete patient is medically stable. The components of bowel man-
portions of the bladder management. Patients with limited agement include clothing management, body positioning,
hand function may need adaptive devices such as orthoses to setup and cleanup of equipment, performance of the bowel
assist with catheter insertion, adaptive scissors to open blad- program, disposal of feces, and cleanup of self. To establish
der packages, leg bags with flip-top openers, and leg bag the most effective bowel training program, the interdisci-
loops (Figure 16-29). Female patients with paraplegia will plinary team must work together. The team will need to
most likely need to begin their training in bed with a mirror discuss patient medications that may affect the bowels, the
to obtain the most ideal position. Touch technique can be time of day when the patient plans to perform the program,
taught so they will not be reliant on a mirror if they have the physical appropriateness related to scapular strength and
good finger sensation and use, and they may progress to us- endurance, and all equipment that will be used.
ing the touch technique in a wheelchair. Some people with Patients with injury above the C6 level will be dependent
SCI may decide to have a suprapubic catheter placed or a in performing the bowel program; however, they should be
bladder augmentation procedure as a lifestyle choice. independent in the verbalization of the technique. Patients
with limited hand function (C6 to C7) may require a digital
Bowel Management bowel stimulator and a suppository inserter with an adapted
The goal of bowel management is to have the patient able to cuff or splint (Figure 16-30). In addition, a roll-in shower
predictably induce regular elimination. As described under chair or upright shower or commode chair with a padded
bladder management, the level of injury will assist in telling cutout in the seat will allow the patient to reach the buttock
if the patient will have either a reflexive bowel or a nonre- area to perform the stimulation. For this level of injury, it
flexive bowel.69 The bulbocavernosus reflex (BCR) is elic- may be advantageous to perform the bowel program in con-
ited by pinching the dorsal glans penis or by pressing junction with the shower to conserve energy with transfers.
the clitoris and palpating for bulbocavernosus and external For individuals with paraplegia, full independence is ex-
anal sphincter contraction.118 If the patient has a positive pected for completion of all bowel management skills.
BCR, this is indicative of a reflexive bowel. With a reflexive These programs are typically performed on appropriate
bowel, tone of the internal and external anal sphincter is bathroom equipment or the bed.
present although the patient will not feel the need to have a To increase the effectiveness of the bowel program the
bowel movement. Voluntary anal contraction and relaxation patient should follow the guidelines identified in Box 16-2.
are not possible, but the nerve connection between the colon
and the spinal cord are still intact, allowing the patient to
reflexively eliminate stool. This can be done with chemical
or mechanical stimulation.118
Flaccid bowel programs are much more difficult to regu-
late because there is no internal or external anal sphincter
tone. Timing and diet are critical for the success of this
program. A suppository may be required to assist with the
Figure 16-30 n Dil stick and suppository inserter with adaptive cuffs.
BOX 16-2 n GUIDELINES FOR BOWEL

PROGRAM
1. Perform the bowel program at the same time each day.
2. Follow a diet high in fiber (25 to 35 g recommended).
3. Drink at least eight glasses of water per day.
Figure 16-29 n Bladder management supplies may include knee 4. Drink a hot liquid 30 minutes before initiating the
spreader with mirror, sterile catheter kit, catheter inserter, leg bag bowel program.
with tubing and adapter, catheter, “HouseHold” for positioning, 5. Perform the bowel program in an upright position.
bungee cord to hold pants, pants holder, small prelubricated female 6. Consider premorbid bowel schedule.
catheter.
Home Management (SGDs) formerly known as augmentative and alternative

Home management may be divided into two components: communication devices.
light home management and heavy home management. Many specialty facilities that treat large numbers of SCI
Light home management includes managing money, prepar- patients will have specialized therapists called Assistive
ing a snack in the kitchen, doing laundry, and making Technology Practitioners (ATPs) and/or AT departments to
the bed. Heavy home management includes shopping for specifically assess, select, and train in the use of device(s)—
groceries, preparing a complex meal in the kitchen, dusting, specifically seating, driving, and electronic access. Seating
and vacuuming. The clinician should discuss the role the departments will evaluate and prescribe customized manual
patient would like to assume at home. The patient may want or powered mobility seating systems. This is essential as
to resume previous home management roles or may want to professionals struggle to assist the patient with reimburse-
discuss changing roles with a family member or caregiver to ment for the mobility devices as well as the always-changing
have energy for other skills. technology. Not everyone has access to someone with this
Patients with C1 to C5 tetraplegia will be dependent in specialized certification; thus consideration should be given
home management. Patients with limited or no hand func- by the therapist in the evaluation phase to include where the
tion will need adaptive kitchen devices, adapted utensils, patient will use the chair and how it will get there, looking
and adapted cleaning equipment. Preplanning activities may at the “big picture” rather than only the mobility device. For
be essential for independent function with patients at all example, how will the wheelchair be transported? Can the
levels of injury. Patients with hand function may require ex- wheelchair be locked down so the patient may drive in a van
tended handles on equipment and must incorporate energy or be a dependent passenger? Can the patient load the
conservation techniques. manual chair into his or her car (Figure 16-31)? There are
specially trained practitioners called driving rehabilitation
Parenting specialists who specialize in recommending equipment and
Research has shown the importance of parenting behavior transportation of the patient as a passenger in addition to
and attitude on children’s ability to adjust to various cir- providing driver education and driver training. They help to
cumstances. This adjustment is not, however, affected by evaluate and to assist with making correct vehicle modifica-
the disability status of a parent.120,121 Patients with C1 to tions and adaptive equipment choices for the patient as a
C5 tetraplegia may be dependent in the physical aspects driver. There are over 600 specialists in the United States
of parenting. Patients with low-level tetraplegia may be represented in each of the 50 states. A professional can be
able to participate in the more physical aspects of parent- found on the Association for Driver Rehabilitation Special-
ing with some level of adaptations such as a wheelchair- ists (ADED) website (www.drivers-ed.org).125 When con-
accessible table with sides to change an infant.122 Parenting sidering a sedan, these specialists will evaluate the patient to
skills for a patient with paraplegia would depend on the see how he or she operates primary and secondary vehicle
environment and the specific activity being performed as controls, opens and closes the door, transfers into the vehi-
well as the mobility of the individual. Therapists can pro- cle, and stores, secures, and retrieves the wheelchair. If the
vide advice and ideas to assist with selection of parenting patient is unable to perform any of those tasks then a van
and baby equipment as well as be a resource in discussing may be an option. Modifications can allow a person to trans-
and adapting equipment options such as slide-down cribs, fer to the van’s driver seat or to drive from the wheelchair.
adjustable-height high chairs, carrying slings or supports, The driving control technology that is available to compen-
and baby strollers that can be more easily pushed with sate for reduced strength or ROM includes steering systems,
one hand.122,123 hand controls, and reduced effort and zero effort steering
and braking. The rehabilitation specialist will provide a
Assistive Technology comprehensive evaluation to determine the patient’s ability
AT can be helpful in letting people resume more indepen- to drive. That evaluation will consider visual, perceptual, and
dent lives in areas of self-care, work, and recreation. AT functional abilities as well as reaction time and behind-the-
is defined by the 1998 Technology-Related Assistance for wheel assessment. In both sedan and van selection, it is
Individuals with Disabilities Act (Public Law 105-394). It always best to recommend that the patient consult the driv-
defines AT devices as “any item, piece of equipment, or ing rehabilitation specialist before purchasing and modify-
product system, whether acquired commercially, modified, ing the vehicle.
or customized, that is used to increase, maintain, or improve In this day of ever-changing technology, the patient may
functional capabilities of individuals with disabilities.” need assistance to access many electronic devices, such as
Hedrick and colleagues124 found that in both civilians computers, televisions, lights, call systems, personal digital
and veterans the most frequently used AT devices were assistants (PDAs), cell phones, music systems, or digital
(1) manual mobility and independent living devices (e.g., readers or the not-yet-invented device that will be coming on
manual wheelchairs, manual exercise equipment, manual the market in the future. Dexterity is required to operate
motor vehicle control devices such as a steering knob, walk- many standard devices such as computers. There are many
ers, reachers); (2) powered mobility and independent living “off-the-shelf” adaptations using universal designs to enable
devices (e.g., power lifts, power doors, motorized wheel- persons with limited dexterity to use a computer. A person
chairs, power-assisted motor vehicle operation devices); with a higher tetraplegic level of injury can use alternative
(3) prosthetics and orthotics (static and dynamic, such as methods to access a computer, such as a microphone with
splints, mentioned earlier); (4) alternative computer access speech recognition software, pneumatic controls (sip-and-
devices, which may be as simple as a typing splint to as puff devices), or an eye gaze system. The most popular and
complex as brain control; and (5) speech-generating devices inexpensive way to access a computer is speech recognition
A B C D
E F G
Figure 16-31 n Car transfer. Most patients with a paraplegic level of injury are modified indepen-
dent (FIM level 6) in the performance of a car transfer. The patient approaches the car on the driver’s
side and opens the door. A, After stabilizing the wheelchair, he may place his foot or feet into the
car or leave them on the footrest or the ground. B, He performs a depression-style transfer onto the
seat of the car; C, positions his lower extremities appropriately inside the car; and D, prepares to get
the wheelchair into the car by removing the wheels (quick release) and cushion and placing these on
the floor in the front passenger area or in the back seat. E to G, The rigid model of wheelchair is
folded and transferred across the patient onto the passenger seat. Transferring out of the car is the
reverse process, beginning with getting the wheelchair out of the car and reassembling it.
software. However, the computer-brain interface is a newer tutorials on both the Microsoft and the Apple websites.
technology currently in clinical trials and uses intact brain Manufacturers do not always consider how persons need-
function to address these needs. In recent years computer ing adaptations will access their device. Cell phones and
companies have been more sensitive to the population with all commercial Bluetooth technology still requires some
disabilities. The operating systems have adjustments that touch to activate; however, some vendors have made
make the keyboard easier to use, referred to as Ease of modifications available, although they are expensive. Sur-
Access or Universal Access features. These are very helpful face touch screens are not disability friendly, and many
for someone who may be entering commands with a single phones and music systems use that technology. The thera-
point such as a mouth stick or a pointer on only one hand. pist’s role is to help assess, decide, and adapt how the
This adjustment can change how the keyboard works or can access should be achieved for the patient, taking advantage
provide an on-screen keyboard. There are shortcuts that of whatever the patient has to use. EADLs such as call
allow the patient to do everything with the keyboard, thus systems or computer systems can be adapted using pneu-
eliminating a mouse, which may be difficult for a mouth matic controls (sip-and-puff devices) or voice-activated
stick user. Many of these keyboard commands are not controls for independence from the bed and the wheel-
needed if the individual uses an already built-in speech chair. There are switches that can be activated with head
recognition program or purchases one. There are detailed or eye control, allowing patients with little movement the
ability to communicate. Patients with C3 to C4 injuries,

depending on neck strength and ROM, can use lower-tech
equipment such as mouth sticks for pushing cell phone
buttons from the wheelchair in addition to the head rest
buttons or a sip-and-puff switch (Figure 16-32). Patients
with C5 injuries begin to use their biceps, deltoids, and
internal and external rotator strength to interact with their
environment. Positioning of the buttons, devices, or mounts
for devices is important for these patients, who may or may
not be using an MAS. Adaptive splinting for support at
the wrist can allow these patients to use their upper arms
in writing, typing, turning pages, and using computers
(Figure 16-33). Patients with wrist function but no finger
function can use utensil holders with a pointer to “dial” a
phone number, for example, or can use their natural teno-
desis to grasp and manipulate objects (Figure 16-34). For Figure 16-34 n Tenodesis brace writing by use of a pen with a
injuries at the T1 level and below, interaction with the built-up grip.
environment in all areas should be independent.
Mobility
Bed Mobility and Coming to Sit
The components of bed mobility include rolling side to side,
rolling supine to prone, coming to sit, and scooting in all
directions while either long or short sitting. Initial training
for bed mobility is usually conducted on the mat, as it is
easier to learn on the firmer surface. When skills on the mat
are mastered, the patient can be progressed to a less firm
surface, such as the bed. Bed mobility is a challenging skill
for clients with tetraplegia to learn because of their limited
upper-extremity strength (Figure 16-35, A to C).7,62 To
accommodate for the loss of upper-extremity musculature,
compensatory strategies and assistive devices, such as bed
loops, may be used (Figure 16-35, F to I). Clients with
paraplegia often master bed mobility skills quickly and
much more easily than clients with tetraplegia because of
Figure 16-32 n Mouth stick writing can be accomplished with their intact upper-extremity musculature.
the client upright in the wheelchair and with the support of a bed-
Pressure Relief in the Upright Position
side table and bookstand.
The client with high tetraplegia achieves independent pres-
sure relief in the wheelchair through appropriately pre-
scribed specialty controls. For example, a pneumatic control
switch may be used to activate the recline mode of a power
wheelchair (Figure 16-36). When the client is unable to
operate a specialty switch, an attendant control may be used.
When powered options are not feasible because of cognitive
deficits, financial limitations, or other reasons, a manual
recliner (Figure 16-37) or tilt wheelchair is used. When
clients are dependent in performing pressure relief, they can
be taught to instruct others in this skill. Clients with mid-
and low-level tetraplegia are taught to perform a side or
forward lean technique for pressure relief if the strength of
the shoulder musculature is appropriate (Figure 16-38). The
client with paraplegia is usually taught to perform a pushup
(depression) for pressure relief (Figure 16-39).
The appropriate time to maintain the change in position
is usually 60 seconds at intervals of 30 to 60 minutes. The
treatment plan should include instructing the client in ways
to ensure that the schedule for pressure relief is maintained
in all settings. The use of watches, clocks, timers, and atten-
Figure 16-33 n Long Wanchick writing device. dant care may be necessary.
A B C
D E F
G H I
Figure 16-35 n Bed mobility and coming to sit. A, The patient rolls from supine to side-lying
position. B, He progresses to supporting his weight through the downside elbow and shoulder. C, He
pushes up onto extended arms. D, While shifting his weight onto the left arm, he unweights the right
arm and hooks his right hand behind his right knee, gaining enough leverage to push and pull himself
toward upright in a long sitting position. E, He continues to shift his weight to the right until he gains
a balanced sitting position with his weight forward over his extended legs. Supine to sitting: F and
G, Starting from a supine position on a bed or a mat, the arms are extended and the hands positioned
under the buttock or in the curve of the back (lumbar spine); the head is lifted; and leverage is used
to pull up until the upper body weight is supported on bilateral elbows. H, The weight is shifted from
right to left or vice versa, and the elbows are extended to support the upper body weight. I, While
the elbows are kept extended, the hands are carefully walked forward until balanced long sitting has
been achieved.
Wheelchair Transfers environment (i.e., car transfer). However, the hydraulic lift
The physical act of moving oneself from one surface to may not be the method of choice because the lift is bulky,
another is described as a transfer. Wheelchair transfers difficult to store, and awkward to transport. Pivot transfers or
may be accomplished in many different ways. The type of manual lifts may be used because of client or caregiver pref-
transfer used by a client is determined by the injury level, erence or when clients are smaller in stature and when other,
assistance needed, client preference, and safety of the more costly lift systems are not available to the individuals.
transfer. When performing transfers, both the client and the Transfers can be performed with the use of a transfer
person assisting must give attention to the use of appropri- board, depression-style, or via the stand or squat and pivot
ate body mechanics. method. The mechanics of teaching an assisted transfer to a
Dependent transfers may be accomplished with an elec- client with C7 tetraplegia is depicted in Figure 16-40. The
tric (power) lift, hydraulic lift, manual pivot, transfer board, client is taught to position the wheelchair, position the trans-
or manual lifts, which may require two or three people. A fer board, use correct body mechanics to get the best lever-
transfer with an overhead power lift is the least physically age to effect movement in the desired direction, remove the
challenging on the part of the caregiver; however, these lifts board, and position his or her body appropriately.7,62
are costly and are not easily transportable. The use of a hy- Wheelchair transfers are performed on many different
draulic lift may be desirable if funding is not available for surfaces. The training procedure begins with the easiest trans-
a power lift or the transfer needs to be done in an outdoor fer and progresses to the more difficult transfer. Instructions
Figure 16-36 n The pneumatic control (sip-and-puff straw) is

usually ordered on a power reclining or tilt-in-space wheelchair for
patients with injury levels above C6. The straw is removable, and
several are supplied with the wheelchair. The straw is attached to a
flexible arm, and it is adjustable to different heights and angles to
fit the needs of the patient.
B
Figure 16-38 n A, Pressure relief: side lean. The tetraplegic patient
with C6- to C7-level injury may use a side lean to achieve pressure
relief over the ischial tuberosities. The patient hooks one upper
extremity around the push handle of the wheelchair on one side and
leans away from the hooked upper extremity until the ischium on the
hooked side is clear of the wheelchair cushion. The position is main-
tained for 1 minute and repeated on the other side. B, Pressure relief:
forward lean. The forward lean method of pressure relief is used for
many different injury levels. The subject must have adequate range
of motion at the hips and in the lumbosacral spine to allow the ischia
to clear the wheelchair cushion at the end range position.
Figure 16-37 n The manual reclining wheelchair is a piece of du- for wheelchair transfers usually begin on level surfaces
rable medical equipment that is prescribed on a temporary or a per- and progress to uneven surfaces as individual strength and
manent basis. The back of the wheelchair fully reclines, and the skill allow.7,62 Given these two principles, the following
legrests elevate to allow for effective pressure relief while the client is list is an example of how one might proceed with transfer
out of bed. Other features of the wheelchair are desk armrests, which training:
may be adjustable in height; a removable headrest; and removable 1. Mat transfer (see Figure 16-40)
legrests. The wheelchair folds and may be transported in a vehicle. 2. Bed transfer
A newer wheelchair that combines the benefits of a manual

wheelchair with a power wheelchair is the pushrim-assisted
power assist wheelchair (Figure 16-45).126 This wheelchair
may be best suited for clients who have some upper-extremity
weakness, joint degeneration, upper-extremity pain from pro-
pelling a manual wheelchair, or reduced exercise capacity or
endurance. This type of wheelchair could potentially delay
secondary injuries of manual wheelchair users.127 Both power
and manual wheelchair mobility training begins on level sur-
faces. When a client is instructed on how to propel a manual
wheelchair, it is suggested that a semicircular pattern be used
to reduce the trauma to the upper extremities.127 Wheelchair
gloves are beneficial in reducing friction over the palms of the
hands during propulsion (Figure 16-46). Research evidence is
available that demonstrates the safety and superior efficacy of
a formal approach to wheelchair skills training of wheelchair
users and their caregivers. The Wheelchair Skills Program
(WSP) is one example of such a program and is available free
on the Internet.128 This program includes useful evaluation
and training tools to help practitioners translate this research
evidence into clinical practice.129
Training progresses toward more difficult skills as
follows:
1. Mobility on level surfaces in open areas
2. Setup for transfers
3. Mobility in tight spaces
4. Mobility in crowded areas
5. On and off elevators
6. Up and down ramps
7. Through doors
8. Wheelies (Figure 16-47)
9. Negotiation of rough terrain
10. Up and down curbs and steps (Figures 16-48 and 16-49)
Figure 16-39 n Pressure relief: depression. This method of
pressure relief is consistent with a full pushup in the wheelchair. Ambulation Considerations—Orthotic
Most patients with a paraplegic level of injury and some patients Disposition
with a low tetraplegic injury level are able to perform this method “Will I ever walk again?” is a question often asked during
of pressure relief. SCI rehabilitation. The team must be empathetic toward and
acknowledge the client’s goals for ambulation, and the sub-
ject should be discussed openly. The professionals involved
3. Toilet transfer in the care of the patient must be careful not to take hope
4. Bath transfer away from the client. Hope is important to maintain positive
5. Car transfer (see Figure 16-31) survival skills in SCI rehabilitation.
6. Floor transfer (Figures 16-41 to 16-43) When ambulation is an appropriate goal, the treatment
7. Other surfaces (e.g., armchair, sofa, theater seat, pool) program may be short and relatively uncomplicated for
some and extremely laborious for others. Treatment tech-
Wheelchair Mobility Skills niques may include therapeutic exercise, biofeedback, neu-
Instructions in the safe and appropriate use of the wheel- romuscular stimulation, locomotor training (discussed later
chair may begin before getting the client out of bed by in this chapter), balance training, standing, and various other
orienting the client to the wheelchair and its component pregait and gait activities. The clinician must consider the
parts. postdischarge environment and include those surfaces in
Ideally, a power reclining or tilt wheelchair is supplied training.
for clients with C1 to C5 tetraplegia to promote maximal The walking disposition of patients with incomplete SCI
independence. The most common drive-system options is challenging owing to the complexity of problems and
available for these clients include, but is not limited to, chin varying degrees of impairment. These patients may have
drive, pneumatic systems (see Figure 16-36), and head con- pain, ROM limitations, ventilator pump dysfunction, weak-
trol (Figure 16-44). A client with mid- to low-level tetraple- ness, and spasticity, as well as sensory and balance dys-
gia may be instructed in the use of both power and manual function. In addition, their premorbid physical condition
upright wheelchairs. The client with paraplegia is instructed must be considered. Musculoskeletal asymmetries, such as
in the use of a manual upright wheelchair unless there are muscle shortening on the stronger side and lengthening on
extenuating circumstances. For example, a power wheel- the weaker side, may lead to pelvic obliquity and scoliosis.
chair is appropriate for a client who is 50 years old and has A team approach to orthotic prescription is desirable to
severe rheumatoid arthritis. meet the needs of patients with incomplete SCI. Even if
A B C D
F G
E
Figure 16-40 n Wheelchair to mat transfer using a transfer board. A, The patient positions the
wheelchair at a 20- to 30-degree angle to the surface to which he is transferring and positions the
board with or without assistance. B, The patient moves forward in the wheelchair to clear the tire in
preparation for lateral movement on the transfer board. C, To achieve the appropriate mechanical
leverage, the patient is instructed to twist the upper body and look over the trailing shoulder (D). He
pushes and lifts to effect movement across the board. E, When the client has achieved a safe position
on the transferring surface, the transfer board is removed. F and G, The patient is helped to get his
feet onto the surface.
A B
Figure 16-41 n Floor transfer. The independent performance of a floor transfer is a goal for most
patients who have a paraplegic level of injury. The patient may use different techniques to get onto
the floor. Forward floor transfer: A, The patient positions his feet off the footrest and moves forward
onto the front edge of his cushion. B, He reaches for the floor, first with one hand then with both, and
C D
Figure 16-41, cont’d C, lowers his knees to the floor. D, He advances his hands forward until his
body is clear of the wheelchair.
A B
C D
Figure 16-42 n Floor transfer sideways. After moving to the front of the wheelchair seat, (A) the
patient leans to the left and reaches for the floor and (B) shifts his weight toward the left arm. C and
D, He balances his weight between both arms and in a very controlled manner lowers his body to
the floor.
A B
C D
Figure 16-43 n Forward lowering floor transfer. A, This trans-

fer method begins from a balanced position on the front edge of
the wheelchair seat with feet on the floor. B, Hips are lifted off the
seat forward enough to (C) lower the buttocks to the footrest.
Note that this requires significant strength and control through the
upper body as well as (D) excellent range of motion in shoulder
extension and a reasonably loose anterior shoulder capsule. E, Legs
are moved forward for balance. Also, a small pillow or cushion
(not shown here) can be used to pad the footrest to protect the
E
patient’s skin.
orthotic devices enable these patients to become indepen- bracing to achieve the maximum amount of function. It also
dent with standing or walking, the energy costs, joint dete- anticipates changes in each patient’s clinical picture. For
rioration, and muscle stresses over the life expectancy of example, braces may be manufactured with joints built into
each individual need to be considered. Orthotic prescription the plastic, or joints that begin as fixed, and are later cut to
should be approached systematically. The patient’s goals, allow articulation. Some models of knee-ankle-foot orthoses
funding, premorbid and current health status, social sup- (KAFOs) can be altered to become ankle-foot orthoses
port, and the environment to which they are returning (AFOs).131
should be considered. A basic clinical algorithm for the The philosophy regarding the use of orthoses for ambu-
selection of orthoses for persons with neurological impair- lation for individuals with complete paraplegia varies
ment has been proposed by researchers at Rancho Los greatly among rehabilitation centers. Some facilities en-
Amigos Medical Center. This algorithm is referred to as courage ambulation for these individuals, whereas others
the Rancho ROADMAP (Recommendations for Orthotic strongly discourage it, given that only a small percentage
Assessment, Decision-Making, and Prescription).130 Suc- of these clients continue to use orthotics after training has
cessful brace prescription uses the minimum amount of been completed.132,133
Figure 16-46 n Para Push gloves. Wheelchair gloves, mesh

back, open fingers, and leather-padded palm. Usually appropriate
for patients with paraplegia-level injuries. Available from multiple
suppliers.
Figure 16-44 n Head array is a headrest and head control for
driving wherein the position of the head activates the drive control
of the wheelchair. It is appropriate for persons with high cervical
injuries. The head array switches can be adjusted to the individual’s
specific needs relative to their active range of motion and control
of their head. The switches are embedded in the headrest panels
and not only control driving but can also control activation of other
devices for environmental control. The side panels of the headrest
can be straight or curbed depending on the needs of the patient.
Additionally, the head array can be sized for either adult or pediat-
ric patient.
Figure 16-45 n Power assist system. The Xtender by Sunrise

Medical is one of three power assist systems available. The motors
are in the wheels. The battery extends off the back of the wheel-
chair. There is a connection between the motors in the wheels. The
system is added to a manual wheelchair. There are two power assist
levels. Figure 16-47 n A wheelie is a functional mobility skill that
enhances functional independence. The performance of a wheelie
is a precursor to negotiating steep ramps, curbs, steps, and rough
terrain.
A B
Figure 16-48 n A, Descending a curb is an advanced wheelchair mobility skill. This man with
AIS A, T12 paraplegia assumes the balanced wheelie position and approaches the curb in a forward
position. The wheelie position is maintained as he rolls off the curb. B, Climbing a curb with assis-
tance is also an advanced skill. This is the same man as in A. He “pops” into a wheelie and advances
his wheelchair to move his casters up onto the curb. He then reaches back to his wheel, leans
forward, and pushes as the helper assists by lifting the back of the chair. A more advanced skill would
be to perform this activity by approaching the curb with speeds fast enough to gain momentum,
“pop” a wheelie, and advance up and over the curb in one continuous movement (not shown). The
curb height, strength, level of injury, and body composition of the patient are determining factors for
speed requirements.
When the philosophy of the rehabilitation center is to use

orthoses for clients who do not have functional motor con-
trol below their level of injury, criteria should be established
so that both the client and the professional staff are consis-
tent in their approach to ambulation. This gives the client
specific information and clarifies goals to be attained, ensur-
ing the most positive outcome (Box 16-3).
The ambulation trial gives the client and the team an
opportunity to simulate orthotic use. If the decision is
made to order orthoses, specific goals should be set. Goals
range from standing and exercise ambulation to commu-
nity ambulation. Most persons with complete injuries above
the L2 level achieve only exercise ambulation because of
the energy necessary for functional ambulation.
Research has demonstrated that the energy cost of ambu-
Figure 16-49 n Descending steps using one handrail. This patient lation for individuals with complete lesions at T12 or higher
with AIS A, level T12 approaches the steps backward, using the is above the anaerobic threshold and cannot be maintained
handrail on his right with both hands, and lowers himself down over time. This study also concluded that ambulation for
three steps. This is one of several methods that may be used to these individuals using a swing-through gait pattern is
negotiate steps. equivalent to “heavy work” or a variety of recreational and
BOX 16-3n CRITERIA FOR AMBULATION

TRIAL FOR COMPLETE INJURIES BOX 16-5 n LOWER-EXTREMITY ORTHOSES137
Expressed desire for ambulation with appropriate goals HIP-KNEE-ANKLE-FOOT ORTHOSES

Body weight not to exceed 10% of ideal Reciprocating gait orthosis (RGO) (see Figure 16-50)
Range of motion: Hip extension 5 degrees, full knee exten- Bilateral knee-ankle-foot orthoses (KAFOs) with pelvic
sion, ankle dorsiflexion 5 to 15 degrees, passive straight band
leg raise 110 degrees KNEE-ANKLE-FOOT ORTHOSES
Intact skin Scott-Craig KAFOs (see Figure 16-51)
Stable cardiovascular system Conventional KAFOs (metal uprights)
Controlled spasticity Polypropylene KAFOs (see Figure 16-52)
Independent function at the wheelchair level Hybrid KAFOs (see Figure 16-52)
ANKLE-FOOT ORTHOSES
Conventional ankle-foot orthoses (AFOs) (metal)
Custom polypropylene AFOs
sporting activities.132-136 Consequently, it is easy to under- Solid ankle AFOsyes (see Figure 16-53, A)
stand why lower-extremity orthoses may end up in the closet Custom polypropylene AFOs, articulated ankle
unused. (see Figure 16-53, B)
The energy cost for ambulation is highest for persons University of California Biomechanics Lab (UCBL)
with complete paraplegia who use a swing-through gait pat- orthotic (see Figure 16-54)
tern and lowest for persons who use bilateral AFOs or a
combination of an AFO and a KAFO. Even individuals re-
quiring only bilateral AFOs have a gait efficiency of less
than 50% of normal, underscoring the importance of the hip
extensor and abductor muscles required for normal ambula-
tion. These muscles are severely or completely paralyzed in
this population.134,135
Assuming that a patient has upper extremities with intact
function, the energy cost of ambulation is progressively re-
duced when more residual motor function is present in the
lower extremities. Conversely, the person with incomplete
tetraplegia has higher energy costs for ambulation despite
spared lower-extremity function because of upper- and
lower-extremity weakness (Boxes 16-4 and 16-5 and
Figures 16-50 to 16-54).132-136
Factors that affect orthotic selection are cost, injury
level, residual motor function, experience bias of the clini-
cian, patient’s medical status, skin and cardiovascular in-
tegrity, and patient’s acceptance. Generally, the hip, knee,
ankle, and foot orthosis (HKAFO) is used when selected
motions of the hip need control or the benefits of the
reciprocating gait orthosis (RGO) are desired, as is the
case with the pediatric population. The use of a KAFO is
indicated when the quadriceps muscle strength is less than
3/5. AFOs are indicated in the presence of ankle instabil-
ity and weakness and to control hyperextension of the
knee joint.134-137
Materials and components of bracing continue to evolve.
Carbon fiber is becoming more common in bracing, includ-
ing in low-profile AFOs, either customized or off the shelf.
n FOUR CATEGORIES OF
BOX 16-4
AMBULATION131
1. Standing only Figure 16-50 n The reciprocating gait orthosis (RGO), although
2. Exercise—ambulates short distances generally used with children, is also used with adults. Its main
3. Household—ambulates inside home or work, uses components are a molded pelvic band, thoracic extensions, bilat-
wheelchair much of the time eral hip and knee joints, and lower limb segments that may be of
4. Community-independent on all surfaces; does not use polypropylene construction with a solid ankle. The RGO uses a
wheelchair dual cable system to couple flexion of one hip with extension of the
other.
There is a trade-off with carbon fiber, however. The benefits

of using carbon fiber are that it is lightweight and rigid.
However, it is not easily modified after it is manufactured
(Figure 16-55, A).
Components have advanced quickly in the last 10 years.
A recently introduced new joint, Sensor Walk, allows per-
sons with no quadriceps function to stand with control from
the joint and then swing the limb freely. Multiple sensors
in the footplate of this KAFO signal to the knee joint the
position and weight-bearing status of the leg. The joint does
not require full knee extension for position of the knee to be
maintained, an advantage over other types of stance-control
mechanisms. If the patient has gluteal function, this device
also allows the individual to ascend and descend stairs using
a step-over-step gait pattern (Sensor Walk Electronic KAFO).138
Other, similar joints in the category of stance-control knee
joints are now available from several different manufactur-
ers and include the E-MAG and Horton knee joints. These
devices use a knee locking mechanism that is either me-
chanically or electronically controlled and is triggered by
heel contact. During stance the knee is locked and with
unweighting the limb is allowed to swing through in a nor-
mal reciprocal pattern. The advantages of this technology
are obvious from a functional perspective. However, the
Figure 16-51 n Scott-Craig knee-ankle-foot orthosis (KAFO) is experience of orthotists and clinicians are that these devices
a special design for spinal cord injury. The orthosis consists of are costly and insurance companies are hesitant to cover the
double uprights, offset knee joints with pawl locks and bail control, additional expense.139-141
one posterior thigh band, a hinged anterior tibial band, an ankle A new system for ambulation that has undergone clinical
joint with anterior and posterior adjustable pin stops, a cushion trials in the United States is the ReWalk system. This exoskel-
heel, and specially designed longitudinal and transverse foot plates eton uses motion sensors, onboard computers, and robotics
made of steel. to assist persons with paraplegia in walking and ascending
Figure 16-52 n A, Polypropylene knee-ankle-

foot orthosis (KAFO) and combination plastic and
metal KAFO. B, Stance-control KAFO knee joint.
This joint combines the stability of a locked knee
during the stance phase of walking but allows flex-
ion of the limb for the swing phase of gait. A
locked knee makes it much harder to clear the leg
over the ground. Some long leg brace users are
perfect candidates for the stance-control KAFOs.
These devices, through a few different types of
joint mechanisms, create a locked knee when the
leg is supporting the weight of the body but unlock
when the leg is lifted to allow for easy advance-
ment of the leg as it is allowed to bend. For the
right patients, this allows them as much mobility to
A B
get around as it does stability.
A B
Figure 16-53 n A, Custom-made solid ankle-foot orthoses (AFOs) in 5 degrees of dorsiflexion
with full footplates. B, Custom-articulated AFOs with adjustable Oklahoma ankle joints.
and descending curbs, ramps, and stairs. Individuals wearing

this exoskeleton have reportedly been able to come from sit-
ting to standing, to ambulate using forearm crutches, to walk
community distances, as well as negotiating environmental
obstacles. Clinical trials have been performed at MossRehab
in Philadelphia as well as internationally. The parent com-
pany, Argo Medical Technologies, began full production and
distribution in early 2011 (Figure 16-55, B). Other companies
are now in the developmental stages and performing clinical
trials with similar exoskeletal devices in preparation for Food
and Drug Administration (FDA) approval. Among these are
Ekso Bionics, Berkeley, California, and Vanderbilt University
research department, Nashville, Tennessee.
Ideally, each patient’s neurological recovery potential is
maximized before or while brace prescription takes place.
Advances in rehabilitation strategies that facilitate neural
plasticity and recovery of function (i.e., walking) include the
use of body-weight support during locomotor training. This
technique of training may employ aquatics, a robot to assist
with movement of the extremities, or manual facilitation of
stepping performed by physical therapists and their reha-
bilitation teams using a body-weight suspension system.
Figure 16-54 n University of California Biomechanics Labora- The principles of locomotor training are discussed later in
tory (UCBL) orthosis. This orthosis is designed with a deep heel this chapter.
cup that holds the calcaneus securely. In addition, the high medial
and lateral trimlines support the joints of the midfoot and allow Equipment
more optimal subtalar joint function. The orthosis also supports the In SCI rehabilitation, the use of equipment is necessary to
longitudinal arch of the foot. (From Lusardi MM, Nielsen CC: achieve the expected outcomes. Clinicians work closely
Orthotics and prosthetics in rehabilitation, Woburn, MA, 2000, with the physician and other team members, including the
Butterworth Heinemann.) rehabilitation technology supplier, to determine the most
A B
Figure 16-55 n A, Allard braces. The Allard family of ankle-foot orthoses (AFOs) include
a prefabricated shell that can be customized by the trained orthotist to the specific needs of the
patients. These dynamic orthoses are constructed of carbon composites, which accounts for their
strength as well as their light weight. There are three different AFOs in the series: Ypsilon, ToeOFF,
and BlueRocker. The AFOs are intended to be used with a custom foot orthotic. B, ReWalk is a
wearable, motorized, quasi-robotic suit. Partially concealable under clothing, ReWalk provides user-
initiated mobility-leveraging advanced motion sensors, sophisticated robotic control algorithms,
on-board computers, real-time software, actuation motors, tailored rechargeable batteries, and com-
posite materials.
appropriate equipment to meet individual needs. It is impor- Seating Principles

tant to have access to trial equipment so the client has the Many individuals spend 8 hours or more per day in their
opportunity to practice with equipment similar to what will wheelchairs after an SCI. Consequently, proper seating of
be prescribed. Ideally the rehabilitation technology supplier these clients may be the most important intervention clini-
should be accessible to the rehabilitation team to allow for cians provide. The seating process should be addressed
necessary adjustments to the equipment. In addition, reha- on admission, continually throughout the rehabilitation pro-
bilitation technology suppliers should be knowledgeable gram, and regularly after discharge to help prevent and
and responsible for educating rehabilitation professionals minimize complications.77,142 The wheelchair is an integral
regarding new products. When possible, all equipment part of the client’s self-image and in many ways will help
should be ordered from a single supplier to reduce confusion define personal lifestyle.62 Goals for seating the client with
when the need for repairs arises. To ensure that the most an SCI are identified in Box 16-6.
appropriate piece of equipment is prescribed, the following Every seating session begins with a thorough examina-
must be considered: durability, function, transportability, tion, as described earlier. Trial simulations are essential to
comfort, cost, safety, cosmesis, and acceptance by the user.62 determine how the patient will function and maintain pos-
Generally, the higher the injury level, the more costly the ture over time in the seating system. Simulations help to
equipment owing to the technology involved. Table 16-7 avoid costly mistakes. The patient must be involved in the
lists equipment according to injury level. decision-making process to ensure that the seating system
Ideally, equipment should be ordered as soon as possi- will work.
ble so the client can be fitted before discharge. Shorter Individuals with SCI are at high risk of pressure ulcers
lengths of stay make early equipment ordering difficult. owing to lack of mobility and impaired sensation. Great care
For example, a client may not have 3/5 wrist extension to must be taken to reduce pressure over bony prominences and
be fitted with a tenodesis brace but with strengthening to distribute pressure over as large an area as possible.143
over time would be an excellent candidate. Clinicians Pressure-distributing cushions should be evaluated clinically
need to negotiate with the funding source so that equip- and with pressure-sensing devices to determine the optimal
ment may be ordered in the outpatient setting. Equipment wheelchair cushion for each individual.142,144 Many patients
required for the SCI population is costly, requiring exten- with muscle paralysis of the trunk find that the effects of
sive review by third-party payers before funding is approved gravity in a sitting position pull the head and upper torso
or denied. Many health care policies do not cover the forward and over the pelvis, resulting in a long kyphosis or a
funding of needed equipment. As a result of these factors, C-curved posture (Figure 16-56).144 Two resulting problems are
many clients are discharged without the equipment they increased weight bearing on the sacrum and development of a
need. Lack of appropriate equipment may result in (1) a feel- thoracic kyphosis, leading to neck hyperextension in an effort
ing of loss of control, (2) contractures and postural deformi- to maintain a horizontal gaze.142 This position is also assumed
ties, (3) skin breakdown, (4) a loss of skills learned in reha- by patients to improve their balance. This occurs when the seat-
bilitation, (5) poor self-image, and (6) increased dependence to-back angle is closed and the client feels as if he or she is
on others. falling forward. Unfortunately, this poor seating posture is
TABLE 16-7 n EQUIPMENT NEEDS CORRELATED TO INJURY LEVEL
INJURY COST INJURY COST

LEVEL EQUIPMENT (IN DOLLARS) LEVEL EQUIPMENT (IN DOLLARS)
C1 to C3 Ventilator (bedside) 14,700 Wheelchair cushion 450-600
Ventilator (portable for wheel- 14,700 Bedside table 225
chair) ECU 250-7000
Power tilt or recline wheelchair 17,000-26,000 Electric hospital bed 2000-4000
Manual recline wheelchair for 2100-4200 Specialized mattress 800-10,000
transport Commode or shower chair 1500
Wheelchair cushion 450-600 ADL equipment 900-1500
Reclining commode or shower 1800-3000 Tenodesis splint 1700
chair Transfer board 100-200
ECU 1900-9000 Hand control for car 300-1500
Call system 1025-1500 Bowel-bladder equipment 125-250
Bedside table 225 C7 to C8 Power upright wheelchair 7500-15,000
Fully electric hospital bed 2000-4000 Manual wheelchair 2000-6500
Specialized mattress 800-10,000 Wheelchair cushion 450-600
Adapted computer 2000-4000 Bedside table 225
Communication devices 400-11,000 ECU 250-1000
Overhead power lift 4000-15,000 Electric hospital bed 2000-3000
Hydraulic lift for transfers 1400-2000 Specialized mattress 600-10,000
C4 to C5 Power tilt or recline wheelchair 17,000-26,000 Commode or shower chair 1500
Manual wheelchair for transport 2100-4200 Hand controls for car 300-1500
Lap tray 250-700 ADL equipment 300-1000
Wheelchair cushion 450-600 Transfer board 100-200
Bedside table 225 Bowel and bladder equipment 125-250
ECU 1500-10,000 Paraplegia Manual upright wheelchair 2000-5500
Fully electric hospital bed 2000-4000 Wheelchair cushion 450-600
Specialized mattress 800-10,000 Raised or padded commode seat 210
Commode or shower chair 1500-3000 (cutout)
Communication devices 300-1500 Tub bench 220
ADL equipment 400-1400 Hand controls for car 400-700
Hydraulic lift for transfers 1400-2000 ADL equipment 100-300
Overhead power lift 4000-15,000 Bowel and bladder equipment 50-250
Mobile arm support 500-1000 Lower-extremity orthotics (if 4000-6000
Upper-extremity orthotics 700-1200 ambulation is a goal)
C6 Power upright wheelchair 7500-20,000
Manual wheelchair 2000-4500
Power assist wheels 9000
ADL, Activity of daily living; ECU, environmental control unit.
Based on 2009-2010 Atlanta, Georgia, retail prices.
quickly learned and difficult to correct.142 This posture can pressure on the sacrum and shearing of the skin. For these
often be prevented by tilting the wheelchair slightly backward patients, a manual wheelchair with adjustable seat and back
while maintaining a fixed seat-to-back angle (Figure 16-57).142 angles can be used to improve stability. Power wheelchairs
In this position, the effects of gravity augment sitting balance with power tilt systems allow users to reposition themselves
and facilitate good spinal alignment. Education regarding and use the power tilt for improved stability.
proper positioning, the use of a sacral block, a firm wheel- Optimal pressure distribution is achieved by maximizing
chair seat and back, and properly applied pelvic positioning the surface area, allowing immersion into the seat cushion,
devices also aid in preventing the kyphotic posture.142 and promoting a symmetrical posture. The width of the seat
Asymmetrical muscle strength, asymmetrical spasticity, should be slightly more than that of the widest body part. The
and preferential use of one upper extremity over another seat depth should come to approximately within 1 to 2 inches
often result in poor trunk alignment. The use of lateral trunk of the popliteal fossae, except when it interferes with lower
supports, lateral pelvic supports, and properly applied seat extremity (LE) management. The height of the back should
belts may aid in maintaining symmetrical trunk posture. reflect the client’s motor function and seated stability. If the
Strong muscle spasms, combined with the effects of back is too high, it can restrict functional activities such as
gravity, may cause the person with severely impaired mobil- wheelchair propulsion and wheelies. Patients with tetraplegia
ity to slide down in the wheelchair, resulting in increased who use the push handles of the wheelchair to hook while
BOX 16-6n GOALS FOR SEATING THE CLIENT

WITH SPINAL CORD INJURY
1. Maximize functional independence
2. Improve pressure distribution and relief of pressure
3. Optimize comfort
4. Enhance the quality of life
5. Optimize good postural alignment and sitting balance
6. Compensate for fixed deformities
7. Allow for transportation of the mobility system
The following are basic seating concepts of proper
postural alignment:
n Neutral pelvic alignment
n Symmetrical alignment of the trunk and neck
n Neutral head positioning over the pelvis
n Maintenance of a horizontal gaze
n Maintenance of ankle in neutral alignment with full
support of the foot
n Maintenance of the thighs in neutral abduction and
adduction with full contact with the cushion
n Neutral shoulder positioning to avoid shoulder
elevation, protraction, or retraction and to provide
adequate upper-extremity support142,144
n Elbow angle that approximates 100 to 120 degrees
when the hand is resting at the top of the wheel or
pushrim126
Figure 16-57 n Example of corrected C-curve posture.
performing functional activities may require custom modifi-

cation of the wheelchair back (Figure 16-58).
The size, weight, and portability of the wheelchair seat-
ing system affect the individual’s lifestyle. The client’s
home or work environment must be evaluated closely for
accessibility so that the wheelchair seating system can be
used effectively in those environments. The buildings must
be structurally sound and spacious to accommodate heavy-
power wheelchair systems. The means of transportation of
the wheelchair (car versus van) may determine whether
a rigid or folding wheelchair frame is indicated. Transit
options and tie-down systems must be considered for safe
transportation. The wheelchair must be adjusted to make it
as efficient as possible to propel to reduce stress on upper-
extremity joints. Many manual wheelchairs are lightweight
(less than 35 pounds) and have multiple adjustments and
choices of tires and casters that make manual wheelchair
propulsion more efficient. Reducing rolling resistance, po-
sitioning the rear axle for maximum propulsion stroke
efficiency, and teaching efficient propulsion techniques
reduce shoulder musculature fatigue and upper-extremity
injury. Rear wheel size should be selected so that when
the wheelchair user is seated, he or she is able to touch the
rear axle with the middle finger. This position increases the
range of contact during propulsion. In addition, shifting
the distribution of the user’s weight back over the rear axle
(usually accomplished by moving the rear wheel axle for-
ward) reduces the percentage of weight on the front cast-
Figure 16-56 n Example of typical kyphotic C-curve posture in ers, making propulsion more efficient.145 This adjustment
the patient with tetraplegia. reduces the rear stability of the wheelchair, so the use of
and the team to identify problem areas and provide addi-

tional education in those areas.
Psychosocial Issues
The immediate reaction to the onset of SCI is physical shock
accompanied by anxiety, pain, and fear of dying. The re-
sponse to such an injury varies greatly and depends on the
extent of the injury, the premorbid activity level, the style
of coping with stress, and family and financial resources.
There may be great sensory deprivation from immobiliza-
tion, neurological impairment, and the monotony of the
hospital routine. Several psychological theories have been
proposed to describe responses and coping mechanisms.65
The process of coping with these changes is referred to as
adjustment (see Chapter 6)
Rehabilitation personnel are becoming more aware of the
need not only to teach functional skills but also to teach psy-
chosocial and coping skills to the client and significant others.
Education in the following areas facilitates the adjustment
process: creative recreation, financial planning, negotiating
community barriers, social skills, managing an attendant, cre-
ative problem solving, accessing community resources, fertil-
ity and child care options, assertiveness, sexual expression,
vocational planning and training, and the use of community
transportation. These skills may be introduced in the inpatient
rehabilitation setting but will be developed further in the home
and community environments. True adjustment and adaptation
begin after discharge from rehabilitation.146,147
Figure 16-58 n Example of custom modification of a wheel- Sexual Issues
chair back to allow a patient with tetraplegia to hook the push
Sexuality is how people experience and express themselves
handle with one upper extremity.
as sexual beings and is a normal part of being human,148 so
it is not surprising that persons with SCI place a high prior-
ity on resuming sexual functions after their injury.149 After
anti-tip bars and/or training in wheelie maneuvers is essen- SCI, men may experience impairments in penile erection,
tial. Finally, along with wheelchair fit, the esthetics of the ejaculation, orgasm, and fertility. Women with SCI may
wheelchair can affect the individual’s self-image and there- experience impairments in the ability to become aroused or
fore the community reentry. This should be considered achieve orgasm and/or may have decreased vaginal lubrica-
when assisting the patient to make wheelchair seating tion.150 Improving sexual functions is a high priority for
decisions. both men and women after SCI.149 Table 16-8 lists the rela-
tionship of the level of spinal injury to sexual function.
Education Treatment of sexual dysfunction should be a coordinated
Education of the client and caregivers is an integral part of effort among the patient, significant other, and appropriate
the rehabilitation process. Formal education includes group health care professionals. Sexual counseling, educational
and individual instruction and family and caregiver train- programs, and medical management provide opportunities
ing. Clients and caregivers are taught preventive skin care, to address the areas of sexual dysfunction, alternative be-
bowel and bladder programs, safe ways to perform all haviors, precautions, and other related areas.150
ADL tasks, nutritional guidelines, thermoregulation pre- Depending on the level and completeness of the SCI,
cautions, pulmonary management, cardiopulmonary resus- most men can attain an erection either through psychogenic
citation, management of autonomic dysreflexia, equipment (via T11 to L2 pathways) or reflexogenic pathways (S2 to
management and maintenance, transfer techniques, wheel- S4)151; however, these erections are often not reliable or
chair mobility, ambulation, proper body positioning, ROM adequate for sexual intercourse. The first-line treatment for
exercises, ADL basics, and leisure skills. Home programs erectile dysfunction after SCI is the use of phosphodiester-
are taught to maintain or increase strength, endurance, ase type V inhibitors such as sildenafil (Viagra), tadalafil
ROM, and function. Energy conservation techniques and (Cialis), and vardenafil (Levitra). Other treatments include
proper body mechanics are incorporated into all aspects of intracavernosal (penile injectable) medications, mechanical
training. methods such as vacuum devices and penile rings, and, as a
Clients are formally tested on their knowledge, and reme- last resort, surgical penile implants.148
dial instruction should be provided in deficient areas. During Male orgasm and ejaculation are likely to occur together;
family training, caregivers are formally evaluated on their however, after SCI an orgasm may not always lead to ejacu-
abilities to safely provide care to the client. Supervised lation, or there may be retrograde ejaculation into the blad-
therapeutic outings and passes allow the client, caregivers, der.152 A study by Sipski and colleagues showed that 78.9%
TABLE 16-8 ELATION OF LEVEL OF SPINAL

R n to conceive; however, there are increased risks to pregnancy,
INJURY TO SEXUAL FUNCTION which may include bladder problems, spasticity, pressure
sores, autonomic dysreflexia, and problems with mobility.158
INJURY LEVEL SEXUAL FUNCTION
Cauda equina/conus Males Discharge Planning
Usually no reflex erections Discharge planning begins from the time the client is being
Rare psychogenic erection considered for admission and continues through the reha-
Ejaculation occasionally occurs bilitation program. It is a continuous process that includes
Females the client, family, treatment team, and community resources,
Vaginal secretions often absent with the goal being successful community reintegration and
Patients generally fertile a perceived good quality of life. The rehabilitation team
Thoracic/cervical Males must identify the specific needs of the client and must struc-
Reflex erections predominate (usually ture the program to enhance the chance of success. Lengths
short duration) of stay are getting shorter in response to pressure from third-
Psychogenic erections generally absent party payers to contain costs. This requires the discharge
Ejaculation occasional planning process to be expedited so that procurement of
Females needed equipment, completion of architectural modifica-
Vaginal secretions present as part tions, and referrals to outpatient and community resources
of genital reflex occur in a timely manner.
Fertility preserved
Sensation of labor pain absent Architectural Modifications
Architectural barriers in the home, transportation system,
workplace, or school may prevent access to opportunities.
The architectural changes required by the person with SCI
for independence in the home and community depend on
of men with incomplete upper motor neuron SCI achieved the degree of impairment, financial resources, and client
orgasm as compared with 28% of men with complete upper and family acceptance of modifications or equipment. The
motor neuron injuries (P , .001), whereas 0% of men with clinician should discuss equipment options with the client
lower motor neuron injuries affecting their sacral cord and family on the basis of the degree of modification they
achieved orgasm.152 plan to make to their home. Thinking creatively about low-
“Will I ever be able to become a father?” is a common tech adaptations should be considered part of the therapist’s
question of men after SCI. Pregnancy rates in partners of role. Problem solving with and by the client is vital to the
men with SCI are lower than in the general population, but process of identifying alternatives as ideas for the future
there is a good chance (greater than 50%) that men with (Figure 16-59).
SCI can become biological fathers with advances in repro- Many available resources describe the dimensions of the
ductive assisted technology. Roughly 2 weeks after an SCI, basic wheelchair and specifications for making homes and
semen quality declines153 to levels approaching those ob- facilities accessible to wheelchair users. See Appendix 16-A
served in males with chronic SCI.154 There is evidence that at the end of this chapter for resources on architectural
bladder management with clean intermittent catheteriza- modification.
tion may improve semen quality over other methods of
bladder management.155 The two most common methods Return to Work or School
of sperm retrieval are vibrostimulation (less invasive) and Successful community reintegration after SCI includes
electroejaculation. These methods are successful for per- returning to preinjury social roles and, more specifically,
sons with lesions above T10. If these methods are not suc- returning to work, school, and/or leisure interests. Public
cessful, there is an option of surgical aspiration. Depend- school systems have a legal obligation to provide an
ing on the semen quality, a progression from intravaginal appropriate school setting for a child with a disability.
insemination, intrauterine insemination, in vitro fertiliza- Rehabilitation teams may assist with school reentry by
tion (IVF), to IVF plus intracytoplasmic sperm injection is adding school visitations and education for faculty or
recommended.148 peers. School accessibility can be assessed and the patient
Women with SCI may have impairment in arousal and and therapists can have an opportunity to share appropri-
orgasm. The vagus nerves are thought to facilitate the pres- ate information about the new impairments before reen-
ence of vaginal-cervical perception of orgasm. Preservation try into the school system. Also, rehabilitation programs
of T11 to L2 sensory dermatomes is associated with psycho- that are CARF accredited must offer academic programs.
genically mediated genital vasocongestion and lubrica- School reentry programs may enhance communication
tion.156 There is some evidence that supports the use of between academic rehabilitation faculty and school, bridg-
sildenafil in women to partially reverse subjective sexual ing the gap for return. Students returning to college may
arousal difficulties.148 For women who have a lower motor need assistance developing problem-solving skills related
neuron lesion, vaginal secretions are often absent, and an to campus accessibility, Americans with Disabilities Act
artificial lubricant is recommended. (ADA) rights, campus transportation options, and self-
Amenorrhea may occur immediately after SCI and last advocacy for sports adaptations.
up to 4 to 5 months. Despite this delay, it is believed that Rehabilitation programs must also emphasize returning
fertility in women is unaffected by SCI.157 Women are able to work throughout the process. For patients who have
community, such as rehabilitation technology, independent

living centers, and job training and placement programs.
Individuals should refer to their state DRS for assistance
with employment.
Engaging in sports and other leisure skills can open doors
for patients returning to the community. Participating in
sports and leisure, whether learning a new skill or adapting
something previously enjoyed, may boost physical capacity
and enhance self-worth. Adapted sporting activities found in
the United States include power soccer, quad rugby, wheel-
chair basketball, tennis, swimming, and snow skiing, to
name a few. The Paralympics provide competitive venues
for elite athletes, and many road races across the United
States have opened the roads for wheelchair athletes to com-
pete alongside able-bodied runners. A handful of colleges
and universities have developed adapted sports teams and
are beginning to offer student scholarships.
A
Health Promotion and Wellness
Individuals with spinal cord injuries are living longer owing
to improvements in medical management, but this has also
led to increased incidence of chronic diseases, such as car-
diovascular disease (CVD) and diabetes mellitus, in this
population.162-164 The Surgeon General’s Report on Physi-
cal Activity and Health identifies persons with disabilities
as among the most inactive subgroup in the United States.165
Cardiopulmonary disease has been identified as a primary
source of morbidity for persons with aging spinal cord
injuries,32,166 and nearly all cardiovascular risk factors are
increased in individuals with SCI. Increased rates of diabe-
tes, impaired glucose tolerance, metabolic syndrome, and
obesity—all conditions that are exacerbated by physical
B inactivity—contribute to the development of CVD.167 Muscle
atrophy is common in people with complete and incomplete
Figure 16-59 n Low-tech home adaptations. A, A strap is added SCIs, with an associated increase in fat mass resulting from
to make a clothes dryer door accessible. B, A hole is drilled and a the imposed immobility caused by the neurological impair-
handle added to a screen door knob. ment. These are all key factors when considering the impact
of resting energy expenditure (REE) on metabolism in
people with SCIs168 and in evaluating the risk of obesity in
sustained a traumatic SCI and are included in SCI model this population.
systems data,13 57.5% report being employed at the time Key components of a health and wellness program for
of the accident. Only 11.6% are employed at the 1-year persons with SCI are exercise, prevention of secondary com-
anniversary, but by year 20 35.4% are employed. At year 10 plications, injury prevention, good nutrition, and good psy-
after injury, people with paraplegia (31.7%) have a slightly chological support. Physical activity after SCI has been
better employment outcome versus those with tetraplegia, shown to improve muscle strength, endurance, mobility, the
of whom 26.4% are employed.41 Many individuals can ability to fall asleep, self-image, and blood lipid profiles and
return to their previous jobs after SCI.159,160 The Americans decrease the risk of premature death. In addition, exercise
with Disabilities Act of 1990 (PL 101-336) prohibits busi- has been shown to decrease anxiety, loneliness, depression,
nesses with 15 or more employees from discriminating stress, heart disease, blood pressure, respiratory illness,
against “qualified individuals with disabilities” with respect diabetes, obesity, and other medical complications.169
to the terms, conditions, or privileges of employment.161 Job Exercise programs for individuals with SCIs must take
site and job responsibilities may need to change to accom- into consideration the musculoskeletal, respiratory, cardio-
modate the new impairments, allowing the patient to fully vascular, and autonomic nervous system changes that occur
participate. For those who are unable to perform previous after SCI. Components of an exercise program should
jobs or who were unemployed before injury, many programs include flexibility, muscular strength, and cardiovascular
exist for training in vocational skills. The Department of endurance, and an appropriate exercise prescription should
Rehabilitation Services (DRS) evaluates clients for skills address exercise mode, intensity, duration, and frequency. It
and functional abilities and provides funding for those is important to find a type of exercise that is enjoyable for
qualifying for job training, job site modification, and the each individual so that it can be easily integrated into his or
purchase of essential equipment that may include transpor- her lifestyle. Required exercise intensity to improve cardio-
tation. Services offered by the DRS vary from state to state. vascular fitness and reduce the risk of CVD is 50% to 80%
Each state agency has a list of resources available in the of peak oxygen uptake.170 American College of Sports
Medicine (ACSM) guidelines suggest that able-bodied indi- through tendon transfers.179,180 Typically, before individuals
viduals exercise 30 to 60 minutes most days of the week, are considered for surgery, their neurological function has
and these guidelines are often extrapolated to the disabled reached a plateau, they are psychologically stable, and they
population as well.171 However, they do not take into ac- have functional goals.180 Individuals seeking restorative
count that individuals with SCIs are also using their upper surgery to the upper extremity undergo a preoperative
extremities to complete many of their ADLs and mobility evaluation using the International Classification for Sur-
tasks, so 30 minutes of exercise two or three times per week gery of the Hand in Tetraplegia (ICSHT).181 Before any
may be sufficient for them to maintain their fitness.170 surgical interventions, therapy may be recommended to
Circuit resistance training programs using alternating resis- ensure that the individual is a candidate for tendon transfer
tance maneuvers and high-speed, low-resistance arm exer- procedures.180 Postoperative rehabilitation varies on the
cise have been shown to be beneficial in improving muscle basis of specific procedures and may consist of 2 months
strength, endurance, and anaerobic power of middle-aged or more, with strength improvements continuing for up to
men with paraplegia while also significantly reducing their 1 year postoperatively.182,183 Tendon transfer procedures
shoulder pain.172 Circuit resistance training has also been may be an option to improve upper-extremity function.183
shown to increase peak oxygen consumption and cardio
respiratory endurance in patients with chronic paraplegia.173,174 Activity-Based Therapy
Exercise programs, both in the clinic and home, may The terms activity-based restorative therapies, activity-
incorporate specialized equipment. The types of equip- based therapies, and activity-based rehabilitation have been
ment available for exercise testing or training in persons coined in the last 10 years to describe a new fundamental
with SCI are well documented in the literature. Arm crank approach for treating deficits induced by neurological
ergometers, wheelchair ergometers, wheelchair treadmills, paralysis. The goal of this approach is to achieve activation
lower-extremity cycling with FES, suspended ambulation of the neurological levels located both above and below the
protocols, and field test protocols are among the more injury level using rehabilitation therapies in order to facili-
widely used equipment in the clinic.175,176 Exercise equip- tate recovery after a debilitating neurological incident.184
ment varies in expense, and each clinic must choose the The theory behind the achievement of recovery from par-
method that best fits its treatment setting and budget. ticipation in intense therapy programs, often called activity-
Home exercise programs may be established with equip- based therapy programs, involves plasticity of the nervous
ment such as weights and cuff weights, elastic bands and system. Dunlop defines plasticity as the ability of neurons to
tubing, and hand cycles. rearrange their anatomical and functional connectivity in
Overuse syndromes are common among long-term response to environmental input, thereby achieving new or
wheelchair users. When any type of exercise program is modified outputs.185 Several lines of evidence suggest that
established, factors that are specific to SCI should be con- the central nervous system is capable of synaptic plasticity
sidered.172 Long-term wheelchair use can lead to an in- and anatomical reorganization occurring at both cortical and
creased incidence of carpal tunnel syndrome, elbow or subcortical levels, including the spinal cord, after SCI.186-188
shoulder tendonitis, early onset of osteoarthritis, and rotator Facilitating reorganization of the injured nervous system is
cuff injuries. The motion and resistance of the upper- the goal of these types of intensive therapy programs, and
extremity muscles during wheelchair propulsion can lead to rehabilitative interventions are thought to affect plasticity in
an overdevelopment of anterior shoulder muscles, scapular several ways, including behaviorally, physiologically, struc-
protraction, and posterior shoulder weakness. This muscu- turally or neuroanatomically, cellularly, and molecularly.189
lature imbalance may lead to elevation and internal rotation For clinicians, this “emerging paradigm shift” in the
of the humeral head that may cause pain as a result of im- practice of SCI rehabilitation has recently been described as
pingement. Injuries can be prevented or slowed if clients a transfer from therapy that focuses on teaching compensa-
perform a proper warmup with stretching and flexibility tory strategies such as learning to use the upper extremities
exercises, wear protective equipment (e.g., helmet and for mobility when the lower extremities are impaired,
padded gloves), alternate modes of exercise, and get proper toward intensive recovery programs specifically designed
rest between exercise sessions. to improve locomotor abilities in people with incomplete
Through an established health and wellness program, a spinal cord injuries.190 Traditional therapy for the treatment
person with SCI has the potential to increase quality of of these types of injuries is designed to improve a client’s
life, improve ADLs, decrease secondary complications, independence using techniques that promote the use of
decrease depression, and decrease the number of related assistive devices to compensate for lost function, such as
hospitalizations. It is a goal that integration to wellness using a wheelchair for mobility. In contrast, intensive ther-
programs for individuals with SCIs will become a standard apy programs for people with SCI focus on recovering the
in all facilities. ability to use their trunk and limbs to stand and walk as they
did before their injury along with promoting lifelong health
and wellness in this population. Although not clearly defined,
RESTORATION AND RECOVERY
activity-based therapy often involves intensive practice and
Upper-Extremity Restoration repetition of task-specific mobility training to promote
Improving hand and upper-extremity function plays a recovery and facilitates revitalization of the central nervous
critical role in achieving independence with ADLs.177,178 system. (Refer to Chapter 4 for additional discussion.)
Surgical restoration of hand grasp, lateral pinch, or elbow Specialized rehabilitation technology is often used in
extension in a patient with tetraplegia can be an option this type of therapy approach, including but not limited to
body-weight–supported treadmill (BWST) systems, robotic the efficacy of this type of training over more traditional
BWST systems, FES bikes, and LE FES systems designed to gait training approaches. However, as interest in locomotor
improve overground walking ability. (Refer to Chapters 9 training interventions continued to grow, so did concern
and 38 for further discussion of rehabilitation technologies.) over the amount and intensity of labor required by thera-
pists to complete this rehabilitation technique. The advent
Improving Walking Function of robotic-assisted locomotor training devices offered a
Research on locomotor training through the use of BWST less burdensome alternative to facilitate walking in persons
systems first began with spinalized cats in the 1980s191-195 with incomplete SCIs while reducing therapist strain
and then progressed to human subjects with increasing (Figure 16-60, B).198,205,206 Over the last several years various
popularity in the 1990s and 2000s (Figure 16-60, A).190,196-200 types of locomotor training approaches have been studied
Much of the theory behind this rehabilitation approach is with regard to incomplete SCIs; however, many questions
based on activating intrinsic connections of spinal cord cir- still remain regarding efficacy of specific intervention choices
cuitry to elicit the appropriate patterns of muscle activation and timing for this population. In a randomized controlled
for walking called central pattern generators (CPGs).201 trial, Dobkin and colleagues reported that after 12 weeks
Research involving the cat model has provided the most of equal administration of locomotor training using manual
conclusive and descriptive evidence for the presence and assistance and conventional overground gait training, no dif-
activity of CPGs,202 including the ability to produce locomo- ferences in walking abilities were reported in patients with
tor output in spinalized animals.203 However, the evidence incomplete SCIs with either intervention.197 Field-Fote and
in humans has been less conclusive and is mainly based on co-workers198 randomly assigned 27 patients with motor in-
the presence of alternating flexor and extensor activity seen complete SCIs into one of four different stepping groups
in fetuses in utero and the presence of “locomotor-type using body-weight support including treadmill training with
patterns” seen in patients with complete SCIs through tonic manual assistance, treadmill training with electrical stimula-
epidural stimulation.204 tion, overground training with stimulation, and treadmill
Although many locomotor training studies have demon- training with robotic assistance. After 12 weeks of training,
strated improved walking function in response to training in all subject groups demonstrated a significant effect of train-
patients with incomplete SCIs, questions remain regarding ing on walking speed, but differences among the four groups
A B
Figure 16-60 n A, Manual treadmill. Treadmill training with body-weight–supported and manual
assistance is being completed on the TheraStride Innoventor, which combines a treadmill and sup-
port harness system with software that measures variables of gait training, including speed, weight
supported, and amount of time walked. Two or three therapists are needed to provide assistance at
the trunk and lower limbs to facilitate an appropriate gait pattern. B, Robotic treadmill. The Lokomat
by Hocoma is a robotic-assisted treadmill that provides adjustable body-weight support and gives
clinicians the ability to adjust gait-specific parameters when completing training with patients who
have mobility deficits.
were not statistically significant.198 Finally, two systematic Lower-Extremity Neuroprosthetics

reviews have also concluded that there is insufficient evi- The first system for standing and stepping to gain FDA
dence at this time to conclude that any one locomotor train- approval was the Parastep II system, developed by Sigmed-
ing strategy is superior to any other to improve walking ics (Northfield, Illinois).213 This type of system generally
function in people with incomplete SCIs.207,208 However, uses two to 12 channels of stimulation. The system in its
Lam and colleagues did suggest that patients with chronic simplest form uses one set of electrodes placed over the
SCIs might benefit more from the combined approach of quadriceps muscle and another set over the sural, saphenous,
locomotor training with electrical stimulation.207 or peroneal nerve. This system consists of a computer con-
trol box, lead wire, electrodes, and a cable connected to a
Upper-Extremity Neuroprosthetics walking device that houses the command switch(es) for step
The first developments in upper-extremity FES began in function. Other walking systems that use implanted elec-
the 1960s with use of a flexor hinge orthosis, much like a trodes are being investigated at this time, but none are FDA
tenodesis brace.210 Later developments focused on im- approved. Both FES systems, the surface electrode system
proving hand function, primarily palmar grasp and lateral and the implanted system, in their present state show prom-
pinch.211 The use of surface electrodes then led to the de- ise for the future. However, these systems currently do not
velopment of implantable FES systems. The NeuroControl present a viable alternative to wheelchair use because of the
Freehand System is an implanted medical device that uses high energy requirement.214,215 In addition, there is limited
electrical stimulation electrodes that are attached to mus- research in the use of epidural spinal cord stimulation to
cles in the hands and forearms and a pacemaker-type facilitate ambulation.143,216
stimulator that is surgically implanted in the chest. Signals Hybrid FES orthosis systems are orthotic systems that
come from the external controller to the electrodes and incorporate FES. Usually the FES is a simple configuration
cause muscles to contract and the hand to open and close. of approximately four channels and uses surface stimula-
The system was approved by the FDA in 1997, but mar- tion. One such system, used with the RGO, was developed
keting was stopped in 2001. The future of upper-extremity by Douglass and colleagues at Louisiana State University
implantable neuroprosthetics is currently uncertain, with Medical School. Systems such as this offer the advantage of
no systems having been made commercially available increased energy efficiency compared with the use of only
since the NeuroControl Freehand. orthoses or only FES. Conversely, the bulkiness of the sys-
More recent development of upper-extremity neuropros- tems impedes the completion of some ADLs, and donning
thetics has been focused around external neuroprosthetics and doffing is more difficult.80,217,218
with products such as the Bioness H200 (Handmaster) or FES devices have also been designed to address foot
the bionic glove by Hanger Orthopedic. The Bioness H200 drop dysfunction to improve overground locomotion in
system enables appropriately selected clients with midcer- patients with incomplete SCIs. Promising results have
vical injuries to flex and extend the thumb and fingers, been demonstrated in all outcome measures of walking,
allowing a useful pinch and grasp, by hitting a switch or a such as functional mobility, speed, spatiotemporal param-
trigger (Figure 16-61). The bionic glove uses three chan- eters, and the physiological cost of walking.219-221 Im-
nels to activate and flex the thumb and extend or flex the provements in walking function could be associated with
fingers by using wrist extensors or flexors to control the plasticity in central nervous system organization, as seen
neuroprosthesis.212 by the modification of the stretch reflex and modifications
A B
Figure 16-61 n The Bioness H200 system enables appropriately selected patients with midcervi-
cal injuries to flex (A) and extend (B) the thumb and fingers, allowing a useful pinch and grasp, by
hitting a switch or a trigger.
in the corticospinal activation of lower leg muscles.222

Commercially available products designed to improve
overground walking in people with neurological injuries
with foot drop include the Bioness L300 (Figure 16-62)
and the WalkAide by Innovative Neurotronics (Figure 16-63).
Both devices are single-channel foot drop stimulators that
synchronize the electrical impulses to the dorsiflexors with
gait. Overcoming many of the technical shortcomings of
conventional FES units, these units have increased compli-
ance and community use of this technology. Several stud-
ies have reported the immediate effects and short-term
gains; however, long-term benefit of foot drop stimulation
for people with SCI is yet to be sufficiently established in
the literature.219-221,223
Functional Electrical Stimulation Cycling

FES cycling has gained acceptance in recent years in treat-
ment of patients with spinal cord injuries. Many authors
have reported significant health and wellness benefits that
are both physiological and psychological when treating
patients with SCIs with this technology.209 During this inter-
vention, electrodes are applied to various muscle groups of
the lower extremities including the quadriceps, hamstrings,
gluteal muscles, tibialis anterior, and gastrocnemius and
soleus. The bike then electrically stimulates the selected
muscle groups at the appropriate intervals to produce the
torque that turns the ergometer at a preset speed. The FES-
driven workload can be supplemented by an internal motor Figure 16-63 n The WalkAide System is a neuroprosthetic
within the ergometer. Currently there are three commer- device that contains an orthotic cuff and electrodes. It fits just
cially available products, including the RT300 by Restor- below the knee to supply functional electrical stimulation (FES) to
ative Therapies (Figure 16-64), ERGYS 2 by Therapeutic the lower leg, stimulating the appropriate muscles and causing
dorsiflexion. It is activated by a tilt sensor mechanism and relies
on the angle of the tibia for appropriate timing of the FES to assist
with foot drop.
Figure 16-64 n RT300-SL leg FES system is a portable func-

tional electrical stimulation (FES) bike that can be easily accessed
from the patient’s wheelchair. Adhesive electrodes can be applied
Figure 16-62 n Bioness L300 system is a neuroprosthetic device to a variety of lower-extremity muscles including the quadriceps,
that contains an orthotic cuff and electrodes. It fits just below the knee hamstrings, gluteals, tibialis anterior, and gastrocnemius. These
to supply functional electrical stimulation (FES) to the lower leg, muscles are stimulated at the appropriate time to facilitate a cycling
stimulating the appropriate muscles and causing dorsiflexion. A gait motion using a lower-extremity ergometer. FES bike software has
sensor is placed in the client’s shoe, and as he or she shifts weight off the capability to store patient-specific parameters between sessions
of the affected limb, FES is triggered to assist with foot drop. and patients.
Alliances, and the RehaMove by Hasomed. The following muscle strength adaptations and can be used by profession-
benefits from FES cycling have been reported in patients als to improve muscular strength in individuals. It was also
with spinal cord injuries: improved cardiorespiratory fitness, determined that vertical platforms elicit a significantly larger
increased leg circulation, increased metabolic enzymes or effect for chronic adaptations than oscillating platforms, but
hormones, greater muscle volume and fiber size, enhanced oscillating platforms elicit a greater treatment effect for
functional exercise capacity, decreased spasticity, decreased acute effects than vertical platforms.232 A variety of litera-
blood glucose and insulin levels, and improved bone mineral ture has recently been published demonstrating the benefits
density.209,224-230 of using this modality in people with a variety of clinical
Newer technology also may include upper-extremity FES conditions including cerebral palsy,233 Parkinson disease,234
(Figure 16-65) cycling in which the electrodes may be stroke,235 and SCI.236 However, there is still only a small
applied to various muscle groups of the arm and scapula. body of evidence describing the effects of WBV on individu-
The most commonly used muscle groups are the anterior als with SCIs.237 Ness and colleague237 found a statistically
deltoids, biceps, and triceps. Use caution in the presence of significant improvement in cadence when treating individu-
unresolved glenohumeral subluxation. There has been less als with chronic SCIs with WBV that was comparable to
research directly looking at the benefits of the upper-extremity improvements seen in individuals who have undergone loco-
FES cycle systems, but clinicians in the field suggest that it motor training. In a follow-up study236 these authors also
may yield many of the same benefits as lower-extremity reported a decrease in quadriceps spasticity after individuals
FES cycling. with chronic SCIs completed 12 sessions of WBV training.
Overall, further research needs to be completed on this
Whole Body Vibration intervention to determine the most efficacious use of param-
Whole body vibration (WBV) training has become increas- eters with patients who have sustained SCIs, but early research
ingly accessible and popular for training individuals with supports that this may be a useful intervention to improve
and without disabilities in recent years. Vibration is used as walking speed and decrease spasticity in individuals with
a mechanical stimulus to increase motor unit recruitment chronic SCIs (Figure 16-66).
through the feet when standing on a vibration platform
(vertical or oscillating) or via the tendon of a muscle belly
when a hand-held unit is used.231 In a meta-analysis looking
at the effects of vibration on muscular development in the
able-bodied population, Pedro and Rhea determined that
vibration exercise can be effective at eliciting chronic
Figure 16-65 n RT300 SA Arm system is a functional electrical

stimulation (FES) upper body ergometer designed to stimulate
muscles of the upper extremities. Adhesive electrodes can be ap-
plied to a variety of muscle groups over the trunk, shoulders, and
arm muscles and are stimulated at the appropriate time to facilitate Figure 16-66 n The Wave vibration plate is used in the clini-
a cycling motion using the upper-extremity ergometer. FES bike cal setting to facilitate upright standing posture and can also
software has the capability to store patient-specific parameters be- be used to facilitate upper- and lower-extremity strengthening
tween sessions and clients. exercises.
In conclusion, recovery of walking is an increasing possi- best defense against SCI is to prevent the injury from
bility for a large number of people with SCI. New modalities occurring. Programs such as ThinkFirst are aimed at help-
of treatment have become available for this population, but ing individuals of all ages learn to reduce their risk of SCI
most still need to be evaluated for their efficacy. by educating them to make safe choices. Key concepts
include “Buckle up. Drive safe and sober. Avoid violent
CONCLUSION situations. Lower your risk to fall. Wear a helmet. Check
Comprehensive treatment of the individual with SCI can the water before you dive.”238
be very challenging. Health care reform issues force the
rehabilitation team to explore new cost-efficient options to References
continue to provide high-quality rehabilitation. New medi- To enhance this text and add value for the reader, all refer-
cal and rehabilitation interventions provide the clinician ences are included on the companion Evolve site that ac-
with a plethora of interventions to improve functional companies this textbook. This online service will, when
recovery as well as promote neurological recovery after available, provide a link for the reader to a Medline abstract
SCI (Table 16-9). Scientists continue to research ways to for the article cited. There are 264 cited references and other
prevent and/or cure paralysis and loss of function after general references for this chapter, with the majority of
SCI; however, until those goals have been achieved the those articles being evidence-based citations.
TABLE 16-9 n SUMMARY OF SPINAL CORD REGENERATION EFFORTS
AGENT/
INTERVENTION MECHANISM SPONSOR TRIAL STATUS/RESULTS
Methylprednisolone Antiinflammatory, blocks glutamate receptors, Pharmacia Standard trauma protocol;
(MP) reduces accumulation of free radicals. effective in high dosages if
given 1st 48 hours post-injury.
Celebrex may be as effective.
Monosialiac gangli- Neurotrophic factor limits cell death by buffer- Sygen May accelerate recovery in 1st
oside (GM-1) ing excitotoxicity and preventing apoptosis 6 weeks but no difference
@ 6-12 mos.
Activated macro- Bolsters immune response, introduces nerve Proneuron Phase II 2003-06; early
phages growth factors. termination due to $$; results
not released.
Cell matrix modifi- Modify inhibitory glial scar matrix, allowing Neuraxo Preclinical studies completed;
ers - Cordaneurin axon sprouting, growth, and functional no Phase I announced at
(acute) Corda- plasticity. present.
Chron (chronic)
Minocycline Synthetic tetracycline antibiotic that inhibits NACTN Promising preclinical work and
activity of inflammatory cytokines, free human trials likely in Canada.
radicals, etc. causing excitotoxicity.
Decorin Naturally occurring protein molecule that Baylor College of Preclinical
suppresses scar tissue formation. Medicine and
Integra Life-
sciences
4-Aminopyridine Potassium channel blocker restores action Acorda Chronic SCI; Two Phase II
(Fampridine or potential conduction in de- or poorly- studies (spasticity, bladder
4-AP) myelinated nerves; enhances synaptic control) 2003-05; moderately
transmission. effective. FDA may approve in
2010 for MS.
HP-184 Synthetic protein that functions as a potassium Aventis Chronic SCI; Phase II completed;
channel blocker to improve nerve conduction. limited efficacy; no further
development planned.
Riluzole Sodium channel blocker and antiexcitotoxic NACTN Phase II trial in acute SCI likely
drug marketed for treatment of ALS. to be funded by NACTN.
Glial growth NGF stimulates myelin production from Acorda Preclinical; may initiate Phase I
factors (Neuregulin) remaining oligodendrocytes. in MS; no date projected.
Monoclonal Several antibodies have been identified with Acorda, Biogen Preclinical studies
antibodies potential to repair CNS myelin and restore Idec, Amgen
neurological function in MS and SCI.
AIT-082 (Neotrofin) NGF promotes axonal sprouting. Neuro- Clinical trial completed but no
therapeutics results released.
Inosine + Axiogen- NGFs that promote axon growth in the Boston Life Phase I trial pending with stroke
esis Factor (AF-1) corticospinal tract. Science patients.
Glial derived neuro- Neurotrophic effect on sensory neurons supe- Amgen Phase II trial with advanced Parkin-
trophic factor rior to other nerve growth factors (NGF, NT- son’s; teminated early due to
(GDNF) 3, NT-4/5) lack of efficacy.
Oscillating Field Implanted electrodes above and below lesion Cyberkinetics Small Phase I in acute SCI;
Stimulator (OFS) deliver OFS, which promotes axonal growth. humanitarian device exemption
Andara requested.
517
INDUCE
Continued
DIFFERENTIATION
REPLACE DEAD
OF PROGENITOR CELLS
Traumatic Spinal Cord Injury
CELLS
IMPLANT
REPLACEMENT
NERVE CELLS
GUIDE AXON
ENCOURAGE AXONS
GROWTH
X
n
TO GROW
CHAPTER 16
BLOCK GROWTH
INHIBITORY FACTORS
X
INTRODUCE
TROPHIC FACTORS
X
X
COMPENSATE FOR
LOSS OF MYELIN
PREVENT DISSIPATION
OF NERVE IMPULSES
X
STIMULATE
MYELIN PRODUCTION
X
PREVENT SECONDARY INJURY EFFECTS
PREVENT
GLIAL SCARRING
X
?
BOLSTER
IMMUNE RESPONSE
X
LIMIT
APOPTOSIS
X
BLOCK
X
EXCITOTOXICITY
TABLE 16-9 n SUMMARY OF SPINAL CORD REGENERATION EFFORTS—cont’d
AGENT/
INTERVENTION MECHANISM SPONSOR TRIAL STATUS/RESULTS
Neurotrophic factor NGF improves bowel function in chronic SCI. Regenereon Phase II trial completed; no
(NT-3) further development planned.
IN-1 antibody Binds to Nogo, a myelin-growth inhibitor, Novartis Phase I in Europe; Phase II in
thereby stimulating axonal growth and remye- US considered but no estimated
linization. start date.
Nogo-66 nogo anti-NgR1 antibody that blocks uptake of Biogen Idec Preclinical studies in MS
receptor blocker Nogo. and SCI.
Chondroitinase Enzyme that breaks down chondroitin-6-sulfate Acorda Preclinical studies
ABC proteoglycans (CSPG), a growth inhibitor, to
promote axonal growth.
Recombinant C3 Blocks rho signaling protein, which mediates Alseres Phase I/IIa completed in ‘07;
toxin (Cethrin) inhibitory Nogo and may be responsible for promising results; Phase IIb
apoptosis; stimulates axon regeneration. trials pending but funding
appears to be an issue.
Schwann cell Myelin producers in peripheral nerves cross Clinical evaluation.
transplants into CNS at dorsal root; may be used to de-
liver trophic factors and as bridges to support
axonal growth.
Olfactory Cells may function in 3 ways: encourage cell Portugal, China, Several uncontrolled treatments
ensheathing glial migration, guide direction of axon growth, Russia, Austra- offered overseas - one safety
(OEG) cells provide a bridge or scaffold over cord damage. lia review article; no published
efficacy findings.
Fetal spinal cord Experimental procedure for treatment of Univ. of Florida Phase I trial completed; cells
transplants syringomyelia. survived and filled syrinx.
No further studies - Replaced
by hESC studies (Geron).
Fetal pig neural Replace neural cells and promote differentiation. Diacrin Appears to be safe in Phase I
stem cells trial; no effectiveness results
released.
Bone marrow Autologous bone marrow-derived cells differ- Brazil, Ukraine No US trials planned.
stromal stem cells entiate into neuron and glial cells and improve
functioning in preclinical studies.
Fetal neural stem Cultured neural stem cells derived from a single NeuralStem US Phase I trial for ALS
cells 8-week fetus. at Emory in 2010.
Human embryonic Embryonic stem cells that have been differenti- Geron Phase I trial on FDA hold as
neural stem cells ated into precursors of neuron-support cells. of August 2009. Expect restart
Source is H1 cell line “approved” human in late 2010.
embryonic stem cell line.
Umbilical cord Cells used in treatment of leukemia, autoim- Stemcyte, China, China conducting CB + Lithium
blood stem cells mune diseases (lupus), and sickle cell anemia. India trial. Possibility of US trial in
>2011?
519
INDUCE
DIFFERENTIATION
REPLACE DEAD
X
OF PROGENITOR CELLS
Traumatic Spinal Cord Injury
CELLS
IMPLANT
REPLACEMENT
X
X
NERVE CELLS
GUIDE AXON
ENCOURAGE AXONS
GROWTH
X
X
n
TO GROW
CHAPTER 16
BLOCK GROWTH
INHIBITORY FACTORS
X
X
INTRODUCE
TROPHIC FACTORS X
X
COMPENSATE FOR
LOSS OF MYELIN
PREVENT DISSIPATION
OF NERVE IMPULSES
STIMULATE
MYELIN PRODUCTION X
?
PREVENT SECONDARY INJURY EFFECTS
PREVENT
GLIAL SCARRING
X
BOLSTER
IMMUNE RESPONSE
LIMIT
APOPTOSIS
?
BLOCK
EXCITOTOXICITY
APPENDIX 16-A n Selected References: Architectural Modification

Accessibility in Georgia: a technical and policy guide to access Harber L, Mae R, Orleans P, et al: UFAS retrofit guide: accessi-
in Georgia, Raleigh, NC, 1986, Georgia Council on Develop- bility modifications for existing buildings, New York, 1993,
mental Disabilities. Van Nostrand Reinhold.
An accessible bathroom, Madison, WI, 1980, Design Coalition. Lebrock C, Behar S: Beautiful barrier-free: a visual guide
An accessible entrance: ramps, Madison, WI, 1979, Design to accessibility, New York, 1993, Van Nostrand Reinhold.
Coalition. Mace RL: The accessible housing design file, New York, 1991,
Handbook for design: specially adapted housing, Veterans Admin- Van Nostrand Reinhold.
istration pamphlet 26-13, Washington, DC, 1978, Department
of Veterans Benefits, Veterans Administration.
CHAPTER 17 Neuromuscular Diseases
ANN HALLUM, PT, PhD, and DIANE D. ALLEN, PT, PhD

amyotrophic lateral sclerosis After reviewing this chapter the student or therapist will be able to:
disuse atrophy 1. Describe the basic pathology and medical treatment of amyotrophic lateral sclerosis,
Duchenne muscular dystrophy Guillain-Barré syndrome, and Duchenne muscular dystrophy.
Guillain-Barré syndrome 2. Describe the current goals and interventions for each condition.
overwork damage 3. Describe the “safe” exercise windows related to disuse atrophy and exercise
polyradiculoneuropathy (overwork) damage.
4. Be able to apply intervention concepts discussed in this chapter to other neuromuscular
diseases.
N euromuscular diseases encompass disorders of upper

or lower motor nerves or the muscles they innervate.
This chapter traces the connections among the central ner-
the myopathies (deficit in ability of muscle fibers to contract
forcefully).2 Secondary movement problems include loss of
range of motion (ROM) in immobile muscles and joints, and
vous system (CNS), peripheral nervous system (PNS), and pain or muscle spasms. Adaptability, the ability to sense
musculoskeletal system through the disordered functioning obstacles or changes in the environment and change the
associated with three neuromuscular diseases: amyotrophic course of a movement in response,1 may be affected with the
lateral sclerosis (ALS), which damages upper and lower mo- sensory loss in GBS but is not typically a problem in ALS
tor neurons; Guillain-Barré syndrome (GBS), which com- or DMD.
promises lower motor neurons and the PNS; and Duchenne
muscular dystrophy (DMD), which affects the muscles
AMYOTROPHIC LATERAL SCLEROSIS
themselves. To review the normal connections, upper motor
neurons originate in the motor cortex of the brain (Betz Pathology and Medical Diagnosis
cells). Axons from these upper motor neurons descend by ALS, commonly known in the United States as Lou Gehrig
means of the corticobulbar and corticospinal tracts to synapse disease, is a relentless, degenerative, terminal disease affect-
with lower motor neurons in the brain stem (neurons of the ing both upper and lower motor neurons. Massive loss of
cranial nerves with motor functions) and spinal cord (anterior anterior horn cells of the spinal cord and the motor cranial
horn cells or alpha motor neurons). Simultaneously, corti- nerve nuclei in the lower brain stem results in muscle atro-
cobulbar tract fibers innervate neurons originating within the phy and weakness (amyotrophy). Demyelination and gliosis
brain stem and descending through the spinal cord to proof the corticospinal tracts and corticobulbar tracts caused by
vide additional input to lower (alpha) motor neurons. Axons degeneration of the Betz cells in the motor cortex result in
from the lower motor neurons within both the brain stem upper motor neuron symptoms (lateral sclerosis).
and spinal cord run within the peripheral nerves, which The cause of ALS is unknown; however, numerous theo-
include motor and sensory fibers, to synapse with muscle ries have been proposed. Ninety percent of the cases of ALS
fibers. The muscle fibers respond to excitation by contract- are sporadic without a known genetic component; however,
ing. Depending on the site of the pathology, neuromuscular most neurodegenerative diseases are now thought to be
diseases can be classified as neurogenic or myopathic. related to complex protein misfolding disorders. The latest
ALS and GBS are neurogenic disorders; DMD is a primary research suggests that ALS and other neurodegenerative
myopathy (Figure 17-1). disorders are related to TDP-43 proteinopathy.3 Approxi-
In considering the movement dysfunction associated with mately 5% to 10% of the cases seem to have a complex
these diseases, strength and endurance are most affected, genetic basis coded on ALS1 through ALS8 and other muta-
with flexibility deficits resulting from these.1 All three dis- tions that are associated with frontal lobe dementias. Twenty
orders decrease a person’s ability to generate force in the percent of genetic causes of ALS are thought to be related
affected muscles, with weakness as a primary symptom. to mendelian mutations in the superoxide dismutase–1
Loss of muscle strength can lead to speech, swallowing, and (SOD1) gene (ALS1). Other factors considered in the gene-
respiratory difficulties along with functional limitations. sis of ALS are vascular endothelial growth factors, toxicity
Fatigue is another primary deficit, although the neurogenic leading to motor neuron death, oxidative stress and mito-
disorders tend to result in central fatigue (deficit in ability to chondrial dysfunction related to microglial inflammation,4,5
recruit motor units) as opposed to the peripheral fatigue of and environmental factors.6
521
Men are affected approximately 1.3 to two times more fre-

quently than are women, although the differences are less
with late onset of disease (ages 701).12
Clinical Presentation
The World Federation of Neurology (WFN) has developed
suggested diagnostic criteria (suspected, possible, probable,
and definite) for patients with ALS entering clinical research
trials. Essentially, a patient with “definite” ALS must show
concomitant upper motor neuron and lower motor neuron
signs in three spinal regions or in two spinal regions with
bulbar signs. Either upper or lower motor signs must also be
evident in other regions of the body.13 Exclusionary criteria
are oculomotor nerve pathway abnormalities (the oculomo-
tor nerve is spared in ALS), significant movement disorder
patterns, sphincter control problems, the presence of sensory
and autonomic nervous system (ANS) dysfunction, and cog-
nitive deterioration.14 (Refer to the WFN ALS website
[www.wfnals.org] section on ALS education for up-to-date
criteria used for clinical studies.)
Although a consistent diagnostic criterion for ALS has
been the absence of sensory involvement, some evidence
exists that there is a progressive functional deficit in sensa-
tion, perhaps related to ongoing immobility.15
Similarly, cognitive deficits are considered exclusionary
criteria for an ALS diagnosis. However, a small subgroup of
Figure 17-1 n Primary sites of pathological features of amyo-
patients with both familial and sporadic forms of ALS has
trophic lateral sclerosis (AMLS), Guillain-Barré syndrome (GBS),
been identified as having concomitant evidence of frontotem-
and Duchenne muscular dystrophy (DMD).
poral dementia (FTD), showing lower scores on executive
cognitive functions, word finding, and phrase length.16 A
combination of ALS and FTD suggests a common cause may
The differential diagnosis for ALS is extensive. The pos- be possible.17 Because of these findings, therapists should be
sibility of cervical or lumbar spondylosis, syringomyelia, aware of the possibility of cognitive deficits in their patients
multiple sclerosis, primary lateral sclerosis, and diseases with ALS, manifested as a decrement in executive skills such
associated with lower motor neuron pathology, among other as planning and organization and language problems. Such
diagnoses, needs to be excluded before the diagnosis of ALS patients may have more difficulty following through on
is made.7 Currently, no single laboratory test is available to medication and therapeutic recommendations, and their fam-
confirm a diagnosis of ALS, although creatine phosphoki- ilies may need more support. Unassociated with overall
nase levels are elevated in approximately 70% of patients cognitive impairment, some deficits in action knowledge as
and tend to be higher in patients with limb onset ALS rather opposed to object knowledge have been noted in patients
than bulbar onset.8 Genetic testing to identify the mutations with ALS, correlating with atrophy in the motor and premo-
in the Cu,Zn SOD1 gene is available when a family history tor cortex.18 Specific cognitive deficits, therefore, may be
of ALS is present. Other laboratory tests, such as identifica- more common than previously noted.
tion of biochemical markers in the blood and cerebrospinal The earliest clinical markers heralding ALS are fascicula-
fluid, are used to exclude other neurological diseases. Elec- tions (especially unequivocal fasciculation in the tongue),
tromyography (EMG) and nerve conduction studies can be muscle cramps, fatigue, weakness, and atrophy.13,19 During
helpful to confirm the presence of widespread lower motor initial diagnostic visits, patients frequently report to their
neuron disease without peripheral neuropathy or polyradicu- physicians a profound sense of fatigue or the loss of exercise
lopathy. Neuroimaging studies are used to rule out condi- tolerance.19 Ninety percent of patients report weakness oc-
tions that may have clinical signs similar to those of ALS.9 curring in a striated muscle or group of muscles. Because
Because of the absence of clear laboratory markers of the onset of ALS is insidious, most patients are not aware of
ALS, the clinical diagnosis must be made on the basis of the strength changes, or they have adjusted to the changes
recognition of a pattern of observed and reported symptoms until they have difficulty with a functional activity such as
of both upper and lower motor neuron disease and persistent tying shoes or climbing stairs. Physical examination usually
declines in physical functions supported by inclusionary and demonstrates more widespread weakness and atrophy than
exclusionary diagnostic testing. Because of the overlap of reported by the patient. By the time most patients report
symptoms with other neuromuscular disorders, misdiagno- weakness, they have lost approximately 80% of their motor
sis is not uncommon.10 neurons in the areas of weakness. This demonstrates the
ALS is the most common form of motor neuron disease, plasticity of the nervous system and its drive to adapt to
with an incidence of approximately three to five cases per meet functional goals. The weakness spreads over time to
100,000 persons. Mean age at onset is 57 years, with two include musculature throughout the body. Succeeding symp-
thirds of patients aged 50 to 70 years old at time of onset.11 toms of weakness in other muscles depends on the continued
CHAPTER 17 n Neuromuscular Diseases 523
loss of motor neurons to the 20% threshold needed for per- with upper-extremity activities, and/or is dysarthric or dys-
ception of weakness.20,21 A typical, but not absolute, pattern phasic. Stage 4 identifies patients with nonfunctional move-
of motor progression is early distal involvement followed ment of at least two regions and moderate or nonfunctional
by proximal limb involvement. In some cases bulbar symp- movement of a third area. Stage 5 is death.22 (See Brooks
toms herald the onset of ALS, but bulbar symptoms more and colleagues14 and Pradas and colleagues28 for informa-
commonly occur later in the disease. Flexor muscles tend to tion on the natural history of ALS and its importance in the
be weaker than extensor muscles.22 design of clinical treatment trials.)
Although the atrophy and weakness component of ALS Along with the primary impairments of weakness and
is most obvious, 80% or more of patients show early clinical fatigue affecting body structure and function in ALS, patients
evidence of pyramidal tract dysfunction (e.g., hyperreflexia also have progressive limitations in activity and participa-
in the presence of weakness and atrophy, spasticity, and tion.29 Activity limitations result in gradual loss of indepen-
Babinski and Hoffmann reflexes).13 Although in some cases dence in community and then household tasks. Mechanical
the upper motor neuron signs may be absent clinically, and electronic adaptive devices can help extend indepen-
Chou23 has shown on autopsy that significant involvement dence in some ADLs past the initial strength losses. Partici-
may be present despite the lack of clinical evidence. pation limitations result in progressive isolation from the
The pattern of ALS onset is highly varied, with several community and family unless extraordinary efforts persist
patterns identified by primary area of onset. Lower-extremity to retain a communication system at home and through elec-
onset is slightly more common than upper-extremity onset, tronic media.
which is more common than bulbar onset. Some patients show
initial symptoms in distal musculature of upper and lower Medical Prognosis
extremities. A significant diagnostic feature of the pattern of In almost all cases ALS progresses relentlessly and leads
disease is the asymmetry of the weakness and the sparing to death from respiratory failure. The rate of progression
of some muscle fibers even in highly atrophied muscles. For seems to be consistent for each patient but varies consid-
example, a patient may have weakness of the right intrinsics erably among patients. Patients with an initial onset of
and shoulder musculature or weakness of the left anterior tibial bulbar weakness (dysarthria, dysphagia) and respiratory
muscles. Bulbar symptoms are presaged by tongue fascicula- weakness (dyspnea) tend to have a more rapid progression
tions and weakness, facial and palatal weakness, and swallow- to death than patients whose weakness begins in the distal
ing difficulties, which result in dysphagia and dysarthria. extremities.30 Death usually follows within 2 to 4 years
Pseudobulbar palsy is sometimes present in ALS, manifested after diagnosis, with a small number of patients living for
by spontaneous laughing or crying unrelated to the situation.24 15 to 20 years.10
Despite the pattern of onset, however, the eventual course of Years of survival after diagnosis may change as drug
the illness is similar in most patients, with an unremitting therapies are developed.31 In addition, increasing numbers
spread of weakness to other muscle groups leading to total of patients are electing to prolong life with home-based
paralysis of spinal musculature and muscles innervated by the mechanical ventilation as opposed to palliative or comfort
cranial nerves. Death is usually related to respiratory failure.25 care only.
In a longitudinal study using monthly questionnaires,
direct patient interviews, record reviews, physician inter- Medical Management
views, and family member interviews, Brooks and col- ALS has no known cure and minimal effective disease-
leagues20 followed 702 patients with ALS. Their findings slowing treatments. Mitchell and Borasio24 have created a
suggest that spread of neuronal degeneration occurred more table (see Table 2 in their study) that summarizes the results
quickly to adjacent areas than to noncontiguous areas. The of trials of the many putative ALS-modifying pharmaceuti-
spread to adjacent areas was more rapid at the brain stem, cals. Only riluzole has been approved for treatment of ALS.
cervical, and lumbar regions. Limb involvement after bulbar Riluzole provides very modest improvement over a placebo
onset was more aggressive in men than in women.20 in both bulbar and limb function, but not in actual strength
One study focused on developing methods to assess the of muscles.32 The drug extended lifespan an average of 2 to
natural history of the progression of ALS so that medical and 3 months. The side effects were minimal in some studies,
supportive treatment planning and interventions could be but fatigue and weakness have been noted in 26% and 18%
instituted.26 Hillel and colleagues27 have developed the ALS of patients taking riluzole compared with a placebo.33
severity scale for rapid functional assessment of disease stage. The popular press has reported on nutritional cures for
Their 10-point ordinal scale allows clinicians and therapists ALS, including regular use of vitamin E. However, Orrell
to score patients in four categories: speech, swallowing, and and colleagues34 found insufficient evidence to support
lower-extremity and upper-extremity function (Box 17-1). clinical use of vitamin E supplements in ALS as an additive
A five-point scale of severity is currently being used in to riluzole treatment or as adjunctive therapy, although no
ALS clinical drug trials. Patients in stage 1 (mild disease) apparent contraindication was found to taking the supple-
have a recent diagnosis and are functionally independent in ment. Other nutritional and nonpharmaceutical supplements
ambulation, activities of daily living (ADLs), and speech. have had some success in animal models of ALS, but this
Stage 2 (moderate) identifies patients with mild deficits in has not yet been confirmed in humans.35
function in three regions or a moderate to severe deficit Cannabis has been studied for its effect on spasticity in
in one region and mild or normal function in two other patients with multiple sclerosis and spinal cord injury. In a
regions. Stage 3 (severe) defines patients who need assis- study of 131 people with ALS, 13 used cannabis, with reports
tance because of deficits in two or three regions; for example, of reduction in spasticity, pain, and depression.36 Because of
the patient needs assistance to walk or transfer, needs help the apparent hopelessness of the diagnosis, many physicians,
BOX 17-1n AMYOTROPHIC LATERAL SCLEROSIS SEVERITY SCALE: LOWER EXTREMITY,

UPPER EXTREMITY, SPEECH, SWALLOWING
LOWER EXTREMITIES (WALKING)
Normal
10 Normal ambulation Patient denies any weakness or fatigue; examination reveals no abnormality.
9 Fatigue suspected Patient experiences sense of weakness or fatigue in lower extremities during exertion.
Early Ambulation Difficulties
8 Difficulty with uneven terrain Difficulty and fatigue when walking long distances, climbing stairs, and walking over uneven
ground (even thick carpet).
7 Observed changes in gait Noticeable change in gait; pulls on railings when climbing stairs; may use leg brace.
Walks with Assistance
6 Walks with mechanical device Needs or uses cane, walker, or assistant to walk; probably uses wheelchair away from home.
5 Walks with mechanical device and Does not attempt to walk without attendant; ambulation limited to less than 50 ft; avoids stairs.
assistant
Functional Movement Only
4 Able to support At best, can shuffle a few steps with the help of an attendant for transfers.
3 Purposeful leg movements Unable to take steps but can position legs to assist attendant in transfers; moves legs purposefully
to maintain mobility in bed.
No Purposeful Leg Movement
2 Minimal movement Minimal movement of one or both legs; cannot reposition legs independently.
1 Paralysis Flaccid paralysis; cannot move lower extremities (except, perhaps, to close inspection).
UPPER EXTREMITIES (DRESSING AND HYGIENE)
Normal Function
10 Normal function Patient denies any weakness or unusual fatigue of upper extremities; examination demonstrates
no abnormality.
9 Suspected fatigue Patient experiences sense of fatigue in upper extremities during exertion; cannot sustain work
for as long as normal; atrophy not evident on examination.
Independent and Complete Self-Care
8 Slow self-care Dressing and hygiene performed more slowly than usual.
7 Effortful self-care performance Requires significantly more time (usually double or more) and effort to accomplish self-care;
weakness is apparent on examination.
Intermittent Assistance
6 Mostly independent Handles most aspects of dressing and hygiene alone; adapts by resting, modifying (e.g., use of
electric razor), or avoiding some tasks; requires assistance for fine motor tasks (e.g., buttons,
ties).
5 Partial independence Handles some aspects of dressing and hygiene alone; however, routinely requires assistance for
many tasks such as applying makeup, combing, and shaving.
Needs Attendant for Self-Care
4 Attendant assists patient Attendant must be present for dressing and hygiene; patient performs the majority of each task
with the assistance of the attendant.
3 Patient assists attendant The attendant directs the patient for almost all tasks; the patient moves in a purposeful manner
to assist the attendant; does not initiate self-care.
Total Dependence
2 Minimal movement Minimal movement of one or both arms; cannot reposition arms.
1 Paralysis Flaccid paralysis; unable to move upper extremities (except, perhaps, to close inspection).
SPEECH
Normal Speech Processes
10 Normal speech Patient denies any difficulty speaking; examination demonstrates no abnormality.
9 Nominal speech abnormalities Only the patient or spouse notices speech has changed; maintains normal rate and volume.
Detectable Speech Disturbance
8 Perceived speech changes Speech changes are noted by others, especially during fatigue or stress; rate of speech remains
essentially normal.
7 Obvious speech abnormalities Speech is consistently impaired; rate, articulation, and resonance are affected; remains easily
understood.
BOX 17-1n AMYOTROPHIC LATERAL SCLEROSIS SEVERITY SCALE: LOWER EXTREMITY,

UPPER EXTREMITY, SPEECH, SWALLOWING—cont’d
Intelligible with Repeating
6 Repeats message on occasion Rate is much slower, repeats specific words in adverse listening situation; does not limit
complexity or length of messages.
5 Frequent repeating required Speech is slow and labored; extensive repetition or a “translator” is commonly used; patient
probably limits the complexity or length of messages.
Speech Combined with Nonvocal Communication
4 Speech plus nonverbal Speech is used in response to questions; intelligibility problems need to be resolved by writing
communication or a spokesperson.
3 Limits speech to one-word responses Vocalizes one-word responses beyond yes and no; otherwise writes or uses a spokesperson;
initiates communication nonvocally.
Loss of Useful Speech
2 Vocalizes for emotional expression Uses vocal inflection to express emotion, affirmation, and negation.
1 Nonvocal Vocalization is effortful, limited in duration, and rarely attempted; may vocalize for crying
or pain.
X Tracheostomy
SWALLOWING
Normal Eating Habits
10 Normal swallowing Patient denies any difficulty chewing or swallowing; examination demonstrates no abnormality.
9 Nominal abnormality Only patient notices slight indicators such as food lodging in the recesses of the mouth or sticking
in the throat.
Early Eating Problems
8 Minor swallowing problems Reports some swallowing difficulties; maintains essentially a regular diet; isolated choking
episodes.
7 Prolonged times, smaller bite size Meal time has significantly increased and smaller bite sizes are necessary; must concentrate on
swallowing thin liquids.
Dietary Consistency Changes
6 Soft diet Diet is limited primarily to soft foods; requires some special meal preparation.
5 Liquefied diet Oral intake adequate; nutrition limited primarily to liquefied diet; adequate thin liquid intake
usually a problem; may force self to eat.
Needs Tube Feeding
4 Supplemental tube feedings Oral intake alone no longer adequate; patient uses or needs a tube to supplement intake; patient
continues to take significant (greater than 50%) nutrition orally.
3 Tube feeding with occasional oral nu- Primary nutrition and hydration accomplished by tube; receives less than 50% of nutrition
trition orally.
No Oral Feeding
2 Secretions managed with aspirator Cannot safely manage any oral intake; secretions managed with aspirator and/or medications;
and/or medications swallows reflexively.
1 Aspiration of secretions Secretions cannot be managed noninvasively; rarely swallows.
Adapted with permission from Hillel AD, Miller RM, Yorkston K, et al: Amyotrophic lateral sclerosis severity scale. Neuroepidemiology 8:142, 1989.
especially those not associated with major medical centers movement, some patients require medications such as qui-
having neuromuscular disease units, do not refer patients nine or baclofen to relieve symptoms (see Chapter 36 for
with ALS for services, yet few primary care physicians or information on drug therapies). In a review of studies on the
neurologists have extensive experience in the care of patients treatment of spasticity in ALS, Ashworth and colleagues38
and families coping with ALS because of the low incidence found only one randomized study addressing spasticity: a
of the disease. Yet, referral of patients with ALS to a multi- moderate-endurance exercise regimen decreased spasticity
disciplinary clinic typically extends the patient’s lifespan, at 3 months after initiation of the program. Stretching and
especially patients with bulbar onset of ALS.25,37 massage may prove helpful for nocturnal muscle cramps.25
Kesiktas and colleagues39 report that in a controlled study of
Muscle Spasms and Pain spasticity in patients after spinal cord injury, adding hydro-
Some patients experience muscle cramps and spasms related therapy to a program of medication and exercise decreased
to upper motor neuron pathology, and up to 73% of patients severity of spasms and decreased the amount of medication
complain of pain, typically in the later stages.24 Although required. A similar response could be hypothesized in
most spasms can be relieved with stretching or increased patients with ALS. In addition to muscle spasms, patients
report nonspecific aching and muscle soreness, probably nutritional plans to maintain nutrition and hydration in
related to immobility and trauma to paralyzed muscles dur- patients with motor neuron diseases. Patients with bulbar
ing caregiving procedures. However, many patients do not symptoms and severe dysphagia who are no longer able to
receive adequate pain medication, or the pain is not con- consume nutrients orally because of motor control problems
trolled by the medication taken.40 A Cochrane review in and recurrent aspiration may need a percutaneous endo-
2008 found no randomized or quasi-randomized controlled scopic gastrostomy (PEG) for feeding, depending on the
trials of drug therapy for pain in ALS, although several patient’s wishes for long-term care. Some evidence exists
case series reported the use of acetaminophen, nonsteroidal that the PEG should be performed early in the disease pro-
antiinflammatory drugs (NSAIDs), or opioids.41 Careful cess to prevent severe weight loss and aspiration.48 Although
administration of medications such as baclofen, tizanidine, a PEG does not appreciably lengthen survival time,49 patients
dantrolene sodium, and diazepam is useful for some patients may have less fear of choking or aspiration. Receiving nour-
with spasticity. Because each has a different action and side ishment from a PEG does not prevent the person from taking
effects, the medications may have to be adjusted to find the food orally if desired.
right dosage and combination. In some patients with severe
cramping, botulinum toxin injections might be helpful, but Dysarthria
they must be carefully administered to prevent further weak- Dysarthria, impairment in speech production, is the result
ness. Because many patients have compromised respiratory of abnormal function of the muscles and nerves associated
function, the physician must take great care when prescrib- with coordinated functions of the tongue and lips, larynx,
ing pain medication, especially opiates, which are often soft palate, and respiratory system. Speech impairments are
used when antispasmodics or antiinflammatory pain medi- the initial symptom in most patients with bulbar involve-
cations no longer work.25 Patients should be instructed to ment. Speech intelligibility is compromised by hyperna-
keep a daily reporting log of the effectiveness of the medica- sality, abnormalities of speed and cadence of speech, and
tion so that the dosage can be adjusted if necessary. reduced vocal volume. Speech is further compromised by
inadequate breath volumes for normal phrasing. A possible
Dysphagia option to help patients with severe hypernasality is a pala-
Dysphagia, a difficulty swallowing liquids, foods, or saliva, tal lift prosthesis to augment velopharyngeal function.50,51
accounts for considerable misery in the patient with advanced Because little can be done medically to delay the loss of
ALS, and it must be dealt with aggressively. Patients with speech control, early referral to a speech therapist is essen-
dysphagia have both nutritional and swallowing problems tial. Numerous augmentative and alternative communica-
associated with weakness of the lips, tongue, palate, and mas- tion systems are now available, the simplest being voice
tication muscles.42 As the progressive loss of swallowing amplification systems or homemade point boards and com-
develops, patients are also at extreme risk for aspiration. Most puter-based head or eye tracking text-to-speech systems
patients with dysphagia also have severe problems with man- that can be modified as the patient status changes. The type
agement of their saliva (sialorrhea). If a patient has difficulty of communication system should be chosen with awareness
transporting saliva back to the oropharynx for swallowing, of the patient-caregiver environment.52
choking and drooling are common.43 This condition is discon-
certing to the affected person, who must constantly wipe the Respiratory Management
mouth or have someone do it for him or her. Progressive respiratory failure is the primary cause of death
In addition, secretions are often thickened because of in ALS patients. Respiratory failure is related to primary
dehydration. With pooling of the thickened saliva, the pos- diaphragmatic, intercostal, and accessory respiratory mus-
sibility of aspiration is increased. Viscosity of saliva can cle weakness.53 Respiratory failure should be anticipated
best be treated by hydration and, in some cases, pharma- and discussed early following the diagnosis of ALS so that
ceuticals. Drugs, such as decongestants, antidepressant patients and their caregivers can express their wishes and
drugs with anticholinergic side effects, and atropine-type develop an advanced directive for care in the terminal phase
drugs, can help control the amount of saliva, provided the of the disease.54
patient is well hydrated.44 In extreme cases, various surgi- Physiological tests used to indicate respiratory dysfunc-
cal procedures such as ligation of the salivary gland ducts, tion include vital capacity, sniff nasal pressure, and nocturnal
severing the parasympathetic supply to the salivary glands, oximetry.10 Clinical signs of increased respiratory dysfunction
and excision of the salivary glands have been used effec- are dyspnea with exertion or lying supine; hypoventilation;
tively.45 Newer treatments to decrease excessive secretions weak or ineffective cough; increased use of auxiliary respira-
are radiotherapy and botulinum A toxin injections into tory muscles; tachycardia (also a sign of pulmonary infection
salivary glands.46 with fever and tachypnea); changes in sleep pattern; daytime
Although dietary treatment is not known to be effective in sleepiness and concentration problems; mood changes; and
changing the course of the disease, a nutritious diet to meet morning headaches.55
caloric, fluid, vitamin, and mineral needs must be main- In early stages of patient care, physical therapists (PTs)
tained. Seventy-three percent of patients with ALS have dif- may help manage respiratory dysfunction by providing
ficulty bringing food to the mouth, making them dependent postural drainage with cough facilitation (suctioning if
on others for their dietary needs. Because of the time it takes necessary), especially during acute respiratory illnesses. The
to be fed, many patients decrease their intake. All patients patient and care providers should also be taught breathing
with dysphagia should be referred for a dietary consultation exercises, chest stretching, and incentive spirometry tech-
to determine the choice and progression of solid and liquid niques, as well as postural drainage techniques if the care-
foods and supplements.47 Appel and colleagues47 describe givers are prepared to provide such support. Although
breathing exercises consisting of resisted inspiratory mus- related to instituting mechanical assistance under either
cle training can facilitate functional respiration, even prac- emergency situations or in response to gradual deteriora-
ticing unresisted breathing for 10 minutes three times a day tion. This discussion should occur before the patient devel-
has been shown to result in improved function.56 An assess- ops respiratory failure. Acute respiratory failure can be
ment of the home environment is imperative to identify frightening, and few patients or family members are pre-
sleeping positions and energy conservation techniques that pared to forego intubation and artificial ventilation during
can be incorporated into the patient’s daily life. the emergency. Patients and caregivers should understand
As respiratory symptoms increase, oxygen at 2 L/min or that not making a decision about mechanical ventilation,
less can be used intermittently at home. When hypoventila- noninvasive or invasive, is a decision to support mechanical
tion with a decline in oxygen saturation becomes common ventilation.63
during sleep, resulting in morning confusion and irritability, Physicians and health care workers who work with the
patients have the option to initiate noninvasive, positive- patient and family must be aware of their own feelings and
pressure ventilation (NIV) such as bilevel positive airway beliefs about prolonging life. For example, a healthy physi-
pressure (BiPAP). BiPAP, which provides greater inspira- cian or therapist who values control and an active lifestyle
tory pressure than expiratory pressure to decrease the effort may envision a life on a ventilator as intolerable and pass
of breathing, can be administered by either mask or con- that value on to the patient, who may or may not have the
toured nasal delivery systems. Some evidence indicates that same needs. The patient’s decision, or change in decision,
early use of NIV can increase survival time by several must be respected by the medical team involved in care.64 In
months and increase quality of life.57 When a patient can no medical centers that use a team approach, patients and
longer benefit from NIV, a decision must be made about families may find support by meeting with counselors or
initiating ventilation by tracheostomy or palliative care.58 peers with ALS who are making or have made decisions
(See also Miller and colleagues59 for an excellent discussion about long-term ventilator care.
of practice parameters in the decision-making process related
to ventilatory support.) Although in the initial stages of ALS Therapeutic Management of Movement
most patients indicate they would not want prolonged respi- Dysfunction Associated with ALS
rator dependence at home, patients may change their minds Perhaps because of the multitude of issues to consider when
as they adapt to the disease restrictions.60 A small study of managing the impairments and limitations associated with
patients who started tracheostomy intermittent positive- ALS, evidence suggests that patients treated by a specialized
pressure ventilation (TIPPV) demonstrated increased long- ALS multidisciplinary team fare better than do those treated
term survival (2 to 64 months).54 In another series of by single-source providers,65 or in general neurology clin-
70 patients on long-term TIPPV, 50% of the patients were ics.33 A Cochrane review of the evidence for multidisci-
living after 5 years; however, 11.4% of these patients had plinary care advantages in this population concluded that the
entered a “locked-in state in which they were unable to com- evidence is of low quality, so far, with no controlled trials
municate in any manner.”61 Decisions about long-term res- identified.66 Whether administered through an ALS-specific
pirator use should be made by the patient and involved team or not, therapeutic management will necessitate ex-
family members or partners, with input from the interdisci- amination of the patient’s current status, evaluation of the
plinary team caring for the patient. Discussions of preferred deficits in relation to patient preferences and needs, and es-
long-term care options should be revisited as the patient’s tablishment of a plan based on mutually determined and
condition changes. realistic goals. The rate of the patient’s disease progression,
If a patient decides that home ventilation is a reasonable the areas and extent of involvement, and the stage of illness
option, those involved in the decision should visit another must be considered. A patient at the initial stages will have
patient who is using in-home mechanical ventilation, if pos- different needs than a patient at later stages who has chosen
sible. Because the decision for home mechanical ventilation NIV or tracheostomy ventilation that may extend life span at
(HMV, NIV, or TIPPV) also affects the life of the patient’s a markedly reduced mobility level. The goal at all stages is
spouse, children, and extended family who may be respon- to optimize health and increase the quality of life. With
sible for some aspects of home care, or whose lives may be guidance and environmental adaptations, patients with
affected by the presence of in-home nurses or attendants, the slowly progressing weakness may be able to continue many
decision for HMV should not be taken lightly. Extensive of their ADLs for an extended number of years. In the final
preparation, ongoing support, and respite options for care- stages of the disease, when the patient is bedridden, pro-
givers are necessary if HMV is to be successful. Success of grams to increase strength or endurance are not appropriate,
HMV also depends on such variables as third-party payment and interventions such as stretching may not effectively
for home care equipment and nurse or attendant staffing, control contracture development. However, patients may
working status of the partner or spouse, age and physical still benefit from positioning and range-of-motion (ROM)
fitness of the spouse and children, pre-ALS family psycho- exercises to decrease muscle and joint pain related to im-
social interactions, and financial factors. HMV should be mobility. The prescription of assistive devices and training
viewed as long term, often extending for more than 1 year. of caregivers will also be needed. The efficacy of therapeutic
Initiation of HMV results in a reasonable perceived quality interventions will be related to the timing of interventions,
of life for the patient, yet caregivers report that their quality the motivation and persistence of the patient in carrying out
of life may be lower than the patient’s because of the burden the program, and support from family members or caregiv-
of care that must be provided.62 ers.67 Objective documentation of outcome measures will
With chronic respiratory insufficiency, the patient and help justify the usefulness of therapeutic interventions at all
family must be involved in the long-term care decisions stages of this disease.
Examination n Baseline testing of muscle strength (manual muscle

The extent of the therapeutic examination of a patient with testing [MMT] or electronic handheld dynamometer
ALS will depend on whether the therapist is working as a testing if standards are clear and can be replicated),
member of a rehabilitative team or as an independent or ROM, spasticity, and endurance; documentation of
clinic-based therapist receiving a referral to evaluate and any areas of atrophy.
treat. PTs and OTs working as team members may have a n Assessment of functional activity level (using a stan-
more circumscribed role related to gross motor function and dardized test or assessment tool whenever possible) to
ADLs, with other consultants focusing on bulbar, respira- include, as appropriate: transfers, gait, upper-extremity
tory, and environmental adjustments. The therapist working function, postural control, and assistive devices; sug-
in a facility without a neuromuscular disease clinic or in a gested tools include the ALS functional rating scale
community or rural environment, however, should be aware (ALSFRS),70 the ALS severity scale (ALSSS),27 timed
of the need to carry out a broad-based assessment. In addi- walk test, or Purdue Pegboard.70
tion to the standard neuromuscular, musculoskeletal, and n Documentation of pain (type, site, and intensity; use
functional-level examinations, the therapist should also body chart and subjective pain scale); identify what
evaluate the patient’s stated or observed functional problems makes pain worse or better.
relative to bulbar and respiratory impairments, environmen- n Assessment of bulbar and respiratory function. (For
tal blocks to independence, and caregiving demands. an in-depth evaluation of bulbar function, the patient
If possible, before the patient’s initial visit, the therapist should be referred to an ear, nose, and throat clinic
should contact the patient and request that he or she keep an or communications disorders clinic unless full eval-
activity log for several days. If an early contact is not possi- uation is available in a comprehensive ALS clinic.
ble, the therapist can assign that task during the initial ses- See Table 17-1 for bulbar and respiratory evaluation
sion. The log should include 15-minute time increments in suggestions.)
which the patient or caregiver can record what she or he was n Environmental assessment with a focus on energy
doing during a specific period. The log should also indicate conservation and safety at current and future func-
whether the patient was experiencing fatigue or pain during tional capabilities.
the activity and how the patient perceived her or his respira- Brinkmann and colleagues70 identify standards for assess-
tory status. An example of an activity log and how it is used ment of patients with ALS in clinical trials. The review and
is shown in Figure 17-2. The sense of fatigue with repetitive description of standardized methods for performing recom-
muscle activity or functional activity should be specifically mended tests and measurements is extremely valuable for
tracked by the patient. any therapist assessing and treating patients with ALS.
Weakness will be the primary deficit, with other problems In evaluating the results of the examination, the therapist
following depending on the location of strength loss. Muscle should synthesize data to define the following, all of which
weakness and the experience of fatigue may be independent are necessary for developing goals with the patient.
measures of ALS pathology, however.68 Although weakness n Rate of the patient’s disease progression
may affect balance during gait, patients with ALS have not n Distribution of weakness and spasticity, respiratory
shown deficits in postural control during quiet stance despite factors leading to hypoxemia, and ease of fatigability
significant paresis or tone changes, possibly because sensa- and bulbar involvement
tion is relatively preserved.69 n Phase of the disease
The therapist’s examination will vary depending on the n Any preexisting impairments and/or activity limita-
patient’s situation29; however, a typical initial assessment tions (see Chapter 8)
may include the following:
n Review of the patient’s medical and activity records, Goals of Therapeutic Intervention
especially time since diagnosis, time course of disease Intervention goals and the recommended exercise and
progression to date, current medications, concurrent activity program designed by PTs or OTs must be based
medical issues, current activities and participation and on the patient’s personal goals. Goals are often a difficult
tolerance for them. area for therapists to discuss with the patient because the
n History should focus on current and recent activities disease is progressive despite intervention. Patients, thera-
and participation signifying patient’s lifestyle, ADL pists, and physicians commonly assume that because noth-
tasks, hobbies or interests, and work focus; primary ing can be done to “cure” the disease, not making addi-
complaints, including weakness, fatigue, pain, respira- tional demands on a patient who is already coping with
tory status, safety, or speech and swallowing issues; daily loss is somehow kinder. Some believe that exercise
psychosocial support issues (family, caregivers, and programs may create false hopes that exercise will delay
agencies); patient’s and family members’ understanding progression. Others believe that exercise will hasten pro-
of ALS and the likely progression and prognosis; and gression.71 The literature on rehabilitation in neuromuscu-
patient’s current concerns and goals. lar disorders, however, suggests that patients with ALS can
n Screening for multisystem involvement should include benefit from carefully designed exercise and activity pro-
checking vital signs at rest, skin integrity, bony abnor- grams. Active participation in determining goals for ther-
malities, sensory integrity, communication ability, and apy can provide the patient and the family with some sense
ability to follow multistep commands. More extensive of control over a difficult situation.7
examination of systems showing deficits may be indi- The general, broad goals for both patient and therapist
cated, or the patient may be referred to appropriate are related to maintaining maximal independence in daily
health care professionals. living and a positive quality of life for as long as possible.
Figure 17-2 n Example of a log for monitoring activity level of patients with amyotrophic
lateral sclerosis.
TABLE 17-1 n COMMON PHYSICAL FINDINGS IN BULBAR AMYOTROPHIC LATERAL SCLEROSIS
METHOD OF PROGRESSION PROGRESSION

ANATOMICAL SITE INNERVATION EVALUATION OF FINDINGS OF SYMPTOMS
GROUP I
Tongue XII Inspect for fasciculations Fasciculations evident Dysarthria (disturbance of
at rest lingual-alveolar consonants
t, d, l, and so on)
Range of motion Slow, incomplete lateral Inability to clear buccal sulcus
movements of food
Loss of lateral force Marked dysarthria (slow rate
Unable to reach palate and slurring of consonants)
with mouth open
Protrusion Unable to protrude beyond Oral transport difficulties
lips Dietary changes
Perform rapid lateral Unable to protrude beyond Speech intelligibility problems
motion incisors
Atrophy evident
Paralysis
Lips VII Suck on gloved finger Lack of suction Inability to whistle
Smile or curl lips over Inability to complete a Inability to use a straw
teeth seal Dysarthria (loss of bilabial
Hold seal and blow out Inability to purse lips consonants p and b)
cheeks Drooling
GROUP 2
Palate V, X, XI Visual examination Unsustained or slow Dysarthria (hypernasal speech)
during phonation and palatal elevation Inability to use a straw
stimulation of gag Soft palate fails to reach Nasal air emission during speech
Puff out cheeks to check Passavant ridge Nasopharyngeal reflex on
for nasal air leak (hold Absence of palatal swallowing
lips closed if necessary) movement
Muscles of mastication V Palpate during bite Noticeable wasting Chewing fatigue
Masseter, temporalis Visual inspection for Unable to palpate Elimination of specific, tough
wasting contraction foods from diet
Dietary changes (soft foods
and liquids)
Mouth breathing and drying
of secretions
Pterygoids Move jaw from side to No observable lateral jaw Unable to use dentures
side movement
GROUP 3
Neck and shoulder XI
Trapezius Hold arm in coronal plane, Progressive inability to Inability to comb hair
hand externally rotated, raise the arm (often Inability to perform facial
as patient elevates arm asymmetrical weakness) grooming
against resistance while
the trapezius is palpated
Sternocleidomastoid or Turn the head against Progressive weakness Inability to lift head when supine
mounted head support resistance applied to in turning the head Inability to support head while
opposite side of against resistance sitting; wears neck collar, has
patient’s chin (often asymmetrical) weakness
Vocal cords X Mirror or fiberoptic Progressive loss of Strained or strangled voice
laryngoscopy abduction of vocal cords: Short of breath (stridor usually not
mild abductor weakness, present because of impaired
near-midline paralysis respiratory function)
Paradoxical vocal cord
movement
TABLE 17-1 n COMMON PHYSICAL FINDINGS IN BULBAR AMYOTROPHIC LATERAL SCLEROSIS—cont’d
METHOD OF PROGRESSION PROGRESSION

ANATOMICAL SITE INNERVATION EVALUATION OF FINDINGS OF SYMPTOMS
GROUP 4
Extraocular muscles III, IV, VI Assessment of extraocular Limitation of extraocular Limitation of gaze
movements movement
Respiratory group
Diaphragm C3-5 Pulmonary function test or Diminishing vital capacity: Shortness of breath during
handheld respirometer 1.5-2.0 L exertion if patient has
for vital capacity remained active
Intercostal C7-L3
Accessory muscles of VII, XI, XII, Cough 1.0-1.5 L Weak cough
respiration C5-8 Sustain a vowel Change in speech phrasing
Blow against a tissue (5-10 syllables per breath)
0.5-1.0 L Speech produced in syllable-
by-syllable fashion (if vocal)
Shortness of breath on swallowing
Modified with permission from Hillel AD, Miller RM: Bulbar amyotrophic lateral sclerosis: patterns of progression and clinical management. Head Neck
11:51–59, 1989. Copyright 1989. Reprinted by permission of John Wiley & Sons, Inc.
More specific therapeutic goals are (1) maintenance of decrease in activity level after the onset of ALS can lead
mobility and independent functioning, to include safe mobil- quickly to marked cardiovascular deconditioning and disuse
ity for patient and caregiver; (2) maintenance of maximal weakness. The disuse weakness lowers muscle force produc-
muscle strength and endurance within limits imposed by tion and reduces muscle endurance.74
ALS; (3) prevention and minimization of secondary conse- Exercise or Overwork Damage. Anecdotal evidence
quences of the disease, such as contractures, thrombophlebitis, that muscle activity or overwork exercise can lead to a loss
decubitus ulcers, and respiratory infections7,67; (4) manage- of muscle strength has been reported since the poliomyelitis
ment of energy conservation techniques and respiratory epidemic of the 1940s and 1950s.75 During that epidemic,
comfort; (5) determination of adaptive equipment needs to physicians and therapists noted that patients with poor- and
include mobility, self-help and feeding devices, augmenta- fair-grade muscles who exercised repeatedly or with heavy
tive communication units, and hygiene equipment that resistance after reinnervation often lost the ability to con-
supports both patient and caregiver7; and (6) eliminating or tract the muscle at all76 (see Chapter 35). Controlled testing
preventing pain.72 of this observation suggests that overwork damage occurs in
mostly denervated muscles, not in all muscles. Reitsma77
Therapeutic Considerations noted that vigorous exercise damaged muscles in rats if less
To prevent more rapid functional loss than expected from than one third of motor units were functional. If more than
the natural history of the disease, both the patient and thera- one third of the motor units remained, exercise led to hyper-
pist must delicately balance the level of activity between the trophy. An additional mechanism of potential overwork
extremes of inadequate exercise and excessive exercise. damage is inhibition of the collateral sprouting of intact
Exercise has been recommended for the general public for axons to innervate “orphaned” muscle fibers when other
its many benefits.73 Inadequate exercise may result in loss of axons degenerate. Yuen and Olney78 provided evidence that
strength and endurance from disuse, as well as secondary collateral sprouting of intact axons can partially reinnervate
problems such as loss of ROM, muscle cramping, and pain. orphaned muscle fibers in ALS. In a rat model, highly inten-
Excessive exercise may result in excessive fatigue and con- sive activity reduced the ability of adjacent axons to sprout
sequent inability to perform ADLs during recovery periods. after fewer than 20% of intact motor units remained.79 In
Overuse injury with excessive strengthening exercise may contrast, vigorous exercise in a mouse model had no adverse
also lead to unnecessary pain and loss of strength. The next effect on the course of ALS.80 Lui and Byl81 systematically
two sections review the evidence for the optimum amount of reviewed the literature reporting exercise effects in animal
activity or exercise. models of ALS and calculated an effective size of 1.39
Disuse Atrophy. Because ALS is a disease of older (where numbers over 0.8 are considered large) in favor of
adults, patients may not have maintained their aerobic fitness exercise. The few negative effects they noted were associ-
or muscle strength before the onset of their neuromuscular ated with either very-high–intensity exercise or a slow rate
problem. Newly diagnosed patients also commonly report of exercise (slower than usual activity for animals when
that they had markedly decreased their activity level in the unrestricted in activity). In addition to generic overwork,
months before diagnosis because of a sense of fatigue or in- evidence exists that repeated maximal eccentric contractions
creasing clumsiness from increasing weakness. If the patient may specifically damage even normal muscle fibers, resulting
had led a sedentary lifestyle before diagnosis, the additional in muscle weakness of several weeks’ duration.82 Although
normal muscle eventually adapts to repeated eccentric exer- the treating therapist every 14 days. Data were evaluated for
cise, whether the reparative effect is possible in patients with 3 and 6 months after initial assessment. All patients showed
neuromuscular diseases is uncertain. Aboussouan55 reviews continued disease progression; however, in all cases, at the
some of the specific mechanisms of exercise intolerance in 6-month assessment patients who exercised showed positive
neuromuscular diseases, including mitochondrial dysfunc- effects in maintenance of muscle strength, less fatigue, less
tion, abnormal muscle metabolism, impaired muscle activa- spasticity, less pain, and higher functional ratings.93 In an-
tion, and central activation failure. other randomized controlled trial, moderate load and moder-
Many researchers have expressed concern about the pos- ate-intensity resistance exercises prescribed individually to
sible relation between high-resistance exercise and muscle patients with ALS in the early stages resulted in significantly
fiber degeneration in humans with motor neuron disease.83,84 less decline in function, small improvements in strength, and
Because of the concerns about damage from stressing sub- no reported adverse effects, compared with patients who
stantially denervated muscles, Sinaki and Mulder85 published performed stretching exercises alone.94 A Cochrane review
recommendations in 1978 that patients with ALS not engage designated the quality of the Drory and colleagues (2001)
in any vigorous exercise and focus instead on exercise asso- study as “fair” and the Dal Bello-Haas and colleagues94 study
ciated with walking and daily activities. On the other hand, as “adequate.”95 Table 17-2 summarizes some of the studies of
McCrate and Kaspar86 review the possible mechanisms by strength training in neuromuscular diseases.
which exercise protects nerves from more rapid degenera- Fewer researchers have considered endurance in neuro-
tion. Evidence regarding the positive benefits of exercise in muscular disorders.73 Sanjak and colleagues87 noted that
ALS has been accumulating, with fewer adverse effects than exercise energy requirements during bicycle ergometry test-
some expected. ing were greater than expected, possibly because of motor
Sanjak and colleagues87 reported that muscle damage inefficiency caused by weakness. Work capacity and maxi-
does not necessarily result from resistance exercise testing mal oxygen consumption were decreased, but heart rate,
or training, although fatigue occurs more easily during both respiratory responses, and blood pressure were within nor-
anaerobic and aerobic exercise. Milner-Brown and Miller88 mal limits. Wright and colleagues96 found small positive
found that mild progressive resistance exercise was helpful physiological effects from an aerobic walking program in
in neuromuscular disorders if the patient had muscle strength patients with slowly progressive neuromuscular disorders.
in the good (4/5) to normal (5/5) range. They determined Pinto and colleagues97 provided eight ALS patients with
that patients should begin their exercise program early NIV during exercise to compensate for respiratory insuffi-
because strength training of muscles with less than 10% ciency. Patients walked on a treadmill for 10 to 15 minutes
of normal function was generally not effective. Aitkens and to the point of subjective fatigue, leg pain, heart rate above
colleagues89 noted strength gains of 4% to 20% without 75% of resting value, or desaturation of oxygen not correct-
deleterious effects after a 12-week program of moderate- able with NIV. In comparison to a nonexercising control
resistance (30% of maximum isometric force) exercises in group, the exercising group had a significant reduction in the
patients with slowly progressive neuromuscular diseases. rate of decline of respiratory function test results, strength,
Kilmer and colleagues,90 in the same population, found no and function over the 1-year training period.97
additional advantage to high-resistance training (12 weeks Endurance training for longer than 10 to 15 minutes
of exercise using the maximum isometric force the individ- in patients with ALS may be restricted by central fatigue,
ual was able to lift 12 times) and noted evidence of over- the decreased ability to recruit all motor units or develop
work in some subjects. In a case report of a patient with high discharge rates,98 and not merely respiratory func-
ALS, strengthening 6 days a week for 10 weeks with pro- tion. Sharma and colleagues99 explored the mechanism of
prioceptive neuromuscular facilitation (PNF) patterns using fatigue in ALS. Both maximum voluntary contraction and
maximal resistance applied manually or with tubing resulted tetanic force decreased in patients with ALS compared
in strengthening of 14 muscle groups out of 18 with no with controls following a 25-minute low-intensity inter-
adverse effects.91 Aksu and colleagues92 compared a super- mittent exercise, but with similar recovery. Fatigue may
vised versus home exercise protocol in 26 ambulatory ALS thus be a consequence of chronic denervation resulting in
patients. They noted that supervised breathing exercises, secondary muscle changes such as altered muscle metabo-
stretching, manually applied resistance exercise with PNF, lism and impaired calcium kinetics along with the loss of
and functional mobility training 3 days a week for 8 weeks motor unit activation.99
resulted in small gains in function in the first 4 weeks and In addition to strength and endurance gains from exercise,
a slower decline over the subsequent 10 months compared ongoing, gentle exercise programs may also help decrease
with home-based breathing, stretching, and active ROM persistent pain and muscle stiffness that often accompany
exercises. The groups were not randomly allocated but were weakened, overtaxed muscle groups.100 A case study of a
not significantly different in the measured variables at base- patient with ALS undergoing a focused exercise program
line.92 In a randomized controlled trial, Drory and col- revealed a positive psychological effect on the patient’s cop-
leagues93 assigned 25 patients with ALS to a group continuing strategies.101 Besides exercise programs, some prelimi-
ing their normal daily activities or a group participating in nary evidence exists to suggest that creatine supplementation
a moderate daily program of exercise individualized for may increase isometric power in patients with ALS over the
each patient. The primary exercise focus was to have mus- short term.102 Modafinil has been noted to have potential in
cles of the trunk and limbs work against “modest” loads helping with severe fatigue in ALS.103
while undergoing significant shortening (not lengthening or Many studies focus on the impact of exercise on mus-
eccentric contractions). The exercises were completed cle strength; however, knowledge of impairments does
twice daily for 15 minutes at home with phone contact by not necessarily correlate directly with functional status.
TABLE 17-2 n SUMMARY OF STRENGTH TRAINING STUDIES IN NEUROMUSCULAR DISEASES
STUDY POPULATION DURATION TRAINING

AUTHOR AND SAMPLE SIZE OF TRAINING MODALITY TRAINING PROTOCOL RESPONSE(S)
Vignos and Various neuromuscular 12 months Weight training Unspecified, but based Strength increased;
Watkins, diseases (NMDs) (multiple muscle on 10-repetition percentage increase
1966291 (24) groups) maximum (RM) correlated with initial
strength
Milner- Various NMDs (12) .12 months Weight training Initially one set of Strength increased
Brown and (variable) (elbow flexion and 10 reps based on significantly when
Miller, knee extension) 15 RM performed on the initial degree of
198888 alternate days; gradu- strength loss was not
ally increased to a severe (,10%)
maximum of five sets
4 days/week; protocol
individualized
McCartney Various NMDs (12) 9 weeks Weight training 3 days/week; initially Strength and muscular
et al, (arm curl and leg two sets of 10-12 reps endurance increased;
1988297 press) at 40% of 1 RM; considerable inter-
gradually progressed to subject variability
three sets of 10-12 reps
(one set at 50%, 60%,
and 70% of 1 RM);
contralateral arm
control
Aitkens et al, Slowly progressive 12 weeks Weight training 3 days/week; resistance Significant improvement
199389 NMD (27) and able- (elbow flexion, at 30% of 1 RM; work in most isokinetic
bodied controls (14) knee extension, increased commensu- strength measures
grip one side rate with ability (not grip) in both
only) groups; cross-training
effect
Kilmer et al, Slowly progressive 12 weeks Weight training 3-4 days/week; high- Results mixed;
199490 NMD (10) and able- (elbow flexion, resistance exercise increase in leg
bodied controls (6) knee extension, (resistance based on strength but decrease
one side only) 12 RM; progressed in arm strength in
from one to five sets of NMD
10 reps)
Lindeman MD (33) and HMSN 24 weeks Weight training 3 days/week; initially In MD group, no
et al, (29); nonexercise (knee extension three sets of 25 reps change in strength
1995325 control group and flexion, hip at 60% of 1 RM; pro- In HMSN group,
extension and gressed to three sets increased strength of
flexion) of 10 reps at 80% of knee extensors; no
1 RM adverse effects
Drory et al, ALS (25): randomly 24 weeks Moderate load, Twice daily, 15 min per Treatment group:
200193 assigned to treatment trunk and limbs, session maintenance of
or control groups concentric strength, less fatigue,
contractions less spasticity, less
pain, higher function
Aksu et al, ALS (26): convenience 8 weeks Breathing exercises, 3 days/week supervised Increased ROM,
200292 assignment to treat- PNF, stretching, vs home strength in treatment
ment and control vs stretching and group; function
groups ROM and breath- sustained better in
ing exercises treatment group
Dawes et al, Various NMDs: 8 weeks Walking and Walking for 20 min at Treatment group had
2006326 11 randomly strengthening light to moderate inten- increase in leg
allocated to control exercises sity alternating days muscle strength; no
group, nine to with progressive resis- change either group
treatment group tance and repetitions in in 2-min walk test
strength
Continued
TABLE 17-2 n SUMMARY OF STRENGTH TRAINING STUDIES IN NEUROMUSCULAR DISEASES—cont’d
STUDY POPULATION DURATION TRAINING

AUTHOR AND SAMPLE SIZE OF TRAINING MODALITY TRAINING PROTOCOL RESPONSE(S)
Dal Bello- ALS (27 early stage): 24 weeks PT-prescribed 3 days/week resisted Slowed decline in
Haas et al., randomly allocated to resistance exercises exercise plus stretch vs treatment group and
200794 resistance exercises performed at home just stretch group small strength gains
plus stretch or just to patient tolerance
stretch groups
HMSN, Hereditary motor and sensory neuropathy; MD, myotonic dystrophy; PNF, proprioceptive neuromuscular facilitation; PT, physical therapist; ROM,
range of motion.
Although some research has shown improvements in mus- patients must be instructed about signs and symptoms
cle force production with strengthening and endurance that indicate overwork, including feeling weaker within
training, associated functional improvements were evident 30 minutes after exercise, having excessive soreness
in some studies92 but not others.104 Jette and colleagues82 24 to 48 hours after exercise, and experiencing severe
calculated the percentage of predicted normal maximal muscle cramping, heaviness in the extremities, or pro-
isometric force (%PMF) relative to four walking levels in longed shortness of breath105; and therapists should
patients with ALS: unable to walk, walking within the check with an independently exercising patient regularly
home only, walking in the community with assistance, and to assess whether any deterioration in strength may be
independent walking in the community. Although they from progression of the disease or overwork weakness.
found great variation in muscle force production between If a patient shows evidence of significant, persistent
and within the different levels of walking for each patient, weakness after institution of an exercise program or persis-
they demonstrated that relatively small changes in force tent morning fatigue after exercise on the previous day, the
production were associated with losses of functional lev- therapist must carefully redesign the patient’s exercise pro-
els. For example, on average, when an independent ambu- gram and activity level and increase the frequency of moni-
lator began to need assistance in the community, the lower- toring the patient’s program. The program must be adjusted
extremity strength dropped to less than 54%PMF. When as the disease progresses. Figure 17-3 is a diagram showing
the patient became an in-home ambulator only, the average the appropriate exercise “window” for use in working with
strength dropped to approximately 37%PMF, and it was a patient with a neuromuscular disorder.
approximately 19%PMF when the patient was no longer
able to walk. Jette and colleagues82 acknowledge that Therapeutic Interventions
many factors need to be considered when interpreting Maintenance of strength and endurance requires daily activ-
their work; however, their study relates functional skills ity and repetitive muscle contractions. In normal persons,
to isometric muscle force production in a concrete way. absence of muscle contraction can result in decreases of 3%
Factors such as spasticity, age at onset of ALS, prior levels to 5% in muscle strength per day. If the patient’s exercise
of fitness and activity, and psychological factors, including level requires less than 20% of the maximal voluntary con-
past responses to extremely challenging situations and traction of the muscles, a decrease in strength will occur; yet
satisfaction with social support, must also be considered. overwork must be avoided.106
Based on the evidence and current practice, exercise Sinaki107 has described three phases and six substages of
prescription in the early stages of ALS should address the ALS with recommended exercise levels (Box 17-2). Although
following72: therapists should not assume that all patients will fit precisely
1. To improve compliance, include both a formal exercise within the stages as described, the stages do provide sugges-
program and enjoyable physical activities.
2. Include activities with opportunities for social devel-
opment and personal accomplishment.
3. Strengthening programs should emphasize concentric
rather than eccentric muscle contractions; use moder-
ate resistance rather than high resistance; and focus on
muscles that have at least antigravity strength.
4. Endurance programs should be monitored for signs of
fatigue, more so when continuous activity lasts longer
than about 15 minutes. Activity programs should
include rest periods.
5. Patients should ensure that they have adequate oxy- Figure 17-3 n Exercise window for normal and damaged or de-
genation, aeration, and carbohydrate loads73 as well as nervated muscles. (From Coble NO, Maloney FP: Effects of exercise
adequate fluids before exercising. on neuromuscular disease. In Maloney FP, Burks JS, Ringel SP,
6. Muscle strength must be monitored to assess for possi- editors: Interdisciplinary rehabilitation of multiple sclerosis and
ble overwork weakness; in unsupervised programs, neuromuscular disorders, New York, 1985, JB Lippincott.)
BOX 17-2n EXERCISE AND REHABILITATION PROGRAMS FOR PATIENTS WITH AMYOTROPHIC

LATERAL SCLEROSIS ACCORDING TO STAGE OF DISEASE
PHASE I (INDEPENDENT) PHASE II (PARTIALLY INDEPENDENT)
Stage 1 Stage 4
Patient Characteristics Patient Characteristics
Mild weakness Hanging-arm syndrome with shoulder pain and sometimes
Clumsiness edema in the hand
Ambulatory Wheelchair dependent
Independent in activities of daily living (ADLs) Severe lower-extremity weakness (with or without spasticity)
Treatment Able to perform ADLs but fatigues easily
Continue normal activities or increase activities if sedentary Treatment
to prevent disuse atrophy Heat, massage as indicated to control spasm
Begin program of range-of-motion (ROM) exercises Preventive antiedema measures
(stretching, yoga, tai chi) Active assisted passive ROM exercises to the weakly sup-
Add strengthening program of gentle resistance exercises ported joints; caution to support, rotate shoulder during
to all musculature with caution not to cause overwork abduction and joint accessory motions
fatigue Encourage isometric contractions of all musculature to tol-
Provide psychological support as needed erance
Stage 2 Try arm slings, overhead slings, or wheelchair arm supports
Patient Characteristics Motorized chair if patient wants to be independently mobile;
Moderate, selective weakness adapt controls as needed
Slightly decreased independence in ADLs, such as: Stage 5
n difficulty climbing stairs Patient Characteristics
n difficulty raising arms Severe lower-extremity weakness
n difficulty buttoning clothing Moderate to severe upper-extremity weakness
Ambulatory Wheelchair dependent
Treatment Increasingly dependent in ADLs
Continue stretching to avoid contractures Possible skin breakdown as a result of poor mobility
Continue cautious strengthening of muscles with manual Treatment
muscle testing (MMT) grades above F1 (31); monitor Encourage family to learn proper transfer, positioning
for overwork fatigue principles, and turning techniques
Consider orthotic support (e.g., ankle-foot, wrist, thumb Encourage modifications at home to aid patient’s mobility
splints) and independence
Use adaptive equipment to facilitate ADLs Electric hospital bed with antipressure mattress
Stage 3 If patient elects home mechanical ventilation (HMV), adapt
Patient Characteristics chair to hold respirator unit
Severe selective weakness in ankles, wrists, and hands PHASE III (DEPENDENT)
Moderately decreased independence in ADLs Stage 6
Easily fatigability with long-distance ambulation Patient Characteristics
Ambulatory Bedridden
Slightly increased respiratory effort Completely dependent in ADLs
Treatment Treatment
Continue stage 2 program as tolerated; use caution not to For dysphagia: soft diet, long spoons, tube feeding,
fatigue to point of decreasing patient’s ADL independence percutaneous gastrostomy
Keep patient physically independent as long as possible To decrease flow of accumulated saliva: medication, suction,
through pleasurable activities such as walking surgery
Encourage deep breathing exercises, chest stretching, For dysarthria: palatal lifts, electronic speech amplification,
postural drainage if needed eye-pointing electronics
Prescribe wheelchair, standard or motorized, with modifi- For breathing difficulty: clear airway, tracheostomy,
cations to allow eventual reclining back with head rest, respirator if patient elects HMV
elevating legs Medications to decrease impact of dyspnea
Modified with permission from Sinaki M: Exercise and rehabilitation measures in amyotrophic lateral sclerosis. In Yase Y, Tsubaki T, editors: Amyotrophic
lateral sclerosis: recent advances in research and treatment, Amsterdam, 1988, Elsevier Science.
tions for interventions on the basis of degree of impairment, For most patients in the early stages of ALS, pleasurable,
functional limitations, and level of disability. In the following natural activities such as swimming, bowling (can gradually
section, staging patterns are used as the framework for ther- decrease weight of ball if shoulder strength is a problem),
apy interventions. Staging information is particularly helpful walking, bicycling (three-wheeler may be needed or in-home
to therapists who do not have the opportunity to work with stationary bicycle, either of which must be evaluated for
large numbers of patients with ALS. easy mounting and dismounting), or tai chi should be rec-
Most patients need specific guidance about what type of ommended. Some patients prefer to exercise alone, whereas
activities and exercises they should do.61 Although many others will gain confidence and companionship by joining a
physicians may suggest to patients that they increase their group activity. Listening to the patient’s desires related to
activity level, their suggestions are seldom specific. Exam- group activities is important. The dropout rate is high among
ples of exercise advice that patients have recalled are “Try those who have been pressured to participate. Some spouses
to move around as much as possible,” “Walk some more,” or family members are supportive of the patient’s activity
and “Be active, but don’t overdo it.” Because changing their needs and will join the patient in his or her regimen. If pos-
typical exercise pattern is difficult for most patients, even sible, the spouse and family members should be engaged in
when they know doing so is important, referral for a physi- the treatment planning process.110
cal therapy consultation can be helpful.108 The therapist must observe the patient completing her or
Phase I (Independent): Stages 1 to 3. A program to his entire recommended activity program. The patient’s re-
increase activity must be specifically designed, with input sponse to the program must be monitored because fatigue
from the patient about willingness to participate and knowl- from exercise sessions can interfere with the ability to carry
edge of the patient’s environmental situations and social out other normal daily activities. If the patient becomes too
support systems. In the early stages of the disease, patients exhausted at the end of a session, he or she may learn to fear
should be encouraged to continue as many prediagnosis exercise and may become depressed about the decreased
activities as tolerated. For example, a golfer should continue activity status. This depression may lead to decreased activ-
to golf for as long as possible. Walking the course should ity and further deconditioning (see Chapter 6).
be encouraged if it is not too fatiguing. When walking or Phase II (Partially Independent): Stages 4 and 5.
balance becomes difficult on uneven terrain, the golfer can During phase II, the goal of physical and occupational
use a golf cart, decrease the number of holes played, move therapy intervention should be to help the patient adapt to
to a par 3 course, or hit balls at a driving range. If upper- limitations imposed by weakness and spasticity, an increas-
extremity weakness is a major problem that interferes with ingly compromised cardiorespiratory status, and possible
swinging the club for distance shots, the player can continue pain from stress related to weakness or muscle imbalance.
playing the greens or on putting courses. Some golfers may This transition stage is often frightening for patients because
need adaptations to club handles with nonskid material such the decrease in function and independence becomes clear;
as Dycem (Dycem Non-Slip products, www.dycem.com) or therapists should accentuate what the person can do and how
Scoot-Gard (Vantage Industries Product) to prevent the club accommodations can be made to help maintain indepen-
from rotating on impact. dence. After a full physical assessment of the patient’s motor
Patients with newly diagnosed ALS who had a sedentary status similar to the initial evaluation, the patient, family
lifestyle before diagnosis should be encouraged to increase members, and therapists (including PT, OT, and speech
their activity level. This may include activities that require therapist if a team approach is possible) should discuss treat-
muscular effort within or around the home, such as sharing ment options and adaptive devices that can help the patient
household and gardening tasks or beginning a walking remain as independent as possible.
program around the neighborhood. After diagnosis, some During late phase I and through phase II, many patients
patients begin searching for in-home exercise devices such show significant weakness of both upper- and lower-extremity
as bicycles and rowing machines. As with healthy persons musculature, but each patient has his or her own pattern and
who start an exercise program after the purchase of exercise rate of progression of weakness and onset of spasticity, bulbar,
equipment, patients with ALS are not likely to use the equip- and respiratory symptoms. A typical patient at this time may
ment consistently if they did not before a diagnosis. The have marked weakness of the intrinsic muscles, shoulder
search for a “perfect” exercise machine may reflect the muscle weakness (in some cases “hanging arm” syndrome)
patient’s desperation to do something tangible. Without tak- with shoulder pain, and generalized lower-extremity weakness
ing away the patient’s motivation to exercise, therapists can (in some cases more severe distally). Patients may be able to
encourage participation in exercise programs that do not walk within the home environment, but many patients have
require expensive equipment, such as walking or working precarious balance and fall easily because of muscle weak-
out to specific exercise routines. A clever therapist can make ness. At this stage, most patients report fatigue with minimal
a video for each patient that includes stretching and gentle work and have to rest frequently when carrying out ADLs.
exercise programs that elicit muscle contractions from all ROM can deteriorate quickly in this phase of the disease,
functional muscle groups (by using inexpensive elastic requiring daily stretching to end range for the calf, quadriceps,
bands or small weights) with follow-up breathing, “warm hip adductors, trunk lateral flexors, and long finger flexors.29
down,” and relaxation exercises. Patients could follow a Moderate exercise can have a modest effect in reducing
program of six maximal isometric contractions held for spasticity.93
6 seconds and isotonic elastic band exercises at submaximal Patients at this point, even if ambulatory, should con-
levels to maintain and improve muscle strength.109 Patients sider using a wheelchair outside the home to conserve
should exercise for short periods several times a day rather energy.72 Factors to consider in choosing a wheelchair
than attempting to exercise all muscle groups in one session. include extent of insurance coverage or financial assistance
programs for purchase of wheelchair (some policies or pro- energy conservation techniques should be aware of the
grams may provide only one type of wheelchair or only one exhaustion that can be associated with communication. A
wheelchair, either motorized or manual); transportability of number of strategies recommended by the American
motorized chair from home to community and work (few Speech-Language-Hearing Association112 can be used by
motorized wheelchair brands fold for stowing in car trunk, the person with ALS to deal with the effects of dysarthria,
and few families can afford to purchase a van that will including the following:
allow the patient to drive or be driven while in a motor n Reduce background noise in the room.
chair); reclining potential of chair back and headrest (pref- n Face the person while talking.
erably electric) to allow the patient to shift weight and rest n Use short, simple phrases rather than long, compli-
while in the chair during later stages of the disease; remov- cated ones.
able arm rests for ease of transfer; potential for headrest n Take the time to say what needs to be said; do not
attachment or extension; potential mounting area for por- allow people to rush conversation.
table respirator equipment if needed; and ease with which n Make extra use of body language, such as gestures and
caregiver can help patient with chair mobility transfers.72 facial expressions, and use writing to supplement
Chairs should have lumbar support and appropriate cush- speech, if possible.
ioning to prevent pressure ulcers.105 n Do not worry about saying things correctly; if the basic
At this stage, patients with more advanced bulbar symp- message being conveyed is understood, then that is
toms begin to experience dysarthria and may need guid- enough.
ance in dealing with communication issues. Murphy111 Also in this stage, some patients and families may need
indicated four major reasons for communication: to identify support to identify adapted feeding systems (special utensils,
needs or request help, share information, respond politely adapted plates, adjustable tables) and hygiene equipment if
in social situations, and maintain social closeness. The transfers within the family bathroom are problematic.113
primary focus of communication for the study participants Because Mr. Turner in Case Study 17-1 was cared for in
was to maintain social closeness. Although few patients a neuromuscular disease clinic, he benefited from input
had any instruction in ways to deal with communication from multiple specialists working as a team to help him
problems, most patients and caregivers created ways to maintain his independence. Unfortunately, many patients do
make themselves understood, such as giving cues about the not have the benefit of such a coordinated treatment environ-
topic and context, creating a “shorthand” language, and ment. Therefore, when necessary, the therapist must be in a
checking with the dysarthric speaker to ensure that the position to provide input on adaptive and safety devices and
listener understands the patient correctly. A number of bulbar issues if other specialist input is not available. Thera-
patients in the study who had significant dysarthria com- pists working in smaller communities and rural areas most
mented that attempting to communicate socially was ex- likely need to be chameleon-like to play many therapeutic
tremely tiring. Therapists who are guiding patients with roles when working with the patient with ALS.
CASE STUDY 17-1 n MR. TURNER

Mr. Turner is a 45-year-old man diagnosed 2 years ago with ALS. On assessment, Mr. Turner showed marked wasting of hand
He lives at home with his wife, who works full time, and two intrinsics. He was unable to abduct or flex either shoulder past
teenaged children. Mr. Turner is a computer programmer for an 90 degrees. His right shoulder showed considerable atrophy,
engineering firm in the area. Since his diagnosis, Mr. Turner has especially of the deltoid and supraspinatus muscles. All other
been able to continue his full-time work schedule, although he upper-extremity movements were weakened but in the G2 (42)
states that he is no longer able to touch type and can type with range. His neck posture was forward: neck extension is F1 (31),
the index fingers only. He has noticed that his shoulders and neck neck flexion is G2 (42). Scapular winging was noted bilater-
hurt (4 out of 10 on a numerical pain rating scale) after an hour ally. No spasticity or loss of passive ROM was evident in the
at the computer. In the last 2 weeks he has found it fatiguing to upper extremities. Lower-extremity musculature showed general-
walk to the cafeteria for lunch (approximately 100 meters), and ized weakness at the F (3) to F1 (31) range, with left muscula-
he fears that he will be knocked down when walking in crowds. ture weaker than right, marked wasting of the foot intrinsics, and
He dropped his tray last week, which was embarrassing, so he a cavus foot position bilaterally. Spasticity of the hip adductors
decided to eat in his office even though he misses the socializa- and hamstrings was noted (Modified Ashworth Scale grade 2),
tion and opportunity to discuss work issues with his colleagues. but no passive ROM loss was detected in the lower extremities.
Mr. Turner has been able to continue most of his nonwork Most obvious during gait was inadequate dorsiflexion for heel
activities, although he is no longer able to operate his sailboat strike and no propulsion during heel-off. He showed a bilateral
independently and is having trouble maintaining his balance corrected gluteus medius pattern on weight bearing. He needed
when golfing. Also when golfing, he now uses a cart and plays to pause to lock each knee during weight bearing and at times
only nine holes. He states that his wife and children are support- he pushed his knee into extension with his hand. He had great
ive and that they have made some changes in the home environ- difficulty ascending and descending the four steps to enter his
ment to accommodate his increasing weakness. He also revealed, home. There were no stairs to negotiate at work.
however, that his children seem frustrated with him because he is Until this appointment, Mr. Turner had not been willing to
so much slower than he was before the illness. discuss the use of adaptive equipment or a wheelchair. During
Continued
CASE STUDY 17-1 n MR. TURNER—cont’d

prior clinic visits his decisions were supported and he was told He felt immediate relief with the adaptations. He was also
that when he was ready, therapists would work with him and fitted for a soft neck collar to wear when he felt he needed
his family to help with equipment decisions. more neck support. (As his condition worsened, he learned
Mr. Turner also showed some early bulbar signs. He noted to rest his head on the headrest of his chair and recline
that he sometimes had to catch drool when working intensely, slightly for a few minutes every 15 minutes.)
and that his pillow was moist in the morning. Food sometimes 3. Maintain maximal muscle strength and ROM. Mr. Turner
got stuck in his cheek area and he could not move it out with was taught as many self-ranging maneuvers as possible,
his tongue. Swallowing was still adequate for eating all foods; which he was encouraged to do in small segments
however, he had had a few coughing episodes when drinking frequently throughout the day. For example, his series of
coffee and wine. He showed increased use of accessory muscu- motions included neck rotations, side bends, and flexion
lature when breathing but had no reports of respiratory distress. and extension within strength limits; upper-extremity
His cough was adequate to clear secretions. motions with the exception of shoulder flexion and abduc-
With input from the therapist, Mr. Turner and his wife tion past 90 degrees; hip flexion, abduction, and rotations;
identified the following general goals: full knee extension; and all ankle motions. When using
1. Increase mobility while conserving energy the walker, Mr. Turner was encouraged to extend each hip
2. Control fatigue and pain of upper extremities and neck fully and to stretch his heel cords. Mrs. Turner and their
during computer work adult children were taught to administer full ROM exer-
3. Maintain maximal muscle strength and ROM (patient cises, including trunk rotations, with special attention to
reported that he felt stiff) ranging of the shoulder to prevent impingement. Simple
4. Identify safety issues within the home and work environ- massage techniques were also taught to all family mem-
ment and adjust household and work environment to bers who felt comfortable with the task.
prepare for the time when Mr. Turner could not ascend Mr. Turner had been active before the onset of ALS
and descend stairs safely and he liked to exercise. He rented a portable pedaling unit
A treatment plan was discussed to achieve the following: to attach to a chair at home. He pedaled two to four times
1. Increase mobility. Because of his increased walking a day, with no additional resistance, to the point at which
difficulties, Mr. Turner decided to use a front-wheeled he felt fatigue (usually 3 to 5 minutes at this stage). He
walker with a seat attachment at home. Because of his carefully monitored his soreness and fatigue level after
hand grip weakness, he felt most stable using attached exercise and increased and decreased his pedaling depend-
forearm troughs. For his worksite, he selected a motor- ing on how he felt immediately and several days after
ized wheelchair so that he could maintain his indepen- exercise. Mr. Turner felt invigorated by this exercise, which
dence at work. Although he found that he could push an he usually did while watching television. He was also
ultralight manual chair, his upper-extremity strength was taught a series of simple elastic band exercises, with
clearly decreasing. Mr. Turner decided that he preferred tensile strength adjusted according to his ability to contract
the motorized chair to an electric scooter because of the his muscles without fatigue. Mr. Turner was also shown
financial cost of switching devices when the scooter no a series of isometric exercises for all muscle groups to do
longer provided adequate postural support. throughout the work day. Because he had some foot and
Because Mr. Turner’s insurance and Medicare would ankle edema, he was encouraged to wear lightweight
not fund an additional manual chair and because the pressure stockings while sitting. Mr. Turner also had
family had no way to transport the electric wheelchair, access to a swimming pool, and he was encouraged to
the ALS Society loaned the family a manual wheelchair carry out walking and upper-extremity exercises as long
for home use. Although not ideal, it was functional. as another adult was with him in the water at all times.
Mr. Turner’s son made some inexpensive adjustments 4. Assess environment of home and work. Occupational
to adapt the chair for a headrest, and his daughter and therapy input was requested to help with ADL aids such
grandchildren repainted the chair to his specifications. as reachers, utensil adaptors to facilitate grip, rubber pen
Because Mr. Turner wanted to keep as active as possi- grippers, key adaptors to permit turning, and thumb
ble and use his walker within the home, he was fitted with abduction splints to assist in pincer grasp. Mr. Turner’s
bilateral ankle-foot orthoses (AFOs) with a flexible ankle OT made several visits to his worksite and home to iden-
joint and pretibial shell to facilitate knee extension. Straps tify adaptations of the environment for safety and inde-
were simple overlap style because Mr. Turner had poor pendence. His wheelchair was eventually adapted with
thumb and grasp control. universal joint arm troughs to decrease his effort during
2. Decrease fatigue and pain of upper extremities. Mr. Turner self-feeding and basic upper-body hygiene. Ramps were
was taught some simple ROM exercises of the neck and recommended for home entry, and nonpermanent safety
arms to perform every half hour while working at the com- rails were placed in the bathroom. Mr. Turner was able
puter. In a simulated work environment the therapist noted to assist with transfer to a shower chair, and the shower
that Mr. Turner had a forward head position when working head was replaced with a handheld unit.
at a computer similar to his workstation. The height of the A speech pathology consultation was also requested.
computer was adjusted to decrease his neck strain, and the Using information from the PT’s manual muscle testing,
desk height was adjusted to allow his wheelchair to fit under the speech pathologist carried out a thorough bulbar
the desk so that his arms could rest fully on the surface. evaluation and provided information about swallowing
CASE STUDY 17-1 n MR. TURNER—cont’d

techniques. The speech therapist focused on ways to As Mr. Turner became totally dependent, he needed 24-hour
decrease drooling and ways to cope with food pocketing care. Professional nurses were provided through his insurance
(tongue mobility was impaired) by using techniques contract 14 hours a day from 6:30 am to 8:30 pm. Family mem-
such as hand pressure on the cheek to push food back bers provided care until midnight. Initially Mr. Turner was able
to the center of the mouth. The therapist also instructed to activate a bell at night to call for help. His wife and children
Mr. Turner and his wife how to prepare foods with textures followed a schedule to turn him every 3 hours throughout the
that were easily swallowed and manipulated. Mr. Turner night. When Mr. Turner became respirator dependent and was
had lost 5 pounds during the last 6 months, so he was no longer able to call for help, it became clear that the nighttime
also referred to the dietician for information about how responsibilities were taking a heavy toll on his wife, who
to maintain nutritious calorie intake. worked full time, and the children, who were in high school and
PROGRESSION OF THE DISEASE college. Fortunately the family was able to pay for a nurse as-
Within 3 to 4 months after initial examination, Mr. Turner was sistant to remain at Mr. Turner’s bedside throughout the night,
no longer able to continue working despite workplace adapta- although the family members all felt that they had no privacy.
tions. At home, he became more dependent. Mr. Turner had great Although the family was committed to having Mr. Turner re-
difficulty adjusting to his physical dependence. Because of main at home until his death, all agreed that they needed respite.
his slow onset of dysphagia and his augmented communication Thus several week-long hospitalizations were made to give the
system, he was able to continue control over his expressive, family a break in the constant care needs.
cognitive, and emotional life for another few months. Initially Although Mr. Turner had elected HMV, he also had signed
Mr. Turner angrily resisted his wife’s attempts to help him with a durable power of attorney for health care, indicating that he
eating and dressing tasks. This began to alienate her and the did not want treatment for infections and that palliative care for
children until a family meeting was held with their medical comfort should direct his treatment. He had a strong lust for
social worker and PTs and OTs. All family members had the life, but he had come to accept his impending death. He did not
opportunity to express their frustrations. A major irritation to have strong religious views, but he had talked with all his care-
the children was what they perceived to be their constant waiting givers and therapists about his concerns related to death. He
for their father to complete a task. Mrs. Turner was most irritated freely expressed his fear of “nonbeing.” Because his caregivers
when Mr. Turner yelled at her when she attempted to help even and therapists were willing to talk about his and their own feel-
though he frequently expressed anger about his clumsiness. ings, Mr. Turner came to believe that he would live on in the
Mr. Turner sadly admitted that he was having increasing diffi- minds, hearts, and behaviors of those he had known. This idea
culty with his ADLs and was sometimes too tired after dressing seemed to give him great comfort. He particularly liked to talk
to participate in family activities. At the end of the meeting, the to others about special times they had had together and how
family had worked out a compromise plan. Mr. Turner would their interactions had affected each other. To help Mr. Turner
continue to do as much as possible for himself. He would specif- process his death, his family, friends, and medical team put
ically ask for help from Mrs. Turner when he wanted it so she together an album of pictures and statements about their time
did not get caught in his anger about needing help. He preferred together. Mr. Turner frequently liked to have his wife read
that the children not have to take any role in his care at this point through the book with him. His family continued to carry out
but realized that he might need their help later. Visiting nurse his ROM exercises and massage because Mr. Turner had indi-
support was requested twice a week to help with bathing, and cated that the treatments provided him physical comfort and
the OT was requested to make another home visit to help with the spiritual closeness he needed with his family. His primary
toileting needs. Mr. Turner felt comfortable with his wife and treatment during the last few days consisted of morphine to
children carrying out ROM exercises. A therapy home visit was decrease his respiratory discomfort. After 5 to 6 months of
arranged to review the exercise and positioning program as well being totally dependent for all care and respiratory function,
as respiratory exercises and postural drainage techniques. Mr. Turner died at home in his sleep after a respiratory illness.
Phase III (Dependent): Stage 6. PTs and OTs are to be in the midst of family activities even when dependent
usually less involved in the care of the patient in phase III, on HMV, other patients feel uncomfortable with their
and nursing personnel become more active. During this dependency and appearance and are reasonably content to
phase, therapists make home visits to support caregivers stay in their room with television and visits from family
and respond to questions about pain control, bed mobility, members. This highly personal decision by patients must
positioning to prevent pressure ulcers, ROM, and equip- be respected. The therapist should review ROM procedures
ment adaptations.29,72,105 Therapists should be sure to teach with family and professional caregivers and provide splint-
all caregivers some basic body mechanics to use during lifting or positioning devices if spasticity or paralysis leads
ing and patient care activities. If possible, caregivers should to caregiving difficulties (e.g., excessive adductor tone and
be taught how to safely move the person with ALS from the contractures interfering with hygiene and bowel care) or
bed to a reclining wheelchair or other reclining chair during tissue damage and pain. If nursing care providers do not
specific times of the day so that the person can continue to give advice on pressure relief beds or mattresses of air or
be part of the family activities. However, the ease of care- foam,105 therapists should be prepared to do so. Unfortu-
givers in transferring and caring for the person in the wheel- nately, many insurance providers and Medicare may not
chair must also be considered. Although some patients want fund special mattresses, and they can be costly. Therapists
may also need to review postural drainage techniques with and their caregivers must have some system to communicate
caregivers. emergency needs; for example, looking to the right means
Of greatest importance in phase III, and sometimes in “help” and looking to the left means “pain.” Therapists
earlier stages, is the patient’s ability to communicate. In should help patients develop alternative modes of communi-
the earliest manifestation of dysarthria, therapists train cation before intelligible speech becomes impossible. (See
patients to slow the speech rate and cadence, exaggerate lip also Cobble117 for information on language impairments.)
and tongue movements, and manage phrasing through In addition to communication systems, environmental
breath control.83 Although spouses and caregivers can control systems can be programmed to turn on and off tele-
often interpret their partner’s or patient’s severely dysar- vision, lights, and other electronic units with the same type
thric speech (see earlier discussion of phase II), most pa- of switching units used for communication (e.g., eye blink,
tients who use NIV or invasive ventilation for a prolonged infrared beam, head movement pressure). Unfortunately,
period need to find nonverbal methods to communicate. If these devices are often expensive and may not be available
severe bulbar impairments precede extremity paralysis, paper to all patients. (See Cook and Hussey114 for a comprehen-
and pencil, alphabet and word boards, and adapted com- sive review of environmental control systems.) Financial
puter keyboards can be used with minimal upper-extremity support is often not extended for high-tech equipment
or finger strength for pointing. The American Speech- by third-party payers because of the patient’s limited life
Language-Hearing Association provides suggestions for expectancy. The ability to communicate and call for help,
developing communication boards with the specific lan- however, is of paramount importance with completely
guage most appropriate for the patient’s situation.112 For dependent patients.
example, the board may be designed with commonly By phase III most patients have significant problems eat-
needed sentences, words used in the person’s daily life, ing and maintaining nutrition, although these problems may
and the alphabet. As the person’s ability to finger point manifest in earlier stages. Patients often report choking or
decreases, the language board can be redesigned. When no coughing after swallowing liquids or problems moving food
extremity movement is possible, subtle neck movements or around in the mouth or to the back of the throat for swallow-
pressures, eye gaze, eye blink, upper facial movements, ing. These problems are best handled medically and can be
and electroencephalographic activity can be harnessed to assessed with videofluoroscopy or videoendoscopy. The
operate communication devices.114,115 Learning to use elec- aggressiveness of treatment intervention depends on the
troencephalographic interfaces, however, takes months of patient’s preference and whether she or he still wants to
intense training and may not provide a reasonable system attempt any oral feeding (e.g., syringe feeding, oral gastric
for communication for most patients with ALS.116 tubes) or wishes to have a PEG or another alternative to oral
Some patients with hypernasality benefit from using an feedings implemented. Therapists, however, can help patients
orthodontic palatal appliance. Patients with a tracheostomy and caregivers develop strategies that improve eating and
may benefit from use of a Passy-Muir (Irvine, Calif) speak- nutrition, such as adjusting eating position, changing head
ing valve tracheostomy tube. These devices require recom- and neck alignments, adding thickeners to liquids, and adjust-
mendation by communication specialists. As speech quality ing portion sizes and texture of foods.7
deteriorates and sound projection wanes, the spouse or care-
giver can use an electronic speech amplifier to magnify the Psychosocial Issues
patient’s speech. Speech pathologists and therapists have Giving the bad news of a terminal diagnosis is difficult for
information on commercially available amplifying devices even the most experienced clinician. In dealing with the
that are often used by persons with hearing problems but can diagnosis of ALS, most physicians now believe that the
be used by hearing people to amplify the speech of a person diagnosis, prognosis, and possible patterns of progression
with severe weakness of phonation. should be shared with the patient and family or partners and
When selecting a communication device, therapists must caregiving friends. Only by knowing the truth can patients
work closely with the patient and family members to ensure and families deal openly with one another and make plans
that the system is compatible with patient skills and com- for the future.118 McCluskey and colleagues119 suggest that
munication needs and preferences. Expensive systems com- those giving the medical or therapeutic diagnosis should
monly lie unused because of simple factors such as lack of attend to good practice parameters when giving bad news,
proximity to the patient, interference of the unit with per- such as creating the appropriate setting, identifying patient
sonal care, increased caregiver workload to manage the unit, and caregiver needs, asking what patients and caregivers
and slowness of communication processing. The best sys- want to know, providing knowledge, exploring feelings of
tems are tailored to the precise needs of the patient; how- the patients and caregivers, and formulating a strategy for
ever, many patients do not have the financial or insurance dealing with the situation. Patients and family members
support to purchase the device, and many patients in the end seldom remember what they are told when first given a
stages of ALS do not have the time to wait for systems de- terminal diagnosis. They do, however, remember how the
signed for their specific needs. Therefore commercially information was given. Therefore information should be
manufactured systems may be most appropriate. (See Cook given honestly but with a sense of hope. All information need
and Hussey114 for a comprehensive list of communication not be given at the time of diagnosis. Rather, the patient and
devices and control interfaces.) family can be exposed to more in-depth information over
Some patients and caregivers learn to communicate a number of sessions when they have the opportunity to
effectively with simple eye gaze, eye blinking, and clicking ask questions that occur during the assimilation process.
techniques with Morse code or self-developed codes. At Therapists, especially those working in isolation from a
minimum, patients with no ability to communicate or move comprehensive clinic, should also follow these guidelines by
providing information, helping the patient and family iden- life.122 Keeping a pain log of intensity, type, location, and
tify goals, and establishing a plan for intervention. Patients time of pain may provide the physician with information
should know that the goals will have to be adjusted and necessary to best prescribe dosages. Many patients with ALS
plans reset as the disease process continues. If patients and do experience significant pain from musculoskeletal sources,
families know that they can contact the therapist for support persistent spasms, or spasticity and pressure sores. Most of
and advice, many of the negative aspects of the illness can these problems can be handled with appropriate pain medica-
be confronted in a positive manner. Preferably, an appoint- tions, muscle relaxants, careful positioning, frequent ROM
ment for a follow-up visit will be set so patients and family exercises, and tissue massage. Undertreated and uncontrolled
members feel that contact with the care provider is expected. pain is associated with a patient’s seeking information on
Information about transitions related to nutrition, com- assisted suicide.123 Some patients who expressed interest in
munication, and respiratory functions should be delivered to assisted suicide options did not follow up because of reli-
patients and families in time to make thoughtful decisions gious beliefs and concerns about possible loss of life insur-
rather than just before a time of crisis, such as after a chok- ance coverage for surviving family members.124
ing episode or during a respiratory arrest. Care should also A major concern of patients with ALS is the dependence
be taken to respect the cultural and spiritual views of the necessary for ADLs associated with late phase II and phase III
patient and family.58 Preferably, patients and family mem- of the disease. Because the process is gradual, most patients
bers will prepare an advance medical directive that should have the opportunity to make adjustments. The dependency
be reviewed with the physician at least every 6 months.120 issues and resulting privacy issues are more uncomfortable
Therapists treating patients who do not have access to a for some patients than for others, especially for the person
multidisciplinary ALS clinic should remember that they are who has always valued self-control and independence. Some
often the person who works most closely with the patient, patients are concerned about their increasing dependence
and they should plan on spending enough time with the fam- because of the consequences of increasing burden of care
ily to respond to concerns and help with problem solving. on spouses or other caregivers.125 That concern for others
Patients will progress through the diagnostic process with sometimes causes patients to choose hospital, nursing home,
different responses and at different rates on a continuum or in-patient hospice care over home care during the terminal
from taking a cognitive approach by asking many questions stage of the disease. Not all patients with terminal illness react
and reviewing the most current research to the extreme of the same way during the dying process. Throughout the pro-
marked denial and disinterest in participating in any medical cess, patients and family members may cycle back and forth
or therapeutic recommendations. through a range of different emotional and coping reactions:
Purtilo and Haddad121 identified four major fears of the depression, anger, hostility, bargaining, and acceptance and
patient who has a terminal condition: fear of isolation, fear adaptation (order is not implied).121 How the patient coped
of pain, fear of dependence, and fear of death itself. Patients with life’s difficulties before the illness and her or his prior
with progressive diseases often see their social contacts relationship patterns often direct how the patient will deal with
decrease. Mr. Turner in Case Study 17-1 was concerned the terminal illness. In one study, patients adjusted most suc-
when he was no longer able to join his colleagues in the cessfully to the changes in their functional status if they did not
company cafeteria. After he received his motorized wheel- look back to the past and compare their losses to their future.126
chair he was able to continue his social contacts until his Health care providers and family members often have
bulbar symptoms progressed to a point that he chose not to great difficulty coping with a patient who is depressed; they
eat in public. When Mr. Turner lost the ability to speak and may make repeated efforts to “talk the person out of” the
had to use his computerized speech system, he noticed that depression. Medical professions must be able to distinguish
fewer colleagues stopped by his office to talk because of the between depression that can be destructive and the mourn-
slowness of the communication process. Although he under- ing or grieving that is a necessary and vital response to deal-
stood the problem, Mr. Turner mourned the loss of friendship ing with loss. In both states the person may feel a level of
and his loss of standing as a competent computer expert. withdrawal, sadness, apathy, loss of interest in activities, and
Because of his need for social contact, Mr. Turner continued cognitive distortions. In a depressive state, however, the
to work until he could no longer tolerate the sitting position. patient experiences an accompanying loss of self-esteem.
His fear of isolation increased when he became homebound. A person in mourning rarely experiences that loss of self-
Although colleagues came for visits regularly at first, as esteem essential to a diagnosis of depression. The grieving
Mr. Turner progressed to a near locked-in state only a few person’s feelings are congruent with the degree of loss
close friends came by for brief visits. Mr. Turner’s greatest experienced.127 A person who grieves for what is lost but
fear was being separated from his family and abandoned to who has adapted to the prognosis may make plans for the
hospital care with inconsistent staffing patterns. Fortunately, impending death. Such behaviors are positive coping strate-
in his community, Mrs. Turner was able to set up visitations gies. However, depressive symptoms related to hopelessness,
from several church members, clerics, and hospice volunteers. uncontrolled suffering, and perceived burden on caregivers
Fear of uncontrolled pain is common among people with are more related to a choice for treatment discontinuance of
terminal diseases. Patients need assurance that their pain will feeding or ventilatory support.124
be controlled. Fortunately, today pain medications can be The issue of depression is complicated by the pseudobulbar
administered in many forms, dosages, and frequencies that effect of emotional lability (inappropriate laughing and cry-
can be tailored to the patient’s specific needs. In a study of ing), which is manifested by approximately 50% of patients
the final month of life with ALS, caregivers reported that a with ALS. This emotional lability is not under complete con-
major emphasis of care was to eliminate as much pain and trol of the patient and is often misunderstood by family mem-
discomfort as possible, even if it shortened the patient’s bers and caregivers. Although current treatment is antidepres-
sant medications, underlying clinical depression may or may social support systems, they expressed frustration that few
not be present that would respond to higher doses of antide- people offered instrumental or direct service support, such as
pressant medication and counseling.120 respite care or help with medical appointments, housekeep-
Yet, pressuring a patient who appears depressed to see a ing, or shopping. Despite the stresses of caregiving, the
mental health clinician can lead to loss of trust if the patient is caregivers felt positive about their roles in helping the depen-
not comfortable talking about feelings or confiding in a coun- dent adult by finding meaning in their acts of caregiving.129
selor. Therefore, OTs and PTs and other persons involved in Fortunately, most families manage to cope with the pro-
the direct care of a dying patient may find that their patients cess—the major contributing factor being the coping ability
feel safer talking with nonprofessional counselors or psycho- of families before the illness. To be really effective, the
therapists about the burden of their care on family members or therapist working with the patient with ALS must be pre-
their own impending death. Rehabilitation personnel should, pared to help families and caregivers find appropriate ways
therefore, be aware of local options for in-home support ser- of coping with the emotional, social, and physical stress of
vices, palliative care, and end-of-life options and services and caregiving. For example, therapists should present, without
be prepared to listen to the patient’s concerns if the patient pressing, adaptive equipment options to patients when they
expresses the need for emotional support. first start to show impairment in functional ability. If shown
how the equipment will help them maintain independence,
Caregiver Issues most patients are receptive to its use. Even when presented in
Often in the concern for the patient’s needs, health care pro- a positive way, however, a wheelchair or adaptive devices
fessionals pay little attention to the effect a person’s degen- may be resisted long after the adaptations would facilitate
erative illness has on other members of the family. ALS sig- mobility and ADLs. Therapists must be attentive to patients’
nificantly affects the person’s extended family because the feelings and fears at this time because use of a wheelchair
patient gradually becomes increasingly dependent on family heralds to many patients the beginning of the end.
members, partners, or caregiving friends for physical care, Other factors that affect the family of a patient with ALS
social arrangements, cognitive stimulation, and emotional include medical insurance and differing levels of long-term
support. For some families, the spouse may have to take on care coverage. Some families are fortunate to have excellent
additional work, return to work, or, in the case of some older coverage that provides extensive home nursing support,
women, join the workforce for the first time to deal with the whereas other families are unable to cope with the financial
financial stresses that occur when chronic illness invades the stresses and must accept public assistance during the final
family unit. Family members must absorb the former family stages of the disease. As opposed to Germany and Japan,
duties of the dependent person. For example, a spouse or which provide long-term nursing care insurance, in the
child may have to handle all the cooking, cleaning, or other United States financial stress on patients with ALS can reach
household chores or work to help support the family. Once more than $150,000 per year for ventilation support at
the patient becomes dependent, the caregiver may need to home.63 Financial burden significantly impacts patient and
reduce or discontinue employment to take care of the patient. caregiver decisions. (See Case Study 17-1 and end-of-life
All family members may have to become involved in the issues resources at www.nlm.nih.gov/medlineplus/endof
physical care of the increasingly dependent person with ALS. lifeissues.html#cat1.)
Children of patients with ALS also have to deal with
major changes in their lifestyle. Although they may love their
GUILLAIN-BARRÉ Syndrome
parent who is sick, at some level most are frustrated with
factors such as the need to provide physical care to parents. Pathology and Medical Diagnosis
This is a difficult problem for children who have not had a In the past 15 years a broad spectrum of inflammatory demy-
positive relationship with that parent. Children living in the elinating polyradiculoneuropathies has been identified. GBS,
home of a parent who is dying of ALS also express frustra- or acute inflammatory demyelinating immune-mediated
tion about the lack of privacy in their home when nursing polyneuropathy, is the most common form of the disease.
personnel and attendants are present, interruptions in family GBS affects nerve roots and peripheral nerves, leading to
and personal life plans, embarrassment because of the par- motor neuropathy and flaccid paralysis with possible sensory
ent’s appearance and dependency, lack of attention from the and ANS effects.130 Purely motor forms and mixed motor and
caregiving and working parent, and fear of financial crises sensory forms of GBS have been identified.131 Unlike ALS,
(e.g., possible loss of home, no financial support for college). GBS usually has a good prognosis, with most patients return-
The entire family is affected by the sick person’s increasing to their prior functional status by 1 year after onset.
ing dependency and impending death. In a small study of The incidence of GBS is approximately one to four cases
11 family caregivers, many caregivers felt frustrated and reper 100,000 persons. A variant form is acute motor axonal
sentful because their lives were consumed with the caregiv- neuropathy, which, like GBS, has a good prognosis. Less
ing responsibilities. Most caregivers had adjusted to some common forms are acute motor and sensory axonal neu-
degree after 2 to 4 years. Caregivers who adjusted most suc- ropathy, which has a less positive prognosis (and which
cessfully learned to take time for themselves without guilt some consider to be a distinct type of peripheral neuropa-
and to tap their social support systems for help.126,128 Simi- thy); Miller-Fisher syndrome, with primarily cranial nerve
larly, 40 caregivers of young adults with severe disabilities symptoms, ataxia, and areflexia132; and chronic inflamma-
reported being overwhelmed by the physical requirements of tory demyelinating polyradiculoneuropathy (CIDP), which
daily care and felt a severe loss of spontaneity in their causes progressive or relapsing and remitting numbness and
lives.129 They also reported a sense of isolation from every- weakness.133 Epidemiological studies show that males are
day social interactions. Although they highly valued their affected by GBS twice as often as are females.134
Approximately 27% of patients with GBS have no identi-

fied preceding illness; however, more than two thirds had
symptoms of an infectious disease 2 weeks before the onset
of GBS symptoms. Although no consistent predisposing
factors are known, evidence exists to support connections
with Campylobacter jejuni, Mycoplasma pneumoniae, cyto-
megalovirus, and Epstein-Barr virus. In GBS the spinal
roots and peripheral nerves are infiltrated with macrophages
and T lymphocytes. Macrophages then attack and strip the
myelin sheaths. In milder cases of GBS the axons are left
intact and the nerves are remyelinated, typically in a matter
of weeks.135 However, in some cases, the axons also degen-
erate, with recovery dependent on axonal regeneration from
intact elements, which takes months and may be incom-
plete.135 In acute axonal motor neuropathy, macrophages
invade the axon directly, leaving the myelin intact.136 Some
evidence exists in a substantial number of patients with GBS
that axonal loss is related to long-lasting or permanent
muscle weakness.137,138
Because of damage to the myelin sheath, saltatory
propagation of the action potential is disturbed, resulting in
slowed conduction velocity, dyssynchrony of conduction,
disturbed conduction of higher frequency impulses, or
complete conduction block.139 Partial conduction block is
most often seen in the early stages of GBS, and the con-
duction block increases as the patient reaches a plateau.
The most common conduction block findings are in the
peroneal nerve, followed by the tibial nerve. Proximal con-
duction block is evident more often than distal conduction
block. In axonal neuropathy, conduction block is more
severe, and the number of functional motor units is decreased
(Figure 17-4).140 The diagnostic criteria for GBS are detailed
in Box 17-3.
Clinical Presentation
GBS in both children and adults is characterized by a rap-
idly evolving, relatively symmetrical ascending weakness or
flaccid paralysis. Motor impairment may vary from mild
weakness of distal lower-extremity musculature to total
paralysis of the peripheral, axial, facial, and extraocular mus-
culature. Severe fatigue is present in 38% to 86% of patients
with GBS, depending on the cutoff point used to define
severity and the age of the sample, with a positive correla-
tion between severe fatigue and age.141 Tendon reflexes are
usually diminished or absent. Twenty percent to 38% of
patients may require assisted ventilation because of paraly-
sis or weakness of the intercostal and diaphragm muscula-
ture.142,143 Impaired respiratory muscle strength may lead to
an inability to cough or handle secretions and to decreased Figure 17-4 n Peripheral nerve showing axonal degeneration
vital capacity, tidal volume, and oxygen saturation. Second- and demyelination.
ary complications such as infections or organ system failure
lead to death in approximately 5% of patients with GBS.144
Approximately 35% to 50% of patients develop some cra-
nial nerve involvement, primarily facial muscle weakness, Sensory symptoms such as distal hyperesthesias, paresthe-
although patients may also develop oropharyngeal and ocu- sias (tingling, burning), numbness, and decreased vibratory or
lomotor involvement.143,145 position sense are common. The sensory disturbances often
ANS symptoms are noted in approximately 50% of patients. have a stocking-and-glove pattern rather than the dermatomal
Low cardiac output, cardiac dysrhythmias, and marked fluc- distribution of loss. Although the sensory problems are sel-
tuations in blood pressure may compromise management of dom disabling, they can be disconcerting and upsetting to
respiratory function and can lead to sudden death. Other typi- patients, especially during the acute stage.147,148
cal ANS symptoms may result in peripheral pooling of blood, Pain was identified as a significant presenting symptom
poor venous return, ileus, and urinary retention.146 reported in the original articles describing GBS. When
Medical Prognosis
BOX 17-3 COMMON DIAGNOSTIC FEATURES
n
OF GUILLAIN-BARRÉ SYNDROME Although some patients have a fulminating course of prog-
ress with maximal paralysis within 1 to 2 days of onset, 50%
A. Motor weakness of patients reach the nadir (the point of greatest severity)
1. Progressive symptoms and signs of motor weakness of the disease within 1 week, 70% by 2 weeks, and 80% by
that develop rapidly 3 weeks.145 In some cases the process of increasing weak-
a. Relative symmetry of motor involvement ness continues for 1 to 2 months. Onset of recovery is var-
b. Usual progression of weakness from distal to ied, with most patients showing gradual recovery of muscle
proximal; self-limiting to distal limbs of upper strength 2 to 4 weeks after progression has stopped or the
and/or lower extremities or may extend to full condition has plateaued. Although 50% of the patients may
quadriplegia with respiratory and cranial nerve show minor neurological deficits (e.g., diminished or absent
involvement tendon reflexes) and 15% may show persistent residual defi-
2. Areflexia of at least distal tendon responses cits in function, approximately 80% become ambulatory
B. Mild sensory symptoms or signs, particularly paresthe- within 6 months of onset of symptoms. The most common
sias and hypesthesias long-term deficits are weakness of the anterior tibial muscu-
C. Autonomic dysfunction such as tachycardia and lature and, less often, weakness of the foot and hand intrin-
arrhythmias, vasomotor symptoms sics, quadriceps, and gluteal musculature. Three percent to
D. Absence of fever at onset of symptoms; history of 5% of patients die of secondary cardiac, respiratory, or other
flulike illness common systemic organ failure.134,151 Fatigue or poor endurance was
E. Laboratory test results nonspecific but may show eleva- also noted as a long-term consequence of GBS, possibly
tion of cerebrospinal fluid protein; cerebrospinal fluid attributable to deconditioning and peripheral fatigue related
cells at 10 or fewer mononuclear leukocytes per cubic to muscle fatigue during the healing process.141,153 Vasjar
millimeter of cerebrospinal fluid and colleagues154 also report that fatigue and poor exercise
F. Electrodiagnostic testing, nerve conduction velocities tolerance were common persisting symptoms in children
usually abnormal who appeared to have fully recovered from acute GBS.
G. Recovery usually begins 2 to 4 weeks after plateau of Although often not the focus of most studies on the long-
disease process term impact of GBS, sensory deficits (impaired response to
pinprick, light touch, and vibration and proprioception in
combination with other sensory losses) are an ongoing prob-
lem for patients 3 to 6 years after recovery from acute GBS.
pain was prominent, patients spontaneously revealed its In a study of 122 subjects, 38% showed sensory deficits in
presence during a medical history. Therefore therapists the upper extremities155 and 66% had ongoing sensory deficits
who may be working with patients with an onset of low of the lower extremities.151 The muscle aches and cramps
back pain not associated with known injury or stress and experienced by some of these patients appeared to be related
reports of paresthesias (pins and needles) and vibratory or to sensory rather than persistent motor dysfunctions as usu-
decreased tendon reflexes should evaluate or monitor for ally thought.
possible GBS.149,150 Overall, factors associated with a poor prognosis are sever-
The most common description of presenting pain was of ity of muscle weakness (especially quadriplegia), the need for
muscle aching typically associated with vigorous or exces- respiratory support, cranial nerve involvement associated with
sive exercise. Pain was usually symmetrical and reported loss of eye movement and swallowing, rapid rate of progres-
most frequently in the large-bulk muscles such as the glu- sion from onset, length of time to nadir, older age at onset,
teals, quadriceps, and hamstrings and less often in the lower history of gastrointestinal illness, and recent cytomegalovirus
leg and upper-extremity muscles. Some pain reported during infections.134,142 In a prospective study of 297 patients with
late stages of the illness was described as “stiffness.” Pain GBS in Italy, disease severity was not associated with time to
was consistently more disturbing at night.150 As the disease clinical recovery, but it did predict ultimate outcome, along
progresses, some patients experience severe burning or with shorter length of time to nadir, older age at onset, evi-
hypersensitivity to touch or even air movement, which can dence of axon damage, and recent gastroenteritis.137
interfere with nursing care and limit therapy interventions.
The types of pain reported include paresthesias, dysesthe- Medical Management
sias, axial and radicular pain, joint pain, and myalgias.151 Medical treatment depends on the rate and degree of ascend-
Dysautonomia (orthostatic hypotension, blood pressure insta- ing paralysis. Because most patients return to their prior
bility, cardiac arrhythmias and sometimes bowel and bladder functional status, excellent supportive care during the acute
dysfunction) is relatively common in patients with GBS stage is imperative. Respiratory compromise should be ex-
requiring ventilatory support; in one prospective study of pected, and all patients, including those with limited paraly-
297 patients, cardiac arrest associated with dysautonomia sis and sensory dysfunction, must be closely monitored for
was the leading cause of death.137 In patients with paraplegia the rapid onset of pulmonary and cardiac decompensation
or quadriplegia, approximately one fourth had problems or cardiac arrhythmias, paroxysmal or orthostatic hypoten-
with urinary retention caused by detrusor areflexia or over- sion, urinary retention, and paralytic ileus caused by dysau-
activity, overactive urethral sphincter, and disturbed bladder tonomia.152 Because of the possibility of sudden respiratory
sensation.134 The possibility of deep vein thrombosis (DVT) failure, patients with evidence of GBS must be hospitalized
and pulmonary embolus must also be monitored and pro- so that immediate cardiorespiratory support can be given
phylactic treatment used.152 if functional vital capacity (FVC) falls below 20 mL/kg or
oxygen saturation falls below 75%.144 Patients who progress the acute through early recovery stages, and rehabilitation
to respiratory paralysis must be treated in an intensive care throughout recovery.163 With the assumption that the patient
environment where adequate respiratory function can be will have significant return of function within months, thera-
maintained, secondary infections can be prevented or lim- pists must help maintain the integrity of functioning systems,
ited, and metabolic functions can be carefully monitored. address pain, teach compensatory strategies, and appropri-
The patient should be intubated if the FVC falls below ately promote increasing activity after the plateau. The im-
12 mL/kg or if the patient is increasingly dyspneic even if mediate needs of the patient will change as the patient moves
FVC is above the cutoff level.145,156 Twenty-five percent of through the acute stage, the plateau at the nadir, and the recov-
patients who experience respiratory failure will develop ery stage of GBS before and after muscles attain antigravity
pneumonia.151 Even if daytime respiration seems adequate, strength. Transitioning between the changes in immediate
night-time respiratory insufficiency (sleep-disordered breath- therapeutic goals necessitates careful examination of the cur-
ing) should be ruled out if patients have persistent sleepiness rent status, progression of disease, and needs of the patient.
or fatigue.141
Patients with GBS in the intensive care unit (ICU) on Examination
ventilation and with varying levels of paralysis and sensory A comprehensive examination of the patient’s movement and
dysfunction feel trapped and out of control because they function includes factors shown in Box 17-4. The extent of
cannot express their needs. These patients can usually hear the examination in any one session depends on the patient’s
well and most can see what is happening around them. They condition and ability to participate. History taking should
benefit from being oriented to time, having the personnel include the course of the disease, along with any recent
explain all procedures, and having some means of obtaining illness, preexisting neuromotor or other medical conditions,
help. Therapists can work with the ICU staff to provide the current concerns, and the patient’s immediate goals. Screen-
patient with alternative forms of communication, such as ing tests can help determine whether sensory and autonomic
eye blink, clicking, and communication boards designed for systems are involved along with motor systems. Checking
their needs. Having some form of communication and vital signs at rest and immediately after activity, assessing
knowing that they will not be left alone will help prevent skin integrity especially in immobile patients, screening
traumatic stress reactions.152 cranial nerve performance, and noting communication ability
In addition to the intensive monitoring of progression and are all important components. Additional testing of sensation
supportive care required for patients with GBS, two specific (and documentation on a body chart, for example) or auto-
immunotherapy-based treatments—plasma exchange (re- nomic systems may be required if the screening tests indicate.
moval of plasma from withdrawn blood with retransfusion In GBS, assessment of muscle strength and ROM as spe-
of the formed elements back into the blood) and intravenous cifically as possible is important so the patient’s course of
immunoglobulin (IVIg) (taking blood from a vein, separat- progression or improvement can be tracked, possible patterns
ing plasma, and returning the blood cells with a plasma leading to contractures can be predicted and prevented, and
substitute)—have been under investigation for their ability the appropriate level of exercise can be implemented. MMT,
to decrease the duration of respirator dependence and the time dynamometry, or isokinetic testing could be useful in various
to onset of improvement. Systematic reviews of these inter- stages; goniometry is typically used for ROM testing. Full
ventions as of 2010 have found that plasma exchange de- MMT and joint ROM may require several sessions in the
creases recovery time and is most beneficial if begun within initial stages, and a few specific muscles and joints may be
the first week of diagnosis and can be beneficial up to 30 days selected (e.g., sternocleidomastoids, deltoids, triceps, flexor
after diagnosis.157 Plasma exchange is also cost-effective as carpi ulnaris, lumbricals, iliopsoas, gluteus medius, anterior
used in patients with mild, moderate, or severe courses of tibialis, flexor hallucis longus; shoulders, fingers, ankles) to
GBS.158 IVIg is somewhat safer and easier to administer than test for changes weekly.
plasma exchange; IVIg speeds recovery by the same amount Several factors may interfere with complete assessment
of time as plasma exchange and is more effective than sup- in the initial stage. Patients who report considerable pain
portive care only. Adding IVIg to plasma exchange did not during handling or active movement may not tolerate or may
improve time to recovery any more than either treatment be unwilling or unable to cooperate with testing. The thera-
alone.159 High-quality evidence is available to support IVIg pist should track the patient’s level of pain, for example, on
use in adults with GBS; the quality of evidence is slightly less a numerical rating of pain scale, to help distinguish between
high to support its use in children with GBS.160 weakness and loss of ROM related to pathological condi-
Although corticosteroids have been used to decrease the tion, immobility, or pain.
inflammatory process in GBS since the 1960s, a review of Fatigue and respiratory difficulties may also preclude
clinical studies of corticosteroid effectiveness showed that complete strength assessment in a single session. Fatigue
corticosteroid treatment alone does not hasten recovery may result from deconditioning, increased effort required to
from GBS.161 Hughes and colleagues have developed prac- perform similarly with weakened muscles, and inability to
tice parameters associated with these findings.162 recruit sufficient motor units to maintain contractions.164
Fatigue can be documented in relation to amount of activity
Therapeutic Management of Movement tolerated (with specific symptoms noted before rest is
Dysfunction Associated with Guillain-Barré required) or with a questionnaire such as the Fatigue Sever-
Syndrome ity Scale (FSS), Fatigue Impact Scale (FIS), or the Visual
Therapeutic management of the movement deficits associated Analogue Scale for Fatigue (VAS-F).141 Functional tests
with GBS includes supportive management during the acute may include standardized scales of independence in ADLs
phase, prevention of long-term medical comorbidities during or balance, tests of manual dexterity, and temporal measures
BOX 17-4 n FACTORS TO CONSIDER IN THE EXAMINATION OF PATIENTS WITH GUILLAIN-BARRÉ

SYNDROME
HISTORY Identify pain type and location (use body chart): what makes
Patterns and sequence of symptom onset it better, what makes it worse?
Recent illness or injury, prior episodes of sensorimotor Identify pressure points or areas that might lead to pressure
problems sores
MOTOR FUNCTION AUTONOMIC SYSTEM
Visual inspection to identify symmetry of muscle bulk and Blood pressure resting and immediately after activity (prone,
function sitting, standing, if possible)
Myotatic reflexes, rule out tonic reflexes Heart rate resting and immediately after activity, dysrhythmias
Manual muscle testing, carefully identifying pattern of weak- Body temperature stability
ness (testing should be as muscle specific as possible Bowel and bladder control
rather than assessing muscle groups only; use form for PSYCHOSOCIAL SYSTEMS
serial recording) Identify patient and family concerns in acute circumstances
Presence of muscle fasciculations and concerns about long-term issues that may affect
Cranial nerves patient and family. Assessment need not be extensive if
Range of motion (use form for serial recording) referral can be made for social service evaluation of
Equilibrium reactions sitting and standing (if testable) patient and family financial concerns, day-to-day living
Current functional status (activities of daily living, including problems (e.g., transportation, child care), support sys-
bowel and bladder function, ambulation) tems, and coping strategies.
Endurance and experienced fatigue
ELECTRODIAGNOSTIC TESTING
SENSORY SYSTEM Nerve conduction velocity. (Physician will order these studies
Identify pattern of sensory loss or changes (use body to be performed by a clinician skilled in the procedures.
chart) This may be a physical therapist, physician, or technician
Identify specific type of sensory change (e.g., paresthesias, depending on facility.)
anesthesia, hypesthesias) (use body chart)
of gait. Chehebar and colleagues163 review some of the pros in a rural or smaller community hospital may be the first
and cons of standardized tests such as the Barthel Index, person to note a patient’s changing respiratory status during
modified Hughes scale of GBS disability, and the Functional an evaluation and treatment session for muscle weakness or
Independence Measure. Health-related quality-of-life mea- back pain. Therefore the therapist must be prepared to advise
sures used in related populations include the Nottingham nursing and medical staff about the need to test oxygen satu-
Health Profile and the SF-36.164 Forsberg and colleagues165 ration levels and FVC. Therapist attention to respiratory
provide a comprehensive list of tests they administered in a complications is particularly important in the managed care
prospective study of 42 patients followed for 2 years after environment, which discourages hospitalization if presenting
the onset of GBS. At 2 weeks postonset, 40 of 42 patients symptoms are not life endangering.145 A simple estimate of
had submaximal scores on total muscle strength, grip FVC can be done at bedside. If after taking a large breath the
strength, balance, and gait speed testing. At 2 months, total patient can count out loud only to 10, the forced vital capac-
muscle strength was still most affected, whereas 25% of the ity is approximately 1 L and intubation should be considered.
patients had regained maximal grip strength, balance, and Complete information on the PT’s evaluation of patients in
gait speed (designated as 1.4 to 1.5 m/sec). By 2 years, over acute respiratory failure is provided by Irwin and Tecklin.167
half of the subjects still lacked the maximum total muscle Patients who have been intubated or who have cranial
score, and 40% claimed fatigue. Sensory deficits were nerve involvement with oral motor weakness commonly have
claimed by up to 36% of patients at 2 years.165 a high incidence of aspiration. Patients with severe oral-motor
Changes in the patient’s condition should be monitored with problems and dysphagia should be evaluated thoroughly and
serial MMT, ROM assessments, sensory testing, and functional treated by a therapist skilled in oral-motor dysfunction and
status examinations. See Karni and colleagues166 for sugges- feeding. This may be a speech therapist, OT, or PT depending
tions on serial functional assessments. Before the patient is on the facility. Patients with a feeding tube (PEG) should re-
discharged from the hospital or rehabilitation unit, therapists ceive their feedings in a relatively upright position and should
should complete an assessment of the patient’s home environ- remain in that position for 30 to 60 minutes after feeding to
ment so that appropriate safety and adaptive equipment can be decrease the chance of aspiration. According to Logemann,168
in place in time for the patient’s return home. approximately 40% of patients receiving bedside swallowing
assessments have undetected aspiration. Therefore the bed-
Respiratory and Dysphagia Examination side evaluation should be considered only a preliminary step
Therapists are usually involved early in the care of patients in the diagnostic process. In addition to careful assessment of
with GBS. For patients with respiratory or bulbar paralysis, oral-motor control, some clinicians recommend cervical aus-
the therapist’s initial contact may be in the ICU. Although cultation to listen to swallowing sounds, particularly during
most hospitals have fully equipped ICUs, a therapist working the acute phase of the illness.
With evidence of swallowing difficulties and possible quick pressure on the neck and thyroid notch timed with
aspiration, the patient should be referred for comprehen- intent to swallow). A conscious swallowing technique is in-
sive testing with videofluoroscopy. Swallowing also can troduced with thick liquids and progressed to thinner liquids
be assessed by techniques such as fiberoptic endoscopy, after the patient’s oral-motor coordination response is enough
ultrasound, electroglottography to determine laryngeal to control movement of fluids. Once the patient has good lip
movement, and scintigraphy, which involves scanning a closure, fluids should be introduced one sip at a time from a
radioactive bolus during swallowing.169 (Refer to section straw cut to a short length to minimize effort. Semisoft, moist
on medical management of ALS for suggestions for deal- foods are gradually introduced (pasta, mashed potatoes,
ing with dysphagia.) squash, gelatin). Any crumbly or stringy foods (coffee cakes,
cookies, snack chips, celery, cheeses) should be avoided, and
Intervention Goals the patient should not attempt to talk or be interrupted during
General goals for the care of the patient with GBS, to be eating until choking does not occur and swallowing is com-
specified with reference to the patient’s preferences, include fortable and consistent.172 Feeding training should occur
the following: during frequent, short sessions to prevent fatigue. Therapists
n Facilitate resolution of respiratory problems and should be prepared to use the Heimlich maneuver if choking
dysphagia occurs or have a suction machine available at bedside.
n Minimize pain
n Prevent contractures, decubitus ulcers, and injury to Pain
weakened or denervated muscles If pain seems to be a major factor limiting the patient’s pas-
n Introduce a graduated program of active exercise while sive or active motion, the treatment team should determine the
monitoring overuse and fatigue best approach to alleviate pain. According to one study, pa-
n Resume psychosocial roles and improve quality of life tients with GBS did not seem to show a consistent response
to any specific pain medication, although six of the 13 pa-
Therapeutic Interventions tients seemed to have a positive response to codeine, oxyco-
In a Cochrane review of exercise in people with peripheral done plus acetaminophen (Percocet), and oxycodone plus
neuropathies, no randomized or quasi-randomized controlled aspirin (Percodan).147 Some patients may find relief with
trials were identified for patients with GBS as of September medications used to treat neurogenic pain, such as the tricy-
2009.135 However, some treatment programs used for patients clic antidepressants, carbamazepine, or gabapentin (anticon-
with other neuromotor dysfunctions can be adapted for use vulsants).151 For patients who do not respond to conventional
with patients with GBS. analgesics or tricyclic antidepressants, a short course of high-
dose corticosteroids can lead to pain relief.144
Respiratory and Cranial Nerve Dysfunction Some patients with neuropathy have noted decreased
Depending on the facility, PTs may be involved in the respira- pain after using transcutaneous electrical nerve stimulation
tory care of patients with GBS. PTs may conduct chest per- (TENS).173,174 Although no study has examined the effect of
cussion, breathing exercises, resistive inspiratory training, or TENS specifically on pain associated with GBS, it might be
strict protocols to prevent overfatigue of respiratory muscles a treatment option to help with desensitization in patients
while weaning patients from mechanical ventilation.170 Goals whose pain is not controlled with passive movement or pain
of treatment are related to increasing ventilation or oxygen- medications.
ation, decreasing oxygen consumption, controlling secretions, Another option is capsaicin, the active ingredient in chili
and improving exercise tolerance. See Irwin and Tecklin167 peppers, which when applied topically interacts with the sen-
for coverage of treatment programs and techniques appropri- sory neurons to relieve pain from peripheral neuropathies.151
ate for the GBS patient with acute or residual respiratory Therapists, wearing gloves, apply a topical anesthetic until the
dysfunction. area is numb. The capsaicin is then applied topically. The cap-
When patients are placed on mechanical ventilators, saicin remains on the skin until the patient starts to feel the
communication can be difficult and frustrating.171 The reha- heat, at which point it is promptly removed. Because the
bilitation team can help develop and execute alternative nerves are overstimulated by the burning sensation, the sen-
means of communication. sory gateway is unable to report pain for an extended period.175
In the more severe cases of GBS, cranial nerve involve- Some patients who experience extreme sensitivity to light
ment can lead to multiple complications such as dysphagia touch, such as from movement of sheets, air flow, and intermit-
and vocal cord paralysis. In many facilities, speech patholo- tent touch contact, benefit from a “cradle” that holds sheets
gists or OTs are responsible for establishing a dysphagia away from the body. Some find relief if the limbs are wrapped
treatment program. Therapists responsible for treatment of snuggly with elastic bandages, which provide continuous low
patients with dysphagia and swallowing problems should pressure while warding off light and intermittent stimuli. Alter-
refer to Logemann’s classic text on the evaluation and treat- natively, the patient’s pain response can be desensitized through
ment of swallowing disorders.168 Therapeutic goals are the methodical stimulation with frequent, consistent stimuli to the
prevention of choking and aspiration and the stimulation of affected area for short durations to allow acclimatization.170
effective swallowing and eating. The act of chewing and
swallowing is complex and requires coordinated reflexive Contractures, Decubitus Ulcers, and Injury to
and conscious action. Intervention is focused on positioning Weakened or Denervated Muscles
(upright with head tilted slightly forward),171 head control, Positioning. In the acute stage of GBS, rehabilitation
and oral-motor coordination (e.g., sucking an ice cube, will focus on positioning and passive ROM to prevent con-
stimulating the gag response, facilitating swallowing with tractures and decubitus ulcers.176 Preventing pressure sores
starts within the first few days of hospitalization, especially needs to find a balance between working for full joint range
for the patient who has complete or nearly complete paraly- and reacting to the patient’s reports of pain. If the ends of
sis. A positioning program for the dependent patient is the ranges start to become stiff, stretch should be slow and sus-
first line of defense, with turning at least every 2 hours for tained at the end point for 10 to 30 seconds.
both pressure relief and lung drainage.171 In addition, the Denervated or weakened muscles can be injured easily;
patient should have a special mattress or unit that constantly therefore the therapist is responsible for ensuring that joint
changes the pressure within the mattress to shift the patient’s structures are not damaged and that ROM activities are done
position or is designed to spread pressure over wide sur- with appropriate support of the limb to prevent sudden over-
faces. Patients who are slender or who have lost significant stretching. Instruction to caregivers regarding passive ROM
muscle mass from GBS-induced atrophy will have promi- activities must include details such as externally rotating the
nent bony surfaces; the therapist may need to fashion foam shoulder during abduction to prevent impingement and ensur-
“doughnuts” or pads or use sheepskin-type protection for ing that the subtalar joint is in the neutral position during dor-
pressure relief. Patients who have muscle pain may prefer to siflexion to avoid overstretching of the midfoot. In hospitals
have their hips and knees flexed. If so, the patient must be where the patient is treated by a changing therapy or nursing
taken out of the flexed position for part of each hour to avoid staff or by family members, a positioning schedule with dia-
muscle shortening. grams, a splinting plan, and ROM recommendations should be
As part of a complete positioning program, therapists presented in poster format at the patient’s bedside to facilitate
should consider how best to maintain the physiological posi- consistent treatment.
tion of the hands and feet. Research has shown that mild ROM can usually be maintained with standard position-
continuous stretch maintained for at least 20 minutes is ing and ROM programs. Nevertheless, some patients, espe-
more beneficial than stronger, brief stretching exercises.177 cially those who have reported severe extremity and axial
Thus the use of splints for prolonged positioning is superior pain early during the disease process and those who have
to the use of short bursts of intermittent, manually applied been quadriplegic and respirator dependent for prolonged
passive stretching for maintaining functional range. Although periods, may develop significant joint contractures despite
some facilities still use a footboard to control passive ankle preventive interventions. As with patients with spinal cord or
plantarflexion, most therapists now use moldable plastic severe head injuries, heterotopic ossification has been reported
splints that can be worn when the patient is in any position. in patients with GBS.178 Meythaler and colleagues179 note
Because ankle-foot splints often prevent visual inspection of that early mobilization was related to therapeutic decreases
the heel position, care must be taken to ensure that the heel in serum calcium levels and suggest that aggressive ROM
is firmly down in the orthosis and that the strapping pattern (but not hard or abrupt movements that may injure the mus-
is adequate to secure the foot. The strap system must be cle) may impede the effects of heterotopic bone overgrowth,
simple enough to be positioned properly by all staff and which can have a severe impact on ROM. Once heterotopic
family members caring for the patient. The ankle-foot splint ossification has been identified, treatment includes modifica-
should extend slightly beyond the end of the toes to prevent tion of ROM exercise to use only active and passive motion
toe flexion and skin breakdown from the toes rubbing on within the pain-free arc.170
sheeting. Care should be taken not to compress the peroneal Soryal and colleagues180 reported on three patients with
nerve with the splint as it crosses the fibula,148 a particularly GBS who had marked residual contractures that limited func-
vulnerable area after the loss of muscle mass in the lower tion after strength improved. None of the patients had radio-
legs from the GBS.139 Wrist and hand splints may be prefab- logical signs of erosive arthropathy or inflammatory joint
ricated, resting-style splints, or molded to meet the patient’s disease. Soryal hypothesized a number of possible mecha-
specific needs. Because spasticity is not a problem in the nisms for the limitations in ROM: (1) therapists and nurses
patient with GBS, a simple cone or rolled cloth may be may have been reluctant to take patients who reported marked
adequate to maintain good wrist, thumb, and finger align- pain during passive movement through the full ROM; (2) the
ment for short-term immobility. contractures may have been a result of pain or damage caused
Range of Motion. To be effective, the ROM program by inappropriate excessive passive movement of hypotonic
must start within the first couple of days of hospitalization and and sensory-impaired joints and muscles (often caused by
include both accessory and physiological motions to increase poor movement of the patient in bed or by poorly trained staff
circulation; provide lubrication of the joints; and maintain or family members moving limbs); (3) the paralysis may have
extensibility of capsular, muscle, and tendon tissue. Passive resulted in lymphatic stasis with accumulation of fluid in tis-
ROM exercises to the ends of normal range for all extremity sue spaces and nutritional disturbances; and (4) vasomotor
joints, fingers and toes, neck, and trunk should be performed disturbances resulting from autonomic neuropathy may have
twice daily—more frequently if the patient has no active led to adhesions and fibrosis. Although the authors found few
movement. Patients can be instructed to perform the ROM reports describing contractures as a significant residual prob-
exercises themselves if they can move actively without pain lem, they suggested that ROM programs must be defined pre-
or fatigue; during the acute stage of declining strength, they cisely as to frequency and duration, particularly for patients
should be observed during ROM activities to ensure adequacy reporting early joint pain.180
of the range and any changes in quality of movement. If the Some patients will prefer to position their limbs so mus-
patient cannot complete movement through full range inde- cle and tendons are in the shortened range in an attempt to
pendently, a therapist or well-instructed and monitored care- decrease muscle pain. This may lead to capsular contrac-
giver can assist the patient in moving to the end of range. This tures. The therapist should be aware of changes in “end feel”
may not be easy if the patient has pain with motion. Knowing over time when testing ROM of each joint to determine if
whether to “push through the pain” or stay within the limits capsular and ligamentous structures are also becoming more
of pain is often a great dilemma for the therapist. The therapist restricted as the muscle and tendon tissue shortens. Patients
who have intact sensation of pain and temperature may In the acute stage of GBS, active exercise is limited to
respond positively to the use of heat (up to approximately whatever the patient can move without pain or excessive
45° C or 113° F) before stretching to decrease muscle pain fatigue. Slings or adaptive devices may help support the
and facilitate tissue elongation before stretching. Several weight of a limb to continue active movement in a gravity-
basic studies of rat tail tendon and the relation between load eliminated plane for those muscles that have lost antigravity
and heat have shown that attaining permanent length increases strength. As the disease reaches its nadir, activity remains
in collagenous tissue is possible with a combination of heat limited. Once weakness stops progressing, passive mainte-
and stretch.181-184 (Caution: Heat should not be used on a nance of ROM may be the only activity possible for immo-
patient with a sensory deficit that inhibits ability to distin- bile patients. As strength begins to return after the plateau,
guish differences in temperature.) therapists must prescribe limited amounts of low-resistance
On the basis of evidence that continuous passive motion activities, with strict avoidance of antigravity strain on the
(CPM) is effective in maintaining joint range in both rabbits muscles until strength reaches the 3/5 (Fair) range of MMT.
and human beings,185 Mays186 described a case study of a Active exercise can be added very slowly, with frequent rest
patient with GBS (quadriplegia with 7 days of mechanical periods and monitoring to avoid fatigue.177,187 Activity
ventilation) who had persistent pain and stiffness of the should be halted at the first point of fatigue or muscle ache;
upper extremities and fingers approximately 3 months after abnormal sensations (tingling, paresthesias) that persist for
the onset of GBS. CPM of the hands and fingers was added prolonged periods after exercise may also indicate that the
to a program of occupational therapy that included ROM, exercise or activity level was excessive. Any progression of
splinting, and ADLs. The author reported an increase in the resistance or repetitions of strengthening exercises should be
rate of recovery of finger range and a decrease in pain after monitored for 3 to 7 days for increase in weakness, muscle
use of CPM. Numerous other studies have reported the value spasms, or soreness before exercises are progressed further.188
of CPM in maintaining or increasing ROM after hip and If additional weakness or soreness ensues, the additional
knee surgery. It may be a useful adjunct to traditional ther- activity must be eliminated for several days, with reinitiation
apy for patients with GBS, especially those who continue to at a lower level of resistance or number of repetitions and
develop contractures with standard, intermittent ROM pro- more gradual increase. Work simplification and energy con-
grams. Patients with severe paresthesias or dysesthesias may servation strategies may be useful to improve function in the
not be able to tolerate CPM equipment. recovery stage of GBS.170 As strength increases, additional
Massage also may play a positive role in maintaining resistance may be applied to those muscles showing good
muscle tissue mobility and tissue nutrition while limiting the recovery while avoiding strain on muscles that have not yet
amount of intramuscular fibrosis development. The use of reached the same level, frequently the most distal muscula-
massage in patients with GBS has not been reported; how- ture. Even when strength has returned throughout, rehabili-
ever, it makes intuitive sense that it may be a useful adjunct tation and exercise may need to continue to address fatigue
to ROM exercises in patients who do not have marked that may persist at each of the International Classification
hypersensitivity to touch, significant muscle pain, or a his- of Functioning, Disability and Health (ICF) levels: body
tory of DVT. Patients with or without a history of DVT who function and structure, activity, and participation.141 For an
are immobile for long periods or who have concomitant example of treatment progression during the acute stage
cardiac illnesses may have marked swelling of the distal from week 1 through week 12, see Table 17-3.
limbs. After medical clearance, edema-specific massage and In the initial stages of upright activity after any period of
limb-elevation techniques may be useful if tolerated by the bed rest, therapists must progress patients with GBS very care-
patient. Early active ROM exercises creating “muscle pump- fully because 19% to 50% of this population show orthostatic
ing” contractions in muscles with at least fair strength can hypotension along with dysautonomia.139,146 A program to
help prevent uncomfortable edema. improve tolerance to upright position can be started in the ICU
if the patient is on a circle electric or Nelson standing bed. If
Progressive Program of Active Exercise while a standing bed is not available, a sitting program can be initi-
Monitoring for Overuse and Fatigue ated as soon as it is tolerated. A progressive standing program
Although most patients with GBS recover from the paralysis, can be instituted when the patient’s respiratory system and
the course and rate of recovery may vary significantly among ANS are no longer unstable and the patient can be moved to
patients. The decline of strength may take 2 days to 4 weeks, a tilt table. Caution should be taken to stabilize the patient
with a plateau of a few days to a few weeks after the nadir. fully to maintain alignment and to limit activity in muscles
Strength returns over the course of weeks to months, depend- having strength below the fair range. When beginning train-
ing on whether the disease process affected only myelin or the ing, some patients benefit from using an abdominal binder or
axons themselves. Strength usually returns in a descending foot-to-thigh compression stockings if tolerated. Because of
pattern—opposite to the pattern noted during onset of the dis- the relation between poor hydration and hypotension, thera-
ease. No evidence exists to indicate that active exercise can pists must ensure the patient is well hydrated before begin-
change the rate of progression of the disease or regrowth of ning upright or standing tolerance programs.139
myelin or axons, although it may improve function through As was discussed in the section on therapeutic consider-
increased strength and aerobic capacity once muscles are ations for patients with ALS, a muscle that has significant
reinnervated. The major goal of therapeutic management denervation is more likely to respond to exercise with over-
throughout the course of GBS must be to maintain the patient’s work fatigue (see Figure 17-3 for the therapeutic window for
musculoskeletal system in an optimal ready state, prevent exercise). Studying the effect of exercise on rat muscle after
overwork, enhance circulation and cardiorespiratory endurance nerve injury, Herbison and colleagues187 identified a loss of
within the limits of active movement, and pace the recovery contractile proteins during initial reinnervation. After rein-
process to obtain maximal function as reinnervation occurs. nervation the same amount of exercise resulted in muscle
TABLE 17-3 n MEDICAL STATUS OF PATIENTS WITH GUILLAIN-BARRÉ SYNDROME AND POSSIBLE
TREATMENT OUTLINE
MEDICAL STATUS TREATMENT*

Tracheostomy Week 1:
Respirator dependent Postural drainage every 3 hours around the clock
Complete cranial nerve paralysis Passive ROM exercises to all joints
Quadriplegia Splinting (molded plastic) of hands and feet to maintain functional position
Positioning, splinting, and ROM program schedule posted at bedside
Weeks 2-5:
Postural drainage decreased to two times each shift (every 8 hours)
Passive ROM exercises, physiological and accessory motions, gentle stretching of intercostal
musculature, trunk rotations
Continue splinting and positioning program
Family education: family members taught gentle physiological ROM techniques, with
attention to correct shoulder patterns and simple massage techniques
Respirator set on intermittent Weeks 6-7:
mandatory ventilation Postural drainage two times each shift (every 8 hours)
Weaning to respirator at night Continue ROM program, splinting, and positioning
by end of week 7 Begin to build tolerance of upright sitting with good trunk alignment
No active muscle contractions Begin facilitation of active facial and tongue muscle activity in patterns necessary for
except eye opening and lip swallowing, eating, and speaking; speech pathology, occupational therapy consultation
movements for dysphagia training
Dysphagia Family members active in care, helping with ROM, splinting, and positioning schedule as
they choose
Palpable muscle activity in neck, Weeks 8-12:
trunk, proximal musculature of Postural drainage one time each shift
upper and lower extremities Chest stretching, breathing exercises
Dysphagia program in collaboration with speech consultant
Muscle reeducation program with electromyographic biofeedback progressing to gravity-
eliminated exercises using suspension slings attached to bed
Tilt-table standing program to increase tolerance to upright (wearing positioning splints if
necessary)
Collaborate with occupational therapist for treatment in wheelchair with suspension
slings to facilitate active arm motion in gravity-limited position
Exercise, rest, positioning schedule posted
Family, patient educated about stimulating activity level to prevent fatigue, overuse of
reinnervating muscles
ROM, Range of motion.
*Treatment depends on rate of recovery.
hypertrophy. Bensman188 reported on eight patients who had patient the expected movement. The therapist then passively
stabilized after acute polyradiculoneuritis (among them moves the patient’s limb while the patient observes. After gain-
patients with GBS). All eight patients had a temporary loss ing a clear picture of what movement is expected, the patient
of function after strenuous physical exercise. Three patients is encouraged to contract muscles. Facilitatory techniques
apparently had significant decreases in strength. All patients such as skin stroking, brushing, vibration, icing, and tapping
were then placed on a program of passive ROM exercises, may be used in conjunction with the muscle reeducation pro-
and an increase in muscle strength was noted. Recurring cess if the sensory and pain status of the patient permits. The
episodes of a temporary loss of function appeared to be patient is taught to reassess his or her movements and make
related to strenuous exercise and fatigue. The current position corrective responses. As the patient gains strength, the move-
for patients with GBS, then, is that excessive exercise during ments are translated into functional activities.187
early reinnervation when only a few functioning motor units Functional activities should be appropriate for the muscle
are present can lead to further damage rather than to the grade of that muscle or muscle group. For example, if the
expected exercise-induced hypertrophy of muscle. patient’s deltoid muscle has a poor (2/5) grade on MMT (full
During the initial stages of exercise, the repetitions per ROM with gravity eliminated), the patient should be cau-
exercise period should be low and the frequency of short tioned not to attempt to elevate her or his arm against gravity
periods of exercise should be high.177 As reinnervation occurs (e.g., to shave or do one’s hair). Patients may exercise when
and motor units become responsive, the early process of the limb weight is supported (using overhead slings, powder
muscle reeducation exercise used by the therapist may be boards, pool exercises) to allow the patient to move actively
similar to that used after polio. To encourage active contraction through a full range until he or she can take resistance in
of the muscle the therapist should carefully demonstrate to the the gravity-eliminated position. Children, teenagers, or adults
with impaired judgment often need a strict schedule of rest imposed sedentary lifestyle.154 Several studies have reported
and activity. Patients and staff also need to be reminded that the effect of endurance exercise training after GBS. In one
prolonged sitting in bed or in a wheelchair, even when sup- case study a 23-year-old woman with a chronic-relapsing
ported, may tax the axial musculature. A program of gradual form of GBS with onset at age 15 years was placed on a
sitting should be instituted, with the final goal being indepen- walking and cycling program at 45% or less of her predicted
dent, unsupported sitting with functional equilibrium reac- maximal heart rate reserve. The low-intensity exercise
tions. In busy hospitals a schedule of sitting and activity program was selected to prevent possible fatigue-related
should be posted in clear view at the patient’s bedside. relapse. After the program, the subject had improved her
As reinnervation progresses and strength and exercise tol- walking time 37%, walking distance approximately 88%,
erance increases, the therapist may choose to use facilitative and cycle ride time more than 100%. Although no standard-
exercise techniques such as neurodevelopmental sequenc- ized or formalized recording of functional level was recorded
ing189 or PNF190,191 to recruit maximal desired contraction of before and after the exercise program, the patient reported
specific muscle groups. Although PNF techniques are excel- that her energy level for ADLs was a “little higher” and that
lent for eliciting maximal contraction, care must be taken not stair walking was easier.194 In another single-subject study
to overwork the weaker components of the movement pattern. of a 54-year-old man 3 years after onset of GBS with re-
A positive aspect of PNF techniques is that they can be tied in sidual weakness, the authors demonstrated similar improve-
with functional patterns such as rolling, which is necessary ments in cardiopulmonary and work capacities as well as leg
for bed mobility, transitions to quadruped, kneeling, sitting, strength after a 16-week course of a thrice-weekly aerobic
standing, and gait. exercise program. The subject also reported expanded ADL
Because patients with GBS are transferred from acute capabilities. The authors suggested that their training regi-
care facilities to rehabilitation, skilled nursing, or home en- men may disrupt the cycle of inactivity after recovery from
vironments more quickly than in the past, therapists must be GBS that leads to disuse atrophy and further deconditioning
careful to document any serial negative changes or plateaus in patients with mild residual weakness.198 Fehlings and
in motor, sensory, or respiratory impairments or functional colleagues199 tested muscle strength and endurance in a
status that may herald a relapse.139 Although 65% to 75% or group of children at least 2 years after acute onset of GBS.
more of patients with GBS show a return to clinically nor- Although the children appeared essentially recovered, endur-
mal motor function, 2% to 5% of patients have a recurrence ance of the arm muscles was lower than that of the lower ex-
of symptoms similar in onset and pattern to the original ill- tremities. They hypothesize that the typical walking, running,
ness.192 Recurrence of symptoms should trigger immediate and cycling activities that the children participated in were suf-
cessation of activity and possibly medical reassessment in ficient to improve strength and endurance of lower-extremity
case of respiratory insufficiency. muscles, and they recommended that children be encouraged
Anecdotal and empirical evidence shows that patients to participate in activities such as swimming to improve upper-
with GBS can continue to show deficits during strenuous extremity endurance. Controlled tetherball and volleyball
exercises that require maximal endurance. Four soldiers activities are also appropriate. Tuckey and Greenwood200
who were considered clinically recovered from GBS (nor- reported positive results of treatment with partial body-weight
mal motor power with or without reappearance of reflexes support (PBWS) treadmill exercise for a patient with severe
and the absence of sensory impairment) were unable to pass GBS. Garssen and colleagues201 reported a 20% reduction in
the Army Physical Fitness Test (APFT), which is designed fatigue levels, along with improved physical condition and
to measure a minimal acceptable age-related level of physi- strength, after a 12-week intensive bicycling exercise program
cal fitness for military duty (maximal effort to challenge for patients several years after the onset of GBS.
respiratory and muscular endurance, strength, and flexibil- Improvements in strength and endurance after GBS may
ity). Before onset of GBS, the four patients had all exceeded continue for months to years. A prospective study following
the APFT standards. None was able to pass the APFT as 6 patients for 18 months after onset of GBS recorded continu-
long as 4 years after the illness, indicating that the persistent ing improvement of muscle strength on average throughout
deficit interfered with their ability to continue their military the assessment period, and yet the average strength of major
careers.193 The possibility of long-term endurance deficits muscle groups had not yet reached that of healthy con-
should be considered when patients appear to have reached trols.202 Although the traditional thought has been that little
full recovery but report difficulty when returning to work or clinical improvement occurs after 2 to 3 years, Bernsen and
activities that require sustained maximal effort.194,195 colleagues203 found that 21% of the patients in a study of
So far, no pharmaceutical agents have been helpful in al- 150 patients after recovery from acute GBS reported improve-
leviating fatigue in this population. In a study of the use of ment after 2.5 to 6.5 years, although the authors thought the
amantadine to relieve severe fatigue in 74 patients with GBS perception of improvement was related to improved sensory
randomly allocated to treatment or placebo groups, the groups function. Of future research and clinical interest are the long-
showed no difference in any of the primary or secondary term consequences of GBS and how the normal aging process
measures recorded.196 Determining the effectiveness of inter- will affect patients who have some mild residual effects—for
ventions to affect fatigue may be complicated by differences example, whether some patients will develop increasing weak-
in measures of experienced fatigue (subjectively reported) ness over time similar to persons with postpolio syndrome.139
versus physiological fatigue (central or peripheral reduction For those patients who experience significant losses in
in voluntary muscle force production) and the weak relation- proprioception after GBS, sensory reintegration activities and
ship between these in many neuromuscular disorders.197 high repetitions of task practice may help to redevelop motor
Cardiovascular fitness may also be compromised after engrams that are based on the altered sensory perception.139
recovery from GBS. This may be caused by altered muscle Patients with GBS have a significantly reduced health-
function, but it is also related to deconditioning from an related quality of life compared with control subjects at
approximately 1 year after onset, associated with de- elastic strap attached to the shoelaces and a calf band may be
creased functional scores and changes in work status.204 sufficient to prevent overuse of the anterior compartment
Although physical training may be expected to improve muscles. An old-fashioned, relatively inexpensive spring wire
functional scores and work capabilities, Bussmann and brace, which can be attached to the patient’s shoes to facilitate
colleagues205 found little correlation between physical fit- dorsiflexion, is a good choice for patients who report sensory
ness and other domains. They hypothesized that training hypersensitivity when wearing a plastic orthosis.
has psychological components, such as positive effect on Most therapy units today have access to varied sizes of plas-
mood and self-confidence, that influence quality of life in tic, fixed-ankle AFOs that can be used until a decision is made
addition to physical changes. to have the patient fitted with custom AFOs. A newer system of
prefabricated AFOs with adjustable ankle motion cams has
Adaptive Equipment and Orthoses been developed that allows the therapist to limit plantar flexion
Judicious use of orthotic devices and adaptive equipment and dorsiflexion to the specific needs of the patient. For patients
should be considered an integral part of the rehabilitation pro- with reasonable control of plantar flexion and dorsiflexion but
cess. The purpose of the orthotic and adaptive devices is two- with lateral instability because of peroneal weakness, a simple
fold: (1) to protect weakened structures from overstretch and ankle stirrup device such as the AirCast Air-Stirrup Ankle
overuse and (2) to facilitate ADLs within the limits of the pa- Brace (AirCast, Summit, NJ) can be used temporarily to pro-
tient’s current ability. Orthotic devices and adaptive equipment vide lateral ankle stability. Although few patients with GBS
should be introduced and discontinued on the basis of serial need knee-ankle-foot orthoses (KAFOs) on a long-term basis,
evaluations of strength, ROM, and functional needs. For ex- inexpensive air splints or adjustable long-leg metal splints to
ample, a hospitalized patient who has poor (2/5) middle deltoid control knee position are sometimes helpful when working on
strength may practice upper-extremity activities such as eating standing weight bearing and during initial gait training. See
while using suspension slings. A thumb position splint may be Chapter 34 for additional information on orthotics.
used temporarily to aid thumb control in grasping tasks.
Most patients will need a wheelchair for several months Psychosocial Issues
until strength and endurance improve. As strength returns, Although most patients with GBS have a good recovery over
patients recovering from severe paralysis may need to a period of 2 or more years, the acute stage of the disease
change from use of a wheelchair with a high, reclining back can be frightening, especially to patients who progress to
with a head rest to use of a lightweight, easily maneuverable complete paralysis and respiratory failure. Nancy, in Case
chair. A quandary for the therapist is to predict how long a Study 17-2, reported that she was terrified during the time
wheelchair will be necessary and whether it should be she was totally paralyzed (including eyelid movement) and
rented or purchased as the patient progresses through differ- on a respirator. She said that nurses, doctors, and hospital
ent stages of recovery. While moving from wheelchair mo- staff seemed to assume she could not hear because she was
bility to independent ambulation, patients will usually prog- unable to respond in any manner. In her words,
ress from parallel bars to a walker with a seat to allow
frequent resting, and then to crutches or a cane. Because “They acted like I was already dead, and I thought I
wheelchairs, walkers, crutches, and canes, especially cus- would be from the way they were talking. The thing I
tom appliances, are expensive and not always covered by hated the most was when the night nurses from the reg-
insurance, the therapist should carefully consider the cost to istry would come in and ask how to make the ventilator
the patient during the recovery process. work! I felt panicked. Can you imagine having your life
Although most patients with GBS are able to walk within depend on a machine and knowing that the person who
8 months of onset, many show a prolonged residual weakness was supposed to make it work had no idea what to do if
of calf and, most commonly, anterior compartment muscula- a tube came unconnected? They were always worried
ture, requiring the use of an AFO. The decision whether to use about my blood pressure. Who wouldn’t have high blood
a prefabricated orthosis or custom appliance is not always pressure in that situation! The thing I liked about my
simple. Several temporary orthotic measures can be consid- therapists was that they told me what they were going to
ered. For example, if the patient shows good gastrocnemius- do even when I couldn’t respond. They didn’t just start
soleus strength with mild weakness of the dorsiflexors, a simple doing things or pulling on me like other people did.”
CASE STUDY 17-2 n NANCY

Nancy, a 16-year-old girl with a history of repeated hospitaliza- and decreased FVC. She was uncooperative during strength
tions for asthma, was admitted to the hospital with tingling in testing, although strength was estimated to be within normal
the hands and feet and mild respiratory distress. Because staff limits except for approximately Fair (3/5) strength of the
thought her asthma attacks had a significant emotional compo- dorsiflexors and everters and Good (4/5) strength of the plantar
nent, her repeated complaints of paresthesias, muscle pain, and flexors. She became extremely upset when her feet were
weakness were largely ignored or attributed to anxiety attacks. touched.
The day after admission, Nancy began staggering while walking Because of her psychological history, she was referred for
and became extremely agitated and hysterical, screaming that psychiatric assessment and was placed on an anxiolytic medi-
she was dying and could not breathe. A medical assessment cation. Two hours later she had a full respiratory arrest and was
showed evidence of wheezing with a normal chest radiograph intubated and maintained on mechanical ventilation. Over the
CASE STUDY 17-2 n NANCY—cont’d

next 3 days she developed flaccid quadriplegia and within MMT grade of Fair (3) or greater, Nancy was allowed to use
5 days she had complete cranial nerve involvement. She was the muscles in functional activities with specified limitations in
weaned from the respirator after 29 days after several episodes activity duration. When she was able to tolerate upright sitting
of pneumonia. After extubation, she had swallowing and and had some bed mobility, Nancy was transferred to a Nelson
speech problems that resolved by discharge at 3 months after bed in which she could begin a gradual standing weight-bearing
onset. During the acute stage, she was catheterized because program.
of urinary retention and was treated for a bowel obstruction. A speech therapist worked with Nancy in the ICU to help
Sensation was normal for perception of temperature changes her relearn safe swallowing patterns and to reintroduce her to
and deep pressure. different-textured foods. A dietician had been working with
Proprioception was diminished at the ankle, knee, and fingers. Nancy throughout her hospitalization to ensure adequate nutri-
Paresthesias and hypesthesias, aggravated by light touch, were tion while intubated, and she worked closely with the speech
present in a glovelike pattern in both hands and a stocking pattern therapist to progress Nancy’s diet as she became able to handle
in both feet. liquids and solids.
Nancy’s physical therapy treatment began in the ICU. Formal After being weaned from the respirator and transferred to the
strength testing was inappropriate; passive ROM was full but felt general floor, Nancy was brought to the physical therapy depart-
stiff at ends of ranges in the wrist, fingers, and ankles. The goals ment for treatment, which was frequently done in conjunction
were to assist in respiratory care, prevent joint contractures, and with occupational therapy. As strength increased, she began
prevent stasis ulcers during the period of immobility. Although a program of resisted exercise. Trunk and upper- and lower-
her postural drainage treatment was performed by using respira- extremity PNF patterns were used as the primary exercise tech-
tory therapy techniques in conjunction with aerosol medication nique; however, great caution was used to avoid overworking
by intermittent positive-pressure ventilation (IPPV), PTs began weak muscle groups evoked during use of the PNF pattern. A
a course of chest stretching techniques in coordination with a full mat program with rolling and coming to sitting was also
fastidious ROM program performed twice a day by a therapist instituted. OTs focused on graduated use of Nancy’s upper
and on the evening and night shifts by a nurse. A pressure relief extremities, first using overhead slings attached to a wheelchair
mattress was ordered for her bed. To prevent contracture devel- and later using a lap board to support her weakened shoulder
opment, an OT fabricated bilateral wrist and finger splints; a PT musculature while practicing hand activities.
molded ankle splints to maintain 90 degrees of dorsiflexion with After 2 months of hospitalization, Nancy was discharged
neutral eversion-inversion. A positioning and ROM schedule in home to return for daily outpatient rehabilitation. Because Nancy
poster form with pictures of positions and ROM patterns was appeared to be regaining strength well, she was provided with an
posted at Nancy’s bedside. ultralight rental wheelchair through her insurance for use until a
Because Nancy reported severe hypersensitivity to light final determination was made for long-term need. Nancy was
touch or to any passive movement of her limbs, a cradle was also fitted with prefabricated adjustable AFOs, which were
placed on the bed to prevent sheets from touching her and to purchased through the physical therapy department. After 4 to
prevent air flow changes from irritating her skin. She was fitted 6 months a determination would be made about expected recovery
for above-knee light pressure stockings, which seemed to of her persistently weakened dorsiflexors. If Nancy appeared to
decrease her sensitivity to light touch. need AFOs for a prolonged period, a set of specifically molded
Progression of the GBS process seemed to plateau at AFOs would be ordered. At discharge, both the PT and OT made
approximately 15 days after onset with a gradual return of a home visit with the hospital social worker and parents to deter-
respiratory function complicated by infections. Weaning from mine what home adaptations and support services would be
the respirator was difficult, and the PT played a major role necessary.
in instructing Nancy, the staff, and her family in appropriate Follow-up of Nancy’s outpatient therapy showed that she
breathing exercises to be performed every 1 or 2 hours. Because continued to make gradual recovery over the next 1.5 years.
her parents wanted to be involved with her care, they were She returned to school 3 months after rehabilitation discharge
taught ROM techniques with special attention to correct shoul- using a wheelchair. She graduated to a walker, then to forearm
der ROM techniques. The PTs continued to follow Nancy twice crutches, and finally to independent ambulation. She refused to
a day to ensure that accessory motions were completed with be seen using a walker at school, so she continued to use the
the physiological motions. Moist hot packs were used effec- wheelchair at school until she was independent on crutches.
tively before ROM exercises for 1 week to minimize severe She continued to wear bilateral AFOs but was weaned from
muscle pain. full-time use approximately 14 months after discharge. During
As part of her positioning program, Nancy was placed in the weaning process, Nancy wore her AFOs at school while
a supported semisitting position while on the respirator. As walking and for any walking distance over four city blocks or
muscle control returned, a muscle reeducation program was if she heard her feet begin to slap from fatigued dorsiflexors.
initiated that focused initially on the head and trunk and then By 14 months, Nancy showed no evidence of overuse weak-
on the upper and lower extremities. Exercise periods were ness after her regular activities, although she had difficulty with
limited to 15 minutes twice a day. She would have benefited endurance activities in her physical education classes. When
from more frequent short sessions; however, this was not possi- hiking, she carried her AFOs to use when she expected a long
ble. Her parents were shown how to guide her active exercise downhill trek to prevent overwork from eccentric muscle activ-
program cautiously so that she was able to exercise more fre- ity. By age 19 years—3 years postonset—Nancy had returned
quently at low repetitions. When each muscle group reached an fully to her normal activity level.
Skirrow and colleagues206 remind clinicians that the “inten- reinnervation process. Although a rehabilitation program
sive care patient is plunged into a world of machines that has been found to make a measurable difference in patient
flash and beep; of tubes and wires that seem to spring from long-term recovery, many patients are being discharged
almost every orifice; and of mind-numbing sedative and without follow-up care.211 Therefore therapists should be
analgesic medications.” Needless to say, evidence is increas- assertive in ensuring that their patients with GBS have ongo-
ing that patients treated in acute trauma rooms or ICUs ing contact with rehabilitation specialists who can guide the
can have posttraumatic stress disorder (PTSD). Particularly recovery process (see Case Study 17-2).
vulnerable are patients who have had previous traumatic
experiences. PTSD places patients at marked risk for increased DUCHENNE MUSCULAR DYSTROPHY
startle responses, extreme vigilance or anticipation of pain-
ful events, sleep disorders, terrifying dreams, and dissociative Pathology and Medical Diagnosis
flashbacks after leaving the ICU; sometimes these symp- Muscular dystrophy refers to forms of hereditary myopathy
toms are left untreated for years after the experience.152,207 characterized by progressive muscle weakness associated
Patients discharged from prolonged ICU experiences, espe- with deterioration, destruction, and regeneration of muscle
cially those who had respiratory failure, have an increased fibers. During the process, muscle fibers are gradually
incidence of anxiety, depression, and panic disorders years replaced with fibrous and fatty tissue. Each of the inherited
after discharge. forms of myopathy (e.g., Becker dystrophy, myotonic dys-
In a nursing study of patient experiences in the ICU, trophy, limb-girdle dystrophy, and facioscapulohumeral
researchers found that patients often felt anxious, apprehen- dystrophy) has its own unique genetic and phenotypic char-
sive, and fearful. The patients expected ICU nurses to be acteristics. (For a comprehensive review of the forms
experienced and technically adept, but those who felt most of muscular dystrophy and myopathy, see Dubowitz.212)
secure despite the traumatic ICU experiences felt that the Because Duchenne (pseudohypertrophic) muscular dystro-
nurses were vigilant to their needs and offered personalized phy (DMD) is one of the most commonly known forms of
care,152,208 a point clearly made by Nancy in the case study. muscular dystrophy, it is used as a model for discussion
Although one might expect ICU staff to be carefully tuned of treatment implications for therapists. DMD is a disease of
in to patient needs, the highly technical nature of modern progressive muscle weakness leading to total paralysis and
ICUs may attract personnel less focused on individual early death in the late teens or young adulthood. It has an
patient care, or it may prevent caring staff from attending incidence of 13 to 33 cases per 100,000 live births and a new
to the little kindnesses that are so comforting to critically mutation rate of approximately 1 in 10,000 (i.e., one third
ill patients. Baxter207 suggests that caregivers in the ICU try or more of cases occur in families without a history of
to orient patients to what is being done, to approach the DMD). The abnormal gene for DMD has been detected on the
patients within their field of vision, and to minimize unex- X chromosome at band Xp21.2, which encodes for dystrophin,
pected noises and sudden touching. a 427-kD cytoskeleton protein in the membrane. Because
Although most patients recover well from GBS, 3 to it has an X-linked recessive pattern, the disease affects
6 years after onset of GBS 38% of patients in a Dutch study males almost exclusively.213 However, in nearly one third of
had to make a job change to accommodate their physical DMD cases, DNA analysis is normal and diagnosis must be
status, 44% had to alter their leisure activities, and nearly confirmed by protein analysis or immunohistology tests.214
50% described ongoing psychosocial changes.203 Similar In almost 100% of patients with DMD there is a complete
findings were reported in a study of Japanese patients recov- absence of dystrophin from muscle tissue. This loss of dystro-
ering from GBS.209,210 phin results in a weakened cell membrane that is easily dam-
In summary, the rehabilitation program for a person with aged in muscle contraction.213 However, loss of dystrophin
GBS must be graded carefully according to the stage of ill- alone is not considered the sole explanation of the severity
ness. In the acute care environment when respiratory deficits and lethality of muscular dystrophy.215
are present, the initial emphasis is directed toward support Laboratory studies show serum creatine kinase (CK) ele-
of maximal respiratory status through postural drainage, vated more than 100 times normal in early stages of the dis-
chest stretching, and breathing exercises. Because of pro- ease. These CK levels decrease over time with loss of muscle
longed bed rest and immobility related to weakness, acces- mass. Elevated CK level is evident at birth long before symp-
sory and physiological ROM must be maintained with toms are evident. Muscle biopsy specimens show degeneration
around-the-clock efforts. Splinting or positioning devices with gradual loss of fiber, variation in fiber size, and a prolif-
are recommended to maintain functional positions during eration of connective and adipose tissue. Histochemical stud-
prolonged periods of immobility. A gradual program to ies indicate loss of subdivision into fiber types, with a tendency
increase upright tolerance is begun when respiratory and toward type I fiber predominance. Electromyographic studies
autonomic functions have stabilized. Therapists must keep show patterns of low-amplitude, short-duration, polyphasic
in mind the potential to damage denervated muscles with motor unit action potentials.
aggressive strengthening programs when developing a reha- Although the absence of dystrophin is usually discussed
bilitation plan and a home-based conditioning program. relative to skeletal muscle, dystrophin is also evident on the
Perhaps as a result of cautious exercise programs, cardio- membrane surfaces of the cardiac Purkinje fibers and is
vascular conditioning appears to lag significantly behind thought to contribute to the cardiac conduction problems
strengthening, so endurance training should specifically fol- seen in DMD. Cardiac involvement is present in more than
low the return of strength. Adaptive equipment and orthoses 60% of boys with DMD across all ages; however, the com-
should be used as needed to protect weakened muscles, mon electrocardiogram and electrocardiographic abnormali-
facilitate normal movement, and prevent fatigue during the ties are reflected early in clinical complications in 30% of
boys until late stages of the disease, when more than 95% weaken, the child must maintain his knees in hyperextension
of boys have significant cardiomyopathy. Because of the to place the axis of rotation posterior to the line of gravity.
increased life span secondary to in-home ventilation for At this point, mild equinus contractures caused by a muscle
respiratory failure, nearly 20% to 30% of deaths can be imbalance between the plantar and dorsiflexors may help
attributed to cardiac disease.216 the child maintain knee control because the gastrocnemius-
The average IQ of boys with DMD is approximately 85, soleus group provides a torque that opposes knee flexion. If
with one third of the boys testing below 75, as reflected in plantar flexion contractures become severe, however, the
delayed developmental milestones. A specific deficit in child will not be able to maintain standing balance because
verbal intelligence and verbal memory that leads to signifi- his base of support is too small and his ankle adaptive strat-
cant impairment in later cognitive development has been egies are nonfunctional.
identified.217,218 Once the child stops weight bearing, development of se-
vere equinovarus deformities is common. Figure 17-6 shows
Clinical Presentation a pattern of progression of muscle imbalance affecting the
Although histological studies have indicated that DMD trunk and lower extremities in stance. Note the increasing
may be identified in the fetus as early as the first trimester, lordosis and plantar flexion as the boys attempt to maintain
symptoms are seldom noted until the child is 2 to 5 years of their center of gravity posterior to the hip joint and anterior
age. When recalling the child’s early development, parents to the knee joint.
often state that the affected child was more placid and less
physically active than expected.219 The earliest obvious Progression of Gait Pattern Changes
manifestations of DMD, however, may be the delay of early The typical changes in gait pattern over time are identified in
developmental milestones, particularly crawling and walk- Figure 17-7; however, age alone is not an adequate index of
ing. In many cases the onset is gradual. Parents or teachers predicted gait pattern. Many factors influence how long a
may first identify a problem because the boy is noted to child will be able to ambulate. Contributing factors are rate of
have difficulty keeping up with peers during normal play progression of weakness; severity of contractures (hip flexion,
activities and to be somewhat clumsy, with frequent falling external rotation, abduction, knee flexion, and plantar flexion—
when attempting to run, jump, climb structures, or negoti- inversion contractures occur as disease progresses); influence
ate uneven terrain. By age 5 years, symmetrical muscle of body weight; degree of respiratory compromise; type of
weakness can usually be clearly identified by MMT. Deep treatment interventions such as bracing, surgery, and exercise;
tendon reflexes may be absent by 8 to 10 years or earlier. extent of family support; and the child’s personal motivation
Sensation is normal.220 to ambulate. When the child can no longer ambulate function-
The typical progression of weakness is symmetrical from ally, a wheelchair must be ordered to fit the specific needs of
proximal to distal, with marked weakness of the pelvic and that child within his home and community environment. (For
shoulder girdle musculature preceding weakness of the trunk an extensive analysis of changes in gait pattern see Sutherland
and more distal extremity muscles. Bowel and bladder func- and colleagues.222)
tion is usually spared. Progression of weakness is slow but
persistent. Weakness of trunk and lower-extremity muscula- Progression of Upper-Extremity Weakness
ture typically leads to changes in gait at 3 to 6 years of age. The upper-extremity pattern of weakness is similar to that
Muscle mass continues to decline, with increasing weakness in the lower extremities, with proximal musculature being
of the trunk, anterior neck, and upper-extremity musculature affected before distal musculature. Functional changes related
affecting functional activities. A typical child will continue to weakness of upper-extremity musculature, however, usu-
walking until about age 12 or 13 years, at which time the ally lag behind those in the lower extremities by 2 to 3 years.
process of transition to a wheelchair becomes imperative. A The early weakness of the scapular stabilization muscles
rapid decrease in strength may occur after prolonged periods interferes with controlled movement of the arms and hands
of immobilization caused by illness, injury, or surgery.221 during reaching. The child gradually loses biceps and bra-
chioradialis function, followed by continued deterioration of
Progression of Lower-Extremity Weakness triceps and more distal musculature. The marked instability
Before age 5 years, hypertrophy of the calf muscles is fre- of scapular musculature is clearly evident when the child
quently noted. Pseudohypertrophy is evident as the muscle tries to elevate his trunk with his arms (e.g., when attempt-
tissue is replaced by fat and fibrous tissue. Even in the early ing to use crutches) or when he is lifted from under the
stages of the disease, few boys with DMD walk with a nor- shoulders.220,223 A classic test of scapular stability is the test
mal gait pattern. Because of early pelvic girdle muscle for the Meryon sign, in which the child slips from the exam-
weakness, most young boys retain a developmentally im- iner’s grip as the child is being lifted from under the arms
mature, wide-based gait pattern. An early distinctive feature (Figure 17-8). Typical progression of upper-extremity weak-
of DMD is the Gowers maneuver, in which the child gets up ness is shown by use of the reaching test (Figure 17-9).
from the floor by using his arms to crawl up his own legs By the time the child reaches stage 3 of the reaching test,
(Figure 17-5).219 he needs considerable help with eating, hair care, and oral
Muscle imbalance occurs in typical patterns as a result of hygiene. Because of major trunk involvement and marked
weakness and contractures. As the posterior hip muscles lower-extremity weakness, the child will also be dependent
weaken, the child must arch his back when standing and for most ADLs, such as hygiene, dressing, and transferring.
retract his shoulder girdle to maintain the center of gravity Weakness of the respiratory muscles (diaphragm, chest wall,
behind the hip joint. This creates a pattern of lumbar lordo- and abdominal musculature) is usually evident by the tenth
sis with protrusion of the abdomen. As the quadriceps or twelfth year, although the diaphragm remains functional
Figure 17-5 n Child demonstrating Gowers maneuver necessary to achieve upright posture because
of pelvic and trunk weakness caused by Duchenne muscular dystrophy.
Figure 17-7 n Early through late stages of ambulation in Duchenne

muscular dystrophy demonstrating changes in alignment at loading
Figure 17-6 n Pattern of progression of muscle imbalance response, midstance, and terminal stance phases of gait. (From Hsu JD,
affecting trunk and lower extremities in Duchenne muscular Furumasu J: Gait and posture changes in the Duchenne muscular
dystrophy. dystrophy child. Clin Orthop Relat Res 288:122–125, 1993.)
Serum CK is elevated in the female carriers, and genetic

molecular probes of possible carriers are now available to
identify deletions within the Xp21 region (the short arm of
the X chromosome) at a 95% accuracy level. Of course,
some families may have belief systems that do not allow
consideration of pregnancy termination to prevent having a
boy with possible DMD. Those views must be respected.
Prenatal diagnosis of DMD for women without a family his-
tory of the disease is not yet practical.225
Despite much effort, an effective pharmaceutical agent
Figure 17-8 n Meryon sign shows lack of scapular stability as has not been identified to treat DMD. In a Cochrane review,
the child slips from the examiner’s grip when lifted from under Manzur and colleagues226 concluded that glucocorticoid
the arms. corticosteroid therapy improves muscle strength in the short
term of 6 months to 2 years; however, adverse effects such
as weight gain, excessive hair growth, osteoporosis, and
longer than do the intercostal and accessory muscles. A behavioral problems were noted. Researchers have also
progressive, sometimes severe scoliosis may contribute to attempted to implant the normal precursor muscle cells or
respiratory compromise. Pure respiratory failure, restrictive myoblasts directly into dystrophic mice and, in several
lung disease, or respiratory failure caused by infection is cases, into children with DMD to precipitate the prolifera-
the usual cause of death, most commonly at age 18 to tion of normal donor muscle cells into the host muscles of
25 years.224 Typical functional stages in DMD are identified dystrophic subjects, but results have not led to significant
in Box 17-5. See Emery and Muntoni213 for a comprehen- improvement.227 Animal studies using helper-dependent
sive review of the clinical process of DMD. adenoviral vectors for dystrophin gene transfer to muscles in
dystrophic mice show promise for patients with DMD.228
Medical Intervention Although no cure for DMD is on the horizon despite the
positive research on gene transfer, the functional status of
Treatment of Primary Pathology the patient, quality of life, and life expectancy can be influ-
DMD has no cure. Some clinicians suggest that until an enced with thoughtful, functionally based treatment and
effective treatment can be found, the best way to decrease the supportive care. Figure 17-10 provides an overall scheme
number of children with DMD is through genetic counseling. for the management of DMD.
Figure 17-9 n Method of evaluating the working hand as demonstrated by the reaching test.
BOX 17-5 n FUNCTIONAL TRANSITIONS IN PATIENTS WITH MUSCULAR DYSTROPHY
1. Ambulates with mild waddling gait and lordosis. Can run with major dressing. Can perform self-grooming but is
with marked effort, gait problems magnified. Can ascend, dependent for toileting and bathing. May need alternating
descend steps, curbs. pressure relief mattress.
2. Ambulates with moderate waddling gait and lordosis. 7. Wheelchair independence in motorized chair but may
Cannot run. Difficulty with stairs and curbs. Rises need to recline intermittently while in chair. Dependent
from floor using Gowers maneuver. Rises from chair in hygiene and most self-care requiring proximal upper-
independently. extremity control.
3. Ambulates with moderately severe waddling gait and 8. As previous stages; will also use two hands for single-
lordosis. Rises from chair independently but cannot hand activities—one hand supports working arm. May
ascend or descend curbs or stairs or rise from floor perform simple table-level hand activities, some self-
independently. feeding with arm support.
4. Ambulates with assistance or in some cases with bilateral 9. Sits in wheelchair only with trunk support and intermit-
knee-ankle-foot orthoses. May have had surgical release tent reclining or transfer to a supine position. Boys
of contractures. May need assistance with balance. Needs attending school may need to be on gurney for part of
wheelchair for community mobility. Propels manual chair day. May benefit from nighttime ventilatory support
slowly. Independent in bed and self-care, although may or intermittent daytime positive-pressure ventilation.
need help with some aspects of dressing and bathing (Some patients may have had an elective tracheostomy
because of time constraints. and need ventilatory support unit attached to wheel-
5. Transfers independently from wheelchair. Unable to chair.) May have some hand control if arms supported.
walk independently but can bear and shift weight to Will need help with turning at night.
walk with orthoses if supported. Can propel self in 10. Totally dependent. Unable to tolerate upright position,
manual chair but has limited endurance. Motorized chair may elect home ventilatory support. Tracheostomy nec-
more functional. Independent in self-care with transfer essary for prolonged ventilation. Tracheostomy may be
assist for bath or shower. adapted for speech if oral musculature adequate. Needs
6. Wheelchair independence in motorized chair. May need 24-hour care. If around-the-clock home care cannot be
trunk support or orthosis. Needs assistance in bed and arranged, patient must be hospitalized.
Treatment of Cardiopulmonary Factors Assistance in respiration progresses in steps.221 In the

Respiratory failure is the cause of death in 70% to 80% of first step, a self-inflating manual ventilation bag may be suf-
patients with DMD. Cardiac and other causes account for ficient. Step two is associated with manual and mechanically
the remaining deaths. Although cardiac involvement is assisted cough techniques. Steps three and four consist of
evident early, because of limited physical activity the the institution of nocturnal and daytime ventilation, respec-
clinical impact of heart disease is not a significant prob- tively. Step five consists of tracheostomy, if the patient and
lem until the adolescent years. Even while ambulatory, family prefer.
children with DMD have a lower exercise performance Sleep-disordered breathing and hypoventilation are
than age-matched healthy children, with higher resting common in the later stages of DMD, and the onset is
heart rates and diminished cardiopulmonary response often subtle. Early symptoms include repeated nighttime
to submaximal and maximal exercise.229 Once the child awakenings, early morning headache, and daytime sleep-
becomes wheelchair dependent, his cardiorespiratory fit- iness. Inexpensive oximetry can be used in the home to
ness deteriorates markedly. With increasing weakness of identify nighttime oxygen desaturation if polysomnogra-
the respiratory musculature and the development of sco- phy with continuous carbon dioxide monitoring is not
liosis, physicians must be vigilant in their treatment of available.231
respiratory infections.230 The American Thoracic Society Because sleep hypoxia is common in the later stages of
consensus statement on the respiratory care of boys with DMD, IPPV or noninvasive ventilation by nasal mask or
DMD suggests the following: mouthpiece is recommended to control oxygen desaturation
n A child should be seen at age 4 to 6 years for baseline at night. Eventually most boys with DMD enter a stage of
pulmonary function testing. constant hypoventilation throughout the day and night, and
n Patients should be seen by a pediatric respiratory phy- a decision needs to be made about the use of 24-hour venti-
sician twice a year after becoming wheelchair depen- lation support. Daytime ventilation should be considered
dent if the FVC falls below 80% or the child is older when waking Pco2 exceeds 50 mm Hg or hemoglobin satu-
than 12 years. ration is lower than 92% while awake.214 Motorized wheel-
n Patients who need mechanically assisted airway clear- chairs can be adapted to handle ventilator systems so that the
ance or mechanically assisted ventilation should be boys can remain active and mobile.
seen by a pulmonary specialist every 3 to 6 months. Once a patient with DMD requires daytime and night-
n All patients should undergo cardiac and pulmonary time ventilation and has severe bulbar muscle weakness, a
assessments before any surgery.214 decision must be made to elect ventilation by tracheostomy
Neuromuscular and skeletal management Assessments Interventions

ROM Stretching
Strength Positioning
Posture Splinting
Function Orthoses
Tools Interventions Alignment Submaximum exercise/activity
Creatine kinase Genetic counselling Gait Seating
Genetic testing Family support Standing devices
Muscle biopsy Adaptive equipment
Assistive technology
Strollers/scooters
Rehabilitation Manual/motorised wheelchairs
Diagnostics
management
Assessments Considerations
Clinical evaluation Age of patient
Strength Stage of disease
Function Risk factors for
ROM side-effects Tools Interventions
Available GCs Assessment of ROM Tendon surgery
Choice of regimen care coordin Spinal assessment Posterior spinal fusion
Corticosteroid cal ati Orthopaedic
Side-effect monitoring ini Spinal radiograph
and prophylaxis
management Cl o management
Bone age (left wrist
Family
n
Dose alteration and hand radiograph)
Bone densitometry
Patient with
Management of other complications
DMD
Tools Interventions GI, speech/

Upper and lower Diet control and swallowing, Psychosocial
GI investigations supplementation nutrition management
Anthropometry Gastrostomy management
Pharmacological
management of gastric Assessments Interventions
reflux and constipation Coping Psychotherapy
Neurocognitive Pharmacological
Speech and language Social
Autism Educational
Pulmonary Cardiac Social work Supportive care
management management
Tools Interventions
Spirometry Volume recruitment Tools Interventions
Pulse oximetry Ventilators/interfaces ECG ACE inhibitors
Capnography Tracheostomy tubes Echo β blockers
PCF, MIP/MEP, ABG Mechanical insufflator/ Holter Other heart failure
exsufflator medication
Figure 17-10 n Management of Duchenne muscular dystrophy (DMD). Coordination of clinical

care is a crucial component of the management of DMD. This care is best provided in a multidisci-
plinary care setting in which an individual and family can access expertise for the required multisystem
management of DMD in a collaborative effort. A coordinated clinical care role can be provided by a
wide range of health care professionals depending on local services, including (but not limited to)
neurologists or pediatric neurologists, rehabilitation specialists, neurogeneticists, pediatricians, and
primary care physicians. It is crucial that the person responsible for the coordination of clinical care be
aware of the available assessments, tools, and interventions to proactively manage all potential issues
involving DMD. ABG, Arterial blood gas; ACE, angiotensin-converting enzyme; DMD, Duchenne
muscular dystrophy; ECG, electrocardiogram; Echo, echocardiogram; GC, glucocorticoids; GI, gastro-
intestinal; MEP, maximum expiratory pressure; MIP, maximum inspiratory pressure; PCF, peak cough
flow; ROM, range of motion. (From Bushby K, Finkel R, Birnkrant DJ, et al: Diagnosis and manage-
ment of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial
management. Lancet Neurol 9:77–93, 2010.)
or palliative care. Ventilation by tracheostomy allows higher and family input after adequate patient education about
ventilation pressures and a better patient-ventilator inter- prolongation of life by tracheostomy ventilation must be
face.232 However, use of a tracheostomy requires careful respected.214
stoma hygiene to prevent infections and mucus plugs and Cardiomyopathy is present in 59% of children with
requires 24-hour caregiver vigilance.233 Although many pa- DMD by 10 years of age, but the cardiac problems seldom
tients and families adapt well to tracheostomy use, the abil- become symptomatic until the end stages of DMD because
ity to speak audibly may be affected. Consideration must be the child’s decreased activity level does not stress the
given to use of a speaking valve system.214 Several cases of weakened heart muscle. In later stages of the disease, how-
pneumothorax have been reported with long-term IPPV.234 ever, cor pulmonale with right-sided heart failure may
Also, as increasing numbers of patients use long-term tra- occur. Medical treatment of any cardiac symptoms gener-
cheostomy-based ventilation, the potential for tracheal ero- ally follows the conventional interventions. Some boys
sion or tracheobronchomalacia, which must be monitored to with severe scoliosis that creates cardiac compression may
prevent hemorrhaging, is increasing.235 As with patients with require correction by spinal fixation.237,238 Retrospective
ALS, many significant treatment and ethical decisions must data suggest that children treated before ventricular dys-
be made by the patient, family, and health care providers function with corticosteroids have a lower incidence of
when submitting to prolonged HMV.236 Patient autonomy cardiac involvement.214
Nutritional Concerns respiratory compromise, chronic pain related to musculo

Excessive weight gain that impairs functional ability is a skeletal problems, sitting tolerance difficulties, and caregiv-
frequent and difficult problem for children with DMD and ing issues. Figure 17-11 presents an example of a boy with
their families. The typical active child needs approximately moderate scoliosis that affects sitting posture. Note the pel-
2400 calories daily to maintain weight and grow; however, vic asymmetry that would seriously affect sitting alignment.
the child with DMD who is more sedentary or who is wheel- Scoliosis tends to occur in two basic patterns: the early-
chair dependent may need 1200 or fewer calories to main- onset form (seen in approximately 23%), which becomes
tain weight. Because of decreased esophageal and intestinal evident before the child begins to use a wheelchair, and the
motility, exacerbated by weak or absent abdominal muscle late-onset form, which develops, on average, 4 years after
strength, a healthy low-fat diet should be encouraged with wheelchair dependency. In the early-onset form the curve
adequate bulk foods, stool softeners, and fluids to facilitate usually becomes severe and progressive, leading to pulmo-
bowel function and motility. Problems with obesity are often nary compromise and structural-based pain. In the late-onset
related to the family’s typical pattern of eating and nurtur- form the course is usually mild. Unfortunately, attempts to
ing. The child and family members may “feed” their anxiety control sitting posture through the use of a spinal orthosis
or depression about the disease.225 In many cases, family and wheelchair seating inserts (inserts that place the child in
members and friends feel that the child’s only pleasure may lumbar lordosis to lock facets, thereby preventing rotation
be eating. Although this may seem true, caring for a totally and lateral collapse, or, more commonly, lumbar and tho-
dependent obese teenager or young adult can become prob- racic lateral supports) have been disappointing.243 Bach244
lematic for both the child and the caregivers. Before obesity states that thoracolumbar bracing is never indicated to slow
becomes an issue, the child and his family should be re- scoliosis development in DMD and it cannot substitute for
ferred for comprehensive nutritional advice from a specialist surgical correction; however, spinal bracing may improve
experienced in dealing with childhood obesity. Suggestions comfort and postural stability in some patients who are not
for adapting eating behavior and food choices will not be eligible for surgical correction because of severe respiratory
followed if they are too restrictive or unreasonable for the or cardiac involvement.242
child’s social situation.239,240 Efforts have been made to delay the time of onset of sco-
Although obesity is a common problem for children liosis with steroid treatment protocols. Evidence supports the
with DMD (greater than 54%), malnutrition is also common. hypothesis that onset of scoliosis can be delayed; however, a
Malnutrition usually occurs in the late stages of the disease as longer follow-up period would be required to determine if
a result of dysphagia.214 Special care must be taken to provide scoliosis can be prevented.245
adequate nutrition after spinal surgery. One review showed Cervellati and colleagues246 reported on a study of
that postsurgical weight loss was related to the inability to self- 20 boys treated from 1985 to 1995 and concluded that early
feed; therefore the investigators suggest that before surgery a surgery significantly reduces the risk factors associated with
feeding evaluation should be done and an appropriate plan severe spinal deformities. The period after spinal surgery
should be put in place to prevent postsurgical malnutrition.241 requires careful coordination of medical, respiratory, and
As the disease progresses, some children develop prob- physical therapy services. Depending on the hospital cul-
lems swallowing, and then weight loss and malnutrition can ture, PTs may be responsible for the pulmonary drainage
become an issue. To decrease the possibility of aspiration, and breathing exercise programs as well as typical passive
careful attention must be paid to food textures and chewing and active exercise programs while the child is in ICU and
and swallowing functions (see page 537 for information on postsurgical care environments. Preferably, therapists should
dealing with bulbar symptoms). Depending on the patient’s introduce postural drainage and breathing techniques as well
and family’s decisions about prolongation of life, some pa- as exercise expectations to the child before surgery to gain
tients now elect to have a permanent PEG placed once self- better cooperation after surgery.
feeding and swallowing become a problem rather than a
pleasure. Even if the patient can still swallow and enjoys eat-
ing in the late stages of DMD, the patient may not be able
to physically take in adequate calories; the PEG allows the
delivery of needed calories and fluids beyond what the patient
can take orally (see page 563 for information on dysphagia
and eating issues).242 Consensus is that body weight and body
mass index should be reviewed regularly and family educa-
tion on nutrition should be an ongoing process. Evaluation
of swallowing should be assessed by taking a history of chok-
ing episodes and observing the child eat different foods and
fluids. Videofluoroscopy should be used to determine if aspi-
ration is a problem, and appropriate adjustments in feeding
should be instituted under the supervision of the appropriate
therapist.214 (See also Bushby and colleagues.221)
Treatment of Scoliosis
Scoliosis is a frequent complication of DMD, with a re-
ported incidence of nearly 90%. Consequences of severe
scoliosis are increased respiratory problems in boys with Figure 17-11 n Moderate scoliosis affecting sitting stability.
Treatment of Other Musculoskeletal Dysfunctions Manzur and colleagues252 carried out a randomized, con-
The primary effect of progressive weakness in DMD gener- trolled trial of 20 boys with DMD (ages 4 to 6 years) to
ally results in secondary effects such as decreases in muscle study the effect of early release of contractures versus con-
extensibility, joint contractures, and bone demineralization. servative (stretching) programs. The boys were followed for
Strength loss diminishes the ability to move actively 12 months or more. Surgery corrected the contractions and
through full range, shift out of static positions, balance improved the speed of gait and transfers over conservative
muscle forces around a joint, and avoid fibrotic changes in treatment as measured at 12 months, but a 2-year follow-up
muscle tissue.221 Loss of ROM from muscle shortening and of six of the boys who had surgery revealed a recurrence of
joint stiffening will occur if not aggressively prevented. ankle contractures. In addition, some of the boys in the oper-
Once present, contractures can severely complicate func- ated group showed more rapid deterioration. The authors did
tion. Long bone fractures in children with DMD are a seri- not recommend routine early surgery to relieve contractures.
ous problem that can have a significant long-term impact on
ambulation. In a study of 378 patients, 21% had incurred Therapeutic Management of Movement
fractures, primarily from falling. Leg fractures predominate Dysfunction Associated with Duchenne
in independent ambulators and wheelchair users, whereas Muscular Dystrophy
upper-extremity fractures more often occurred in boys Like ALS, DMD has a relentless and incurable progression
using KAFOs. Twenty percent of those who had fractures toward total dependence and eventual early death. The dif-
lost the ability to ambulate.247 ferences are the population (children rather than adults) and
In standard treatment protocols for children with DMD time course, with DMD taking 15 to 25 years rather than the
who have impending loss of ability to walk independently, 3 to 5 years typical of adults with ALS. As in ALS and GBS,
bilateral KAFOs are used in conjunction with surgical strength and endurance remain the primary impairments of
release of contractures.248 At the point of surgery, a pattern of DMD, with secondary problems such as contractures and
contractures has magnified the effect of weakness from the respiratory problems following from immobility. Unlike in
loss of approximately 60% of muscle mass.249 Surgery is the other neuromuscular disorders, the endurance problems
typically followed by an aggressive therapy program. Bach in DMD are related to peripheral fatigue, fatigue stemming
and McKeon250 studied 13 boys with DMD who had surgery from the muscles themselves rather than from the lack of
to release lower-extremity contractures. Seven boys were ability to recruit additional motor units.253 As in ALS and
ambulating independently before surgery (early surgery GBS, therapeutic management in DMD will involve evolu-
group), and six boys were preparing to use or had begun tion of the intensity and frequency of exercise to correspond
to use a wheelchair before surgery (late surgery group). to changes in the strength and endurance of the patient. In
Depending on the contracture patterns, the boys underwent all three disorders, the general therapeutic goals are to
surgical procedures that typically included subcutaneous re- maximize function, manage discomfort, and promote opti-
lease of the Achilles tendons and hamstring muscles and mal quality of life. The differences among the disorders
fasciotomy of the iliotibial bands. Four patients had rerouting mean that the actual form of the exercises and interventions
of the posterior tibialis to the dorsal surface of the second or in DMD may require adaptation to suit a child or adolescent.
third cuneiform to balance the foot and prevent the often se- Ideally, a team of specialists should be involved in the long-
vere varus position of the foot. Boys in the late surgical group term care of a child with DMD and his family. The thera-
required more extensive inpatient rehabilitation, whereas pist’s primary role is twofold: to perform serial examina-
boys in the early surgical group were treated as outpatients tions of the child’s movement capabilities and to adjust the
after a short hospitalization. Physical therapy was started on child’s intervention program as the disease progresses. Even
the second postoperative day. The program consisted of gen- with relentlessly progressive diseases, rehabilitation pro-
eral conditioning exercises of the trunk and extremities (e.g., grams can have potential psychological benefits, such as
rolling, trunk stabilization, neck and head control), stretching more positive coping strategies, while physical activity con-
exercises, and intensive weight bearing in standing while tinues to decline.254
wearing bilateral long-leg casts or below-knee casts, depend-
ing on the surgery. One child participated in a pool therapy Examination
program. Bach and McKeon250 suggest that early surgery for A typical therapy examination should include a history,
contractures followed by intensive physical therapy can pro- systems review, and tests and measures to assess muscle
long brace-free ambulation. The number of falls experienced strength, endurance, and ROM impairments along with
by the boys decreased markedly after the surgery and reha- levels of activity and participation. In some facilities the
bilitation period. Boys in the early intervention groups ben- therapist also collects data on the child’s pulmonary sta-
efited from the surgical interventions more than the boys in tus.167 History taking should include the course of the dis-
the later intervention groups. All patients and their families ease, any recent illnesses or losses of function, coexisting
in the early surgery group thought that the procedures were neuromotor or other medical conditions, current concerns,
helpful. Boys in the late surgery group, however, stated either and the goals of the patient and family. Screening tests can
that they would not have had the surgery if they had a chance help rule out sensory deficits, identify cardiac and respira-
to decide again or that they had no opinion. Roposch and tory issues, and determine skin integrity, especially in im-
colleagues251 reviewed the records of 91 boys with the typi- mobile patients. Checking vital signs at rest and immedi-
cal equinovarus deformity in DMD and strongly recom- ately after activity, noting communication ability, and assessing
mended surgical intervention, including a posterior tibialis ability to follow multistep commands are all important
transfer, over conservative, nonsurgical treatment to maintain components. When screening tests indicate a deficit, fol-
foot position and lengthen time of ambulation. low-up should occur with additional testing or referral to
the appropriate professional. The tests and measures appro- the Muscular Dystrophy Functional Rating Scale (MDFRS)
priate for assessing the movement dysfunction of patients to standardize assessment of the functional impact of mus-
with DMD include measures of strength, ROM, function, cular dystrophy, including people with DMD—more than
activity, and quality of life. Palmieri and colleagues255 half of those tested. The MDFRS consists of 33 items cover-
review many of the measures reported in the literature for ing mobility, basic ADLs, arm function, and impairment
use in this population. (including contractures, strength of the trunk and neck, sco-
Manual Muscle Testing. MMT is used extensively for liosis, and respiratory issues). The developers reported test-
measuring muscle strength of children with DMD255,256 and retest and interrater reliabilities of 0.98 to 0.99 and good
is relatively reliable if consecutive examinations are made by evidence of validity. Brooke and colleagues264 and Vignos
the same rater. Intrarater reliability of scores in the gravity- and colleagues265 have described previous functional scales
eliminated position have been shown to be highest in this for use in DMD; the MDFRS compares favorably with each
population.257 DMD shows a linear pattern of decreased of these, with some advantages for determining the child’s
muscle strength (loss of about 0.25 MMT unit per year from status and for predicting appropriate care, perhaps because
ages 6 to 13, and 0.06 MMT unit per year from age 13 on)258 it is longer.263 As part of any functional assessment in DMD,
without marked increases in the rate of deterioration in adaptive behaviors should be noted. For example, a child
strength over time. Thus, marked, precipitous changes in may not be able to lift his arm overhead, but he may use his
muscle strength noted in a few months with initiation of fingers (strength often remains intact even after respiratory
bracing or wheelchair use,223 for example, or immobilization support is necessary) to “crawl” up his chest to reach his
after fracture, generally reflect disuse atrophy rather than head or he may lean forward to approximate his chest to his
disease progression. Such transitory weakness may respond hand or use his other arm or a lever system to assist with
to increased activity and exercise. The history and medical activities.244
records can help differentiate weakness stemming from For ambulatory patients, gait velocity can help predict
various sources and thus determine the potential for strength- how long the patient has before transitioning to a wheel-
ening. Cable and strain-gauge tensiometers, handheld myo- chair. In a longitudinal study of 51 boys with DMD, 100%
meters and dynamometers, and isokinetic dynamometers of those who took 9 seconds or more to walk 30 feet
may also be useful for a more discriminating documentation were wheelchair bound within 2 years.258 McDonald and
of muscle strength.255,256 colleagues266 also recommend the use of the 6-Minute Walk
Range of Motion. ROM is assessed with goniometry in test as a standardized and functional measure of endurance
most cases of DMD.256 As with MMT, serial ROM evalua- for this population. Slight modifications may be necessary to
tions should be completed by the same therapist because keep younger children on-task for this test.266 Observational
intrarater reliability is higher than interrater reliability in this gait analysis can help to identify adaptive behaviors and use
population.259 The two-joint muscles are most prone to de- of compensatory strategies during locomotion.256
veloping shortness, so the positioning of limbs for testing of Respiratory Function. The PT’s role in evaluating
ROM must be considered. The lack of upright weight bear- respiratory status in children with DMD will vary depending
ing and reliance on a wheelchair for mobility tend to accel- on the facility and area of the country in which the therapist
erate contracture development in DMD; therapists should be works. For more in-depth information regarding evaluat-
particularly vigilant about monitoring lower-extremity ROM ing pulmonary status, refer to Chapter 30 or see Irwin and
as the child becomes more sedentary.260 Some boys with Tecklin.167 At a minimum the therapist should evaluate
significant shoulder girdle weakness begin to develop con- bulbar function, cough effectiveness, and FVC (a simple
tractures even before they become wheelchair dependent. spirometer available in most clinics is adequate). For more
Early attention should be paid to possible subluxation of the sophisticated testing, the child should be seen by a pulmo-
shoulder.261 nary function specialist. In addition, the therapist may
Particular attention should be given to the accuracy of monitor activity levels via armbands or pedometers or may
measuring hip ROM. Rideau and colleagues262 recommend assess metabolic equivalents or caloric consumption to
the “dangling leg” test, in which the child is placed supine design the optimal activity program for children with DMD
with his lower legs hanging over the end of the table. An and obesity.255 One method of testing a child’s energy cost
inability to bring the thighs to midline indicates shortening during ambulation is the energy expenditure index,267 which
of the iliotibial band and hip abductors. One can quantify the divides walking heart rate (WHR) minus resting heart rate
shortening by measuring the distance of the thigh from (RHR) by walking speed (distance [D] divided by time
the midline and from the surface of the table. In addition, the [T]):(EEI 5 [WHR 2 RHR]/[D/T]). Determinations of energy
therapist should note pelvic obliquity, preferably with serial expenditure while walking may factor into the decision to
photographs taken with the child in the sitting and supine transition to a wheelchair, at least for longer distances.
positions. Ideally, the patient can be photographed from In late stages the therapist may need to assess the child’s
the back in sitting position against a simple clear, framed bulbar function to prevent swallowing and aspiration prob-
plastic sheet with grid squares to allow easy, nonradio- lems caused by tongue and oral-facial muscle weakness.
graphic tracking of scoliosis. Therapeutic Goals. The basic goals for a therapeutic
Functional Status. The child’s functional status contin- program are straightforward: (1) to prevent contractures that
ues to be relatively stable for some time even when MMT can lead to further disability and pain, (2) to maintain maxi-
indicates that the child is losing strength. Because the weak- mal strength and endurance and prevent disuse atrophy,
ness is gradual, many children develop remarkably adaptive (3) to facilitate maximal functional abilities by using appro-
adjustments in movement patterns to remain functional even priate adaptive equipment, (4) to maintain maximal respira-
with marked strength loss. Lue and colleagues263 developed tory muscle strength and movement of secretions, and (5) to
foster realistic child and family expectations within the con- Although inspiratory exercises tend to be the focus of inter-
text of the environment. These are broad-based goals; the ventions, expiratory inefficiency may play a major role in the
therapist will need to write more specific, time-oriented inability to clear secretions.274 Once the child begins to have
goals for a particular episode of care. difficulty clearing secretions, the family should be taught
manual or mechanically assisted postural drainage techniques
Therapeutic Interventions as long as the patient has an adequate cough. Patients who
Younger children with disabilities are usually eligible for need support with coughing can be taught “air stacking” tech-
school-based therapy services. However, therapists increas- niques (taking a series of breaths without exhaling between
ingly act primarily in the role of consultant rather than direct breaths) to increase intrathoracic pressure needed to cough
service provider, especially for older children. Much of the effectively. Some patients respond well to manual coughing
child’s exercise program must be carried out at home by assistance. Increasingly patients and caregivers are being
parents or caregivers. When both parents work outside the taught to use a mechanical insufflator-exsufflator (positive
home or when the child lives in a single-parent home with a pressure followed by negative pressure) to stimulate cough-
working parent, compliance with home programs can be ing.275,276 These techniques should be reviewed and used
problematic. As many exercise activities as possible should aggressively whenever the child is bed bound for more than
be encouraged within the child’s school day so that parents 1 or 2 days and before and after all surgical procedures.214
can focus on parenting, nurturing, general caregiving, and Physical therapy interventions, such as postural drainage and
simple positioning and bedtime exercises. Under the super- breathing exercises, are invaluable in preventing early death
vision of a consulting therapist, the child’s therapy often can from respiratory failure. The Muscular Dystrophy Association
be provided in some form at the child’s school if on-site continually updates its information on breathing and respira-
therapists, personal attendants, or adaptive physical educa- tory care.277,278
tion teachers are available. In end stages of DMD when the child is dependent, deal-
Respiratory and Dysphagia Care. In the school ther- ing with oral-motor problems that may interfere with eating
apy environment, where most children with DMD are and swallowing is imperative. Techniques such as position-
monitored, the therapist should be prepared to provide the ing, increased sensory input (texture, temperature), and
child and family with methods to improve breathing effi- volume changes in foods may improve the child’s swallow-
ciency. In the early stages of the disease, the child and ing and allow the child to continue taking food orally.279 The
family can be taught simple breathing exercises stressing interventions are similar to those described for ALS. The
diaphragmatic breathing, full chest expansion, air shifts, and Muscular Dystrophy Association also publishes informa-
rib cage stretching. Most children enjoy playing with hand- tional manuals dealing with dysphagia problems (see www.
held incentive spirometer units and playing blowing games mdausa.org).
(e.g., bubbles, pinwheels). Respiratory exercise in different Prevention of Contractures. Diligent ROM exercises
studies has resulted in improvement in respiratory endur- for the whole body will require cooperative efforts of the
ance,268 ventilatory muscle endurance but not respiratory rehabilitation team and the patient and family. Stretching
muscle strength,269,270 and both respiratory muscle strength may progress as weakness dictates, from active to active-
and endurance.271 In the last study, two thirds of the 27 sub- assisted to passive to prolonged elongation phases using
jects had DMD, with percent predicted vital capacities of positioning, splinting, orthoses, and standing devices.221
27% to 96% that had decreased over the 6 months immedi- During the ambulatory phase of the disease, focus should be
ately preceding the exercise protocol. The exercise protocol, on the hips, knees, and ankles. Later, focus will shift to the
monitored via a visual feedback system, consisted of twice- shoulders and the elbow, wrist, and finger flexors. At the first
daily sessions of 10 cycles of resisted inspiratory breaths at sign of loss of end ROM, the therapist should adjust the
70% to 80% of the patient’s maximum inspiratory pressure, child’s program to include specific stretches.261,280
plus 10 maximal static inspiratory efforts that reached at Evidence provides a protocol for stretching in people with
least 90% of the maximally generated inspiratory pressure. normal muscles to increase ROM: stretches performed 2 to
The intervention lasted for 2 years, with increases noted in 5 days a week, once per day, held for 10 to 30 seconds for
the first 10 months and a plateau maintained through the end three or four repetitions over a 6-week time frame.281,282
of the training period. Winkler and colleagues272 noted Unfortunately, no such evidence exists for the best stretching
similar effects in a 9-month training protocol, somewhat protocols in DMD to maintain ROM. Palmieri and col-
dependent on the rapidity of respiratory function decline leagues recommend that stretching be performed a minimum
in the year preceding the training. In the 6-month training of 4 to 6 days per week for any joint or muscle group.255
period of another study, subjects training with resisted inspi- The stretch should be slow to avoid muscle reflex contrac-
ratory and expiratory breathing had significantly greater tions, and sustained at the end point for 10 to 30 seconds. To
benefit than subjects randomly allocated to the group per- increase muscle extensibility, dry or wet heating, electromag-
forming the same breathing exercises without resistance. netic stimulation, or a warm bath may help; for best effect,
The static inspiratory and expiratory pressures returned follow a bath by drying with prewarmed towels to avoid
to baseline within 3 months after training ceased, but shivering and muscle stiffening.255
improvements in perceived exertion persisted for up to In a 2010 Cochrane review283 of the best methods for
1 year postintervention.273 increasing ankle ROM in patients with neuromuscular
Respiratory exercise cannot reverse the process of respira- disease, only two studies of DMD were noted, with inter-
tory failure; however, attention to pulmonary hygiene can ventions of early surgery252 or prednisone use.284 Surgery
help the child cope more effectively with respiratory infections eliminated the contractures, but in most cases the contrac-
and the discomfort accompanying respiratory compromise. tures had recurred by the 2-year follow-up.252 Prednisone
had no significant effect on ROM in comparison to a pla- protocols can be provided at this time. In general, however,
cebo or when comparing two different doses.284 Hyde and both strengthening and aerobic exercises should be con-
colleagues285 noted an annual delay of 23% in the develop- sidered, the frequency and intensity of which should be
ment of contractures at the Achilles in boys with DMD appropriately prescribed based on the disease course and the
randomly allocated to a group receiving both stretching and patient’s abilities and goals.
night splints compared with boys who had stretching alone. Strengthening exercises have had mixed support in the
Brooke and colleagues220 reported similar findings, and past.289 de Lateur and Giaconi290 noted small gains in
Scott and colleagues286 noted that boys who had both AFOs strength of the exercised compared with the unexercised
and stretching were able to continue walking longer than quadriceps muscle of four boys with DMD during and for
boys who did not. This evidence indicates that multiple 18 months after a 6-month exercise program of submaxi-
simultaneous strategies may be most beneficial in prevent- mal isokinetic contractions, 30 repetitions, 4 to 5 days per
ing shortening and maximizing function. Patient and family week. No postexercise weakness or increases in deteriora-
preferences must be considered for any plan to be effective, tion were noted in the exercised muscles. Vignos and
however. Some young patients do not tolerate night splinting Watkins291 instituted a home program of maximal resis-
well. In such cases, AFOs to control plantarflexion contrac- tance exercises for 1 year; the 14 patients with DMD in
tures may be preferred over long-leg orthotics that prevent the exercised group improved in strength for the first
knee flexion contractures or align hips (using an additional 4 months and then reached a plateau, compared with
bar between legs to control rotations). declines in strength of the control group. Scott and col-
Early in the course of the disease process, both parents leagues292 noted diminished strength after a strengthening
and the child must be educated about the expected changes home-exercise program for 18 boys, although with no
in muscle balance and how they can play an active role in control group, possible reductions in disease progression
preventing or limiting the impact of contractures caused by could not be confirmed.
muscle imbalance. Because contractures at the hip, knee, Evidence for the effectiveness of strengthening exer-
and ankle interfere with the mechanical alignment neces- cises in other muscle disorders is insufficient293 and cannot
sary to stand erect and walk, each day the child should be thus be generalized to DMD. Elder294 reviewed animal
encouraged to move his own limbs to end ranges through studies suggesting that dystrophic mice trained on a tread-
normal play activities to slow development of contractures mill showed increased damage to muscle tissue, whereas
related to sedentary positioning. Some research supports forced swimming in dystrophic mice had no adverse effect.
the view that the combination of positioning, stretching, In a case review of three generations of patients with
and splinting should begin before contractures exist. For facioscapulohumeral muscular dystrophy (seven cases and
example, the child can be encouraged to watch television or one suspected case), Johnson and Braddom295 noted asym-
play video games while lying prone with legs aligned out of metrical weakness of the upper extremities. They related
the common “frog leg” (hip abduction and external rota- the weakness to patterns of overuse (dominant side or
tion) pattern. Once a child has significant hip flexor or ilio- side used most often in work activities). On the basis of
tibial band contractures, stretching techniques must be their information and additional evidence that muscle-
specific because simple prone positioning can force the derived enzymes (CK and myoglobin concentrations in
lumbar spine into excessive lordosis. Although difficult to blood) were markedly elevated in patients with DMD after
accomplish in some mainstreamed school environments, prolonged exercise,296 repetitive exercise may be contrain-
positioning the child in a standing frame during several dicated.297 In contrast, Cup and colleagues293 reported that
class periods helps provide prolonged stretch to hip, knee, in their review of 33 studies of exercise therapy for neuro-
and ankle musculature. Later in the course of the disease, muscular diseases, they found absent or negligible adverse
resting hand splints are appropriate to control shortening of effects; one study reported that “3 of 20 patients decreased
the long finger flexors.221 their training for 1 or 2 sessions due to delayed-onset
Although development of contractures of the hip, knee, soreness.”
and ankle from muscle imbalances has been thought the Given the evidence to date, Hasson298 concluded that
cause of early loss of ambulation instead of weakness,287 exercise consisting of brief periods of low- or high-intensity
others believe that weakness causes the loss of ambulation activity can improve strength for patients with minimal to
instead.280 Some authors note that loss of ambulation can moderate weakness. The increased recruitment of motor
occur from either case.255 Limiting contracture development units from training effects also may improve muscle coordi-
facilitates mobility and handling throughout the course of nation and reduce disuse atrophy. However, exercise pro-
the disease, however, and the best approach to contractures grams have minimal effect on strength of muscles already
is to prevent them.260 severely weakened.
Exercise and the Maintenance of Maximal Functional In addition to active and resistive exercise programs,
Level. Because DMD affects muscles throughout childhood Scott and colleagues286 completed a small study of the effect
and adolescence, when strength and endurance are generally of intermittent, long-term, low-frequency electrical stimula-
developing, effectiveness of strengthening and aerobic exertion on dystrophic anterior tibialis muscles. They demon-
cise has been difficult to assess.267 Training programs may strated a significant increase in mean voluntary contraction
maximize muscle and cardiorespiratory function, but they force and suggested that electrical stimulation can have
have also led to reports of weakness after physical exer- a beneficial effect if used with children whose muscles are
tion.288 The debate over the value of exercise in DMD and not already markedly weakened. Zupan299 supports this find-
the relative lack of controlled trials have limited the ability of ing, but children under treatment were unable to maintain
clinicians to provide evidence-based therapy. No definitive strength beyond 4 to 5 months.
Evidence for the effect of aerobic training in DMD is Percent of Maximum Potential Stress
Clinical
sparse.300 Hasson,298 in a review of exercise studies of patients 0 20 35
THERAPEUTIC WINDOW
80 90 100
Correlates
100 Normal
with muscular dystrophy, reports that oxygen consumption
Percent of Normal Muscle Available for Exercise

improved with endurance training, although whether repeti-
hy
nc e
Atrop
Degree of Disease Involvement

tive endurance training at moderate or high intensity (70%
a
Early
ten
Mild
se
DMD
of V̇o2max) causes muscle damage is unknown. Muscle
ct
in
u
fe
ea ge
Disu
Ma
Ef
at m
Pl
ng
Da
biopsies in DMD have revealed reduced or missing nitric
ni
Limb Girdle MD
rk
ai
Tr
wo FSH MD
er
oxide synthase, necessary for sufficient nitric oxide levels.55 Ov
Moderate
Nitric oxide normally limits vasoconstriction in muscles dur-
ing and after exercise and also provides cytoprotection and Kugelberg-
antiinflammation in muscle tissue. Muscle fatigue in DMD Welander
Severe
Disease
may thus be exacerbated by ischemic exercise.55 However, 0 Late
Effective Extraordinary
aerobic training in other muscular diseases has shown indi- Bed Rest Daily Activities Mild
Training Training Exercise
DMD
cations of positive effect on aerobic capacity as well as

measures of activities and participation,293 so generalization Normal Exercise Correlates
to DMD has a possible rationale. In addition, strengthening Figure 17-12 n Idealized response of normal and impaired
exercises in combination with aerobic exercises in other muscle to exercise. The therapeutic window of safe exercise nar-
muscle disorders have been shown to have a likely positive rows progressively. Activities (lower X axis) causing normal
effect.293 exercise effects in normal muscle (upper X axis) correlate with
Overall, the data from animal and human studies suggest different effects in impaired muscle. (From Coble NO, Maloney
that submaximal exercise is not harmful and it may be help- FP: Effects of exercise on neuromuscular disease. In Maloney FP,
ful in maintaining maximal function if the patient does not Burks JS, Ringel SP, editors: Interdisciplinary rehabilitation of
exercise into marked fatigue. Because muscle endurance multiple sclerosis and neuromuscular disorders, New York, 1985,
and peak power are diminished in addition to muscle JB Lippincott.)
strength, a focus on program design related to functional
exercises individualized to each child’s functional require-
ments is recommended.274
Ideally, the child’s exercise can be incorporated into plea- children gradually discontinue walking about a year after
surable activities adapted for children with movement and they lose their ability to deal with stairs or when daily am-
weakness-related balance problems. Many ambulatory chil- bulation time decreases to less than 30 minutes per day.212
dren enjoy ball activities, walking-based simple obstacle Toward the end of the child’s independent walking stage, he
courses, parachute games, table tennis, cycling (preferably has a marked anterior pelvic tilt with lordosis and a protu-
tandem), and especially swimming. Swimming is an excellent berant abdomen. His shoulders are retracted and he may
exercise for children with DMD because they often are quite hold his hands behind his hips or elevated in a mid-guard
buoyant because of their increased fat/muscle ratio. Many position to stabilize his hips. He has a severe waddling gait
children can continue to float or swim independently on their with a shortened stride, and he must carefully lock his knees
backs even when nonambulatory (if supervised) and able at each step. He falls frequently, which may result in frac-
to move only distal musculature. The Muscular Dystrophy tures of the lower or upper extremities.
Association has an excellent guide to water-based exercises: If the child and his family have followed an aggressive
“No Sweat Exercise: Aquatics.”301 ROM, positioning, and activity program, the child’s walking
A safe indicator of extent and intensity of exercise is that time may be extended by months. In most cases, however,
the patient should recover from exercise fatigue after a the contractures from muscle imbalance continue relent-
night’s rest. When designing an active play program, thera- lessly and the child begins to need support when walking.262
pists should review the types of muscle contractions that the When contractures at the hip, knee, and ankle show evi-
activity requires, considering that possible muscle damage dence of interfering with the child’s ability to stabilize each
occurs when muscles are active and functioning in an joint during stance, most children are referred for surgery to
eccentric manner.302 Concerns about damage from eccentric restore functional joint motion. Figure 17-13 shows the
muscle contractions were supported in animal studies in typical walking pattern of a boy with DMD who is being
which dystrophic muscles were found to be more susceptible considered for release of contractures and bracing.
to stretch-induced muscle damage.303 Figure 17-12 shows Bracing either before or after surgery may be indicated to
responses of normal and impaired muscle to exercise. (See assist with positioning and stabilizing joints for function.
Eagle’s report on exercise in neuromuscular diseases.304) In Ideally, bilateral KAFOs should be measured and fitted in
a summary of findings on effects of physical exercise on final form before surgery to release contractures so the child
conditioning in muscular dystrophy, Ansved305 found that can begin upright weight bearing in the KAFO the day after
the scientific basis for clear recommendations on exercise surgery. KAFOs are commonly fabricated of molded plastic
prescription is poor, but evidence does show the importance thigh units (ischial weight-bearing quadrilateral socket) with
of maintaining an active lifestyle with limitations on high- metal joints at the knee (drop locks) and ankle (or a flexible
resistance and eccentric training activities.305 plastic ankle component) (Figure 17-14).306 If the orthoses
Maintenance of Ambulation. As DMD progresses, are not immediately available, the child can begin the stand-
the child’s posture (a result of both weakness and contrac- ing program in long-leg casts. Casting must be kept to a
ture) and gait pattern abnormalities become extreme and he minimum because of the risk of disuse atrophy in immobi-
must work harder to maintain balance while walking. Most lized muscles. (See Grossman and colleagues307 for a review
of the effect of immobilization on normal muscle and

appropriate therapy interventions.)
In the hospital, standing in bilateral KAFOs can be initi-
ated on a tilt table. Most children are fearful after surgery
and report significant pain when their legs are moved or if
they are placed upright. For therapy to be successful during
this early standing stage and during passive ROM exercises,
the child must have adequate pain medication. If the child is
not properly medicated in the first few days after surgery, the
therapist may have to deal with difficult, resistant behaviors
of the child that persist long after the pain should have sub-
sided. Pain protocols must be discussed before the child’s
surgical procedures. The child should be medicated at least
30 minutes before the therapist’s visit.
Gait training is usually begun within 48 hours after sur-
gery. Initial work focuses on helping the child regain his
sense of standing balance because his old patterns of equi-
nus, lordosis, and shoulder retraction may no longer be
adaptive. The child should be allowed to find his own best
center of balance, and he should be allowed to use compen-
satory gait deviations necessary to allow the best mobility
and stability. Depending on the child’s upper-extremity
strength and control, he may progress from parallel bars for
balance assist, to pushing a wheelchair or weighted walker,
to balance assist from a therapist with a safety strap to pre-
vent falls. Some children who seem to need a walker for
balance transition do best if they use a walker with forearm
Figure 17-13 n Typical walking pattern of a boy with Duchenne rests and vertical hand grips, which seem to help them sta-
muscular dystrophy who is being considered for release of contrac- bilize their arms more effectively than a standard walker.
tures and bracing. Fortunately, most children do learn to walk independently
without support again after surgery, although they are
unable to negotiate steps or inclines or rise from the floor
independently.308 Hyde and colleagues306 report that 24 of
30 boys treated with KAFOs were able to achieve functional
ambulation again. Vignos and colleagues309 report in a re-
view of long-term treatment of DMD that a combination of
operative procedures, orthotics, stretching, and a program of
standing and walking resulted in extended walking until a
mean age of 13.6 years and standing for 2 years after that.
With the early use of surgery and bracing procedures to
maintain ambulation, the expected deterioration in muscle
strength and function as a result of becoming sedentary in a
wheelchair is deferred.310
Because most children with DMD are discharged home
within a few days of surgery, PTs must provide options for
continuing standing within the home. Standing frames are
often available through the child’s school district or therapy
unit. If they are not, the therapist can help the family build a
simple standing frame for home. This frame often can be
made from a piece of plywood, or a gluteal strap system can
be attached to a table at home. If possible the child should
be positioned just forward of the line of gravity to encourage
back extension with facet stability and to allow the child
better head control in the presence of weak anterior neck
muscles. Use of swivel walkers has been recommended by
some therapists and physicians because the child does not
need upper-extremity control for support. Although the con-
cept of hands-free walking seems logical, boys with DMD
had more difficulty using the walkers compared with chil-
Figure 17-14 n Example of boy walking in knee-ankle-foot dren with paraplegia because of the more delicate postural
orthoses showing ischial weight-bearing quadrilateral socket, knee adjustments needed by children with dystrophy and their
drop locks, and plastic ankle component. greater sensitivity to the motion restriction of the swivel
walker. In addition, older children with DMD are seldom progression takes considerable thought. Therapists, the
willing to wear externally visible bracing outside the home patient, and the parents or caregivers must review environ-
or school system. Some therapists have reported success mental constraints, access issues, social goals, and work and
with the ORLAU variable center of gravity swivel walker recreational needs.
(Mopac Ltd., Eau Claire, Wisconsin)311; however, support Because of the problems associated with increased
for its use is not widespread. wheelchair use, the therapist must work closely with the
Bakker and colleagues312 reviewed the literature on the family and any school-based personnel to design a realistic
effectiveness of treatment with surgery and KAFOs. They plan to prevent rapid deterioration in strength and indepen-
found that the scientific strength of the studies was poor. dent function. If possible, the child’s standing program in
Although the treatment approach seemed to prolong the KAFOs should be continued at school and at home as long
walking time, whether it extended functional walking was as possible, with a goal of 3 to 5 hours of standing per day.
not clear. The children who benefited most were highly With mainstreaming, however, continuing a standing pro-
motivated and had slower rates of deterioration. gram at school is sometimes difficult because attendants and
Transition to Wheelchair. Although surgical and equipment are not available, the child may need to move
orthotic interventions may prolong ambulation within the from room to room for different classes, and the child may
home and classroom past the predicted time for cessation of not like being singled out for special treatment. It is helpful
independent walking (8 to 12 years), most children begin to to caregivers if the child continues to wear his KAFOs when
use a wheelchair for community mobility and long distances using the chair until he is totally dependent for transfers and
before this time. When children begin to spend more time in can no longer be pivoted from the chair to another surface.
their chair, the rate of development of contractures, disuse If the child uses a motorized wheelchair, directional con-
weakness, and obesity increases.249,306 Because of this more trol systems must be adapted to each child’s needs. Most
rapid deterioration in the child’s functional skills, profes- young people with advanced DMD do well for years with a
sionals and parents often discourage the child from using a standard joystick hand control system; however, because of
chair for mobility. Children, however, tend to welcome use extended survival times relative to the long-term use of me-
of the chair because they have more energy for their social chanical ventilation, many patients must have their control
interactions and learning tasks.306 systems adjusted frequently to minimize the need for muscle
Selection of the appropriate wheelchair is often difficult control, such as pinch strength. The need for ventilation sup-
for the patient and family because of the multiple decisions port while using the wheelchair does not seem to interfere
that must be made. Few children with DMD can propel a with the ability to drive.314 (See Cooper315 for a comprehen-
manual wheelchair for more than a few years because of sive manual on wheelchair selection. This information is
their increasing upper-extremity weakness. In addition, their equally valuable for patients with ALS and GBS.)
propulsion speed in their manual chair is seldom adequate to When the child can no longer tolerate the sitting position,
keep up with their peers. Eventually, the child will need a some children have continued to attend school on a gurney.
motorized chair. Although this provides tremendous free- Once the person with DMD is no longer able to attend
dom for the child, a motorized chair presents problems to school or work, the home environment will need to be
many families because transporting the chair requires a van adapted for maximal self-direction despite significant physi-
and lift unit, which is seldom funded by insurance. Ideally, cal dependence. Both low- and high-tech environmental
the child should have both a manual and a motorized chair; control systems are more readily available today than they
however, in today’s health policy climate, parents or advo- were 10 years ago. Television control units, voice-activated
cates often must engage in protracted efforts to obtain adap- telephones, switch-activated bed controls, and page turners are
tive equipment for the patient. among the low-tech systems. Sip-and-puff, blink-operated,
An important consideration when purchasing a wheel- and voice-activated control units can be adapted to operate
chair is the trunk support system. Traditionally, boys with most electronic devices. OTs and PTs can provide invalu-
DMD are thought to develop a gravity collapse of the spine able support to the person with DMD and the caregivers by
related to their functional sitting posture. To control the col- making several home visits to suggest modifications and
lapsing spine, spinal orthoses and seat inserts to lock the adaptive devices and systems. (See Cook and Hussey114 for
spine in extension (to prevent lateral bending and rotation) detailed information on assistive technology systems. Also
are frequently recommended. Unfortunately, the effective- see an excellent website for home automation, environmen-
ness of positioning devices to control the development of tal control, and electronic aids for daily living [EADLs]:
scoliosis has been disappointing.243,244 The therapist there- www.makoa.org/ecu.htm.)
fore should work with the child, the family, and the orthope-
dist to determine the best system to maintain optimal spinal Psychosocial Issues
alignment and trunk stability as the child weakens. In addi- Psychosocial issues related to DMD are family issues.
tion, as the child becomes more physically dependent, the At the time of the child’s diagnosis, the parents are often
chair may need to be fitted with a pressure-relief molded seat emotionally devastated and cycle back and forth through
and trunk cushions, elevating leg rests, and a reclining back many phases of denial, anger, sadness, and active coping,
with a head rest.313 The Tilt-in-Space chair (LABAC Sys- especially if they feel guilt that they “caused” their child’s
tems, Denver) is a good example of a chair that can be mo- disease. This process tends to recur when the child does
torized to allow mobility as well as maximal adjustment of not meet expected normal physical and social milestones
seat position by using mouth control systems. It can also be or when he reaches predicted stages of deterioration, such
adapted for a respirator attachment. The decision about the as the transition to a wheelchair. Because children with
type of power chair necessary in the later stages of disease DMD have concomitant developmental and cognitive
delays or issues, educational and social interactions can be emergency that might occur to ensure that the patient’s de-
compromised in addition to the physical changes. Because sires are respected.214,318
DMD is a multisystem multiprocess disease, early in the Because of the extended life opportunities for DMD pa-
child’s life the family should be guided to encourage the tients who may now live into their 20s, home care require-
child’s independence and to discourage overprotection.316 ments, the impact of in-home care on family members, and
Therapists can play an important role in helping the child the financial impact must be fully reviewed and support
and family identify realistic goals for independence. In systems put in place before caregiving stress becomes over-
addition, therapists can be instrumental in extending inde- whelming. Positive family functioning while caring for a
pendence and a sense of self-direction by anticipating dependent child or adult with DMD is correlated with care-
patient needs for adaptive equipment and identifying giver health and hardiness and requires multiple levels of
appropriate assistive devices and environmental control family support from family, friends, and professionals.319
systems that empower the person with DMD and provide Increasingly, young men with DMD are attending college
relief for caregivers from the constant attention required by even though they may require 24-hour assistance with ADLs
a completely dependent person. Key to family support is and monitoring of ventilation equipment. To date, parents
access to a multidisciplinary clinic with specialists in neu- are providing most of the care to their children with DMD
rology, pulmonology, orthopedics, rehabilitation services, by attending colleges or living in dorms or apartments with
psychology, social work, and dietetics. Only through com- their child. With life extended with ventilation, parents and
prehensive clinics do families of children and adults with the young person with DMD should begin early to plan for
DMD receive the level of education and support necessary a future with maximal decision making by the young adult
to deal with the changing levels of function and demands with DMD. This mindset of a “future” requires considerable
on family systems.214 problem solving by all people involved in the care of the
Psychosocial support should be made available to the young adult. Parents of children with DMD should involve
child and family during predictable times of crisis. Major their child early in life to make appropriate decisions about
times of crisis occur around the age of 5 years when the care, learn about medical needs and practices, and deal with
child begins to realize his differences, at age 8 to 12 years finances necessary to run a home or hire an attendant. These
when the child loses the ability to walk independently, dur- issues related to independence (even though physically de-
ing the adolescent years when social interactions become pendent) and caregivers are now being discussed by patients
restricted, and around the time of high school graduation with DMD and their caregivers.320
when the child and family must face vocational limitations Parents and the child should be given the opportunity to
and almost certain death within the next decade.317 Transi- discuss the impending death in an accepting environment
tion times are often accompanied by depression, with- with persons who are experienced in dealing with degenera-
drawal, and anxiety in the child and family members be- tive diseases. Because the child and family have long antici-
cause parents had a marked preoccupation with their sons pated the child’s death and have made transitions through
and a diminished expression of enjoyment.313 Predictably, many levels of grieving, the process of separation and
the integrity, strength, and intragenerational and intergen- mourning may have occurred before the child’s death. Each
erational function and coping styles of the child’s family child and family member should therefore be helped to deal
contribute a great deal to the way the family responds to the with the process according to his or her own pace and in
child’s progressive deterioration. Extended periods of anxi- response to individual needs. The child’s death is sometimes
ety and depression should be treated vigorously with cogni- considered a welcome relief.321 This feeling of relief, how-
tive interventions, support groups, respite care, and, when ever, is often accompanied by survivor guilt and a tremen-
appropriate, short-term anxiolytics and antidepressants. Re- dous sense of loss of life focus for the family members
peated opportunities to discuss end-of-life care must be whose lives have been so intertwined with that of the child’s.
given to both the child and parents. Professionals, however, Ideally, arrangements should be made for the family to meet
tend to underestimate the quality of life for patients with with the professionals with whom they feel most comfort-
end-stage DMD; therefore patients and family members able several weeks after the child’s death and again several
must be educated about long-term options for ventilatory months later so that the family (and caregivers) can deal
support or palliative care well ahead of any respiratory with their thoughts and feelings (Case Study 17-3).322,323
CASE STUDY 17-3 n JEREMY

Jeremy was 3 years old when he was diagnosed with DMD. He difficulty rising from the ground and needed to hold on to a
lived at home with his mother and a 5-year-old sister. There railing when stepping up a stair.
was no known family history of DMD, although family lore During a medical history, Jeremy’s mother said that she had
suggested that a cousin died quite young from pneumonia and noticed that he was “slow to develop” but was not worried
a “wasting disease.” Jeremy was referred for a medical evalua- because she thought he was just a “late bloomer.” A muscle
tion when a playground supervisor at his preschool noted that biopsy was positive for a diagnosis of DMD. A physical therapy
he was clumsy when running and that he had difficulty on the evaluation 3 months after diagnosis showed ROM to be within
playground climbing equipment and the slide. He also had normal limits for all joints. Muscle weakness was evident on
CASE STUDY 17-3 n JEREMY—cont’d

MMT with G2 (42) hip abduction and extension and quadriceps On the fourth day, he practiced walking for 10 minutes six
strength bilaterally. Hip flexion, knee flexion, dorsiflexion, and times a day with full physical therapy treatment twice a day.
toe extension were in the G (4) range. Plantar flexion was G1 Because Jeremy was from a rural area and daily physical
(41) with evident hypertrophy. Shoulder abduction and flexion therapy would not be available on discharge, he was kept in the
was in the G (4) range, although the patient had difficulty hospital for 3 additional days for intensive rehabilitation. An
sustaining abduction for more than 5 seconds. OT worked with Jeremy to provide adaptive equipment for
Jeremy had a moderate head lag when moving from supine reaching, self-care, and eating (he was unable to raise his arms
to sitting, because of G2 (42) anterior neck muscles. The above 45 degrees and needed his left arm to assist the right
therapist made an on-site school visit to help the teachers iden- when reaching). He was discharged home on the eighth day.
tify obstacles to Jeremy’s full integration with his classmates. An Elks traveling therapist arranged to visit the family once a
The school custodian built some ramps to help Jeremy use the week for the next month to continue ambulation training and to
playground equipment. guide the mother in a home positioning and ROM program.
Jeremy ambulated independently until age 8 years. His gait The therapist also helped the mother adapt the home environ-
pattern was typical of late-stage ambulation (marked equinus, ment and his school to adjust expectations of Jeremy so he was
knee hyperextension during stance, bilateral Trendelenburg on less prone to falling and excessive fatigue.
stance, marked lordosis with a protuberant abdomen with arms The family was lost to follow-up, but by report Jeremy con-
held posterior to hips). He had 40-degree hip flexion contrac- tinued to ambulate in his KAFOs for approximately 9 months
tures with iliotibial band tightness, no knee contractures, and after surgery, when he chose to use his wheelchair full time. A
25-degree plantar flexion contractures. MMT showed the motorized wheelchair was recommended; however, his mother
expected decrease in strength, with pelvic and shoulder girdle believed that Jeremy was easier to handle in his manual chair.
muscles being weaker than more distal musculature, except The Muscular Dystrophy Association loaned Jeremy a motor-
that the anterior tibialis and the peroneals were F1 (31). He ized wheelchair for school use. He had developed moderate
was unable to rise independently from the floor and needed scoliosis but did not report pain. He refused to wear a molded
assistance with stairs. Because his gait pattern was slow and spinal corset, but the padded thoracic pads fitted to his chair
he needed to rest frequently when walking more than 20 feet increased his comfort. By age 15 years, Jeremy was dependent
at school, Jeremy had been using a manual wheelchair for for all care except feeding. He was able to sit with support in a
long-distance mobility since the age of 7 years. large living room chair and he enjoyed watching television and
On the recommendation of orthopedist consultants, Jeremy playing card games with a few friends who visited his home.
underwent bilateral percutaneous hip flexor lengthening, ilio- He was disinterested in continuing school and missed more
tibial band fasciotomy, and heel cord release. Bilateral KAFOs days than he attended. He was not cooperative with his
had been fitted before surgery, and Jeremy was placed in the home-based teacher.
braces after surgery. No casting was done. Despite his com- During his fifteenth year, Jeremy had repeated episodes
plaints, he was gradually brought to the full weight-bearing of chest congestion and difficulty handling stringy foods. The
standing position by late afternoon on the day after surgery. visiting therapist taught his mother some postural drainage and
Adjustments were made in his pain medication schedule to breathing exercises for Jeremy; however, the mother did not
allow him to tolerate the process more comfortably. By the follow through with the recommendations. Because his mother
third hospital day, Jeremy participated in two therapy sessions had to work full time, a public agency provided in-home care
per day and was standing in the parallel bars, where he was during the days when Jeremy was not at school or after he
taught lateral and anteroposterior weight shifting in preparation returned from school. The mother refused in-home nursing
for ambulation. Active assisted and passive ROM exercises care, preferring to continue with the attendant, who was not
were performed without the KAFOs twice a day. On the fourth comfortable carrying out Jeremy’s exercises or pulmonary care.
hospital day, Jeremy began to take short steps using the parallel The family refused counseling or support from parents of other
bars for balance. His mother was also taught his exercises so children with disabilities. Jeremy died at home after a brief
that Jeremy could have more than two therapy sessions a day. bout with pneumonia.
SUMMARY Therapists must be aware of their own feelings and

In this chapter, discussion of three different diseases reveals reactions to patients with severe neuromuscular diseases.
the varied effects of neuromuscular pathology on a person’s Working with patients with GBS is usually a positive experi-
day-to-day function. ALS is an adult-onset degenerative ence because most patients attain full recovery despite their
disease of the upper and lower motor neurons; GBS is an often severe disability during the acute illness and long
inflammatory process affecting the PNS of children and recovery period. Working with patients with degenerative
adults; and DMD is an inherited degenerative disease mani- terminal diseases, however, draws deeply on the therapist’s
festing in childhood that affects muscle tissue. In all three emotional and spiritual strength. A typical response of health
conditions the therapist must design a therapy program that care professionals is to view these patients’ conditions as
will provide the patient with the impetus to become or re- hopeless and to assume that the patients must also perceive
main as active as possible without causing possible muscle their existence as hopeless, depressing, and without value.
damage from excessive exercise demands or overwork. Research does suggest an increased incidence of depression
and demoralization in patients with degenerative, terminal Because few medical-clinical facilities see a large enough
diseases compared with nonaffected populations. Other sample of patients with any of these three diagnoses,
research, however, has indicated that many patients perceive therapists must align with their professional organizations
their own life satisfaction much more positively than pro- to institute nationwide, multisite research studies to pro-
fessionals would believe.318,324 Therapists must tap into vide clear evidence of effectiveness of therapy in these
patients’ positive energy to design treatment programs that populations.
respect patients’ goals and life plans within the context of
their environment. References
Limited evidence exists to document the effectiveness To enhance this text and add value for the reader, all refer-
of rehabilitation for patients with progressive neurological ences are included on the companion Evolve site that
diseases. Determining the most appropriate exercise and accompanies this textbook. This online service will, when
therapeutic intervention programs therefore requires diligent available, provide a link for the reader to a Medline abstract
examination of the dysfunctions and needs of the indi- for the article cited. There are 326 cited references and other
vidual patient and assessment of the effects of interven- general references for this chapter, with the majority of
tions appropriately adapted from use in other populations. those articles being evidence-based citations.
CHAPTER 18 Beyond the Central Nervous System:
Neurovascular Entrapment Syndromes
BRADLEY W. STOCKERT, PT, PhD, LAURA J. KENNY, PT, OCS, FAAOMPT, and
PETER I. EDGELOW, PT, MS, DPT
OVERVIEW space. There are no lymphatic channels within the endoneu-

The purpose of this chapter is twofold. The first purpose is to rial space. The pressure within the endoneurial space increases
develop the concept that the entire nervous system forms a with compression of the neuron.4
continuous tissue tract. This concept is central to the idea that The second level of organization consists of a collection
movements of the trunk and/or limbs can have a profound of many nerve fibers (a fascicle) surrounded by a layer
biomechanical and physiological impact on the peripheral of connective tissue called the perineurium.2,3 The perineu-
nervous system (PNS) and central nervous system (CNS). rium acts as a selective barrier to diffusion and as such
Mobility of the nervous system and some of the responses of exerts significant control over the local movement of fluid
the system to movement in normal and sensitized states are and ions. This connective tissue layer acts like a pressurized
discussed. container—that is, extrusion of the contents occurs if the
The second purpose of this chapter is to develop in the membrane is cut. The compartment enclosed by the peri-
reader an understanding of neurovascular entrapment syn- neurium does not contain lymphatic channels.4 This may
drome. This is an underrecognized impairment present in be a problem during inflammatory states when edema is
some patients with a wide variety of diagnoses—for example, present deep to the perineurium. The perineurium is the
nonspecific arm pain, repetitive strain injury, carpal tunnel last connective tissue layer to rupture in tensile testing of
syndrome, and thoracic outlet syndrome. Standard medical peripheral nerves.5 The outermost connective tissue layer of
care frequently fails with these patients. A theoretical model a peripheral nerve is called the epineurium. The epineurium
for the development and perpetuation of neurovascular surrounds, protects, and enhances gliding between the fas-
entrapment syndrome is presented. Background information cicles. Lymphatic channels are found within the epineurial
regarding the syndrome is provided, and the appropriate compartment.
screening tools for assessment of the impairment are dis- All three connective tissue layers are interconnected—
cussed. Treatment suggestions and a case study are presented they are not separate and distinct, but continuous tissue lay-
at the end of the chapter. ers.2,3 Each of the connective tissue layers contains free
nerve endings from the nervi nervorum. As a result, all three
PERIPHERAL NEUROANATOMY connective tissue layers are a potential source of pain. In
The PNS is generally regarded as the portion of the nervous addition, all three layers are continuous with the homolo-
system that lies outside the CNS (i.e., the brain and spinal gous connective tissue layers of the CNS—for example, the
cord).1,2 The major components of the PNS include motor, dura mater and the epineurium.
sensory, and autonomic neurons found in spinal, peripheral, The vascular supply for peripheral nerves is designed to
and cranial nerves. Although this partitioning is valid from provide uninterrupted blood flow regardless of the position
an anatomical perspective, it often leads to a lack of appre- of the trunk and limbs. Extrinsic vessels provide blood flow
ciation as to the truly continuous nature and integrative to segmental vessels that in turn supply an extensive intrin-
function of the nervous system as a whole. The concept that sic (intraneural) vasculature within the PNS. These seg-
the entire nervous system is a continuous tissue tract rein- mental vessels branch off of the extrinsic vessels and enter
forces the idea that limb and trunk movements can have a peripheral nerves in areas of low nerve mobility relative to
mechanical effect on the PNS and the CNS that is local and the surrounding tissue. The intrinsic vasculature supplies all
global. three connective tissue layers within the PNS. Arterioles
The nervous system is composed of two functional tissue and venules are found in the epineurial and perineurial
types. One type of tissue is concerned with impulse conduc- spaces, but only capillaries are found in the endoneurial
tion. This functional category includes nerve cells and Schwann compartment.
cells. The second functional tissue type provides support and Peripheral nerves are regularly subjected to compression
protection of the conduction tissues—that is, the connective and elongation (stretching), which have been shown to
tissues. increase intraneural pressure. An increase in intraneural
Three levels in the organization of a peripheral nerve have pressure decreases the diameter of the intrinsic blood ves-
been described2,3 (Figure 18-1). At the innermost level the sels and results in a reduction in blood flow within the nerve.
nerve fiber is the conducting component of a neuron (nerve Compressive forces of 20 to 30 mm Hg have been shown to
cell). A connective tissue layer called the endoneurium sur- adversely affect intraneural blood flow,6 and compressive
rounds each nerve fiber. The endoneurium surrounds the forces of 50 to 70 mm Hg have been shown to result in com-
basement membrane of the neuron and plays an important plete arrest of blood flow7 and cause damage to myelin and
role in maintaining fluid pressure within the endoneurial axons.6 A strain (elongation) of 6% to 8% has been shown
571
Figure 18-1 n Three levels of organization of a peripheral nerve or nerve trunk. A, Nerve trunk
and components. B, Microscopic structure of nerve fiber.
to decrease intraneural blood flow by 50% to 70% in the MOBILITY OF THE PERIPHERAL NERVOUS
sciatic nerve of rats.4,8,9 A strain of 15% in the sciatic nerve SYSTEM
of a rabbit has been shown to result in complete arrest of Several types of tissues (e.g., bone, fascia, and muscle) sur-
blood flow,7 and the same strain produces an 80% reduction round peripheral nerves as they “travel” to target tissues.
in blood flow in the rat sciatic nerve.9 Strains of 11% Peripheral nerves can be thought of as passing through
or greater are produced by some of the positions used in a series of tissue tunnels composed of various biological
neurodynamic tests of the upper limb.6 Significant increases materials. The composition of the tissue tunnel changes with
in intraneural pressure and concomitant decreases in intra- the passage of the nerve from the vertebral column (an osse-
neural blood flow have been shown to adversely affect neu- ous tunnel) to the target tissue—for example, from an osse-
ronal conduction.7,10,11 ous tunnel to a soft tissue and/or fibro-osseous tunnel. A
The cytoplasm in cells moves and has thixotropic “mechanical interface” exists at the junction between the
properties—that is, the viscosity of cytoplasm is lower nerve and the material adjacent to the nerve that forms the
when it is continuously moving.4 In neurons the move- tissue tunnel. Movement of the trunk and/or limbs can cause
ment of the cytoplasm from the cell body through the three types of movement to occur in the peripheral nerves:
axons (anterograde movement) occurs at two speeds. unfolding, sliding, and elongation.15
Fast axoplasmic flow occurs at a rate of about 100 to When there is little or no tension in a peripheral nerve,
400 mm/day and is used to carry ion channels and neu- the axon typically contains undulations (folds). As tension
rotransmitters (i.e., materials required for normal impulse is applied the axon will “unfold” so that the undulations
conduction) to the nerve terminals. Slow axoplasmic flow disappear. “Sliding” can be defined as movement between
occurs at a rate of about 6 mm/day and is used to transport the nerve and the surrounding tissues at the mechanical
cytoskeleton proteins, neurofilaments, and other materials interface (extraneural movement). Sliding by itself does
used to maintain the physical health of the cell. A third not cause significant elongation or tension to develop
flow occurs in the opposite direction (retrograde) at a within the nerve, so intraneural pressure remains relatively
rate of about 200 mm/day. Retrograde transport carries unchanged. Ultrasound studies have shown that the median,
unused substances and exogenous materials taken up ulnar, sciatic, and tibial nerves undergo extraneural move-
at the terminus—for example, neurotrophic factors. The ment (sliding) with movement of the upper and lower
material carried back to the cell body by retrograde trans- limbs, respectively.16-19
port has been shown to influence activity in the cell “Elongation” of the nerve occurs when tension is applied
nucleus.4 to a nerve and there is little or no unfolding and sliding at
Compression raises intraneural pressure, which has a the mechanical interface. Elongation causes movement to
negative impact on the flow of cytoplasm.4 Anterograde and occur between the neural elements and connective tissue
retrograde flow of axoplasm is impaired with 30 mm Hg layers (intraneural movement). Elongation decreases the
compression on the nerve, hypoxia, or a strain of 11% or diameter of the nerve, resulting in an increase in the intra-
greater.12-14 Prolonged or intense exposure to compression neural tension and pressure.15 An increase in intraneural
can result in conduction abnormalities, endoneurial edema, pressure has been shown to decrease the flow of blood and
fibrin deposition, demyelination, and axonal sprouting. Each axoplasm, resulting in altered neural function (see previous
of these events increases the likelihood of developing adhe- section). Elongation within the median and ulnar nerves has
sions and abnormal impulse-generating sites (AIGSs).4 (The been shown to occur with movements of the upper limb.6
negative impact of AIGSs is discussed in the section on Both extraneural and intraneural movements may occur
adaptive responses to pain.) simultaneously within a nerve, but they may not be uniformly
CHAPTER 18 n Beyond the Central Nervous System: Neurovascular Entrapment Syndromes 573
distributed. When a body moves, some parts of the PNS will pain, inflammation, proliferation of fibroblasts, and scar
undergo primarily extraneural movement (sliding) with little formation (fibrosis). Ultrasound studies have shown that
or no development of tension while other areas undergo intra- the median nerve in patients with carpal tunnel syndrome is
neural movement (elongation) that results in an increase enlarged approximately 30%.24 An intraneural scar decreases
in intraneural tension and pressure. As a consequence, some the compliance of the nerve and increases the amount
areas within a nerve slide, developing little or no tension, of intraneural pressure and tension generated with elonga-
whereas other areas of the same nerve elongate significantly, tion.21 Intraneural lesions can impair or completely block
increasing the amount of intraneural tension.6 In areas repeat- the ability of the nerve to conduct action potentials.2,15,25
edly exposed to high amounts of tension, for example, the Partial or complete conduction blocks can result in abnor-
median nerve at the wrist, the nerves are found to contain a mal sensation, loss of motor function, autonomic dysfunc-
higher-than-average amount of connective tissue.15 tion, and atrophy of target tissue, for example, muscle and/
If one considers the entire nervous system as a continu- or skin.
ous tissue tract, then the idea that movement and/or tension Microtrauma can produce an extraneural lesion.2,15,21,25
developed in one region of the nervous system can be dis- The damage in an extraneural lesion occurs in the tissue sur-
tributed and dissipated throughout the entire nervous system rounding the nerve or at the mechanical interface. Swelling
becomes apparent.20,21 The inability of a component within within the tissue tunnel can produce compression of the
the nervous system to dissipate and/or distribute movement nerve. Fibrosis can produce adhesions at the mechanical
and tension can lead to abnormal force development and interface leading to a decrease in sliding of the nerve.
lesions elsewhere in the continuous tissue tract.22 A decrease in the ability of a nerve to slide within a tissue
tunnel will result in an abnormal increase in intraneural
PERIPHERAL NERVE ENTRAPMENT tension and pressure as movement is imposed on the nerve.
Seddon’s classification of nerve injury is based upon me- The increase in local intraneural tension can produce abnor-
chanical trauma.23 Schaumberg2 modified this paradigm mal changes in the conduction of action potentials, and the
into an anatomically based scheme containing three tension will be distributed in an aberrant pattern throughout
classes of injury (Table 18-1). Injuries in class II and III the continuous tract of the nervous system. The resultant
are caused by macrotrauma that results in some disruption abnormal distribution of tension predisposes the nervous
to the integrity of the nerve fiber. The following discus- system to the development of lesions at other sites.15
sion of entrapment is focused on microtrauma in which Friction, compression, and tension can produce micro-
there is no breach in the anatomical integrity of the nerve trauma that results in intraneural and extraneural pathology.2,15
fiber (class I). Mechanical microtrauma resulting in nerve For example, fibrosis can produce a combined pathological
entrapment can occur with excessive or abnormal friction, state that results in a substantial reduction in the ability of a
compression, and/or tension (elongation).2 nerve to slide within the tissue tunnel and a substantial in-
Tissue tunnels, peripheral nerves, and the mechanical crease in intraneural tension during nerve elongation as the
interfaces between them are all vulnerable to mechanical compliance of the nerve is decreased. Movement of the me-
microtrauma—that is, abnormal friction, compression, and/ dian nerve at the carpal tunnel has been shown to occur with
or tension.2,15 Some peripheral nerves are exposed to bony movements of the upper limb.16-19 Longitudinal and transverse
hard interfaces, for example, the lower cords of the brachial movements of the median nerve at the carpal tunnel have been
plexus at the first rib, which are potential sources of abnormal shown to be reduced in the presence of microtrauma, that is,
friction. Inflammation and swelling within a tissue tunnel can carpal tunnel syndrome,24,26 nonspecific arm pain,27,28 and
produce compression of a nerve, for example, the median whiplash injury.28
nerve within the carpal tunnel. The point at which a nerve Intraneural and extraneural lesions result in an abnormal
branches limits the amount of gliding (extraneural movement) distribution of sliding and tension throughout the nervous
available at that location and increases the amount of local system with movement of the trunk and/or limbs. The
intraneural tension developed with movement, for example, abnormal distribution of tension within a nerve increases the
the tibial nerve in the popliteal fossa.2,15 probability of a second lesion or abnormality developing
Microtrauma can produce an intraneural lesion that within the nerve. This situation led Upton and McComas29
causes a decrease in intraneural flow of blood and axoplasm, in 1973 to first use the term “double crush injury.” (This
demyelination, and/or conduction defects.2,15 If the lesion term should be considered a misnomer because a “crush”
occurs in the connective tissues of the nerve, there may be does not necessarily occur.) For example, entrapment of the
TABLE 18-1 n CLASSIFICATION OF ACUTE TRAUMATIC PERIPHERAL NERVE INJURY
ANATOMICAL
CLASSIFICATION CLASS I CLASS II CLASS III
Previous nomenclature Neuropraxia Axonotmesis Neurotmesis
Lesion Reversible conduction block Axonal interruption but basal Nerve fiber and basal lamina
resulting from ischemia or lamina remains intact interruption (complete nerve
demyelination severance)
Modified from Schaumberg HH, Spencer PS, Thomas PK: Disorders of peripheral nerves, Philadelphia, 1983, FA Davis.
median nerve at the carpal tunnel can cause the development P and calcitonin gene–related peptide,4 which in turn cause
of abnormal tension in cervical spinal nerves, resulting in a mast cells to release histamine and serotonin.35,36 Together
lesion at that site. Upton and McComas29 have shown that these chemical mediators augment the inflammatory state
a lesion at the carpal tunnel increases the risk of having a and cause the endothelial cells of capillaries to further dete-
second neural lesion in the cervical region. riorate by becoming flatter, larger, and leakier, enhancing
exudation and edema.
PATHOGENESIS OF NEUROVASCULAR Deterioration of the capillary endothelium results in exu-
ENTRAPMENT dation and the formation of a protein-rich edema in the inter-
Neurovascular entrapment can occur at any point along the stitial space. Protein-rich edema stimulates proliferation of
continuous tract of the nervous system. The carpal tunnel, a fibroblasts, resulting in fibrosis, and intensifies the abnormal
common site of entrapment, has been studied and provides a pressure gradients, resulting in more tissue hypoxia—that is,
framework of information regarding the pathogenesis of neu- a positive feedback or self-perpetuating cycle of pathology is
rovascular entrapments. Sunderland30 has reasoned that a initiated. Intraneural fibrosis decreases compliance of the
change in the normal pressure gradients within the carpal nerve, and extraneural fibrosis results in the formation of
tunnel can lead to compression of the median nerve. In order adhesions at the mechanical interface between the nerve and
to maintain homeostasis in the carpal tunnel and the median tissue tunnel. Fibrosis causes a nerve to become stiffer and
nerve, blood must flow into the tunnel, then into the nerve less mobile, resulting in an abnormal increase in tension
and back out of the tunnel. For normal blood flow to occur in when movement is imposed on the nerve.
the median nerve the blood pressure must be highest within The set of circumstances described earlier may be referred
the epineurial arterioles and becomes progressively lower to as a neurovascular entrapment syndrome, and it has the
in the capillaries and epineurial venules and lowest within potential to cause the development of problems elsewhere in
the extraneural space of the carpal tunnel. Any increase in the the system—that is, a double crush injury (see previous sec-
pressure of a single compartment has the potential to disrupt tion). Upton and McComas29 studied 115 subjects with carpal
the normal pressure gradients and impair the flow of blood tunnel syndrome or ulnar impingement at the elbow. They
within the compartments of the carpal tunnel and median found that 81 of the 115 subjects also had evidence of a neural
nerve. Impaired intraneural blood flow can lead to localized lesion at the neck. Because all nerves essentially travel within
hypoxia, edema, inflammation, and fibrosis.30 tissue tunnels, the potential exists for this scenario to occur
An increase in pressure within the carpal tunnel can elsewhere in the continuous tissue tract of the nervous system,
occur for a variety of reasons, for example, synovial hyper- for example, the capsule of the dorsal root ganglion and the
plasia, thickening of tendons, venous congestion, inflam- thoracic outlet.5,15,37
mation, and/or edema. Venous blood flow within a nerve
will be impaired and venous stasis will develop if pressure ADAPTIVE RESPONSES TO PAIN
within the extraneural space of the carpal tunnel becomes A thorough discussion of the pain associated with neuro-
greater than the pressure within the epineurial venules. vascular entrapment is beyond the scope and intent of
Because blood pressure within venules is relatively low, this chapter. The topic of pain management is discussed
partial occlusion of blood flow can begin to occur with in Chapter 32 of this book. However, we would like to
pressures as low as 20 to 30 mm Hg.15,31 describe the development of hyperexcitable states and
The pressure within the carpal tunnel is normally about AIGSs in neurons as well as their role in the development
3 mm Hg with the wrist in a neutral position.4 The pressure of pain associated with neurovascular entrapment.
can rise to over 30 mm Hg when the wrist is placed in “Normal” or physiological pain occurs when peripheral
90 degrees of extension32 or with the functional task of using nociceptors are subjected to a stimulus that is at or above the
a computer mouse to drag or point at an object.33 Studies threshold for firing. “Abnormal” or pathological pain can
have shown that the pressure within the carpal tunnel in occur when there is a change in the sensitivity (threshold) of
someone with carpal tunnel syndrome can be 30 mm Hg, the somatosensory system.38 Devor39 wrote that “the crucial
or more, with the wrist in neutral and can increase to about pathophysiological process triggered by nerve injury is an
100 mm Hg when the wrist is in 90 degrees of flexion4,12,13 increase in neuronal excitability.”
or extension.12 Compressive forces of 20 to 30 mm Hg have Neurons that become inflamed, hypoxic, and/or demy-
been shown to adversely affect intraneural blood flow,6 elinated can enter a hyperexcitable state.2,39-47 A neuron
whereas compressive forces of 50 to 70 mm Hg have been in a hyperexcitable state can begin to discharge spontane-
shown to result in complete arrest of blood flow7 and cause ously and/or develop a sustained rhythmic discharge after
demonstrable damage to myelin and axons.6 Motor and sen- stimulation. In addition, hyperexcitable neurons can develop
sory abnormalities begin to manifest at about 40 mm Hg, mechanosensitivity,41 chemosensitivity,4 and/or thermal
and complete blockade of the median nerve has been shown sensitivity,45 all of which can result in the production
to occur at 50 mm Hg.34 The pressure found in the carpal of allodynia, a form of pathological pain.* These changes
tunnel of people with carpal tunnel syndrome is clearly in the behavior of a nerve can occur in the absence of de-
adequate to disrupt the normal flow of blood, axoplasm, and tectable degeneration.40-42 The changes in impulse genera-
action potentials within the median nerve, causing severe tion and neuronal sensitivity are characteristics of an
impairment to normal nerve functions. AIGS.4 A hyperexcitable state and an AIGS can develop
Sunderland30 proposed that venous congestion or stasis with the mechanical microtrauma and inflammation often
within the carpal tunnel will lead to localized hypoxia,
edema, and fibrosis. Hypoxia causes capillary endothelial
cells to deteriorate and local C fibers to secrete substance *References 28, 39, 43, 45, 47, 48.
associated with peripheral nerve pathology, for example, patients with neurovascular entrapment may enhance the
compression, tension, and friction.2,39,48,49 A variety of effectiveness of their treatment.
chemical mediators have been implicated in the develop-
ment of a hyperexcitable state in a neuron—for example, CLINICAL EXAMINATION AND TREATMENT
neurotrophins,50,51 histamine,52 and other inflammatory OF NEUROVASCULAR ENTRAPMENT
mediators,53 which are thought to act through changes in For an effective evaluation of a patient with a neurovascular
gene expression,45,50,51 changes in voltage gated sodium entrapment problem, the whole person must be addressed
channel expression,45 and a reduction in anterograde axo- and involved in the evaluation and treatment processes. This
plasmic transport.44,52 philosophy requires the therapist to become the evaluator,
The dorsal root ganglion appears to play a significant role teacher, and guide for the patient. Wherever possible the
in the pain associated with peripheral nerve pathology.38,41 testing procedures should be performed by the patient so
Mechanical microtrauma to and inflammation of peripheral that he or she can learn to self-assess his or her status before
nerves can cause the dorsal root ganglion to become hyperex- and after treatment procedures. This self-assessment gives
citable (sensitized).41 The change in sensitivity allows what the patient control, thus decreasing the fear of movement or
were weak, subthreshold stimuli to evoke pain and supra- reinjury. In some cases, if a therapist uses his or her hands it
threshold stimuli to evoke exaggerated pain (hyperalgesia). In may be detrimental to the patient in a lifelong sense if it
addition, the dorsal root ganglion can develop mechanosensi- leads to dependence. The concept of the patient gaining
tivity, chemosensitivity, and thermal sensitivity, resulting control of the problem(s) is fundamental and must be inte-
in allodynia.45 This change in sensitivity reflects a change in grated into the initial patient contact for development of
the physiology of the nerve and may be a component in an effective self-management approach. Without an effective
the development of enhanced central sensitivity to pain and self-management strategy, the patient is at risk for recurrent
development of a chronic pain state.38 problems and development of a chronic condition.
As noted previously, the PNS and CNS represent a con- The Edgelow protocol for examination and treatment of
tinuous tissue tract. The pain and symptoms associated with neurovascular entrapment challenges the traditional muscu-
musculoskeletal injury and/or peripheral nerve pathology loskeletal paradigm by placing the primary emphasis on
can include changes that are the result of an alteration in the the response of the neurovascular and neuromotor systems
autonomic nervous system, which is considered part of the to injury.62-64 The standard musculoskeletal evaluation
continuous tissue tract of the nervous system.2,54 For exam- centered on a biomechanical model of the musculoskeletal
ple, catecholamines do not normally elicit pain. However, and nervous systems is adequate for patients with straight
if a nerve is injured or if there is local inflammation, the forward symptoms that appear to be of biomechanical ori-
catecholamines can induce pain (chemosensitivity) and they gin. However, a biomechanical approach is inappropriate for
can maintain or enhance pain in inflamed tissues.4 patients with severe or irritable signs and symptoms that
Some patients who are treated for musculoskeletal inju- may be neurological or vascular in origin. Patients with
ries have signs that may be related to autonomic dysre- neurovascular entrapment often have severe, irritable symp-
flexia.37 Wyke55 demonstrated that stimulation of nociceptors toms. First a subjective evaluation is conducted in a patient
in spinal joints resulted in reflex changes in the cardiovascu- with a potential neurovascular entrapment problem to deter-
lar, respiratory, and endocrine systems. Dysregulated breath- mine how the objective examination should proceed. The
ing has been documented in patients with chronic pain.56 history of the condition is discussed with the patient. Key
Patients with nonspecific arm pain have a reduced sympa- components that should be discussed include history of
thetic vasoconstrictor response in the hand of the affected trauma, repetitive activities, sustained static or tension pos-
limb.57 Thermal asymmetry has been documented in the tures, such as computer keyboard work, or physical activi-
hands of patients with neurogenic thoracic outlet syndrome.58 ties performed with a high level of cognitive demand, as
Feinstein59,60 has shown that injecting saline solution into seen in a pianist. The history should include a discussion of
the thoracic paraspinal muscles caused pallor, diaphoresis, general health, including any potentially relevant medical
bradycardia, and a drop in the blood pressure. These cardio- conditions (e.g., asthma, diabetes, hypothyroidism). Phase I
vascular and respiratory changes are often associated with of differential diagnosis (medical screening) should be com-
an alteration in the output from the autonomic nervous pleted to ensure that the patient is appropriate for evaluation
system.37,54,61 and intervention. (See Chapter 7 on medical screening.)
In patients with cumulative trauma disorder (CTD), A discussion of the patient’s symptoms and complaints
signs of abnormal autonomic nervous system output can should include questions that determine whether the neural
include (1) vasomotor reflexes leading to cool, pale skin,57 or vascular system is a potential source of the problem.
(2) changes in the pattern of sweating (hypohidrosis and/or Symptoms relevant to the potential problem of neurovascu-
hyperhidrosis), (3) trophic changes in the skin, (4) hyperac- lar entrapment include complaints of fullness in the upper
tive flexor withdrawal reflexes, and/or (5) paradoxical extremity; a feeling of swelling, tingling, pain, coldness, or
breathing patterns.37 Edgelow has described paradoxical numbness; or dropping things. In addition, the progression
breathing as the predominant use of the scalene muscles for of the symptoms or complaints and the level of irritability
ventilation during quiet breathing versus normal ventila- should be determined. If pain is a major factor, then a func-
tion, which is predominantly a function of the diaphragm.37 tional pain questionnaire should be completed (see Chapter
Edgelow found that paradoxical breathing is present in 32 on pain management). Motor changes of relevance to the
most patients with CTD of the upper extremity.37 A better potential problem of neurovascular entrapment include
appreciation of the contribution of the autonomic nervous complaints of dropping things, weakness, or an inability
system to the pathology and symptoms present in some to perform motor tasks that were done previously without
difficulty. The level of neural irritability and the presence of end feel. In patients with neurovascular entrapment, this
peripheral or central sensitization should be determined by procedure may evoke a significant flare and worsening of
asking the patient what activities aggravate and ease the symptoms. In patients with neurovascular entrapment the
symptoms. When an extended period of time is required for “feel” of involuntary muscle tension can be the first sign of
symptoms to ease after provocation, irritability may be a abnormality in assessing movement. This tension is often
cause. Sensitization is indicated when minor mechanical or subtle and may occur earlier in the range of motion than
normally nonnoxious stimuli, such as clothing on the skin, where traditional biomechanical symptoms or the end feel
provoke pain. Vascular complaints relevant to the potential normally occurs.65 Moving into the range of motion to the
problem of neurovascular entrapment include complaints of initial onset of tension minimizes the risk of provoking
fullness, swelling, abnormal skin color, or cool skin tem- adverse neurological or vascular consequences. In patients
peratures. A change in the vascular symptoms with a change with suspected neurovascular entrapment who have symp-
in limb position is particularly significant. toms suggestive of neural irritability and sensitization,
In a biomechanical evaluation model the therapist ex- the biomechanical examination and treatment techniques
amines the quantity and quality of active movements and should be modified or deferred until the sensitivity and irri-
determines whether there is pain, spasm, or resistance at an tability of the nervous system are improved. See Table 18-2
n SUGGESTED MODIFICATIONS TO A STANDARD

TABLE 18-2
BIOMECHANICAL EVALUATION
OBSERVATION
Cervical and thoracic: WNL Kyphosis Flat
Scapula: Equal High R/L Low L/R
Lumbar: WNL Lordosis Flat
Hands and feet: swelling, discoloration, other
ACTIVE RANGE OF MOTION (FOR A PATIENT WITH UPPER QUADRANT SYMPTOMS)
Cervical
Flexion: __________degrees causes/increases symptoms
Extension: __________ degrees causes/increases symptoms
Rotation: (R): __________ degrees causes/increases symptoms
Rotation: (L): __________ degrees causes/increases symptoms
Lateral flexion (R): __________ degrees causes/increases symptoms
Lateral flexion (L): __________ degrees causes/increases symptoms
Shoulder Flexion
(R) (with elbow extension): ______ degrees causes/increases symptoms
(L) (with elbow extension): ______ degrees causes/increases symptoms
(R) (with elbow flexion): ______ degrees causes/increases symptoms
(L) (with elbow flexion): ______ degrees causes/increases symptoms
Shoulder Internal Rotation (Reaching behind Back) (Functional Tension Test with
Radial Nerve Bias)
(R) position: causes/increases symptoms
(L) position: causes/increases symptoms
NEURAL EXAMINATION
Passive Neck Flexion
no/yes _________ degrees causes/increases symptoms
Upper Limb Neural Dynamic Test4
(R) position: _________ causes/increases symptoms
(L) position: _________ causes/increases symptoms
Straight Leg Raising Test or Lasegue Test66
Right: _________ degrees causes/increases symptoms
Left: _________ degrees causes/increases symptoms
Tinel Sign66
(Normal = 0; Mild = 1+; Moderate = 2+; Severe = 3+)
Supraclavicular region: Right Left
Elbow: Right Left
Wrist: Median Right Left
Ulnar Right Left
n SUGGESTED MODIFICATIONS TO A STANDARD

TABLE 18-2
BIOMECHANICAL EVALUATION—cont’d
KABAT TESTS71
Strength Tests71
Flexor carpi ulnaris: (R)/5 (L)/5
Adductor pollicis: (R)/5 (L)/5
Thinker Pose71 (Isometric Contraction of Longus Colli) (Temporary Strengthening of the
Flexor Carpi Ulnaris and Adductor Pollicis)
no/yes—Which muscles are affected and by what amount?
VASCULAR INTEGRITY
Temperature of Hands (Ambient Room Temperature)
Right: (index) (digiti minimi)
Left: (index) (digiti minimi)
Adson Test66 (Change in Pulse Pressure)

Right after: 1 minute 2 minutes 3 minutes
Left after: 1 minute 2 minutes 3 minutes
Elevated Arm Stress Test (EAST)66 (Change in Pulse Pressure)
Right after: 1 minute 2 minutes 3 minutes
Left after: 1 minute 2 minutes 3 minutes
SENSATION66
Localization
Stereognosis
Graphesthesia
BREATHING PATTERN (ABILITY TO RELAX THE SCALENE MUSCLES WITH
QUIET BREATHING)
Normal or dysfunctional pattern
PALPATION FINDINGS
(Tenderness: Normal 5 0; Mild 5 11; Moderate 5 21; Severe 5 31)
Scalene muscles: Right: Left:
Subclavius: Right: Left:
Pectoralis minor: Right: Left:
L, Left; R, right; WNL, within normal limits.
for suggested modifications to a standard biomechanical common complaint with neurovascular entrapment of the
evaluation. upper extremity is “I drop things,” yet standard tests of
One component of the examination involves evaluating strength, light touch, and two-point discrimination may
the integrity of the vascular system in the extremities. The have normal results. Therapists often think of this problem
hands or feet should be inspected for discoloration, and as motor until our standard tests fail to demonstrate motor
the skin temperature should be determined in each of the dysfunction. Subtle changes in the somatosensory cortex
peripheral nerve territories present in the affected limb. can occur as a consequence of repetitive motions, particu-
Cool, cyanotic skin can be an indication of arterial insuffi- larly when performed under conditions of intense concen-
ciency or sympathetic dysreflexia in the area, whereas swell- tration or in the presence of pain.67-69 Byl observed severe
ing can be an indication of inflammation and venous or degradation in the representation of the hand in the
lymphatic insufficiency. An Adson test and the elevated arm somatosensory cortex of owl monkeys that were trained in
stress test (EAST) can be used to evaluate vascular integrity a behavior of rapid, active opening and closing of the hand
by determining whether the pulse pressure decreases with a under conditions of high cognitive drive.68 In addition,
change in the position of the limb.66 The Adson test and Byl67 found a significant difference in response on some
EAST should be performed on both upper extremities, and sensory integration and praxis tests in human subjects
the pulse pressure evaluated at 1, 2, and 3 minutes. These with diagnoses of tendinitis and focal dystonia. Byl
tests may be modified or deferred depending on the level of has postulated that similar changes can be identified in
neural irritability found. humans with repetitive strain injuries with the use of
Sensory changes may be subtle and are not always Jean Ayers’s tests of sensory localization, stereognosis,
accompanied by obvious motor dysfunction. The most and graphesthesia.69
An assessment of the patient’s breathing pattern at rest the more traditional musculoskeletal signs, are used as
and palpation of the subclavius, pectoralis minor, and sca- guides in determining the effectiveness of treatment.
lene muscles should be performed. The normal breathing
pattern at rest is primarily diaphragmatic (Figure 18-2). Six Common Signs of Neurovascular Entrapment
However, patients with neurovascular entrapment often 1. Abnormal hand temperature within the following
demonstrate a breathing pattern at rest that relies predomi- parameters:
nantly on the scalene muscles. The scalene breathing pattern a. Cold hands defined as in the 70° F range at rest and
mechanically narrows the thoracic outlet area, thus poten- during activity at the target task
tially perpetuating a neurovascular entrapment syndrome in b. Asymmetry between the temperatures of the second
the area. The scalene breathing pattern may be a sign of digit and the fifth digit, with the fifth digit being
protective posturing. Palpation is used to determine whether colder.70
tenderness or tightness is present. Palpation of the subcla- c. Asymmetry between hands in which there is an abnor-
vius, pectoralis minor, and scalene muscles is significant mal temperature cooling response to diaphragmatic
because of the relationship these muscles have with the sub- breathing, aerobic walking, and repeated use of the
clavian vein, brachial plexus components, and subclavian upper extremities in an activity such as bouncing a
artery, respectively. The results of the palpation should be gymnastic ball.
correlated to the neurological and vascular changes found 2. Abnormal breathing pattern: accessory, chest, or para-
elsewhere in the extremity. doxical rather than diaphragmatic.
3. Abnormal mobility and sensitivity of the nervous system:
Neurovascular Entrapment Examination specifically the dura, the brachial plexus, or the sciatic
There are some common symptom patterns characteristic nerve or sacral plexus.
of neurovascular entrapment that alert the therapist to 4. Cardiovascular deconditioning: patient has a low level of
modify the physical examination. In addition to the symp- endurance and is easily fatigued.
toms mentioned previously, the following patterns help the 5. Sensory dysfunction of the hand at the cortical
therapist recognize a patient with a potentially sensitized level: abnormal tactile localization, graphesthesia, and
nervous system. stereognosis.
6. A positive Kabat71 sign: weakness of the flexor pollicis
Symptom Patterns Characteristic of brevis in the shortened range of adduction that is unilat-
Neurovascular Entrapment eral and reversed with a gentle 30-second isometric con-
1. Symptoms are severe and irritable. traction of the longus colli obtained with the “thinker
2. Function is markedly reduced in the target task (injury- pose” (Figure 18-3).
producing activity) and activities of daily living. This combination of symptoms and signs identifies neu-
3. The patient reports feeling that his or her emotions are in rovascular consequences of the injury. Improvements in
a state of “being out of control.” these signs and symptoms serve as markers that identify
In a modified examination scheme designed to evaluate treatment effectiveness—namely, decrease in pain, improve-
for the presence of a neurovascular entrapment syndrome, ment in function, and a feeling of being more in control.
patients typically have six signs. These signs, in addition to
Figure 18-2 n Diaphragmatic breathing. As the client inhales,

the stomach should rise and the lordosis in the low back should Figure 18-3 n The “thinker pose.” Self-traction is applied by
increase. During exhalation the stomach should fall and the back using gentle upward pressure from one upper extremity onto
should flatten against the floor. the chin.
Neurovascular Entrapment Examination extension; thus position four of the arm self-test can be too
Procedures provocative to use on the affected extremity in a patient with
Neurodynamics of the upper extremity is assessed with severe symptoms. (Please refer to the references at the end
the use of upper limb neural dynamic tests as described by of this chapter for more information on neural dynamic
Butler.4 Passive neck flexion is examined to assess dural tests.)
sensitivity, whereas the straight leg raise test is used to Hand temperature is assessed with an infrared hand-held
assess the sensitivity of the sciatic nerve and sacral plexus. thermometer. Measurements are made of the second digit
In addition to these passive neural dynamic tests performed (innervated by the upper roots of the brachial plexus) and
by the examining therapist, the patient is taught an “arm fifth digit (innervated by the lower roots). Temperature is
self-test” to use as a self-assessment technique. assessed during rest, diaphragmatic breathing, walking on a
The arm self-test is an adaptation of the brachial plexus treadmill, and repeated movements of the upper extremities
tension test. This is an active test that the patient, the medical while a gymnastic ball is being bounced. A normal response
provider, and the physical therapist can use as an indicator of is an increase in temperature in response to these activities.
upper quarter neural sensitivity. The test provides immediate A cooling response is considered abnormal.
feedback regarding the patient’s response to an exercise or Breathing pattern is assessed by palpating the scalene
other form of intervention. The test results can be used as an muscles in the area between the inferior border of the ster-
indicator of a change in patient status. The test is nonspecific, nocleidomastoid and superior to the clavicle. This procedure
meaning that it does not indicate which structure is the source is best done while the patient performs relaxed inhalation.
of the protective tension response. The test is an important tool The scalene muscles are normally quiet during relaxed inha-
that helps the patient recognize and manage symptom flares. lation. Contraction of the scalene muscles and elevation
The arm self-test is one of the self-assessment tools that of the sternum are considered to be abnormal during quiet
encourage the patient to take control of his or her treatment. inhalation. Patients are instructed to breathe with the “belly”
The arm self-test is performed by guiding the standing only (diaphragmatic breathing). If they are unable to do this,
patient through a series of positions using the upper extremity breathing is considered to be paradoxical.
(Figure 18-4). The sequence goes from position zero to five, Cardiovascular fitness is assessed by treadmill walking.
with zero being the position of least general tension on the The patient is instructed to walk at a speed that does not
brachial plexus and five being a position of maximum elon- cause an increase in symptoms for up to 20 minutes. Over
gation and general tension on the brachial plexus. Care must time, patients are encouraged to increase their walking
be taken to educate the patient to stop at the first sensation speed until they reach a level where they are aerobically fit
of tension and not linger with the arm in any self-test posi- on the basis of standard measures.
tion that provokes symptoms. The test sequence begins in CNS sensory dysfunction of the hand (specifically tactile
the zero position with the patient’s hand resting on the chest. localization, graphesthesia, and stereognosis) is assessed by
In position one the patient’s arm is straight at the side; in the methods of Byl.69
position two the patient abducts the arm to shoulder height Hand strength is assessed by examining for the presence
with the palm pronated. In position three the patient maintains of a Kabat sign.71 The patient is instructed to hold the arm at
abduction to shoulder height while supinating the forearm the side with the elbow flexed to 90 degrees and fully supi-
and hand; position four is performed by maintaining position nated. The wrist is positioned in neutral flexion-extension
three while extending the wrist. If the patient does not expe- with the fingers fully extended and the thumb in the short-
rience tension or pain in the previous positions, he or she can ened range of adduction and flexion (thumb in the plane of
progress to position five by adding cervical lateral flexion the palm). The distal phalanx of the thumb is held in full
away from the side being tested. Coppieters,72 in a cadaver extension. This starting position inhibits the median inner-
study of nerve gliding, noted increased strain on the medial vated muscles of the palm and finger flexors. A manual
nerve in positions of wrist extension combined with elbow muscle test is done to test the strength of flexor pollicis bre-
vis and adductor pollicis in the shortened range. If there is a
“giving way” at the metacarpophalangeal joint, then this is
quantified using a “thumbometer,” an inexpensive device
consisting of an eye drop bottle attached to a blood pressure
cuff sphygmomanometer. Clinical experience demonstrates
that after longus colli isometric contraction there is a
strengthening of the affected muscles in the thumb. There
will be a weakening effect on thumb strength if the patient
has cervical instability during the performance of activities
or exercises. This indicates the activity is too much for the
patient at that time. If there is no effect on thumb strength
then the patient is stable enough for the activity.
Neurovascular Entrapment Interventions

Treatment must follow the same principles that guide the
examination. The patient is taught self-assessment tech-
niques and strategies so that the patient has control of
Figure 18-4 n Demonstration of arm self-test positions. The arm the progression of treatment and activities of daily living.
self-test is an active upper extremity neural dynamic test. The patient may use any of the following self-assessment
techniques, as appropriate, to guide the course of treatment:

a pain scale, a thermometer to test skin temperature, a neuro-
dynamic test, or a Kabat strength test. Any treatment or activ-
ity that increases symptoms, protective posturing, or tension
is modified or discontinued.
Treatment is begun using sensory motor integration with an
emphasis on functional skills (e.g., breathing, balance, and
hand function) in a manner that does not cause irritation of the
patient’s condition. The patient is guided through a series of
breathing exercises designed to improve the circulation to the Figure 18-6 n Patient in a quadruped position on a therapy ball
extremities, calm the nervous system, and retrain the scalene with the chin tucked and the neck straight. The patient can lift an
muscles, if appropriate. The breathing exercises are progressed upper or lower extremity to provide a challenge to the muscles that
through the use of foam rollers (Figure 18-5). These are used stabilize the spine.
to increase the mobility of the spine and rib cage. The breath-
ing exercises are combined with functional movements of the
trunk and extremities in a manner that mobilizes the nervous the longus colli with Jull’s protocol.73 The expected effect
system. Once the patient is able to manage the symptoms, the of the thinker pose is to reverse the identified weakness of
treatment can progress to stabilization exercises with a gym the thumb. The patient is taught to minimize mechanical
ball (Figure 18-6). If the patient has vestibular, balance, or stress to the cervical and thoracic spine through instruction
sensory integration deficits, then specific techniques for ves- in body mechanics. An important concept is to train the
tibular, balance, or sensory retraining would be added. patient to identify the coactivation position for stability of
Our intention is not to present every component of a total the neck and to visualize that position before moving the
treatment program but rather highlight those core compo- body away from the center of gravity or moving the arm.
nents that address the neurovascular consequences of the This method has the patient assume the thinker pose to
injury. Our experience is that modifying these neurovascular stimulate the deep neck flexors to contract before the move-
consequences is the first step and the foundation for recovery. ment is performed.
The temperature, neurodynamic, and breathing dysfunc-
Core Components of Treatment tions are addressed by training the patient to perform relaxed
The reversible weakness of the thumb is addressed by diaphragmatic breathing with spinal motion (see Figure 18-2).
strengthening the longus colli muscle. This is accomplished The expected outcomes are to normalize hand temperature and
by a 30-second isometric contraction using the “thinker increase the range of motion while decreasing sensitivity to
pose” (see Figure 18-3) and specific muscle reeducation for the neurodynamic tests. Low cardiovascular endurance is
addressed by having the patient begin a progressive aerobic
conditioning program of walking. Because the examination
identifies six signs of dysfunction, the goals of treatment are to
normalize these six signs. Clinical experience teaches that as
these signs improve there is a reduction in pain and an increase
in function.
Role of the Patient

Maximizing the treatment response requires a unique partner-
ship between the physical therapist and the patient that facili-
tates the patient’s feeling of being in control. The feeling of
being in control is thought to have a positive impact on the
response to treatment. Patients are taught methods for self-
assessment of the immediate effect of the treatment. They are
taught to assess their responses to the core program on the
basis of signs of hand strength and hand temperature and
mobility and sensitivity of the nervous system. The patients are
provided with self-assessment tools and treatment devices.
The details of the exercise program are provided in a patient
booklet, audiotapes, and a videotape, which can be found at
www.edgelow.com.
References
ences are included on the companion Evolve site that accom-
panies this textbook. This online service will, when available,
Figure 18-5 n Foam roller exercise for mobilization of the spine. provide a link for the reader to a Medline abstract for the
The roller is placed underneath the spine with the client in the article cited. There are 73 cited references and other general
supine position. The client gently rolls from side to side to increase references for this chapter, with the majority of those articles
mobility of the spine. being evidence-based citations.
CASE STUDY
The following case example describes brief components of this Clinical Reasoning
patient’s physical therapy encounter. This presentation is not Diagnostic hypothesis is based on physical examination
meant to describe a complete case but rather to illuminate key findings:
concepts in the clinical reasoning behind the physical therapy 1. Cervical postural dysfunction
examination, assessment, and plan of care in a patient with 2. Altered neural dynamics based on the presence of:
signs and symptoms of neurovascular entrapment. Video clips a. Early onset of protective muscle tension and repro-
of the patient using self-assessment techniques to evaluate his duction of symptoms with arm self-test (indicator of
response to treatment are included. possible neurovascular entrapment)
PATIENT DESCRIPTION b. Scalene breathing pattern (indicator of possible adap-
The patient is a 27-year-old, right-handed caterer who tive response to pain)
3 weeks ago, while lifting a box, felt a “pop” and strain in INTERVENTION
his right forearm. He was referred to physical therapy with a The patient was taught a neural dynamic self-assessment
diagnosis of “forearm strain.” He has avoided using his right technique to evaluate his response to activity. We called this
arm for 3 weeks, but his symptoms have not significantly his “arm self-test.” If he had a negative response to an exercise
improved. or activities of daily living, as evidenced by an increase in
The patient drew a body chart indicating symptoms not only symptoms or a decrease in the range of his arm self-test, he
in his right forearm but also in his upper arm, his wrist, and the was instructed to modify or discontinue the activity and
right side of his neck (Figure 18-7). On further questioning he perform a self-treatment that restored his tension-free range.
stated that another component of his work was developing Videos 18-1 through 18-3 demonstrate the patient perform-
menus. He develops menus using a laptop computer perched ing his arm self-test, engaging in an exercise on a foam roller,
on some shelves in a cramped office space with his right arm and repeating his arm self-test immediately after engaging in
sustained overhead in an awkward position. the foam roller exercise. The intention of the foam roller exer-
Clinical Reasoning cise was to help him mobilize his thoracic spine to improve his
Diagnostic hypothesis is based on the subjective examination ability to correct his cervical posture on his own. After the
findings: roller exercise he demonstrated a dramatic increase in tension-
1. Potential soft tissue strain of forearm flexors based on free range of his right arm self-test. The difference was readily
the mechanism of injury apparent to the patient and helped him grasp the concept of
2. Potential altered neural dynamics with sensitization sensitivity of the nerves as well as the concept of the nerves
on the basis of: as a continuous tract in which cervical posture correction was
a. History of receptive use of the upper extremities in a key component of his treatment.
sustained awkward positions After the foam roller exercise his right-sided neck discom-
b. Pattern of symptoms that do not fit localized forearm fort was unchanged. A trial of cervical traction with the use of
strain or cervical radiculopathy a towel (Figure 18-9) was found to relieve his neck discomfort
c. Lack of response to 3 weeks of rest and self-care without producing forearm symptoms or worsening his arm
measures. self-test. During the first treatment, the techniques that he
PHYSICAL EXAMINATION found successful for restoring his neural mobility were foam
Figure 18-8 is a photograph of the patient’s sitting posture on roller exercises, diaphragmatic breathing (see Figure 18-2),
initial examination. He demonstrated forward head posture and supine cervical traction (see Figure 18-9). Therefore
and mildly protected posture of his right arm. Based on the those interventions were the focus of his home program
patient’s history, the nervous system was considered a potential instruction.
source of dysfunction. (See Table 18-2 for suggested modifica- The patient was issued a foam roller and instructed in spine
tions to a biomechanically based musculoskeletal examination mobilization exercises (see Figure 18-5). He was instructed in
to use when the nervous system is considered a significant the towel cervical traction technique for symptom management
source of dysfunction.) (see Figure 18-9). A cervical posture correction exercise termed
The patient was instructed to complete active movement the “thinker position” (see Figure 18-3) was added to his
testing just to the point of feeling tension or resistance to home exercise program as a form of dynamic cervical posture
movement. This modification to the physical examination correction. In addition, he was instructed to walk daily to
was meant to minimize the potential for a significant flare of maintain his aerobic capacity. Figure 18-8 shows the patient’s
symptoms from provocation testing of potentially irritable posture before the initial examination. Figure 18-10 shows the
neurovascular structures while still providing a repeatable patient’s posture immediately after the initial treatment that
measure for reassessment. included foam roller exercise, towel cervical traction, instruc-
The patient moved through full cervical range without tion in the self-assessment of upper extremity neural dynamic
complaints of tension or resistance. On palpation the scalene test, and dynamic cervical posture correction via the thinker
muscles were noted to be active during quiet breathing. The position.
arm self-test indicated a restriction of mobility in his right At 4 weeks (visit 4) treatment progressed to light resistive
brachial plexus as compared with his uninvolved left side. exercises. At this stage the patient no longer demonstrated
He indicated the onset of tension and the reproduction of his signs of neural irritability. The patient was placed prone on
forearm symptom with the arm self-test. a therapy ball to perform exercises that promote scapular
Continued
CASE STUDY—cont’d
stabilization, postural strengthening, and functional grip (see localized soft tissue strain. Other objective indicators not as-
Figure 18-6). At visit 5 he reported a flare of his symptoms and sessed initially that may have further guided the treatment
what he did to resolve the problem. The patient experienced a would be Kabat testing,71 measuring the temperature of the
flare of symptoms after an attempt to progress his strengthen- hands,70 and sensory testing of localization, graphesthesia, and
ing exercises. He discontinued the strengthening exercise and stereognosis.69
used his symptom relief techniques of breathing exercises and A key concept to keep in mind is the role of education in
towel cervical traction. He subsequently used the arm self-test treating patients with a problem such as neurovascular entrap-
to determine which strengthening exercise he could tolerate ment. Patient-clinician communication is extremely important
without producing a protective tension response of his arm. when dealing with all patients, but the clinician’s communica-
The sixth and final treatment session focused on problem tion skills are really challenged when dealing with a patient
solving related to symptom management, upper-quarter who has a neurovascular entrapment. Describing the dysfunc-
stabilization during simulated work tasks, and progression to tion of a neurovascular entrapment to the patient in succinct,
recreational activities. Emphasis was placed on continued nonmedical terminology can be quite difficult, but it is a criti-
self-assessment of the response of the nervous system to the cal step in the patient encounter to help him or her develop
progression of activity. an understanding of what is wrong so he or she can engage
OUTCOME in self-treatment. Teaching the patient self-assessment tools
The early success with self-guided treatment set the stage restores the patient’s control, allowing the patient to guide his
for teaching the patient to evaluate the effect of any activity, or her own treatment and to be more responsible for his or her
manage symptoms with one or two easing techniques, and own well-being.
ultimately progress his own activity level. This approach gave QUESTIONS
the patient control of his problem so that he was capable of 1. How would you describe a form of neural sensitization
managing a flare of his symptoms. (mechanical allodynia) to a patient?
The patient received a total of six treatments. At the time of Answer: I say to the patient: Have you ever touched a hot
discharge his grip strength was equal bilaterally and his upper plate? When you touched that hot plate what did your
extremity neural dynamic test results were equal bilaterally. hand do? It quickly pulled away. Your body has reflexes
At this point he was working full-time, regular duty with that protect you, like tightening the muscles in your arm
ergonomic improvements at his workstation. so you can pull your hand away from the hot plate. What
DISCUSSION if you touched this smooth, cool sink, but your finger did
This case illustrates the importance of evaluating the role of not recognize the smooth, cool sink. Instead your finger
the nervous system in patients with symptoms associated with sent a signal to your brain that it was touching a hot plate.
repetitive use of the upper extremity. In this case example That’s what your body does when it has experienced pain
the patient’s problem did not seem to be chronic, because he for a period of time—it becomes sensitized, meaning it
reported a specific recent injury. However, on further investiga- starts to sense things that are normally not painful as now
tion he also reported symptoms that were chronic in nature, being painful. The body then tenses or tries to withdraw
which could have delayed his recovery if not appropriately and get away from what it senses as pain.
assessed on initial examination. There can be a wide spectrum 2. How would you instruct a patient to do an active self-test of
of presentations of neurovascular entrapments ranging from the arm?
subtle signs and symptoms of nervous system involvement to Answer: I say to the patient: I am going to ask you to place
dramatic, life-altering, complex problems in patients who your arm in a sequence of positions. The positions are
have undergone multiple medical and surgical interventions numbered from 0-5. I want you to stop when you feel
without obtaining symptom relief. The key to success in tightness anywhere in your arm or if you feel an increase
treating patients with neurovascular entrapments is recognizing or change in your pain. Remember the number where
the signs and symptoms of subtle nervous system involvement you stopped. We will redo the self-test after we have
early. Neural sensitization4 and possible processing changes done some exercises. That way you can decide which
in the CNS67-69 necessitate evaluation of the nervous system exercise helps you the most.
as a potential source of symptoms in patients with symptoms After the patient has completed one self-test and one form of
of CTD. If the issues of nervous system irritability and treatment followed by a reassessment self-test, then I help
sensitization are not addressed during evaluation and the patient interpret the body’s response. If your self-test
throughout treatment, then the risk for increasing the patient’s result was worse (lower number, less arm range), it means
symptoms and continuing the cycle of nervous system that your body responded as though it were touching the
hypersensitivity is high. hot plate—it tightened and tried to withdraw. This means
The indicators that this patient may have had a nervous that the exercise we tried is not a helpful exercise for you
system dysfunction were his history of repetitive work in an at this time. If your self-test result was better (higher
awkward position, the pattern of his symptoms, and his lack number, greater range), then this exercise was helpful;
of response to standard medical care and rest. The indicators your body responded in a way that indicated calming of
of nervous system dysfunction on physical examination were the protective response. The self-test is something you
the restricted upper limb neural dynamic test (arm self-test), can always use to help you evaluate whether an exercise or
altered breathing pattern, and lack of objective signs of a activity is going to be helpful or hurtful for your arms.
Figure 18-7 n Body chart with symptomatic areas marked by

the patient.
Figure 18-9 n Towel traction unit. Through arching of the low

back, the amount of traction is increased slightly. Through flattening
of the low back, the amount of traction is decreased slightly.
Figure 18-8 n The patient’s posture before initial examination.
Figure 18-10 n The patient’s posture immediately after the

initial treatment.
CHAPTER 19 Multiple Sclerosis
GAIL L. WIDENER, PT, PhD

autoimmune disease After reading this chapter the student or therapist will be able to:
axonal damage 1. Describe the pathological processes, prevalence, and clinical presentation of people
benign with multiple sclerosis.
demyelination 2. Compare and contrast the types of multiple sclerosis and the common disease
disease-modifying agents progression in each.
exacerbation 3. Discuss the medical management of the disease and the disease symptoms.
immune system 4. Describe how the International Classification of Functioning, Disability and Health
lesion provides a common language for describing the impact of disease on people with
neuroprotection multiple sclerosis and how it provides a framework for rehabilitation management.
plaques 5. Describe the outcome measures that can be used to examine people with multiple
primary progressive sclerosis that cover body system problems (impairments), functional skill and activity
progressive relapsing limitations, and participation restrictions.
relapse 6. Develop a rehabilitation plan of care using evidence-based interventions to maximize
relapsing remitting patient function and quality of life.
remission
secondary progressive
OVERVIEW OF MULTIPLE SCLEROSIS spared in the majority of WM lesions. Treatment in the

initial stages of the disease is aimed at reducing inflamma-
Pathophysiology tion and immune system infiltration with disease-modifying
Multiple sclerosis (MS) is a chronic, inflammatory disease of agents (DMAs).
the brain, optic nerve, and spinal cord mediated by the im- Later in the course of the disease, inflammation be-
mune system.1 It is characterized by lesions of disseminated comes uncommon while demyelination and axonal loss
focal demyelination accompanied by variable axon damage continue, suggesting replacement by a neurodegenerative
and destruction and reactive gliosis. Initially, MS was thought disease process. Disease progression becomes more con-
to be a disease of the white matter (WM); however, recent stant with a lack of exacerbation. The motor, sensory, and
investigations have shown that the gray matter (GM) is cognitive disability that accumulates in the advanced
significantly involved. Lesions found in the GM typically stages of the disease appears to be associated with the cor-
contain demyelination and loss of neurons without the tical GM pathology.2 Owing to the lack of inflammation,
immune system infiltrates and inflammation characteristic of DMAs have not been shown to be beneficial in the later
lesions in the WM. Tissue damage has been found outside stages of the disease.
the focal lesions throughout the GM that is associated with
brain and spinal cord atrophy. These areas of demyelination Incidence and Prevalence
and axonal damage interfere with normal conduction of MS is the primary cause of nontraumatic disability in
neural signals, leading to a disruption of function. young and middle-aged adults and the most common in-
Early in the course of the disease, focal inflammatory flammatory condition of the central nervous system (CNS).
WM lesions are composed of immune system components It is reported that approximately 350,000 to 400,000 people
that produce demyelination, axonal injury, and loss of oli- in the United States and over 2.5 million people worldwide
godendrocytes. Astrogliosis activated by the damaged neu- have the disease.3,4 People are most commonly diagnosed at
rons produces gliotic scarring (visualized as sclerosis in age 20 to 50 years, with an average age of 32. However, MS
postmortem brain tissue) called plaques. Active disease is can be diagnosed in people of any age. Approximately 5%
followed by periods of remission in which acute inflamma- of all patients with MS are diagnosed before their sixteenth
tion is reduced. Axonal remyelination occurs but is highly birthday.5
variable and is related to recovery of function during peri- MS is found in people who reside above the northern or
ods of remission. The degree of axonal loss is associated below the southern 40° latitude with greater frequency than
with the severity of the inflammation; however, axons are those who live closer to the equator (Figure 19-1). Given the
585
80° 80°
60° 60°
45° 45°
40° 40°
20° 20°
0° 0°
20° 20°
Key
High Risk
40° Probable High Risk 40°
45° Low Risk 45°
Probable Low Risk
North-South Gradient
in Risk
Other Gradient in Risk
Figure 19-1 n World distribution of multiple sclerosis. (From Multiple Sclerosis Resource Center,
www.msrc.co.uk.)
increased sun exposure of people living closer to the equator, flairs, relapses) that can last days to months and are typically
lack of vitamin D is being investigated as a potential factor followed by periods of improved function. During remis-
contributing to disease development.6 Many researchers sions, function can return to prerelapse levels, but most
believe that exposure to an infectious agent may trigger the frequently it does not recover fully. Attacks normally occur
disease process: Epstein-Barr virus is currently considered a with a frequency of one or two per year. Approximately 90%
likely candidate. of people with RRMS transition to SPMS after 20 years or
Women are affected two to four times more frequently around 40 years of age.
than men. Even so, men are more likely to have a more ag- In secondary progressive MS (SPMS), relapses decrease
gressive disease progression and a worse prognosis.4,7 Cau- in frequency over time and convert to a slow steady progres-
casians with Northern European ancestry have the greatest sion of increasing disability or disease severity. Relapses may
incidence of MS, whereas people of Asian, African, or His- occur early in SPMS but gradually lessen over time. People
panic ethnicity are at lower risk. African Americans have a with RRMS eventually convert to SPMS 10 to 20 years after
lower incidence, but become disabled earlier than Cauca- diagnosis.11
sians, suggesting that tissue destruction occurs earlier It is thought that the clinical disability associated with
and more rapidly.8 Inuits, Yakutes, Hutterites, Hungarian SPMS results from the neurodegeneration that occurs as a
Romani, Norwegian Lapps, Australian Aborigines, and result of tissue injury that accumulates from early in the
New Zealand Maoris do not appear to develop MS.9 Being disease process. In addition to less inflammation, there is
diagnosed with MS may be related to age, gender, genetics, a greater amount of brain atrophy in people with SPMS
geography, or ethnic background. An identical twin with compared with RRMS. Figure 19-3 shows the natural
MS means that the other twin will have a 25% chance of history of RRMS and SPMS, comparing the change in
diagnosis, suggesting something beyond genetics. Having brain volume with increasing clinical disability and
a first-degree relative with MS will increase the risk of disease burden.2
disease from 1/750 to 1/40.3 Primary progressive MS (PPMS) is less common, affect-
ing only 10% to 15% of people with MS. From disease on-
Types of Multiple Sclerosis and Clinical set, progression results in a gradual worsening of symptoms
Characteristics without relapses. People tend to be older when diagnosed
At least four types of MS have been identified (Figure 19-2). (late 30s or early 40s), have fewer abnormalities on brain
Although the course of the disease is highly variable even MRI, and respond less favorably to standard MS therapies.
within a subtype of MS, there are characteristics common Progressive myelopathy is commonly associated with
to each. PPMS.
The initial neurological episode or attack is typically Progressive relapsing MS (PRMS) is the least common
identified as clinical isolated syndrome (CIS). Symptoms form (5%). This form of MS typically begins with a
must last for at least 24 hours and can be monofocal or mul- progressive course with clear relapses or exacerbations.
tifocal. If there are lesions present on magnetic resonance Benign MS is identified when symptoms occur once
imaging (MRI), there is a high risk of developing MS. and never recur. This happens in roughly 25% of cases.12
In one group of people with CIS followed for 20 years, 63% Recently, Sayao and colleagues13 reported that 52% of
were diagnosed with definite MS.10 people with benign MS had not developed MS 20 years
Relapsing remitting MS (RRMS) represents about 85% later. However, the remainder of people went on to
of people with MS, characterized by exacerbations (attacks, develop MS, with at least 21% requiring the use of a cane.
CHAPTER 19 n Multiple Sclerosis 587
Relapsing Remitting MS Clinical Manifestations

MS can affect the optic nerve and any tissue within the brain
or spinal cord, so almost any neurological symptom can re-
sult. Individual assessments are required to identify the
Increasing
disability
problems present. Even so, the following list constitutes the

most common problems encountered by people with MS.
Fatigue
Of people with MS, 65% to 97% report fatigue during the
course of the disease; as many as 40% of people with MS
Time state that fatigue is their most disabling symptom.15 There
are two types of fatigue in people with MS: primary and
Secondary Progressive MS secondary. Primary fatigue, often called lassitude, is caused
by the effects of the demyelination and axonal destruction
and its effect on nerve conduction. Restorative rehabilitation
has little effect on primary fatigue from neurodegeneration.
Increasing
disability
Secondary fatigue results from problems such as decon-

ditioning, infections, sleep disturbances, poor nutrition,
medication side effects, other medical conditions (such as
thyroid disease), and heat intolerance. Clinicians should be
extremely careful to separate the types of the fatigue in order
to determine the most appropriate interventions.
Time
Sensory Impairments
Sensory impairments are among the most common symp-
Primary Progressive MS
toms associated with MS and can affect the visual, somato-
sensory, and vestibular systems.16 The most common prob-
lem of the visual system is optic neuritis, which can produce
Increasing
blurry or double vision and/or painful eye movements and

disability
nystagmus. Somatosensory or proprioception disturbances

can include dysesthesias (tingling, buzzing, or vibrations) or
anesthesias (complete loss of sensation in part of the body).
People may experience paresthesia or anesthesia in half of
the body—upper or lower or side to side—or below a certain
spinal cord level. Dysesthesias may be limited to small body
Time areas such as a patch of skin on the head or a single upper
or lower extremity. Vestibular system involvement occurs in
20% of people with MS at some time during their disease
Progressive Relapsing MS course17 and may manifest as dizziness and/or vertigo.
People with MS can have pain associated with the dam-
age to neural tissue (neuropathic pain). This may manifest as
neuralgias with burning, itching, or electric shocklike sensa-
Increasing
disability
tions. Lhermitte sign is an electric shock–like shooting

sensation that can run into the upper extremities or down the
back in response to flexion of the neck.
Motor Systems Impairments

Deficits in the motor system include weakness, spasticity,
Time ataxia, and tremor. Paresis or muscular weakness is fre-
Figure 19-2 n Types of multiple sclerosis.
quently seen in people with MS and is associated with sev-
eral causes. Like fatigue, weakness can be caused by damage
to the myelination and axons of motor and premotor neurons
in the CNS that can manifest in many different patterns
The authors could not identify any criteria associated with including monoparesis, paraparesis, hemiparesis, or quadri-
either developing MS or continuing to have the benign paresis. However, additional causes of muscle weakness can
form. also be associated with disuse deconditioning and may also
The risk of a more rapid disease progression is corre- result in muscle atrophy. When muscle weakness or loss of
lated with older age at diagnosis; male sex; initial symp- motor control is seen in the muscles of speech, it results in
toms involving the motor, sphincter, or cerebellar systems; dysarthria. Paralysis or total loss of muscle strength occurs
multifocal disease at onset; shorter time between first and with less frequency but can be devastating for patients. Sev-
second attacks; and frequent attacks in the first 5 years eral patterns of paralysis (or “-plegia”) occur in people with
postdiagnosis.7,14 MS including paraplegia, hemiplegia, and quadriplegia.
Brain volume
Clinical disability
MRI lesions
RRMS SPMS
Inflammation
Neurodegeneration
TIME
Figure 19-3 n Natural history of relapsing remitting multiple sclerosis (RRMS)—conversion to second-
ary progressive multiple sclerosis (SPMS). Figure shows the typical clinical course of RRMS with conver-
sion to SPMS. Magnetic resonance imaging (MRI) activity (gray line and boxes) indicates the inflamma-
tory lesions; they occur more frequently early in the disease and occur with greater frequency than in
clinical disability (solid black line). Brain volume indicated by the stippled line shows brain atrophy
increasing as the inflammatory component of the disease slows and is replaced by neurodegeneration.
A broad clinical definition of spasticity is a velocity-sensitive cerebellum as a likely source (see Chapter 21). Tremors af-
resistance to muscle stretch or a muscle spasm during move- fect the head, neck, vocal cords, limbs, and torso, with the
ment.18 Some people report heaviness in the limbs, difficulty upper extremities having the greatest occurrence.21,22
moving a joint, jumping of the extremities, or involuntary MS affects many of the systems required for postural
painful movements. Muscle spasms or cramping are fre- control and balance, including sensory input (visual, so-
quently experienced by people with MS. Eighty four percent matosensory, and vestibular), central processing, and motor
of people with MS report spasticity, with 34% indicating output. Therefore it is not surprising that over 50% of people
that their spasticity is moderate to severe.19 Female sex or with MS report falling one or more times in the previous
longer disease duration are both associated with higher 6 months.23-26
prevalence of spasticity. Spasticity has been highly corre-
lated with patient-reported disability and poorer quality of Bowel and Bladder Dysfunction
life (QOL).19 Spasticity may change according to position The incidence of bowel problems (35% to 68%) and bladder
and may result from increased effort during activity or from problems (52% to 97%) make them common in people with
the presence of a noxious stimulus such as an infection, MS, as reported by two research studies.27,28 Symptoms
skin lesions, fractures, renal stones, distention of bladder or include urinary urgency, nocturia, or retention of urine or
colon, or other physiological stressors such as certain medica- feces.29 Incontinence of either system can also occur.
tions (DMAs or serotonin reuptake inhibitors) or psy Neurogenic detrusor muscle overactivity is the most com-
chological distress. Environmental factors such as tight cloth- mon urological impairment in people with MS; 20% have
ing, hunger, or elevated body or air temperature may also lead detrusor muscle underactivity, and only 10% report no
to increased spasticity. Spasticity can cause muscle contrac- symptoms.28
tures, skin breakdown, pain, and sleep disturbances, which
often lead to secondary activity limitations and participation Sexual Dysfunction
restrictions that limit performance of activities of daily living Sexual dysfunction affects 40% to 85% of women with MS
(ADLs) and mobility. and 50% to 90% of men. It can manifest as erectile dysfunc-
Ataxia occurs in up to 80% of people with MS at some tion, impotence, inability to achieve orgasm, and, in men,
point in their disease progression.20 This motor deficit can retrograde ejaculation.28,30,31
occur from disturbances in the vestibular system or cerebel-
lum or a loss of proprioception. Ataxia or a lack of coordina- Cognitive Impairments
tion can manifest as difficulty with walking to difficulty Cognitive dysfunction occurs in roughly 40% to 70% of
with movements of the extremities such as overshooting or people with MS, with 70% demonstrating mild to moderate
undershooting targets (dysmetria) or an inability to produce impairment.32,33 Although cognitive problems can occur at
rapid alternating movements (dysdiadochokinesia). Occa- anytime, abilities affected early in the course of the disease
sionally, patients experience sustained body positioning are verbal fluency and verbal memory.34 Other cognitive
(dystonia) of the extremities or head and neck. In different dysfunctions common in people with MS include impair-
research studies, tremor is reported by 25% to 58% of peo- ments in memory, processing speed, executive functioning,
ple with MS, with the majority of people experiencing mild attention, and visuospatial learning. There is a fair correlation
to moderate dysfunction.21,22 Action tremor, both postural between cognitive decline and ability to work and unemploy-
and intention, are found in people with MS, pointing to the ment because of the impairments in short- and long-term
memory, problems with concentration, forgetfulness, and

slowed word recall.35,36 This is a likely source of frustration
for both patients and caregivers alike.
Depression
Depression is two to three times more common in people
with chronic health conditions than in the general popula-
tion and has a greater incidence than other neurological
conditions.37 From 26% to 50% of people with MS have
been reported to experience depression during the course
of the disease.32,38 Several factors contribute to the high
incidence of depression in people with MS. The fact that
MS is a chronic, progressive, and unpredictable disease
that affects people in their early to middle adult years, is
often invisible, and limits participation in many life roles
often leads to a perceived reduction in QOL.39 Suicide is of
great concern for people with depression, and rates are
significantly higher in people with MS than in the general
population.40 Depression is associated with a lower QOL
and other symptoms of MS including fatigue, disability,
pain, and cognitive impairment.41
Figure 19-4 n T2-weighted magnetic resonance imaging (MRI)
Heat Intolerance scan of plaques associated with multiple sclerosis. Plaques are indi-
Uhthoff phenomenon is a temporary worsening of MS- cated by arrows. (From Frey H, Lahtinen A, Heinonen T, Dastidar P:
related problems associated with an increase in core body Clinical application of MRI image processing in neurology. Int J
temperature. Such increases can occur with physical exer- Bioelectromagnet 1(1), 1999.)
tion such as exercise or with a change in the environment
such as hot baths or showers, hot weather, and hot air
temperature. conditions. The first is the analysis of CSF. This requires a
lumbar puncture in which CSF is gathered and analyzed to
identify oligoclonal bands representing the presence of im-
MEDICAL MANAGEMENT
mune system proteins indicating that the body is attacking
Diagnosis itself. The majority of people with MS have oligoclonal
Historically, people with MS would wait for a diagnosis for bands; however, because people with other diseases or con-
a year or more. Although there are no definitive tests that ditions also have oligoclonal bands, the test is not specific
diagnose MS, the addition of MRI has accelerated diagno- for MS. The lack of oligoclonal bands at diagnosis has been
sis. In 2001 the International Panel on the Diagnosis of related to a slower progression of the disease and increased
Multiple Sclerosis updated criteria to include MRI, visual time to reach markers of disability such as walking with an
evoked potentials, and cerebrospinal fluid (CSF) analysis. assistive device or confinement to a wheelchair.
The 2005 Revised McDonald Criteria for MS diagnosis Evoked potentials record the nervous system’s response
were designed to make the diagnostic process even more to stimulation of a specific sensory pathway (visual, audi-
efficient and easier.42 The Poser criteria require the presence tory, vestibular, or general somatosensory). Demyelination
of two separate episodes over time, plus evidence of two and axonal degeneration cause a slowing of signal transmis-
or more lesions in separate brain or spinal cord regions sion along neurons and therefore will increase the response
identified by radiological imaging studies. Even with the time to an externally applied sensory stimulus. Damage to
improved technological measures used to facilitate diagno- the optic system is a common first symptom in MS, and
sis, an accurate clinical history is critical. Often patients will therefore visual evoked potentials are often most helpful in
recall episodes of transient symptoms that did not last long diagnosis.
enough to require attention by a primary care provider. Disease severity and progression are monitored by ongo-
In addition to the clinical history, MRI studies have ing medical checkups, MRI imaging, and the use of several
improved diagnosis of MS. Although T2-weighted MRI outcome measures. The Kurtzke disease severity scale was
images show MS lesions as hyperintense and identify new developed to allow primary care providers a way to measure
or active lesions, MRI has been shown (Figure 19-4) to clinical disability and chart disease progression. It has been
overestimate clinical relapses. Conventional MRI with T1 replaced by the Expanded Disability Status Scale (EDSS)
weighting identifies lesions as hypointense (black holes) and (Table 19-1).43 The EDSS is a 10-point ordinal scale com-
is able to identify brain atrophy. T1 imaging demonstrates a pleted by a physician or physician extender, with 0 indicat-
stronger correlation with clinical status and disease severity ing no disability and 10 indicating death caused by MS.
than the lesion load found with T2 weighting. Gadolinium- Using a cane relates to an EDSS score of 6.0. The National
enhanced T1-weighted MRI images show active MS lesions MS Society (NMSS) Task Force on Clinical Outcomes
as hyperintense (white). Assessment also recommends the Multiple Sclerosis Func-
Two additional medical tests can be used to aid in the tional Composite (MSFC)44 as a measure of disease severity
diagnosis of MS and differentiate it from other diseases and and progression. This set of outcome measures is used to
TABLE 19-1 ABBREVIATED EXPANDED

n
aimed at reducing inflammation will be less effective as the
DISABILITY STATUS SCALE disease progresses. Fox2 suggests that early treatment is
needed to compensate for the later stages of the disease when
inflammation is less prevalent.
SCORE FUNCTION
1.0 Normal neurological examination findings
Medications
2.0 Minimal disability
3.0 Moderate disability Disease-Modifying Agents
4.0 Ambulates 12 hours without aid DMAs are aimed at reducing immune system dysfunction,
5.0 Disability impairs activity (walks 1500 feet thereby reducing damage to neural tissue and long-term dis-
without assistance) ability for people with RRMS. There are several different
6.0 Intermittent or unilateral constant assistance medications that act on various components of the immune
6.5 Bilateral support required (walker, crutches, system with the intention of modifying the course of the
two canes) disease (Table 19-2). In general, these drugs are approved for
7.0 Unable to walk 15 feet without assistance use with RRMS and are used off-label for other forms of MS
8.0 Basically constrained to bed and have been shown to reduce the number of attacks expe-
9. 0 Bedridden rienced. The majority of the drugs require injections; how-
10.0 Death from multiple sclerosis ever, in 2010 the U.S. Food and Drug Administration (FDA)
approved the first oral DMA, fingolimod. Measurement
of therapeutic effectiveness includes relapse rate, progression
chart change in physical and cognitive function and will be of disability (EDSS), and quantitative evidence of lesions on
discussed later in this chapter. It includes three tests that MRI. All DMAs have side effects (see Table 19-2), but rarely
measure upper-extremity function (Nine-Hole Peg Test are they serious. These medications are costly, and some
[NHPT]), lower-extremity function and mobility (25-Foot people do not respond well or tolerate the side effects. It is
Timed Walk [25FTW]), and cognitive function (Paced Audi- common that people will try more than one type before find-
tory Serial Addition Test [PASAT]). ing the DMA they tolerate the best.
Medical management of MS has two major goals: long-
term management of the disease and exacerbations and symp- Antiinflammatory Medications
tomatic management. Early after diagnosis with CIS, it is High-dose corticosteroids (such as prednisone or methyl-
recommended that people take DMAs. Recent evidence prednisolone) are used to reduce inflammatory response
suggests that as the disease progresses it becomes less inflam- during exacerbations for people with RRMS. Although no
matory and more neurodegenerative. Therefore medications medications have demonstrated effectiveness in people with
TABLE 19-2 n DISEASE-MODIFYING AGENTS: INDICATIONS AND SIDE EFFECTS
FDA-APPROVED DISEASE-MODIFYING AGENTS INDICATION COMMON SIDE EFFECTS

IFN beta-1a (Avonex) CIS Flulike symptoms
IFN beta-1a (Rebif) RRMS Injection-site reactions
IFN beta-1b (Betaseron) SPMS Depression
IFN beta-1b (Extavia) Elevated liver enzymes
Glatiramer (Copaxone) CIS Injection-site reactions
RRMS Systemic reactions, immediately postinjection
Elevated liver enzymes
Natalizumab (Tysabri) RRMS Progressive multifocal leukoencephalopathy
Infusion reactions
Hepatotoxicity
Mitoxantrone (Novantrone) RRMS Cardiotoxicity
Intravenous infusion SPMS Treatment-related leukemia
PRMS Infection risk
Alopecia
Amenorrhea
Fingolimod (Gilenya) RRMS Flulike symptoms
Increased liver enzymes
Headache
Diarrhea
Back pain
Cough
CIS, Clinical isolated syndrome; FDA, U.S. Food and Drug Administration; IFN, interferon; RRMS, relapsing remitting multiple sclerosis; SPMS, secondary
progressive multiple sclerosis.
PPMS, anecdotal evidence suggests that intermittent pulses the hygiene score, but no changes were noted in spasm
of intravenous methylprednisolone can help slow progres- frequency score.45 Blocks last 1 to 3 months with relatively
sion of clinical disability in some patients.2 few side effects. Similarly, botulinum toxin type B was
A host of additional medications are used to manage the shown to reduce hip adductor spasticity.46 Clinical practice
symptoms associated with MS. Each will be discussed as guidelines18 recommend that neuromuscular blocks be
part of symptom management. Also refer to Chapter 36 for performed by appropriate specialists in conjunction with a
additional information. rehabilitation program.
Refractory spasticity is defined as unsuccessful treatment
Symptom Management with oral medications and/or rehabilitation. In this situation
two other options exist: surgery or placement of an intrathe-
Fatigue cal baclofen pump (ITB). Surgical procedures include
The fatigue experienced by people with MS is generally tendon lengthening or tendon transfer and are performed to
divided into primary and secondary causes. Fatigue from maintain adequate hygiene or prevent or correct contractures
primary causes results from the disease itself or to heat and therefore preserve function. Intrathecal pumps, inserted
intolerance and is defined by the term MS lassitude. Heat into the spinal cord, allow adjustable drug delivery.
intolerance may result in a temporary worsening of symp- Baclofen, the drug of choice for the intrathecal pump, can be
toms. It is sometimes referred to as pseudoexacerbation given in higher doses; use of the pump avoids the side
and occurs when core body temperature rises with expo- effects often encountered when the drug is taken orally.
sure to raised ambient temperature or metabolic activity Relapses are more commonly reported in people on oral
such as exercise. However, in addition to MS lassitude, medications than those using ITB. People using ITB also
other causes can include side effects of medications used report higher levels of satisfaction, less spasticity, and fewer
in the treatment of MS, deconditioning from reduced ac- painful spasms compared with those on oral medications.19
tivity levels, poor nutrition, infections or other medical
conditions, depression, or sleep disturbances. Several Pain
medications combined with rehabilitation strategies have Both nociceptive and neuropathic pain can be present in
been recommended for management of fatigue. Amanta- people with MS. Therefore it is important to discern the type
dine (Symmetrel) and modafinil (Provigil) are frequently of pain in order for the most appropriate treatment to be
prescribed. rendered. Nociceptive pain can often be treated with analge-
sics (acetaminophen, nonsteroidal antiinflammatory drugs
Spasticity [NSAIDs], or opioids) and is more amenable to physical
Spasticity can interfere with physical function and hygiene. therapy (discussed later under rehabilitation management).
However, spasticity can also add support to weakened Neuropathic pain generally requires pharmacological inter-
limbs, allowing more effective mobility. The goal of medi- vention, although an interdisciplinary team approach may
cal management of spasticity is to maintain full range of be valuable. First-line medications for neuropathic pain
motion (ROM) of muscle and soft tissue structures to allow that occurs in the spinal cord are calcium channel blockers
maximal physical function and proper hygiene. Haselkorn (gabapentinoids) or N-methyl-d-aspartate (NMDA) antago-
and colleagues18 describe the clinical practice guidelines nists (ketamine). When pain is present in the head, the
for managing spasticity in people with MS written by primary treatment is opioid drugs such as antidepressants
the Multiple Sclerosis Council. A complete assessment of (tricyclics) or anticonvulsants (gabapentin or pregabalin).47
the spasticity and how it affects the individual’s life is In the case of trigeminal neuralgia, the first choice is often
required. Typically, successful management includes both carbamazepine. Refer to Chapter 32 on pain management
pharmaceuticals and rehabilitation. for additional information.
When spasticity is the result of CNS impairments,
medical management often includes the use of oral pharma- Mobility
cotherapy including baclofen (Lioresal) or tizanidine Physical rehabilitation is the primary intervention used
(Zanaflex). Adjuvant therapies include diazepam (Valium) to manage mobility dysfunctions. However, one medica-
or clonazepam (Klonopin), dantrolene (Dantrium), gaba- tion has recently been FDA approved to improve gait. In
pentin (Neurontin) or levetiracetam (Keppra), clonidine clinical studies dalfampridine (Ampyra) demonstrated the
(Catapres), or muscle relaxants. Each of these drugs can ability to improve walking speed in people with MS.48
have negative side effects that interfere with movement and However, changes in the quality of gait or movement were
therefore rehabilitation. not measured.
Management of focal spasticity may include local anes-
thetics such as lidocaine, bupivacaine, etidocaine, all of Tremor
which are short acting with side effects of CNS and cardio- Tremor management using medications such as isoniazid,
vascular toxicity and hypersensitivity. Neurolysis treatment carbamazepine, ondansetron, or cannabis extract has been
with phenol or alcohol is longer acting; however, these minimally effective.49 Surgical interventions including ste-
agents can have the side effects of pain, swelling, fibrosis, reotaxic thalamotomy and deep brain stimulation have been
and dysesthesias. Focal spasticity affecting functional mus- studied, but the evidence to support the effects on functional
cle groups can also be effectively treated with neuromuscu- status and disability is lacking. The effectiveness of other
lar blocking agents including alcohol, phenol, or botulinum options including physical therapy, tremor-reducing ortho-
toxin. Botulinum toxin type A (Botox) has been shown to ses, and extremity cooling have yet to be proven beneficial
improve spasticity as measured by the Ashworth Scale and in clinical trials.49
Bowel and Bladder Management activities, and exercises that provide an adequate stimulus
Behavioral modification and rehabilitation are used to help to produce adaptation is critical to restore function or
alleviate the symptoms of bladder incontinence or detrusor improve motor and cognitive performance. Although each
muscle overactivity. A few medications have been shown to patient case is unique, the most likely answer is that both
be helpful: anticholinergic agents are used to manage detru- strategies will be employed. The challenge for rehabilita-
sor overactivity or dyssynergia, and underactivity is treated tion professionals is to sort out how much of a patient’s
with cholinomimetic agents.2 dysfunction arises from neurodegeneration, which necessitates
People with constipation are encouraged to combine compensation, and how much occurs from inactivity and
adequate fluid intake with dietary fiber or bulk-forming system deconditioning, in which case system capacity can
medications.2 be restored to some extent. Rehabilitation professionals
must choose therapeutic interventions based on whether
Depression and Cognitive Impairments compensation or restoration is the goal.
Depression is very common in people with MS, yet it is Rehabilitation for people with MS occurs in every setting:
infrequently identified or treated.50 Therapy can include inpatient hospitals, outpatient clinics, skilled nursing facili-
supportive psychotherapy and medication given individually ties, home care settings, and the community. With the current
or in combination. To date two pharmacological therapies climate of decreasing access to and reducing coverage for
have shown the most promise in reducing cognitive deficits rehabilitation, therapists must be able to make evidence-
(l-amphetamine sulfate and donepezil), and neither has based arguments to primary care providers and insurers, as
serious adverse effects.51,52 well as patients, to support effective therapeutic interventions
that will achieve the goals of optimal physical and cognitive
REHABILITATION MANAGEMENT functioning, safety, and QOL.
For rehabilitation professionals managing people with
Overview MS, the International Classification of Functioning, Disabil-
Chronic neurodegenerative conditions, such as MS, result ity and Health (ICF) model (refer to Chapter 1) provides
in a loss of physical and cognitive function from the dean excellent framework for assessment and management
struction of neurons and from a lack of activation of the regardless of the setting in which the patient or client is
affected systems. People with MS experience physical and encountered.53 Although guided by the opening interview
cognitive impairments potentially leading to inactivity and and chart review, the initial assessment must include how
resultant deconditioning (Figure 19-5). This often becomes the individual with MS is functioning in home, at work, and
a cycle that is difficult to break. One question that frames in recreation environments and which impairments of bodily
the rehabilitation strategy chosen is whether the focus structure or function might be contributing to the identified
should be compensation for or restoration of lost function. activity limitations and participation restrictions. Rehabilita-
Compensation includes interventions such as wheelchairs tion professionals must consider how personal and environ-
or walkers to assist with mobility or braces for absent or mental factors may impede or facilitate achievement of
inadequate muscle power. Restoration is aimed at increas- rehabilitation goals. Personal factors in people with MS may
ing the capacity of the system—for example, maximizing include whether the patient is heat intolerant, experiences
cardiovascular endurance by increasing maximal oxygen MS-related fatigue, or has the confidence or motivation to
uptake or restoring full ROM. Therefore, prescribing programs, perform certain tasks. Environmental factors that may be of
particular importance for the patient with MS may be living
in a hot climate or having access to cooling equipment such
as air conditioning or cooling garments. It is critical to un-
Physical Impairments Cognitive Impairments derstand how the disease affects the lives of both individual
patients and their caregivers. Outcome measures designed
to test impairments, activity, and participation, along with
assessments of environmental and personal factors, will help
health care professionals understand the deficits of their
Inactivity
patients and determine the best place to focus rehabilitation
efforts and monitor the patient’s response to intervention.54
Because of the myriad CNS lesions and variable clinical
presentations in people with MS, there is no one approach
Deconditioning/Disuse that is the gold standard for rehabilitation management.
Whatever the approach, evidence is growing that rehabili-
Figure 19-5 n Interaction among impairments, inactivity, and tation is beneficial. Intensive inpatient therapy programs
deconditioning. People with multiple sclerosis often experience provide long-term improvement in a number of functional
physical and/or cognitive impairments that can lead to or be in- skills, participation, and QOL but may not change under-
creased by inactivity. Deconditioning and disuse can reduce activ- lying impairments. Prospective studies have shown that
ity levels or be caused by inactivity. This can become a cycle that intensive inpatient rehabilitation improves disability
is self-perpetuating. (Modified from Multiple sclerosis treatment: and QOL and that these benefits can be long lasting.55-58
impact on quality of life (Clinical monograph, p. 10): Proceedings High-intensity programs in the outpatient clinic or home
from Clinical Medical Education/Clinical Education Symposium environment offer evidence of short-term symptomatic
at the Consortium of Multiple Sclerosis Centers Annual Meeting. changes that have translated into improved participation
Washington, DC, June 2007.) and QOL.56,59
Assessment Scales.63 However, because these scales measure spasticity

The initial interview must include a quick screen or question- at rest, they may not reflect the degree to which spasticity
ing about the body systems and areas that are commonly may be interfering with function. Careful observation of the
impaired in people with MS and the problems commonly patient’s movements may also inform the clinician about
encountered: motor strength, coordination, spasticity, sensory how spasticity is affecting the patient’s ability to move.
disruption (vestibular, visual, and somatosensory), bladder Ataxia and Incoordination. Few standardized tests
control, depression, and cognition. If impairments are present, have been developed to specifically measure ataxia. One
there is a strong likelihood of negative impact on the patient’s recent test is the Scale for the Assessment and Rating
ability to perform ADLs or participate in activities related to of Ataxia (SARA). Although this test has yet to be validated
work, home, and leisure. All patients with MS must be asked in people with MS, it has good reliability and validity in
if they have fallen in the last 6 months because of the high patients with cerebellar dysfunction, a common problem in
rate of falling in people with MS.23-25,26 Results of the inter- people with MS.64,65
view and chart review will help develop hypotheses about Tests of nonequilibrium coordination are designed to mea-
which potential impairments might be contributing to the sure the presence of dysmetria or dysdiadochokinesia, both of
patient’s or client’s physical or cognitive dysfunctions. which occur in patients with MS. However, these tests (includ-
Therefore the examination needs to be designed to observe ing finger to nose, heel to shin) are somewhat subjective and
the problematic tasks and test the hypotheses developed. are therefore difficult to use to demonstrate improvement after
During the assessment, examiners must determine if the an intervention. However, using a stopwatch during these tests
problems identified by the patient (or those found by the as- can be an important tool to record objective data. Count the
sessor) fit within their scope of practice or whether the pa- number of repetitions of a given activity performed in a set
tient requires a referral to an appropriate health care profes- amount of time (e.g., how many alternating forearm supina-
sional. A good example is identifying people with depression tions and pronations can be performed in 30 seconds), or
using a quick two-question screen. According to Mohr and record the time it takes to complete a set number of repetitions
colleagues,60 these questions are 98.5% sensitive for identi- of a given activity (e.g., how long it takes to complete five
fying major depressive disorder. The two questions are alternating supination-pronation movements). Refer to
(1) “During the past 2 weeks, have you often been bothered Chapter 21 for additional assessment tools.
by feeling down, depressed, or hopeless?” and (2) “During Vestibular Dysfunction. The vestibular system is
the past two weeks, have you often been bothered by having affected by MS both centrally (lesions in the vestibular
little interest or pleasure in doing things?”61 An answer of nuclei or cerebellum) and at the entry site of cranial nerve
yes to either question should trigger a referral to the pa- VIII.66 However, benign paroxysmal positional vertigo
tient’s primary care provider for follow-up. (BPPV) can also occur.67 The techniques used to assess
The physical examination might then start with testing the and treat the effects of vestibular disorder in an individual
patient’s ability to perform functional activities that the with MS are the same as those discussed in Chapter 22.
patient or his or her caregivers have identified as problematic. When vestibular symptoms are present, Williams and col-
This might include performance of transfers, gait, ADLs, or leagues68 suggest evaluation using computerized platform
cognitive tasks as well as the specific activities that the person posturography (CPP) in people with MS with minimal to
states are compromised in his or her work, home, and recre- mild disability. It is important to keep in mind that the
ational life. There are several measures of QOL that also patient with MS will often have additional problems that
cover participation issues relevant to people with MS. might require modification of the vestibular intervention—
Comprehensive lists of standardized tests and measures for example, heat intolerance or additional visual or so-
for impairment, activity limitations, and participation re- matosensory deficits.
strictions or QOL are provided in Chapter 8. The next Fatigue. Identifying if and when fatigue occurs in indi-
section of this chapter will primarily focus on the tests and viduals with MS is important to assessment and the structuring
measures found to be valid and reliable in the examination of intervention. Questions should address the type of fatigue,
of individuals with MS. whether mental or physical; when during the day it occurs;
whether it is related to physical or mental exertion; and what
Assessing Body System Problems Contributing to the person with MS does, if anything, to relieve it. In addition,
Activity Limitations fatigue-related self-report scales can help the rehabilitation
In general, standardized methods of examining muscle professional gain an understanding of the perceived impact
strength and endurance, somatosensation, vision, coordi- that fatigue may be having on a patient with MS. Two of the
nation, cardiovascular status and endurance, posture, mus- commonly used scales are the Modified Fatigue Impact Scale69
cle tone, reflexes, ROM, pain, and cognition are useful in and the Fatigue Severity Scale.70 These measures may also aid
examining patients with MS. As with many neurological the therapist in determining if the intervention had any impact
conditions, abnormal posturing or pain may necessitate on the patient’s perceived level of fatigue.
using nonstandardized test positions or methods that must Cognition. The PASAT,71 recommended by an expert
be noted in the patient documentation. If a patient is panel of the National MS Society, is a test for cognitive
unable to attain the normal test position while performing impairments in people with MS. A more recent test, the
a muscle strength test, the assessed strength is noted along Audio Recorded Cognitive Screen (ARCS),72,73 appears to
with the position in which the muscle or muscle group be a more comprehensive cognitive assessment developed
was tested. for people with dementia but the psychometric properties
Spasticity. Spasticity can be measured using resistance have not yet been determined in people with MS. However,
to passive ROM and Ashworth62 and Modified Ashworth Lechner-Scott and co-workers74 found that compared with
the PASAT, the ARCS was similar in detecting impairments anticipatory postural reactions, reactive postural responses,
of cognition and more sensitive at identifying problems with sensory orientation, and stability in gait. Both interrater reli-
memory or executive impairments. ability in people with parkinsonism and content validity are
good, but testing in other populations has not yet been com-
Assessing Activity Performance and Participation pleted. There is an abbreviated version of the BESTest, the
Outcome measures assess the ability of an individual to mini-BESTest,88 that covers four of the six systems, focus-
perform an activity or task as well as assess the perception ing on dynamic balance. These promising tests may offer
of the person to use those tasks to fulfill life roles. Following the clinician a better way of identifying which components
are activity and participation measures commonly used in of orientation and postural control are dysfunctional, which
people with MS. An individual’s perceived ability to partici- may allow more targeted interventions.
pate may also be included in some QOL outcome measures The Activities-specific Balance Scale (ABC)89 is a ques-
that are included in the following sections. tionnaire that rates people’s self-perception of how confi-
Balance. Balance is foundational to upright movement dent they are to perform activities that challenge their bal-
and is produced by a complex interaction among sensory ance. The Dizziness Handicap Inventory (DHI)90 assesses
inputs, central processing, and motor responses. It can be three domains of disability related to dizziness: physical,
discussed under both body structure and function or emotional, and functional. The sum score or each subscale
activity. In either case balance dysfunction has been identi- score can be reported. Higher scores mean greater levels of
fied in people with MS with minimal as well as more handicap and disability. Cattaneo and colleagues91 found
advanced disability.75-79 Cameron and Lord80 report the that both the ABC and DHI tools discriminated between
three most common problems with balance to be delayed fallers and nonfallers and were therefore good predictors of
response to postural perturbations, increased body sway fall status in people with MS. Refer to Chapter 22 for addi-
while standing quietly, and an inability to move outside the tional information on balance.
base of support. Gait. Gait can be measured in myriad ways depend-
Whereas some balance tests focus on stationary or static ing on the goal of the assessment. Speed, distance, and
tasks that allow observation of body sway in standing, in- quality may all be important to the patient and therapist.
cluding single-leg stance test, Romberg test with eyes open Observational gait analysis is the gold standard for
or eyes closed, tandem stance, and CPP, others add move- clinical measurement of gait quality. Although motion-
ment and challenge dynamic balance (Functional Reach analysis laboratories are able to provide detailed kinetic
Test,81 Tinetti Performance-Oriented Mobility Assessment and kinematic assessment of joint angles and gait cycle,
[POMA],82 and Berg Balance Scale [BBS]83). Other tests it is costly and typically not available in most clinical
challenge anticipatory balance (reactions to perturbations settings. Instrumented mats such as the GaitRit can provide
related to self-generated movement) or reactive balance clinicians with temporal and spatial gait parameters such
(perturbation tests, CPP). Frzovic and co-workers84 found as step length, step width, cadence, and single-leg sup-
that single-leg stance, tandem stance, response to external port and double-leg support times. Although this is less
perturbations, and the Functional Reach Test were able to costly than motion analysis, it may still be out of reach
distinguish people with MS from healthy controls. for many clinics. Gait speed and velocity can also be
Several authors have studied measures of balance in measured by having the patient walk a given distance
people with MS. Cattaneo and colleagues85 determined that while being timed. These walks can occur at a self-
four tests measuring balance during standing and gait and selected pace or as fast as the person can walk safely.
self-perception of balance had good intrarater and interrater Several short-distance timed tests exist, the 25FTW
reliability. The two tests measuring balance during standing and the timed 10-meter gait test,92 both of which have
and movement were the BBS and the Dynamic Gait Index been shown to have good reliability and sensitivity to
(DGI). change.93,94 The 6-minute walk test (6MWT) measures
CPP provides an objective assessment of sensory contri- walking endurance and is recommended by the NMSS
butions to balance dysfunction in people with MS.86 In par- Task Force on Clinical Outcome Measures as a measure
ticular, the Sensory Organization Test is useful in identify- of walking ability that is sensitive to change. Gijbels and
ing the relative sensory contributions (visual, vestibular, and co-workers95 report that the 6MWT was better at predict-
proprioceptive) to stationary balance and response to pertur- ing habitual walking in people with mild to moderate MS
bation. Understanding the sensory conditions under which than the 25FTW. However, the 25FTW may be more sen-
the patient loses balance and falls assists the therapist in sitive to change when compared with the EDSS.96 The
providing exercises that will challenge those conditions in a 6MWT distance was reduced in people with MS com-
safe and controlled manner. For example, the patient who pared with healthy controls and was inversely related to
relies heavily on visual input to maintain balance (condi- disability.97
tions with eyes closed in the Sensory Organization Test) Two additional performance-based tests, the DGI and the
would be provided exercises and activities that challenge the Timed Up-and-Go Test (TUG), combine walking with other
vestibular and proprioceptive systems, such as standing on functional tasks. The DGI measures the ability of an indi-
foam while the eyes are closed. vidual to walk while adding various challenges such as
Developed by Horak and colleagues,87 the Balance Eval- slowing down or speeding up, head turning, stepping over or
uation Systems Test (BESTest) is an instrument examining around obstacles, and stair climbing. It was developed to
complex balance disorders that includes the six domains that assess gait dysfunction associated with peripheral vestibular
underlie orientation and postural stability: biomechanical disease.98 McConvey and Bennett99 found the DGI to be a
constraints, stability limits and verticality, transitions and reliable and valid tool for use in people with MS. The TUG
test combines walking with transfers and turning. It is fre- 45 minutes to administer the complete set of questionnaires
quently used in both clinical and research settings and has and does not provide a sum score for all tests. There is good
been shown to be reliable in measuring function in people test-retest reliability for the MSQLI even in people with MS
with MS.93 and cognitive dysfunction.108 A shortened version of the
The Multiple Sclerosis Walking Scale–12 (MSWS-12) is tool exists, but the psychometric properties have not been
a 12-item patient-rated questionnaire that measures the per- thoroughly tested.
ception of the impact of MS on walking ability. This scale
has good reliability and validity and may be very useful to Disease Severity Measures
document patient perceived change in walking ability before Disease severity is a measure of disablement. Interventions
and after intervention.100,101 that change function (e.g., improve walking distances or
Upper-Extremity Tests of Function. Movement im- decrease reliance on assistive devices to move) can reduce
pairments of the upper extremities can result in decreased disability. There is also compelling evidence that exercise
ability to perform ADLs and other functional activities. may actually modify disease progression in people with MS.
Standardized tests such as the Box and Block Test (BBT)102 Therefore disease progression may be used to assess the
or the NHPT103 provide objective data about unilateral impact of an intervention on the patient’s perceived level of
manual dexterity or the ability to manipulate objects. Both disability. Although the EDSS43 is the gold standard for as-
tests are inexpensive but do require some equipment and a sessing disease severity, it requires a trained primary care
stopwatch. The NHPT is part of the MSFC and therefore has provider to administer. Disease Steps111,112 and Guy’s Neu-
been used extensively in evaluating people with MS. rological Disability Scale (GNDS)109 are two additional
Composite Tests. An expert panel of the NMSS rec- disability scales that have demonstrated good correlation
ommended the use of the MSFC,44,104 including the 25FTW, with the EDSS. Whereas Disease Steps must be adminis-
the NHPT, and the PASAT. The MSFC has been tested tered by a professional, GNDS can be given to patients to
against lesion load as measured via MRI, EDSS scores, and complete on their own.110
QOL measures, showing that it has good validity and reli-
ability and is sensitive to change.104-106 Each component Interventions
scale of the MSFC can also be used independently to The goals of rehabilitation for persons with MS are to
monitor physical and cognitive function as written previously. maximize and maintain function and prevent complications
so that they can participate fully in all aspects of their lives.
Assessing Quality of Life The variable presentation that people with MS can manifest
QOL measures are patient-report tools that evaluate the requires rehabilitation professionals to be flexible and cre-
value a person places on his or her abilities and limitations ative. The plan of care developed to manage a patient must
and how these affect the individual’s social, emotional, and be linked to the impairments, activity limitations and par-
physical well-being. Many of these tools include questions ticipation restrictions identified during the assessment. Re-
that address an individual’s perception of how well he or search provides evidence for the most effective interventions
she is able to fulfill life roles and how the disease affects and must be coupled with the desires and needs of the indi-
this participation. In a meta-analysis of exercise training on vidual with MS. The rehabilitation program must be negoti-
QOL in people with MS, Motl and Gosney107 found that ated with the patient/client in consultation with caregivers
disease-specific measures of QOL detected larger changes when available or appropriate.
than generic QOL measures. Several measures have been The National Clinical Advisory Board of the National
commonly used to evaluate people with MS: the Multiple MS Society recommends that rehabilitation occur when-
Sclerosis Quality of Life–54 (MSQOL-54)113 and the Mul- ever there is a sudden or gradual decline in function or
tiple Sclerosis Quality of Life Inventory (MSQLI).114 The an increase in impairment that has a negative impact on
multidimensional MSQOL-54 was based on the Health an individual’s safety, independence, mobility, or QOL.
Status Questionnaire (SF-36), with 18 additional items spe- In addition, it is recommended that rehabilitation be a part
cific to MS covering fatigue, and cognitive and sexual of a comprehensive health care plan at all stages of the
functioning. There are 12 subscales that cover physical disease.115
function, role limitations—physical, role limitations— Regardless of the type of intervention chosen, evidence
emotional, pain, emotional well-being, energy, health per- is growing that increased activity, whether cognitive or
ceptions, social function, cognitive function, health dis- physical, may have a neuroprotective effect on the brains
tress, overall QOL and function, and change in health. The of people with neurological insults. In fact, Golzari and
measure takes about 15 minutes to complete and requires colleagues116 demonstrated that an 8-week, 24-session,
15 to 20 minutes to score. Reliability is good to excellent in combined exercise program improved muscle strength and
people with MS.113 balance and reduced disability in people with MS. In this
The MSQLI was developed by the Consortium of Mul- study, levels of proinflammatory immune system mediators
tiple Sclerosis Centers Health Research Subcommittee in were measured before and after the intervention. The au-
1997. It is composed of 10 components covering issues im- thors demonstrated that this dosage of exercise reduced
portant in MS. It includes the Health Status Questionnaire, markers of inflammation in the blood. This is one of the first
Modified Fatigue Impact Scale, MOS Pain Effects Scale, studies in people with MS showing that inflammation and
Sexual Satisfaction Survey, Bladder Control Scale, Bowel therefore the disease process may be altered by the applica-
Control Scale, Impact of Visual Impairment Scale, Per- tion of an exercise intervention, suggesting a role for reha-
ceived Deficits Questionnaire, Mental Health Inventory, bilitation in neuroprotection and not simply symptom man-
and MOS Modified Social Support Survey. It takes about agement. This also implies that rehabilitation, specifically
exercise, should occur early in the course of the disease and each individual. In addition, a number of health providers
not only after clinical disability has occurred. However, can be members of the rehabilitation team, including
the exact dosage, intensity, or type of exercise required to nurses, occupational therapists, physical therapists, speech-
produce activity-dependent neuroplasticity is not yet known. language pathologists, psychologists, neuropsychologists,
At least one study in an animal model of MS, experimental and physicians.
allergic encephalomyelitis, has shown the beneficial effects
of exercise.117 Exercise
In prescribing a rehabilitation program for persons Historically, exercise was thought to worsen disability and
with MS, each individual’s level of fitness and physical bring on exacerbations. Medical advice warned patients
and cognitive resources including memory, judgment, that overexertion could hasten relapse and progression.
strength, endurance, spasticity, balance, and coordination There now exists clear evidence that this is not the case.
must be taken into consideration. In addition, therapists Regular, appropriate exercise has been shown to increase
must investigate the person’s level of fatigue and heat strength, aerobic capacity, overall function, and QOL. In
sensitivity. If present, these factors will require modifica- 1996 Petajan and colleagues published a seminal study in
tion of the rehabilitation program, including where the which a 60% V̇o2max aerobic ergometer exercise program
activity is performed, in what environment, and the time was well tolerated in people with MS and did not provoke
of day in relation to fatigue level and the other tasks the remission.118 After 10 weeks, participants had improve-
individual must perform. In other words, to be successful, ments in V̇o2max, work capacity, isometric strength, and
the rehabilitation program must fit into the framework of blood lipids and reduced depression, anger, and fatigue. In
the person’s life. a 2009 systematic review of the literature,119 exercise was
Rehabilitation can occur in a variety of locations: inpa- shown to be an effective intervention for people with MS
tient, outpatient, home, and the community. Figure 19-6 to improve muscle strength, endurance, mobility-related
shows a physical therapy–led community-based exercise actions, and to a lesser extent mood compared with control
program for people with MS in which group activities ad- conditions. This evidence did not suggest the superiority of
dressing strength, balance, and endurance are modified for one particular type of exercise program over others. It is
very important to note that adverse effects were rarely seen
in any of the exercise studies, and when they did occur
they did not last for longer than 24 hours, indicating that
exercise is safe for people with MS.
In a review of the exercise literature, White and
Dressendorfer120 recommend that endurance exercise pro-
grams for people with MS with mild to moderate disabil-
ity use the following guideline: perform regularly, two or
three sessions per week, at an intensity of 65% to 75%
heart rate maximum, and last 20 to 30 minutes per session.
Resistance exercise should include 15 to 18 repetitions for
one to three sets initially with a goal of increasing to three
to four sets. Training should last at least 12 weeks.121 Ow-
ing to heat intolerance, exercise should incorporate inter-
mittent rest periods that allow heat to dissipate.120 Heesen
and colleagues122 developed a guideline for exercise
prescription for people with MS for all levels of disability
(Table 19-3).
Prescribed early in the course of the disease when mild
to moderate disability is present, exercise can be used to
restore function by reducing physical or cognitive decline
from disuse or deconditioning. As clinical disability ac-
crues in the later stages of the disease, exercise may then be
used to compensate for missing function or prevent second-
ary complications—for example, stretching hip adductor
muscles with decreased range of motion to allow adequate
personal hygiene to occur.
Evidence-Based Interventions for

Specific Problems
Fatigue
Fatigue is one of the most frequent and disabling symptoms
Figure 19-6 n Community exercise class for individuals with associated with MS and is best managed with a multidisci-
multiple sclerosis. A physical therapy–led community exercise plinary team composed of physicians, physical therapists,
class is shown. Participants perform group strengthening and bal- occupational therapists, and nurses. As described earlier, the
ance activities that are modified for each individual. causes of fatigue can be divided into two basic categories:
TABLE 19-3 n GENERAL EXERCISE GUIDELINES FOR LEVELS OF DISABILITY
LEVEL OF DISABILITY EDSS LEVEL TRAINING PROGRAM

None: no fatigue or thermosensitivity 0 Full exertion, aerobic and resistance exercise, no extreme sports
Minimal: limited fatigue and heat sensitivity; 1-2 Monitored exercise program including strengthening and
minor balance or gait problems endurance using a variety of exercise types, precooling if
heat-sensitive, avoid overtraining
Moderate: limited gait; may have spasticity, 3-5 Deficit-driven exercise protocols including strengthening and
weakness, ataxia, balance problems endurance training using methods tolerated, walking, cycle
ergometry, precooling if needed
Severe: cannot participate in all daily 6-7 Movement preservation, stretching, targeted strengthening
activities; short-distance, aided walking only needed for task-specific training
Bedridden 8-9 Primarily passive movements to maintain motion, breathing
exercises
Modified from Heesen C, Romberg A, Gold S, Schulz KH: Physical exercise in multiple sclerosis: supportive care of a putative disease-modifying treat-
ment. Expert Rev Neurother 6:347–355, 2006.
EDSS, Expanded Disease Severity Scale.
primary and secondary. Primary fatigue related to demyelin- Practice Guidelines128 as energy effectiveness and includes
ation and neurodegeneration may have fewer options an analysis of individuals’ home, work, and leisure activities
for treatment. Secondary fatigue caused by deconditioning, and the environments in which they occur in order to de-
comorbidities, depression, poor nutrition, heat intolerance, velop activity modifications designed to reduce fatigue. This
sleep disturbance, and medications may be more easily can include a variety of strategies such as reducing energy
managed. Several strategies for fatigue management have expenditure through activity and modification, workspace
been reported and show promise; however, few research organization and improving efficiency of movements;
studies have demonstrated effectiveness in randomized con- balancing work and rest periods; delegating tasks; evaluat-
trolled trials or in comparisons among approaches. Interven- ing standards and prioritizing activities; and using assistive
tions for fatigue management include cooling devices, en- technologies that conserve energy usage.129,130 In a random-
ergy conservation education training, exercise, and a ized controlled trial, a 6-week community-based energy
multifaceted class aimed at teaching people with MS how to conservation class using the strategies listed previously was
manage their fatigue. compared with a wait-list control group. Immediate post-
One study found that the cooling suit was shown to course improvements in fatigue were noted129 and were
improve all dimensions of fatigue on the Fatigue Impair- present after a 1-year follow-up period.131
ment Scale (physical, cognitive, and psychosocial) in a The multidimensional fatigue management class “Fatigue:
small multiple-case study.123 Although recommended in the Take Control” was developed based on the recommendations
clinical practice guidelines on fatigue and MS by expert of the Fatigue Management Guidelines of the NMSS from
opinion and anecdotal reports of people with MS, little ad- 1998.132 The content of the 6-week class includes many of the
ditional evidence exists to support cooling as a therapeutic aspects of fatigue management education and training that
intervention. Two additional studies have shown that cool- were described previously. The pilot study found that partici-
ing garments can reduce symptoms of fatigue and improve pants had less fatigue compared with a wait-list control
ambulatory ability.124,125 group.132 These classes are often offered by local chapters of
Exercise shows promise as an intervention that can the NMSS.
improve fatigue for people with MS that may improve Patients may need to be prescribed assistive devices for
muscle weakness caused by disuse and deconditioning. ADLs. People with MS who have spasticity have a greater
However, no one type of exercise, resistance or aerobic, or cost of walking.133 Using wheeled mobility for longer-
program has been proven most effective. One program distance outings (to the shopping mall, an extended event,
included a 5-day-per-week, 30-minute bicycle aerobic on vacation) can conserve energy and extend the time a per-
training program for 4 weeks that improved fitness and son can participate in activities of importance to him or her.
showed a tendency for reduced fatigue. This study had an However, therapists should be aware that using assistive
age, sex, and activity level control group.126 Di Fabio58 devices such as walkers or crutches actually increases en-
showed that a prolonged outpatient rehabilitation program ergy expenditure for elderly people,134 and therefore the
in patients with progressive MS led to a decrease in MS- need for improved support must be balanced with the in-
related symptoms, including fatigue. However, there was creased energy burden an assistive device might add.
no control group. A randomized study comparing bicycle
training with yoga found that fatigue improved in both Spasticity
groups, with neither group shown to be better than the Several rehabilitation strategies to manage spasticity are
other.127 available, including ROM, stretching, light pressure or
Energy conservation is defined by the fatigue and MS stroking,135 cold therapy, electrical stimulation, and educa-
guidelines of the Multiple Sclerosis Council for Clinical tion. Although none of these interventions is supported by
strong research evidence, many are used routinely in clinical produce a wide variety of potential impairments that can
practice (ROM, stretching). Other approaches (cold therapy, adversely affect gait. In a review article by Kelleher and
light pressure or stroking) are recommended for use in con- colleagues,141 imbalance, fatigue, spasticity, incoordina-
junction with stretching or ROM programs. Regardless of tion, muscle weakness, and sensory system impairments
the technique employed, educating individuals and caregiv- were all reported to negatively affect ambulation ability.
ers about the importance of adhering to a spasticity manage- Therefore addressing each of these impairments has the
ment program is essential. The Multiple Sclerosis Council potential to improve gait. A recent literature review of
for Clinical Practice Guidelines18 recommends, based on therapeutic interventions for mobility problems suggests
expert opinion, stretching a muscle with spasticity for that a variety of different methods can be used to improve
60 seconds or longer or using a prolonged stretch, lasting ambulation.142 Snook and Motl143 performed a meta-
hours, with braces or splints. analysis of exercise studies aimed at improving walking
Cold can be applied in a number of ways: baths, towels, mobility in people with MS and found that greater effects
or cooling garments. There are multiple quasi-experimental were associated with supervised exercise training, pro-
research studies that suggest an improvement in spasticity grams of less than 3 months’ duration, in mixed samples of
for a brief period after cooling18; however, the number of people with RRMS and progressive MS.
subjects and study methods make these results equivocal. Task-specific gait training has been evaluated in people
Nilsagård and co-workers136 found subjective reports of with MS. A randomized controlled trial compared two differ-
improved spasticity after a single session of cooling, ent treatment groups—facilitation and task-specific training—
although no statistically significant differences in spasticity that each received 15 to 19 1-hour treatment sessions over 5 to
measures were found. 7 weeks and found that both improved 10-m gait speed, stride
length, and balance; however, there was no control group.144
Balance and Postural Control Treadmill training has been investigated in several small, pilot
Balance is foundational to the ability to stay upright and or case studies with promising results of improved QOL,
perform dynamic movements. It is a frequent problem in energy expenditure, and gait parameters.145-147
people with MS and results in a person limiting his or her Several exercise studies have an association with im-
participation in home, work, and leisure activities. Abnor- proved gait. A combined resistance and aerobic home pro-
malities of balance along with cane use and poor perfor- gram lasting 23 weeks improved gait speed for short and
mance on tests of balance and ambulation can increase the longer distances in exercise compared with a control group.148
risk of falling.26 Other fall risk factors that have been iden- Rampello and co-workers149 compared a neurorehabilitation
tified include fear of falling, male sex, poor concentration program with an aerobic training program of similar duration
or forgetfulness, and urinary incontinence.25 Rehabilita- (three times per week for 8 weeks). The authors found that
tion programs must be based on a thorough understanding aerobic training improved walking distances and speeds and
of the impairments and personal and environmental fac- measures of aerobic capacity over the neurorehabilitation
tors that may be contributing to the balance dysfunction. group. Both groups had QOL improvements in emotional
Cattaneo and co-workers137 compared the effects of three well-being and health distress; the neurorehabilitation group
balance interventions on falling and other measures of demonstrated improved mental health.
balance. Three rehabilitation groups were included: one in An additional technique that shows promise for improv-
which motor and sensory strategies were targeted, the ing mobility in people with MS is an evaluation and inter-
second focusing on motor strategies alone, and the third vention approach that uses small amounts of weight placed
group not receiving balance-specific training. The greatest on the torso in response to identified balance dysfunction.
reduction in falls and improvement on the BBS were Balance-Based Torso-Weighting (BBTW) is an intervention
associated with group one, and the least with group three. that uses directional loss of balance in both static and dy-
Hayes138 compared 12 weeks of standard physical therapy namic assessment to determine where small amounts of
with high-intensity resistance exercise (60% to 80% weight (generally less than 1% to 1.5% of body weight)
maximal contraction) added to standard therapy and found are placed in a treatment orthotic called BalanceWear. The
that standard therapy produced better balance outcomes. BalanceWear orthotic can be worn during the performance
In addition, strength and the ability to ascend and descend of activities in therapy or daily for home, work, or leisure
stairs were all better in the standard therapy group. Impor- activities. A recent randomized controlled trial in people
tantly, people with MS tolerated the high intensity resis- with MS who reported gait abnormalities showed that when
tance exercise without problems. One pilot study found wearing the weighted BalanceWear orthotic participants
that a 12-week, biweekly aerobic exercise program did not increased their gait speed compared with no weight controls,
improve balance as measured by the Functional Reach and improved TUG scores compared with a standard
Test but did result in an improvement in walking dis- weighted control.150
tance.139 For additional intervention strategies on balance, When people with MS do not respond to therapeutic in-
refer to Chapter 22. terventions to restore function, mobility assistive devices
such as canes, crutches, walkers, wheelchairs, and scooters
Mobility are used to enhance mobility through compensation. Mobility-
People with MS rate gait as one of the most important assisted technology (MAT) can improve function in people
bodily functions122; gait is often adversely affected in peo- with moderate to severe impairments of ambulation and
ple with MS. Gait disturbances have been observed in may reduce activity limitations and participation restric-
people with MS even before disability is measured on the tions by reducing fatigue and enhancing energy conserva-
EDSS scores.140 Lesions in the brain and spinal cord tion to allow greater involvement in work, family, social,
vocational, and leisure activities. Other MAT technologies people cope with and adjust to their impairments.156,157 How-
include functional electrical stimulation (FES), neuropros- ever, the evidence is not yet convincing for specific programs
theses, and orthotics. FES is applied to specific muscles or addressing attention and executive functioning. O’Brien156
muscle groups to activate weak muscles. Some of these was able to recommend the use of a modified story technique
stimulators can be built into a neuroprosthesis that can be to address learning and memory deficits in people with MS. In
set up for use during exercising or walking.151 Orthotics a systematic review Maitra158 found that cognitive behavioral
such as the ankle-foot orthosis (AFO) or hip flexion assist therapy programs performed by occupational therapists were
orthosis (HFAO)152 can compensate for muscle weakness in positively correlated with improvement in Functional Indepen-
the lower extremity, improve foot and knee positioning, and dence Measure (FIM) scores. Refer to Chapter 27 for addi-
reduce energy expenditure. Therapists often work coopera- tional information regarding interventions with individuals
tively with orthotists to ensure proper fit. Use of wheeled with cognitive problems.
mobility devices such as a manual wheelchair, power
wheelchair, or scooter requires a formal evaluation by an Dysphagia and Dysarthria
occupational or physical therapist with justification that it is Dysphagia or difficulty with chewing and swallowing be-
required for mobility at home at least on a part-time basis. comes more prevalent in people with MS as the disease
Therapists must take a long-term view of the projected progresses.159 Therapists facilitate proper swallowing with
needs of the patient when prescribing wheeled mobility, as exercises that will improve posture to prevent aspiration and
most insurance companies will replace this equipment only strengthen muscles of mastication. Other interventions may
every 5 years. include diet modifications and education for the patient and
his or her family or caregivers. Dieticians may be consulted
Pain and Dysesthesias to facilitate proper food choices.
The occurrence of pain in people with MS is often underes- Dysarthria from the disruption of muscular control in the
timated. Pain can be acute, as in optic neuritis or Lhermitte central and peripheral speech mechanisms leads to abnor-
syndrome, or chronic, as in dysesthesias in the limbs or malities of speed, range, timing, strength, sound, and accu-
joints or mechanical pain related to abnormal positions or racy of speech movements. Speech-language pathologists
repeated movements that cause abnormal wear and tear on determine therapy programs that take into consideration the
the musculoskeletal system. Occupational and physical ther- stage of the disease and speech quality. Typical programs
apists can address poor body mechanics and weakness and may include exaggerating articulation, increasing voice
poor movement patterns with retraining, and soft collars volume, and increasing strength of oral musculature. Exer-
may help reduce Lhermitte syndrome. However, little cise programs designed to increase respiratory muscle
evidence supports these interventions.153 Transcutaneous strength have not been successful in improving voice quality
electrical nerve stimulation has been suggested anecdotally or production.160
by Kassirer154 as beneficial for reducing pain. Cognitive-
behavioral therapy has been researched for managing chronic SUMMARY
pain155; however, little evidence exists for using it in people This chapter has focused on the pathophysiology, clinical
with MS. presentation, medical management, and rehabilitation of
people with MS. Understanding the type of MS, clinical
Bladder Dysfunction disability, and stage of the disease will help therapists
Urinary incontinence and retention are common and often determine the best assessment and intervention strategies
embarrassing problems for people with MS. Patients may be for management of the rehabilitation program. Using
advised to avoid bladder irritants including caffeine, alcohol, the ICF framework will facilitate the assessment of the
concentrated urine, and infection. Physical therapists may impairments, activity limitations and participation restric-
work with patients to assess the factors contributing to blad- tions affecting patients and clients. In addition, including
der dysfunction by retraining hyperactive or weak pelvic the environmental and personal factors present will help
floor muscles using biofeedback techniques and exercise. tailor the program to the patient’s needs. Using QOL
Nurses may need to teach patients with urinary retention in- measures developed for people with MS should help the
termittent catheterization. Refer to Chapter 29 for additional therapist understand the entire range of problems that
information on pelvic floor dysfunction and its treatment. patients may have.
Many websites are available to assist therapists and their
Cognition patients with MS to understand the disease and find resources
Strategies for managing cognitive impairments include com- to help them manage the disease. The National MS Society
pensation techniques such as memory notebooks, diaries, (www.nationalmssociety.org) and the Multiple Sclerosis
calendars, and computer-assisted programs for memory, at- Foundation (www.msfocus.org) are both excellent resources.
tention, or other executive functions. Neuropsychologists,
speech-language pathologists, and occupational therapists References
can all direct cognitive rehabilitation programs. Strategies To enhance this text and add value for the reader, all references
for coping with cognitive impairments are often shared with are included on the companion Evolve site that accompanies
the other members of the health care team for reinforcement this textbook. This online service will, when available, provide
with patients. a link for the reader to a Medline abstract for the article cited.
There is growing evidence to support psychological inter- There are 160 cited references and other general references for
ventions for people with mild to severe MS-related cognitive this chapter, with the majority of those articles being evidence-
deficits, aimed at alleviating depressive symptoms and helping based citations.
CASE STUDY 19-1

INITIAL INTERVIEW PLAN OF CARE AND GOALS
Mrs. P. is a 54-year-old woman with a 28-year history of Mrs. P. had weakness in B LEs, balance problems, and an
RRMS. She was first diagnosed after her third daughter was unsteady gait with an increased risk of falling, interfering with
born and remembers having a lot of trouble walking. Mrs. P. is her functional mobility and QOL. The physical therapy plan
concerned about her trunk weakness, back pain, and difficulty included balance and gait training, improved posture and time
with walking; she often stumbles, especially when she does standing, and increased endurance and cardiovascular fitness.
not use her single-point cane. She reports having fallen twice Goals included a decreased fall risk with an improved Tinetti
in the past year when she lost her balance and was unable to score of 25/28, improved B LE and trunk strength (4/5 in all
catch herself. One fall was at home, and one in the backyard. muscle groups), improved endurance to stand and walk to 30 to
Therefore she has been using the cane more, especially on days 45 minutes, decreased back pain to 0 to 1/10 on most days, and
when she feels off balance. Mrs. P. is limited to 10 minutes of improved endurance and cardiovascular fitness to 45 minutes to
walking and standing secondary to trunk fatigue and difficulty 1 hour in 12 to 24 weeks.
balancing. She is overweight and reports some bladder inconti- INTERVENTION
nence and heat sensitivity. Mrs. P. is a homemaker with 4 BBTW placement of 1.5 pounds of weight to the torso to
children; the youngest is 11. Leisure activities include playing address the perturbation and rotational asymmetries (posterior
the piano, singing, doing the Wii balance exercise for 20 min/ right upper and lower trunk and anterior near navel) was effec-
day, and doing 10 minutes of treadmill walking at 3.0 mph tive in reducing her kyphotic posture and impaired reactive
after using a cooling vest. After her treadmill walking, she feels balance control. The rigid component of the BBTW resolved
fatigue for 3 to 4 hours. Recently she has noted having more back pain immediately; she felt better breath control and trunk
difficulty with singing and at times feels out of breath. Mrs. support, and her Tinetti balance 15/16 and gait 12/12 5 27/28
P’s goals are improved posture, better breath control, no back significantly improved. TUG score remained the same, but she
pain, the ability to walk without stumbling or using a cane, and no longer needed her UE to stand up. Her posture also
the ability to keep up with her children and her busy life. improved to 50% less kyphosis. During gait while weighted, a
ASSESSMENT softer (inaudible) right heel strike was noted, and her step
Mrs. P.’s Disease Steps classification is 3 (she uses a cane inter- length was even. Mrs. P. expressed that she felt much steadier
mittently and is able to walk for 100 feet without it), and her and more balanced with the BBTW BalanceWear vest and was
EDSS score is 6.0. Vital signs are within normal limits (WNL) thrilled to have no back pain.
at rest and for exercise. This patient is cognitively intact and Mrs. P. was seen in a managed care setting and was able to
reliable in her response to questions. Her active and passive make significant progress with her physical therapy program. She
ROM is WNL throughout her extremities, trunk, and neck. She was seen in physical therapy (13/week 3 1 month, 23/week 3
has selective motor control with normal tone. Manual muscle 2 months, 13/month 3 3 months) to improve her posture,
tests of bilateral upper extremity (UE) were normal, with the strength, balance, and fitness. Breath-control exercises using her
lower extremity (LE) 4/5 except for right hip flexion 31/5, hip diaphragm were implemented to improve singing, monitoring
extension-abduction and plantarflexion 3/5. Abdominals 2/5, progress with an inspiratory spirometer. Because this patient
back extensors 3/5 (able to lift trunk against gravity through understood the principles of exercise, she was advised to perform
full range with difficulty and unable to take resistance). the specific exercise until she experienced a decrease in the
Sensation to light touch (LT), pain, and proprioception are in- quality of movement or the muscles fatigued. A general stretching
tact throughout except for bilateral (B) feet, noted to have program was initiated, including specific stretches to improve her
diminished sensation to LT. In sitting her posture is extremely posture in sitting and standing. For strengthening exercise she
slumped (from 30 to 45 degrees when fatigued) with notable started with one set of 8 to 15 reps; resistance was increased by
thoracic kyphosis. She requires standby assist from supine to 2% to -5% when 15 reps were efficient for major muscle groups
prone secondary to trunk weakness and instability. She requires including hip flexion-extension-abduction, heel raises for plantar-
use of B UEs in weight bearing to move from sitting to stand- flexion, rowing (shoulder retraction) with yellow Thera-Band in
ing. During observational gait analysis, she demonstrates an standing, curl-ups in hook-lying, and quadruped weight shifts,
asymmetrical step length with the left longer than the right and which were performed 23 to 33/week. To address her
a right heel strike that is notably loud or audible. Her TUG deconditioning, an interval treadmill-training program was imple-
score is 8 seconds using B UEs to stand up. Tinetti balance mented on alternating days. Recommendation was to maintain
(POMA) 5 14/16 and gait 5 6/12 for a total score of 20/24 the intensity of 3.0 mph at 65% to 75% of HRmax and do inter-
(19 to 24 risk for falls). Single-limb stance on the right 5 4 vals of four blocks of 3 minutes with 1- to 2-minute rest breaks in
seconds and left 5 6 seconds, tandem stance 5 4 seconds. between and to continue to use the cooling vest before exercise.
Perturbation tests reveal loss of stability with an anterior nudge She continued with the Wii balance program, increasing to
(posterior loss of balance [LOB]), posterior nudge (anterior 30 minutes on the days she was not doing the treadmill. On those
LOB), and lateral and upper and lower trunk (LOB to opposite days she also performed specific balance exercises in the corner,
side). Rotational resistance tests to the right upper and lower beginning with her eyes open (single-leg stance; tandem stance).
trunk result in a stepping response, and the patient is unable to Mrs. P. was advised to use the BBTW rigid vest for 2 hours
maintain stability, resulting in a stepping response. Results during functional activities and walking and to perform her
of rotational resistance tests to left upper and lower trunk are exercises with it every other day. If she became less steady, she
normal. was advised to wear the vest an additional 1 to 2 hours per day.
CHAPTER 20 Basal Ganglia Disorders
MARSHA E. MELNICK, PT, PhD

Basal ganglia After reading this chapter the student or therapist will be able to:
Dystonia 1. Describe the circuitry of the basal ganglia.
Huntington disease 2. Relate the anatomy and physiology of the basal ganglia to its roles in sensorimotor and
Parkinson disease cognitive processes.
3. Use the information on anatomy, physiology, and pharmacology to explain the signs and
symptoms seen in classic disease states—for example, Parkinson disease, Huntington
disease, and dystonia.
4. Develop an evaluation plan for patients with diseases of the basal ganglia.
5. Develop an intervention plan for patients, with the rationale for treatment methods.
6. Determine treatment effectiveness, especially in the case of degenerative disease.
7. Integrate the information in this chapter with the information provided in Section I of
this book to develop treatment plans for patients with metabolic or toxic disorders.
T his chapter considers the degenerative, metabolic, Anatomy

hereditary, and genetic disorders that typically have The dorsal or sensorimotor basal ganglia are composed of
their onset in adulthood, including Parkinson disease, par- three nuclei located at the base of the cerebral cortex—
kinsonian syndromes, Huntington chorea, Wilson disease, hence their name. These nuclei are the caudate nucleus, the
dystonias, heavy metal poisoning, and drug intoxication. putamen, and the globus pallidus. Two brain stem nuclei, the
Because of the wide variety of diseases with their wide substantia nigra and the subthalamic nucleus, are included
variety of causes, the concentration is on understanding as part of the basal ganglia because they have a close func-
the clinical problems and commonalities that exist within tional relation to the forebrain nuclei. In addition, connec-
this grouping. In general, the practice parameter of the tions between the basal ganglia and the pedunculopontine
diseases discussed in this chapter is the physical therapy nucleus (PPN) are important in regulating underlying tone.
diagnostic parameter 5E: Impaired motor and sensory Other parts of the basal ganglia, the ventral basal ganglia,
integrity associated with progressive disorders of the are intimately related to the limbic system and are discussed
central nervous system, from the Guide to Physical Thera- in Chapter 5. The anatomical location of the various parts of
pist Practice.1 Although the occupational therapy guide the basal ganglia is shown in Figure 20-1.
does not classify practice parameters in that manner, the The caudate nucleus and the putamen are similar struc-
concepts and clinical reasoning process can be used by tures embryologically, anatomically, and functionally and
both professionals. The predominant area of the brain are often referred to together as the neostriatum—a term
affected by these disorders is the basal ganglia: this group derived from the word striate and used to denote pathways
of central nervous system (CNS) structures is therefore from and to the caudate and putamen. An older term, corpus
discussed in some detail. striatum, refers to the caudate, putamen, and globus palli-
dus. The various connections and interconnections of this
THE BASAL GANGLIA system are discussed on the basis of these definitions.
The most commonly seen disorders affecting the basal gan-
glia include Parkinson disease, Huntington chorea, and Afferent Pathways
dystonias, including drug-induced dyskinesias. All of these Functionally, the basal ganglia can be divided into an afferent
medical diagnoses involve impairments in muscle tone, portion and an efferent portion (Figure 20-2). The afferent
movement coordination and motor control, and postural structures are the caudate and putamen. They receive input
stability and the presence of extraneous movement. Taken from the entire cerebral cortex, the intralaminar thalamic
together, these disorders now affect approximately 1 million nuclei, and the centromedian-parafascicular complex of the
people in the United States.2-4 thalamus as well as from the substantia nigra and the dorsal
To understand how this area of the brain can account for raphe nucleus, both located within the brain stem. The pro-
such a wide variety of symptoms, the anatomy, physiology, jections from the cortex are systematically arranged so that
and neurochemistry of the basal ganglia structures must be the frontal cortex projects to the head of the caudate and
considered. putamen and the visual cortex projects to the tail. In addition,
601
Figure 20-1 n A coronal section of the anatomical location of various parts of the basal ganglia.
(Reprinted from Nolte J: The human brain: an introduction to its anatomy, St Louis, 1981, CV
Mosby.)
Cortex Thalamus
Centromedian-parafascicular
complex and intralaminar nuclei
Caudate/Putamen
Substantia nigra Globus pallidus
Subthalamic nuclei
Thalamus and motor cortex

Superior colliculus
Reticular formation
Figure 20-2 n Afferent and efferent portions of the basal ganglia.

CHAPTER 20 n Basal Ganglia Disorders 603
the prefrontal cortex projects mainly to the caudate, whereas through the superior colliculus and then to the tectospinal
the sensorimotor cortex projects mainly to the putamen.5-8 tract. Pathways exist from the globus pallidus and substantia
Projections from the cortical regions that represent the nigra that terminate in areas of the reticular formation (e.g.,
proximal musculature, and those from the premotor regions the PPN) and thus may influence the motor system through
may be bilateral.6,9-11 These close and profuse connections the reticulospinal pathways. Anatomically the basal ganglia
between the cortex and the basal ganglia suggest a close in- are therefore in good position to affect the motor system at
terfunctional relationship. The projections from the thalamus many levels. Many of these connections are also areas that
to the caudate-putamen are also somatotopically arranged. receive cerebellar input, and thus these two regions of the
The heaviest projections are from the centromedian nucleus, brain have ample opportunity to further integrate movement
and these nuclei also receive massive input from the motor responses.17
cortex.7-10 The basic circuitry of the basal ganglia comprises two
The somatotopic arrangement of the cortico-striatal– loops.7 The loops for the sensorimotor system are shown in
thalamic-cortical pathways is maintained throughout the Figure 20-3. The direct loop is the loop that begins in the
loop. This finding has led to an important functional hypoth- motor regions of the cortex and projects to the putamen and
esis that the basal ganglia form parallel pathways subserving then directly to the globus pallidus, the internal segment,
specific sensorimotor and associative functions.5 The puta- and on to the thalamus. The indirect pathway adds the sub-
men is linked to the sensorimotor functions and the caudate thalamic nucleus between the globus pallidus, external seg-
to the associative, including cognitive functions.9,12 ment, and internal segment before sending the signal on to
As knowledge of the circuitry of the basal ganglia has the thalamus. The subthalamic nucleus also receives direct
advanced, so has the knowledge regarding the microscopic
structure. The caudate-putamen looks somewhat homoge-
neous because of the predominance of one cell type. Careful
analysis using precise staining methods has demonstrated
the appearance of patches within these nuclei. It is hypoth-
esized that this organization is important for the ability of
the basal ganglia to modulate ongoing sensory input and
choose the appropriate motor response.12 The intrinsic struc-
ture of the caudate-putamen also suggests that at least nigral
input occurs in a way that could immediately modulate the
input coming from the cortex.13,14
Efferent Pathways
The input that has been processed in the caudate-putamen is
sent to the globus pallidus (pallidum) and substantia nigra
(nigra), which constitute the efferent portion of the basal
ganglia. The globus pallidus and substantia nigra are each
divided into two regions. The globus pallidus has an external
and an internal region; the substantia nigra consists of
the dorsal pars compacta and the ventral pars reticulata.
Embryologically and microscopically, the internal segment
of the globus pallidus and the pars reticulata of the substan-
tia nigra are similar. These two regions are the primary
efferent structures for the basal ganglia. The projections
from the caudate and putamen to the pallidum and nigra
maintain a somatotopic arrangement.10,15,16 From these
structures the information is transmitted to the thalamus and
then to the cortex, still maintaining somatotopy. The supe-
rior colliculus, the PPN, and other, less defined brain stem
structures (perhaps the reticular formation) also receive pal- Figure 20-3 n Diagram of the sensory motor portion of the basal
lidal and nigral output. All output of the basal ganglia has ganglia depicting the direct and indirect pathways. Black circles
then been processed through the globus pallidus and/or the represent inhibitory neurons; open circles represent excitatory
substantia nigra before proceeding to other areas of the brain neurons. CM, Centromedian nucleus of the thalamus; GPe, globus
(see Figure 20-2). pallidus external segment; GPi, globus pallidus internal segment;
MC, motor cortex; PMC, premotor cortex; SMA, supplementary
Pathways to the Motor System motor cortex; SNr, pars reticularis of the substantia nigra; STN,
Information processed in the basal ganglia can influence the subthalamic nucleus; VApc/mc, ventral anterior pars parvocellu-
motor system in several ways, but no direct pathway to laris and pars magnocellularis of the thalamus; VLo, ventral latera-
the alpha or gamma motor neurons of the spinal cord exists. lis pars oralis nucleus of the thalamus. (Reprinted from Alexander
The first route is the projection to the ventroanterior and GE, Crutcher MD: Functional architecture of basal ganglia
ventrolateral nuclei of the thalamus, which then project pre- circuits: neural substrates of parallel processing, Trends Neurosci
dominantly to the premotor cortex. Another pathway is 13:266-271, 1990.)
input from the premotor and motor cortex as well as from

the pallidum.18,19 The darkened neurons represent inhibitory Striatum
connections, and the open neurons represent excitatory con-
nections. In general, the direct pathway, by disinhibition,
activates the thalamocortical pathway; the indirect pathway
inhibits the thalamocortical system. The role of these loops
in normal and diseased states is clarified in the discussion of
the physiology and pharmacology of the basal ganglia.
In summary, input from the motor cortex, all other areas GPi
of the cortex, parts of the thalamus, and the substantia nigra

enter the basal ganglia through the caudate and putamen.
Here they are processed and sent on to the globus pallidus STN
and substantia nigra. The appropriate “gain” of the system is
adjusted, for example, how large a movement is necessary or
how much postural stability is needed. The information VLo/VA
is sent to the muscles by way of the thalamus and motor
cortex, the superior colliculus, and/or the reticular formation.
Physiology
Other Other
The caudate and putamen are composed of neurons that fire Motor Motor
slowly; the globus pallidus neurons fire tonically at high Programs
Desired Motor Program
Programs
rates. The low firing rates of the caudate-putamen are par- Excitatory
tially a result of the nature of thalamic inputs. Input from the Inhibitory
cortex seems to have priority over input from the thalamus
and substantia nigra. These data indicate that the cortex is Figure 20-4 n The net effect of basal ganglia circuitry to pro-
instrumental in regulating the responsiveness of caudate and duce an area of excitation (the desired program) surrounded by an
putamen neurons.20 In turn, basal ganglia stimulation may area of inhibition (all other unnecessary programs). GPi, Globus
prepare the cortex for subsequent inputs; this might be espe- pallidus internal segment; STN, subthalamic nucleus; VLo/VA,
cially important when a response must be withheld until an ventral lateralis oralis, ventral anterior. (Adapted from Mink JW:
appropriate stimulus occurs, such as keeping the foot on the The basal ganglia: focused selection and inhibition of competing
brake until the light turns green.20-23 Mink hypothesized that motor programs, Prog Neurobiol 50:381-425, 1996.)
basal ganglia inputs to the cortex activate only the most
necessary pathways and inhibit all unnecessary pathways
(Figure 20-4).24
The pattern of neuronal firing in the direct and indirect individual will have difficulty in selecting the environmen-
pathways also suggests that the basal ganglia modify input tally appropriate behavior.24-27 Aldridge and colleagues found
to the cortex. The neurons of the efferent portion of the basal that the basal ganglia were modulated dependent on the
ganglia respond with either phasic increases or phasic purpose of the impending movement.27
decreases in activity, which in turn will affect the activity in
the thalamus and hence the cortex. A decrease in activity of Relationship of the Basal Ganglia
the internal segment of the globus pallidus removes inhibition to Movement and Posture
to the thalamus and thus enables cortical activation. Whether Lesion experiments; single and multiple unit recordings in
the two pathways are activated concurrently or whether differ- awake, behaving animals; careful observations of the
ent activities activate the two pathways separately is not yet sequelae of human disease processes; and the results of
known; either way, the basal ganglia would have a role in functional magnetic stimulation studies in humans have
cortical activation and modulation. One of the current views provided some answers regarding the precise role of the
in relationship to disease processes is that an underactive basal ganglia in movement and posture.
direct pathway and/or an overactive indirect pathway would
lead to decreased activation of the cortex and hence bradyki- Automatic Movement
nesia and akinesia, whereas an overactive direct pathway The earliest view of the basal ganglia came from Willis in
and/or underactive indirect pathway would lead to the pres- 1664. He hypothesized that the corpus striatum received
ence of extraneous movements (see Figure 20-3).6,25 “the notion of spontaneous localized movements in ascend-
How do these pathways relate to everyday function? ing tracts . . . . Conversely, from here tendencies are dis-
Rigidity could be explained by too much muscle activity patched to enact notions without reflection [automatic
(through the pathways from the basal ganglia to the PPN and movements] over descending pathways” (p. 7).28 Willis pos-
on to the spinal cord). Akinesia and bradykinesia typical of sessed great insights in the discussion of the signs and
individuals with Parkinson disease are caused by insufficient symptoms of basal ganglia disease. Magendie in 1841 dem-
excitation or too many conflicting patterns of movement. onstrated that removal of the striatum bilaterally produced
Increased extraneous movements are characteristic of basal compulsive movements, whereas removal of only one stria-
ganglia diseases and can be attributed to the dysfunctions tum produced no visible effect.29 Studies by Nothnagel30
within these pathways. If the amount of muscle activity and demonstrated that lesions of the nigra tended to produce im-
the sequence and timing of activation are inappropriate, the mobility. With the advent of the use of electrical stimulation
in the late nineteenth century, further information on the

function of the basal ganglia was gathered. Stimulation of
the caudate nucleus did not (and does not) produce move-
ment of muscles or limbs as occurs with stimulation of the
motor cortex. However, at higher levels of current, total
body patterns and postures were usually evoked. The earliest
stimulation of the caudate nucleus produced an increase of
flexion of the head, trunk, and limbs and tonic contraction of
the facial muscles.31 These early studies are mentioned
because of the insights they provide for the symptoms of the
disorders of today.
Motor Problems in Animals

Contemporary experiments using lesion paradigms show
a wide variety of motor problems in a variety of animals.
Hypokinesia, a decrease or poverty of movements, a
decreased amount of exploration of novel environments,
and a tendency to assume a fixed posture are the most com-
mon problems after a lesion in the basal ganglia. These
motoric dysfunctions are seen regardless of the method by
which the lesion is made: pharmacologic, surgical, or by
stimulation. In essence, movements are altered in scale
(related to the gain), take longer for completion, and
take place under altered conditions of antagonistic muscle
interactions (e.g., contraction).32-40
Movement Initiation and Preparation

The hypothesis that the basal ganglia are involved in move-
ment initiation and preparation is an area of some research
disagreement. A “readiness potential,” recorded from the
scalp of human beings before movement and thought to
reflect basal ganglia activity, is more apparent in complex A B
than in simple movements, for example, before dorsiflexion Figure 20-5 n Typical posture of a patient with Parkinson dis-
with gait but not before dorsiflexion when sitting.41-44 ease from the front and from the side. Note the flexed spine, mild
Neuronal recordings from awake, behaving animals flexion at the hips and knees, and excessive dorsiflexion with
found that units in the basal ganglia alter their activity weight predominantly on the heels. Patient was at Hoehn and Yahr
before changes in the electromyographic activity of the stage 2.5.
prime movers of the task.45-51 Studies recording from mul-
tiple units in animals moving freely in their home environ-
ment suggest that neurons in the caudate-putamen and in the
substantia nigra are activated in sequential, purposeful described deficits in the longer loop reflexes but not in the
movements.27 short latency reflex associated with the stretch reflex.
Others have investigated the interactions of the sensory sys-
Postural Adjustments tems involved in balance in those with Parkinson disease.62-66
The basal ganglia have been implicated in the process of Bloem and colleagues60 and Visser and colleagues62 concluded
posture and postural adjustments. People with diseases of that postural instability was caused by a decrease in propriocep-
the basal ganglia assume flexed or other fixed postures as the tion. In a recent review of proprioception and postural stability
disease progresses (Figure 20-5). In addition, these individ- and motor control, Nicola and colleagues also describe the
uals have decreased postural stability and are therefore at kinesthetic and proprioceptive deficits in people with Parkinson
risk for falls. Animal experiments indicate that a deficit disease. Nicola and colleagues concluded that there was a
exists in determining response based on one’s own body po- “failure” in the body map similar to the failure in egocentric
sition, or “egocentric localization.”52-54 This deficit decreases localization described previously.54 A decrease in the ability to
the ability of a person with basal ganglia disease to modify a use proprioceptive and kinesthetic information to properly
postural response to the precise environmental demands. scale the input and response also contributes to a loss of balance
Martin,55 in his extensive studies of individuals with reactions.
Parkinson disease, was the first to describe severe distur-
bances in posture, especially when vision was occluded. Perceptual and Cognitive Functions
Melnick and colleagues56 showed that a decrease in static The basal ganglia are not solely motor systems. The previ-
postural adjustments in persons with Parkinson disease ous paragraphs demonstrate the role of the basal ganglia
could be seen early in the disease process.57 Bloem and col- in sensory integration. The basal ganglia are also involved
leagues58-60 and Visser and colleagues61,62 meticulously stud- in cognitive functions and responses associated with
ied the reflexes involved in postural adjustments and reward.36,37,48,50,67-70 Researchers have found that learned
movements are more affected by basal ganglia lesions For movements to be properly controlled and properly
than reflexes, that neurons in the basal ganglia are respon- sequenced, the two sides of the body need to be well inte-
sive to some sensory input, especially proprioceptive input, grated. There is anatomical evidence that suggests some
and that neurons in other parts of the basal ganglia are re- means of bilateral control for the basal ganglia. A lesion of
sponsive to reward and anticipation of the reward.26,71,72 one caudate nucleus or nigrostriatal pathway produces a
Klockgether and Dichgans73 as well as Jobst and colleagues74 change in the unit activity of the remaining caudate.78,83
found that patients with Parkinson disease likewise had im- Studies of the dopaminergic pathway also indicate interac-
pairments in kinesthesia and that as a person moved a limb tions between the two sides of the body.83 For this reason
further from the body’s center, kinesthetic sense decreased. one may find deficits in function even on the “uninvolved”
Schneider and colleagues75 found that animals that devel- side of an individual with disease of the basal ganglia. It
oped parkinsonian symptoms from a neurotoxin had deficits is also possible that diseases of the basal ganglia may go
in operantly conditioned behavior. They suggested that the unnoticed until damage is found bilaterally.
decrease in performance resulted from a “defect in the link- This summary of experimental results on the function of
age” between a stimulus and the motor output centers. These the basal ganglia illustrates several points. At least in some
sensory difficulties may be important factors in evaluation general way the basal ganglia are involved in the processes
and treatment of basal ganglia diseases, especially those of movement related to preparing the organism for future
associated with dystonia. motion and future reward. This may include preparing the
The basal ganglia appear to be involved in the process of cortex for approximate time activation, setting the postural
withholding a response until it is appropriate.76 A deficit in reflexes or the gamma motor neuron system, organizing
alternation of response may be the result of a tendency sensory input to produce a motor response in an appropri-
toward perseveration of a previously reinforced cue.77 ate environmental context, and inhibiting all unnecessary
Additional deficits exist in remembering or relearning tasks motor activity. Because of the multilevel involvement of
requiring a temporal sequence.78 Graybiel26 integrated the the basal ganglia in movement, it is crucial that clinicians
behavioral findings with information from her anatomical carefully observe all aspects of movement (simple and
and chemical studies to suggest that the basal ganglia are complex) with and without interference of sensory cues or
important in providing behavioral flexibility. She hypothe- performance of dual tasks as well as postural tone during
sizes that the basal ganglia are involved in procedural learn- examination and treatment and the responses to treatment
ing that leads to the development of habits. These habits (see Chapter 9).
become routine and are easily performed without conscious
effort. Because these activities can proceed without thought, Neurotransmitters
we are free to react to new events in our environment and to Before a detailed analysis of the diseases of the basal
think. She and colleagues have performed electrophysiolog- ganglia can be considered, a brief description of the neu-
ical experiments that explain this learning process, and these rotransmitters of this region is necessary. The most prevalent
studies demonstrate great plasticity in basal ganglia net- diseases discussed in this chapter indicate a deficit in spe-
works.79 This enables the individual to select the proper cific neurotransmitters. The pharmacological treatment of
movements in the proper environmental context. An elegant Parkinson disease and, in the future, perhaps other “basal
study by Brown and colleagues80 demonstrates a model of ganglia plus” diseases, is based on these neurochemical
the basal ganglia that can reflect these cognitive and learning deficits. The basal ganglia possess high concentrations of
activities. Their model seems to integrate many of the func- many of the suspected neurotransmitters: dopamine (DA),
tions of the basal ganglia with the physiology and pharma- acetylcholine (ACh), g-aminobutyric acid (GABA), sub-
cology of the entire system. These cognitive dimensions are stance P, and the enkephalins and endorphins. This discus-
important to remember when developing a plan of care for a sion, however, includes only the first three neurotransmit-
patient with basal ganglia dysfunction. ters. A diagram of the basal ganglia pathways, which
Humans with basal ganglia disease also show problems includes the neurotransmitters, is shown in Figure 20-6.
in perceptual abilities, including deficits in tasks that involve DA is the major neurotransmitter of the nigrostriatal
perception of interpersonal and intrapersonal space.81 In pathway. It is produced in the pars compacta of the substan-
pursuit-tracking tests individuals with Parkinson disease had tia nigra. The axon terminals of these dopaminergic neurons
particular difficulties in correcting errors77; if the motor are located in the caudate nucleus and putamen. DA appears
system is inflexibly set, corrections can be made only by a to be excitatory to the neurons in the direct pathway (GABA
complete reprogramming. and substance P neurons) and inhibitory to the neurons in
The ability to perform cognitive activities involves inte- the indirect pathway (GABA and enkephalin neurons).2 This
grating sensory information and, on the basis of this infor- dual effect means that a loss of DA will lead to a loss of
mation, making an appropriate response. The basal ganglia excitation in the direct pathway and an excess of excitation
seem to have a sensory integrative function as evidenced of the indirect pathway, leading to a powerful decrease in
by experiments that show a multisensory and heterotopic activation of the thalamocortical pathway.
convergence of somatic, visual, auditory, and vestibular Several DA receptors exist; however, their chemical
stimuli.26,71,72 Segundo and Machne82 hypothesized that the interactions permit the continued use of D1 and D2 receptor
function of the basal ganglia was not subjective recognition classes.7 The role of DA may modulate the effects of other
of the stimuli but rather in the regulation of posture and neurotransmitters such as glutamate. Many new drugs
movements of the body in space and in the production (called the dopamine agonists) influence only one of these
of complex motor acts. Nicola and colleagues had similar receptors. Recent experiments have been trying to determine
conclusions.54 which behaviors are mediated by which DA receptor in the
alkaloids and atropine-like drugs, were one of the first class

of drugs used in the treatment of Parkinson disease. ACh
antagonists are still used as adjuncts to treatment for patients
with Parkinson disease. As some of the drugs to treat
dementia are ACh agonists, care must be used when these
are prescribed for the person with basal ganglia dysfunction,
especially Parkinson disease.
GABA is an inhibitory neurotransmitter that is found
throughout the brain. In the basal ganglia it is synthesized in
the caudate nucleus and putamen and transmitted to the
globus pallidus and substantia nigra.86 GABA in the basal
ganglia may permit movement to occur by allowing a distri-
bution of neuronal firing. It also may provide a means of
feedback inhibition in the efferent parts of the basal ganglia
so that the program of activity is not repeated unless
needed.86 Individuals with Huntington disease have a defi-
ciency of this chemical. Although agonists of GABA exist
(e.g., muscimol and imidazole acetic acid), a successful
drug for the treatment of Huntington disease has not yet
been found. This may be a result of either the ubiquitous
nature of GABA or the very complex circuitry and interrela-
tionships that exist among GABA, ACh, and DA.
In addition to the transmitters discussed, co-transmitters
may be found in the basal ganglia. Two such co-transmitters
are cholecystokinin and neurotensin. The interactions of
these co-transmitters may alter the sensitivity of DA recep-
tors. Fuxe and colleagues87 suggest that the interactions of
co-transmitters may alter the “set point” of transmission in
Figure 20-6 n The neurotransmitters of the direct and indirect
synapses. They may therefore be important in one of the
pathways of the basal ganglia. Black circles represent inhibitory neu-
side effects of DA therapy, supersensitivity.
rons; open circles represent excitatory neurons. enk, Enkephalin; glu,
Lastly, the neurotransmitter from the cortex to the cau-
glutamate; GPe, globus pallidus external segment; GPi, globus palli-
date nucleus and putamen is glutamate. Studies are ongoing
dus internal segment; SNr, pars reticularis of the substantia nigra;
to investigate glutamate antagonists as a treatment for
STN, subthalamic nucleus; subst P, substance P; Thal, thalamus.
Parkinson disease. Glutamate receptors use calcium, and in
(Reprinted from Alexander GE, Crutcher MD: Functional architec-
the future, drugs affecting calcium channels may also have
ture of basal ganglia circuits: neural substrates of parallel processing,
a therapeutic effect.
Trends Neurosci 13:266–271, 1990.)
SPECIFIC CLINICAL PROBLEMS ARISING

FROM BASAL GANGLIA DYSFUNCTION
hope that this research may lead to more effective drug treat- Parkinson Disease
ment with fewer side effects. Parkinson disease, first described by Parkinson in 1807, is
Because various drugs and chemicals can act as agonists a disease characterized by rigidity, bradykinesia (slow
(similar to) and antagonists (blocking the action of) of DA, movement), micrography, masked face, postural abnor-
they are used in treating disease involving the basal ganglia. malities, and a resting tremor. As might be suspected from
Agonists include amantadine, apomorphine, and a class the review of functional physiology of the basal ganglia,
of drugs called the ergot alkaloids (e.g., bromocriptine). the postural abnormalities include an assumption of a
Amphetamine, which prevents the reuptake of DA, can en- flexed posture, a lack of equilibrium reactions, especially
hance the effect of any DA present in the system. Antago- of the labyrinthine equilibrium reactions, and a decrease
nists include haloperidol, clozapine, and antipsychotic drugs in trunk rotation. Parkinson disease is among the most
of the phenothiazine class. With time these drugs may prevalent of all CNS degenerative diseases. Presently
deplete the basal ganglia of DA and thus cause Parkinson there are an estimated 1 million people in the United
disease or tardive dyskinesia. Similar effects on the DA sys- States with this disease, with approximately 60,000 new
tem are observed in a single dose of methamphetamine cases each year; the incidence is 4.5 to 20.5 and the
(see Chapter 36).84 prevalence is 31 to 347 per 100,000. (Refer to the list of
ACh is believed to be the neurotransmitter of the small websites at the end of this chapter.) Incidence increases
interneurons of the caudate and putamen. It is presumed to with advancing age, and it is estimated that one in three
inhibit the action of DA in this region and classically must adults over the age of 85 will have this disease.2 The per-
be “in balance” with DA (and GABA). Dopaminergic axon sonal and societal burden of Parkinson disease is great and
terminals are found on cholinergic neurons. Substances that includes the costs of actual treatment, the burden of care-
increase dopaminergic activity decrease release of ACh and giving, and the costs of lost earnings in patients under the
vice versa.85 The antagonists of ACh, such as belladonna age of 65.88
The pathology of Parkinson disease consists of a decrease involved than simple movements, such as dorsiflexing the
in the DA stores of the substantia nigra with a consequent foot at toe-off in walking as opposed to dorsiflexing the foot
depigmentation of this structure and the presence of Lewy in a seated position.71,106-109 In addition, patients with par-
bodies (intracellular inclusions). It is DA that gives the sub- kinsonism have increased difficulty performing simultane-
stantia nigra its coloration (and hence its name); therefore the ous or sequential tasks, over and above that seen with simple
lighter the nigra, the greater the DA loss. tasks. Parkinsonian patients must complete one movement
The cause of Parkinson disease remains unknown, and before they can begin to perform the next, whereas control
the consensus is that it is multifactorial.89,90 A slow viral subjects are able to integrate two movements more smoothly
process or long-term effects of early infection were impli- in sequence. This deficit has been shown in a variety of tasks
cated in postencephalitic parkinsonism. Some evidence indi- from performing an elbow movement and grip to tracing a
cates involvement of environmental factors and that interac- moving line on a video screen. The patient with Parkinson
tion of environment and aging lead to a critical decrease in disease behaves as if one motor program must be completely
DA. Several investigators have found a link between grow- played out before the next one begins, and there is no ad-
ing up in a rural area and Parkinson disease; the important vance planning for the next movement while the current
factors include pesticide use, insecticide use, and elements movement is in progress.106-108,110,111 Morris and colleagues
in well water.91-97 Accumulation of free radicals, cell death demonstrated a similar phenomenon in walking. Patients
to excitatory neurons from toxins, and dysfunction of nigral with parkinsonism were unable to perform walking while
mitochondria have all been implicated in the pathological carrying a tray with a glass of water and had even more dif-
process. The genetics of Parkinson disease is still debated. ficulty when walking and reciting a numerical sequence.112,113
Although twin studies indicate that there may not be a single Sequential movements become more impaired as more
gene involved in Parkinson disease, as in Huntington dis- movements are strung together; for example, a square is
ease, a family history may be an important risk factor.93,98-101 disproportionately slower to draw than a triangle; a penta-
Very recently a large-scale study found two genetic loci gon, more difficult than a square.5,106 These results indicate
to be associated with Parkinson disease.102 So the debate that patients with Parkinson disease have difficulty with
continues, with most neurologists agreeing that the multi- transitions between movements. Transitional difficulties are
factorial approach will yield the best opportunity to develop more impaired in tasks requiring a series of different move-
a cure. ments than tasks requiring a series of repetitive movements.
In view of possible treatment effects for Parkinson disease, For example, an individual will have less difficulty continu-
it is interesting that a study by Sasco and others103 found an ally riding a stationary bike than movement requiring transi-
inverse relationship, albeit small, between participation in tions such as coming from a chair to standing, walking, and
exercise or sports and later development of Parkinson disease. turning a corner. Therefore treatment must include complex
The loss of DA from the substantia nigra leads to alterations movements with directional changes to ensure that the
in both the direct and indirect pathways of the basal ganglia, patient is safe outside the treatment setting.
resulting in a decrease in excitatory thalamic input to the cor- Bradykinesia is not caused by rigidity or an inability to
tex and perhaps a decrease in inhibitory surround that leads to relax. This was demonstrated in an electromyographic anal-
the symptoms of Parkinson disease. ysis of voluntary movements of persons with Parkinson
disease.114 Although the pattern of electromyographic agonist-
Symptoms antagonists burst is correct, these bursts are not large
Bradykinesia and Akinesia. Bradykinesia (a decrease enough, resulting in an inability to generate muscle force
in motion) and akinesia (a lack of motion) are characterized rapidly enough. Even in slow, smooth movements, however,
by an inability to initiate and perform purposeful move- these individuals demonstrated alternating bursts in the
ments. They are also associated with a tendency to assume flexor and extensor muscle groups. This type of pattern,
and maintain fixed postures. All aspects of movement are expected in rapid movements that require the immediate
affected, including initiation, alteration in direction, and the activation of the antagonist to halt the motion, interferes
ability to stop a movement once it is begun. Spontaneous or with slow, smooth, continuous motion. Other researchers
associated movements, such as swinging of the arms in gait have found an alteration in the recruitment order of single
or smiling at a funny story, are also affected. Bradykinesia is motor units.115,116 These alterations included a delay in re-
hypothesized to be the result of a decrease in activation of the cruitment, pauses in the motor unit once it was recruited,
supplementary motor cortex, premotor cortex, and motor and an inability to increase firing rates. These persons there-
cortex.104 The resting level of activity in these areas of the fore would have a delay in activation of muscles and an
cortex may be decreased so that a greater amount of excit- inability to properly sustain muscle contraction for move-
atory input from other areas of the brain would be necessary ment, and a decreased ability to dissipate force rapidly.24,115,117
before movement patterns could be activated. In the individ- Such changes may account for perceived decreases in strength
ual with Parkinson disease, an increase in cortically initiated that are seen in persons with Parkinson disease. They are also
movement even for such “subcortical” activities as walking important to remember in both treatment planning and the
supports this hypothesis. Automatic activities are cortically efficacy of treatment efficiency.
controlled, and each individual aspect seems to be separately Rigidity. The rigidity (increased resistance to passive
programmed. Associated movements in the trunk and other movement) of Parkinson disease may be characterized as
extremities are not automatic. This means that great energy either “lead pipe” or “cogwheel.” The cogwheel type of
must be expended whenever movement is begun.105 rigidity is a combination of lead-pipe rigidity with tremor. In
Bradykinesia and akinesia affect performance of all types rigidity there is an increased resistance to movement
of movements; however, complex movements are more throughout the entire range in both directions without the
classic clasp-knife reflex so characteristic of spasticity. Pro- likelihood of falling as the duration of the disease increases.
caine injections can decrease the rigidity without affecting Drug treatment is not usually effective in reducing the inci-
the decrease of spontaneous movements, confirming that dence of falls. Deep brain stimulation and exercise, on the
rigidity is not the same phenomenon as bradykinesia.118,119 other hand, have been shown to be effective in increasing
Rigidity is not caused by an increase in gamma motor functional skills and/or motor performance; these improve-
neuron activity, a decrease in recurrent inhibition, or a gen- ments may decrease the number of falls.131-134 Large ran-
eralized excitability in the motor system.120 Long- and domized clinical trials have been performed to determine the
middle-latency reflexes are enhanced in parkinsonism, and efficacy of exercise.135
the increase in long-latency reflexes approximates the Although the causes of balance difficulties are not known,
observable increase in muscle tone. Short-latency reflexes several hypotheses exist. One explanation for postural insta-
(i.e., deep tendon reflexes), on the other hand, may be nor- bility is ineffective sensory processing. Several investigators
mal in persons with Parkinson disease. have found deficits in proprioceptive and kinesthetic
Tatton and others121 found differences in certain cortical processing.55,74,117,136 For example, Martin55 found that laby-
long-loop reflexes in normal and drug-induced parkinsonian rinthine equilibrium reactions were delayed in patients with
monkeys, which led them to speculate that the “reflex gain” Parkinson disease. Studies of the vestibular system itself,
of the CNS may lose its ability to adjust to changing envi- however, have shown that this system functions normally.
ronmental situations. For example, in normal persons the Pastor and colleagues137 studied central vestibular process-
background level of motor neuron excitability is different ing in patients with Parkinson disease and found that the
for the task of writing than for the task of lifting a heavy vestibular system responds normally and that patients can
object; in individuals with Parkinson disease motor neuron integrate vestibular input with the input from other sensory
excitability would be set at the same level. Similarly, in the systems. This group hypothesized that the parkinsonian
normal individual there would be a difference in excitability patients had an inability to adequately compensate for base-
if the environmental demands were for excitation or inhibi- line instability. This theory is in partial agreement with stud-
tion of a muscle; for the individual with Parkinson disease, ies by Beckley, Boehm, and others58,59,65 demonstrating that
there would be similar motor neuron excitability regardless patients with Parkinson disease were unable to adjust the
of task demands. Furthermore, this lack of modulation may size of long- and middle-latency reflex responses to the
mean that the person with parkinsonism perceives himself or degree of perturbation. These patients are therefore unable to
herself to be moving farther than he or she is actually mov- activate muscle force proportional to displacement. Melnick
ing. It is also consistent with a decrease in system flexibility and colleagues56 found that subjects with Parkinson disease
and an inability to adjust to equilibrium perturbations.58,59,65 were unable to maintain balance on a sway-referenced force
An important aspect of rigidity is that it might increase plate. Glatt138 found that patients with Parkinson disease did
energy expenditure.122 This would increase the patient’s not demonstrate anticipatory postural reactions and, in fact,
perception of effort on movement and may be related to behaved exactly as a rigid body with joints. Horak and col-
feelings of fatigue, especially postexercise fatigue.123 leagues,139,140 in a variety of studies, reported similar find-
Tremor. The tremor observed in Parkinson disease is ings and found defects in strategy selection as well; patients
present at rest, usually disappears or decreases with move- with Parkinson disease chose neither a pure hip strategy nor
ment, and has a regular rhythm of about 4 to 7 beats per a pure ankle strategy but mixed the two in an inappropriate
second. Some people with Parkinson disease may have a and maladaptive response. Investigators have found that
postural tremor. The electromyographic tracing of a person antiparkinsonian medications could improve background
with such a tremor shows rhythmical, alternating bursting of postural tone but did not improve automatic postural re-
antagonistic muscles. Tremor can be produced as an isolated sponses to external displacements.58,59,65,139-141 Other studies
finding in experimental animals that have lesions in various have demonstrated deficits in proprioceptive perception—
parts of the brain stem or that have been treated with drugs, what has been termed an “impaired proprioceptive body
especially DA antagonists. DA depletion, however, is not the map.” Patients with Parkinson disease did not alter anticipa-
sole cause of tremor. It appears that efferent pathways, espe- tory postural adjustments in response to step width changes,
cially from the basal ganglia to the thalamus, must be intact unlike control subjects.142 Increased step width requires
because lesions of these fibers decrease or abolish the increased lateral reactive forces to unload the stance leg. The
tremor.124 Poirier and colleagues124 proposed that tremor lack of ability to prepare for these extra forces may indicate
results from a combined lesion of the basal ganglia and cer- that narrow stance width, start hesitation, and freezing of
ebellar–red nucleus pathways. Because both the basal gan- gait are compensatory mechanisms to proprioceptive loss.136
glia and the cerebellum project to the thalamus, a lesion of Likewise, when patients could not see their limbs, they
the thalamus can abolish the tremor regardless of the spe- had difficulty moving the foot to a predetermined location
cific pathway(s). Although tremor may be cosmetically disin response to perturbation. Control subjects had no
abling, the tremor rarely interferes with activities of daily difficulty.143,144 Taken together, it appears that postural insta-
living (ADLs). bility results from inflexibility in response repertoire; an
Postural Instability. Postural instability is a serious inability to inhibit unwanted programs; the interaction of
problem in parkinsonism that leads to increased episodes of akinesia, bradykinesia, and rigidity; and some disturbance in
falling and the sequelae of falls. More than two thirds of all central sensory processing.
patients with parkinsonism fall, and more than 10% fall Gait. The typical parkinsonian gait is characterized by
more than once a week.125 People with Parkinson disease decreased velocity and stride length.145,146 As a conse-
have a ninefold risk of recurrent falls compared with age- quence, foot clearance is decreased, which again places the
matched control subjects.60,126-130 Patients have an increased individual at greater fall risk.147 In many patients, especially
as the disease progresses, speed and shortening of stride Gait and postural difficulties are the two impairments that
progressively worsen as if the individual is trying to catch up cause the greatest handicap to persons with parkinsonism.
with his or her center of gravity; this is termed festination. They have been found to be the major elements of disability
Forward festination is called propulsion; backward festina- at home and work for these patients.
tion is known as retropulsion. One hypothesis is that festi- Perception, Attention, and Cognitive Deficits. Espe-
nating gait is caused by the decreased equilibrium responses. cially in recent years, researchers have tried to address
If walking is a series of controlled falls and if normal the cognitive and perceptual impairments of people with
responses to falling are delayed or not strong enough, then Parkinson disease.136,149-152 Whereas the movement deficits
the individual will either fall completely or continue to are hypothesized to be caused by a decrease in putaminal
take short, running-like steps. The abnormal motor unit fir- excitation of the cortex, the learning and perceptual deficits
ing seen with bradykinesia may also be the cause of ever- are hypothesized to be caused by a decrease in cortical exci-
shortening steps. If the motor unit cannot build up a high tation from the caudate nucleus.111 The deficits are of frontal
enough frequency or if it pauses in the middle of the move- lobe function and include an inability to shift attention, an
ment, then the full range of the movement would decrease; inability to quickly access “working memory,” and difficulty
in walking this would lead to shorter steps. Festination may with visuospatial perception and discrimination. Research
also be the result of other changes in the kinematics of gait. attention has focused on the specific deficits of parkinsonian
The changes in gait kinematics include changes in patients compared with patients with Alzheimer disease,
excursion of the hip and ankle joints (Figure 20-7). Instead patients with frontal lobe damage, and those with temporal
of a heel-toe, the patient may have a flat-footed or, with lobe damage.149,152,153 The perceptual deficits of all groups
disease progression, a toe-heel sequence. The patient with appear to increase with progression of the disease process.
Parkinson disease appears to have lost the adult gait pattern In general, patients have difficulty in shifting attention to a
and is using a more primitive pattern. The flat-footed gait previously irrelevant stimulus,154 learning under conditions
decreases the ability to step over obstacles or to walk on requiring selective attention,154 or selecting the correct
carpeted surfaces. The use of three-dimensional gait analy- motor response on the basis of sensory stimuli.155-157 There
sis has shown that there is a decrease in plantarflexion at is also evidence that DA is involved in selection of responses
terminal stance. Changes are also seen in hip flexion, which that will be rewarding.54 These impairments will affect treat-
may alter ankle excursion. However, qualitative aspects ment strategies.
of the timing of joint excursion appear intact. Figure 20-7 Learning deficits also have been found in patients with
illustrates the joint angles in a 55-year-old patient with parkinsonism; procedural learning has been particularly
Parkinson disease compared with adults without basal implicated, as would be indicated based on the physiology
ganglia dysfunction.148 of the system. Procedural learning is learning that occurs
47.1 47.1
20.0 20.0
Hip flex./ext.
0.0 0.0
–17.3 –17.3
84.3 84.3
60.0 60.0
Knee flex./ext. 40.0 40.0

20.0 20.0
–12.4 –12.4
26.3 26.3
10.0 10.0
Ankle flex./ext.
0.0 0.0
–10.0 –10.0
–22.0 –22.0
Figure 20-7 n Angles of excursion during gait in a patient with Parkinson disease. Shaded areas
are mean 6 standard deviations for adults without Parkinson disease; black lines represent a patient
with Parkinson disease. Movement shown for right and left lower extremities. Note decreases, espe-
cially in left lower extremity for extension and bilateral decreased plantarflexion.
with practice or, as defined by Saint-Cyr and colleagues,158 of rostral progression of dopaminergic involvement. Cog-
“the ability gradually to acquire a motor skill or even a cognitive involvement can include memory loss, confused
nitive routine through repeated exposure to a specific activ- thinking, and dementia. Parkinson disease medications
ity constrained by invariant rules.” In their tests, patients may worsen these cognitive impairments. The nonmotor
with Parkinson disease did very poorly on tests of proce- symptoms of Parkinson disease have been addressed in a
dural learning, but their declarative learning was within practice parameter recommendation by the American
normal limits. Pascual-Leone and colleagues111 studied pro- Academy of Neurology.161
cedural learning in more detail. They found that patients
with Parkinson disease could acquire procedural learning Stages of Parkinson Disease
but needed more practice than control subjects did. They Parkinson disease is a progressive disorder.165 The initial
also found that the ability to translate procedural knowledge motor symptom is often a resting tremor or unilateral
to declarative knowledge was more efficient if it occurred micrography (bradykinesia of the upper extremity). With
with visual input alone rather than the combination of visual time, rigidity and bradykinesia are seen bilaterally, and pos-
input with motor task. This may be a rationale for more tural alterations and axial symptoms then begin to occur.
therapy, not less. This commonly starts with an increase in neck, trunk, and
Nonmotor Symptoms. Nonmotor symptoms are con- hip flexion that, accompanied by a decrease in righting and
sistently seen in patients with Parkinson disease and may be balance responses, leads to a decreased ability to maintain
attributable to dopaminergic pathways outside the basal the center of gravity over the base of support.
ganglia. Braak159 hypothesized that Parkinson disease actu- While these postural changes are occurring, so does an
ally begins with DA deterioration in the medulla and pro- increase in rigidity, which is most apparent in the trunk and
gresses rostrally. Often the first signs are loss of sense of proximal and axial musculature. Trunk rotation becomes
smell, constipation, vivid dreams (rapid-eye movement severely decreased; there is no arm swing during gait and
[REM] behavior disorder), and orthostatic hypotension.160,161 no spontaneous facial expression; and movement becomes
Orthostatic hypotension may cause some dizziness and more and more difficult to initiate. Movement is usually
requires coordination of medications for other medical prob- produced with great concentration and is perhaps corti-
lems. l-Dopa and DA agonists may lower blood pressure; cally generated, therefore bypassing the damaged basal
blood pressure medication may need to be altered once ganglia pathways. This great concentration then makes
antiparkinsonian drugs have been prescribed. Although not movement tiring, which heightens the debilitating effects
all people with these problems have Parkinson disease, of the disease.
when they are combined they may indicate risk for this dis- Eventually the individual becomes wheelchair bound
order. Because physical therapy may be most effective when and dependent. In the late and severe stages of the disease,
started early, researchers are trying to learn more about these especially without therapeutic attention for movement dys-
early symptoms. functions, the client may become bedridden and may dem-
Other nonmotor symptoms that decrease quality of life onstrate a fixed trunk-flexion contracture regardless of the
include incontinence in men and women, sexual dysfunc- position in which the person is placed. This posture has
tion, excess saliva, weight changes, and skin problems. been called the “phantom pillow” syndrome because, even
Nonmotor symptoms that can interfere with and complicate when lying supine, the person’s head is flexed as if on a
physical and occupational treatment include fatigue, pillow.
fear, anxiety, and depression. Urinary incontinence is Throughout this progressive deterioration of movement,
important because it increases the risk of hospitalization there is also a decrease in higher-level sensory processing.
and mortality.162 In addition, the patient can perform only one task at a time.
Sleep disorders are widespread in Parkinson disease and Reports of dementia range from 30% to 93% in patients
include more than just REM sleep disorder.163 The patient with Parkinson disease.166 The presence of dementia in this
may experience daytime drowsiness and decreased sleep at population may indicate involvement of the ACh or norad-
night. There appears to be a lack of consolidation of sleep renergic mesolimbic system. In this case, treatment with
with decreased total sleep time as well as the presence of anticholinergic drugs may increase a tendency toward
restless leg syndrome.164 Daytime drowsiness may be a side dementia, especially in older patients. Sometimes cognitive
effect of medication; however, it can also be exacerbated deficits are inferred because of slowed responses, spatial
after therapeutic exercise, so a cool-down period is neces- problems, sensory processing problems, and a masked face
sary before the patient sits down and relaxes. (see Chapter 36).
Another side effect of medication is presence of halluci- The most serious complication of Parkinson disease is
nations. Many patients report seeing very ugly creatures bronchopneumonia. Decreased activity in general and
or monsters, and when such hallucinations occur in the decreased chest expansion may be contributing factors. The
therapeutic session they can be most uncomfortable for the mortality rate is greater than in the general population, and
therapist and the patient. These hallucinations also make it death is usually from pneumonia.
difficult for the patient to use adjunct treatments such as Staging of Parkinson disease uses the Hoehn and Yahr
computer games and virtual reality activities. scale (Table 20-1).165 Originally developed as a 5-point
Nonmotor symptoms often predominate as the disease scale, in recent years 0, 1.5, and 2.5 measurements have
progresses.160 They contribute to severe disability, im- been added. The 1.5 and 2.5 ratings have not been vali-
paired quality of life, and shortened life expectancy. As the dated, but because their use is so common, the latest recom-
disease progresses, cognitive problems also become more mendation is to continue using them while the validity is
frequent. Braak159 hypothesized that this was an indication studied.167
TABLE 20-1 OEHN AND YAHR STAGING

H n
l-dopa does not appear to be closely correlated with the stage
SCALE FOR PARKINSON DISEASE of the disease.
The use of l-dopa alone or in combination with carbi-
dopa has not provided a cure or even prevented the degen-
STAGE PROGRESSION OF SYMPTOMS eration of Parkinson disease.178,179 As more has become
0 No signs of disease. known about the DA receptor, specific agonists have been
1 Unilateral symptoms only. developed. Ropinirole, pramipexole, pergolide, and bro-
1.5 Unilateral and axial involvement. mocriptine are examples of DA receptor D2 agonists that are
2 Bilateral symptoms. No impairment of balance. used alone or with l-dopa. The agonists are thought to de-
2.5 Mild bilateral disease with recovery on pull test. crease the wearing-off effects as well as decrease the dyski-
3 Balance impairment. Mild to moderate disease. Physically nesias that occur with long-term l-dopa use, but l-dopa
independent. remains the most effective medication. It is quite likely that
4 Severe disability, but still able to walk or stand newer D2 and/or D2-D1 (DA receptor D1) agonists will be
unassisted. developed. Pharmacological interventions also include
5 Needing a wheelchair or bedridden unless assisted. drugs that prevent the breakdown of DA (e.g., catechol-O-
The Hoehn and Yahr scale is commonly used to describe how methyltransferase [COMT] inhibitors) and/or its reuptake.
the symptoms of Parkinson disease progress. The original Entacapone is an example of a COMT inhibitor.180
scale included stages 1 through 5.165 Stage 0 has since been Another approach to pharmacological treatment of indi-
added, and stages 1.5 and 2.5 have been proposed to best viduals with Parkinson disease was developed from research
indicate the relative level of disability in this population.167 on a designer drug that contained the neurotoxin 1-methyl-
4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). It was found
that the conversion of MPTP to the active neurotoxin MPP1
could be prevented by monoamine oxidase inhibitors such
as deprenyl and pargyline.73,179 Deprenyl, rasagiline, and
Pharmacological Considerations selegiline are now used before the initiation of, or in con-
and Medical Management junction with, l-dopa and carbidopa.
The knowledge that the symptoms of Parkinson disease are Another treatment alternative is surgery performed in
caused by a decrease in DA led to the pharmacological man- precise areas of the basal ganglia, known as stereotaxic sur-
agement of this disease. Because DA itself does not cross the gery. Stereotaxic surgery is an old technique that has made
blood-brain barrier, levo-dihydroxyphenylalanine (l-dopa), a a comeback based on the new knowledge of basal ganglia
precursor of DA that does, has been used to treat Parkinson connectivity and improvements in the procedural instrumen-
disease since the late 1960s.168-170 An inhibitor of aromatic tation.* Initially, one of the structures of the basal ganglia
amino acid decarboxylation (carbidopa) is usually given with was lesioned with freezing or high-frequency stimulation.
l-dopa to prevent the conversion to DA before entering the Today the globus pallidus internal segment or the subtha-
brain. The decarboxylase inhibitor allows a reduction in dos- lamic nucleus is stimulated with implanted electrodes. This
age of l-dopa itself, which helps decrease the cardiac and technique is known as deep-brain stimulation (DBS). DBS
gastrointestinal side effects of DA. has now been approved by the U.S. Food and Drug Admin-
Amantadine is another drug that has been effective in the istration (FDA). An advantage of deep brain stimulation
treatment of patients with Parkinson disease. Although over permanent lesions is that DBS is reversible and is safer
the mechanism of action of this antiviral medication is for bilateral surgeries. Stimulation of the globus pallidus
unknown, it is thought to include a facilitation of release of internal segment or subthalamic nucleus has been shown to
catecholamines (of which DA is one) from stores in the decrease all symptoms; subthalamic nucleus stimulation is
neuron that are readily releasable. It is often administered in also effective in reducing dyskinesias and may lessen the
combination with l-dopa. amount of medication taken.183-185 Effects of stimulation are
Treatment of Parkinson disease with l-dopa in these vari- greater for symptoms manifested in the “off” state. Deep
ous combinations is extremely helpful in reducing bradyki- brain stimulation has been demonstrated to improve rigidity,
nesia and rigidity. It is less effective in reducing tremor and bradykinesia, and akinesia, as well as gait57,184,186-189 and
the postural instability. Because Parkinson disease involves balance.56,190 It has also been demonstrated to improve
the nigral neurons, the receptors and the neurons in the stria- movement velocity and speed of muscle recruitment for
tum (which are postsynaptic to dopaminergic neurons) reactivity.190,191 The proposed mechanism of action is interfer-
main intact and initially are somewhat responsive to DA.171,172 ence with the abnormal neuronal firing.192,193 In a random-
With time, however, the receptors appear to lose their sensi- ized, controlled, clinical trial, DBS was more effective in
tivity, and the prolonged effectiveness (10 years or more) reducing symptoms and increasing quality of life than
of l-dopa therapy is questionable.173-175 A further complica- medication.194,195 This group also found that although some
tion of l-dopa therapy is the development of involuntary side effects were worse (e.g., brain hemorrhage), the total
movements (dyskinesias) and the “on-off” phenomenon—a number of adverse reactions was greater in the medication
short-duration response resulting in sudden improvement of group. Whether stimulation of the subthalamic nucleus is
symptoms followed by a rapid decline in symptomatic relief neuroprotective, that is, prevents further degeneration, is
and perhaps the appearance of dyskinesias and/or dysto- presently under investigation. Thalamic stimulation is used
nias.176,177 With time the “on” effect becomes of shorter and
shorter duration.173,176,178,179 Controlled-release or slow-release
l-dopa may decrease these side effects. The effectiveness of *References 6, 7, 76, 173, 181, 182.
for decreasing tremor. Therapists may find that intense treat- and without vision and the differences between the two con-
ment immediately after these surgeries may be able to take ditions (see the section on balance in Chapter 22). Assessing
advantage of neural plasticity. challenges to balance such as tandem walking or standing
Fetal transplantation of the substantia nigra to the cau- on a compliant surface is important, especially in the early
date nucleus remains under investigation. A double-blind, stages of the disease. This may be the first sign of balance
placebo-controlled trial was completed with mixed re- impairment. Posturography is the most sensitive measure of
sults.192,196-198 Studies continue, including those of dose, cell postural instability, especially in the early stages of the dis-
type, and placement of cells. Recently, however, there was a ease (Hoehn and Yahr stages 1 and 2).58 A clinically useful
report of Lewy-body inclusions in grafted cells 14 years tool to assess dynamic balance is the functional reach test,
after the transplant.199 The authors concluded that Parkinson which has been shown to be an effective, predictive tool
disease was an ongoing process and that what caused the in people with Parkinson disease as it is in the elderly.204 The
disease initially, also affected the grafted cells. Balance Evaluation Systems Test (BESTest) is also an ap-
propriate comprehensive measure for those with Parkinson
Examination of the Client with Parkinson Disease disease. Obtaining a falls history continues to be a reliable
The previous sections introduced the symptoms of Parkinson predictor of future falls and is easy to measure. (Refer to
disease and hypothesized pathophysiological explanations Chapters 8 and 22 for specifics on these tests.)
for these symptoms. Examination of the client with Parkinson An assessment of chest expansion and vital capacity
disease should include the degree of rigidity, bradykinesia, should also be included. This is important because of their
balance impairments, and gait abnormalities and how contribution to the complication of pneumonia. For this
much these symptoms interfere with ADLs—that is, how reason, when rigidity is assessed, the muscles of respiration
the symptoms are influencing the client’s participation in should be included, along with extremity and trunk assess-
life. The outcome measures used in the examination of ment. Active and passive range of movement, general
patients with Parkinson disease should be as objective as strength, chest expansion, and vital capacity should also be
possible. measured on regular intervals. At present a complete and
The Hoehn and Yahr Scale (see Table 20-1) is frequently easy-to-use form for evaluation does not exist for Parkinson
used to describe the general severity of disease.165 The disease.
Unified Parkinson’s Disease Rating Scale (UPDRS) is the
most widely used assessment tool to describe all facets of General Prognosis, Treatment Goals,
impairment: cognitive and emotional status, ADL ability, and Rationale
motor function, and side effects of medication.200-202 The As with all treatment, the prognosis (functional goals and
UPDRS is also frequently used to measure the efficacy of established time parameters) is based on the general goals
treatments. Another clinical scale is the Core Assessment related to the findings from the examination of each client
Program for Intracerebral Transplantation (CAPIT), which and the client’s expectations and functional requirements.
includes timed tests.203 This scale was designed to standard- Parkinson disease must be understood as a degenerative
ize assessments of patients with Parkinson disease who disease when establishing the prognosis and treatment plan.
undergo surgical intervention. It is comprehensive and more Nonpharmacological and surgical interventions, especially
time-consuming and therefore tends to be used more in physical therapy treatment, are especially important in the
research than in the clinic. Knowledge of these scales will beginning of the disease.205 In general, goals include in-
help the physical therapist in communication and interac- creasing movement and range of motion (ROM) in the entire
tions with other health care professionals even though the trunk as well as the extremities, maintaining or improving
scales may not be ideal for planning physical and occupa- chest expansion, improving balance reactions, and maintain-
tional therapeutic interventions. ing or restoring functional abilities. Increased movement
Assessment of functional activities will be most benefi- may in fact modify the progression of the disease.206,207 It
cial for treatment planning and reevaluation. In addition to may further help to retard dementia. Although l-dopa
assessing how the patient performs the activity, the time it decreases the bradykinesia, it alone will not be effective
takes to complete an activity must be measured. For examin increasing movement or improving balance; therefore,
ple, gait is assessed by general pattern, speed, and distance, aggressive intervention in the early stages is necessary.
as well as the effects of interfering stimuli including walking Increasing trunk rotation goes hand in hand with increasing
while performing cognitive tasks. It is advantageous to range of movement and motion in general. The longer cli-
evaluate forward and backward walking as well as braiding ents are kept mobile, the less likely they are to develop
and the ability to alter gait speed in each of these condi- pneumonia and the longer they can maintain independence
tions.145,146 Available objective tests of gait and functional in ADLs. Ideally, rehabilitation interventions should begin
mobility include the Timed Up-and-Go Test, 10-meter walk at the first sign of the disease, but this is not always possible.
test, the 5 or 10 Times Sit-to-Stand Test, the Dynamic Gait Treatment initiated while the disease is still unilateral
Index, or any of the objective standardized tests presented in (Hoehn and Yahr stage 1) is more advantageous.208,209
Chapter 8. Careful observation of how the person performs
a task would be useful for treatment planning. For example, Treatment Procedures
when rising from a chair, does the patient move forward Overall, physical rehabilitation is effective in the treatment
in the chair, place the feet underneath the knees, and lean of people with Parkinson disease. The results are greater
forward before rising? when treatment is started early in the disease process, but it
A careful analysis of balance is imperative for the patient has been shown to be effective in Hoehn and Yahr stages 1
with Parkinson disease. This must include assessment with to 3. The American Academy of Neurology recommends
physical therapy in its practice parameters.210 The bottom Many relaxation techniques appear to be effective in re-
line is that treatment by movement specialists that incorpo- ducing rigidity, including gentle, slow rocking, rotation of the
rates complex, sequential movements with multiple sensory extremities and trunk, and the use of yoga (see Chapters 9 and
inputs creates demands for responses that are environmen- 39). In the client with Parkinson disease, success in relaxation
tally appropriate, challenges balance, uses large-amplitude may be better achieved in the sitting or standing positions
movements, and is fun and effective. Many treatment regi- because rigidity may increase in the supine position.91 Further-
mens have been used, and almost all have been successful. more, because the proximal muscles are often more involved
Animal research indicates that exercise and forced functional than the distal muscles, relaxation may be easier to achieve by
movements may protect the dopaminergic neurons.211 The following a distal-to-proximal progression. The inverted posi-
following paragraphs will provide more precise information tion may be used with care. Initially this position facilitates
and more precise details. some relaxation (increase in parasympathetic tone) and then
Basic principles for treatment of the person with Parkinson increases trunk extension, which is important for the parkin-
disease will, of course, depend on the areas of impairment and sonian client. Relaxation may also be effective in reducing the
handicap revealed in the evaluation. Certain principles, how- tremor of Parkinson disease. Once a decrease in rigidity has
ever, are true for all stages of the disease. First, the activities been achieved, movement must be initiated in order to use the
selected must engage the patient: the patient must find the newfound range in a functional way.
activities interesting enough to do them regularly. Variety is Therapeutic Programs. Exercise itself is important for
important to facilitate shifts in movement as well as in the person with Parkinson disease. There is a relationship
thought. And movements must be big! (In fact, one treatment between longevity and physical activity.229 Those who exer-
technique even uses that word in its name.) Activities that are cise have lower mortality rates.229 Some evidence also indi-
designed to improve balance are valuable even in the early cates that exercise may alter the magnitude of free radicals
stages of the disease. To date, many rehabilitative techniques and other compounds linked to aging and parkinsonism.
and exercises have demonstrated improvement in function for Immunological function may also be improved with exercise.
people with Parkinson disease, and there have now been a few Sasco and colleagues103 demonstrated a link between a lack
randomized clinical trials with small numbers of patients to of exercise and development of parkinsonism. Finally, the
test efficacy of the varied techniques. Programs that empha- role of aerobic fitness itself may be a factor in reducing dys-
size sensory-motor integration, agility, and motor learning function.103 Animal data indicate that functional exercise de-
demonstrate decreased progression of disease and improved creased DA loss after a variety of lesion models.129,208,209,211,230
motor function.212-227 Programs that involve the coordination Some of the animal activities were similar to the complex,
of dual motor-cognitive tasks and complex sequences of sensory-motor and agility programs now used in patient
movements and that force the participant to quickly change programs.213,218,221,222 Aerobic exercise may improve pulmo-
movements dependent on environmental conditions have re- nary function in patients with Parkinson disease because these
sulted in improved performance on the Timed Up-and-Go functions appear to suffer from deficiencies in rapid force
Test, the UPDRS, the 10-meter walk test, and a variety of bal- generation of the respiratory muscles, similar to limb muscu-
ance tests. Some of these programs include the Lee Silverman lature.231 Exercise is most beneficial when it is begun early in
Voice Treatment (LSVT BIG) program, sensory attention the disease process as is recommended in all books, pam-
focused exercise (PD SAFEx), ballroom dance, Zumba, tai phlets, and websites for the patient.232 (Refer to the list of
chi, karate, computer game playing, and alpine hiking. websites at the end of this chapter.) All research on the effects
Most of the studies referenced previously have included of exercise programs in parkinsonism indicates this point.
people at Hoehn and Yahr stages 1 to 3. As the person pro- When the use of forced functional activities is delayed too
gresses, practice of precise ADLs is advisable. These in- long, few beneficial effects of exercise on the DA system have
clude rising from a chair, getting out of bed, turning in bed, been shown in animal studies.208,209 Hurwitz233 found that
adjusting covers, and being aware of posture. At the later patients who were still independently mobile at home and in
stages of the disease, breathing exercises will need to be a the community benefited the most from a home program.
more prominent aspect of treatment. Big movements still Schenkman and Butler228 also indicate that patients in the
need to be stressed. At these later stages of the disease, use earlier stages of the disease had the best potential for im-
of assistive equipment may also need to be taught. What fol- provement. If patients practice regular physical exercise in
lows are ideas for treatment of more specific aspects of the conjunction with disease-specific exercises, the ill effects of
disease. These are ideas and are not exhaustive. The words inactivity will not potentiate the effects of the disease process
big, fun, and novel are good words to remember when plan- itself. Although most patients with Parkinson disease can
ning treatment. achieve an adequate exercise level, many clients have fitness
Decreasing Rigidity. Movement throughout a full ROM levels that are poor or very poor before the medical diagno-
is crucial, especially early in the disease process, to prevent sis.122 Exercise, even once a week, can be effective in improv-
changes in the properties of muscle itself. In Parkinson dis- ing gait and balance in clients with Parkinson disease when
ease the contractile elements of flexors become shortened practiced over several months.212,234
and those of the extensor surface become lengthened, en- So far almost all studies have found that exercise under
hancing the development of the flexed posture that is tradi- the guidance of a therapist is effective.213-227 Palmer and col-
tionally present.228 For most patients, treatment proceeds leagues235 used precise, quantitative measures to assess mo-
better if rigidity is decreased early in the treatment session. tor signs, grip strength, coordination, and speed as well as
In fact, movement therapy interventions appear to have more measurements of the long-latency stretch reflex after
lasting effects when the treatment is performed during the two exercise programs in patients with Parkinson disease.
“on” phase of a medication cycle. These two programs were the United Parkinson Foundation
program and karate training. Their results indicated im- One program designed for those at Hoehn and Yahr stage 2.5
provement over 12 weeks in gait, grip strength, and coordi- or 3 begins with seated activities for upper extremities
nation of fine motor control tasks and no change in a decline (Figure 20-8, A) and combination movements for warmup
in movements requiring speed. The patients all felt an (Figure 20-8, B). The participants then progress to standing
increase in general well-being. A study by Comella and and marching activities that incorporate coordinated move-
colleagues236 as well as one by Patti and colleagues237 also ments of arms and legs as well as balance and trunk rotation
found decreases in parkinsonian symptoms with physical (Figure 20-9). All movements are performed to music similar
and occupational therapy. However, these studies found no to that used in aerobics classes in any gym or health club
long-term carryover once therapy had been discontinued. (Figure 20-10). (The rationale for the use of external cues and
The authors never explain the exercise program precisely the role of rhythm in gait training are discussed in subsequent
nor the instructions provided for a home program. paragraphs.) A cool-down period allows participants to prac-
Rhythmical exercise has been shown to decrease rigidity tice fine motor coordination activities of the hands (Figure
and bradykinesia and improve gait over time.212,238-254 Ball- 20-11). Many Parkinson disease associations also have audio-
room dancing is a form of rhythmical therapy for patients tapes for exercises (e.g., United Parkinson Foundation).
with Parkinson disease that incorporates rhythmical move- The use of computer games to improve symptoms is cur-
ment, rotation, balance, and coordination.212 A program of rently under investigation. These games force the participant
tango versus waltz and foxtrot indicated that although both to move in precise ways or to shift weight to score points.
groups improved on the UPDRS motor scale, Berg Balance Many “off-the-shelf” games exist and have been used with
Scale, 6-minute walk distance, and backward stride length, older adults (the predominant patient population of those
the tango group had greater improvements.214,217,225 The with Parkinson disease) to increase activity levels. For some
waltz and foxtrot, which are easier dances, may be beneficial with Parkinson disease, even in the early stages, these games
for those at more advanced stages of the disease. The use of
dance also facilitates changing direction. Our program using
Latin dance (predominantly mambo and cha-cha) and other
weight-bearing exercise demonstrated similar improvements
in balance and especially initiation of gait.212,234,247,255 Similar
effects were seen in tai chi, which demands attention to
movement and increases challenges to balance and control of
movement. Tai chi has been shown to be effective in improv-
ing gait and balance parameters.220
Studies using a program emphasizing sensory awareness
of the size of movement have shown improvement in both
speed of movement and gait parameters.224,226,227 PD
SAFEx,226 a program that focuses attention on sensory
awareness, was shown to improve gait and function on the
UPDRS in a randomized controlled study. A group that
engaged in aerobic exercise alone improved gait but not
symptoms. A group that continued usual activities did not A
demonstrate improvements on any outcome measure. These
authors concluded that programs emphasizing increased
sensory feedback and awareness were superior in reducing
the symptoms and improving the function of patients with
Parkinson disease.
Treadmill training has been used in Parkinson disease
exercise programs. Use of the treadmill with body-weight
support increases safety and allows the therapist to control
speed of movement as well as perturbations. Some studies
have used cued treadmill training with good results and car-
ryover to the home.256 Cognitive tasks and other dual tasks
have also been added during treadmill training with good
results.257 These studies found collectively improved mea-
sures of balance and gait, as well as reduced fear of falling
and number of falls.213,221 B
Physical activity and movement appear to increase quality
of life by decreasing depression and improving mood and Figure 20-8 n Seated aerobics or warmup exercises. A, Clients
initiative.248,258 Group classes can serve as an extra support are using bilateral upper-extremity patterns to facilitate trunk rota-
system for patients with Parkinson disease and their spouses.* tion. Instruction was to let the head follow the hands. B, This exer-
A carefully structured low-impact aerobics program appears cise encourages trunk rotation, large movements, and coordination
to be beneficial to patients even with long-standing disease.234 of the upper and lower extremities. Clients are to reach with the
arms and touch the opposite foot. This coordination is difficult for
those with Parkinson disease, and many clients initially could not
*References 210, 226, 227, 238, 249, 258. move the arms and legs at the same time.
are too fast or too confusing. A feasibility study showed that

disease-specific games could be used, and enjoyed by those
with Parkinson disease.259
The most successful exercise programs appear to be
those that incorporate context-dependent responses and a
varied environment. All of the previously listed examples
of these activities are presented in Box 20-1. Aerobic
exercises that are not as effective in requiring context-
dependent responses are presented in Box 20-2. Research
has shown the importance of adjusting the response to the
specific task and has also demonstrated the importance of
practice for the parkinsonian patient.157,260 The principles
of motor learning are of paramount importance in the
treatment program of these patients. Random practice
may enable the patient to learn the correct schema by
Figure 20-9 n Initial warmup in standing. Clients are to walk which to regulate the extent, speed, and direction of the
with the head up, with the back as straight as possible, and to take movement. Random practice also may be important in
large steps. When the group began, walking was the major aerobic facilitating the ability of the patient to shift attention and
activity and was used to increase endurance and encourage move- to learn to access “working memory.” The parkinsonian
ment. Nonambulatory patients march in place while seated. patient may benefit from visual instruction and mental
rehearsal before performing the movement.137,157 In addi-
tion, the instructions used need to be pertinent to the task
at hand.
n EXERCISES THAT PROMOTE

BOX 20-1
CONTEXT-DEPENDENT RESPONSES
The following exercises promote context-dependent responses
and are recommended for people with movement disorders:
1. Walking outdoors
2. Karate and other martial arts
3. Dancing (all forms)
4. Ball sports (various types)
5. Cross-country and downhill skiing
6. Well-structured, low-impact aerobics classes
7. Treadmill training with guidance of a movement
Figure 20-10 n Walking in a “waltz rhythm” (slow, quick, quick) specialist
emphasizes a big step for the slow step. Note lack of automatic arm This list is a sample of activities; it should not be
swing. Also note flexed posture of seated patient during rest period. considered all inclusive.
BOX 20-2 n EXERCISES THAT PROMOTE

FITNESS AND INCREASE RANGE OF MOTION
BUT NOT CONTEXT-DEPENDENT RESPONSES
The following exercises promote fitness and increase range
of motion but not context-dependent responses and are
recommended for people with movement disorders.
1. Treadmill walking without guidance or supervision
2. Stationary bicycle riding
3. Using strengthening machines and free weights (with
low weights or low resistance)
4. Using step exercises and stair climbers
5. Using rowing machines
Figure 20-11 n Cool-down period allows time to work on fine 6. Swimming laps
finger movements. Thumb abduction with rounded fingers and This list is a sample of activities; it should not be
various rhythms are used to increase coordination. Note “masked considered all inclusive.
face” appearance.
Strengthening. Strengthening exercises have been pro- present a visual cue for the patient who has freezing epi-
moted for the patient with Parkinson disease. With disuse sodes. Canes can be especially useful for patients who fall
comes decreased strength. Weakness occurs with initial con- because of freezing. If a specialized cane is not available,
traction and also with prolonged contraction. Manual mus- the client can turn his or her own cane upside down. Other
cle testing may not reveal losses in strength; however, most visual cues have been used to help initiate movement after
of the successful exercise programs previously mentioned freezing. For example, one patient tosses pennies ahead of
did include functional strength training as part of the pro- him and steps over them. (He cautions that one should not
gram. High-resistance eccentric resistance can produce mus- bend to pick them up as this will again lead to freezing.)
cle hypertrophy and may effect improvements in mobility.261 Another watches the movement of a person walking beside
Another study used “sports activities” in a twice-per-week him; the movement of that person’s feet encourages his feet
program.158 The program included exercises on land de- to move. The U walker has a laser line that can be added to
signed to improve gait and balance and exercises in the provide lines on the ground. Morris and colleagues147 have
water to increase strength. These investigators reported sig- tried to increase carryover of visual stimuli by incorporating
nificant improvements in UPDRS scores, cognitive function, them with a program of visualization. Their clients practiced
and mood in addition to ADL and motor scores during the walking with lines until the steps were near normal in size;
14-week program. Interestingly, they also found decreases the clients were then to visualize the lines on the floor as
in dyskinesia. The greatest changes in exercise appeared they walked. Their visualization program met with initial
early and were maintained up to 6 weeks after cessation of success. Increasing the magnitude of the step or the ampli-
the exercise program. According to the literature, functional tude of the movement appears to be the most important
strength training seems to be more effective than weightlift- component for improvement in gait and a decrease in freez-
ing if the goal is improvement in ADLs.158 An important ing.147 Tactile cueing has also been demonstrated to improve
part of any strengthening program is the trunk musculature. gait ability.266
Spinal extensors need to be exercised, and spinal flexibility Gait rehabilitation must include walking in crowds,
likewise encouraged.262 through doorways, and on different surfaces. Practicing walk-
Use of Cues for Improving Gait. As the disease pro- ing slowly and quickly is important, as is walking with dif-
gresses, intensive exercise programs may need to be revised fering stride lengths, because in the real world step length
or altered. By stage 2.5, gait disorders are the most common and speed must change with environmental demands. The
diagnosis for which the person with Parkinson disease will principles of motor learning presented in Chapter 4 appear to
see a therapist. Many aspects of gait are amenable to treat- be very helpful for facilitating carryover of the therapeutic
ment. The problems that cause the biggest ambulation limi- effects in our preliminary studies. One word of caution, how-
tation are freezing and small steps. Both auditory and visual ever; as previously mentioned, the person with Parkinson
stimuli have been used in treatment of parkinsonian gait disease has difficulty performing two tasks at once, such as
disorders. Thaut and colleagues263 demonstrated that use of walking and performing math problems or walking with a
a metronome or carefully synthesized music improves stride glass of water on a tray.113,130,155 The patient may have to
length and speed and that these improvements remain up concentrate only on walking as the disease progresses, to
to 5 weeks after the cessation of the auditory stimulus.234 increase patient safety.
Melnick and colleagues234 also demonstrated both immedi- Balance. Another problem for which therapy is indi-
ate and longer-lasting improvements in gait with a rhythmi- cated is impaired balance, especially because drug and
cal exercise program once a week in patients needing assis- surgical treatments are ineffective in remediating this prob-
tance to walk. A study by Nieuwboer and colleagues256 used lem. This problem will eventually affect all persons with
auditory, visual, or somatosensory cues in the patient’s Parkinson disease.267 If at all possible, the client should be
home. The patient chose the cue that was best for him or her. instructed to practice balance exercises at the early stages
The cues were provided in a variety of tasks including walk- of the disease. Equilibrium reactions in all planes of move-
ing with dual tasks and walking sideways and backward. ment and under different controls should be encouraged.
Cues were effective in decreasing freezing, but there was Techniques to increase dynamic balance control should be
only a small effect when the cues were stopped. There may included, especially turning the body and turning the head.
be a difference in the use of cueing for those who freeze All three balance strategies need to be addressed and then
during gait and those who do not. The use of cues may be practiced in a variety of environmental conditions. The
more effective for those who do not have frequent freezing newer computerized games that target balance have pro-
episodes.264 vided a fun and therapeutic method for keeping interest in
People with Parkinson disease find climbing stairs easier balance exercises.130,135,136,142,143 (See Chapter 22 for other
than walking on a flat surface because of the visual stimula- procedures to improve balance.)
tion provided by the stairs. Visual stimuli have been effec- Dual Task Performance. Rarely will the client with
tive in freezing episodes. These include the use of lines on Parkinson disease state that he or she has difficulty perform-
the floor and stair climbing. Martin55 found that parallel ing two tasks at once. Nonetheless, this is quite apparent in
lines were more facilitating than other lines and that the very simple activities, such as requiring the patient to count
space between lines was also important; the lines cannot be backward and walk at the same time.146,268 One solution is
too close together. The use of visual stimuli has scant evi- to instruct the client to attend to only one task at a time.
dence of carryover. One client used visual stimuli in special Another is to have the client practice doing two things at the
glasses that provided constant lines for the client to step same time and constantly alter activities in a random prac-
over. At present these glasses are not commercially avail- tice mode during treatment. The efficacy of these two
able. Dunne and colleagues265 described a cane that could approaches has yet to be studied.
Activities of Daily Living. Transitional movements Patients frequently ask about the timing of medication
pose great problems for the client, especially by Hoehn and and exercise. For any form of exercise in parkinsonism to be
Yahr stage 3. This is most likely because normal postural effective, movement must be possible, especially movement
adjustments are no longer automatic and they become a through the full arc of the joint. It seems plausible, therefore,
sequential task. Practice with frequent review is helpful. that exercise should be performed during the “on” period of
Some researchers report improvement in moving from a the medication cycle. On the other hand, perhaps a more
seated to a standing position after practicing techniques long-lasting effect would result if the patient with Parkinson
designed to increase forward weight shift (e.g., leaning on a disease tried to exercise without medication. The question of
chair while standing up).269,270 Visualization of this task has the effects of exercise on DA agonist absorption was inves-
demonstrated carryover. If getting up from a chair becomes tigated by Carter and colleagues.272 These authors con-
too difficult, chairs with seats that lift up have been used cluded that the effect was variable from patient to patient,
effectively. but the response of each patient was consistent. However,
Bed mobility is another important consideration for pa- none of the patients exercised vigorously, which may have
tients with Parkinson disease. Rolling in bed and rising from skewed the results. Reuter and colleagues273 interpreted a
the supine position become difficult and need to be practiced, decrease in dyskinesia seen after an exercise program as
with increased emphasis on trunk rotation. A firm bed may indicative of more efficient DA absorption. Nevertheless,
make getting in and out of bed easier. Rolling and getting out this study supports the concept that the patient needs to be
of bed is a task that may be easy for the patient on the hard mat “in tune” with his or her own response and adjust medica-
tables in the clinic but difficult on the softer bed at the client’s tions and exercise to a schedule accordingly. The therapist is
home. Tempur-Pedic beds may make movement even more also involved in the prescription of assistive devices. The
difficult than traditional beds by Hoehn and Yahr stage 3. Most use of ambulatory aids for patients with Parkinson disease is
patients report that satin sheets with silk or satin pajamas make an area with no clear-cut guidelines. Because coordination
moving in bed far easier. This is true in both the early and later of upper and lower extremities is often difficult, the ability
stages of the disease. Teaching the client to roll onto the side to use a cane or walker is often limited. The patient may
and lower the legs off the bed facilitates getting out of bed; the drag the cane or carry the walker. Walkers with wheels
client may not be using this method and so learning or relearn- sometimes increase the festinating gait, and the patient may
ing this movement is important. Beds with a head that can be simply fall over the walker. Nonetheless, four-wheel walkers
raised electrically may be helpful as the disease progresses, but with pushdown brakes appear to work best for many clients.
while sleeping the patient should lower the head as close to A walker that is in the brake condition at the start and
horizontal as possible. requires the patient to push on handles to walk may also
Breathing exercises are crucial for the patient with Parkinson be safer. For patients with a tendency to fall backward, an
disease. As stated previously, the most common cause of assistive device may simply be something to carry backward
death is pneumonia. Chest expansion may be included in with them. Therefore the reason for using the assistive
upper-extremity activities such as swinging the arm. The cli- device must be carefully assessed. Walkers, walking sticks,
nician may also have the client shout—especially with some or canes can be helpful for the person who is able to walk
kind of rhythmical chant, even a simple “left, right” while with a heel-toe gait pattern but lacks postural stability. The
walking. With disease progression, specific breathing exer- height of walker or cane should be adjusted carefully to
cises need to be incorporated. This is crucial for the patient promote trunk extension and avoid an increase in trunk flex-
who is no longer able to walk. ion. A walking stick, or the use of two sticks as in hiking, is
In addition to treatment in the therapy department, the less likely to promote flexion than a cane. A survey by
parkinsonian client also should be given a home program. Mutch and colleagues250 in Ireland found that nearly half of
The home program should encourage moderate, consistent the patients responding used some type of assistive device.
exercise as part of the normal day. Periodic checks may These devices included devices for walking, reaching, rising
enhance compliance. Fatigue should be avoided and the from bed, and performing ADLs. Patients with Parkinson
exercise graded to the individual’s capability. The therapist disease may also benefit from assistive devices for eating or
should keep in mind that learned skills such as various writing.
sports are sometimes less affected than automatic move- As Parkinson disease progresses the patient may experi-
ments, perhaps because these skills may rely on cortical ence difficulty in swallowing and even chewing. Therapy for
involvement.16 oral-motor control should be initiated, and a dietician con-
Fatigue is a frequent complaint of people with Parkinson sultation may be necessary to ensure adequate nutrition.
disease. Although it has been correlated with disease pro- A dietician may also be beneficial in guiding the patient’s
gression, depression, and sleep disturbances, it also exists in protein intake. A diet high in protein may reduce the respon-
up to 44% of those without depression or sleep difficulty.271 siveness of the patient to DA replacement therapy.146 Regu-
This type of fatigue is over and above what is associated lating the amount and timing of protein ingestion can im-
with the exertion of an exercise program and may be one prove the efficacy of drug treatment in some patients. Use of
reason people with Parkinson disease no longer exercise. vitamins is a subject that appears on many websites for pa-
The client with Parkinson disease frequently experiences tients with Parkinson disease, and the patient should be re-
postexercise fatigue. If a person is so tired after exercise that minded to consult with his or her physician when changing
he or she cannot perform normal ADLs, exercise will not vitamins.
become a part of the client’s daily routine. Postexercise The resurgence of surgery as a treatment alternative in
fatigue is easily alleviated by a gradual and extended cool- Parkinson disease, including stimulation of deep brain sites
down period. that may alter neuroplasticity, means that the therapist will
face new and exciting challenges in treatment.274 Intense with ubiquitin and induces intranuclear inclusions and inter-
physical therapy, especially incorporating complex motor ference with mitochondrial function. The defect is charac-
skills, has been demonstrated to be effective in improving terized by severe loss of the medium spiny neurons and
function after a subthalamic nucleus lesion in animal preservation of the ACh aspiny neurons. There are decreases
studies.275 Therefore, intense physical therapy after surgery in choline acetyltransferase (CAT), ACh, the number of
may be necessary to maximize benefits from all surgeries in muscarinic ACh receptors, glutamic acid decarboxylase, and
Parkinson disease (as well as in Huntington disease and the substance P. There is generally no decrease in DA, norepi-
dystonias). nephrine, or serotonin (5HT), although more recent studies
Finally, therapeutic rehabilitation and exercise may mod- with single-photon emission computed tomography (SPECT)
ify but cannot halt or reverse the progression of this degen- indicate that DA does diminish significantly in the later
erative disease. The therapist can assist the client and family stages of the disease.279
in coping with the constraints of this disease, enhancing the Huntington disease is usually manifested after the age of
patient’s quality of life throughout its course. As stated in 30 years, although childhood forms appear rarely. Those
one study of Parkinson disease, the total cost of treatment younger than 20 years with the disease account for approxi-
must also include the cost to the spouse or other family mately 10% of all people with Huntington disease. Death
members.2 from this disease occurs about 15 to 25 years after the onset
of symptoms, although as in Parkinson disease the earliest
Differences between Parkinson Disease symptom is not known.
and Parkinson Plus Syndromes: Theoretical A marker for the Huntington gene has been detected.278
and Practical Considerations If the family pedigree is known and the chromosomes of the
Several other neurodegenerative diseases are grouped to- parents can be obtained, detection of which offspring have
gether as “Parkinson plus” syndromes. Clients with these the faulty chromosome is possible presymptomatically. Of
syndromes usually do not respond to l-dopa intervention. course, early detection of this disease involves ethical and
The most common of these is progressive supranuclear practical issues. At present, although testing is available, it
palsy (PSP). Symptoms of this disease include bradykinesia, is not widely used. Furthermore, testing for Huntington dis-
gait instability with frequent falls, rigidity, and a vertical ease is typically available only to those older than 18 years.
gaze palsy. These clients can be evaluated and treated in a Despite these problems, localization of the gene and the
manner similar to clients with Parkinson disease. However, repeat is promising and offers hope for improved means of
PSP usually involves more cognitive impairment and the treatment.
progression is more rapid; within a decade the patient is Huntington disease affects neurons in the basal ganglia as
typically immobile. well as the frontal cortex. The movement disorders are
Multiple system atrophy (MSA) is a degenerative disease presumed to be related to degeneration of the striatal neu-
that affects various areas of the CNS, causing problems with rons, specifically the enkephalinergic neurons.16 The cogni-
movement, balance, and autonomic functions. The disease is tive and emotional symptoms are associated with cortical
characterized by bradykinesia and rigidity and a tendency to destruction.
walk with a wide base of support. A person with MSA often
has frontal lobe dysfunction as well. Unfortunately, l-dopa Symptoms
is not effective in treating this disorder. Some of the signs and symptoms of Huntington disease are
Because these syndromes are more rare than Parkinson similar to those of Parkinson disease: abnormalities in pos-
disease and far more variable, no studies have been under- tural reactions, trunk rotation, distribution of tone, and ex-
taken regarding rehabilitation intervention efficacy. Be- traneous movements. Individuals with Huntington disease,
cause accurate differential diagnosis is important in patient however, are at the other end of the spectrum; rather than a
planning, a thorough evaluation by a neurologist is highly paucity of movement, they exhibit too much movement,
recommended. which is evident in the trunk and face in addition to the ex-
tremities. The gait takes on an ataxic, dancing appearance
Huntington Disease (in fact, chorea means to dance in Greek), and fine move-
Huntington disease (formerly Huntington’s chorea) is an- ments become clumsy and slowed.280 As with the person
other degenerative disease of the basal ganglia.276 It is the with parkinsonism, there is a decrease in associated move-
classic disorder representing hyperactivity in the basal gan- ments (e.g., arm swing). The extraneous movements are of
glia circuitry.277 This disease gets its name from the family the choreoathetoid type, that is, involuntary, irregular iso-
of physicians who described its patterns of inheritance. lated movements that may be jerky and arrhythmical as in
Huntington disease is inherited as an autosomal dominant chorea, to rhythmical and wormlike as in athetosis. Usually,
trait and affects approximately 6.5 per 100,000 people.3 The however, these occur in successive movements so that the
defect is on the short arm of chromosome 4.278 The defect entire picture is one of complex movement patterns. The
alters DNA so that there is an increase in the cytosine- “movement generator” aspects of the basal ganglia seem to
adenine-guanine (CAG) sequence; in normal individuals be continuously active, as would fit the hypothesis of a dis-
there are 10 to 28 CAG triple repeats, but in the individual ruption in the indirect pathway. As the disease progresses,
with Huntington disease there are 36 to 120 repeats.64 the choreiform movements may give way to akinesia and
The longer the length of the CAG triple repeats, the earlier rigidity.
the onset of the disease. The CAG repeat is related to gluta- Gait patterns of the person with Huntington disease are in
mine. The target protein affected by the polyglutamine ex- some ways similar to those of Parkinson disease. Gait veloc-
pansion has been named huntingtin. Huntingtin combines ity and stride length are decreased. The decrease in velocity
is correlated with disease progression. Unlike the person contractions in those patients with rigidity. Presence of ath-
with Parkinson disease, however, the person with Huntington etosis or dystonia was associated with slow, reciprocal con-
disease has a decreased cadence as well.281 The base of sup- tractions. During sustained contractions, EMG activity dem-
port is increased (again unlike the pattern seen in Parkinson onstrated brief, irregular cessation of activity in the choreic
disease). In addition, lateral sway is increased along with patients. Thus patients with Huntington disease have inter-
great variability in distal movements. ruption of normal motor function at rest and during sus-
Disruptions in movement for the person with Huntington tained activity (e.g., stabilizing contractions).
disease reflect the role of the basal ganglia in movement. For The abnormal postural reactions of the person with
example, the person with Huntington disease, like the per- Huntington disease may occur from a misinterpretation of
son with Parkinson disease, has difficulty responding to in- sensory input, especially vestibular and proprioceptive (sim-
ternal cues; he or she also has difficulty with internal ilar to the parkinsonian syndrome). However, the dementia
rhythms. Kinematic analysis of upper-extremity complex of Huntington disease precludes further testing.
tasks demonstrates that the person with Huntington disease In addition to the involvement of the motor systems, the
must rely on visual guidance in the termination of a move- individual with Huntington disease also shows signs of
ment. This has been interpreted to indicate impairment in dementia and emotional disorders that become worse as the
the development and fine-tuning of an internal representa- disease progresses. Neuropsychological tests are therefore
tion of the task.282 These clients have increasing difficulty part of the Unified Huntington’s Disease Rating Scale
with more complex movements in the absence of advanced (UHDRS). The client may show lack of judgment and loss
cues.283,284 The lack of internal cuing in the person with of memory, deterioration in speech and writing (i.e., severe
Huntington disease has been linked to the increased vari- decrease in ability to communicate), depression, hostility,
ability of response seen in these clients.285 and feelings of incompetence. IQ decreases, with perfor-
The face is also affected in Huntington disease. Speech, mance measures decreasing more rapidly than verbal levels.
breathing, and swallowing lack normal control and coordi- Evidence of ideomotor apraxia is also present, especially as
nation. Speech lacks rhythm, as might be expected with the disease progresses.291 Suicide is fairly common.
decreased internal timing, and is often soft. Swallowing and
therefore eating may also be difficult, are common prob- Stages of Huntington Disease
lems, and often are accompanied by weight loss. In fact, Huntington disease is a progressive disorder. The initial
some suggest that a person with decreased body weight symptoms are most often incoordination, clumsiness, or
and a parental history of the disease is at greater risk.286,287 jerkiness. A classic test for eliciting choreiform movements
Impaired voluntary eye movement is often the first sign of in this early stage is a simple grip test. The client grips the
Huntington disease. The person with Huntington disease examiner’s hand and maintains that grip for a few seconds.
has difficulty with initiation and control of saccadic eye The person with Huntington disease displays what is de-
movements. scriptively called the “milkmaid’s sign”; alternate increases
The exact mechanisms for the production of choreoathe- and decreases in the grip that are perhaps the equivalent of
toid movements are unknown. Because these extraneous the electromyographic abnormalities seen during sustained
movements are part of a person’s normal repertoire of move- contractions. Facial grimacing or the inability to perform
ment patterns, they may be “released” at inappropriate times complex facial movements also may be present very early.
and without any modulation. A postmortem examination In many cases the dementia and psychological symptoms
showed a decrease in GABA that was greater in the globus of Huntington disease occur after the onset of the neuro-
pallidus external segment than the internal segment. This logical signs. In cases in which very subtle personality
agrees with the previously described current model.6 Recent changes occur first, the diagnosis may be more difficult.
use of positron emission tomography (PET) scans demon- Such persons may appear forgetful or unable to manage
strates loss of ACh and GABA neurons.288 A pattern therefore appointments and financial affairs. They may be thought to
may be executed before it is necessary, and inappropriate por- have early senility, or they may show signs of severe depres-
tions of a movement pattern cannot be inhibited. Petajan289 sion or schizophrenia. Early diagnosis may be important,
found motor unit activity indicative of bradykinesia. Record- and SPECT is showing promise for early detection of the
ings of single motor units in the muscles indicates that per- disease.292
sons with Huntington disease have a loss of control evi- With time, the combination of the psychological and
denced by an inability to recruit single motor units.289 As the neurological problems causes the individual to lose all
efforts at control increased, these individuals demonstrated ability to work and perform ADLs. This person eventually
an overflow of motor unit activity that resulted in full cho- can be cared for only in an extended care facility. By this
reiform movements. Those in the earlier stages of the dis- time the choreiform movements have given way to rigidity,
ease demonstrated what the experimenters termed “micro- and the patient is bedridden. Death is usually caused by
chorea,” or small ballistic activations of motor units.289 As infection, but suicide is also common. Figure 20-12 shows
in Parkinson disease, difficulty occurs in modulating the stages of Huntington disease according to Shoulson
motor neuron excitability. Another finding in this experi- and Fahn.293
ment revealed motor unit activity indicative of bradykinesia.
Yanagasawa290 used surface EMG recordings to classify Pharmacological Considerations
involuntary muscle contractions in Huntington disease and Medical Management
patients with varying movement disorders from chorea to Advances in the pharmacological management of Parkinson
rigidity. He found brief, reciprocal, irregular contractions in disease have led to a great deal of research in an effort to
those patients with classic chorea, and tonic nonreciprocal find appropriate drugs for the management of Huntington
Capacity to Capacity to Capacity to

handle manage perform
Engagement financial domestic activities of Care can be
in occupation affairs responsibility daily living provided at
Score Score Score Score Score

Stage 1 Usual level 3 Full 3 Full 2 Full 3 Home 2
Stage 2 Lower level 2 Requires 2 Full 2 Full 3 Home 2

slight help
Stage 3 Marginal 1 Requires 1 Impaired 1 Mildly 2 Home 2

major help impaired
Stage 4 Unable 0 Unable 0 Unable 0 Moderately 1 Home or extended 1

impaired care facility
Stage 5 Unable 0 Unable 0 Unable 0 Severely 0 Total care 0

impaired facility only
Figure 20-12 n Functional stages of Huntington disease. (Reprinted from Shoulson I, Fahn S:
Huntington’s disease: clinical care and evaluation, Neurology 29:2, 1979.)
disease.294-299 At present, however, no fully effective medi- The dementia and personality problems interfere more
cation is available for this disease. Each symptom is treated with life tasks than do the presence of movement disorders.
with its own medication. Medications are usually prescribed as combinations of drugs
The symptoms of Huntington disease indicate an in- to treat the specific emotional and psychological symptoms.
crease in dopaminergic effect. At autopsy a decreased num- Cortical degeneration is most certainly involved, but disrup-
ber of intrinsic neurons of the striatum that contain the tion of the heavy corticostriate projections also may be a
neurotransmitter GABA or ACh are found. Biochemical factor in the progression of this disease. Although alterations
studies reveal a definite decrease in GABA concentration in in DA have been implicated in psychotic problems such as
addition to a decrease in ACh concentration in the basal schizophrenia, the role of the basal ganglia in thought pro-
ganglia. Therefore drug therapy depends on drugs that are cesses is, at best, little understood. In the words of Woody
cholinergic or GABA-containing agonists and those that act Guthrie, “There’s just not no hope. Nor not no treatment
as DA antagonists. To date, the DA antagonists have been known to cure me of my dizzy called Chorea.”304
more effective in ameliorating neurological symptoms; At present the best hope for the person with Huntington
however, these drugs have severe side effects including disease lies in a better understanding of the genetic mecha-
parkinsonism—for example, bradykinesia and rigidity—and nisms causing destruction of the GABA-containing cells in
tardive dyskinesia.294 There is no evidence that improvement the striatum and cortical destruction. In the meantime, cor-
of the choreiform movements leads to improved function. rect and early diagnosis is important in providing the proper
In general, pharmacological treatment is not started until early intervention, which must include counseling.305 In an
the choreiform movements interfere with function because effort to facilitate research into the causes as well as the
these drugs have side effects that may be worse than the treatment of the disease, the Commission for the Control of
chorea.300 Perphenazine, haloperidol (Haldol), and reser- Huntington’s Disease has set up several research centers,
pine are still the most commonly used medications. The including a brain and tissue bank. Research has also begun
first two block the DA receptors themselves; reserpine on the use of tissue transplantation. As with Parkinson dis-
depletes DA stores in the brain. Side effects include depres- ease, the tissue does survive, but the results are even more
sion, drowsiness, a parkinsonian type of syndrome, and preliminary than for parkinsonism.
sometimes dyskinesia. Drugs such as choline, which would
increase ACh concentrations, have produced only transient Examination of the Client
improvement.188 Many efforts have been undertaken to with Huntington Disease
find a GABA agonist that would reduce the symptoms of The standard medical evaluation is the UHDRS.306 This
Huntington disease, but these have been unsuccessful so comprehensive evaluation examines cognitive function as
far.300,301 The problem with finding a medication to increase well as motor function. The physical or occupational ther-
GABA is that such a drug will probably cause inhibition apy evaluation of a person with Huntington disease must
throughout the brain, not just in the basal ganglia. Thus the include an assessment of the degree of functional ability and
individual’s level of alertness and ability to function might how the chorea interferes with function. Which extremities,
be reduced—something the person with Huntington disease including the face, are involved? Does the client have any
can ill afford.301 Riluzole, a drug that blocks glutaminergic cortical control of the chorea or any means (i.e., tricks) of
neurotransmission, has been tried with initial success.302 A allaying these extraneous movements? What exacerbates the
2009 Cochrane review concluded that no pharmacological symptoms? What lessens them? A simple rating scale is the
intervention demonstrated disease-modifying or disease- capacity to perform ADLs (see Figure 20-12). A standard-
progression effects.303 ized ADL assessment with space to write in how the client
performs these activities or why she or he cannot perform occupational therapy treatment techniques for holistic ther-
them would be helpful. apy and for treating specific problems.311-313 See Chapter 9
Gait analysis can include a timed walk test and cadence for other specific therapeutic interventions.
assessment; stride length can then be calculated. A subjec- A study by Zinzi and colleagues311 was a nonrandom pilot
tive assessment of variability and incoordination should also study that incorporated gait, balance, and transfer training.
be made. In addition, posture and equilibrium reactions Strengthening of the extremities, trunk, and muscles of
should be tested. What associated reactions, if any, are pre respiration as well as coordination and postural stability
sent? In assessing posture, care should be taken to observe activities were included. The program was undertaken with
the posture of the extremities in addition to the trunk, head, occupational therapy to include cognitive, rehabilitation,
and neck. Dystonic posturing should be carefully noted, and ADL training. Participants were engaged in the inten-
especially if the client is taking medication. Any changes sive inpatient program for 3 weeks for 8 hours per day,
should be reported to the physician. 5 days a week, 3 times a year. The data indicate that there
A gross assessment of strength should be made, with was significant improvement in motor function and in ADL
particular attention paid to the ability to stabilize the trunk performance and that these subjects did not show deteriora-
and proximal joints. To reduce the effects of rigidity, ROM tion over the 3 years of the study—a positive outcome for a
measurements become important as the disease progresses. degenerative disease.
In the assessment of the client with Huntington disease, Treatment of the person with Huntington disease has
the stage of psychological involvement and mental state some parallels with the treatment of cerebral palsy athetosis.
must be reliably assessed during both evaluation and treat- These techniques, however, must be adapted to the adult.
ment. SPECT and other computer tomography scans may Of critical importance are the techniques for improving co-
give some clues to the amount of cortical and basal ganglia activation and trunk stability. The use of the pivot-prone and
degeneration, which can assist in determining possible corti- withdrawal patterns of Rood are helpful, and their benefit
cal functioning. There does not seem to be a consensus in may be increased with the use of Thera-Band. Neck co-
research or clinical practice for which measurements are contraction and trunk stability may improve, or at least oral
most sensitive to change or best reflect function for the functions may be maintained. In addition, the techniques of
patient with Huntington disease. rhythmical stabilization in all positions as well as heavy
work patterns of Rood should be helpful.314 Yet movements
General Treatment Goals and Rationale practiced out of context may not carry over into functional
Maintenance of the optimal quality of life is the most impor- activities; thus practicing coactivation in functional patterns
tant goal for treatment of persons with Huntington disease during treatment if at all possible is recommended. Whereas
and their families, including maintenance of functional in Parkinson disease the emphasis is on large-amplitude
skills and advice to the family on adaptive equipment. Tech- movements, movements for the person with Huntington
niques that reduce tone may also reduce choreiform move- disease need to be of smaller amplitude and controlled.
ments. Increasing stability about the shoulders, trunk, neck, The gait disorder of Huntington disease has been shown
and hips helps maintain function. Respiratory function to respond to rhythmical auditory stimuli in one study.315
should be kept as high as possible. Again, the evaluation The ability to respond decreases in those most severely in-
results dictate treatment procedures. volved, indicating that treatment in the later stages of the
disease may not be amenable to rhythmical stimuli. Another
Treatment Procedures finding of this study was that cadence was a larger problem
The Commission for the Control of Huntington’s Disease307 than stride length, especially at normal and fast speeds
stated that these individuals are underserved by physical and (compare this with the findings in Parkinson disease). Inter-
occupational therapy. Peacock308 surveyed physical thera- estingly, people with Huntington disease were able to modu-
pists in one state. Of the 585 therapists who responded, only late gait to a metronome but had more difficulty with musi-
15.5% had worked with at least one patient with Huntington cal cues even when the tempos were identical. Subjects with
disease, and 6.2% had worked with more than one patient; Huntington disease demonstrated short-term carryover of
this confirmed the underutilization of physical and occupa- metronome auditory stimuli to gait without auditory stimuli.
tional therapy today. Hayden304 and Peacock308 suggest that Although the long-term carryover was not studied, using a
therapy can improve quality of life for this population. A metronome in gait training may be helpful in clients with
2008 article by Busse and colleagues309 demonstrated that Huntington disease. A more recent study using a metronome
there is still underutilization of therapy services. They also to cue gait during single and dual task gait activities found
found that there no routine outcome measurements for the that participants with Huntington disease had difficulty syn-
stages of the disease, and they suggested that management chronizing steps to a metronome in all conditions.313
of falls and decreased mobility dysfunction could be a treat- Relaxation aids the reduction of extraneous movements.
ment goal of physical therapy interventions. Although ani- In the early stages of the disease methods that require active
mal models of Huntington disease exist, there have been few participation of the client, such as biofeedback and tradi-
studies investigating possible movement interventions. In tional relaxation exercises, may be included. As dementia
one animal study even a little environmental enrichment becomes more apparent, more passive techniques such as
improved the ability of Huntington mice on a rotarod test slow rocking and neutral warmth must be used. These tech-
and slowed the progression of the disease.310 The mice get- niques are also helpful in reducing the choreiform move-
ting even more enrichment showed improvement on more ments of the mouth and tongue, which may prove useful for
behavioral tests as well as changes in the striatum. Recently the dentist and those responsible for proper nutrition of the
a few articles have been published examining physical and client. In most cases of Huntington disease, the individual is
quite thin (almost emaciated) and begins to age rapidly as Symptoms

the disease progresses. The extraneous movements, espe- The deposition of the excess copper in the cornea results in
cially as they become more severe, increase metabolic the classic diagnostic sign of Wilson disease, the Kayser-
demands, and nutrition therefore becomes increasingly im- Fleischer ring: a brownish-green or brownish-red ring found
portant. Attention therefore must be paid to head, neck, and in the sclerocorneal junction.
oral-motor control. Increased pressure on the lips may aid in Several forms of Wilson disease have been classified on
lip closure and facilitate swallowing. Special straws with a the basis of the signs and symptoms. One type entails only
mouthpiece similar to a pacifier may be useful. A dietician liver involvement and no neurological signs. A dystonic
should be consulted for assistance in teaching the family form is most common in those with an onset of the disease
how to prepare balanced and appetizing meals and snacks after age 20 years. The individual shows the same abnormal
that are still easy to swallow. positioning of the limbs and trunk that characterizes the
The degree of dementia influences treatment options. dystonia, rigidity, and bradykinesia seen in Parkinson dis-
Conscious efforts to control extraneous movements will be ease. Associated reactions and facial expressions are absent.
more difficult as cognitive function decreases. New memo- Festinating gait and flexed posture are present. Tremor of
ries and new patterns of movements are more difficult to the hand, head, and body may be present.
establish. The therapist therefore must use techniques that If the onset of the disease occurs before age 20, the
require subcortical control and must keep in mind that the appearance of choreoathetoid movements of the face and
client can sometimes remember old, normal patterns of upper extremities is usually present. The gait resembles that
movement. An encouraging treatment method may be the of the individual with Huntington disease. This early-onset
use of imagery. Yágüez and colleagues312 found that patients form is accompanied by a rapid deterioration.317
with Huntington disease could use imagery to compensate Common to all forms of Wilson disease that involve brain
for impairments in a graphomotor design task. structures are difficulty in speaking and swallowing, incoor-
Peacock’s study308 suggests that group programs includ- dination, and personality changes. The personality changes
ing strength, flexibility, balance, coordination, and breath- are the first signs of the disease, especially emotional lability
ing exercises may be very successful, especially in the and impaired judgment. If the disease progresses, dementia
early stages of the disease. This was confirmed in the study and cirrhosis of the liver increase and motor function pro-
by Zinzi.311 No amount of physical or occupational therapy, gressively decreases.
however, can prevent neuronal cell loss. Because Huntington The postures and movement patterns seen in people with
disease is a progressive, degenerative disease, the client’s Wilson disease include dystonic movements involving twist-
condition will get worse, although the Zinzi study sug- ing and rotation of limbs, with sustained contraction at the
gested that the progression might be slowed with inte- end of the movement.16 As Wilson disease progresses, the
grated therapy.311 Eventually, goals must be aimed at classic abnormal posture of increased flexion occurs, along
preventing total immobility and assisting caretakers in with rigidity that can progress to the inability to move if
transfer techniques and advising them in the use of adap- severe enough. Dystonia, like bradykinesia and choreoathe-
tive equipment. One aspect of treatment that cannot be tosis, belongs on a continuum of the extraneous movements
measured but is important in my view is the degree of hope present with basal ganglia involvement. Dysfunction of the
offered just by the fact that a health professional is provid- cerebellum and intralaminar nuclei of the thalamus may also
ing ongoing care. This may lessen the client’s degree contribute to these impairments of posture and movement.318
of despair and depression and may help maintain quality A peculiar aspect of dystonia is that it can be decreased with
of life. proprioceptive or tactile inputs.16 As with other diseases of
the basal ganglia, an imbalance or abnormal response in the
Wilson Disease neurotransmitters occurs in Wilson disease; however, the
Wilson disease, or hepatolenticular degeneration, is a dis- precise imbalance is not yet known.
ease caused by faulty copper metabolism. The toxic effects
of copper lead to degeneration of the liver and the basal Stages of the Disease
ganglia. Wilson disease, inherited as an autosomal recessive The first symptom of Wilson disease is usually a change in
trait, affects a very small percentage of the population. If the the individual’s personality. Either when this becomes se-
disease is recognized and properly treated, the patient can vere enough or when the movement disorder appears, a di-
expect function without restriction and a normal life span. agnosis can be made by the presence of the Kayser-Fleischer
Wilson disease is characterized by an increase in the ring in the eye or by an analysis of copper metabolism.
amount of copper absorbed from the intestinal tract, a sub- Because Wilson disease is now treatable by chemical means,
sequent elevation in the amount of copper in the blood the full progression of this disease is usually not seen. If left
serum, and an increase in the amount of copper deposited untreated, the dystonia becomes worse and the person be-
in tissue.316 Ceruloplasmin is concomitantly reduced. The comes more rigid. In addition, muscle weakness can occur
increase in tissue copper may interfere with various en- and progress, seizures may develop, and the dementia and
zyme systems of particular cells. The connection of copper personality disorder also become worse.
with DA metabolism may account for the basal ganglia
involvement. Medical Management
Neuronal degeneration is present in the globus pallidus Wilson disease is usually one of the first diseases to be ruled
and putamen and to a lesser extent in the caudate nucleus. out when a patient manifests movement disorders and
Atrophy may be present in the gray matter of the cortex and behavioral problems, especially in the younger patient. Be-
the dentate nucleus of the cerebellum. cause the signs and symptoms of Wilson disease are caused
by an increased absorption of copper, treatment consists of Symptoms

drugs that will inhibit this absorption. Concomitantly, cop- Dyskinesia is defined as an inability to perform voluntary
per intake in the diet is restricted; no nuts, chocolate, liver, movement.305 In practical terms, however, dyskinesia is
shellfish, dried fruit, or mushrooms. Zinc salt, which blocks usually a series of rhythmical extraneous movements. In
the absorption of copper in the stomach and has no side tardive dyskinesia this typically begins with, or may be
effects, is now the treatment of choice. Penicillamine and confined to, the region of the face. These extraneous move-
trientine increase urinary excretion of copper, but there are ments may include choreoathetoid or dystonic movements.
serious side effects to these drugs. If the copper imbalance Because of abnormality in basal ganglia function, abnor-
is treated, the neurological signs do not progress. malities in postural tone and postural adjustments are also
present. Instead of the typical flexed posture of Parkinson
Examination and Treatment Intervention disease, clients with tardive dyskinesia show extension of
Because Wilson disease is fully managed medically and the trunk with increased lordosis and neck flexion.319 This
can be diagnosed early, it may not be of concern to the description of the disease is rather broad, but the problems
therapist. If the client is referred for therapy, treatment of drug-induced movement disorders are varied. They may
techniques should be wholly based on symptoms. Exami- take the form of drug-induced Parkinson disease or dysto-
nation is similar to that of the person with Parkinson or nia. In tardive dyskinesia, akinesia and rigidity similar to
Huntington disease. It consists of describing the type of that seen in parkinsonism may exist simultaneously with
extraneous movement present, when it is present, and fac- the choreoathetoid-like movements. The key factor in tar-
tors that influence the degree of dystonia. Ease of move- dive dyskinesia is its slow onset after the ingestion of
ment should also be assessed and may be timed as for the neuroleptic medications.
patient with Parkinson disease. In addition, range of move-
ment and strength should be evaluated, especially if the Etiology
disease is progressing. Although many people take neuroleptic medication, only a
Treatment is then designed to alleviate the problems. small percentage acquires tardive dyskinesia. Many factors
Extraneous movements may be reduced by any technique may predispose an individual to movement disorders. One
that will reduce tone. Positioning is important. If bradykine- of these is age.320 This might be expected because of the
sia is the major sign, then treatment would be similar to that influence of aging processes on the concentration of DA.
used for people with Parkinson disease; if trunk stability is Gender may also be a factor. Women, and older women in
poor, the therapist proceeds as in Huntington disease. The particular, are more at risk for tardive dyskinesia, perhaps
client with Wilson disease has knowledge of what normal because of decreased estrogen.319 The absolute amount of
movement feels like and usually has good cognitive abilities neuroleptic ingested may also be a factor, but to date
at the time treatment is started. Because of the emotional definitive studies have not been completed. So far the
lability, which is one of the first symptoms in this disease, length of time the individual takes medication does not ap-
the treatment session should be well planned and quite pear to be a strong predisposing factor. As the biological
structured. abnormalities of schizophrenia become better understood,
further understanding of the causes of tardive dyskinesia
Tardive Dyskinesia also may be elucidated. The development of tardive dyski-
Tardive dyskinesia is usually a drug-induced disorder and nesia is hypothesized to be caused by supersensitivity.305,321
thus will be used to indicate the problems that can arise from With the use of drugs that deplete the brain of DA, the
drug intoxication. In particular this section concentrates on brain becomes more sensitive to it. And, in fact, in humans
the problems associated with drugs that affect DA metabo- the withdrawal of neuroleptics tends to heighten the dis-
lism and/or reuptake, including amphetamine, methamphet- ease; essentially, withdrawal of the DA antagonist means
amine, haloperidol, and classes of drugs used in treatment of that far more DA is able to act on these already sensitive
psychotic disorders: the phenothiazines, butyrophenones, terminals.305,321,322
and thioxanthenes. As the use and misuse of drugs becomes Because of the effectiveness of long-term treatment for
more common, these types of disorders may become more schizophrenia provided by neuroleptics, research into the
frequent. (Refer to Chapter 36 for additional information.) underlying cause and therefore treatment of the major side
The use of phenothiazines (one of the neuroleptics) has effect, the motor disorders, has greatly increased.323 But as
become a very effective and common treatment for schizo- with Parkinson disease and Huntington disease, animal
phrenia. This treatment protocol has enabled many schizo- models are difficult to produce. However, experimental evi-
phrenics to leave the mental institution. These drugs are DA dence indicates that the basal ganglia are involved in move-
antagonists and thus decrease the amount of DA in the brain. ments about the face, especially the mouth, and buccolin-
The exact site of the brain involved in schizophrenia itself is gual dyskinesia is the most frequently encountered symptom
not within the scope of this chapter, but the neurological in tardive dyskinesia.69,70 The response of basal ganglia
signs that occur will be discussed. As might be expected, neurons to sensory input shows increasing localization of
they involve structures within the basal ganglia. Tardive dys- response with age; the region about the mouth becomes
kinesia is a gradual disease that occurs after long-term drug increasingly sensitive.70 Further research along these lines,
treatment. The most typical involvement is of the mouth, both in normal animals and in those with lesions, may
tongue, and muscles of mastication; therefore tardive dyski- answer the question of what is happening at a neuronal
nesia may be called orofacial or buccolingual-masticatory level. This would facilitate pharmacological and therapeutic
(BLM) dyskinesia. interventions.
Pharmacological and Medical Management Ameliorating the oral grimacing, of course, would be
The most important treatment for tardive dyskinesia is pre- helpful for the schizophrenic person who is trying to return
vention. Today, DA receptor agonists are prescribed only to society. The effectiveness of physical and occupational
when other, newer medications are not effective. Tardive therapy treatment cannot be assessed until therapists
dyskinesia is often irreversible. The withdrawal of medica- become involved with these clients and record the effective-
tion, in fact, may increase the movement disorders. Or re- ness of their interventions. In cases in which the parkinsonian-
covery may take even more time than that required for the like symptoms are stronger than the dyskinetic movements,
onset of the disease. Strangely, sometimes the drug that treatment would follow the plan for the individual with
caused the disease may be the drug that reduces the symp- Parkinson disease. As yet, physical therapy for drug-induced
toms; that is, increasing the dose may lessen the movement dyskinesias is not mentioned on websites to the physician or
disorder. This might be expected if supersensitivity to DA is the patient.
involved. But again, with time the increased dose will
also cause a reappearance of the symptoms. The Movement Other Considerations
Disorder Society and WE MOVE recommend that the phy- Other drugs besides neuroleptics may also produce move-
sician evaluate the schizophrenic patient at 3-month inter- ment disorders. Amphetamine, for example, has been shown
vals to prevent the disease. (Refer to the list of websites at to cause long-term changes in brain function even with very
the end of this chapter.) small doses.324-326 Adults who were hyperactive as children
The use of other drugs in conjunction with the neurolep- sometimes show a decrease in the readiness potential.327
tics has been tried in various animal models of the disease. Further longitudinal research and research using PET scans
As might be expected, anticholinergic drugs (which would and functional magnetic resonance imaging (fMRI) are un-
worsen an imbalance between DA and ACh) worsen the derway to determine the role that medications used in treat-
dyskinesia. Lithium has been successful in one animal ing hyperactive children, such as methylphenidate (Ritalin),
model of dyskinesia.305 Some neuroleptic drugs seem to might play in changing the architecture of the basal ganglia
have less effect on movement than others; however, the side and causing movement disorders.84 The problem of drug-
effects of one such drug, chlorpromazine, are life-threatening. induced movement disorders may become an ever-increasing
Reducing the buildup of phenylalanine is also indicated as a one for the therapist.
way to decrease occurrence of tardive dyskinesia. A medical In 1982 several young people were treated for rigidity
food comprising branched-chain amino acids seems to re- and “catatonia” after the use of what they thought was her-
duce concentration of phenylalanine and was effective in oin. Careful examination of these patients revealed that they
reducing the movement disorder in one clinical trial. More had parkinsonian-like symptoms.38,105 The chemical respon-
research is needed into both the mechanisms of schizophre- sible for the symptomatology was MPTP, a meperidine ana-
nia and the mechanisms for the production of the abnormal log that was an impurity in the designer heroin. This discov-
movements. ery has enabled research in animals and clinical studies in
humans and may enable better understanding of the patho-
Evaluation and Treatment Interventions genesis and, in turn, of the treatment of the disease. One
for Dyskinesia hypothesized cause of Parkinson disease implicated envi-
The effectiveness of rehabilitation therapy intervention in ronmental toxins (because some herbicides such as paraquat
drug-induced dyskinesia is, as yet, not completely known. resemble the chemical structure of MPTP) and the involve-
However, because the neuroleptics do provide an effective ment of superoxide free radicals.92,328,329 More complete
long-term treatment of schizophrenia, and because amphet- epidemiological studies are now underway to investigate
amines and methamphetamine are being abused, therapists Parkinson disease to determine the relationship to specific
need to become aware of the problem and offer some assis- herbicides and pesticides.
tance. Early drug holidays (time without use of drugs) may Methamphetamine use also induces movement disorders.
be of value in treatment of tardive dyskinesia, and therefore The MRI of even infrequent users shows damage to the
early awareness of incipient changes in motor function may basal ganglia.330,331 A child born to a mother using metham-
be of value. Assessment of patients receiving drug therapy phetamine may also have movement disorders and delayed
could perhaps begin before treatment and then at prescribed achievement of developmental milestones. Another recre-
intervals. The knowledge that postural adjustments are ab- ational drug, cocaine has long been known to produce par-
normal in most basal ganglia diseases means that analysis of kinsonian movement disorders.332
posture statically and in motion might provide early clues of
development of movement disorders. The same would be
Dystonia
true for balance reactions and changes in tone with changes
in position. Once movement disorders appear, an assessment General Information
of when and where the extraneous movements occur is Dystonia is a movement disorder characterized by sustained
important. (See Chapter 8 for general examination tools and muscle contraction in the extreme end range of a movement,
Chapter 22 specifically for tests of balance.) frequently with a rotational component. There are inherited
General treatment is similar to that used in Huntington dystonias that usually involve the entire body. These dysto-
disease; oral treatment corresponds to that for the athe- nias are most prevalent in those of European Jewish descent.
totic child with cerebral palsy. If a hyperreactivity to sen- Focal dystonias involve just one joint or a few neighboring
sory stimulus exists, then oral desensitization may be of joints, such as spasmodic torticollis or writing cramps.
value. Full-body dystonia is a disease of the basal ganglia, and the
current view is that focal dystonia also involves lesions of unlike other focal dystonias. An interesting phenomenon of
precise areas of the basal ganglia. dystonia is the fact that many patients will develop a sensory
Generalized and focal dystonias manifest differently and or motor “trick” that will decrease the severity of the muscle
have different pathophysiologies and therefore different contraction(s) and may even stop these movements.333,336
treatments. They will therefore be separated in this part of Symptoms. Symptoms of focal dystonia will depend on
the chapter. However, in all cases of dystonia, excessive the site of involvement. For example, in the case of spas-
coactivation of agonists and antagonists occurs that inter- modic torticollis, the symptom is pain and an inability to
feres with the timing, execution, and loss of independent control a movement of the head to the side.
joint motions. Rarely are any abnormalities of muscle tone The signs and symptoms of focal hand dystonia are vari-
present, per se—that is, no increase in deep tendon reflexes able. The problem may initially manifest as an abnormality
or rigidity occurs. Muscle strength and ROM are usually in the quality of sound produced by a musical instrument
within normal limits unless disuse leads to weakness. (e.g., a deterioration of vibration in a violinist),337 increas-
ing errors in task performance, unusual fatigue or sense of
Generalized Dystonia weakness, or involuntary or excessive movement of a single
Symptoms. The person with generalized dystonia will digit or multiple digits. Initially the symptoms are subtle
begin a movement (such as walking) and then will experi- and virtually indistinguishable from the normal variations
ence a torsional contraction of the trunk; of the upper that may be seen in the execution experienced by all musi-
extremity, especially at the shoulder; and in the ankle, foot, cians studying technically demanding music or software
and toes. These contractions may be so strong that further engineers who spend excessive hours at the computer. Fre-
movement is impossible. Many patients experience pain as quently, a person engaged in a profession with high repeti-
the muscles remain contracted for long periods of time.333 tion of tasks who has minimal pain but vague motor control
Etiology. The cause of generalized dystonia is predomi- problems or somatosensory dysfunction is manifesting
nantly genetic, involving the DYT gene.334 early signs of focal dystonia.338 Although a co-contraction
Pharmacological and Medical Management. There of flexors and extensors can be observed while an individ-
are few treatments for generalized dystonia. DA agonists ual with hand dystonia performs the target task, at rest and
and l-dopa are sometimes effective. during the performance of nontarget tasks the hand appears
Evaluation and Treatment Intervention. Evaluation to function normally. Some patients demonstrate a variety
of the person with generalized dystonia will be similar to the of subtle abnormalities such as a reduced arm swing; loss
evaluation of the person with tardive dyskinesia or Huntington of smooth, controlled grasping; a physiological tremor;
disease. Several ADLs should be examined. And the way that hypermobility of the interphalangeal joints; decreased
dystonia interferes with these ADLs is of most importance for ROM in some upper limb joints (e.g., shoulder abduction,
treatment. In addition to the full extent of the motoric abnor- external rotation, finger abduction, forearm pronation);
mality, it is also important to test sensation, especially higher neurovascular entrapment; compression neuropathy; or poor
level sensory processing such as precise localization of touch, posture.339-344
graphesthesia, and kinesthesia. Etiology. The cause of focal dystonias is unknown and
Movement therapy interventions are only now being de- multifactorial. Frucht344 observed that task-specific hand
veloped for generalized dystonia. Overall, treatment similar dystonia seemed to begin after motor skills had been
to that in Huntington disease that emphasizes treating the acquired rather than during skill acquisition. Thus, focal
symptoms may be beneficial. One successful program uses hand dystonia in a musician is probably not a disorder of
sensory integration and relearning techniques performed motor learning but a disruption of acquired, complex, motor
with attention.335 Practice is a crucial element of treatment, programs. The data also suggested that peripheral environ-
and the client must be willing to practice the sensory tasks mental influences seem to play an important role in molding
many, many times throughout the day for benefit. the dystonic phenotype. For example, the hand performing
Other Considerations. As with other extraneous move- the more complex musical tasks (e.g., right hand in pianists
ments associated with basal ganglia disorders, relaxation and guitarists, left hand in violinists), seemed to be more
can reduce the muscle contraction. However, I have found predisposed to the development of dystonia. In addition, the
that the time to incorporate the relaxation is before the full- dystonia usually began in one finger and spread to adjacent
blown development of the muscle contraction—a difficult fingers, rarely skipping a finger. Furthermore, the ulnar side
task. Therefore clients should practice relaxation on a regu- of the hand (fingers 4 and 5) was disproportionately
lar basis. There is frequently a psychological aspect to the affected, potentially because of the challenging ergonomics
focal dystonias that may necessitate intervention from a and technical stresses of the musical instrument required for
psychiatrist or psychologist. this part of the hand in terms of gripping and activation of
individual finger movements.345
Focal Dystonias Pharmacological and Medical Management. The
Spasmodic torticollis is the most common focal dystonia. most common medical treatment for the focal dystonias is
The person with this disorder will have involuntary contrac- botulinum toxin. This toxin binds with the ACh receptors on
tions of neck muscles that result in head turning and head the muscle and prevents the muscle from contracting. The
extension and flexion movements that are often sustained for injections are made under electromyographic guidance so
long periods of time. Other common sites of focal involve- that only those motor units involved in the production of the
ment are the vocal cords; the tongue and swallowing mus- extraneous movements are paralyzed. However, the treat-
cles; the facial muscles, especially about the eye; the hand; ment does not cause permanent change, so the patient must
and the toes. Writer’s cramp is a task-specific dystonia, repeat these injections every 3 to 4 months. Some people
develop antibodies to the toxin, rendering it then ineffec- day for benefit. The client practices cognitively demanding
tive.346,347 Therefore, medical management prevents the sensory discrimination tasks throughout the day and tries to
abnormal movement but is not a cure. use only tension-free movements.349
Evaluation and Treatment. Overall, treatment of focal Treatment for the person with torticollis must include a
dystonia will depend on the joint or joint involved. The du- relearning of midline before the person can begin to practice
ration of the dystonia, the trigger, and the person’s trick, if normal movement away from midline. The client may find
any, to relieve the dystonia must be noted. Tricks are sensory this relearning process easier after botulinum injection. See
in nature and help relieve the pain often associated with the Chapter 9 for further treatment interventions appropriate for
extreme movement. The Toronto Western Spasmodic Torti- patients with focal dystonias.
collis Rating Scale (TWSTRS) is one evaluation for the Other Considerations. As with other extraneous move-
person with spasmodic torticollis.348 ments associated with basal ganglia disorders, relaxation
Several ADLs should be examined. For example, in hand can reduce the muscle contraction. However, the time to
dystonia, the person should be evaluated using the instru- incorporate relaxation is before the full-blown development
ment producing the dystonia (i.e., the pen in writer’s cramp) of the muscle contraction—a difficult task. This task re-
as well as other tools (e.g., a fork). In addition, there seems quires a shift in paradigm to a health and wellness model
to be position dependence, so writing while prone may not and prevention (see Chapter 2). Therefore clients should
evoke the dystonia despite severe inability to hold the pen at practice relaxation on a regular basis. A psychological as-
a desk.349 pect to the focal dystonias frequently necessitates interven-
In addition to the full extent of the motoric abnormality, tion from a psychiatrist or psychologist.
sensation, especially higher-level sensations such as precise
localization of touch, graphesthesia, and kinesthesia must be METABOLIC DISEASES AFFECTING
assessed. Byl and colleagues found changes in the sensory OTHER REGIONS OF THE BRAIN
cortex after development of focal hand dystonia.335,349-352 All alterations of metabolism, if allowed to continue, will
Recent evidence suggests that balance, particularly dynamic affect nervous system function. This includes alterations in
balance, should also be assessed in patients with torticol- sodium, water, sugar, and hormonal balance. Table 20-2 lists
lis.353 These balance difficulties have not been relieved with metabolic diseases that often have neurological sequelae.
botulinum toxin. Proper treatment is usually medical management of the im-
Movement therapy interventions are now being devel- balance. Physical therapeutic intervention, if necessary,
oped. One successful program uses sensory integration and should address specific neurological symptoms.
relearning techniques performed with attention.354 Practice Ingestion of or exposure to heavy metals may also lead to
is a crucial element of treatment and the client must be will- CNS disease. Table 20-3 describes the sequelae of these
ing to practice the sensory tasks many times throughout the problems.
TABLE 20-2 n NEUROLOGICAL COMPLICATIONS OF METABOLIC DISORDERS
METABOLIC PROBLEM TREATMENT NEUROLOGICAL COMPLICATION

Decreased sodium (too Restriction of water intake Muscle twitching, seizures, coma
much water)
Increased sodium Slow rehydration Cerebral edema, muscle rigidity, decerebrate rigidity
Decreased potassium Restoration of potassium levels after Changes in resting potential of neuron; hyperpolarization;
(hypokalemia), often assessing primary cause muscle weakness and fatigue with eventual total paralysis
caused by aldosteronism
Magnesium imbalance Improved diet, intravenous magnesium Mental confusion, muscle twitching, myoclonus, tachycardia,
hyperreflexia, extraneous movements, seizures
Diabetes mellitus Proper control of diabetes Peripheral neuropathy, pseudotabes, possible seizures
and coma
Hypoglycemia Treatment of primary cause; diet adjustment Anoxia of the brain, seizures, mental confusion
Hyperthyroidism Thyroid-blocking agents; intravenous fluids, Hyperkinesia, irritability, nervousness, emotional lability,
hydrocortisone, and propranolol if patient symmetrical peripheral neuropathy
is in thyroid crisis
Hypothyroidism Thyroid supplement Sluggishness, mental and motor retardation, muscle
weakness, sometimes muscle pain
Hypercalcemia Treatment of primary cause, which is often Headache, weakness, fatigue, proximal neuropathy, rigidity,
hyperparathyroidism, vitamin D malignancy tremor, disorientation
(therefore surgical removal)
Hypocalcemia Intravenous administration of calcium Hyperexcitability of the peripheral and central nervous
(possible medical emergency) systems, which can lead to tetany and convulsions
TABLE 20-3 n NEUROLOGICAL COMPLICATIONS OF HEAVY METAL POISONING
TYPE OF METAL TREATMENT NEUROLOGICAL COMPLICATION

LEAD
Source: lead paint, industrial Elimination of source, reduction Interstitial edema and hemorrhage (especially in cerebellum) in
(fumes of molten lead) of fluids, intravenous urea or acute poisoning; all levels of central nervous system affected in
mannitol, use of chelating agents chronic long-term poisoning
In children: seizures, mental retardation, behavior problems,
hyperactivity
In adults: spasticity, rigidity, dementia, personality changes
Peripheral neuropathy may occur in adults and children
ARSENIC
Source: paint and insecticides Removal of source, gastric lavage, Demyelinization of peripheral nerves in all extremities
intravenous fluids, maintenance of
electrolyte balance; penicillamine
used in acute poisoning
MANGANESE
Source: industrial if manga- Levodopa Neuronal loss in basal ganglia, substantia nigra, and cerebellum
nese dust is not removed; Initially psychiatric disturbances, including nervousness,
symptoms appear 2-25 yr irritability, and a tendency toward compulsive acts
after exposure Later, muscular weakness and parkinsonian symptoms
MERCURY
Rare, but may affect farmers Penicillamine; function returns only Loss of neurons, especially in cerebellum; also in cortex near
and dental office workers with physical, occupational, and calcarine fissure
speech therapy Alternating periods of confusion, drowsiness, and stupor with
restlessness and excitability
Ataxia, dysarthria, visual deterioration
SUMMARY patients and provide information on making day-to-day life

This chapter has focused on the pathophysiology, evalua- easier. Local and national support groups and foundations
tion, and treatment of genetic, hereditary, and metabolic also provide information and support for the patient and
diseases affecting adults. In all of these diseases the thera- caregiver. Most also have separate sections for health care
pist or movement specialist is an important (though some- providers.
times underused) part of the rehabilitation team. Knowl-
edge of the possible mechanisms involved in the production Websites
of the varying movement disorders may make the appro- National Institute of Neurological Disorders and Stroke,
priate evaluation and subsequent treatment more meaning- National Institutes of Health
ful. Even with degenerative, progressive disorders the A comprehensive index of neurological disorders.
therapist plays an important role in maintaining quality of www.ninds.nih.gov/disorders/disorder_index.htm
life and assists the client and family in coping with the WE MOVE—Worldwide education and awareness for move-
disease. The importance of documentation (see Chapter ment disorders
10) and publication of cases and larger controlled studies This organization is for professionals and patients and
cannot be overstressed. Both will assist in the develop- includes information regarding all movement disor-
ment of improved therapeutic techniques and may help ders included in this chapter. Refer to Web booklet, A
researchers in planning and interpreting appropriate ex- Caregiver’s Guide to Huntington’s Disease.
perimental studies. Establishment of efficacy will be the www.wemove.org
first step toward evidence-based practice and a critical The Parkinson’s Disease Foundation
link in the evolution of professionals who have been iden- This website includes an exercise program for people
tified as movement specialists. with Parkinson disease.
With the advent of the Internet, many websites have been www.pdf.org
created to focus on diseases and conditions mentioned in The National Parkinson Foundation
this chapter. In addition, the organization WE MOVE News, medical information, events calendar, and more
(Worldwide Education and Awareness for Movement Disor- for people with Parkinson disease, families, and
ders) has a website for both patients and care providers caregivers.
(www.wemove.org). These sites answer many questions for www.parkinson.org
American Physical Therapy Association Podcast on Parkinson References

Disease To enhance this text and add value for the reader, all
Schenkman M, Gill-Body K; Craik RL, moderator. Out- references are included on the companion Evolve site that
come measures for people with Parkinson disease. http:// accompanies this textbook. This online service will,
ptjournal.apta.org/content/suppl/2011/08/17/91.9.1339. when available, provide a link for the reader to a Medline
DC2/ptj_201109_discussion_outcomes.mp3 abstract for the article cited. There are 354 cited refer-
Huntington’s Disease Society of America ences and other general references for this chapter,
Information, research, and help for people with Huntington’s with the majority of those articles being evidence-based
disease, families, and caregivers. citations.
www.hdsa.org
CASE STUDY 20-1 n PATIENT WITH PARKINSON DISEASE, HOEHN AND YAHR STAGE 1
Ms. T. is a 55-year-old woman who was diagnosed with her tennis, an activity she enjoys with her husband twice a
Parkinson disease 1 year ago. The disease began in her left week. Her only medication is deprenyl.
arm and leg when she noticed increasing stiffness and difficulty This patient is in Hoehn and Yahr stage 1, with some begin-
moving. She complains of some instability in walking and ning of bilateral symptoms and progression to stage 2. She is
recently has developed a slight resting tremor in the left hand. young, is employed full-time, and has been involved in regular
On initial evaluation she had full active and passive ROM in all exercise for the past 10 years. Her complaints are of stiffness,
extremities, neck, and trunk. There is a mild resting tremor slowed movements, and foot dystonia. Because her symptoms are
present in the left hand. There is mild cogwheel rigidity in the mild at present and she has good balance in standing and walk-
left upper and lower extremities; there is some intermittent ing, this patient should be encouraged to continue exercising reg-
resistance to passive movement in the right upper extremity as ularly. She should try to maintain her tennis, as this requires com-
well. Strength is grossly within normal limits throughout. plex, sequential, context-dependent movements. Although tennis
Sensation is intact throughout. Equilibrium reactions are involves motor responses to external cues, it does necessitate
delayed, but the patient demonstrates an ankle strategy on a rapid force generation and anticipatory movements. This should
flat surface and a hip strategy when standing on the balance encourage continued motor learning. In addition, she should be
beam; there is no mixing of the synergies, and her balance encouraged to continue walking out of doors and practice alter-
responses are appropriate to the degree of displacement. nating speed of walking. The dystonia is more difficult to resolve.
The patient is able to stand in the sharpened Romberg It may be tied to medication, and differing medication schemes
position for 30 seconds with the eyes open and 20 seconds with are now being tried. She is also on a program of stretching and
eyes closed. She can stand on the right leg for 30 seconds with strengthening of the ankle as well as a sensory stimulation pro-
eyes open and 15 seconds with eyes closed; she can stand on gram for the feet. Foam between the toes has helped to decrease
the left leg for 15 seconds with eyes open and 10 seconds with dystonia early in the day.
eyes closed. When walking, she has a heel-toe sequence, short- Ms. T. has also been informed about the importance of
ened stride length, and normal stride width. There is no arm maintaining chest expansion and monitoring her breathing.
swing on the left and a diminished arm swing on the right. This will be important as the disease progresses. She attends a
There is no trunk rotation and very slight trunk flexion support group for young parkinsonian patients to increase her
throughout the gait cycle. Speed is within normal limits for a awareness of the disease, new treatments, and support. As the
25-foot walk. The patient is able to turn freely. She has re- disease progresses, she will need a home program appropriate
cently begun to experience a foot dystonia, which is worse with for her symptoms. The home program will be reassessed every
fatigue. It has interfered with her daily walking program and 3 to 6 months.
CASE STUDY 20-2 n PATIENT WITH A SEVEN-YEAR HISTORY OF PARKINSON

DISEASE, HOEHN AND YAHR STAGE 3
Mr. R. is a 68-year-old man with a 7-year history of Parkinson in the lower extremity have range within functional limits.
disease. He is currently in Hoehn and Yahr stage 3 of the dis- Strength is grossly 4 to 41/5 on manual muscle testing
ease progression. He falls two or three times a day, has diffi- throughout including grip strength. Sensation is within normal
culty eating, and has noticed weakness in his right hand. He limits throughout. Sitting balance is good during static and
would like to return to full activity including golf twice a dynamic activities.
week, swimming, and skiing. On evaluation he has moderate The patient sits with a posterior pelvic tilt, rounded shoul-
rigidity in all extremities; the right side is worse than the left. ders, and flexed neck. On rising to a standing position, he does
It is most marked in the right wrist, forearm, and hand. Shoul- move forward in the chair, which positions his feet under his
der flexion and abduction lack 15 degrees bilaterally. He has a knees. He does not lean forward as he stands and momentarily
15-degree knee flexion contracture on the right; all other joints loses his balance on rising from a chair. Static standing balance
Continued
CASE STUDY 20-2 n PATIENT WITH A SEVEN-YEAR HISTORY OF PARKINSON

DISEASE, HOEHN AND YAHR STAGE 3—cont’d
is fair, and dynamic balance is fair. When pushed on the ster- breathing with the strokes. He does not play golf because it
num, he takes one or two steps backward, even to a gentle takes him so long.
push. When pushed from behind he takes several steps forward. This patient was encouraged to continue both to exercise
He lost his balance and required assistance when trying to and to socialize while exercising. He was encouraged to re-
catch a large ball thrown to the side. His gait pattern is typical sume golf at times when his course is less crowded. In addi-
of a parkinsonian patient. There is a shortened step and a flat- tion, he was given a home program consisting of activities to
footed foot contact. He complains of festination and of freez- be performed in seated and standing positions that encourage
ing, but neither are observed during the evaluation. He turns trunk rotation and large movements and are coordinated with
“en bloc,” exhibiting no arm swing and no trunk rotation. He good breathing practices. He was given some balance exercises
walks slowly and is unable to increase his speed measurably in that challenge his equilibrium in a safe environment. His home
a 25-foot walk. He is taking l-dopa–carbidopa and deprenyl. program was monitored every 3 months because of the distance
He tried taking another D2 agonist but experienced hallucina- he must travel to come to the clinic. His wife was instructed to
tions. He was able to ski until last winter. At that time he found exercise with her husband and to exercise to music with him.
that he could not stand up once he fell down, and sometimes he He was referred to the speech pathologist for a swallowing
fell without realizing that he was falling. He stated that he “did evaluation and was given a therapeutic program for his speech
not think it was safe to ski.” He also no longer swims because and breathing. He is now able to play golf once a week;
he has difficulty breathing in the pool and coordinating his however he is not yet ready to resume swimming or skiing.
CHAPTER 21 Movement Dysfunction Associated
with Cerebellar Damage
SUSANNE M. MORTON, PT, PhD, and AMY J. BASTIAN, PT, PhD
OVERVIEW strokes.1 These strokes can involve any of the three arteries
The cerebellum is a highly unique brain structure, easily that supply the cerebellum: the superior cerebellar artery,
recognizable by its location on the dorsal surface of the anterior inferior cerebellar artery, and posterior inferior cer-
brain stem and the distinct, dense folia, or foldings, of its ebellar artery. Depending on the territory supplied by the
cortex. For centuries the cerebellum has been the object of damaged vessel (Table 21-1), there are stereotyped patterns
intense investigation by scientists, in particular because of of cerebellar and extracerebellar motor dysfunction that re-
the extreme uniformity in the arrangement of neurons in the sult. However, there is certainly some variation in distribu-
cerebellar cortex and the presence of very large Purkinje tion from person to person. Stroke involving the superior
cells, which have an extensive fanlike dendritic arbor. The cerebellar artery often leads to dysmetria of ipsilateral arm
human cerebellum contains more neurons than any other movements, unsteadiness in walking, dysarthric speech, and
brain region, suggesting that whatever its role in behavior, it nystagmus.1 Stroke involving the anterior inferior cerebellar
requires the integration of vast amounts of information and artery often causes both cerebellar and extracerebellar signs
may perform rather complex computations. Researchers (owing to involvement of the pons) including dysmetria,
agree that the cerebellum plays a critical role in coordinating vestibular signs, and facial sensory loss.1 Finally, stroke in-
and adapting movements, although how it does so is still not volving the posterior inferior cerebellar artery is usually, in
fully understood. It is now also clear that the cerebellum is the long run, the most benign, though initially it often
connected to nonmotor regions of the brain, such as the manifests with vertigo, unsteadiness, walking ataxia, and
prefrontal cortex, and therefore likely plays a role in cogni- nystagmus.1 The best predictor of recovery from cerebellar
tive and other nonmotor functions. Yet the most striking and stroke is whether the deep cerebellar nuclei are involved:
debilitating effect of damage to the cerebellum is ataxia, recovery is best when they are not damaged.2
which comes from the Greek and translates literally to Tumors in the posterior fossa (i.e., in or near the cerebel-
“without order.” We will focus on this hallmark feature of lum) do occur, though they are more common in children
cerebellar damage, which is incoordination of movements than adults. Depending on the type and location, tumors
without overt muscle weakness. may be treatable with surgical resection, chemotherapy, ra-
In this chapter we will review critical features of cerebel- diation therapy, or some combination of these. Children
lar anatomy and physiology that help reveal the role of the with cerebellar tumors often have a good prognosis for re-
cerebellum in motor control, and we will describe the major covery because many of the types of tumors most common
movement deficits associated with damage to the human in this population are benign and can be removed. Children
cerebellum. We will highlight the most valuable and unique also typically recover very well after cerebellar damage
components of the physical therapy examination for clients from tumor resection and show little signs of cerebellar
with suspected cerebellar dysfunction and review the evi- ataxia. Tumors in adulthood often are caused by a more ag-
dence for and against specific rehabilitation interventions gressive form of cancer and therefore may carry a poorer
targeting recovery of body functions, activities, and partici- prognosis. Second to tumor type, damage of the deep cere-
pation. Emphasis is placed on the importance of the physical bellar nuclei is an important factor that predicts recovery,
therapist’s judgment in determining whether a recovery or a even more so than age.2
compensation approach should be implemented. Several neurodegenerative diseases can damage the cerebel-
lum (Table 21-2). One of the more common types of degenera-
Types of Cerebellar Damage tive diseases is a group of hereditary, autosomal dominant dis-
Cerebellar ataxia can result from damage to the cerebellum eases referred to as the spinocerebellar ataxias (SCAs).
itself or the pathways to or from it. Damage can occur from Currently there are 30 known distinct SCAs, which are named
a number of different causes, such as stroke, tumor, degen- by numbers (e.g., SCA1, SCA2). Depending on the genetic
erative disease, trauma, or malformation. The cause of cer- abnormality, they can cause either purely cerebellar damage or
ebellar dysfunction is often an important consideration combined cerebellar and extracerebellar damage.3,4 Most of the
when determining a prognosis and developing a treatment SCAs have onset in midlife and are slowly progressive, which
plan. Other factors to consider include whether the cerebel- means that children of an affected parent will likely not know
lar lesion is static versus progressive, whether it involves if they are affected until adulthood. There are genetic tests for a
only the cerebellum or multiple neural structures, and subset of these diseases. Because onset of symptoms is delayed
whether it was present at birth or acquired. and there are no effective pharmacological treatments, genetic
Cerebellar strokes are rarer than cerebral strokes, but not counseling is a must before families decide whether or not to
entirely uncommon. They account for less than 5% of all have children undergo genetic testing. A related set of diseases
631
TABLE 21-1 n TERRITORIES OF THE CEREBELLAR ARTERIES
ARTERY CEREBELLAR TERRITORY SUPPLIED

SCA Superior or upper half (approximately) of the dorsal and upper third (approximately) of the ventral surface of the cerebellum
except for the extreme lateral wing of the hemisphere
Portions of the vermis and nodulus
Substantial upper portions of the intermediate and lateral hemispheres
Portions of the deep cerebellar nuclei
Superior cerebellar peduncle
AICA Middle 10%-30% of the ventral cerebellum, sometimes wrapping laterally to encompass a small portion of the most lateral
aspects of the dorsal cerebellum
Flocculus
Small portions of the lateral hemisphere
Middle and inferior cerebellar peduncles
PICA Inferior or lower half (approximately) of the dorsal cerebellum and the inferior fourth to third of the ventral cerebellum
Portions of the nodulus, vermis, intermediate and lateral hemispheres
Portions of the deep cerebellar nuclei
Data from Amarenco P: The spectrum of cerebellar infarctions. Neurology 41:973–979, 1991; and Tatu L, Moulin T, Bogousslavsky J, Duvernoy H: Arterial
territories of human brain: brainstem and cerebellum. Neurology 47:1125–1135, 1996.
AICA, Anterior inferior cerebellar artery; PICA, posterior inferior cerebellar artery; SCA, superior cerebellar artery.
Note: The cerebellar arteries also supply extracerebellar regions (i.e., portions of brain stem) not listed here.
TABLE 21-2 n SELECTED FORMS OF CEREBELLAR DAMAGE102
ACQUIRED DEGENERATIVE NONHEREDITARY

1. Stroke (infarct, hemorrhage) 1. Multiple system atrophy (MSA)
2. Tumor (primary brain tumor, metastatic disease) 2. Idiopathic late-onset cerebellar ataxia (ILOCA)
3. Structural (Chiari malformation, agenesis,
hypoplasia, etc.)
4. Toxicity (alcohol, heavy metals, drugs, HEREDITARY
solvents, etc.) 1. Autosomal dominant disorders (episodic ataxias, spinocerebellar
5. Immune-mediated (multiple sclerosis, gluten ataxias)
ataxia, etc.) 2. Autosomal recessive disorders (Friedreich ataxia, early onset
6. Trauma cerebellar ataxia, etc.)
7. Infection (cerebellitis, etc.) 3. X-linked disorders (mitochondrial disease, fragile X-associated
8. Endocrine (hypothyroidism) tremor, etc)
Data from Manto M, Marmolino D: Cerebellar ataxias. Curr Opin Neurol 22:419–429, 2009.
is the hereditary episodic ataxias,5 which are rare autosomal damage the cerebellum by increased pressure and mechani-
dominant diseases. As the name implies, clients with episodic cal deformation. Recovery from cerebellar malformation is
ataxia will have periods of ataxia lasting minutes to hours, not understood; often these children have substantial dam-
brought on by exercise, stress, or excitement. Some of the epi- age to the brain stem or other neural structures, which may
sodic ataxias respond well to medications.6 make therapy more challenging. A more comprehensive list
Cerebellar damage can occur from other sources as well. of the variety of types of cerebellar damage is provided in
In traumatic brain injury, damage of the cerebellum is Table 21-2.
almost always found in the presence of widespread brain Given the wide range of cerebellar disorders, it is useful
damage and is seen as a predictor of poorer outcome.7 The for the clinician to categorize the damage as progressive or
cerebellum is also particularly sensitive to toxins, including nonprogressive. Clients with progressive disorders, such as
certain heavy metals and alcohol. Chronic alcoholism causes the SCAs, are likely to experience worsening ataxia and
cerebellar atrophy preferentially involving the anterior supe- decreased mobility over time, and will need periodic therapy
rior vermis.8,9 The inflammatory disorder multiple sclerosis over the life span for optimal function. In contrast, the con-
also frequently produces lesions in the cerebellum. Finally, dition of patients with nonprogressive disorders would not
congenital brain abnormalities such as Chiari malformation be expected to worsen, and some may have the potential for
CHAPTER 21 n Movement Dysfunction Associated with Cerebellar Damage 633
substantial recovery. Note that when additional brain areas Looking at a sagittal slice through the cerebellum, dis-
are involved, rehabilitation may be more challenging, theo- tinct cellular regions can be visualized. The most superficial
retically because other, compensatory brain mechanisms region is the cerebellar cortex, which, unlike the cerebral
may be impaired. This type of information is vital for mak- cortex, contains only three layers. The arrangement of cells
ing an appropriate prognosis and developing a long-term within the cortex is strikingly uniform across all cerebellar
plan of care for clients with cerebellar dysfunction. lobes and plays a vital role in determining cerebellar func-
tion, which will be described later. Deep to the cerebellar
CEREBELLAR ANATOMY AND PHYSIOLOGY cortex is the white matter layer, which contains the axons of
A brief review of specific anatomical and physiological Purkinje cells projecting out from the cerebellar cortex and
features is critical to understanding the mechanisms by the axons of mossy and climbing fibers entering the cortex
which the cerebellum helps coordinate and adapt move- from other brain and spinal regions (see Figure 21-1). The
ment. Recall that most pathways between the cerebellum cerebellar nuclei are the output structures of the cerebellum,
and spinal cord are uncrossed or double crossed, whereas and they make up the deepest region. Groups of neuronal
pathways between the cerebellum and cerebrum are crossed. cell bodies receive information coming into the cerebellum
Hence a lesion to one side of the cerebellum produces from a variety of brain and spinal cord regions and also from
ataxia and related cerebellar deficits involving the same the cerebellar cortex, via Purkinje cell axons. The deep nuclei
side of the body as the lesion. Also note that the cerebellum are arranged in pairs, with one nucleus of each pair on each
has relatively few direct projections to the spinal cord. In- side of the cerebellum. Most medially are the fastigial
stead, it exerts a strong influence on movement through its nuclei, followed by the globose and emboliform nuclei and
projections to cerebral and brain stem motor structures, as most laterally the broad dentate nuclei (see Figure 21-2).
described later. The medial and lateral vestibular nuclei also receive input
directly from the cerebellar flocculonodular lobe and are
Anatomical Divisions therefore considered to play a role as an additional set of
The cerebellum is part of the hindbrain and is positioned on cerebellar output structures.
the dorsal surface of the brain stem at approximately the
level of the pons (Figure 21-1). It is connected to the brain Functional Divisions and Their Afferent
stem by the superior, middle, and inferior cerebellar pedun- and Efferent Projections
cles. The cerebellar peduncles contain all of the axons that Probably the most useful way of thinking about the anatomy
transmit information to and from the cerebellum. The cere- of the cerebellum is to divide it into distinct functional longi-
bellum can be anatomically divided into three lobes: the tudinal “zones.”10 Each cerebellar zone consists of a region
anterior, posterior, and flocculonodular lobes. The primary of cerebellar cortex and its own pair of deep cerebellar nu-
fissure divides the anterior and posterior lobes, and the pos- clei. Each zone also has projections to and from distinct areas
terolateral fissure divides the posterior and flocculonodular of the brain and spinal cord. Thus, despite the regular ar-
lobes (Figure 21-2). rangement of cells over the entire cerebellum, each func-
tional longitudinal zone is uniquely positioned to control
certain types of movement but not others.10-12 See Table 21-3
for a summary.
The medial zone consists of the midline structure, the
vermis, and the fastigial nuclei. This region of the cerebel-
lum predominantly receives afferent information from the
brain stem vestibular and reticular nuclei and the dorsal and
ventral spinocerebellar pathways,13-18 which convey impor-
tant information regarding the current sensorimotor state of
the trunk and limbs.19-21 In turn, its outputs, through the
fastigial nuclei, are largely to reticular and vestibular nuclei
that will form part of the medial descending system (reticu-
lospinal and vestibulospinal tracts), with some additional
projections to the cerebral cortex via the thalamus.22-24 The
medial cerebellar zone is involved in the control of posture
and muscle tone, upright stance, locomotion, and in gaze
and other eye movements.
The intermediate zone is made up of the intermediate
hemispheres and the globose and emboliform nuclei. This
region also receives inputs from the dorsal and ventral spi-
nocerebellar pathways and brain stem reticular nuclei, as
well as some projections from the cerebral cortex that arrive
via the cerebropontocerebellar pathway.11,13,14,25,26 Major
projections from this cerebellar zone are to the cerebral cor-
Figure 21-1 n The cerebellum, bisected through the midsagittal tex via the thalamus and to the red nucleus.23,24,27 The inter-
plane. (Redrawn from Kandel ER, Schwartz JH, Jessell TM: Prin- mediate zone is considered to be important in controlling
ciples of neural science, ed 4, New York, 2000, McGraw-Hill coordination of agonist-antagonist muscle pairs during a
Medical.) variety of activities including walking and voluntary limb
Figure 21-2 n The cerebellum, flattened, showing key structures. A, Different shading distinguishes the three lobes of the cerebellum. The
cerebellar vermis and hemispheres are also identified. B, Functional longitudinal cerebellar zones, distinguished by different shading, and
the locations of the deep cerebellar nuclei within each zone. (Redrawn from Kandel ER, Schwartz JH, Jessell TM: Principles of neural
science, ed 4, New York, 2000, McGraw-Hill Medical.)
movements. The medial and intermediate zones of the cer- Physiology of Cerebellar Neuronal Circuits
ebellum are collectively referred to as the spinocerebellum, Within a longitudinal zone, thousands of microzones may
because these are the only cerebellar regions that receive exist,11 each consisting of a highly organized group of con-
afferents from the spinal cord. nected cerebellar cortical neurons. A microcomplex is the
The largest region of the cerebellum is the lateral zone, name given to a neural circuit made up of a single micro-
which contains the two broad lateral hemispheres and their zone plus the other connected neurons with which it com-
output structures, the dentate nuclei. Afferents to the lateral municates directly. The following section provides a very
zone predominantly come from the cerebrum, from a wide brief overview of the circuits important for cerebellar func-
variety of cortical areas including motor, premotor, and pre- tion and reviews the flow of neuronal signals into and out of
frontal cortices, parietal somatosensory and sensory associa- cerebellar microzones (Figure 21-3).
tion areas, and primary visual and auditory cortices.25,26 Most afferent information enters the cerebellum through
Outputs from the dentate travel mostly back to large areas of one of two pathways: the mossy fiber pathway or the climb-
the cerebrum (through the thalamus), to many of the same ing fiber pathway. Both have important actions on cerebellar
areas from which afferents arrived in the cerebellum. Again, Purkinje cells. The mossy fiber pathway affects “beams” or
these include vast regions of sensorimotor cortices.27-33 rows of Purkinje cells oriented along the cerebellar folia.
Other efferent fibers project to the red nucleus in the brain Dense mossy fiber inputs arise from a wide variety of
stem. The lateral cerebellar zone plays a major role in con- regions, including the cerebral cortex, several subcortical
trol of complex, multijoint voluntary limb movements, par- areas, the brain stem, and the spinal cord. Mossy fibers enter
ticularly those involving visual guidance, and in the plan- the cerebellar cortex and synapse onto granule cells, whose
ning of complex movements and the assessment of movement axons ascend and branch into parallel fibers. Each parallel
errors. Because this region of the cerebellum interacts pre- fiber extends long distances longitudinally and synapses
dominantly with the cerebrum, it is also commonly called onto many Purkinje cells, all located along the same beam.38
the cerebrocerebellum. It is also sometimes referred to as the Each parallel fiber has a relatively weak effect on single
neocerebellum because it is considered to have arisen fairly Purkinje cells, but the mass effect of many thousands of
recently in the phylogenetic tree, being much more expan- parallel fiber contacts with Purkinje cells drives the Purkinje
sive in primates than in lower animals.34 cells to fire at high rates.39 In contrast, each climbing fiber
The flocculonodular lobe can be considered a fourth zone arises exclusively from the inferior olive, located in the
of the cerebellum. It receives afferent projections directly brain stem, and contacts only a few (approximately 1 to 10)
from the vestibular primary afferents (semicircular canals Purkinje cells.12,40,41 Each Purkinje cell receives information
and otoliths) as well as from vestibular nuclei and visual from only one climbing fiber, yet the climbing fiber’s effect
brain regions.11,13,14,16,18 Outputs from the flocculonodular on the Purkinje cell is powerful, causing large complex
lobe project directly to the medial and lateral vestibular nu- spikes.
clei of the brain stem, without a synapse in a deep cerebellar The Purkinje cell provides the output for the cerebellar
nucleus.12,22,35 For this reason, these vestibular nuclei are cortex; each Purkinje cell axon projects to one of the deep
sometimes considered an additional set of deep cerebellar cerebellar nuclei. The mossy fiber and climbing fiber path-
nuclei. This cerebellar zone helps control eye movements ways affect Purkinje cells differently and are thought to
and balance. The well known vestibuloocular reflex (VOR), transmit different types of information. Mossy fibers are
which provides gaze stabilization during head turning or active at very high rates (generating action potentials at
walking, relies on the cerebellum for proper functioning.36,37 approximately 100 Hz) and are highly modulated by vari-
Because of its critical ties to the vestibular system, the floc- ous sensory stimuli and motor activity. They have been
culonodular lobe is also known as the vestibulocerebellum speculated to relay information related to the direction,
(see Figure 21-2). velocity, duration, or magnitude of movements or sensory
TABLE 21-3 n FUNCTIONAL LONGITUDINAL CEREBELLAR ZONES
FUNCTIONAL ROLE IN CLINICAL SIGNS

ZONE ALTERNATE NAME MAJOR AFFERENTS MAJOR EFFERENTS MOVEMENT IF DAMAGED
Flocculonodular Vestibulocere- n Vestib primary n Med & n VOR n Nystagmus
lobe bellum afferents lat vestib nuclei
Flocculo- n Vestib nuclei n Gaze and eye n Impaired VOR
}
nodular lobe; movements
med & lat n Visual areas n Posture and n Imbalance
vestib nuclei balance
Medial n Vestib & retic n Vestib & retic n Gaze and eye n Oculomotor
zone nuclei nuclei movements deficits
Vermis; fastigial n DSCT & VSCT n Cerebrum n Postural tone n Hypotonia
nuclei
n Balance n Imbalance
n Locomotion n Falls
Spinocerebellum n Gait ataxia
Intermediate n DSCT & VSCT n Cerebrum n Limb movements n Imbalance
zone
Intermediate n Retic nuclei n Red nucleus n Coordinate n Gait ataxia
hemispheres; agonist-
globose & antagonist muscle
emboliform pairs
nuclei
n Cerebrum n Tremor
n Lack of check
n Dysdiadocho-
kinesia
n Dysmetria
Lateral zone Cerebro- n Cerebrum (wide n Cerebrum (same n Complex, n Dysdiadocho-
Lateral cerebellum range of areas: areas as afferent multijoint kinesia
hemispheres; or motor, premotor, projections) voluntary limb
dentate nuclei Neocerebellum prefrontal, movements
somatosensory, n Red nucleus n Visually guided n Dysmetria
sensory movements
association, visual,
auditory cortices)
n Motor planning n Dyssynergia
n Sensorimotor n Decomposition
error assessment
Med, medial; Lat, lateral; Vestib, vestibular; Retic, reticular; DSCT, dorsal spinocerebellar tract; VSCT, ventral spinocerebellar tract; VOR, vestibuloocular
reflex.
stimuli.42-45 Climbing fibers, however, are active at very CEREBELLAR FUNCTION IN ADAPTING
low rates (approximately 1 to 4 Hz) and do not appear AND CONTROLLING MOVEMENT
to be as strongly modulated by sensory stimuli or motor The cytoarchitecture of the microzones is extremely stereo-
activity.40,46,47 There is still some disagreement regarding typed throughout the cerebellum, suggesting that it performs
what sort of information is encoded in the climbing fiber the same overall function regardless of whether it is acting on
signals, but the frequency of discharge appears to be too circuits controlling standing balance, eye movements, reach-
low to transmit information pertaining to specific param- ing and grasping, and so on. So what is the function of this
eters of sensory or motor events. The role of the climbing cerebellar circuit? What aspect of motor control does it
fiber is clearly important, however, because its firing uniquely provide? Despite centuries of study, these questions
produces large complex spikes in the Purkinje cells and have still not been answered completely. Although numerous
can also powerfully affect subsequent Purkinje cell theories of cerebellar function exist, here we limit the discus-
firing.41,48 sion to just a few that we view as particularly relevant.
Figure 21-3 n Schematic of the major cell types and their connections within the cerebellum. Excitatory synapses are indicated with a
triangle; inhibitory synapses with a bar. DCN, Deep cerebellar nucleus; IO, inferior olive. (Redrawn from Ito M: Cerebellar circuitry as a
neuronal machine. Prog Neurobiol 78:272–303, 2006.)
Theories of Cerebellar Function corrections would be issued too late. Instead, the brain
One general theory states that a primary function of the generates motor commands based on an internal prediction
cerebellum is in coordinating multiple limb segments to of how the command would move the body. This “feed-
generate smooth and fluid multijoint movements.49-52 This forward” control requires stored knowledge of the body’s
“motor coordinator” theory has support from behavioral dynamics, the environment, and the object to be manipu-
studies demonstrating that multijoint movements appear to lated, and it is learned from previous exposure. The neural
be particularly impaired in clients with cerebellar lesions.50 representation of this knowledge is referred to as an internal
Multijoint movements are inherently more complex than model,59-62 as it provides the ability to reproduce the effects
single joint movements because they require control of me- of motor actions in the brain. The internal model theory for
chanical interaction torques; those occurring at one segment cerebellar function states that the cerebellum serves as the
but caused by movement of other linked segments.53 This site of an internal model for movement. Accordingly, the
model suggests that the cerebellum predicts the mechanical incoordination of movement associated with cerebellar
interactions between segments based on a stored internal damage is a consequence of an inaccurate internal model,
knowledge of limb dynamics, and helps generate the correct which disrupts nearly all aspects of feed-forward motor
motor commands for appropriate multijoint movements. control.63 This idea is appealing, as it could help explain the
A second popular theory is the timer hypothesis. This wide variety of motor behaviors (e.g., reaching, standing
idea proposes that the cerebellum is the main site for the balance, eye movements) and movement parameters (e.g.,
temporal representation of movements.54,55 Supporters of force, direction) that can be impaired after cerebellar dam-
this theory suggest that cerebellar output ultimately encodes age. Likewise, human behavioral studies have recently
the precise temporal sequence of muscle activation with pointed out that cerebellar damage is frequently associated
such precision that a cerebellar lesion produces obvious with impaired feed-forward control but relatively intact
deficits in the spatial domain (e.g., movement direction and feedback mechanisms.64,65
magnitude) as well as the temporal domain.56 Other studies A related theory originates from the seminal works of
have shown that individuals with cerebellar damage also Marr,66 Albus,67 and Ito,68 in which the cerebellum was theo-
have impairments in perceiving time intervals, suggesting rized to be a sort of “learning machine.” This theory was
that this could be a more general cerebellar function.57,58 based on careful examination of the anatomy and physiol-
A third idea is that the cerebellum acts as an internal ogy within cerebellar microcircuits, and today it continues
model to allow predictive control of movement. Sensory to provide the basis for many of the current theories of cere-
feedback is inadequate for movements that need to be both bellar function (i.e., those described earlier). Central to the
fast and accurate: it is too slow, and as a result, motor idea of cerebellar involvement in learning was the discovery
that Purkinje cell output can be radically altered by climbing

fiber induction of long-term depression (LTD) of the parallel
fiber–Purkinje cell synapse.48 Hence, climbing fiber inputs
onto Purkinje cells can be viewed as providing a unique type
of teaching or error signal to the cerebellum. More recently,
LTD, long-term potentiation (LTP), and nonsynaptic plastic-
ity have all been shown to exist at numerous sites within the
cerebellum, both in the cortex and in the deep cerebellar
nuclei.69-72 Thus there are multiple avenues for activity-
dependent plasticity to occur within the cerebellum over
relatively short time scales. It is presumed that the plastic
changes in cerebellar output are responsible for changing
motor behavior during the process of learning new skills.
To summarize, although the precise mechanisms are still
under debate, a majority of researchers can agree on a few
central themes of cerebellar function. First, the cerebellum
is an integral structure for the coordination of movements.
Second, precisely timed interactions between neurons pro-
duce activity-dependent plasticity at a number of different
sites within the cerebellum. Presumably this plasticity plays Figure 21-4 n Trajectories of the wrist (solid lines) and final end
a fundamental role in motor learning. Convergence of these point positions of the tip of the index finger (filled circles) during
two themes would seem to indicate that a major cerebellar (A) slow and accurate and (B) fast and accurate reaches from a
function is to maintain optimal motor control through con- typical nondisabled individual (left) and a subject with cerebellar
stant adaptive learning processes, so that movements are damage (right). The subject with cerebellar damage shows dysmet-
appropriately adjusted for varying environmental demands. ria and an abnormally curved wrist path. Note also the tendency for
hypometria during slow movements and hypermetria during fast
CLINICAL MANIFESTATIONS OF CEREBELLAR movements (the same control and cerebellar subjects are shown in
LESIONS A and B). Several reaches are overlaid for each subject. Arrow in-
Ataxia is the primary sign of damage to the cerebellum or its dicates the reach direction. Large open circles indicate the target
input structures. Ataxia refers generally to uncoordinated or locations. (From Bastian AJ, Martin TA, Thach WT: Cerebellar
disordered movement, which, though most often associated ataxia: abnormal control of interaction torques across multiple
with gait (gait ataxia), can also be used to describe uncoordi- joints. J Neurophysiol 76:492–509, 1996.)
nated arm or leg movements (limb ataxia). Ataxia is exacer-
bated by moving multiple joints together and by moving
quickly. Because ataxia is a nonspecific term, it is important in
both clinical and research settings to use more precise termi- In particular, clients with cerebellar lesions have been dem-
nology to describe the specific aspects of motor performance onstrated to have a specific deficit in the ability to account
that are impaired. Around the beginning of the twentieth for interaction torques,49,50 the rotational forces that act on a
century, Joseph Babinski and Gordon Holmes were two of the limb segment when another linked limb segment is in mo-
earliest investigators to describe many of these specific fea- tion.68 When the cerebellum is intact, the central nervous
tures we now discuss here.73-75 system is able to predict the effects of interaction torques
and appropriately counter or exploit them so as to produce a
Dysmetria smooth, straight, and accurate reach in a feed-forward man-
Dysmetria specifically refers to an impaired ability to prop- ner. When the cerebellum is damaged, an incorrect or absent
erly scale movement distance. Movements are described as accounting for interaction torques leads to an incorrect feed-
either hypermetric or hypometric, referring to overshooting forward motor plan and subsequently an uncoordinated,
or undershooting of targets, respectively. Many clients with overly curved and hypermetric or hypometric reach that re-
cerebellar lesions will show both forms of dysmetria even quires feedback corrections to reach the target location.
during successive movements (Figure 21-4).49,50 Dysmetria
can be seen in both proximal and distal joints and occurs dur- Dyssynergia
ing both single-joint and multijoint movements, though mul- Originally, Babinski80 coined the term asynergia to mean a
tijoint movements worsen dysmetria (Figure 21-5).49,76-78 deficit in the coordination of movements of one body region
Slow movements tend to produce hypometria, whereas fast or in one limb segment with movements of another. Today,
movements almost always bring about hypermetria.49 For dyssynergia is used to describe impairment of multijoint
this reason, it has been speculated that hypometria represents movements, wherein movements of specific segments are
more of a voluntary compensation for hypermetria than a not properly sequenced or of the proper range or direction,
primary impairment from cerebellar damage. Sometimes resulting in uncoordinated multijoint movement. As indi-
large end point errors can be reduced to some degree with cated earlier, it is nearly universally true that clients with
visual feedback, but even the corrective movements them- significant cerebellar damage show greater impairments
selves are still abnormal.79 during multijoint movements than single-joint movements.
One proposed mechanism for dysmetria is an impaired However, it is not fully understood whether the reason for
ability to predict and account for the dynamics of the limbs. that is because the deficits of single-joint movements are
movement.75 An example of this is the well known finding

that clients with cerebellar damage, when asked to reach to
a target in front of and above the resting arm, will often flex
the shoulder first and then, while holding the shoulder fixed,
extend the elbow.49 This approach is generally slower and
will produce a more curved trajectory of the finger to the
target compared with nondisabled individuals, who would
typically perform the shoulder flexion and elbow extension
at the same time to produce a nearly straight-line finger
trajectory. Most likely, decomposition reflects more of
a compensatory strategy for dealing with impaired multi-
joint movements than it does a primary sign of cerebellar
damage.49,83
Lack of Check
Lack of check, sometimes also referred to as excessive
“rebound,” refers to the inability to rapidly and sufficiently
halt movement of a body part after a strong isometric force,
previously resisting movement of the body part, is suddenly
released. Individuals without cerebellar damage can very
quickly halt, or “check,” this unintended movement. Indi-
viduals with cerebellar damage, on the other hand, are
known to have considerable movement in the direction
Figure 21-5 n Final end point positions of the tip of the index opposite the previous resistance, to the point that the
finger (numbers; each value corresponds to a different subject) dur- unchecked movement risks loss of upright balance or self-
ing (A) single-joint (elbow only) and (B) multijoint (combined injury. This phenomenon is presumed to be caused by
shoulder and elbow) reaches from nondisabled (left) and cerebellar delayed cessation of agonist and/or delayed activation of
(right) subjects. Cerebellar dysmetria (both hypometria and hyper- antagonist muscles.
metria) is greatly exacerbated during the multijoint reaching condi-
tion. Multiple reaches are shown for all subjects. Arrow indicates Cerebellar Tremor
the reach direction. Large open circles indicate the target Despite being a very common neurological sign, tremor is
locations. (From Bastian AJ, Zackowski KM, Thach WT: Cerebel- poorly defined and not well understood. There are several
lar ataxia: torque deficiency or torque mismatch between joints? different forms of tremor, many with different causes, only
J Neurophysiol 83:3019–3030, 2000.) some of which are related to cerebellar dysfunction, so it is
important to distinguish among them. Tremor associated
with damage to the cerebellum is typically called action
compounded during a multijoint movement or because the tremor, reflecting the fact that it is absent at rest and elicited
cerebellum plays a special and unique role in multijoint during muscle activation and distinguishing it from the rest-
control. Dyssynergia appears to be related to dysmetria and ing tremor associated with Parkinson disease. Action tremor
therefore is probably also related to a deficit in predicting can be classified as postural or kinetic tremor.84 Postural
limb dynamics.49 tremor occurs in muscles maintaining a static position
against gravity (e.g., holding arms out in front of the body
Dysdiadochokinesia or standing in place), whereas kinetic tremor occurs in
Dysdiadochokinesia specifically refers to a deficit in the muscles producing an active voluntary movement. There-
coordination between agonist-antagonist muscle pairs elic- fore the movement oscillations are most visible in the same
ited during voluntary rapid alternating movements.80 Such plane as the voluntary movement. Kinetic tremor typically
coordination is typically tested during performance of sim- occurs at relatively low frequencies (approximately 2 to
ple, fast alternating movements such as forearm supination- 5 Hz) and can be observed during simple nontarget-directed
pronation or hand or foot tapping. Characteristic deficits are movements such as forearm pronation and supination or foot
excessive slowness along with inconsistency in the rate and tapping, or during targeted movements such as pointing dur-
range of the alternating movements, which worsen as the ing the finger-to-nose test. Intention tremor is a specific
movement continues.74 Dysdiadochokinesia appears to be form of kinetic tremor that occurs during the terminal por-
caused by poor regulation of the timing of cessation of ago- tions of visually guided movements toward a target. It may
nist muscle activity and the initiation of antagonist muscle actually represent the multiple corrective movements, driven
activity,81,82 which could be related to a deficit in predicting by visual feedback, to reach the target. As such, intention
limb dynamics. Indeed, rapid reversals in movement are tremor can be tested by repeating the test movement with
dynamically difficult to control. eyes closed; if the tremor decreases substantially or disap-
pears, it is an intention tremor.79
Decomposition Classic cerebellar tremor is kinetic tremor with intention
Movement decomposition refers to the breaking down of a tremor at movement termination. In general, cerebellar
movement sequence or a multijoint movement into a series tremor is thought to be caused by an insufficient ability to
of separate movements, each simpler than the combined anticipate the effects of movement and excessive reliance on
sensory feedback loops.85 Cerebellar tremor is highly influ- step initiation; that is, dynamic instability.96,97 Specifically,
enced by sensory conditions and has a strong mechanical clients tend to produce larger-than-normal surface-reactive
component; it is significantly reduced during isometric con- torque responses and exaggerated and prolonged muscle ac-
ditions or when vision is removed. It also can be decreased tivity, thereby overshooting the initial posture during the re-
in some clients by adding an inertial load to the limb,86 turn phase of the recovery from a perturbation (Figure 21-6).
though that strategy may also increase dysmetria.87 There
may also be a significant central component to cerebellar Gait Ataxia
tremor, possibly related to influences from the thalamus or Probably the greatest complaint and the most obvious sign
the inferior olive.81,88 of cerebellar damage is gait ataxia. This abnormal pattern of
walking is often described as a “drunken” gait because cli-
Hypotonia ents often stagger and lose balance as if intoxicated. Early
Hypotonia in clients with cerebellar damage was first de- work of Holmes showed that patients with cerebellar lesions
scribed by Holmes.75 It appears to arise from decreased ex- have severe difficulty maintaining balance during walking,
citatory drive to vestibulospinal and reticulospinal path- which often leads to falls, typically directed backward and
ways, two major output pathways from the cerebellar vermis toward the side of the lesion. Holmes reported specifically
and flocculonodular lobe. The hypotonia usually manifests that walking is slowed, with steps that are short, irregular in
as a decrease in the extensor tone necessary for holding the timing, and unequal in length. The legs sometimes lift
body upright against gravity. In cats, lesions of either the overly high during the swing phase by excessive flexion at
vestibular or fastigial nuclei cause this sort of postural hypo- the hip and knee and then lower abruptly and with uncon-
tonia.51,89-91 More recent observations in humans indicate trolled force. The trajectory of walking often veers errati-
that hypotonia is typically most problematic in cases of se- cally and patients have difficulty with stops or turns, espe-
vere cerebellar hypoplasias affecting the vermis, such as cially if performed quickly.75
Joubert syndrome,92 or in adults during the acute stage of
cerebellar injury only. In cases of adult-onset acute injury,
hypotonia usually resolves naturally over time and clients
recover normal passive muscle tone and normal reflexes
quickly. Thus hypotonia typically presents minimal to no
problems for physical function.81
Imbalance
Another cardinal sign of cerebellar damage is postural insta-
bility in both static and dynamic conditions. Specifically, cli-
ents with cerebellar damage usually show increased postural
sway; either excessive or diminished postural responses to
perturbations; poor control of equilibrium during voluntary
movements of the head, arms, or legs; and sometimes abnor-
mal oscillations of the trunk, called titubation.
Classically, cerebellar imbalance during stance was con-
sidered to be of a similar magnitude whether or not the eyes
are open; that is, little improvement noted with visual feed-
back73,75 and a negative Romberg test result. However, more
recently, investigators using posturography measures have
been able to distinguish several different categories of cer-
ebellar imbalance during quiet standing, some of which
do show improvement with visual stabilization.93,94 For
instance, clients with cerebellar damage relatively isolated Figure 21-6 n Postural responses from nondisabled control and
to the anterior lobe typically show increased postural cerebellar groups (average of 10 trials from 10 subjects in each
sway, which is of a high velocity and low amplitude and group) after backward platform translations of 15 cm/s for 6 cm.
occurs mainly in the anterior-posterior dimension. These Traces show (top to bottom) electromyographic recordings from
individuals also tend to have associated postural tremor and various postural muscle groups, postural sway, shear force, surface
increased intersegmental movements of the head, trunk, torque, and platform displacement. Filled areas indicate the first
and legs and tend to improve when allowed visual informa- 400 ms of activation in the electromyographic traces and the active
tion. On the other hand, localized damage to the vestibulo- surface-reactive forces in the shear force and torque traces. Postural
cerebellum more often leads to increased postural sway that responses of the cerebellar subjects are increased, with excessive and
consists of low-frequency and high-amplitude movements prolonged muscle activity (note especially the abnormal activation of
without a preferred direction and without increased interseg- flexor muscle groups), larger sway, and greater torque production.
mental movements. These individuals typically show no ABD, Rectus abdominis; fwd, forward; GAS, gastrocnemius; HAM,
improvement with visual information. Clients with damage biceps femoris; PAR, paraspinals; pf, plantarflexion; QUA, rectus
limited to the lateral cerebellum tend to have only slight or femoris; TIB, tibialis anterior. (Adapted with permission from
even no postural instability at all.93-95 the American Physiological Society. From Horak FB, Diener HC:
Human cerebellar damage is also associated with hyper- Cerebellar control of postural scaling and central set in stance.
metric postural responses to surface displacements or during J Neurophysiol 72:479–493, 1994.)
Those initial reports have now been confirmed numerous Oculomotor Deficits
times; clients with cerebellar damage walk without the con- Eye movements are often dramatically impaired after cer-
sistency in timing, length, and direction of steps typical of ebellar damage. Saccades are often slowed and dysmetric
healthy adults.77,98 In some cases gait appears wide based. (can be hypermetric or hypometric).103 Smooth pursuit may
There is also increased variability in both the timing and be “choppy,” referred to as saccadic pursuit, wherein the
movement excursion at the hip, knee, and ankle joints and smooth tracking of a target is degraded into a series of
irregularities in the resulting path of the foot during swing. shorter saccadic movements following behind the target.104
Coordination between joints of one leg and between legs The ability to cancel, or suppress, the VOR may be im-
(intralimb and interlimb coordination, respectively) is also paired or absent.105 Finally, abnormal nystagmus may also
abnormal.77,98,99 As an example, the timing of peak flexion be present. The nystagmus may occur during central gaze,
at one joint with respect to other joints’ positions may or there may also be alternating nystagmus or rebound nys-
be altered or inconsistent. Often decomposition is also tagmus. The most common form of nystagmus in cerebellar
observed between hip and knee, knee and ankle, and/or hip dysfunction is gaze-evoked nystagmus, indicating nystag-
and ankle joints.99,100 mus elicited toward the end ranges of lateral and/or vertical
A critical component of locomotor control is the require- gaze.106,107
ment for stability and dynamic balance while maintaining Clients with significant oculomotor abnormalities may be
forward propulsion. Thus imbalance, described previously, is referred to vestibular specialists, but these deficits should
also a major contributor to many features of gait ataxia. In never be ignored. Impaired eye movements may have a sig-
fact, it has been shown that clients with cerebellar damage nificant negative impact on physical function. For example,
and significant balance deficits also typically demonstrate impaired saccades can prevent a client from reading, and
nearly all the classic features of gait ataxia (i.e., reduced saccadic pursuit can exacerbate already poor visually guided
stride lengths, increased stride widths, reduced joint excur- limb movements.108 Perhaps most devastating, deficits re-
sions, abnormal swing foot trajectories, increased variability lated to impaired oculomotor control and vestibular reflexes
in foot placement, and joint-joint decomposition). In con- often worsen dynamic balance and walking abilities.
trast, clients with cerebellar damage and significant leg coor-
dination deficits but minimal or no balance deficits typically Speech Impairments
have very few walking abnormalities (Figure 21-7).101,102 Speech production may also be impaired when the cerebellum
Therefore during typical conditions of level walking, balance is damaged. Classically, the speech deficit associated with
deficits contribute much more strongly to cerebellar gait cerebellar damage is referred to as scanning speech, though
ataxia than do leg coordination deficits. it may be more generally referred to as ataxic dysarthria.
Figure 21-7 n Angular excursions at the ankle (top row), knee (middle), and hip (bottom) during fast walking from a typical nondisabled
individual (left column), a subject with cerebellar dysfunction who has significant leg incoordination but minimal imbalance (middle), and a
subject with cerebellar dysfunction who has significant imbalance but minimal leg incoordination (right). Several strides (from initial contact
to next initial contact) are overlaid for each subject. The client with cerebellar imbalance (shaded) shows significant evidence of gait ataxia,
including reduced joint excursions, excessive stride-to-stride variability, and abnormal timing between joints, whereas the client with
cerebellar leg incoordination and no imbalance shows no evidence of gait ataxia. DF, Dorsiflexion; F, flexion; PF, plantarflexion. (Adapted
with permission from the American Physiological Society. From Morton SM, Bastian AJ: Relative contributions of balance and voluntary
leg-coordination deficits to cerebellar gait ataxia. J Neurophysiol 89:1844–1856, 2003.)
Similar to limb control deficits, the primary impairment commands, demonstrated by large negative aftereffects (step
of speech may be related to the planning and prediction of length asymmetry in the reverse direction compared with
movements rather than the execution of speech components early adaptation) when the treadmill belts are initially re-
directly.109 Also like limb movements, most speech impair- turned to a regular (nonsplit) pattern. In contrast, individuals
ments appear to be attributable to alterations in timing and with cerebellar damage typically show a slower rate of adap-
coordination.143a The most consistent characteristics of ataxic tation, a reduced magnitude of adaptation, or no adaptation
dysarthria are impaired articulation (the correct pronounce- at all, and small or no aftereffects (see Figure 21-8). All of
ment of speech sounds) and impaired prosody (the pattern these findings indicate a significant deficiency in the capabil-
of stress and intonation of certain syllables or words). Other ity for motor adaptation in individuals with cerebellar dam-
common findings include slowed speech and either a lack age. As indicated earlier, adaptation deficits have been dem-
of or excessive loudness variability.109 Traditionally, speech onstrated in this patient population with numerous behavioral
impairments are treated primarily by speech and language tasks.*
pathologists. Cerebellum-dependent adaptation is not the only form of
motor learning, but it is an important one for rehabilitation
Impaired Motor Learning for several reasons. First, adaptation is a highly automatic
A critical problem associated with cerebellar damage is im- process to rapidly adjust movements for new, predictable
paired motor learning. In humans the cerebellum has been demands (e.g., adjusting the walking pattern for snow or
linked to learning of a wide variety of motor behaviors, in- sand; adjusting eye movements for glasses). Individuals
cluding recovering balance after a perturbation,96,97 learning with impaired cerebellar adaptive learning must use other
new walking patterns,64,100,111 adjusting voluntary limb means to handle new task demands, such as conscious con-
movements,65,112,113 and making eye movements.114,115 The trol strategies. This is obviously inefficient and difficult, as
type of learning that appears most reliant on the cerebellum it means that the individuals must think much more about
is associative and procedural. Specifically, the cerebellum their movements and cannot tolerate distractions. Adapta-
appears to be essential for learning to adjust a motor behav- tion is also important because when it is repeated many
ior through repeated practice of, or exposure to, the behavior times, it can result in more permanent storage of a move-
and using error information from one trial to improve per- ment pattern that can be called on immediately (i.e., no
formance on subsequent trials. It is important to note that error-based period of adaptation required). A clear example
cerebellum-dependent motor learning is driven by errors of this is the use of new bifocal glasses. Initially, there is an
directly occurring during the movement rather than by other adaptation process to adjust eye movements when switching
types of feedback, such as knowledge of results after the between the top and bottom lenses because eye movements
fact (e.g., hit or miss). Studies have suggested that the type have to be bigger for magnified objects. Yet with repeated
of error that drives cerebellum-dependent learning is not adaptation, the brain eventually stores two calibrations, one
the target error (i.e., “How far am I from the desired for making eye movements when viewing through the top
target?”), but instead what has been referred to as a sensory lens and one for making eye movements when viewing
prediction error (i.e., “How far am I from where I predicted through the bottom lens, that can be switched between in-
I would be?”).113,116 stantly. Thus adaptation can lead to a more permanent,
In the laboratory setting, cerebellar learning is most easily learned calibration that is used in specific situations. Clients
tested via motor adaptation, a form of motor learning that with cerebellar damage will not be able to make these short-
requires a modification of an already well learned motor term adaptations normally, and theoretically one would
behavior for new environmental or physical demands (in expect that they will not be able to form the more permanent
contrast to learning of a completely novel skill). Adaptation calibrations with repeated adaptation.
is an error-driven learning process that is acquired on a time Other forms of motor learning may not depend on the
scale of minutes or hours, as opposed to days or weeks.117,118 cerebellum and thus may be particularly useful for reha-
It is an active process: movement adaptation takes trial- bilitation for clients with cerebellar lesions, though this
and-error practice of the task, in which errors during one trial has never been formally tested. One example is use-
change movement on the subsequent trial. Storage of the dependent motor learning, in which a person strengthens
adapted movement is shown by the presence of aftereffects a movement pattern with repeated practice of that same
when the new demand is removed. Specifically, aftereffects pattern.120 It is not clear what mechanisms subserve this
are movement errors in the opposite direction to the original form of learning, though a Hebbian-like process in the
errors during adaptation and provide strong evidence that the cerebral cortex seems likely (i.e., repeated use strengthens
central nervous system adjusts the predictive control for the synapses in the brain that are engaged). Another form
body movements with practice.62,119 Thus when the new de- of motor learning is reward or reinforcement learning.
mand is removed, a process of active “unlearning” or dead- This may involve basal ganglia circuits to strengthen
aptation must occur to return the movement to its original movements that are rewarded.121 Whether individuals with
form. An example of a locomotor adaptation is shown in cerebellar damage can use either of these other forms of
Figure 21-8. In this case, a walking adaptation is induced by learning has not been experimentally tested. Yet if they
having subjects walk on a split-belt treadmill, where one belt can, these learning mechanisms might provide important
is moving at twice the speed of the other, forcing the two legs compensatory strategies for the loss of error-dependent
to walk at different speeds. Control subjects are able to rap- adaptations.
idly restore appropriate step length symmetry after only a
few minutes walking on the split-belt treadmill. They also
appear to store the newly learned set of (predictive) motor *References 56, 64, 65, 96, 97, 111-113.
choice.130,131 Nevertheless, anatomical studies have shown

clearly that the cerebellum has connections to brain areas
considered relatively purely cognitive in function, suggest-
ing that a cognitive role for the cerebellum is likely.132
Clinical Signs by Functional Division

Because of the organizational structure of the cerebellum
into functional longitudinal divisions, discrete areas are as-
sociated with specific signs and symptoms. Thus, depending
on the location and volume of cerebellar damage, clients
may have just a few or nearly all of the clinical manifesta-
tions described earlier. Clients with damage to the flocculo-
nodular lobe or midline zone typically demonstrate some
(though usually transient) loss of postural tone, impaired
upright posture and balance, gait ataxia, and oculomotor
deficits.2,51 On the other hand, damage to the intermediate
zone often results in action tremor, dysdiadochokinesia, and
dysmetria of the limbs.2,51 Finally, damage to the lateral
zone commonly produces dyssynergia, dysmetria, and dif-
ficulty planning complex limb movements, especially those
Figure 21-8 n Motor adaptation of step length symmetry during
that are visually guided (see Table 21-3).2,51
walking on a split-belt treadmill by a nondisabled individual (top)
Often, however, multiple longitudinal zones are affected at
and a subject with cerebellar damage (bottom). Each data point
the same time. For instance, the arteries supplying the cere-
represents the difference in step lengths between the legs (fast mi-
bellum each span more than one longitudinal zone, such that
nus slow) for all strides during regular walking (belts tied, speed
a stroke involving a single cerebellar artery would be likely to
0.5 m/s), split-belt adaptation walking (fast belt speed 1.0 m/s;
cause signs and symptoms associated with more than one
slow belt speed 0.5 m/s), and deadaptation walking (belts tied,
zone (see Table 21-1). Degenerative disorders affecting the
speed 0.5 m/s). Perfect symmetry between the legs is represented
cerebellum also are typically pancerebellar, affecting large
by a step length difference value of 0. Note that the split-belt condi-
regions of the cerebellum. Hence these clients will also typi-
tion perturbs step length symmetry initially in both subjects. The
cally have a wide variety of signs and symptoms. A careful
control subject rapidly adapts the walking pattern to restore ap-
and thorough clinical examination is therefore a requirement
propriate step length symmetry while walking on split belts. The
before a diagnosis for physical therapy can be made in clients
cerebellar subject does not adapt. Once the belts are returned to the
with cerebellar damage.
tied condition, the control subject shows a large negative afteref-
fect; that is, perturbed step length symmetry in the reverse direc-
MEDICAL MANAGEMENT OF CEREBELLAR
tion, which again is rapidly adjusted to restore near-symmetric step
DAMAGE
lengths. The cerebellar subject shows a reduced aftereffect and no
The greatest limitation in caring for clients with cerebellar
deadaptation. (From Morton SM, Bastian AJ: Cerebellar contribu-
dysfunction is the lack of any curative treatment for most
tions to locomotor adaptations during splitbelt treadmill walking.
forms of cerebellar damage. Pharmacological agents have
J Neurosci 26:9107–9116, 2006.)
been used, but with limited success. Often, clients with
ataxia and degenerative forms of cerebellar damage may be
prescribed vitamin E, coenzyme Q10, and/or its synthetic
Nonmotor Impairments analogs, but the effectiveness of these drugs in minimizing
Within the last 25 years, researchers have exposed a possible symptoms or otherwise slowing or halting the disease is
role of the cerebellum in a number of nonmotor, cognitive largely unsubstantiated.133,134 Other drugs that may be used
behaviors. Much of the early evidence for cerebellar in- include acetazolamide, amantadine, 4-aminopyridine, and
volvement in nonmotor tasks came from functional imaging buspirone chlorhydrate,6,135-137 but these also have question-
studies showing increased activation within the cerebellum able effectiveness. Studies of the effectiveness of medica-
during performance of certain tasks with a predominant tions for cerebellar ataxia have been significantly limited by
cognitive component, such as language processing.122,123 inadequate sample sizes, inappropriate outcome measures,
Speculation has since arisen that the cerebellum may be in- and/or limited follow-up. Given the unsatisfactory results
volved in not only language, but also working memory,124 from pharmacological interventions thus far, most clients
learning nonmotor associations between objects,125 and with cerebellar ataxias must rely primarily or solely on
higher-order executive functions.126 Loss of control over physical rehabilitation approaches to restore or reduce the
emotional behaviors127 and certain neurodevelopmental and symptoms of their disease.
neuropsychiatric disorders have also been said to be linked
to cerebellar damage.128,129 However, interpretation of some PHYSICAL AND OCCUPATIONAL THERAPY
investigations of the relationship between the cerebellum MANAGEMENT OF CEREBELLAR MOVEMENT
and cognition is limited in that it is sometimes difficult to DYSFUNCTION
separate the cognitive and motor components of a task, par- As is the case with all brain lesions, there is nearly always
ticularly in imaging studies in which subjects are instructed some level of natural, or spontaneous, recovery after dam-
to perform some motor task to indicate a cognitive age to the cerebellum. The extent of recovery depends on
complex interactions among numerous factors including the be repeated multiple times with the same limb, as subse-
source of damage; the severity, location, and volume of quent movements may look strikingly different (e.g., hypo-
damage; the presence or absence of damage to other brain metric on some trials, hypermetric on others). Comparing
regions; the presence or absence of other coexisting medical slow or preferred with “as fast as possible” speeds allows
conditions; age; and other factors. Several studies have now the clinician to determine the severity of the ataxia (gener-
indicated that motor recovery from a first-ever ischemic ally worst with fastest movements) and how well the client
cerebellar stroke is generally excellent, with minimal to no is able to compensate when allowed full use of feedback
residual deficits in up to 83% of patients.138-140 On the other mechanisms (the degree to which the movement is improved
hand, individuals with degenerative cerebellar disorders when performed slowly). It is also generally useful to com-
tend to have progressively worsening clinical signs and pare the same movements with and without vision, to deter-
symptoms.141 One study has shown that people with damage mine whether or not visual feedback improves movement
to the deep nuclei do not recover as well as those with dam- quality. A final caution about the tests of voluntary move-
age to only the cerebellar cortex and white matter.2 Another ment coordination is that the examiner must carefully
consideration is the degree to which other brain regions are dissociate limb incoordination from deficits of balance and/
lesioned. Individuals with cerebellar stroke or tumor may or vision. For example, if the client has difficulty maintain-
also have damage of the brain stem. Clients with multiple ing quiet unsupported sitting, he or she will most likely
sclerosis or head injury often also have cerebral and spinal demonstrate several abnormal movement patterns that re-
cord lesions. Moreover, the majority of the spinocerebellar semble classic limb ataxia when asked to move the limbs
ataxias (SCAs) affect other neural structures well beyond (e.g., dyssynergia, dysmetria) if tested in this unsupported
the cerebellum; these are often the corticospinal tract, cere- position. In this situation the examiner cannot distinguish
bral cortex, basal ganglia, and sometimes the peripheral whether the deficits observed are caused by a true incoordi-
nervous system. Generally speaking, the presence of multi- nation of voluntary limb movements or because of an
ple lesion locations is associated with a poorer outcome, inability to maintain the trunk in a stable and upright posi-
possibly because circuits that could otherwise serve a com- tion that provides the limb a stable base from which to gen-
pensatory role are also damaged.142 erate movement. Thus, to test coordination, the examiner
must give the client the necessary head and trunk support
Physical/Occupational Therapy Evaluation required for the limb movement task (e.g., test sitting in a
A majority of the components of the physical therapy ex- high-backed chair with manual support at the shoulders or
amination for clients with cerebellar dysfunction are the perform in supine position) if the client is unable to provide
same as would be performed with any client with a health this support by himself or herself. Similar confusion may
condition that is primarily neurological in origin. Therefore arise if the client has significant visual or other oculomotor
we will discuss only the features that are unique or of critical impairments such as diplopia. Here, the client would be
importance for the client with cerebellar pathology. In the likely to show apparent dysmetria during visually targeted
following sections we highlight just a few of these specific movements, but it would not be possible to determine
tests. Box 21-1 contains a more complete list. One important whether it was caused by real limb ataxia or a visual impair-
note is that many of the tests for cerebellar movement ment preventing the client from accurately identifying the
dysfunction are sensitive but not specific to cerebellar target location in space. This is not to say that it would not
pathologies. That is, although they often will reveal abnor- be beneficial to test limb coordination in positions or situa-
malities in a client with cerebellar damage, their results are tions that also challenge balance and/or vision; only that
frequently also observed to be abnormal in clients with during the initial examination of the client, one should be
other, noncerebellar disorders of a neuromuscular origin. careful to ensure an accurate determination of the source of
Therefore they should not be used in isolation to rule in or the movement impairment.
rule out cerebellar pathologies. A major component of almost all physical therapy
examinations of clients with cerebellar dysfunction is the
Tests of Impairments of Body Functions or posture and balance examination. Posture and balance should
Structures always be observed in both static and dynamic conditions
At the level of body functions or structures, physical/ and in both sitting and standing positions, as the client’s ca-
occupational therapists use a variety of simple tests and pabilities allow. The examination is performed in the same
measures to detect the typical movement impairments as- manner as for clients with other neurological disorders and
sociated with cerebellar dysfunction. Many of these fall into so is not described in detail here (see Box 21-1 for suggested
the category of limb movement coordination tests. Particu- components to emphasize with clients with cerebellar dys-
larly useful in the upper extremity are the finger-to-nose test, function). Important considerations specific for clients with
the alternating forearm supination-pronation test, and the cerebellar dysfunction include careful monitoring for symp-
hand or finger tapping test. The heel-to-knee test and foot or toms of nausea or vertigo (common in acute cerebellar
toe tapping test provide similar information for the lower stroke, may resolve quickly), observation for postural tremor
extremity. Each of these tests informs the therapist about the or titubation, and observation of the recovery from perturba-
presence and severity of many components of ataxia, includ- tions and the presence of lack of check. Although physical
ing dysmetria, dyssynergia, dysdiadochokinesia, decompo- therapists most often deal with posture and balance, occupa-
sition, and kinetic and/or intention tremor. Some general tional therapists also need to identify and examine these
rules should be applied when performing and interpreting components in order to analyze movement activity problems
the findings from these clinical tests. First, it is important to that are based upon a functional trunk such as dressing,
perform the test on and compare both sides. Each test should feeding, and other daily living activities.
BOX 21-1n TYPICAL CLINICAL TESTS AND MEASURES FOR THE PHYSICAL THERAPY EVALUATION
OF CEREBELLAR DYSFUNCTION
TESTS OF IMPAIRMENTS OF BODY FUNCTION OR STRUCTURE
1. Muscle tone
Hypotonia: Particularly involving postural extensor muscle groups. Can be assessed in supine, sitting, and/or standing position.
2. Voluntary movement coordination
For all coordination tests, the following guidelines are recommended, when appropriate: (1) compare both sides; (2) repeat each
test multiple times; (3) compare slow or preferred versus “as fast as possible” speeds; (4) compare with and without vision.
Finger-to-nose test: Vary the target (fingertip) locations. Require near-full excursion at elbow and shoulder. Observe for speed,
dysmetria, dyssynergia, decomposition, kinetic tremor, intention tremor.
Alternating forearm supination-pronation: Do not allow bracing of upper arm against trunk. Require elbow flexion and extension
along with forearm movements. Observe for speed, dysmetria, dyssynergia, decomposition, dysdiadochokinesia, kinetic tremor.
Hand or finger tapping: Observe for speed, dysmetria, dysdiadochokinesia, kinetic tremor.
Drawing or handwriting sample: Compare with and without permitting bracing upper arm against trunk and/or forearm on writing
surface. Observe for speed, dysmetria, dyssynergia, decomposition, kinetic tremor, intention tremor.
Holding static position, arms outstretched: Client holds position for several seconds. Observe for drift, postural tremor.
Resisted movements: Compare different levels of resistance. Observe for lack of check (rebound). Note: Ensure client safety;
rebound may be extreme, destabilizing, and/or injurious in severe cases.
Heel-to-knee test: Client performs with shoes removed and in supine position to test full excursion at hip and to prevent bracing
of thigh or hip on support surface. Client performs (1) repeated taps on knee with heel and (2) sliding the heel up and down the
lower leg. Observe for speed, dysmetria, dyssynergia, decomposition, dysdiadochokinesia, kinetic tremor, intention tremor.
Foot or toe tapping: Observe for speed, dysmetria, dysdiadochokinesia, kinetic tremor.
3. Static and dynamic balance
Sitting balance: Client performs with and without upper and lower extremity support or with and without trunk support (as skill
level permits), with and without vision. Test for recovery from self-imposed perturbations (upper extremity or head movements)
or external perturbations (gentle pushes by examiner). Observe resting preferred posture, tremor, sway, ability and effort re-
quired to maintain position, recovery from perturbations or other loss of balance. Inquire about vertigo, nausea, and subjective
perception of stability.
Standing balance: Client performs with and without upper and lower extremity support, with and without vision, and with feet
apart and together. Test for recovery from self-imposed perturbations (upper extremity or head movements) or external pertur-
bations (gentle pushes by examiner). Advanced testing: assume and maintain tandem stance and single limb stance. Observe
resting preferred posture, natural foot position (base of support), tremor, sway, ability and effort required to maintain position,
recovery from perturbations or other loss of balance. Inquire about vertigo, nausea, and subjective perception of stability.
4. Oculomotor performance
Smooth pursuit: Sitting, keeping the head still, client follows pen tip or similar small object with eyes. Test in all movement planes
and directions and through full range of motion. Vary speed. Observe for saccadic (choppy) pursuit.
Saccades: Sitting, keeping the head still and when verbally prompted, client alternately fixes gaze on one of two pen tips, or a pen
tip and the examiner’s nose, or other small objects. Vary the target (pen tip) locations, testing a variety of end point locations,
directions of movement, and distances traveled, including full range of motion. Observe for dysmetria, particularly on initial
trials.
Gaze-evoked nystagmus: Sitting, keeping the head still, client maintains gaze in a variety of locations, including near end ranges
of lateral gaze. Observe for nystagmus, particularly toward the direction of gaze.
TESTS OF ACTIVITY LIMITATIONS
1. Bed mobility
2. Transfers
3. Gait
As skill level permits, client performs natural, narrow, and tandem gait. Compare preferred versus fastest speed and with and
without vision. Test for recovery from self-imposed perturbations (upper extremity or head movements) or external perturba-
tions (gentle pushes by examiner). Observe on inclines and declines, uneven surfaces, negotiating introduced obstacles.
Observe walking while distracted (dual-task situation) and while holding an object with the upper extremity (e.g., hold a
Styrofoam cup half-filled with water). Test turns and control through narrow areas such as a doorway. Observe for speed,
irregularity of step height and distance, veering walking path, loss of balance, seeking out of upper extremity support surfaces
or guarding, leg dyssynergia, decomposition (e.g., stiff knees, reduced ankle motion), widened base of support, truncal sway,
kinetic tremor. Note distance tolerated and need for assistive devices and/or orthotics.
4. Stair climbing
5. Activities of daily living (ADLs): dressing, grooming, feeding, bathing, and so on
As described previously, because other areas of the ner- perform five repetitions of a block-stacking task with the
vous system are also frequently damaged along with the right upper extremity in 9.4 seconds without significant
cerebellum, it may be appropriate to test for impairments of drops or placement errors. Decomposition was observed
body functions or structures associated with pathology in- between shoulder and elbow joints and obvious intention
volving extracerebellar regions of the neuromuscular system. tremor was present during terminal block placement. When
Most commonly this involves testing of muscle strength, asked to repeat at a faster pace, the client performed the
somatosensation (including cutaneous sensation and proprio- same task in 6.0 seconds with one block dropped and two
ception), reflexes, passive muscle tone, and observation for misplaced on the stack (dysmetria). Notable dyssynergia
signs of other motor abnormalities such as spasticity, dysto- was also present.”
nia, abnormal synergies, chorea, bradykinesia, or resting
tremor. Standardized Clinical Scales
It is also important to examine the client’s initial level of There are two common standardized rating scales that quan-
endurance (fatigability), both in the cardiovascular and mus- tify the severity of cerebellar ataxia. The more well known
cular systems. For the cardiovascular system, this can be is the International Cooperative Ataxia Rating Scale
approximated by recording the response to sustained aerobic (ICARS).143a This scale was first published in 1997 by the
exercise on one or more measurement scales (e.g., perceived Ataxia Neuropharmacology Committee of the World Fed-
exertion, heart rate, blood pressure, respiratory rate). See eration of Neurology in response to an established need for
chapter 30 for additional information. For the muscular a universal scale to quantify ataxia in randomized clinical
system, this can be approximated by recording the maximal trials of pharmacological interventions for treating ataxia.
number of repetitions of a specific set of muscle contrac- The ICARS measures a client’s ability to perform 19 spe-
tions or limb movements that can be tolerated before force cific activities or movements using an ordinal scale. The
output or range of motion is reduced. These types of mea- activities are grouped into categories based on whether they
sures can provide a gross gauge of the overall level of relate to cerebellar dysfunction affecting (1) posture and
cardiovascular and musculoskeletal fitness of the client. gait, (2) limb movements, (3) speech, or (4) oculomotor
Because cerebellar dysfunction often leads to movements performance. A subscore is tallied for each category and
being much more effortful and often exaggerated, it is ex- a total score is obtained, ranging from 0 (no ataxia) to 100
tremely important that the client with cerebellar dysfunction (most severe ataxia). The ICARS has been found to be reli-
obtain adequate endurance for safe participation in daily able in clients with cerebellar dysfunction and has estab-
activities. lished criterion-related and external validity.144-147 More re-
cently, it was shown that the ICARS is sensitive to increases
Tests of Activity Limitations in ataxia severity over one year in persons with chronic cer-
Tests of activity limitations should typically proceed simi- ebellar degeneration.141 The Scale for the Assessment and
larly to the standard neurological examination. The observa- Rating of Ataxia (SARA) is a newer tool that was devised,
tion of gait should be given particular attention, as gait at least in part, out of concern over the construct validity of
ataxia is considered one of the most sensitive signs of cere- the ICARS subscale structure.148 The SARA is similar to
bellar damage and the inability to walk safely is a major the ICARS in that it quantifies performance of specific
participation limitation for most clients with motor disor- movements or activities on an ordinal scale (many of the test
ders.143 Box 21-1 provides suggested components of the gait activities are the same or similar to those in the ICARS), but
analysis for clients with cerebellar dysfunction. In addition, it does not categorize individual test items by body part. The
it may be necessary to evaluate the activity limitations re- SARA has fewer test items (only eight) and can therefore be
lated to speech, visual, and/or oculomotor deficits common administered faster in the clinical setting. The SARA has
to cerebellar dysfunction. With careful interviewing, the been shown to be reliable and valid for clients with SCAs.148
client may relay information about limitations in conversa- Either the ICARS or SARA scale may be useful for longitu-
tion and communication, reading, and so on that should be dinal tracking of disease progression in cerebellar degenera-
addressed in rehabilitation. The physical or occupational tion or for quantifying improvement of ataxia in clinical
therapist may be the first health care professional to note trials. The scales are detailed in Appendixes 21-A and 21-B.
these types of activity limitations and should refer the client
to the proper professional for additional services. Diagnosis, Prognosis, and Plan of Care
Documentation of the body function or structure impair- Clients with cerebellar dysfunction should be given a diag-
ments and activity limitations should include not only the nosis by physical and occupational therapy that identifies
level of assistance, if any, required to perform the skill (or the primary movement dysfunction and helps direct treat-
for the voluntary limb coordination tests, the degree of im- ment interventions to return the client to his or her desired
pairment, e.g., none, mild, moderate, or severe), but also a level of activity and participation, if possible. Typically,
detailed description of the deficits observed during the at- determination of a movement diagnosis is based largely on
tempted movement; that is, the movement quality. Specifi- both the results of the therapist’s examination and evalua-
cally, the severity and frequency of the specific features of tion, as well as on knowledge of the cause of the disorder
ataxia (e.g., decomposition, lack of check) should be re- and the extent of the lesion. The prognosis for recovery
ported when they accompany a given movement. Also use- likewise depends highly on the cause and extent of the
ful is documentation of the time taken to perform certain lesion. It is important to make a determination whether
tasks. For example, one might document performance of a the impairments in body function or structure and activity
functional reach and place task by a client with cerebellar limitations are expected to improve or expected not to im-
dysfunction in the following way: “The client was able to prove or potentially to worsen over time (e.g., with disease
progression). For impairments and activity limitations that with cerebellar dysfunction, incorporating both aerobic ex-
are expected to improve, emphasis should be placed on re- ercise to improve cardiovascular endurance and submaximal
covery of those skills; for those expected not to improve or resistive exercise to improve muscle fatigue resistance ap-
to worsen, emphasis should be placed on instruction and pears appropriate. Aerobic exercise activities might include
practice of compensatory strategies and general condition- walking, dance, recumbent or stationary cycling, rowing,
ing to minimize degradation due to fatigue. arm ergometry, swimming and aquatic exercise, as well as
many other possibilities.
Physical/Occupational Therapy
Interventions for Clients with Cerebellar Consider More Intensive, Longer Duration
Dysfunction Interventions
The literature on the effectiveness of rehabilitation interven- Because the cerebellum is thought to be a primary site of
tions for individuals with primary cerebellar damage is ex- motor learning and individuals with cerebellar damage often
tremely limited: there have been no randomized controlled have motor learning deficits, it is reasonable to consider
trials published. Of the few studies on the effects of rehabilita- whether these clients are capable of benefiting from any inter-
tion interventions in this patient population, all have been vention that relies on trial-and-error motor practice.101 It has
nonrandomized, noncontrolled small group101,149,150 or case been suggested that rehabilitation for clients with significant
study151-155 designs. The fact that there are so few studies avail- cerebellar dysfunction may take longer (more trials, more
able, each featuring different client populations (e.g., post- sessions) and might not ever be complete. The question re-
stroke versus post–surgical tumor resection versus cerebellar mains unanswered thus far. Of course, cerebellum-dependent
degeneration) and different outcome measures, makes deter- motor adaptation is only one of many motor learning mecha-
mining the most effective interventions difficult. Therefore we nisms, so it is possible that other processes can be engaged
provide here only a description of the major themes that have during rehabilitation. In support of this idea, it now appears
arisen from these studies to date. that at least partial “re-learning” of more normal movement
patterns is possible with selected cerebellar patient popula-
Gait and Balance Interventions tions.101,150 Notably, in the literature, gains were reported un-
Many of the intervention studies for cerebellar ataxia empha- der conditions of very frequent (10 hours/week) or very long
size stability and balance, especially during gait.101,149,153,155 (6 months) training schedules. This could be a necessity for
This likely is a reflection of the tight link between gait and clients with health conditions in which motor learning is
balance102 and the fact that gait ataxia is one of the most com- impaired. Figure 21-9 shows improvements in SARA scores
mon and debilitating signs of cerebellar damage.100 Common and self-selected walking speeds in a group of individuals
interventions include combinations of exercises targeting with significant progressive ataxia, in response to an inten-
gaze, static stance, dynamic stance, gait, and complex gait sive rehabilitation program targeting balance and dynamic
activities.101,153 Some examples of exercises in each of these intersegmental control. Much more research is needed to
categories are detailed in Box 21-2. Dynamic balance activi- determine the full range of improvements possible, the mini-
ties performed while sitting, kneeling, and quadruped have mal dosage of intervention required, and whether the benefits
also been advocated.101 Other interventions specific to the can be retained over the long term in this difficult patient
client’s individual impairments of body structure or function population.
should be implemented as necessary, for example, stretching
of short or tight ankle plantarflexors and exercises for Compensatory Strategies
the VOR.101,153 Locomotor training over ground and on Compensation is a common component of the plan of care
treadmills and with and without body weight support has for clients with cerebellar dysfunction. Many clients begin
also been used with some success in single case exam- using compensations subconsciously, whereas others need
ples.151,156 It is not clear how imbalance is corrected in the to be taught these strategies and when to use them. If the
body weight support environment, however. With all gait client is not expected to recover premorbid movement pat-
and balance activities, it is critical that the exercise be suffi- terns, compensation can enable the individual to regain a
ciently and increasingly challenging, so as to facilitate plas- certain prior level of activity or societal participation despite
ticity in the nervous system.157,158 In a recent study in mice, an abnormal movement pattern. Instruction in compensation
it was suggested that trial-and-error practice is a require- can also be valuable in situations when full recovery is ex-
ment for regaining full motor recovery during cerebellar pected but the client would benefit from the use of compen-
remyelination.159 satory strategies for the short term, such as for safety pur-
poses. One compensatory strategy that works well for
Aerobic Exercise and Resistance Training clients with cerebellar dysfunction is the instruction to sim-
Integration of aerobic exercise and resistance training into ply slow down movements. Recall that slower movements
the treatment plan is recommended for a majority of clients are less dyssynergic and less hypermetric.49 Voluntarily re-
with cerebellar dysfunction, particularly if it is expected the ducing the number of segments moving at the same time
client will not regain premorbid status. If full recovery is not (i.e., decomposition) also helps reduce dyssynergia and dys-
attained, nearly all types of movements will be generally metria.49,83 In some cases, reminding clients to use visual
more effortful, requiring increased energy expenditure and cues can also be helpful, for instance, to use vertical mark-
demanding greater concentration. It is well known that re- ings of a doorway to maintain upright stability, although for
petitive fatiguing activity worsens postural control160,161 and certain types of cerebellar damage this is not effective.93,94
therefore may contribute to trips, falls, or other injuries.162 For gait, deliberately widening stance can be helpful, both
Because imbalance is such a common outcome for clients for maintaining balance and for preventing tripping over a
n COMMON GAIT AND BALANCE INTERVENTIONS FOR CLIENTS WITH CEREBELLAR

BOX 21-2
DYSFUNCTION
1. Gaze and eye movements
a. VOR: (i) visually fixate on stationary target, slow head movements; (ii) visually fixate on target moving in opposite
direction, slow head movements; (iii) VOR cancellation, visually fixate on target moving in same direction, slow head
movements. Progression: increase and vary speed, perform eyes closed, add complexity to background.
b. Saccades: active eyes alone and combined eye and head movements between two stationary targets. Progression: increase
and vary speed.
2. Static stance
a. Feet together, arms across chest, eyes open and closed, with and without slow head movements. Progression: increase time
with eyes closed, increase and vary speed of head movements.
b. Stand on foam, feet apart, arms across chest, eyes closed briefly and intermittently. Progression: narrow base of support,
increase time with eyes closed.
c. Semitandem stance, arms across chest, eyes closed briefly and intermittently. Progression: narrow base to full tandem stance,
increase time with eyes closed, perform semitandem stance on foam.
d. Unilateral stance, arms across chest, eyes open. Progression: perform with intermittent then longer periods with eyes closed,
perform on foam.
3. Dynamic stance
a. March in place, arms across chest, eyes open and closed. Progression: increase time with eyes closed, add and incrementally
increase pause time in unilateral stance, add head movements.
b. Standing toe taps, forward, backward, to the side, alternating legs, arms across chest, eyes open and closed. Progression:
increase step distance, increase time with eyes closed, add head movements.
c. March in place on foam, arms across chest, eyes closed briefly and intermittently. Progression: increase time with eyes
closed, add and incrementally increase pause time in unilateral stance.
d. Standing 360-degree turn, rightward, leftward, arms across chest, eyes open and closed. Progression: tighten turns, increase
speed.
e. Standing reaches, feet apart and together, eyes open and closed. Progression: increase time with eyes closed, increase reach
distance, vary directions, narrow base of support to feet together position.
f. Standing bends and squats, feet apart and together, eyes open and closed. Progression: increase time with eyes closed,
narrow base of support to feet together, reach to touch the floor.
g. Transitions: standing to supine on floor and back up. Progression: transition into and out of all possible positions, with and
without upper-extremity support, eyes open and closed, including kneel, half-kneel, quadruped, side sit, squat, and so on.
4. Gait
a. Narrow base of support, arms at sides. Progression: increase gait speed.
b. Normal base of support, arms at sides with periodic head movements. Progression: narrow base of support, increase and vary
speed and frequency of head movements.
c. Gait with wide turns, arms at sides. Progression: sharpen angle of the turn, increase gait speed, add head movements.
d. Gait with eyes closed, arms at sides. Progression: add turns, add head movements, increase gait speed.
e. Gait with perturbations: (i) self-imposed (e.g., large arm movements); (ii) external (e.g., pushes by therapist). Progression:
increase speed and amplitude of perturbations, make external perturbations unexpected.
5. Complex gait
a. Sideways and backward gait, eyes open, arms at sides. Progression: narrow base of support, add head movements, perform
with eyes closed, increase gait speed.
b. Incline and decline gait, eyes open, arms at sides. Progression: narrow base of support, add head movements.
c. Gait on foam, padded or other compliant surface, eyes open, arms at sides. Progression: narrow base of support, add turns,
add head movements, perform with eyes closed.
d. Semitandem gait, eyes open, arms at sides. Progression: narrow base to tandem gait, add head movements, perform with eyes
closed.
e. Gait while negotiating obstacles, eyes open, arms at sides, avoid, step onto, step over. Progression: increase obstacle number
and size, vary obstacle placement.
f. Gait while distracted, eyes open, arms at sides. Add cognitive task as a distracter; start with responding to simple yes-no
questions, progress to difficult tasks (e.g., counting, performing two- or three-digit addition, subtraction).
Adapted from Gill-Body KM, Popat RA, Parker SW, Krebs DE: Rehabilitation of balance in two patients with cerebellar dysfunction. Phys Ther 77:534–552,
1997; and Ilg W, Synofzik M, Brötz D, et al: Intensive coordinative training improves motor performance in degenerative cerebellar disease. Neurology
73:1823–1830, 2009.
VOR, Vestibuloocular reflex.
clients with significant imbalance and limb ataxia but who

are ambulatory will often not be safe with any type of cane
and instead require a wheeled walker and/or the physical
assistance of a caretaker to walk. A final strategy is to re-
move or minimize distractions during effortful activities.
Distraction, for example, dividing attention between a motor
and a cognitive task, can worsen ataxia.163
The Controversy of Weighting

Rehabilitation textbooks have traditionally supported the
use of weights for adults and particularly children with
cerebellar dysfunction and ataxia. Either the trunk may
be weighted by having the client wear a vest or pack that
is weighted, increasing the load on the body axially,164 or
the limbs may be weighted individually165,166 with simple
wrist or ankle weights. Presumably this trend was based on
the common finding, first noted by Holmes,74 that clients
with cerebellar dysfunction appear improved (move more
smoothly and with reduced tremor) if they are asked to hold
a heavy weight in the hand while moving the arm. Today it
is known that cerebellar tremor is at least in part mechanical
in nature, such that anything that adds inertia to the moving
segment would reduce the amplitude of the tremor.86 This
most likely explains the improvement that has been re-
ported. However, this apparent benefit would certainly not
continue once the weight is removed, and a reduction in
tremor does not indicate a normalization of any other pa-
rameters of movement. In fact, weighting of the extremities
Figure 21-9 n Changes in (A) SARA scores and (B) self-selected would seem a questionable choice, given the known diffi-
walking speeds for 16 individuals with progressive ataxia who com- culty of clients with cerebellar dysfunction to predict and
pleted 4 weeks of intensive (1 hr/day, 3 days/wk supervised training account for limb dynamics or to account for interaction
plus 1 hr/day, 7 days/wk independent home exercise) rehabilitation torques.49,50 Addition of weights, particularly to the distal
emphasizing balance. Measures were recorded at four time periods segments, would seem only to make this task more difficult.
(E1 to E4), corresponding to 8 weeks preintervention (E1), imme- Evidence for this has been demonstrated in a study of pa-
diately preintervention (E2), immediately postintervention (E3), tients with cerebellar ataxia in which it was shown that
and 8 weeks postintervention (E4). Subjects were categorized as weighting the extremities worsens the amount of hyperme-
having either a predominant cerebellar ataxia (e.g., SCA2, SCA6, tria during a simple single-joint wrist movement task.87
idiopathic cerebellar ataxia, or another autosomal dominant cere- One unintended consequence of weighting is that it
bellar ataxia) or a predominant afferent ataxia (e.g., Friedreich tends to slow movements down. As described earlier,
ataxia or other significant somatosensory neuropathy). The group slower movements are less hypermetric and less dyssyner-
overall showed significant improvements in both measures (reduced gic than preferred-speed or fast movements. In early studies
SARA scores and increased walking speeds) after the 4 weeks of of weighting, speed was not monitored or controlled for, so
exercise and retained these improvements over a further 8 weeks. a simple reduction in movement speed could account for
When broken down by ataxia category, subjects with a predominant the immediate apparent improvement described in some
cerebellar ataxia, but not the subjects with a predominant sensory reports.165,166 Therefore weighting may indeed provide a
ataxia, showed a significant improvement in walking speed. (Asterisks [*] benefit to some clients with ataxia but it is most likely an
denote significant improvements immediately following the inter- indirect one, related more to decreased movement speed.
vention, while no significant changes occurred during pre and post Accordingly, the significant potential for negative conse-
intervention periods.) SARA, Scale for the Assessment and Rating quences of weighting (i.e., worsened hypermetria, increased
of Ataxia. (Adapted with permission from the American Academy interaction torques, and the annoyance and physical awk-
of Neurology. From Ilg W, Synofzik M, Brötz D, et al: Intensive wardness of weights) makes this intervention option seem
coordinative training improves motor performance in degenerative inferior to the simpler strategy of instructing the client in
cerebellar disease. Neurology 73:1823–1830, 2009.) voluntarily moving more slowly.167
SUMMARY
foot. The use of assistive devices for gait should be consid- The cerebellum is highly unique, in terms of its anatomy,
ered on a case-by-case basis. For some clients, facilitating its physiology, and the movement-related impairments indi-
upper-extremity support and increasing the base of support viduals experience if it becomes damaged. Ataxia, or move-
improve balance during gait. However, for others, the level ment incoordination, is the major sign of cerebellar damage
of skill required to coordinate controlling the device with the and can affect limb movements, eye movements, speech,
movements of arms and legs is too difficult, and using the balance, and walking. Depending on which cerebellar
device actually worsens the instability. Generally speaking, functional longitudinal zone is lesioned, one or more of
these specific categories of movements will be impaired. strategies and general conditioning may be effective in at
Although the precise mechanisms are not yet fully under- least partially restoring prior participation levels.
stood, it is widely acknowledged that the cerebellum is inte-
gral to (1) coordination of movements, particularly fast, Acknowledgments
multijoint movements and (2) adaptation of movements to The authors are supported by the following grants: NIH R01
changes in body conditions or the environment and learning HD040289 (AJB), R01 HD048741 (AJB), K01 HD050369
new movement patterns based on trial-and-error practice (SMM), and R21 NS067189 (SMM). Special thanks to
(motor learning). Unfortunately, evidence for the effective- Ms. Jennifer Keller for assistance with the videos and
ness of rehabilitation interventions for individuals with pri- Ms. Tara Hackney for assistance with the illustrations.
mary cerebellar damage is extremely limited and incom-
plete. One critical factor in the evaluative process is the
determination of whether the body function or structure References
impairments and activity limitations are expected to im- To enhance this text and add value for the reader, all refer-
prove or expected not to improve or even worsen, as is ences are included on the companion Evolve site that ac-
the case in progressive diseases. If expected to improve, companies this textbook. This online service will, when
emphasis should be placed on trial-and-error practice of in- available, provide a link for the reader to a Medline abstract
creasingly challenging motor activities. Balance and gait for the article cited. There are 167 cited references and other
skills may be highlighted. On the other hand, if recovery general references for this chapter, with the majority of
is not expected, instruction and practice of compensatory those articles being evidence-based citations.
APPENDIX 21-A n International Cooperative Ataxia Rating Scale (ICARS)

I. POSTURE AND GAIT DISTURBANCES 4 5 Immediate falling
1. Walking capacities (Observe walking 10 m, near a wall, Score: _____
including half-turn.) 6. Body sway with feet together, eyes closed
0 5 Normal 0 5 Normal
1 5 Almost normal naturally but unable to walk with feet in 1 5 Slight oscillations
tandem position 2 5 Moderate oscillations (,10 cm at the level of
2 5 Walking without support but clearly abnormally and head)
irregularly 3 5 Severe oscillations (.10 cm at the level of head),
3 5 Walking without support but with considerable threatening the upright position
staggering; difficulties in half-turn 4 5 Immediate falling
4 5 Walking with autonomous support not possible; uses the Score: _____
episodic support of the wall 7. Quality of sitting position (on flat, hard surface, thighs
5 5 Walking possible only with one stick together, arms folded)
6 5 Walking possible only with two special sticks or with a stroller 0 5 Normal
7 5 Walking only with accompanying person 1 5 With slight oscillations of the trunk
8 5 Walking impossible, even with accompanying person 2 5 With moderate oscillations of the trunk and legs
(wheelchair) 3 5 With severe disequilibrium
Score: _____ 4 5 Impossible
2. Gait speed (Observe only if subject scores ,4 on preceding Score: _____
test; otherwise automatically score this test as 4.) Posture and Gait Subscore: _____/34
0 5 Normal
II. KINETIC FUNCTIONS
1 5 Slightly reduced
8. Knee-tibia test: decomposition of movement and intention
2 5 Markedly reduced
tremor (Subject is supine with head tilted to allow visual control.
3 5 Extremely slow
Ask subject to raise one leg in the air (to a height of approximately
4 5 Walking with autonomous support no longer possible
40 cm) and place heel on opposite knee and then slide the heel
Score: _____
down the anterior tibial surface of the resting leg toward the ankle.
3. Standing capacities, eyes open (Ask subject to stand on one
On reaching the ankle joint, the leg is again raised, and the action
foot; if impossible, ask to stand with feet in tandem; if impos-
repeated. Repeat for at least three trials on each side.)
sible, ask to stand with feet together. For the natural position,
0 5 Normal
ask subject to find a comfortable standing position.)
1 5 Heel lowering in continuous axis, but movement is
0 5 Normal; able to stand on one foot .10 s
decomposed in several phases (without real jerks) or
1 5 Able to stand with feet together but no longer able to
abnormally slow
stand on one foot .10 s
2 5 Heel lowering jerkily in the axis
2 5 Able to stand with feet together but no longer able to
3 5 Heel lowering jerkily with lateral movements
stand with feet in tandem position
4 5 Heel lowering jerkily with extremely strong lateral
3 5 No longer able to stand with feet together but able to
movements or test impossible
stand in natural position without support, with no or
Score right: _____ Score left: _____
moderate sway
9. Action tremor in the heel-to-knee test (Same test as preced-
4 5 Standing in natural position without support, with
ing one; visual control required. Observe action tremor of the
considerable sway and considerable corrections
heel when asked to hold the heel on the knee for a few seconds
5 5 Unable to stand in natural position without strong support
before sliding down the leg.)
of one arm
0 5 No trouble
6 5 Unable to stand at all, even with strong support of two arms
1 5 Tremor stops immediately when the heel reaches the knee
Score: _____
2 5 Tremor stops in ,10 s after reaching the knee
4. Spread of feet in natural position without support, eyes
3 5 Tremor continues .10 s after reaching the knee
open (Ask subject to find a comfortable position, then measure
4 5 Uninterrupted tremor or test impossible
the distance between medial malleoli.)
0 5 Normal (,10 cm)
10. Finger-to-nose test: decomposition and dysmetria (Subject
1 5 Slightly enlarged (.10 cm)
sitting; visual control required. Start trials with hands resting
2 5 Clearly enlarged (25-35 cm)
on knees. Repeat for at least three trials on each side.)
3 5 Severely enlarged (.35 cm)
0 5 No trouble
4 5 Standing in natural position impossible
1 5 Oscillating movement without decomposition of the
Score: _____
movement
5. Body sway with feet together, eyes open
2 5 Segmented movement in two phases and/or moderate
0 5 Normal
dysmetria in reaching nose
1 5 Slight oscillations
3 5 Segmented movement in more than two phases and/or
2 5 Moderate oscillations (,10 cm at the level of head)
considerable dysmetria in reaching nose
3 5 Severe oscillations (.10 cm at the level of head),
4 5 Dysmetria preventing the subject from reaching nose
threatening the upright position
APPENDIX 21-A n International Cooperative Ataxia Rating Scale (ICARS)—cont’d

11. Finger-to-nose test: kinetic tremor of the finger (Same test Limb Kinetics Subscore: _____/52
as preceding one; visual control required. Observe movement III. SPEECH DISORDERS
tremor occurring during the ballistic phase of the movement.) 15. Dysarthria: fluency of speech (Ask subject to repeat a
0 5 No trouble standard phrase several times; for instance, “A mischievous
1 5 Simple swerve of the movement spectacle in Czechoslovakia”.)
2 5 Mild tremor, estimated amplitude ,10 cm 0 5 Normal
3 5 Moderate tremor, estimated amplitude 10 to 40 cm 1 5 Mild modification of fluency
4 5 Severe tremor, estimated amplitude .40 cm 2 5 Moderate modification of fluency
Score right: _____ Score left: _____ 3 5 Considerably slow and dysarthric speech
12. Finger-finger test: action tremor and/or instability (Subject 4 5 No speech
sitting. Ask subject to flex, abduct and internally rotate shoul- Score: _____
ders and then flex elbows so as to have both index fingers 16. Dysarthria: clarity of speech
pointing at each other at midline. Subject is to maintain index 0 5 Normal
finger positions for approximately 10 s, at distance of ap- 1 5 Suggestion of slurring
proximately 1 cm apart (not touching) and hands at the level 2 5 Definite slurring, most words understandable
of the thorax.) 3 5 Severe slurring, speech not understandable
0 5 Normal 4 5 No speech
1 5 Mild instability Score: _____
2 5 Moderate oscillations of finger, estimated amplitude
Dysarthria Subscore: _____ /8
,10 cm
3 5 Considerable oscillations of finger, estimated amplitude IV. OCULOMOTOR DISORDERS
10 to 40 cm 17. Gaze-evoked nystagmus (Ask subject to look laterally at
4 5 Jerky movements, amplitude .40 cm examiner’s finger. Observe for nystagmus, mainly horizontal,
Score right: _____ Score left: _____ but could be oblique, rotary, or vertical.)
13. Pronation-supination alternating movements (Subject 0 5 Normal
sitting. Ask subject to raise the forearm vertically and alternate 1 5 Transient
supinating and pronating the forearm on the lap, combining 2 5 Persistent but moderate
elbow flexion and extension with forearm supination and 3 5 Persistent and severe
pronation.) Score: _____
0 5 Normal 18. Abnormalities of ocular pursuit (Ask subject to follow the
1 5 Slightly irregular and slowed slow lateral movement of the examiner’s finger.)
2 5 Clearly irregular and slowed, but without sway of the 0 5 Normal
elbow 1 5 Slightly saccadic
3 5 Extremely irregular and slowed movement, with sway of 2 5 Clearly saccadic
the elbow Score: _____
4 5 Movement completely disorganized or impossible 19. Dysmetria of the saccade (Examiner’s two fingers are placed,
Score right: _____ Score left: _____ one in each temporal visual field of the subject, whose eyes are
14. Drawing of the Archimedes’ spiral on a predrawn pattern in the primary position. Ask subject to shift eyes to look later-
(Subject sitting at a table with a sheet of paper fixed in place. ally at the finger, first on the right and then on the left. Average
Ask subject to trace the spiral with a pen using the dominant overshoot or undershoot of the two sides is then estimated.)
hand; no timing requirements.) 0 5 Absent
0 5 Normal 1 5 Bilateral clear overshoot or undershoot of the saccade
1 5 Impairment and decomposition, the line quitting the Score: _____
pattern slightly, but without hypermetric swerve Oculomotor Subscore: _____/6
2 5 Line completely out of the pattern, with recrossings TOTAL ATAXIA SCORE (SUM OF FOUR SUBSCORES):
and/or hypermetric swerves _____/100
3 5 Major disturbance caused by hypermetria and
decomposition
4 5 Drawing completely disorganized or impossible
Score: _____
Modified with permission from Trouillas P, Takayanagi T, Hallett M, et al: International Cooperative Ataxia Rating Scale for pharmacological assessment of
the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. Neurol Sci 145:205–211, 1997.
APPENDIX 21-B n Scale for the Assessment and Rating of Ataxia (SARA)
1. Gait (Subject (a) walks at a safe distance parallel to a wall in- unpredictable directions in a frontal plane, at about 50% of
cluding a half-turn (turn around to face the opposite direction of subject’s reach distance. Movements are approximately 30 cm
gait) and (b) walks in tandem (heels to toes) without support.) in distance and occur at a rate of approximately one reach every
0 5 Normal, no difficulties in walking, turning, or walking 2 s. Ask subject to follow the movements, pointing with his or
tandem (up to one misstep allowed) her index finger as fast and precisely as possible. Average per-
1 5 Slight difficulties but visible only when walking 10 con- formance of last three movements is rated. Rate separately for
secutive steps in tandem each side.)
2 5 Clearly abnormal, tandem walking .10 steps not possible 0 5 No dysmetria
3 5 Considerable staggering, difficulties in half-turn, but 1 5 Dysmetria, undershooting or overshooting target ,5 cm
walks without support 2 5 Dysmetria, undershooting or overshooting target ,15 cm
4 5 Marked staggering, intermittent support of the wall 3 5 Dysmetria, undershooting or overshooting target .15 cm
required 4 5 Unable to perform five pointing movements
5 5 Severe staggering, permanent support of one stick or light Score right: _____ Score left: _____ Mean score
support by one arm required ([R 1 L]/2): _____
6 5 Walking .10 m only with strong support (two special 6. Nose-finger test (Subject seated comfortably, feet and trunk
sticks or stroller or accompanying person) supported if needed. Ask subject to point repeatedly with the
7 5 Walking ,10 m only with strong support (two special index finger from his or her nose to examiner’s finger, which is
sticks or stroller or accompanying person) in front at about 90% of subject’s reach distance. Movements
8 5 Unable to walk, even supported are performed at moderate speed. Average performance of
Score: _____ movements is rated according to the amplitude of the kinetic
2. Stance (Subject stands (a) in natural position, (b) with feet to- tremor. Rate separately for each side.)
gether in parallel (big toes touching each other), and (c) in 0 5 No tremor
tandem (both feet on one line, no space between heel and toe). 1 5 Tremor with an amplitude ,2 cm
Subject does not wear shoes, eyes are open. For each condition, 2 5 Tremor with an amplitude ,5 cm
three trials are allowed. Best trial is rated.) 3 5 Tremor with an amplitude .5 cm
0 5 Normal, able to stand in tandem for .10 s 4 5 Unable to perform five pointing movements
1 5 Able to stand with feet together without sway but not in Score right: _____ Score left: _____ Mean score
tandem for .10 s ([R 1 L]/2): _____
2 5 Able to stand with feet together for .10 s but only with 7. Fast alternating hand movements (Subject seated comfort-
sway ably, feet and trunk supported if needed. Ask subject to perform
3 5 Able to stand for .10 s without support in natural posi- 10 cycles of repetitive alternation of pronation and supination
tion but not with feet together of the forearm on his or her thigh as fast and as precisely as
4 5 Able to stand for .10 s in natural position only with in- possible. Demonstrate the movement at a speed of approxi-
termittent support mately 10 cycles in 7 s. Record times for movement execution.
5 5 Able to stand .10 s in natural position only with constant Rate separately for each side.)
support of one arm 0 5 Normal, no irregularities, performs in ,10 s
6 5 Unable to stand for .10 s even with constant support of 1 5 Slightly irregular but performs in ,10 s
one arm 2 5 Clearly irregular, single movements difficult to distin-
Score: _____ guish or relevant interruptions, but performs in ,10 s
3. Sitting (Subject sits on an examination table without support of 3 5 Very irregular, single movements difficult to distinguish
feet, eyes open and arms outstretched to the front.) or relevant interruptions, performs in .10 s
0 5 Normal, no difficulties sitting .10 s 4 5 Unable to complete 10 cycles
1 5 Slight difficulties, intermittent sway Score right: _____ Score left: _____ Mean score
2 5 Constant sway but able to sit .10 s without support ([R 1 L]/2): _____
3 5 Able to sit for .10 s only with intermittent support 8. Heel-shin slide (Subject supine on examination bed without
4 5 Unable to sit for .10 s without continuous support sight of his legs. Ask subject to lift one leg, point with the heel
Score: _____ to the opposite knee, slide down along the shin to the ankle, and
4. Speech disturbance (Speech is assessed during normal lay the leg back on the examination bed. The task is performed
conversation.) three times. The slide component of the movement should be
0 5 Normal performed within 1 s. If subject slides down without contact to
1 5 Suggestion of speech disturbance shin in all three trials, rate as 4. Rate separately for each side.)
2 5 Impaired speech but easy to understand 0 5 Normal
3 5 Occasional words difficult to understand 1 5 Slightly abnormal, contact to shin maintained
4 5 Many words difficult to understand 2 5 Clearly abnormal, goes off shin #3 times during 3 cycles
5 5 Only single words understandable 3 5 Severely abnormal, goes off shin 4 times during 3 cycles
6 5 Speech unintelligible, anarthria 4 5 Unable to perform the task
Score: _____ Score right: _____ Score left: _____ Mean score
5. Finger chase (Subject seated comfortably, feet and trunk ([R 1 L]/2): _____
supported if needed. Examiner sits in front of subject and per- Total Ataxia Score (Sum of Eight Test Scores): _____/40
forms five consecutive sudden and fast pointing movements in
Modified with permission from Schmitz-Hubsch T, du Montcel ST, Baliko L, et al: Scale for the Assessment and Rating of Ataxia: development of a new
clinical scale. Neurology 66:1717–1720, 2006.
CHAPTER 22 Balance and Vestibular Dysfunction
LESLIE K. ALLISON, PT, PhD, and KENDA FULLER, PT, NCS

activity limitation After reading this chapter the student or therapist will be able to:
anticipatory postural adjustments 1. Describe both central and peripheral sensory and motor components of the postural
automatic postural responses control system.
balance 2. List common postural control impairments found in clients with neurological problems.
base of support 3. List commonly used balance tests, and distinguish which are appropriate for clients at
body system problems low, moderate, and high levels of function.
center of gravity 4. Differentiate how test results are used to identify body system impairments and
impairment activity limitations that limit participation.
limit of stability 5. Analyze the interaction of individual, task, and environmental factors that affect
motor learning stages balance.
participation 6. Describe how to plan and progress balance exercise programs to increase the use of,
sensory conflict or compensation with, available sensory inputs.
sensory environment 7. Describe how to plan and progress balance exercise programs to facilitate anticipatory
strategies postural adjustments to prevent balance loss and provoke automatic postural
systems model or systems approach responses to regain balance after unexpected disturbances.
volitional postural movements 8. Describe how to plan and progress balance exercise programs to increase the control
of center of gravity in upright postures and during gait.
9. Describe how to increase the difficulty level of balance exercise programs in order to
promote the automaticity of postural control during functional activities.
10. Identify and analyze the function of the vestibular system.
11. Describe how to facilitate adaptation and central nervous system reorganization to
regain control of balance and decrease dizziness.
12. Identify patterns of recovery that influence choices of intervention.
Balance Function and Disorders deficits are permanent or temporary, and whether recovery or
Leslie Allison, PT, PhD progressive decline is expected, is critical. This medical prog-
nostic information will assist physical and occupational thera-
pists in goal setting and intervention planning.
N o matter what the neurological diagnosis, a disease or

injury that affects the nervous system is likely to compro-
mise one or more of the postural control mechanisms. For
The International Classification of Functioning, Disabil-
ity and Health (ICF) model described in Chapter 1 and
illustrated in Figure 1-1 describes the interactions of body
example, clients with such diverse diagnoses as stroke, head function and structure problems (impairments) and activity
trauma, spinal cord injury, peripheral neuropathy, multiple limitations as seen in clients with balance disorders, and
sclerosis (MS), Parkinson disease, cerebellar dysfunction, how these functional activity limitations restrict an individ-
cerebral palsy, and Guillian-Barré syndrome all experience ual’s ability to participate in life situations, thus decreasing
disequilibrium problems. One common thread among all these quality of life. Balance impairments negatively affect func-
different medical diagnoses is the presence of balance impair- tion, often reducing the individual’s ability to participate
ments. Clients with different medical diagnoses may have the fully in life.2 These impairments often restrict activity levels,
same balance impairments, and clients with the same medical produce abnormal compensatory motor behavior, and may
diagnosis may have different balance impairments depending require devices for support or assistance from another per-
on which portions of the postural control system are involved.1 son. Falls can result when imbalance is severe, leading to
To optimally understand and manage balance problems, a test secondary injuries. To avoid these consequences and ad-
of each balance component and the interactive nature of the vance the functional status of clients, therapists should un-
components is important. The traditional medical “diagnostic” derstand both the demands that various environments and
model does not provide this information and is not the most functional tasks place on postural control systems and the
beneficial model for planning balance rehabilitation interven- impairments that may diminish the ability of those systems
tions. The medical diagnosis is relevant: knowing whether to respond adequately.
653
BALANCE almost constantly, such as shifting from foot to foot, reach-

ing for the telephone, or catching an object that is falling
Definitions of Balance from a high shelf. Even reactions to involuntary balance
Balance is a complex process involving the reception and disturbances, such as a slip or trip, are modified on the basis
integration of sensory inputs and the planning and execution of the immediate task. A man carrying a bag of groceries
of movement to achieve a goal requiring upright posture. It who slips may drop the bag to reach with both hands and
is the ability to control the center of gravity (COG) over the catch himself. If he is instead carrying his infant child, he
base of support in a given sensory environment.3,4 The COG may reach with only one hand or even take the fall if by do-
is an imaginary point in space, calculated biomechanically ing so he can protect the infant from harm. Often in real life
from measured forces and moments, where the sum total of we perform several tasks at once, such as carrying a laundry
all the forces equals zero. In a person standing quietly the basket while walking, or talking on a cellular phone while
COG is located just forward of the spine at approximately climbing a flight of stairs. When tasks are undertaken con-
the S2 level. With movement of the body and its segments, currently, attention must be divided between them, which
the location of the COG in space constantly changes. The may also affect balance abilities.
base of support is the body surface that experiences pressure All these variables—the location of the COG, the base
as a result of body weight and gravity; in standing it is the of support, the limit of stability, the surface conditions, the
feet, and in sitting it includes the thighs and buttocks. The visual environment, the intentions and task choices—are
size of the base of support will affect the difficulty level of inconstant, producing changing demands on the systems
the balancing task. A broad base of support makes the task that control balance. The integrity and interaction of pos-
easier; a narrow base makes it more challenging. The COG tural control mechanisms allow a wide range of movements
can travel farther while still remaining over the base if the and functions to be achieved without loss of balance.
base is large. The “shape” of the base of support will alter
the distance that the COG can move in certain directions. HUMAN CONTROL OF BALANCE
Any given base of support places a limit on the distance Early studies of postural control mechanisms using selec-
a body can move without either falling (as the COG exceeds tively lesioned cats and primates focused on reflexive and re-
the base of support) or establishing a new base of support by active equilibrium responses that are relatively “hard-wired.”6
reaching or stepping (to relocate the base of support under These valuable studies brought to light certain stereotypical
the COG). This perimeter is frequently referred to as the motor responses to specific sensory stimuli, such as the
limit of stability or stability limit.3,5 It is the farthest distance crossed extension reflex or tonic neck reflexes. There is no
in any direction a person can lean (away from midline) with- doubt that these reflexive and reactive responses—for exam-
out altering the original base of support by stepping, reach- ple, the vestibuloocular reflex (VOR) and the protective ex-
ing, or falling. tension reactions—are foundational to normal postural con-
trol. However, the postural control system encompasses much
Environmental Context more than these subcortically driven components. Balance
This biomechanical task of keeping the COG over the base abilities are heavily influenced by higher-level neural circuitry
of support is always accomplished within an environmental and other systems (e.g., cognitive, musculoskeletal), as well.5
context, which is detected by the sensory systems. The sen- In addition, the nervous system is influenced by and respon-
sory environment is the set of conditions that exist, or are sive to the demands placed on it by the tasks being accom-
perceived to exist, in the external world that may affect bal- plished and the environments in which those tasks are per-
ance. Peripheral sensory receptors gather information about formed.7-9 All of these facets are included in a systems
the environment, body position and motion in relation to the approach to dynamic equilibrium.10-12 Examination and inter-
environment, and body segment positions and motions in vention methods based on this systems model have conse-
relation to the self. Central sensory structures process this quently evolved.11,13
information to perceive body orientation, position, and
motion and to determine the opportunities and limitations The Systems Approach
present in the environment. Gravity is one environmental The dynamic systems model for dynamic equilibrium recog-
condition that must be dealt with to remain stable. It is a nizes that balance is a result of interactions among the indi-
constant condition for everyone except astronauts in space. vidual, the task(s) the individual is performing, and the en-
Surface and visual conditions, however, may vary signifi- vironment in which the task(s) must be performed. These
cantly and may be stable or unstable. Unstable surface con- interactions are represented in Figure 22-1. Within the indi-
ditions might include the subway, a sandy beach, a gravel vidual, both sensory inputs and processing systems (left side
driveway, or an icy parking lot. Common unstable visual of figure) and motor planning and execution systems (right
conditions are experienced on mass transit, in crowds, or on side of figure) are critical. Both peripheral components
a boat. Rapid head movements may render even a stable (lower part of figure) and central components (upper part of
visual environment unusable for postural cues, and darkness figure) of the systems are involved in the cycle. The cycle is
may preclude the use of vision. The more stable the environ- driven both by purposeful choices of the individual (tasks)
ment, the lower the demand on the individual for balance and by demands placed on the individual by the environ-
control. Unstable environments place greater demands on ment. Successful function of the sensory systems allows
the postural control systems. recognition of body position and motion in relation to self
Balance is also affected by an individual’s intentions to and the world. The desired outcome from the motor systems
achieve certain goals and the purposeful tasks that are un- is the generation of movement sufficient to maintain balance
dertaken. Volitional balance disturbances are self-initiated and perform the chosen, goal-directed task(s).
CHAPTER 22 n Balance and Vestibular Dysfunction 655
Figure 22-1 n The systems model of postural control illustrates the constant cycle that occurs simul-
taneously at many levels. (Reprinted with permission from NeuroCom International, Clackamas, Ore.)
Peripheral Sensory Reception of the head. The position of the head in relation to gravity is
The three primary peripheral sensory inputs contributing to detected through the otolith system. Horizontal and vertical
postural control are the bilateral receptors of the somatosen- accelerations, as in riding in a car or an elevator, are also
sory, visual, and vestibular systems.4,10 Somatosensory re- detected by the otoliths.14 Movements of the head are de-
ceptors located in the joints, ligaments, muscles, and skin tected through the semicircular canals. Head movement
provide information about muscle length, stretch, tension, stimulates both sets of semicircular canals, so that the ves-
and contraction; pain, temperature, and pressure; and joint tibular nerve on one side becomes inhibited while the other
position. The feet, ankles, knees, hips, back, neck, and eye becomes excited. The vestibular system provides sensory
muscles all furnish useful information for balance mainte- redundancy in the information obtained from each separate
nance. Somatosensation is the dominant sense for upright vestibular apparatus. If the peripheral vestibular system is
postural control and is responsible for triggering automatic damaged on one side, the information can be captured by the
postural responses (APRs). Somatosensory loss significantly intact canals on the opposite side. The vestibular system is
impairs balance. Loss of peripheral somatosensation occurs critical for balance because it uniquely identifies self-motion
in clients with loss or disease of or injury to the peripheral as different from motion in the environment. Box 22-1
sensory receptors or afferent sensory nerves. Examples in- describes the sensory components of the vestibular system.
clude clients with diabetic neuropathy, peripheral vascular Vestibular loss also impairs balance. Loss of peripheral
disease, spinal cord injury, and amputation. vestibular inputs occurs in clients with disease of or injury
Visual receptors in the eyes perform dual tasks. Central to the peripheral sensory receptors or afferent cranial nerves.
(or foveal) vision allows environmental orientation, contrib- Examples include clients with head injury involving tempo-
uting to the perception of verticality and object motion, as ral bone damage, acoustic neuroma, benign positional
well as identification of the hazards and opportunities pre- vertigo (BPV), or Meniere disease. For a comprehensive
sented by the environment.10 For example, a kayaker may review of the vestibular system and vestibular disorders,
see rocks in a stream as a hazard to be avoided, whereas a please see the vestibular section of this chapter beginning on
hiker who wants to cross the stream may see the same rocks page 689.
as a welcome opportunity. Peripheral (or ambient) vision Orientation to the wider environment, primarily from vision,
detects the motion of the self in relation to the environment, allows feed-forward, or anticipatory, postural adjustments.
including head movements and postural sway. Peripheral Prior experience and high attentional capacity improve antici-
vision is largely subconscious, whereas central visual inputs patory postural adjustments significantly. Detection of head
tend to receive more conscious recognition.10 Both are nor- movement by the vestibular and cervical somatosensory sys-
mally used for postural control. Vision is critical for feed- tems and of body sway by somatosensory and peripheral visual
forward, or anticipatory, postural control in changing envi- systems provides feedback for APRs. Note that the better an-
ronments. This includes planning for functional movements ticipatory abilities become, the fewer balance errors occur.
such as reaching and grasping, and especially for successful Fewer balance errors mean fewer losses of balance and a re-
navigation during gait. Vision loss also impairs balance. duced need to produce APRs.
Loss of peripheral visual inputs occurs in clients with Disease of or damage to any of the peripheral sensory
disease of or injury to the eyes or afferent cranial nerves. receptors or afferent pathways impairs or removes the
Examples include clients with cataracts, macular degenera- detection capabilities of the system, rendering sensory in-
tion, glaucoma, or diabetic retinopathy. formation unavailable for use in postural control. Many
The vestibular system provides the central nervous sys- patients with neurological diagnoses have peripheral sen-
tem (CNS) with information about the position and motion sory impairments.
BOX 22-1 n SENSORY

COMPONENTS will increase in one vestibular organ and decrease propor-
OF THE VESTIBULAR SYSTEM LABYRINTHS tionately in the other. This is known as push-pull function,
and the information from each side is considered to “match.”
SEMICIRCULAR CANALS With the same example, if the eyes are open while the head
n Semicircular canals are ring-shaped, fluid-filled struc- moves, the rate of the visual flow will be equal and the direc-
tures containing hair cells that respond to fluid move- tion of the visual flow will be opposite to the rate and direc-
ment when the head moves. tion of information from the vestibular inputs. The muscles
n The canals are arranged so that when the head moves, on one side of the neck will shorten and on the other side will
the direction and velocity in each canal are compared stretch. The inputs from these three systems are congruent. If
with a mutually perpendicular canal on the opposite side both sides and all three systems provide compatible inputs,
of the head. the process of sensory organization is simplified.
n The brain uses the relative change in firing from each When changes in the environment occur, the relative
side to identify the direction of rotation. This provides availability, accuracy, and usefulness of information from
for sensory redundancy; the brain interprets motion by the three sensory systems may also change. Sensory organi-
comparing information from each side to calculate the zation also includes an adaptive process, called multisensory
velocity and direction of head turns. reweighting, that permits the CNS to prioritize the sources
n The orientation of the three semicircular canals on each of sensory information when environmental conditions
side corresponds to the directions of neck motion, flex- change.15,16 Available, accurate, and useful information is
ion and extension, right and left rotation, and right and “upweighted,” whereas unavailable, inaccurate, or less-
left side-bending. This relation supports the integration useful information is “downweighted.” For example, in dark
of vestibular inputs with somatosensory information environments, vision would be downweighted and somato-
from the cervical spine. sensory and vestibular information would be upweighted.
OTOLITHS This adaptive process is imperfect, however, and balance is
n Located in the vestibule or central component of the lab- not as well controlled when any sense must be down-
yrinth, the otoliths respond to gravity and linear acceler- weighted as it is when all three senses are available and
ation through hair cell deflection. accurate. Individuals with peripheral sensory loss or central
nThese compartments contain multiple hair cells attached to the sensory processing deficits may have difficulty reweighting
walls and connected to the vestibular nerve. These hair cells quickly and fully. This impairs their ability to adapt to, and
are embedded in a gel-like substance called the macula. remain stable in, changing environments.17
n Sitting on top of the macula are crystals of calcium car- Sensory conflict can arise when information between
bonate, known as otoconia. The purpose of the otoconia sides or between systems is not synchronous. Sensory orga-
is to establish mass so that the hair cells can measure the nization processing then becomes more complex because the
effects of gravity as well as movement. brain must then recognize any discrepancies and select the
n The otoliths respond to movement of the head by the correct inputs on which to base motor responses. The ves-
shearing effect of the otoconia pressing on the macula tibular system may be used as an internal reference to deter-
and displacing the hair cell in the opposite direction of mine accuracy of the other two senses when they conflict.
the movement. These signals from the otoliths are used For example, a driver stopped at a red light suddenly hits the
to compare this motion with the resting tone established brake when an adjacent vehicle begins to roll. Movement of
by gravity. the other car detected by the peripheral visual system is mo-
mentarily misperceived as self-motion. In this situation, the
vestibular and somatosensory systems do not detect motion,
but the forward visual flow is interpreted as backward mo-
tion. Because the brain failed to suppress the (mismatched)
Central Sensory Perception visual inputs, the braking response was generated.
The brain processes all the environmentally available sen- When the brain recognizes that the information coming
sory information gathered by the peripheral receptors in from one sensory input is inaccurate or unavailable, as is the
varying degrees. This processing is usually referred to as case when somatosensory information is diminished post-
multisensory integration or sensory organization.4,10 Central stroke, it must depend more on the remaining senses (in this
sensory structures function first to compare available inputs case, vision and vestibular system) to determine position
between two sides and among three sensory systems. The and motion in space. The brain then compares and uses in-
somatosensory system alone is unable to distinguish surface formation from senses it considers accurate for balance. An
tilts from body tilts. Also, the visual system by itself cannot individual with the problem just described may compensate
discriminate movement of the environment from movement for the loss of somatosensory function by becoming visually
of the body.14 The vestibular system by itself cannot tell if dependent for balance during movement. If vision subse-
head movement through space is produced by neck motion quently also becomes disrupted as this client ages, his ability
or trunk and hip motion. Therefore the brain needs informa- to orient in space will be further compromised. This will
tion from all three senses to correctly distinguish self- impair balance and increase risk of falls.
motion from motion in the environment. Activities or environments that create sensory conflict or
How are sensory inputs from separate senses combined to demand sensory resolution become more difficult to manage
form perceptions of position and motion? For example, con- when the vestibular system is deficient or underused. These
sider the movement of turning your head to one side to look situations, such as going down stairs, riding escalators or
over your shoulder. When the head turns to one side, firing elevators, walking on uneven ground, and making quick
turns, are often avoided. When sensory conflicts cannot be The motor plan must be transmitted to the peripheral mo-
resolved rapidly, dizziness or motion sickness occurs. tor system to be enacted. A copy of the intended movement
Intrinsic central sensory processing impairments also can plan is sent to the cerebellum during the transmission. When
produce sensory conflict. An adult hemiplegic patient with the movement begins, incoming sensory inputs (feedback)
pusher syndrome illustrates an inability to integrate visual, about the actual movements and performance outcome are
vestibular, and somatosensory inputs for midline orienta- compared with the intended movements and performance
tion. Within a single system, discrepancies between the outcome. Movement errors (the difference between the in-
sides are also problematic. Unequal firing from opposite tended and the actual movement) and performance errors
sides of the vestibular system, as in unilateral vestibular (desired goal not achieved) are detected, and plans for cor-
hypofunction, produces a mismatch that is subsequently in- rection are then formed and transmitted. This process of
terpreted as head rotation when head movement does not error detection and error correction is the foundation of
occur. This spinning sensation is known as vertigo.14 Vertigo motor learning.
is resolved if the brain is able to adapt to the mismatch. For Clients with CNS disorders often have central motor
further information on vertigo, refer to the section on the planning and control system problems. After a stroke, cli-
vestibular system. ents may have hypertonus and poor reciprocal inhibition;
Finally, the central processing mechanisms combine any clients with head trauma may have difficulty initiating or
available and accurate inputs to answer the questions “Where ceasing movements; clients with Parkinson disease exhibit
am I?” and “How am I moving?” This includes both an in- bradykinesia; and those with cerebellar ataxia display mod-
ternal relation of the body segments to one another (e.g., ulation problems.22
head in relation to trunk, trunk in relation to feet) and an
external relation of the body to the outside world (e.g., Peripheral Motor Execution
feet in relation to surface, arm in relation to handrail). CNS Movement is accomplished through the bilateral joints and
disease or trauma involving the parietal lobe may impair muscles. Normal range of motion (ROM), strength, and en-
these processing mechanisms so that even available, accu- durance of the feet, ankles, knees, hips, back, neck, and eyes
rate sensory inputs are not recognized or incorporated into must be present for the execution of the full range of normal
determinations of position and movement.18,19 Impairments balance movements. Decreased ankle dorsiflexion ROM, for
of central sensory processing may occur after stroke, head example, restricts the forward limits of stability. Strength
trauma, tumors, or aneurysms; with disease processes such deficits are a primary cause of movement abnormalities in
as MS; and with aging. both central and peripheral nervous system disorders. In ad-
dition, weakness may be the result of force modulation defi-
Central Motor Planning and Control cits or disuse.11 Balance is directly affected by loss of
Whereas sensory processing allows the interaction of the strength. For example, weakness of the hip extensors and
individual and the environment, motor planning underlies abductors will impede successful use of a hip strategy for
the interaction of the individual and the task. Aside from upright trunk control. Initially adequate toe clearance may
reflexive activity such as breathing and blinking, most motor diminish with fatigue. Many clients with neurological issues
actions are voluntary and occur because some goal is to be also have stiffness and contractures as a result of persistent
achieved. That is not to say that reflexes occur separately weakness or hypertonus. Restrictions in ROM also limit bal-
from volitional movements; for example, the vestibuloocu- ance abilities.
lar reflex is active concurrently with visual tracking activity, The ability to achieve static postural alignment, although
but most actions occur because of some purposeful intent.14 necessary for normal balance, is not sufficient to allow voli-
These task intentions precede motor actions.10,20 Wrist and tional functions. Adequate strength (to control body weight
hand movements vary depending on what is to be grasped (a and any additional loads) through normal postural sway
cup versus a doorknob); foot placement and trunk position ranges is needed to permit dynamic balance activities such
vary depending on what is to be lifted (a heavy suitcase as reaching, leaning, and lifting. Postural control demands
versus a laundry basket). The initiation of volitional motor are increased during gait because the forces of momentum
actions depends on intention, attention, and motivation.10,21 and the interaction between recruitment, timing, and veloc-
Once an objective (“Where do I want to be?” “What do I ity also must be regulated.23 Traditionally considered ortho-
want to do?”) has been chosen, the next step in motor plan- pedic problems, deficits in strength, ROM, posture, and en-
ning is to determine how to best accomplish the goal given durance have a great impact on balance abilities. Attention
the many options that are potentially available. For example, must be given to these musculoskeletal system problems in
when the task demands fine skills or accuracy, the dominant examination of and intervention for clients with neurologi-
hand is preferred; when the task involves lifting a large or cal diagnoses.
heavy object, both hands are preferred. In addition to which
limbs, joints, and muscles will be used, motor planning also Influence of Other Systems
adjusts the timing, sequencing, and force modulation. This Balance abilities are also influenced by other systems. Atten-
can be demonstrated in various reaching tasks. Reaching to tion, cognition and judgment, and memory are critical for opti-
remove a hot item from the oven will occur slowly, whereas mal balance function and are often impaired in hemiplegic and
reaching to put an arm through a sleeve will occur more head-injured clients as well as those who have progressive
quickly. Optimal motor plans are developed with knowledge neurological disorders. Attentional deficits reduce awareness of
of self (abilities and limitations), knowledge of task (charac- environmental hazards and opportunities, interfering with an-
teristics of successful performance), and knowledge of the ticipatory postural control.12 When balance is threatened, an
environment (risks and opportunities).21 inability to allocate attention to the necessary task of balance
versus a secondary, less necessary task increases the risk of the body in a state of equilibrium.3,4 Functionally organized
falls. Cognitive problems such as distractibility, poor judgment, means that the responses, although stereotypical, are
and slowed processing also increase the risk of falls. Memory matched to the perturbing stimulus in direction and ampli-
loss may preclude recall of safety measures. Depression, emo- tude. If the stimulus is a push to the right, the response is a
tional lability, agitation, or denial of impairments also can in- shift to the left, toward midline. The larger the stimulus, the
crease the risks for loss of balance. In addition to having a direct greater the response. Automatic postural responses always
impact on balance abilities themselves, these cognitive and occur in response to an unexpected stimulus and are typi-
behavioral problems impede motor learning processes, which cally triggered by somatosensory inputs. Because they occur
are crucial for the relearning of balance skills. rapidly, in less than 250 ms, they are not under immediate
volitional control.
Constant Cyclic Nature Four automatic postural responses have been described.
The systems model of postural control previously presented Ankle strategy describes postural sway control from the
illustrates the constant cycle that simultaneously occurs at ankles and feet. The head and hips travel in the same direc-
many levels. Attention and intention allow feed-forward pro- tion at the same time, with the body moving as a unit over
cessing for active sensory search of the environment and motor the feet (Figure 22-2, A). Muscle contractile patterns are
planning, both of which are needed for anticipatory postural from distal to proximal (i.e., gastrocnemius, hamstrings,
control. Movements are initiated and executed with resultant paraspinals). This strategy is used when sway is small, slow,
sensory experiences and error detection, or feedback. Success- and near midline. It occurs when the surface is broad and
ful movements are repeated and refined; unsuccessful ones are stable enough to allow pressure against it to produce forces
modified. The nature of this cycle presents the clinician with that can counteract sway to stabilize the body. Ankle strat-
opportunities for intervention after the appropriate examina- egy is typically used to control anterior-posterior sway, be-
tion of sensory, motor, and cognitive functions. Through feed- cause most of the degrees of freedom at the ankle are in this
back and practice, balance abilities can improve.24 direction.
Hip strategy involves postural sway control from the
Motor Components of Balance pelvis and trunk. The head and hips travel in opposite direc-
tions, with body segment movements counteracting one an-
Reflexes other (Figure 22-2, B). Muscle contractile patterns are from
Many levels of neuromuscular control must be functioning proximal to distal (i.e., abdominals, quadriceps, tibialis an-
to produce normal postural movements. At the most basic terior). This strategy is observed when sway is large, fast,
level, reflexes and righting reactions support postural orien- and nearing the limit of stability or if the surface is too nar-
tation. The VOR and the vestibulospinal reflex (VSR) con- row or unstable to permit effective counterpressure of the
tribute to orientation of the eyes, head, and body to self and feet against the surface. Hip strategy is used to control both
environment.10 anterior-posterior and medial-lateral sway. Hip strategy in
When motion of the head is identified by the semicircular the medial-lateral direction involves weight shifts from foot
canals, it triggers a response within the oculomotor system to foot; any client with difficulty weight-shifting quickly
called the vestibuloocular reflex. This causes the eyes to and accurately will have difficulty with medial-lateral hip
move in the opposite direction of the head but at the same strategy.
speed. Stimulation of the otoliths drives the eyes to respond Suspensory strategy involves a lowering of the COG to-
to linear head movement. Quick movements of the head will ward the base of support by bilateral lower-extremity flexion
trigger the VOR.25 or a slight squatting motion (Figure 22-2, C). By shortening
The VOR allows the coordination of eye and head move- the distance between the COG and the base of support, the
ments. When the eyes are fixed on an object while the head task of controlling the COG is made easier. This strategy is
is moving, the VOR supports gaze stabilization. Visuo-ocular often used when a combination of stability and mobility is
responses often work concurrently with the VOR. They per- required, as in windsurfing.
mit “smooth pursuit” when the head is fixed while the eyes Stepping and reaching strategies involve steps with the
move and visual tracking when both the eyes and the head feet or reaches with the arms in an attempt to reestablish a
move simultaneously.10 new base of support with the active limb(s) when the COG
The VSR helps control movement and stabilize the body.
Both the semicircular canals and the otoliths activate and
modulate muscles of the neck, trunk, and extremities after
head movement to maintain balance. The VSR permits sta-
bility of the body when the head moves and is important for
the coordination of the trunk over the extremities in upright
postures. Righting reactions support the orientation of the
head in relation to the trunk and the head position relative to
gravity and include labyrinthine head righting, optical head
righting, and body-on-head righting.10
Automatic Postural Responses Figure 22-2 n Automatic postural responses. A, Ankle strategy.
At the next level, automatic postural responses operate to B, Hip strategy. C, Suspensory strategy. D, Stepping strategy.
keep the COG over the base of support. They are a set of (From Hasson S: Clinical exercise physiology, St Louis, 1994,
functionally organized, long-loop responses that act to keep Mosby.)
has exceeded the original base of support (Figure 22-2, D). and instructions permit a stepping response, stepping strategy
A successful stepping strategy is the best way to avoid a fall may be preferred. Individuals who are fearful of falling often
after a slip or trip. perceive even slight body sway as threatening instability. They
Misconceptions about these APR strategies are common. may use stepping and reaching strategies exclusively whether
First, these strategies do not function in daily life as sepa- or not these “rescue” strategies are actually necessary.
rately as they are described in the early research literature.
In quiet standing, for example, frequency analysis of unper- Anticipatory Postural Adjustments
turbed postural sway in healthy adults reveals that both Anticipatory postural adjustments are similar to automatic
ankle and hip strategies occur in combination, simultane- postural responses, but they occur before the actual distur-
ously.26 In perturbation studies, mixed use of strategies is bance.20 If a balance disturbance is predicted, the body will
often seen unless the perturbation is clearly below or above respond in advance by developing a “postural set” to counter-
certain-sized thresholds. Second, these strategies occur in act the coming forces. For example, if an individual lifts an
response to disturbances from all directions, not just in empty suitcase thinking it is full and heavy, the anticipatory
pure anterior-posterior or medial-lateral directions.27 Third, forces generated before the lift (to counter the anticipated
although these strategies are stereotypical in humans, great weight) will cause excessive movement and brief instability.
individual variation in strategy selection and performance Failure to produce these anticipatory adjustments increases
comes from other influential factors. For example, many the risk of sudden balance loss, creating the need to use rapid,
people use stepping strategy for most perturbations unless reactive automatic postural responses to prevent a fall. For
specifically instructed not to step or unless the conditions do clients with deficits in reaction time or automatic postural
not permit a step. An anxious person may reach or step responses, superior use of anticipatory postural control can
much sooner than a relaxed person with similar physical help the client avoid the unexpected perturbations that make
deficits. Last, all these strategies do not occur in sequence automatic postural responses necessary.
with every balance disturbance.28,29 In other words, individ- In balance laboratories, anticipatory postural adjustments
uals normally do not try ankle strategy and wait until it fails are studied using electromyography so that muscle activity
before trying hip strategy, then wait until it fails before try- before observable movement can be measured. In the clinic,
ing stepping strategy (although early learning may involve problems with anticipatory adjustments may be observed
such exploration). Because these responses must occur ex- when the client fails to counteract a predicted disturbance,
tremely rapidly to prevent balance loss, such a sequential such as “don’t let me push you backward,” or fails to inte-
approach would be inefficient and ineffective. Instead, the grate postural control tasks during other activities, such as
normal response is the emergence of the single strategy best the inability to step smoothly over an anticipated obstacle
suited to the particular perturbation, the limitations of the during gait or inability to maintain sitting balance when both
individual, and the conditions in the environment. arms are intentionally lifted overhead.
Abnormal use of automatic postural responses is often
observed in individuals with neurological disorders. Clients Volitional Postural Movements
with vestibular deficits typically rely on ankle strategy, Volitional postural movements are under conscious control.
which permits the head to remain aligned with the body and Weight shifts to allow an individual to reach the telephone
sustains congruence between vestibular and somatosensory or put the dishes in the dishwasher, for example, are self-
inputs. Use of hip strategy may be modified or limited be- initiated disturbances of the COG to accomplish a goal.
cause when the head is moving in the opposite direction as Volitional postural movements can range from simple weight
the COG, vestibular and somatosensory inputs are not con- shifts to complex balance skills of skaters and gymnasts.
gruent. Activities that require use of hip strategy, such as They can occur after a stimulus or be self-initiated. Volitional
standing in tandem or on one leg, can be a problem for cli- postural movements can occur quickly or slowly, depending
ents with bilateral vestibular loss or an uncompensated ves- on the goal at hand. The more complex or unfamiliar the task,
tibular lesion. However, some cases involve excessive use of the slower the response time. Use of a variety of movements
hip strategy on a level surface (when an ankle strategy that might successfully achieve a goal is possible. Volitional
would suffice).30 This may reflect abnormal integration of postural movements are strongly modified by prior experi-
the somatosensory and vestibular information. If peripheral ence and instruction. Automatic and anticipatory postural
somatosensation is impaired, as in diabetic neuropathy, or responses allow the continuous unconscious control of bal-
central sensory weighting of somatosensory inputs is inad- ance, whereas volitional postural movements permit con-
equate, hip strategy may dominate. scious activity. This level of postural motor control is
Clients with somatosensory loss, distal lower extremity the most frequently tested and treated in clinical practice,
weakness or hypertonus, restricted ankle ROM, and/or re- but it is by no means sufficient by itself to produce normal
duced limits of stability typically rely on hip strategy. This balance.
occurs because the client cannot feel the surface or the feet
well enough to modulate foot pressure against the surface, CLINICAL ASSESSMENT OF BALANCE
because the person cannot generate sufficient force against
the surface with the ankle muscles, or because restricted Objectives of Testing
ankle ROM prevents COG sway. The use of hip strategy is When present, activity limitations need to be identified and
normal when the COG is at or near the limits of stability and measured. Functional scales are typically used to determine
a step is either not possible or not desired. the presence and severity of these limitations, not necessar-
When the hip or ankle strategy is not efficient enough to ily why those limitations exist. From these functional tests,
control the movement of the center of pressure, or if conditions decisions can be made about whether to treat and, if so, what
tasks need to be practiced. If treatment is indicated, clini- test result? Or, alternatively, because the Romberg test is
cians must make judgments about what to treat. Further performed with feet together, what effect would hip weak-
testing to identify and measure impairments is then neces- ness have on the ability to stand with a narrowed base of
sary to know what systems are involved. A comprehensive support? When using a test whose results may be altered by
evaluation of balance includes both functional and impair- problems in more than one system, any relevant system
ment tests.12 should be evaluated. If multiple system deficits exist, and
No single quick-and-easy test of balance can adequately they often do in clients with neurological conditions, then
cover the many multidimensional aspects of balance, al- use caution in making “commonly assumed” conclusions on
though many such tests have great value as screening tools. the basis of clinical test results.
However, a comprehensive test battery, called the Balance Because so many balance tests are available, several
Evaluation Systems Test (BESTest), based on the systems questions must be asked to determine whether a test is ap-
model has been developed that provides clinicians with a propriate for use.33 For what purpose and population was the
thorough examination at the impairment level (Figure 22-3).31 test designed? Can that test be used legitimately for a differ-
The BESTest takes more time to administer than, for exam- ent purpose or with a different population? Is it valid? Is it
ple, a single-leg stance test, but results from the BESTest give repeatable by different examiners or by the same examiner
the clinician a far more complete and accurate picture of the multiple times? Are results reliable? In what populations are
client’s balance impairments than any single-item test or they reliable? What is the threshold for this test—that is,
screening test can. Armed with these results, the clinician can how large must performance changes be before this test can
develop interventions specifically targeted to the impaired detect them? Are normative data available for comparison?
systems. For clients whose primary problems include imbal- These questions are being investigated but have not yet been
ance, the clinician’s investment of time to perform this com- answered for many of the clinical balance tests commonly
prehensive test battery yields a valuable outcome. A shorter used by therapists with the many different neurological
version of this test, the mini-BESTest, has subsequently been populations they treat. Some of the evidence already re-
published.32 It takes less time to administer but likewise pro- ported may be frustrating to clinicians. For example, the
vides a less complete picture of the client’s balance systems. Timed Up-and-Go test (TUG) predicts falls in community-
Specifically, it does not include any items from the biome- dwelling older adults but not in acute-care hospital popula-
chanical constraints or stabilities limits categories. Even so, tions.34,35 For several balance tests such as the Functional
it is superior to single-item tests or screening tests that are Reach Test, the Berg Balance Scale, and others, the cutoff
not based on the systems model and do not identify balance scores used for accurate prediction of falls in clients with
system impairments that should be addressed in the interven- Parkinson disease are different from the cutoff scores used
tion plan. in older adults without Parkinson disease.36 These examples
No single, simple test for balance is possible because bal- make it clear that clinicians must understand their clients
ance is such a complex sensorimotor process.33 Many rela- and the characteristics of the various balance tests in order
tively simple balance tests exist, but not all tests are appro- to select the most appropriate tests and interpret test results
priate for all clients. Different tests may be needed to answer for each client.
specific questions. For example, several good tests have
been developed to determine the risk of falls in elderly Types of Balance Tests
people. These would be insufficient to discern whether an Balance tests can be grouped or classified by type. Different
injured dancer can resume practice or an injured roofer is types of tests measure different facets of postural control
ready to return to work. Clinicians should understand the (Table 22-1). Quiet standing (static) refers to tests in which
advantages and limitations of different balance tests to be the client is standing and the movement goal is to hold still.
able to select appropriate evaluative tools. Disturbances to balance, called perturbations, may or may
In general, a balance test will not be useful unless it suffi- not be applied. Active standing (dynamic) tests also position
ciently challenges the postural control system being tested. the patient standing, but the movement goal involves volun-
Tests for stability (“static balance”) are appropriate for clients tary weight shifting. Sensory manipulation tests use altered
who are having difficulty simply finding midline or holding surface and visual conditions to determine how well the
still in sitting or standing. They are of much less value for CNS is using and reweighting sensory inputs for postural
clients with higher-level abilities. Conversely, single-leg stance control. Functional balance, mobility, and gait scales involve
tests or sensory tests with a foam surface may be far too dif- the performance of whole-body movement tasks, such as
ficult for clients with lower-level abilities to perform. sitting to standing, walking, and stepping over objects. A
A word of caution about interpreting test results is indi- few test batteries offer a combination of the preceding tests.
cated. Most clinical tests rely on observations of motor be- The BESTest is the most comprehensive test battery to date.
havior to arrive at some conclusion about what systems have Dual-task tests have been developed to examine the effect of
problems and how they affect movement. Abnormal motor concurrent activities and divided attention on balance and
behavior has many causes, and clinicians should be careful mobility performance. A commonly accepted test for sitting
before concluding that an observed behavior is caused by balance in adults is not yet available, although clients with
problems in a certain system. For example, the Romberg test neurological problems often need sitting balance retraining
is commonly assumed to test the use of vestibular inputs. Yet in early stages. Clinicians typically modify standing tests or
during the test, both somatosensory and vestibular inputs are pediatric sitting tests to assess sitting balance in adult clients
(normally) used for balance control. If balance control is with neurological conditions. For example, the Functional
deficient, is the vestibular system necessarily the culprit? Reach Test has been used to measure excursion in seated
Could somatosensory system deficits also result in a poor individuals with spinal cord injuries.37
I. Biomechanical 3. Ankle strength 4. Hip/trunk lateral 5. Sit on floor and
1. Base of support 2. CoM alignment
Constraints and ROM strength stand up
8. Functional
II. Stability 6. Sitting verticality 7. Functional
reach lateral
Limits/Verticality (left and right) reach forward
(left and right)
and lateral lean
(left and right)
III. Anticipatory 11. Stand on one

12. Alternate stair 13. Standing arm
Postural 9. Sit to stand 10. Rise to toes leg (left and
touching raise
Adjustments right)
14. In-place 15. In-place 16. Compensatory 17. Compensatory

IV. Postural 18. Compensatory
response, response, stepping correction, stepping correction,
Responses stepping
forward backward forward backward
correction,
lateral (left
and right)
CHAPTER 22
19. Sensory integration on balance (modified CTSIB). A: Stance on
V. Sensory
firm surface EO, B: stance on firm surface EC, C: stance on foam 20. Incline, EC
Orientation
EO, D: stance on foam EC
VI. Stability in 21. Gait, level 22. Change in gait 23. Walk with head 24. Walk with pivot 25. Step over 26. Timed “Get up 27. Timed “Get up
surface speed turns, horizontal turns obstacles & Go” Test & Go” Test with
n Balance and Vestibular Dysfunction

Gait
dual task
Figure 22-3 n Balance Evaluation Systems Test (BESTest) with modifications of both long and
short forms. Short form identified by 14 components shown in BOLD. CoM, Center of mass; CTSIB,
Clinical Test of Sensory Integration on Balance; EC, eyes closed; EO, eyes open; ROM, range of
motion. (Data from Horak FB, et al: Phys Ther 89:484–498, 2009.)
661
places hands on the hips or crosses the arms over the chest,
TABLE 22-1 n TYPES OF BALANCE TESTS then picks up one leg and holds it with the hip in neutral and
the knee flexed to 90 degrees. The lifted leg may not
TYPE TESTS be pressed into the stance leg. This test is scored with a
Quiet standing (with Romberg stopwatch. Five 30-second trials are performed for each
or without perturba- Sharpened Romberg or tandem leg (alternating legs), with a maximum possible score of
tion) Romberg 150 seconds per leg. Normal young subjects are able to
One-legged stance test (OLST) stand for 30 seconds, but this may not be a reasonable
Timed stance battery expectation for frail older clients.38
Postural sway In both the Romberg test and the OLST, problems in
Nudge or push sensory organization processes can be observed. To deter-
Postural Stress Test mine how much of the stability is achieved through visual
Motor Control Test stabilization, each test can be repeated with eyes closed. The
Active standing Functional Reach Test client with visual dependency for balance will often have an
Multi-Directional Reach Test immediate loss of balance when the eyes are closed. (Re-
Limits of stability member, visual dependency may be a sign of somatosensory
Sensory manipulation Sensory Organization Test (SOT) or vestibular loss, or both.) As noted earlier, the client with
Clinical Test of Sensory Interaction and somatosensory or vestibular loss may have difficulty pro-
Balance (CTSIB) ducing the hip strategy necessary to perform these tasks.
Vestibular Vertiginous positions A battery of timed stance tests has been developed by
Hallpike-Dix maneuver Bohannon and Leary.42 This set of tests varies the foot posi-
Nystagmus tion (apart, together, tandem, and single leg) and the avail-
Semicircular canal function ability of visual information (eyes open and closed) to pro-
Visual-vestibular interaction duce eight different combinations. Maintenance of balance
Visual acuity in each condition is timed for a maximum of 30 seconds; the
Oculomotor tests assigned score is the total number of seconds that balance
Fukuda Stepping Test could be maintained. The best possible score on this test is
Dizziness Handicap Inventory 240 seconds. This test is reliable, valid, and sensitive to
Functional scales Berg Balance Scale change over time.42
Timed Up-and-Go Test A related test is the Balance Error Scoring System, or
Tinetti Performance-Oriented Assess- BESS test, which was developed for use with athletes to
ment of Balance screen for concussion effects.43,44 The original BESS test in-
Tinetti Performance-Oriented Assess- volved three stance positions (double-leg stance with feet
ment of Gait together, single-leg stance, tandem stance) on two surfaces
Gait Assessment Rating Scale (GARS) (firm and foam), thus providing six conditions. The eyes are
Dynamic Gait Index closed in all conditions. Each trial is 20 seconds in duration.
Functional Gait Assessment The examiner observes and counts the number of balance
Combination test Fregly-Graybiel Ataxia Test Battery errors that are made in each condition. The six observed er-
batteries Fugl-Meyer Sensorimotor Assessment rors include hands lifted off waist; opening eyes; step,
of Balance Performance stumble, or balance loss; moving a hip past 30 degrees of
Dual task Stops walking when talking abduction; lifting the forefoot or heel; and remaining out of
Multiple Tasks Test the test position for more than 5 seconds. If more than one
error occurs at the same time, for example, opening eyes and
hands off waist, only one error is counted. When measured in
high-level young adults, the reliability of this test improved
Quiet Standing with the removal of the double-leg stance condition, which
The classic Romberg test was originally developed to “ex- produced few to no errors in this high-functioning popula-
amine the effect of posterior column disease upon upright tion, and the addition of three trials in each of the remaining
stance.”38 The client stands with feet parallel and together four conditions. The modified BESS test thus includes only
and then closes the eyes for 20 to 30 seconds. The examiner four conditions.45 This test has not been investigated for use
subjectively judges the amount of sway. Quantification of in traditional neurological rehabilitation populations.
sway can be accomplished with a videotape, forceplate, or, Objective postural sway measures can be obtained by
more recently, accelerometer.39,40 Excessive sway, loss of computerized force plates (Figure 22-4) or wearable acceler-
balance, or stepping during this test is abnormal. The sharp- ometers (Figure 22-5).40,46-48 The client is asked to adopt a
ened Romberg,38 also known as the tandem Romberg, re- standardized foot placement if possible (this varies by manu-
quires the client to stand with feet in a heel-to-toe position facturer) and to stand quietly with arms at the sides or hands
and arms folded across the chest, eyes closed for 60 seconds. on hips for 20 or 30 seconds. Sway with both eyes open and
Often four trials of this test are timed with a stopwatch, for eyes closed is commonly measured. Graphic and numerical
a maximum score of 240 seconds. quantification is provided. Normative data may be provided.
One-legged stance tests (OLSTs) are commonly used.38,41 These more technical measures are able to detect more subtle
Both legs must be alternately tested, and differences be- problems and are more sensitive to change in performance
tween sides are noted. The client stands on both feet and after treatment than are rating scales or timed measures.
Eyes open Eyes closed Automatic postural responses are assessed by the client’s
response to perturbations. It is imperative that clinicians in-
clude APR testing in their balance assessment because APRs
are the motor responses necessary to prevent loss of balance
and falls. The push-and-release test is a clinically useful
method with a five-point ordinal rating scale.31,49,50 Testing
for ankle and hip strategies (“in-place” strategies) requires
Target Target that the clinician (1) place his or her hands on the front or
sway sway back of the patient’s shoulders, (2) ask the patient to remain
(% max area) (% max area) still and centered by resisting the pressure applied by the
0.05 0.10
hands (producing isometric muscle activity), (3) watch for
the toes or heels to begin to raise slightly (the clinician in-
Eyes open Eyes closed creases pressure until this occurs), then (4) suddenly release
the push. Both forward and backward directions are tested;
the clinician always stands where he or she can support the
client in case of balance loss. Testing for stepping strategy
follows the same concept but is performed differently. In-
stead of keeping the client’s COG at midline, the client leans
his or her weight into the clinician’s hands, shifting the COG
Target Target away from midline toward the outer limit of stability before
sway sway the release. The correct client response is to step to reestab-
(% max area) (% max area) lish a new base of support underneath the new position of the
0.48 2.20
COG. Forward, backward, and both lateral directions are
Figure 22-4 n Graphic and numerical postural sway measures us- tested. When nudge or push tests are performed predictably
ing a computerized force plate system. Top left, Normal subject, eyes (i.e., “don’t let me push you backward”), this is assessment
open. Top right, Healthy subject, eyes closed. Bottom left, Client with of anticipatory postural control. When the release happens
Parkinson disease, eyes open. Bottom right, Client with Parkinson unpredictably (no cues, unpredictable timing), automatic
disease, eyes closed. (Reprinted with permission from NeuroCom postural responses can be assessed. Perturbations of different
International, Clackamas, Ore.) strengths from multiple different directions should be given.
A B
C
Figure 22-5 n A, A wearable accelerometer for motion detection that can be used to measure
postural sway and other physical motions. B, The accelerometer worn in a belt at the L5-S1 level.
C, Postural sway data recorded by the accelerometer. (Photographs courtesy McRoberts B, The Hague,
The Netherlands.)
A B
Figure 22-6 n Surface perturbations during (A) the adaptation
test and (B) the motor control test using computerized dynamic
posturography. Force plate measures include latency and amount
of response and adaptation of the response to repeated perturba-
tions. (From Hasson S: Clinical exercise physiology, St Louis,
1994, Mosby.)
The Motor Control Test (MCT) is a computerized test of

automatic postural responses that perturbs the client through
surface displacement (Figure 22-6, B).3 The client stands on
a dynamic (movable) forceplate with feet parallel and arms
at sides. The support surface rapidly translates (slides) for-
ward or backward. This surface displacement results in a
rapid shift in the relation between the COG and the base of
support. The expected responses are directionally specific
(to the direction of the stimulus) forces generated against the
surface to bring the COG back to the center. Response laten-
cies, strength, and symmetry are measured. Normative data
are available. This test can be used to look for abnormal
stepping strategies when failure to select hip strategy occurs.
The MCT is the most standardized and reliable test of auto-
matic postural responses, but it is not widely used because it
requires computerized equipment.
Active Standing
Volitional control of the COG is evaluated by asking the
client to make voluntary movements that require weight Figure 22-7 n During the Functional Reach Test, the client is
shifting. The Functional Reach Test was developed for use asked to reach forward as far as possible from a comfortable stand-
with older adults to determine risk of falls.51 The client ing posture. The excursion of the arm from start to finish is
stands near a wall with feet parallel. Attached to the wall at measured by a yardstick affixed to the wall at shoulder height.
shoulder height is a yardstick. The client is asked to make a A, Functional reach, starting position. B, Functional reach, ending
fist and raise the arm nearest the wall to 90 degrees of shoul- position.
der flexion. The examiner notes the position of the fist on the
yardstick. The client is then asked to lean forward as far as
possible, and the examiner notes the end position of the fist four directions.55 The multidirectional reach test is concep-
on the yardstick (Figure 22-7). Beginning position is sub- tually equivalent but measures sway anteriorly, posteriorly,
tracted from end position to obtain a change unit in inches. and laterally to both sides. This test should provide a more
Three trials are performed. Normative data are available, comprehensive picture of volitional COG control limita-
and the test is reliable. However, the standard error of mea- tions. Validity and mean values have been established for
surement for this test may be as high as 2 inches, meaning community-dwelling older adults.56
that a change in score of less than 2 inches cannot be attrib- The limits of stability test uses a computerized forceplate
uted to clinical improvement because it may reflect only to measure postural sway away from midline in eight direc-
measurement error. Subsequent studies have not shown that tions.46,57 Clients assume a standardized foot position and
this test is useful for fall prediction.52-54 control a cursor on the computer monitor by shifting their
One serious limitation of the Functional Reach Test is weight. They are asked to move the cursor from midline to
that it measures sway in only one direction (forward). An eight targets on the screen (Figure 22-8). Measures include
expansion of this test has been devised to measure sway in movement velocity, directional control (path sway), measures
A B C
Figure 22-8 n Graphic postural sway measures from the limits of stability test using a computer-
ized force plate system (numerical measures not shown). Clients are asked to move away from and
return to midline. A, Subject with normal postural sway. B, Hemiplegic client on initial evaluation.
C, Hemiplegic client on discharge evaluation. (Reprinted with permission from NeuroCom Interna-
tional, Clackamas, Ore.)
of excursion (length of the trajectory of the COG), and reac- with the other leg. The original SEBT included eight di-
tion time. This test should be performed once for familiariza- rections; currently the SEBT is typically performed in
tion, then a second time for scoring purposes. Second and three directions: center-forward, right-rear, and left-rear.60
subsequent tests are reliable. Normative data are available. Three tape measures are taped to the floor, radiating out
A very challenging test used primarily in athletic from the same center point. The two rear tape measures
populations is the Star Excursion Balance Test (SEBT) are at a 45-degree angle from the center line. The client
(Figure 22-9).58,59 The SEBT could be used, for example, stands on one foot with the great toe on the center point,
in high-level traumatic brain injury (TBI) clients who re- then reaches the maximum distance away from the center
quire more demanding test conditions. However, although with the lifted foot. The distance is recorded by the exam-
there is evidence for the validity and reliability of this test iner. This is done in all three directions, with the lifted leg
in orthopedic populations, as yet this test has not been having to cross behind the stance leg to reach to the oppo-
investigated for use in neurological populations. The site-side rear tape. Six practice trials in each direction are
SEBT is in concept a lower-extremity functional reach given before recording scores to eliminate a learning
test, requiring single-leg stance on one leg and a reach effect, although recent evidence suggests four practice tri-
als may be sufficient.61 Three scored trials in each direc-
tion are performed. Both legs are tested.61a
Anterior Sensory Manipulation

Sensory inputs play a critical role in postural control, but few
Anteromedial Anterolateral tests to measure their use to produce a balance performance
outcome have been developed. The Sensory Organization
Test (SOT) uses a computerized, movable forceplate and
movable visual surround to alter the surface and visual envi-
ronments systematically.3,4 The client stands on the force-
plate with feet parallel and arms at the sides and is asked to
Medial Lateral
stand quietly. Three 20-second trials under each of six sen-
sory conditions are performed (Figure 22-10). In conditions
one, two, and three the support surface (forceplate) is fixed.
During conditions four, five, and six the support surface is
sway referenced to the sway of the client. In other words, the
movement of the surface is matched to the movement of the
Posteromedial Posterolateral
client in a 1:1 ratio. This responsive surface movement main-
tains a near-constant ankle joint angle despite body sway,
rendering the somatosensory information from the feet and
Posterior ankles inaccurate for use in balance maintenance. Visual in-
Figure 22-9 n The floor grid layout for the original Star Excur- puts are undisturbed in conditions one and four. Vision is
sion Balance Test (SEBT) with eight directions. Bolded arrows in absent (eyes are closed) in conditions two and five. The mov-
the anterior, posteromedial, and posterolateral directions indicate able visual surround is sway referenced in conditions three
the three directions used in the modified SEBT. (From Brumitt J: and six. This responsive visual surround movement main-
Assessing athletic balance with the Star Excursion Balance Test. tains a near-constant distance between the eyes and the visual
NSCA Perform Train J 7:6, 2008.) environment despite body sway, rendering visual inputs from
Under condition five, the eyes are closed (visual cues are
absent) and the support surface is sway referenced (somato-
sensory cues are inaccurate), leaving the vestibular inputs as
the only remaining sense that is both available and accurate.
Comparison of sway during condition five with sway during
condition one indicates how well the client is using vestibular
inputs for balance control. Clients with vestibular loss caused
by head injury, MS, or acoustic neuroma may have difficulty
with condition five. Many elderly clients also may be unsta-
ble in this condition. Functional situations in which these
clients may be at risk for falls would have both inadequate
lighting and compliant or unsteady surfaces (e.g., walking on
a gravel driveway or thick carpet in the dark).
Under both conditions three and six, the visual surround
is sway referenced (visual cues are available but inaccurate).
By comparing sway during these two conditions with sway
in the absence of vision (conditions two and five, with eyes
closed), determining how well the client can recognize and
subsequently suppress inaccurate visual inputs when they
conflict with somatosensory and vestibular cues is possible.
Some clients with CNS lesions (e.g., head injury, stroke,
tumor) may have difficulty with this condition. Clients who
cannot recognize and ignore inaccurate visual cues cannot
distinguish whether they are moving or the environment is
moving. If they perceive that they are moving (away from
Figure 22-10 n The six Sensory Organization Test conditions. midline) when they are not, they may often actively generate
The Sensory Organization Test determines the relative reliance on postural responses to “right” themselves. These responses,
visual, vestibular, and somatosensory inputs for postural control invoked to bring the COG to midline, then result in move-
using computerized dynamic posturography. (From Hasson S: ment away from the midline. The inaccurate perception
Clinical exercise physiology, St Louis, 1994, Mosby.) leads to a self-initiated loss of balance. Functional situations
that correlate with this test condition include public trans-
portation, grocery and library aisles, and moving walkways.
The SOT is valid and reliable in the absence of motoric
the eyes inaccurate for balance maintenance in those two problems, which increase sway for reasons unrelated to sen-
conditions. sory reception and perception. Normative data are available.
Under condition one, all three senses (vision, vestibular The Clinical Test of Sensory Interaction on Balance
sense, and somatosensory sense) are available and accu- (CTSIB) is a clinical version of the SOT that does not use
rate. Body sway is measured by the forceplate; this initial computerized forceplate technology.62 The concept of the
measurement forms the baseline against which subsequent six conditions remains intact (Figure 22-12). Instead of
measures are compared (Figure 22-11). Under condition sway measures, the examiner uses a stopwatch and visual
two the eyes are closed, so only somatosensory and ves- observation. A thick foam pad substitutes for the moving
tibular cues remain. In an individual with normal move- forceplate during conditions four, five, and six. In normal
ment function, the somatosensory inputs will dominate in individuals and clients with peripheral vestibular lesions,
this condition. By comparing sway during condition two measures with foam correlate to moving forceplate mea-
with sway during condition one, detection of how well the sures.63 Originally, a modified Japanese lantern substituted
client is using somatosensory inputs for balance control is for the moving visual surround in conditions three and six.
possible. Clients with somatosensory loss from spinal cord Studies have not shown that measures using the Japanese
injury, diabetes, or amputation have difficulty in condition lantern correlate with the moving visual surround measures.
two. Functional situations with inadequate lighting or Most clinicians now perform the modified CTSIB with just
unusable visual cues (e.g., busy carpeting) are similar to four conditions, eyes open and closed on a firm surface and
condition two. eyes open and closed on the foam surface. The client is
Under condition four, the support surface is sway refer- asked to stand with feet parallel and arms at sides or hands
enced (somatosensory cues are available but are inaccurate), on hips. At least three and up to five 30-second trials of each
so only visual and vestibular cues remain useful. In a normal condition are performed.18 The watch is stopped if the client
client the visual inputs will dominate in this condition. Com- steps, reaches, or falls during the 30 seconds. If the client is
paring sway during condition four with sway during condi- very steady for 30 seconds on the first trial of a condition,
tion one indicates how well the client is using visual inputs some clinicians choose not to test the remaining trials in that
for balance control. Clients with visual loss caused by dia- condition and will give the client a full score for that condi-
betes, cataracts, or field loss have difficulty in condition tion. A maximum score for five trials of each condition is
four. Functional situations that correlate with condition four 150 seconds. Individuals with normal movement abilities
include compliant surfaces (beach, soft ground, gravel drive- are able to stand without stepping, reaching, or exhibiting
way) and unstable surfaces (boat deck, slipping throw rug). loss of balance for 30 seconds per trial per condition. It is
Figure 22-11 n Postural sway measures from each of the six Sensory Organization Test condi-
tions are compared, and the ratios are used to identify impairments in the use of sensory inputs for
postural control. (From Jacobson GP, Newman CW, Kartush JM: Handbook of balance function
testing, St Louis, 1993, Mosby.)
normal for sway to increase slightly as the conditions in- stand with both feet in the right-rear quadrant, steps leftward
crease in difficulty. The CTSIB may not be a reliable mea- over the fourth bar to stand with both feet in the left-rear
sure in clients with hemiplegia or other conditions that in- quadrant (starting location), then reverses direction, going
volve motor deficits in, or abnormal response time through, back through each quadrant in the same way until standing
the lower extremities and trunk.64 The clinician can use the again with both feet in the rear-left quadrant. The outcome
information regarding client response in a variety of envi- measure is the time it takes the client to perform this task cor-
ronmental conditions to determine intervention management rectly, clearing each bar completely with each foot. This test
strategies.65 has been used with older adults, individuals with vestibular
disorders, and clients poststroke.67,68
Vestibular System Tests
Please refer to the vestibular section for a thorough presenta- Functional Scales
tion of vestibular disorders and their management. A comprehensive balance evaluation must include both
impairment-based tests of body systems and activity-based
Active Stepping functional measures. Functional scales help address the ac-
The ability to change the base of support without balance loss tivity limitations. By asking the client to perform functional
then to reestablish COG stability over the new base of support tasks that demand balance skills, the clinician can determine
is a balance-dependent skill critical for functional activities. the presence of activity limitations that will affect the indi-
The four square step test is a timed stepping test in a standard- vidual’s ability to participate in life, and identify the tasks
ized, structured format in forward, backward, and lateral di- that the client needs to practice. Three mobility scales and
rections (Figure 22-13).66 A simple plus sign–shaped grid is three gait scales focus on postural control; five of these were
laid out on the floor using four straight canes, dowel rods, or developed for the elderly population to determine risk of
plastic piping. This creates four quadrants. The client begins falls. Many clinicians are also using them to assess clients
standing in the rear-left quadrant, steps forward over the first with neurological conditions, although their usefulness with
bar to stand with both feet in the front-left quadrant, steps neurological populations is less well-documented. Far fewer
rightward over the second bar to stand with both feet in the standardized tests for high-level balance skills have been
right-front quadrant, steps backward over the third bar to developed; some clinicians adapt tests used by athletes, but
Figure 22-12 n The Clinical Test of Sensory Interactions on Balance uses foam and a Japanese
lantern to replicate the six sensory conditions. A stopwatch is used to time trials.
these are often too difficult for many neurologically in-

volved clients.
Start
The Berg Balance Scale consists of 14 tasks that the cli-
Finish ent is asked to perform.69 The examiner rates the client on
each task by using an ordinal rating scale of 0 to 4, in which
0 is unable to perform and 4 is able to perform without dif-
ficulty. This test is highly reliable. It was originally designed
for assessing risk of falls in older adults, and cutoff scores
1 2 for fall risk vary depending on which of several studies is
consulted.70,71 Use of higher cutoff scores may erroneously
identify nonfallers as fallers; use of lower cutoff scores may
4 3 erroneously identify fallers as nonfallers. The Berg Balance
Scale has also been used with clients after stroke.72-75
The original Get-Up-and-Go Test is made up of seven
items and subjectively scored on an ordinal rating scale of 1
to 5, in which 1 is normal and 5 is severely abnormal.76 This
test has been modified by making it a timed measure to in-
crease its objectivity and reliability, which is now high. The
TUG test eliminates the “standing steady” segment and uses
a stopwatch to time the performance.77 Clients are asked to
rise from a chair with arms, walk 3 meters as fast as they
Figure 22-13 n The floor grid for the timed Four Square Step safely can, turn, walk back to the chair, and sit down. This
Test (FSST). Arrows indicate the direction of the steps. (From Dite test may be performed with an assistive device; however, the
W, Temple VA: A clinical test of stepping and change of direction use of a device will alter the speed at which the task can be
to identify multiple falling older adults. Arch Phys Med Rehabil accomplished, and any retesting must be done with the same
83:1568, 2002.) device to produce comparable results. Originally designed
to assess frailty in older adults, the test is now more com- falls. When combined, the Tinetti POMA balance and gait
monly used to assess fall risk in this population. Young scales offer a best possible score of 28, with scores of 19 or
adults typically perform this task in 5 to 7 seconds, healthy less indicating a high fall risk.
older adults in 7 to 9 seconds (low risk), moderate-risk older The original Gait Assessment Rating Scale (GARS) is a
adults in 10 to 12 seconds, and high-risk older adults in list of 16 abnormal aspects of gait observed by the examiner
13 seconds or more.78,79 These cut-off scores are for older as the client walks at a self-selected pace (see Table 22-3).82
adults walking without assistive devices. Improvements in These abnormalities are commonly seen in older adults who
test performance that are not captured by the time score fall, who are fearful of falling, or both. The items are scored
alone should also be documented, for example, if the client on a scale of 0 to 3, with lower numbers reflecting better
can now perform the test without the use of chair arms to (less abnormal) performance. The best possible score is 0.
stand up, or without an assistive device. This gait scale provides some relative numerical indication
The Tinetti Performance-Oriented Mobility Assessment— of the quality of gait. A shorter, modified version of this test,
Balance subscale (POMA-Balance) is a list of nine items the Modified GARS, has been developed. The Modified
scored on scales of either 0 to 1 or 0 to 2, with the higher GARS (GARS-M) includes nine of the original items plus a
numbers reflecting better (more normal) performance.80 gait velocity measure. It provides equivalent sensitivity and
The score value is specific to the item. The best possible score takes less time to perform.83 These two gait scales were de-
is 16, with a score of 10 or lower indicating a high risk veloped to assess risk of falls in older adults.
of falls.81 The Dynamic Gait Index (DGI) is a gait test specifically
Most balance and mobility scales have been developed to designed to look at postural control during gait.12 It includes
assess risk of falls in older adults. Many share similar items. eight items requiring changes in gait speed, walking with
See Table 22-2 for a summary of scale items. horizontal and vertical head turning, whole-body turns dur-
The Tinetti Performance-Oriented Mobility Assessment— ing gait, stepping over and around obstacles, and stair ascent
Gait subscale (POMA-Gait) is a list of seven normal aspects and descent. Items on this test are scored on a 4-point ordi-
of gait that are observed by the examiner as the client walks nal scale of 0 to 3, with 3 being normal performance and 0
at a self-selected pace and then at a rapid but safe pace.80 indicating severe impairment. The best possible score on
Scoring scales are again either 0 to 1 or 0 to 2, and higher this test is a 24, and scores of less than 19 points have been
numbers indicate better performance. Score values are spe- associated with impairment of gait and fall risk. The pres-
cific to the item being observed (Table 22-3). The best possi- ence of head motion and whole-body turns in this test may
ble score is a 12; scores of 8 or below indicate a high risk of help identify clients with potential vestibular dysfunction.
TABLE 22-2 n BALANCE AND MOBILITY SCALE ITEMS
BERG BALANCE DYNAMIC GAIT TIMED UP-AND-GO TINETTI BALANCE

ACTIVITY SCALE INDEX TEST ASSESSMENT
1. Sitting unsupported  
2. Sitting to standing   
3. Standing to sitting   
4. Transfers 
5. Standing unsupported   
6. Standing with eyes closed  
7. Standing with feet together 
8. Tandem standing 
9. Standing on one leg 
10. Rotating trunk while standing 
11. Retrieving object from floor 
12. Turning 360 degrees  
13. Stool stepping 
14. Reaching forward while standing 
15. Sternal nudge 
16. Walking  
17. Abrupt stop 
18. Walking then turning  
19. Stepping over obstacle 
20. Stairs 
21. Walking at preferred and varied 
speeds
22. Walking with horizontal and 
vertical head turns
23. Stepping around obstacles 
control system to permit a more complete assessment of the

TABLE 22-3 n GAIT SCALE ITEMS client’s balance abilities.
The BESTest is an excellent comprehensive test
GAIT ASSESSMENT battery based on the systems model of postural control (see
TINETTI RATING SCALE Figure 22-3).31 It includes seven categories representing
GAIT ACTIVITIES GAIT SCALE (GARS) components of postural control: biomechanical constraints;
1. Initiation (hesitancy)  † stability limits; anticipatory postural adjustments; automatic
2. Step length  † postural responses; sensory orientation, and stability during
3. Step height   gait. Within each category are individual test items and, in
4. Step symmetry   some cases, existing tests. For example, the sensory orienta-
5. Step continuity  † tion category contains the individual item “Stand on incline
6. Path deviation   with eyes closed” and the four-condition CTSIB discussed
7. Trunk   earlier. Although the BESTest takes approximately 30 minutes
8. Walking-heel  to administer, the information acquired helps to identify which
distance underlying components of the postural control system are
9. Staggering † causing the observed balance problems. Armed with this
10. Heel strike † critical information, the clinician can design a customized,
11. Hip ROM* † individualized intervention program that targets the sources of
12. Knee ROM*  imbalance in each client.
13. Elbow extension*  A shorter version of this test has been developed, the
14. Shoulder extension* † Mini-BESTest (see Figure 22-3).32 It includes 14 of the
15. Shoulder abduction*  original 36 items, has a compressed rating scale, and takes
16. Arm-heel strike † approximately 15 minutes to administer. It does not include
synchrony any items from the biomechanical constraints category or
17. Forward head*  the stability limits category. This does not mean that these
18. Shoulders held  components need not be measured. Biomechanical con-
elevated* straints such as hip and ankle weakness, and constricted
19. Forward flexed  limits of stability, seriously negatively affect balance and
trunk* should be tested in addition to administration of the Mini-
20. Gait speed † BESTest. The compressed rating scale may reduce the abil-
ROM, Range of motion.
ity of this test to reflect a client’s progress over time.
*
During gait. The Fregly-Graybiel Ataxia Test Battery is a more chal-
†
Items that were retained for the Modified GARS. lenging test appropriate for clients with higher-level balance
skills. It includes eight test items that the client must per-
form (Figure 22-14).38 Standing trials in tandem stance both
The reliability of this test is high.71,84,85 A modified and off and on a rail with eyes open and closed are timed. Timed
slightly more difficult version of this test, the Functional single-leg stance trials also are performed for each leg.
Gait Assessment (FGA), has been developed specifically for Walking 10 steps with eyes closed is included. Five trials of
use with patients with vestibular disorders.86 The best pos- each task are given. Trials are stopped if the client uncrosses
sible score on the FGA is 30 points, and a score of 22 points the arms, opens the eyes (during eyes-closed trials), steps
or below can be used to classify fall risk and predict unex- (during standing trials), or falls. Trials are judged on a pass-
plained falls in community-dwelling older adults.87 fail basis. This test battery is valid for use with clients who
The three gait tests listed previously are distinct from have peripheral vestibular dysfunction. Normative data are
traditional gait tests because they focus on elements of pos- available from a normative database composed primarily of
tural control during gait. One very important traditional gait findings in young men. As noted earlier, clients must be at a
measure that should be included in the assessment of bal- high level motorically to perform these tasks. This test is a
ance and gait in older adults and clients with neurological good choice for clients with higher-level abilities because it
disorders is gait speed. This measure has been termed “the does provide more demanding balance tasks. Interpretations
sixth vital sign” for older adults because of its strong asso- regarding a client’s use of sensory inputs when motor in-
ciation with level of dependence in activities of daily living volvement is also present cannot be made with certainty.
(ADLs) and instrumental ADLs (IADLs), probability of The Fugl-Meyer Sensorimotor Assessment of Balance
hospitalization, risk of falls, eventual discharge location, and Performance is a subset of the Fugl-Meyer Physical Perfor-
ambulation category.88 mance Battery, which was designed for use with hemiplegic
clients (Figure 22-15).18 Three sitting and four standing bal-
Combination Test Batteries ance activities are listed. The items are scored on a 0 to
Because no single test can give a complete picture of a cli- 2 scale, with score values specific to each item. Higher
ent’s balance abilities, individual test items are often com- scores indicate better performance; the maximum (best)
bined to form a test battery. Several of the tests described score is 14. However, a client could achieve this score of 14
earlier, such as the Berg Balance Scale and the Tinetti and still not have normal balance.
POMA, include a combination of multiple items and could The Dizziness Handicap Inventory (DHI) was developed to
be categorized as combination test batteries. Different items identify specific functional, emotional, or physical problems
on a test may challenge different components of the postural associated with an individual’s reaction to imbalance or
Figure 22-14 n A combination of tasks (Romberg test, one-legged stance test [OLST], walking)
and environments (eyes open, eyes closed, rail) are included in the Fregly-Graybiel Ataxia Test
Battery. (From Newton R: Review of tests of standing balance abilities. Brain Inj 3:335, 1989.)
Figure 22-15 n The Fugl-Meyer Sensorimotor Assessment of Balance Performance includes both
low-level and high-level tasks. (From DiFabio RP, Badke MB: Relationship of sensory organization
to balance function in patients with hemiplegia. Phys Ther 70:20, 1990.)
dizziness.89,90 The DHI assesses the client’s perception of the The DHI can be given before the initial evaluation to help
effects of the balance problem and the client’s level of emo- determine which physical tests should be performed. An
tional adjustment. It also looks at perceived physical limitations astute clinician can see patterns of dysfunction within the
as a consequence of the disorder. Twenty-five items are divided reported symptom level. For example, visual motion sensi-
into three subscales in this self-assessment inventory. Included tivity and visual dependency can be indicated from the an-
are a nine-item functional scale, a nine-item emotional scale, swers about grocery stores, crowds, riding in a car, or diffi-
and a seven-item physical scale. Each item is assigned a value culty at night. Imbalance usually is indicated when the client
of four points for a “yes,” two points for a “sometimes,” and has difficulty walking down a sidewalk and using stairs.
zero points for a “no.” This inventory is reliable, is easy to ad- Patients with chronic mild head injury often will report
minister, and can be used to evaluate treatment outcomes.91 many activities as most provoking because of their inability
Changes in scores on the functionally based DHI correlate to integrate the sensory systems and poor motor control for
highly with changes in scores on the impairment-based SOT.89 balance.
Dual-Task Tests are questionnaires that are easy to administer. The ABC Scale
In everyday life tasks, normal balance is largely unconscious consists of 16 items that range in difficulty from “walk around
and does not compete for attentional resources. In clients the house” to “walk outside on icy sidewalks.” Several of the
with balance disorders, however, the challenge of maintain- items inquire about activities in public places, for example,
ing postural control during upright activities and gait is often parking lots and escalators. Clients are asked how confident
sufficient to demand the use of attentional resources. The they are that they could do each of the activities without losing
interaction of cognitive demands and postural control de- their balance or becoming unsteady. Responses are given on a
mands is examined in dual-task tests that add concurrent scale from 0 to 100 in increments of 10, with higher numbers
cognitive and motor tasks to gait tasks. At the simplest level indicating higher confidence. More recently a short version,
are the walking while talking (WWT) and stops walking the ABC-6, has been developed. It has six of the original
when talking (SWWT) tests.92-95 In these tests the client is 16 items and takes less time to administer, yet retains good
asked to walk and, while the client is walking, the clinician reliability and correlation with balance and fall risk mea-
asks the client one or more questions and observes if the cli- sures.107 The FES consists of 10 activity items that are less
ent must stop walking to answer the question(s). If so, the difficult than the items on the ABC Scale. Items on the FES
test result is positive—that is, the client must stop attending include getting in and out of a chair and answering the door or
to the postural control demands of walking to reallocate a phone. All of the items refer to activities done in the home.
attention to the cognitive task. These are gross measures, apt Clients are asked how confident they are that they could do
to identify only those with more severe attentional balance each of the activities without falling. Responses are given on a
problems or to misidentify clients who prefer to chat and rest scale from 1 to 10, with lower numbers indicating higher con-
rather than keep walking. A more formalized dual-task test is fidence. The Modified FES (MFES) has 14 activity items and
the Multiple Tasks Test (MTT), which includes eight items includes two activities done outside the home and three activi-
involving gait plus other verbal cognitive and motor tasks ties done in public spaces. It also takes into account whether or
such as carrying a tray and avoiding obstacles.96,97 Two dual- not an assistive device is used.108 Scoring is identical to that of
task versions of the TUG have been developed. The TUG- the original FES.
Manual involves performing the TUG while carrying a cup A third measure of fear of falling is the Survey of Activi-
nearly full of water. The TUG-Cognitive involves performing ties and Fear of Falling in the Elderly (SAFFE).109 This
the TUG while subtracting backward from a randomly se- measurement instrument is more involved than the ABC
lected number or spelling words backward.98 The Walking Scale or MFES; however, it provides additional information
and Remembering Test (WART) requires the client to re- specific to activity restriction that is valuable to the clinician.
member a set of numbers that the tester speaks aloud while The SAFFE has 11 activity items that are similar in nature to
the client walks as quickly as possible while trying not to the items on the other two scales, including community ac-
step off of a narrow path.99 Once the walk is completed, the tivities. This questionnaire asks if the client actually does the
client must repeat the numbers in sequence. For all dual-task activity or not. If he or she does the activity, the questionnaire
tests, performance of each single task is measured separately asks how worried the client is that he or she might fall during
first. Then the dual-task performance is recorded. The differ- the activity, on a scale from 1 to 4. Lower numbers indicate
ence for each of the two scores (physical and cognitive per- increased worry. If the client does not do the activity, the cli-
formance) between undivided attention and divided attention ent is asked whether the reason he or she does not do the
conditions is calculated. activity is fear of falling, with degree of fear scored on the
scale from 1 to 4, or whether the client does not do the activ-
Balance Confidence Tests ity for reasons other than fear, and what those other reasons
Reduced participation in functional activities may occur not are. For each item, clients also indicate whether the fre-
only because balance impairments impede participation, but quency of doing the activity has increased, decreased, or re-
also when clients are anxious about falling. Fear of falling mained the same. The SAFFE takes longer to administer than
may lead individuals to avoid activities that they remain the ABC Scale or MFES but provides explicit results about
quite capable of doing.100 In turn, prolonged self-restriction activity restriction not obtained from the other two scales.
of activity leads to the many negative consequences of being The Fear of Falling Avoidance Behavior Questionnaire
sedentary—decreased ROM, weakness, low endurance, and (FFABQ) is a recently developed instrument with a focus on
so on—and thus ironically further impairs balance and inactivity avoidance versus fear.110 It lists 14 different activi-
creases fall risk.101,102 As this worsening balance and in- ties, ranging in difficulty from walking and preparing meals
creased risk is perceived by the client, further activity re- to going up and down stairs to engaging in recreational ac-
striction occurs, creating a self-perpetuating downward tivities such as sports or traveling. Clients rate whether or
spiral leading to social isolation, anxiety, and depres- not they agree with a statement that they avoid a specified
sion.103,104 It is just as important to address poor balance activity on a 5-point scale from 0 (completely disagree) to
confidence as it is to address poor balance, for without suf- 4 (completely agree). This questionnaire is reliable, with
ficient balance confidence a client will not participate in scores that discriminate between previous fallers and non-
activities even if balance abilities permit him or her to do so. fallers, and more versus less active individuals. The shift
The client will lose all the gains made in therapy if he or she from an emphasis on fear or confidence as in the ABC and
does not remain active, and he or she will not be active if FES, to activity avoidance as in the SAFFE and FFABQ is
fearful of falling. an important and positive one for physical and occupational
The two most commonly used measures of balance confi- therapists. As the ICF health and disablement model de-
dence are the Activities-specific Balance Confidence Scale scribes, our goals are to increase activity and participation to
(ABC Scale), and the Falls Efficacy Scale (FES).105,106 Both achieve an improved quality of life for our clients.
Considerations in the Selection of Balance In addition, additional tests are necessary to assess the
Tests systems that may affect postural control to help identify and
To determine the type and level of challenge of the tests to be measure impairments (e.g., ROM, strength, sensation and
used during the examination, a thorough subjective history is sensory organization, motor planning and control). These
critical. In describing the symptoms and the situations that types of measures should be sensitive, objective, and quan-
cause dizziness or imbalance, the client offers clues to possible tifiable. Unfortunately, some body system components do
deficits and thereby the measures that will help identify them. not have objective, quantifiable clinical measures (e.g., mo-
Many of the functional scales previously reviewed were tor planning, coordination). In these cases, clinicians must
designed to determine whether balance is abnormal in el- continue to use subjective rating scales.
derly clients who have no medical diagnosis, in other words, Other factors to include when deciding what tests to use
as screening tools. Clinicians working with clearly diag- are the time required to perform the test, the number of staff
nosed clients with neurological conditions often do not need members who must be present, and the space and equipment
such tools to establish that balance skills are abnormal be- needed. Clinicians must weigh the potential benefits of tech-
cause the deficits are patently obvious. These screening nological tools (e.g., computerized forceplates, isokinetics,
tools can be useful, however, to identify disabilities, estab- motion analysis, electromyography) against their cost and
lish a baseline, monitor progress, and document outcomes. practicality (i.e., their cost-effectiveness). The test must be
Many clinical facilities have their own therapy evaluation suitable for the client’s level of functioning (physical and
forms that include a section on balance. Items and scoring are cognitive). Many head-injured clients, for example, cannot
usually defined by the facility. They are not standardized initially participate in traditional forms of testing because of
across sites, as are published scales, and are rarely tested for cognitive limitations.
measurement qualities such as validity and reliability. As reha-
bilitation professions evolve toward evidence-based practice, PROBLEM IDENTIFICATION, GOAL SETTING,
nonstandardized tests with unknown measurement quality are AND TREATMENT PLANNING
no longer acceptable. Clinicians should use standardized, ob-
jective, quantifiable, valid tests with high reliability, sensitivity, Clinical Decision Making
and specificity whenever possible. Facilities insistent on using Treatment of clients with neurological diagnoses is based on
their own tests should conduct research to ensure that they are the particular set of impairments and activity limitations
valid, reliable, and responsive to change over time. A func- possessed by each individual. Remediation of balance defi-
tional balance rating scale is important in the evaluation of cits similarly must be specific to the involved body systems
clients with neurological impairment. To be responsive enough and functional activity losses in each client. Clinicians
to measure changes in clients who clearly are not (and may should generate an overall problem list for each client; if
never be) clinically normal, scales should have at least five, imbalance is a listed problem, then a sublist of balance prob-
and perhaps seven, possible relative scores. lems also can be developed (Figure 22-16).
Figure 22-16 n An example of a balance-specific problem list (as a subset of a general problem
list), which should be developed to guide balance rehabilitation treatments.
others with progressive diseases such as Parkinson disease

or MS, the rate of decline is unknown and abilities may
fluctuate. In these cases the clinician should consider the
following issues: Would a consultation provide the required
information? If so, referral is appropriate. Do any contrain-
dications to treatment exist? What are the risks and benefits
of providing versus withholding treatment? Is some amount
of functional improvement possible? If no contraindications
are present, the benefits outweigh the risks, and functional
improvement is expected, then a trial of treatment may be
given, even if knowing for certain whether the problem(s)
will respond to the treatment is not possible. In these cases
especially, a baseline must be established against which to
measure any change. Change for the worse or no change
after a reasonable trial period indicates that treatment should
be altered or discontinued.
Figure 22-17 n A clinical decision-making tree to illustrate the Using the Systems Model to Identify Postural
treatment-planning process in balance rehabilitation. Control Impairments
The systems approach is useful to develop a balance prob-
lem list because it can be applied to different diagnoses
To direct and establish priorities for treatment, clinicians equally well and allows deficits in multiple systems to be
must review the problem list and ask themselves the follow- recognized. Table 22-4 illustrates several examples of ways
ing questions (Figure 22-17): Which impairments are tem- this framework is used to identify balance deficits in clients
porary and can be remediated? How much improvement can with different neurological diagnoses.
be expected? How soon will it occur? Which impairments For each client, problems affecting postural control
are permanent or progressive and must be compensated for? should be described in objective, measurable terms when-
What other body systems can be counted on to substitute? ever possible. For example, the term “impaired vision” is
What external compensations may be needed? too vague; “four-line drop on eye chart” is more specific.
For some clients with neurological impairments, know- “Poor use of visual inputs for balance control” is an inter-
ing whether a problem is permanent or temporary is not pretation; the objective result could be stated “Loss of bal-
possible, as in recovery from a stroke or head injury. In ance after less than 15 seconds on 5/5 trials of standing on
TABLE 22-4 n EXAMPLES OF MEDICAL DIAGNOSES AND RELATED IMPAIRMENTS AFFECTING

BALANCE
IMPAIRMENTS FROM CLIENT WITH DIABETIC CLIENT WITH PARKINSON CLIENT WITH INCOMPLETE
SYSTEMS MODEL STROKE DISEASE PARAPLEGIA
PERIPHERAL SENSORY
Vision Retinopathy Cataracts
Vestibular Hair cell loss
Somatosensory Peripheral neuropathy Slowed transmission time Complete loss
CENTRAL SENSORY
Vision Hemianopia Vision dominant Needs superior use to compensate
Vestibular Failure to use inputs Needs superior use to compensate
Somatosensory Failure to use inputs Failure to use inputs
Strategy selection Step dominant Ankle dominant Hip dominant
Perception of position in space Midline shift with left neglect Restricted limits of stability
CENTRAL MOTOR
Timing Increased reaction time Bradykinesia
Sequencing Disordered Co-contraction
Force modulation Spasticity Rigidity
Error correction Use right side only
PERIPHERAL MOTOR
Range of motion Knee hyperextension Bilateral ankle plantar flexor Hip flexion contractures
contractures
Strength Decreased left side Decreased bilateral extremities Severe weakness bilateral lower
and trunk extremities and trunk
Endurance Severely impaired Moderately impaired Mildly impaired
foam, eyes open.” Documenting problems in this manner with this type of multiple-problem approach to maximize the
makes goal writing (and subsequent treatment planning) time available with clients.
much easier. The clinician must thoughtfully choose environments and
tasks that together stimulate and challenge the appropriate
Writing Goals on the Basis of Body Structure postural control systems. To stimulate one sensory system,
and Function Impairments and Activity the other systems must be placed at a disadvantage to force
Limitations reliance on the targeted system. The environment is then
Goals also should be stated in objective and measurable structured to put the other systems at a disadvantage (e.g.,
terms so that their achievement can be judged. “Improved training with eyes closed or in the dark puts vision at a
balance” is open to any interpretation, whereas “able to disadvantage and forces the use of somatosensory and ves-
stand on right leg for 30 seconds on 3/3 trials” and “walks tibular inputs). If one side or limb is significantly more
tandem entire length of balance beam without misstep affected, such as in hemiplegia, then the other side must be
7/10 times” are measurable goals. These types of goals may disadvantaged to force reliance on the targeted side. Tasks
be helpful to the clinician who understands the link between are then selected to disadvantage the less affected side. For
impairments and function, but they may seem nonfunctional example, placing the less affected leg on a step or small ball
(and therefore unnecessary) to others who read them (e.g., makes it more difficult to use for balance and forces the
case managers, third-party payers). From their standpoint, transference of weight to the more affected leg. To achieve
incorporating the functional task that will be positively af- optimal function, however, all systems and all sides must be
fected by its achievement into the impairment goal is benefi- capable of working together, so training to improve balance
cial; for example, “able to stand on right leg for 10 seconds impairments must be incorporated and interspersed with
at a time so that stairs can be ascended and descended step- training functional tasks. For carryover of improvements
over-step without railing,” or “walks tandem on balance into real-life situations, training tasks should be varied
beam to demonstrate ability to avoid falls using hip strat- enough to promote motor problem solving on the part of the
egy.” By describing the specific system problem (e.g., client.111 For example, sitting balance and transfers should
power, range, balance strategies) as it relates to function be taught using stable and unstable surfaces, with different
in the treatment objectives, clinicians force themselves to heights and firmnesses, with and without armrests and back
focus on functional outcomes and illustrate for others why supports, and using both right and left sides. This technique
these goals are meaningful. The need for and validity of the may improve the client’s abilities to perform safe sitting
treatment are then more likely to be clearly perceived. At and transfers in new situations not previously practiced in
times, goal documentation requirements may specify that therapy.24
the goals be purely functional in nature. Writing goals with- Tables 22-5 through 22-7 illustrate the process of test
out impairment components may meet the needs of the re- choice, problem identification based on test results, goal set-
viewer, but they will not help to direct clinical interventions ting based on impairments and disabilities, and treatment
to the specific components that need to be addressed in each planning based on goals in three different types of clients.
individual client. Documentation must meet reviewer re- Note that only selected tests were performed for each client.
quirements, but for one’s own benefit, writing a separate set Goals were directly related to the problems that were identi-
of goals with the dual impairment-function component will fied by the tests, and treatment plans followed directly from
assist the clinician with planning and prioritizing treatment. the goals.
If a problem cannot be alleviated and requires compensa-
tion, the goal(s) should reflect this as well. For example, a
BALANCE RETRAINING TECHNIQUES
client with diabetes has progressive peripheral neuropathy
with somatosensory loss and ineffective ankle strategy. If Motor Learning Concepts
the client’s visual and vestibular sensory systems and proxi- Although covering the principles of motor learning is not
mal strength are relatively intact, however, then the goals within the scope of this chapter (refer to Chapter 4), the
might mention improved use of visual cues and successful discussion of balance retraining methods is not possible
substitution of hip and stepping strategies. Educational and without some consideration of several motor learning con-
environmental modification goals for safety also are appro- cepts that must be incorporated into treatment. The clinician
priate in these situations. must remember that successful treatments address the inter-
action of the individual, the task, and the environment
Developing a Treatment Plan (Figure 22-18).12,24
Once the goals have been listed and priorities established, the
treatment plan is developed. The most effective and efficient Individual
treatments focus first on those problems with the greatest Therapists should know their clients’ impairments: sensory
impact on function and address more than one problem at a and motor, peripheral, and central. Whenever possible,
time. Training balance on an unstable surface contributes to therapists should know which impairments can be rehabili-
the use of visual and vestibular inputs as well as to the use of tated and which require compensation or substitution. Be-
hip strategy, increased lower-extremity strength, and increased cause of the nature of neurological insult, this includes an
motor control (skill) on that type of surface. Training gait on awareness of cognitive and perceptual impairments that may
an inclined treadmill with eyes closed or head movement in- affect the ability to relearn old skills or develop new ones.
creases the use of somatosensory and vestibular inputs, endur- Optimal learning of skilled movement requires that the
ance, postural control, ROM, and lower-extremity strength. client have (1) knowledge of self (abilities and limitations),
Creative clinicians develop comprehensive treatment plans (2) knowledge of the environment (opportunities and risks),
n AN EXAMPLE OF TEST SELECTION, PROBLEM IDENTIFICATION, GOAL SETTING,

TABLE 22-5
AND TREATMENT PLANNING IN A CLIENT WITH PERIPHERAL VESTIBULAR DEFICIT
Patient profile: 50-year-old woman
Diagnosis: Uncompensated (R) unilateral peripheral vestibular deficit for 6-7 years
Course of examination and treatment: Otolaryngologist A psychologist A neuro-otologist A outpatient physical therapy
TEST PROBLEMS IDENTIFIED GOALS SET TREATMENT PLAN
Visual acuity Four-line drop on Snellen eye chart Able to read chart with only one-line Gaze stabilization exercises
drop
Oculomotor Positive nystagmus with Frenzel lenses
Saccades
Pursuit
Nystagmus
Gaze stabilization Unable to perform test Able to rotate head horizontally Gaze stabilization exercises
Visual-vestibular ( Fixation with horizontal 1 vertical 2 min without problems
interaction head movements after 5-10 s
VOR cancellation Able to perform visual-vestibular
Hallpike interaction test
SOT Decreased use of somatosensory inputs Somatosensory use 100/100 Sensory environment
70/100 stimulation
Decreased use of visual inputs 55/100 Vision use 90/100
Absent use of vestibular inputs 0/100 Vestibular use 70/100
Unable to resolve visual conflict 0/100 Visual conflict resolution 90/100
Limits of stability Restricted anteriorly and posteriorly to Limits of stability expanded to 85% COG control training
35% limit of stability anterior and posterior at 5-s pacing
Slow movement time
ROM and strength None
Gait Gait training
Eyes open Weaves side to side
Eyes closed Deviates to (R) with eyes closed Walks in a straight line with eyes closed
Head turning Dizzy with horizontal head turns Walks with only slight deviation with
eyes closed and head turning
Pivots Loss of balance and dizzy with (R) Spins to (R), (L) with eyes closed
pivot
Abrupt stops Very unsteady with abrupt stop; feels Comes to abrupt stop steadily
“off”
Reprinted with permission from NeuroCom International, Clackamas, Ore.
COG, Center of gravity; (L), left; (R), right; ROM, range of motion; SOT, Sensory Organization Test;
VOR, vestibuloocular reflex.
(3) knowledge of the task (critical components), (4) the abil- errors are frequent and performance is inefficient and incon-
ity to use those knowledge sets to solve motor problems, and sistent. Within the nervous system only temporary changes
(5) the ability to modify and adapt movements as the task are occurring. Skill refinement is the second stage. The goal
and environment change. To the extent that a client is miss- is for the client to improve the performance, reduce the
ing these characteristics, the clinician should attempt to number and size of the errors, and increase the consistency
support his or her development or even supply them until and efficiency of the movements. Skill retention is the final
they are present. Different types of clients vary with regard stage. The ability to perform the movements and achieve the
to which characteristics are likely to be missing. For exam- functional goal has been accomplished, and the new objec-
ple, a cognitively impaired, head-injured client may lack tive is to retain the skill over time and transfer the skill to
awareness of self and environment, even though his or her different settings. Retention and transfer are the hallmarks
physical abilities make modifying and adapting movements of true learning, in which some relatively permanent changes
possible. Conversely, a quadriplegic client may be aware of have occurred within the nervous system. A client may have
his or her limitations, the environment, and the task de- attained the skill retention phase for sitting balance tasks, be
mands but may initially have limited experience to know in the skill refinement stage for standing balance tasks, and
how to solve a motor problem and limited physical ability to be in the skill acquisition stage for locomotor balance tasks.
modify movements. Therapists use practice and feedback to teach motor
The clinician must also ask what motor learning stage the skills. Repetition is necessary to develop skill; feedback is
client is in for different tasks. Skill acquisition is the first necessary to detect and correct errors. During skill acquisi-
stage. The objective is for the client to “get the idea of tion, frequent repetition of a movement or task and frequent
the movement” to begin to acquire the skill.112 In this stage, feedback are beneficial to help the client begin to be able to
TABLE 22-6n EXAMPLE OF HOW TREATMENT PLANNING FLOWS FROM TEST RESULTS
IN AN ELDERLY CLIENT WITH FREQUENT FALLS
Diagnosis: Disequilibrium of aging, frequent falls
Course of examination and treatment: Cardiologist A neurologist A outpatient physical therapy
Peripheral sensory Mildly decreased vibration sense Compensate for permanent sensory Educate about safe surfaces and
bilateral lower extremity loss lighting
Somatosensory Home safety evaluation
Vision ( Acuity, cataracts
( Depth perception
SOT Absent use of vestibular inputs 0/100 Increase use of vestibular inputs to Somatosensory and vestibular
Decreased use of somatosensory 30/100 stimulation*
inputs 60/100 Increase use of somatosensory inputs
Dependent on vision to 75/100
Static postural sway Excessive sway—2 standard devia- Standing sway within normal limits COG control training
tions outside normal range for age for age
Nudge or push test No use of ankle or hip strategy Survives 5/10 pushes with hip Hip strategy exercises*
Steps immediately strategy
LOS No ankle strategy—uses hip strategy Uses ankle strategy to reach 40% COG control training
Sway to 45% LOS anterior, 35% LOS anterior and posterior
LOS posterior Reaches 8/8 targets at 75%
Slow movement time LOS using hip or ankle strategy
within 4 s
ROM ( Neck extension 0-10 degrees ) Spinal extension neck 0-20 degrees ROM exercises*
( Lumbar extension 1-15 degrees ) Lumbar extension 0-20 degrees
( Hip extension 0-5 degrees ) Hip extension 0-10 degrees
Strength Flexion 4/5 )(B) Hip abduction and extension to Progressive resistive exercises,
(B) Hip abduction 31/5, extension 3/5 greater than 4/5 including bicycle*
(B) Knee extension 41/5, flexion 4/5 ) (B) Ankle dorsiflexion and
(R) Ankle dorsiflexion 32/5 plantarflexion to 42/5
(L) Ankle dorsiflexion 2/5
(B) Ankle plantarflexion 31/5
Gait (GARS) Score 35/48 GARS scales 25/48 Gait training*
Deviations (I) Ambulation with walker in home, 1—starts, stops, turns
Forward flexed trunk community 2—treadmill
Double limb stance prolonged 3—uneven surfaces, curbs,
bilaterally stairs, carpet, outdoors
Short step length
Endurance Fatigue after ambulating 60 ft Ambulates more than 200 ft without Gait training as earlier
stopping
Tinetti balance 6/16 score Tinetti balance score 10/16 Gait training as earlier
scale
Tinetti gait scale 5/12 score Tinetti gait score 8/12 Gait training as earlier
Falls and catches self
LOS, Limit of stability; (B), bilateral; COG, center of gravity; GARS, Gait Assessment Rating Scale; (I), independent; (L), left; (R), right; ROM, range of
motion; SOT, Sensory Organization Test.
*Also included in home exercise program.
perform the desired movements and tasks. As soon as the objects of various weights, shapes, and sizes; and take the
client progresses to the skill refinement stage (the clinician object from higher and lower heights and alternately reach
observes reduced errors and less variable performance), to right and left sides. This variation introduces a problem-
however, then practice should be varied and feedback briefly solving demand for the client: modifications in timing,
delayed. For example, the task of standing and reaching to force, and sequencing are now necessary.111
one side to take an object from the therapist might initially Feedback, which is especially helpful for those with sen-
be repeated to the same side and at the same height several sory reception or perception problems, initially may contain
times. Then the therapist should begin to vary the task information to assist the client in detecting errors about the
demands gradually: reach farther or faster; take different goal achievement (knowledge of results, such as “you did
TABLE 22-7n AN EXAMPLE OF HOW TREATMENT PLANNING FLOWS FROM TEST RESULTS
IN A CLIENT WITH RIGHT HEMIPARESIS
Diagnosis: Left cerebrovascular accident with right hemiparesis
Course of examination and treatment: Acute rehabilitation A home health A outpatient rehabilitation
Peripheral somatosensory None
SOT Average overall stability 47/100 Average stability 60/100 Vestibular stimulation with forced
Absent use of vestibular inputs 0/100 ) Use of vestibular inputs 15/100 use and head movements
Postural sway COG control training
Functional reach Forward lean restricted to 5 inches Able to reach forward 8 inches
Static balance Weight shift asymmetry to left in ) Control of COG
static standing and medial or lateral Stands midline
sway, 25% LOS to left of midline
Limit of stability Forward weight shift restricted to ) Forward LOS to 50%
25% LOS ) Right LOS to 50%
Rhythmic weight shift Extraneous sway off desired path ( Extraneous sway scores by 50%
OLST Unable on right leg, 30 seconds on Stands on right leg, 10 seconds COG control training
left leg
Nudge, push (motor Switch from ankle to hip strategy Able to stand upright after mild Hip and stepping strategy training
strategy selection) noted but unable to withstand per- perturbations 5/10 times
turbation Able to “catch” self by stepping
or reaching 5/10 times
Range of motion None
Strength: right leg 4/5 Knee extension ) RLE strength Progressive resistive exercises
3/5 Knee flexion 5/5 Knee
2/5 Ankle dorsiflexion 4/5 Ankle
3/5 Ankle plantarflexion
Endurance Standing tolerance less than Able to stand unaided for Standing tolerance tasks
10 minutes 15 minutes
Gait ( Step length—RLE Symmetrical step height and Gait training on treadmill
( Step height—RLE length 5/10 times
( Heel strike—RLE ) Heel strike RLE 5/10 times
( Toe-off—RLE
Tinetti Gait Subscale Unable to turn, reach, or bend 8/12 score Gait training on uneven surfaces,
without loss of balance No falls with head movements, with low
Falls: Gait independent without cane in lighting
Uneven surfaces household; with cane in com- Safety education
Low lighting munity
Head turning
No community ambulation
Requires cane
Requires supervision for household
ambulation
COG, Center of gravity; LOS, limit of stability; OLST, one-legged stance test; RLE, right lower extremity; SOT, Sensory Organization Test.
not lean far enough to reach this last time”) or about a move- describe her or his own errors, and afterward providing the
ment error (knowledge of performance, “you did not feedback, the therapist allows the client to compare her or
straighten your knee enough last time”).113 Early feedback his own developing internal frame of reference with the cor-
also may contain cues about what to do better next time, rect external frame of reference. By asking clients to suggest
such as “straighten your knee before you shift weight onto what might be done to correct the errors, the therapist shifts
that leg.” If feedback is always provided by an external the error correction process from the external source to the
source, such as the therapist, a mirror, or a computer moni- clients, supporting motor problem-solving processes. As
tor, then the client is not given the opportunity to develop clients progress to the skill retention level, variations should
internal error detection and error correction mechanisms and increase (including task and environmental demands) and
will not be as likely to retain or transfer the skill. By delay- feedback delays should be longer. The clinician must de-
ing the feedback and asking the client to estimate or velop a sense of how to use practice variation and feedback
patory postural adjustment (e.g., preceding a lift)? Clients

need to learn to respond in all three conditions, which are
often combined. For instance, lifting is a voluntary move-
ment. Predicting the load to be lifted leads to anticipatory
postural preparation. Counteracting the destabilizing force
of a greater-than-predicted load requires an automatic pos-
tural response. If, during therapy, the clinician says “don’t
let me push you” before nudging the client, the demand is
for anticipatory postural adjustment. If the disturbance is
provided without warning, the demand calls for APRs. If the
Figure 22-18 n Interactions of the individual, the environment, clinician requests a lean to the right, that is a voluntary pos-
and the task are critical to postural control skills. Although they tural adjustment. Activities that demand all three types of
may be isolated in the mind of the clinician for assessment pur- balance control, either one at a time or in combination,
poses, they are never isolated in the function of the client. should be included in balance retraining programs.
Environment
Just as tasks can be purposefully selected to promote pos-
delay therapeutically to progress clients through the stages tural control responses, environmental conditions also must
of motor learning. Too much variation and too little feed- be included in the design of the therapy plan to stimulate the
back early on impede skill acquisition; insufficient variation necessary systems. Gravity cannot be manipulated by the
and excessive feedback later on hamper skill retention and clinician, but the client needs to learn to counteract it at dif-
transfer. ferent speeds and from different positions, among other
things. Familiarity with how gravity can aid movement, as
Task in walking, is also important. The therapist can vary the
Functional rating scales performed as a part of the evalua- surface conditions. They may be stable, even, and predict-
tion yield information about what tasks, or functional ac- able (hospital hallway, sidewalk), unstable (boat, subway,
tivities, are limited by the postural control impairments. Bed gravel driveway), uneven (grass, curbs, stairs), or compliant
mobility, sitting, sitting to standing, transfers, standing, (beach, padded carpeting). Visual conditions also may be
walking, working, and sports participation may be affected. manipulated. Visual cues may be available and accurate
Repeating the problematic tasks over and over is one ap- (daylight, fluorescent lighting), unavailable (darkness or
proach; however, analyzing the problematic tasks to deter- poor lighting, or lack of environmental cues such as a busy
mine what postural control demands are placed on the client carpet pattern on a stairway), unstable (moving crowd, pub-
when undertaking those tasks is far more productive for lic transportation), used for purposes other than balance
the clinician. Does a task demand predominantly stability? (fixation on a ball in tennis), or dependent on head move-
Mobility? Both? For example, standing to take a photograph ments. Clinicians should help prepare their clients to func-
demands the ability to hold still, standing to move laundry tion in the real world by training them to maintain balance
from the washer to the dryer requires weight shifting, and under different combinations of surface and visual condi-
standing to don a pair of pantyhose calls for both steadiness tions. This includes situations in which cues from the envi-
and movement. All three are standing tasks, but each places ronment agree—that is, visual, somatosensory, and vestibu-
different postural control demands on the client. By using lar inputs are all sending the same message, so to speak—as
task analysis, the therapist may consciously select or design well as in sensory conflict environments, where cues from
tasks to place specific demands on the client such that the one system may disagree with (not match) cues from the
postural control systems that need improvement will be other sensory systems. Functional situations in which sen-
challenged to respond. sory conflicts may exist include elevators, escalators, people
Analysis of mobility tasks includes attention to timing, movers, airplanes, and subways. An emphasis on being able
force, and duration of movements. Consider the different to adapt to changes in environmental conditions rapidly and
timing demands for weight shifting and reaching to catch an effectively is important.
item falling from a shelf, take a hot casserole out of the
oven, or open a door. Compare the different amounts of Intervention
force necessary to pick up a heavy suitcase, pick up a baby Successful intervention for the individual with a balance
from a crib, or replace a ceiling light bulb. The duration of a disorder depends on the ability of the clinician to identify
balance demand may be brief, as in recovering from a trip, the components of the problem. The therapist must create a
or extended, as in walking across an icy parking lot. Clini- program that addresses several components at a time, not
cians should choose tasks that vary these parameters to just for efficiency, but because these systems should be able
prepare clients for activities with various mobility demands. to function together to perform functional activities in real-
Activities that incorporate changing head positions will fur- world environments. Treatment is oriented toward multiple
ther challenge the individual with vestibular insufficiency. impairments, with tasks and environments selected to best
Therapists also need to consider whether the elements of correct involved or facilitate compensatory systems.
the task are predictable or unpredictable. In other words, The intervention must be matched to the level and com-
will the postural control demand be a voluntary movement bination of body system impairments. For example, tasks
(e.g., sweeping the porch), an automatic postural response related to the different functions of the sensory systems
(e.g., missing the last step on a flight of stairs), or an antici- should be identified and not treated as a single body system
problem. The clinician should have a good idea of the level or compliant surfaces, with vision either absent (eyes
of stimulus during each exercise program so that the facilita- closed), destabilized (eye movements or head movements),
tion is as accurate as possible. Progression of the program or confused (e.g., optokinetic stimulation) provides chal-
follows the changes seen from one intervention to the next lenging combinations. Adding neck extension and rotation
to promote carryover and retention of learning. The exercise to place the vestibular organs at a disadvantaged angle can
progression integrates activities that reflect those changes. increase difficulty. Gaze stabilization with head turns while
This usually involves more complex movement skills in a standing on an uneven surface or while walking creates a
greater range of gradually more challenging environments. higher-level challenge. Quick movements of the head, head
tilts, or forward bending trigger vestibular signals to add
Sensory Systems input to the system. Combining these types of activities can
In general, the less sensory information available, the more create progressively more complex challenges. Standing or
difficult the task of balancing. A treatment progression weight shifting on foam with eyes closed, and head and eye
might therefore start with full sensory inputs (vision, so- movement while walking all require vestibular input for suc-
matosensory, and vestibular: 3/3) available in the environ- cessful performance.
ment and perhaps augmented feedback if intrinsic sensory Additional vestibular challenge can be added by includ-
channels are deficient, as with somatosensory loss or a ves- ing activities that require quick changes of position in a su-
tibular disorder. Challenge is added by manipulating either perior or inferior direction, such as a lunge or going up and
visual or somatosensory inputs, so that equilibrium must be down stairs. Other exercises involving up-and-down body
maintained by using only two of three senses (vision and movements, such as sitting to standing, seated bouncing on
vestibular or somatosensory and vestibular). If both vision a Swiss ball, and standing bouncing on a mini-trampoline,
and somatosensory inputs are manipulated, then only the all with eyes closed to eliminate use of vision for stability,
vestibular inputs are a reliable source of sensory information increase the demand on the vestibular system. To train the
and balance is accomplished with only one of three senses.114 client who is overreliant on vision to improve the use of
Most patients with permanent or progressive vestibular or vestibular inputs versus vision, activities such as watching a
somatosensory losses naturally compensate and become vi- ball being tossed from hand to hand while walking, walking
sually dependent. In cases in which improving the use of backward, or walking with eye movements can be used.
somatosensory or vestibular inputs is necessary, the training Reading while walking requires the use of vision for reading
of vision for stability can be counterproductive, teaching so that it cannot be used for postural orientation, forcing the
compensation versus improvement of normal function. On other sensory sources to be used for orientation.
the other hand, visual retraining is entirely appropriate for
the client with severely compromised somatosensation that Multisensory and Motor Control Dysfunction
cannot be changed, as is common in persons with diabetes. Older clients often have dysfunction in all three sensory
To stimulate the use of visual inputs, environments are systems—that is, a multisensory balance disorder. Disease-
designed to disadvantage somatosensation while providing related disruptions of the somatosensory or visual system
reliable visual cues (stable visual field with landmarks). (e.g., a peripheral neuropathy or cataracts) are combined
Somatosensation cannot be removed as can vision, but it can with age-related declines in the vestibular system. In some
be destabilized by having the client sit or stand on unstable cases therapy aimed at increasing vestibular function can
surfaces (rocker board, biomechanical ankle platform sys- have a significant impact on postural stability. If sensory
tem [BAPS] board, randomly moving platforms) or con- loss is permanent or progressive, safe function may require
fused by having the client sit or stand on compliant surfaces the use of an assistive device.115 Choosing an assistive de-
that give way to pressure, such as foam, “space boots,” or vice for these clients can be a challenge. An individual with
responsively moving platforms. cerebellar or visual-perceptual problems may have more dif-
To stimulate the use of somatosensory inputs, environ- ficulty using an assistive device, and thus it may be contra-
ments are designed to disadvantage vision while providing indicated. For these clients, careful assessment of safety and
reliable somatosensory inputs (stable surfaces, level or in- gait both with and without the device is demanded. A single
clined). Having the client close the eyes or practice in low cane often does not allow for compensation for changes in
lighting or darkness removes or decreases visual inputs. For direction of an impending fall, and a standard aluminum
clients with an overreliance on visual input for balance, the walker does not provide support when changing directions
somatosensory system needs to be facilitated while the vi- because it must be lifted. The ideal walker has four rotating
sual system is disrupted. This can be accomplished by hav- wheels and thus the ability to change direction without be-
ing the client sit or stand on a stable surface while performing lifted. This device greatly increases stability, and the
ing quick head turns. For the client with self-limited head client usually describes a significant increase in confidence.
movement, the intervention may begin with head movement Of course, the use of a walker also limits normal use of the
during quiet standing and progress to head movements dur- upper extremities and trunk during gait and restricts the
ing weight shifts and then walking. Eyes-closed standing types of environments that can be negotiated. Making sure
and weight shifting also increase the use of somatosensation the client stands erect in walking versus leaning forward into
for balance. Optokinetic stimuli in the visual surround also a permanent flexed position is important in order to retain
stimulate use of somatosensory inputs. and/or improve existing postural function.
To stimulate the use of vestibular inputs for adaptation of Many clients with neurological conditions have tempo-
the CNS, environments are designed to disadvantage both rary difficulty with head control early in their recovery, and
vision and somatosensation while providing reliable vestib- others have chronic head control problems. Their ability to
ular cues (detectable head position). Practicing on unstable orient the vestibular organs, eyes, and neck proprioceptors
properly is impaired, which negatively affects the ability to Balance during Gait. During gait, the COG follows a
perceive internal and environmental cues that could assist in sinusoidal path as forward progression of the body mass
balance maintenance. Thus, developing head control is very combines with alternating lateral weight shifts to the stance
important. (Refer to Chapter 9 for examples of how to im- foot (Figure 22-19).23 Each step creates a new base of sup-
prove head control.) Clients with spasticity or contractures port. Assistive devices such as canes, walkers, and crutches
of the ankles and feet who cannot place their feet in full extend the base of support and thus reduce the demands on
contact with the floor are at a biomechanical disadvantage the intrinsic balance control system. In sitting, standing, and
and also have difficulty receiving somatosensory inputs that walking, control of the COG involves the ability to establish
could support postural control processes. The more accurate a stable base of support and transfer weight over it. Treat-
and reliable sensory information available, the greater the ment progressions for COG control then involve training to
chances that the sensoriperceptual processes that contribute establish, maintain, and reduce the base of support and to
to balance can fulfill their role. Treatment progressions
should include attention to increasing the client’s ability to
receive and process sensory information pertinent to balance
control through oculomotor, head, and peripheral limb posi-
tioning and movement.
Control of the Center of Gravity

Effective control of the COG depends on accurate awareness
of body position and motion in space and the relation be-
tween body parts (perception) as well as biomechanical and
musculoskeletal systems (execution). Trunk and head con-
trol abilities are primary. For clients with paralysis or degen-
erative disease that limits the ability of arms and legs to as-
sist with postural stability, head and trunk control may be
the dominant means of balance. Both the head and neck and
the trunk need to be able to achieve and hold a midline posi-
tion, rotate around this midline axis, and move away from
and return to the midline without loss of balance. The term
midline here refers not to a line between right and left sides
but to a point at which the right and left and forward and
backward components are centered in all planes—medial-
lateral, anterior-posterior, rotary, and side bending (shorten-
ing and elongation on either side). Midline problems can
certainly be caused by body system problems such as weak-
ness, pain, or sensory deficits but can also be caused by
perceptual problems that have nothing to do with balance
deficits but do cause balance problems.
Sitting Balance. In sitting the pelvis and posterior
thighs form the primary base of support, with additional
stability provided by the feet in contact with the floor. The
axis of anteroposterior movement rotates around the greater
trochanter, and forward and backward leans are achieved
through pelvic and trunk movement. Anterior pelvic tilt with
upper trunk extension allows forward reaching and begins
the sitting-to-standing transition. Lateral weight shifts with
trunk elongation precede right and left reaching and scoot-
ing. Lateral weight shifts with trunk rotation permit cross-
midline reaching and begin the sitting-to-supine progres-
sion. The use of arms to prop in sitting is an extension of the
base of support.
Standing Balance. In standing, the feet form the base
of support. The axis of anteroposterior movement rotates
around the medial malleolus. Mediolateral movement oc-
curs with weight shifts from foot to foot. Weight shifts move
the COG through space for reaching and lifting tasks as well Figure 22-19 n The trajectory of the center of gravity (C of G)
as in preparation for stepping. Ankle strategy is most effec- and the center of pressure (C of P) during a gait cycle. LHC, Left
tive for forward and backward movement of the COG heel contact; LTO, left toe-off; RHC, right heel contact; RTO, right
through the limits of stability on a stable surface. As the toe-off. (From Palia AE, Winter DA, Frank JS, et al: Identification
COG nears the sway boundary, hip strategy works to restrict of age-related changes in the balance control system. In Duncan P,
its travel. If this fails, stepping or reaching strategies are editor: Balance: proceedings of the APTA forum, Alexandria, VA,
used to reestablish a new base of support. 1990, American Physical Therapy Association.)
produce automatic, anticipatory, and voluntary postural re- Standing Balance. Standing balance tasks also can be-
sponses to restrict or produce weight shifts. gin with finding midline and becoming stable there. Con-
Early treatment progression for COG control may in- trolled mobility (volitional) should be encouraged as soon as
clude “neurodevelopmental sequence activities” (e.g., prone possible, first on a stable surface with slow, small weight
on elbows, all fours, kneeling, right or left side sitting, half- shifts. Challenge is added by increasing the distance trav-
kneeling), not for the purpose of “reflex development” in the eled away from midline, moving toward restricted regions of
traditional sense but because the task demands are to bal- the limits of stability, altering speed of sway, adding com-
ance with progressively less surface contact (i.e., shrinking bined upper-extremity activities (e.g., dribbling a basketball,
the base of support). Additional benefits include greater reaching), or adding resistance (manual, flexible bands).
control, coordination, and generation of power of the neck, Narrowing the base of support (Romberg, tandem, single
trunk, and axial muscles. It also is useful for simultaneously leg) makes control of the COG more demanding. Placing the
addressing impairments such as lower-extremity extensor feet in a diagonal stride position is more desirable for pregait
tone, trunk weakness and asymmetries, and head and neck weight shifting than is symmetrical double stance. Attention
extensor weakness. Functionally, bed mobility and floor- should be given to the stance (loading) leg with regard to
to-stand transfers are related to these progressive position pelvic protraction, hip and knee extension, and ankle dorsi-
exercises and should be practiced concurrently in low- and flexion, with the tibia traveling forward over the foot. Focus
high-level clients, respectively. on the swing (unloading) leg should include pelvic drop
Sitting balance can be progressed by (1) removing upper- with knee flexion as the heel comes up and pressure through
extremity support (hands on firm surface to moveable sur- the ball of the foot and toes to load the opposite leg maxi-
face [e.g., ball, bolster, rolling stool], one hand free and both mally. Standing balance exercises can be made more diffi-
hands free); (2) making the seating surface less stable (mat cult by training on a less stable surface (carpet, foam, rocker
to bed to rocker board to Swiss ball); and (3) removing the board, BAPS board) and by adding combined head and eye
use of one foot by crossing the leg or of both feet by raising movements or closing the eyes. The goals for dynamic sit-
the height of the seat so they do not touch the floor. Tasks ting and standing balance exercises are to increase the size
might include multidirectional weight shifts with the hands and symmetry of the limits of stability and improve the abil-
in contact with a bolster or ball that is pushed or pulled to ity to transfer weight to different body segments with con-
and fro, reaching or passing objects, performing upper body trol at different speeds and with varied amounts of force. To
tasks (grooming, dressing), managing socks and shoes and facilitate somatosensory and vestibular integration, these
wheelchair armrests and footrests, and so forth. activities can be performed with decreased or distorted
Sitting to Standing and Transfer Balance. Transi- visual cues. Closing the eyes, turning the head quickly, turn-
tional movements such as sitting to standing and transfers ing the lights down low, or wearing sunglasses may decrease
involve large COG excursions over a stable base of support. the use of vision for stabilization.
For sitting to standing, the base of support must change from Strategy Training. Training ankle, hip, and stepping
the seat to the feet. The feet begin to accept the weight first by strategies may begin in a voluntary manner but must prog-
downward pressure through the heels as the pelvis rolls ante- ress to an automatic level of use to develop more normal
riorly. The weight moves to the front of the feet as the trunk balance and for real-life prevention of balance loss. Before
comes forward and the pelvis lifts from the surface, then strategy training, the clinician should be sure that the client
backward toward midline as the trunk extends into standing. has the ability to develop the desired strategies. The ob-
The COG stays near midline if both legs are participating served dominance of other strategies is appropriately com-
equally, but it will often deviate to a preferred side during the pensatory, not dysfunctional, if a missing strategy cannot be
transition in clients with hemiplegia. Training should include effectively executed. Clients use these strategies to prevent
disadvantaging the preferred leg (perhaps by moving it a bit loss of balance, so the clinician must take care not to reduce
forward) to allow the more affected leg and foot to experience reliance on an effective strategy but to add additional strate-
the weight transference. During transfers, a lateral weight gies to the repertoire.
shift is required in addition to the partial stand. The COG does Ankle strategy should be practiced on a firm, broad sur-
not remain near midline; it instead moves forward to load the face. Clients can be asked to sway slowly in anterior-poste-
feet and then laterally toward the side of the transfer. Progres- rior, right-left, and diagonal directions, first to and from
sion of balance skills in sitting to standing and transfer tasks midline, progressing to passing midline, and finally pro-
may involve gradually lowering the height of the surface, re- gressing to sway toward the periphery without return to
moving armrests to preclude upper-extremity assistance, and midline. Head and pelvis should be traveling in the same
transferring to surfaces of different heights and firmnesses. direction at the same time. Clients can practice standing near
Remember that velocity is a normal part of sitting-to-standing a wall with a table in front of them, swaying forward to
movements because the momentum is used to assist the touch the table with the stomach (leading with the pelvis)
weight transfer from seat to feet, so the clinician must allow and backward to touch the wall with the back of the head.
some speed during this task. If the client is unsteady on aris- Cues are given not to “bow” to the table and not to touch the
ing (cannot dampen or slow the speed in a controlled man- wall with the buttocks. As soon as the client is able to per-
ner), working gradually from standing to sitting initially may form this protocol, functional meaning should be added with
be beneficial before progression to sitting to standing. Prac- maneuvers such as forward or lateral reaching tasks, hands
tice of sitting to standing with the eyes closed can be an effec- over head to take things off shelves, and leaning backward
tive way to train clients who are overdependent on vision for to rinse hair in the shower. To improve anticipatory and
balance. Without the use of vision for stability, integration of automatic ankle strategy use, add slight perturbations to
vestibular and somatosensory systems can be facilitated. the body or the surface when midline, progress to gentle
perturbation when away from midline, and finally progress permit hands-free motion but provide tangible reassurance
from predictable to unpredictable perturbations. that a fall will be prevented if balance loss occurs. In this
Hip strategy is practiced on either a narrow or an unstable case, treatment progression would include weaning the cli-
surface, such as standing sideways on a balance beam, a ent off the use of the harness. If the only harness available is
two-by-four, a half-slice foam roller, foam, or a rocker on a body-weight support treadmill or gait system, move the
board. The head and pelvis travel in opposite directions to balance retraining to that harness. The treadmill does not
counterbalance each other, in a forward bow–backward need to be moving.
bending motion for anterior-posterior sway. Rapid sway is Gait Training. The initial focus for controlling the COG
requested in forward-backward, right-left, and diagonal di- during gait is a stable base of support that can be continually
rections. By using the wall and table setting previously reestablished quickly and reliably through stepping. Unlike
mentioned, clients can be cued to how to touch the nose to- standing balance, in which the base is stable and the COG
ward the table while simultaneously touching the wall with moves over it, during locomotion the base is moving and the
the buttocks. Lateral hip strategy can be trained similarly, COG moves to stay over the base. Achieving a symmetrical,
with the client standing sideways to the wall, touching the smoothly oscillating COG movement is the objective, with
table with one hip and the wall with the opposite shoulder. the forces of gravity and momentum being exploited.
Sway close to the edge of the client’s limit of stability The training is begun first in the forward direction but
should produce a shift from ankle to hip strategy, so to en- also includes backward and sideways directions (sidestep-
hance the use of hip strategy the client should practice sway ping, braiding, or carioca) to increase postural control de-
control as far away from midline as possible without step- mands. Challenge can be added by narrowing the base of
ping. As soon as the client demonstrates the ability to per- support (tandem) or reducing the foot-surface contact (walk
form this strategy, it should be incorporated into functional on toes or heels). Training to integrate postural control with
tasks such as low reaching (e.g., trunk of car, laundry dryer). locomotor skills is best accomplished not through continu-
To promote anticipatory and automatic use of hip strategy, ous, steady-pace walking, but by starting, stopping, turning,
the client is in midline and given moderate, rapid perturba- bending, varying the speed, and avoiding or stepping over
tions to the body or the surface such that ankle strategy will obstacles. Difficulty is added by increasing the abruptness,
be insufficient to counteract the force. Then the size of the frequency, and unpredictability of these types of tasks and
disturbance is increased, and the client is positioned away by adding tasks such as carrying or reading while walking.
from midline when the perturbation is given so that righting Altered surface conditions (carpets, ramps, curbs, stairs,
to midline is appropriate. The shift should be made from grass, gravel) or reduced lighting conditions also heighten
predictable (“don’t let me make you step or fall”) to unpre- the challenge. Head and eye movements while walking
dictable perturbations. should be added as the client improves. Walking quickly
Stepping strategy can be practiced first from atop a step, while reading signs on the wall or room numbers, for ex-
curb, or balance beam. Both legs should be included in train- ample, or looking toward and away from the therapist while
ing because real-life situations such as a slip or trip often walking makes vision more difficult to use for stability.
preclude the use of one limb and demand the use of the Walking in crowds or in busy, cluttered environments is
other. It may be necessary to fix one foot in position to pre- also challenging. Locomotion training on the treadmill
vent stepping by the less affected leg in order to allow reduces some abnormal asymmetries and increases control
a stepping response on the more affected leg to emerge of gait with increased extension of the trailing limb.116
(a forced-use paradigm). Progress is made by stepping on a Again, gait training specifically for balance enhancement
level surface and then to stepping up onto a step or curb or should occur without holding onto fixed surfaces with the
over progressively larger obstacles (appliance cord, shoe, hands, for example, parallel bars or the side rails of the
phone book). All directions should be practiced, including treadmill. This is because the nervous system needs to learn
lateral and diagonal perturbations, if safe recovery from to solve the balance problem using the legs and trunk, not
real-life unexpected balance losses is to be learned. Large, the hands.
rapid perturbations are given such that ankle and hip strate- Clients with somatosensory loss in the feet should use a
gies will be inadequate and stepping or reaching is de- cane or walker. They may not need the device for biome-
manded. Again, progress should be made from predicted chanical support, but they do need to obtain as much infor-
to unpredictable disturbances. For any automatic postural mation about the surface as possible. Through use of a cane
response—ankle, hip, stepping, protective reaching—to be or walker, preserved somatosensation in the hands can de-
effective in real life, successful demonstration of the re- tect surface information that is important for balance con-
sponse to unexpected perturbations is imperative. trol, and biomechanical support is available if needed in
Many clients, especially those who are fearful of falling, case of balance loss.
are dependent on the use of hands for stability. Therapeutic
balance retraining activities should provide the maximal
Other Considerations
level of challenge that can be managed without the need for
upper-extremity support. If the client physically needs to Treatment Tools
hold on, then the activity is at too high a level and should be Therapists use both high-technological and low-technologi-
modified. Otherwise, what is being taught is a “hand strat- cal equipment in the remediation of balance deficits; each has
egy” that will not be useful if the client experiences loss of advantages and disadvantages. High-technological options
balance when nothing firmly fixed is available to grasp for include accelerometers with motion biofeedback, forceplate
stability. Extremely anxious clients may initially benefit systems with postural sway biofeedback, electromyographic
from training with an overheard harness system that will biofeedback, optokinetic visual stimulation (from visual
surround or moving lights), videotaping, and treadmills with hands-free training as soon as possible, to increase reliance
biofeedback. Options for the evaluation and treatment of on the lower extremity and trunk reactions critical for bal-
balance and gait deficits are expanded with the addition of ance recovery strategies. Rapid and recordable measurement
advanced technology such as forceplate measures of postural of gait characteristics (e.g., velocity, step length, step width)
sway and pressure mat measures of gait, giving the therapist is possible with instrumented systems. Some systems are
a more quantitative and sensitive measurement than visual made for overground walking (Figure 22-23, B) and are
observation or timed measures. Most high-technological portable. Other systems are incorporated into treadmills
systems provide computer-generated reports with charts and and provide feedback during gait training. All motorized
graphs quickly. For training, overhead harness systems systems provide the ability to manipulate the environment
allow safe, hands-free practice, and computerized sway feed- easily and efficiently and to graduate tasks and environmen-
back supports motor learning (Figures 22-20 through 22-23). tal challenges safely. Drawbacks to high-technological
Computerized systems allow advanced monitoring of prog- equipment include cost, space requirements, and operator
ress and biofeedback, which supports motor learning.117 training requirements.
Figure 22-20 is an example of technology in which force- Low-technological options include mirrors, soft foam
plates measure pressure-generated signals (center of force pads, hard foam rollers, rocker boards, BAPS boards, tilt
[COF]). The systems shown use height and COF data to boards, Swiss balls, mini-trampolines, balance beams, and
calculate the COG, which is used to measure postural sway. wedges or incline boards. All these items are accessible (low
The COG icon may be displayed on the monitor screen for cost, easy to obtain), portable, and easy to use. The main
feedback to the individual if desired. Figure 22-21 is an ex- drawbacks for low technological equipment are that it does
ample of how surface motion provides both biomechanical not provide novel feedback, objective scoring, or graphic
and somatosensory challenges. Balance measurement char- recording, and clinicians must be skilled and creative in the
acteristics vary: some systems measure the motion of the use of such equipment in order to provide appropriate grada-
surface (Figure 22-21, A), whereas others use motion sensors tion of task difficulty and environmental conditions.
on the body placed at the level of the COG (Figure 22-21, B).
Other systems provide the ability to generate visual motion. Safety Education and Environmental Modifications
Figure 22-22, A, shows a system with a three-sided booth Remediation of balance deficits is not always possible, but the
with unidirectional motion combined with a moveable force- clinician is always responsible for ensuring the safety of each
plate with unidirectional motion. Both visual and somatosen- client. When permanent deficits exist, the client and the family
sory inputs can be manipulated for testing (e.g., SOT) and should be taught in what environments the client is at risk (e.g.,
training. Omnidirectional visual motion (Figure 22-22, B) a client with vestibular loss on a gravel driveway at night),
can be produced by rotating display systems that are used in what tasks are unsafe (e.g., ladder climbing, changing ceiling
a dark room. light bulbs), how the client can compensate (e.g., use a cane at
The ability to challenge balance during gait training is night or in crowds), and what changes in the home or work-
improved if the client is secure in an overhead harness sys- place are needed (e.g., night lights, stair stripes, raised toilet
tem as seen in Figure 22-23, A and C. These systems allow seats). Clinicians can ask the client (or family) to problem
solve risky situations: “What would the client do?” Home
evaluations should be followed by a list of recommended
safety modifications. Falls are frightening and dangerous; cli-
nicians should do their utmost to prevent them. If falls are
likely, clients and families should be taught what to do if a fall
occurs and, once the client is on the floor, how to perform
floor-to-standing or floor-to-furniture transfers. Home moni-
toring services such as Lifeline may be indicated if the client
lives alone and is prone to falling. Hip protectors will not pre-
vent falls but do significantly reduce the risk of hip fracture.
Home Programs
A Strengthening, stretching, posture, and endurance exercises
can all be performed safely at home so that time in the clinic
can be spent on balance-challenge exercises requiring super-
vision. Improvements in strength, ROM, posture, and endur-
ance support improvements in balance. Many balance exer-
cises can and should be performed at home if safety and
adherence can be ensured; however, unstable clients should
always be supervised. Standing balance tasks can be com-
pleted in a corner or near a countertop so that in case of
balance loss the client can use the hands (reaching strategy)
B to prevent a fall if other automatic postural response strate-
Figure 22-20 n Advanced technology to support balance and gait gies are inadequate. However, balance exercises should not
retraining. The forceplates shown here are static, or fixed. They are be routinely done while holding onto countertops, furniture,
somewhat portable. A, Balance Master Basic. B, Balance Master. or other surfaces. If the client needs to use her or his hands
(Courtesy NeuroCom, a division of Natus, Clackamas, Ore.) to perform the balance task, the task is too difficult and
A B
Figure 22-21 n Advanced technology to support balance and gait retraining. Other systems pro-
vide surfaces that can be made unstable (A) or made to move (B). The surface motion provides both
biomechanical and somatosensory challenges. Amplitude and velocity capacity also vary from sys-
tem to system. Both systems shown here provide omnidirectional motion. A, Biodex Balance System
SD measures the motion on the surface, B, Proprio Reactive Balance System uses motion sensors
on the body placed at the level of the COG (A, Courtesy Biodex Medical Systems, Shirley, NY;
B, Courtesy Perry Dynamics, Decatur, Ill.)
should be modified so that it can be safely performed with- necessary to accomplish this; the conscious brain is focused
out needing to hang onto a stable object. The community on accomplishing some other goal(s) and thus balance con-
setting is ideal for postural control gait training. Grocery or trol must be achieved at a less conscious level. Alternative
library aisles, public transportation, elevators, escalators, tasks can be physical in nature, such as carrying a tray or
grass, sandboxes or beaches, ramps, trails, hills, and varied dribbling a basketball, or cognitive, such as conversing or
environmental conditions in general provide both challenge solving verbal or math problems, or a combination of phys-
and functional relevance. ical and cognitive demands.
This objective is not universal. Clients with permanent or
Concurrent Tasks progressive deficits in automatic motor processing, particu-
Normal balance is largely subconscious. One objective in larly those with Parkinson disease, lose automaticity. They
balance retraining is to force the nervous system to solve must learn to produce motor actions volitionally, with attention
postural control problems at the automatic, subconscious and intention, unless there are external sensory cues to drive
level. A great deal of practice and dual-task training are the motor system.
A B
Figure 22-22 n Advanced technology to support balance and gait retraining. Visual motion
may provoke instability for certain individuals. Some systems provide the ability to generate visual
motion. A, SMART Balance Master is a system with a 3-sided booth with unidirection motion
combined with a unidirectional movable forceplate. Both visual and somatosensory inputs can be
manipulated for testing and training. B, Stimulopt Optokinetic Ball. Omnidirectional visual motion
can be produced by rotating display systems that are used in a dark room. (A, Courtesy NeuroCom,
a division of Natus, Clackamas, Ore; B, courtesy Framiral, Cannes, France.)
occur because of a combination of intrinsic and extrinsic risk

Fall Prevention factors. For example, a client with hemiplegia who has lim-
Client safety is always paramount; for any client with bal- ited ability to rapidly and maximally dorsiflex the ankle who
ance deficits the risk of falls is increased and must be ad- encounters a trip hazard such as a rumpled doormat may not
dressed in every clinical management program. In older be able to clear the obstacle by lifting the hemiplegic leg and
adults without documented neurological conditions, falls are foot quickly. If the number and/or severity of intrinsic risk
prevalent and lead to severe injury (including head trauma) factors is great, falls may occur without any provoking ex-
and death. Fall risk is even greater and is especially high in trinsic hazard.
persons with stroke and Parkinson disease. Fall prevention Additional factors influence fall risk levels. The location
is a critical primary objective for clinicians serving clients of the client may have an effect on fall risk. While in an in-
with neurological conditions who have impaired balance stitutional setting, the physical environment may be safer
and gait. and the level of supervision and assistance higher, thus low-
Fall risk factors are categorized as intrinsic, relating to ering risk. Yet if the surroundings are unfamiliar or confus-
the individual, and extrinsic, relating to the environment. ing to the client, the client does not remember to call for
Intrinsic risk factors include but are not limited to medical assistance before getting up, or the environment holds barri-
conditions (e.g., stroke, Parkinson disease); medications; ers such as bedrails, wheelchair footrests, and so on that
impaired balance and gait; somatosensory, visual, or vestibular must be dealt with when hurrying to the bathroom, then an
sensory loss; central processing problems; slow reaction time institutional setting may pose increased risk. The amount of
and other central motor deficits; lower-extremity weakness supervision or assistance the client receives may alter risk
and decreased ROM; cognitive deficits; depression; urinary level. Confused or forgetful clients in facilities with high
urgency or incontinence; and footwear. Extrinsic risk factors staff-to-client ratios or who have family or caregiver super-
are hazards in the environment, such as inadequate lighting vision most of the time will have lower risk than those in
or excessive glare, slippery or cluttered surfaces, lack of facilities or homes where supervision and assistance are
handrails or grab bars, attention distracters, and timing de- sparse. Lastly, the relative dependence-independence level
mands (e.g., hurrying to answer the phone). The more risk of the client, both physical and cognitive, affects risk level.
factors present, the greater the likelihood of falls. Most falls Very dependent clients who cannot get up by themselves,
in community-dwelling older adults are trips and slips and and very independent clients with high-level balance and
A B C
Figure 22-23 n Advanced technology to support balance and gait retraining. High technology used
to challenge gait. The ability to challenge balance during gait training is improved if the client is secure
in an overhead harness system. These systems allow hands-free training to increase reliance on the
lower extremities and trunk reactions critical for balance recovery strategies. Rapid and recordable
measurement of gait characteristics is possible with instrumented systems. A, Biodex FreeStep SAS
uses overhead harness system to challenge balance during gait. B, GAITRite Portable Walkway System
challenges gait during overground walking. C, Biodex Gait Trainer 3 with Unweighting System is
another example of how to challenge balance during gait training. (A and C, Courtesy Biodex Medical
Systems, Shirley, NY; B, courtesy CIR Systems, Havertown, Pa.)
gait skills, are both at lower risk than clients in the middle the concrete parking lot without getting her arms out in time
of that spectrum. Clients who have sufficient ability to get to protect her would have a higher risk of injury.
up out of the bed or chair, and perhaps to walk, but who have Clinicians should consider fall risk and injury risk factors
impaired balance and gait skills and poor judgment or as they carry out their assessments and evaluation. This
memory are at a much higher risk level. would begin with the chart review or history taking; as prob-
A separate but equally important risk to consider is the lems are noted, the clinician should be “red-flagging” those
risk of injury from a fall. Injury risk also depends on both that are risk factors for falls. For example, you might note
intrinsic and extrinsic factors. Clients with low bone mineral that the client is on more than six prescription medications;
density, low body mass, and impaired protective responses polypharmacy is a risk factor for falls. You also note that one
(automatic postural responses, especially reaching or proof the medications is a drug to remediate bone loss, and
tective extension) are more likely to be injured. Falls that further inquiry reveals that the client does have a diagnosis
occur from a greater height onto a harder surface are more of osteoporosis, a risk factor for injury. During your own
apt to result in injury. An overweight client with adequate therapy assessment, you find substantial lower-extremity
bone mineral density (BMD) who, while in her yard garden- weakness, balance impairments, and gait limitations requir-
ing, stumbles and falls to the grassy ground from standing ing the use of an assistive device, all major risk factors for
height with both arms out to break her fall would have a falls. One of the identified balance impairments includes
lower risk of injury. A thin client with osteoporosis who, deficient automatic postural responses, a risk factor for in-
while at the store shopping, stumbles off a curb and falls to jury. Later, during a team meeting to discuss the new client,
you learn from the occupational therapist that the home should then have a frank discussion about recommended
safety survey completed by the client’s spouse indicates home safety modifications. The clinician should convey
numerous safety hazards, extrinsic risk factors for falls what is recommended and why, highlighting the benefits.
and perhaps injury. For fall prevention and injury prevention However, factors such as time, expense, and personal prefer-
purposes, a list of all fall and injury risk factors pertaining ence also influence client and family decisions. Identifica-
to that client should be generated for use in treatment tion of barriers to safety modification implementation is
planning. helpful and may lead to solutions that permit initial resis-
The aim of intervention for fall prevention is to eliminate tance to be overcome.
or minimize risk factors, with emphasis on four risk factors Vision management is critical for any client with visual
that appear to be more influential than others (in community- deficits. (Refer to Chapter 28 for a discussion of disorders of
dwelling older adults). These four interventions are exercise, vision and visual-perceptual dysfunction.) These visual im-
medication management, home safety modification, and pairments might be at the peripheral level, such as macular
vision management. The single best intervention for fall pre- degeneration, or the central level, such as homonymous
vention is exercise—specifically, individualized exercises hemianopsia. Occupational therapy is recommended for a
that target balance, gait with balance challenges, and leg visual-perceptual evaluation and potentially for low-vision
strength. The challenge level of the balance and gait exer- rehabilitation if needed. Vision professionals (ophthalmolo-
cises should be high. The balance and gait training program gists, developmental optometrists), preferably those with
must be of high intensity and frequency and of long duration. specialization in neurological populations if indicated (e.g.,
For a reduction in fall rates in community-dwelling older TBI, cerebrovascular accident [CVA], MS), should also be
adults, a bare minimum of 5 to 6 weeks, with sessions two to involved. Objectives include maximizing vision for the cli-
three times a week, is required. For more neurologically in- ent and including visual support within the home safety
volved clients, the overall amount of practice would need to modification plan if needed.
be greater. Gains that are made during therapy will not be Footwear assessment is important. Walking indoors bare-
maintained unless exercise or physical activity that includes foot or in socks is associated with increased fall risk. The
balance challenge is continued after therapy. Clients should footwear most highly associated with hip fractures is slip-
be intentionally transitioned from therapy to a community- pers. Shoes and slippers that do not provide adequate foot
based balance exercise or physical activity program as an support, or that have slick soles, are unsafe and not recom-
integral part of their discharge plan. Clinicians may consider mended. Footwear lacking a secure back (flip-flops, mules,
doing their last treatment or two at the community-based or sling-backs), high-heeled shoes, and platforms are poor
program to support the client through the transition and choices for clients at risk for falls. Running shoes with very
increase the probability of follow-through. It is critical for thick, cushioned soles and a heavy tread are also not ideal.
clients to persist with physical activity to maintain or even The optimal shoes for fall risk reduction are well fitted with
further lower their fall risk level. thin, hard soles. Shoes with a tread sole and a tread beveled
The second area of intervention is medication manage- heel are more stable on wet or slippery surfaces.118 Just as
ment. This requires a team approach and tactful, profes- with home safety modifications, factors such as expense,
sional communication with the client’s physician(s). The habit, and personal preference may create obstacles to client
goal is to have the client take as few medications as possible, adoption of suggested footwear changes. These obstacles
in the smallest doses possible, and to eliminate or when should be recognized, respected, and addressed directly with
necessary replace certain drugs that are known to raise the professional communication strategies designed to facilitate
risk of falls substantially (e.g., benzodiazepines). (Refer to positive behavior change.
Chapter 36 for a discussion of the impact of drug therapy Clinicians should assume that clients at risk for falls will
on patients undergoing neurological rehabilitation.) Clini- fall when they are discharged home. Though we work to
cians must understand that medication management for ensure this will not happen, we also prepare for the possibil-
fall prevention is a difficult balancing act for the physician. ity that it will. Clients who are able must be taught how to
For example, antidepressants and sleeping pills raise the risk get up from the floor independently, with and without furni-
of falls. Yet depression and sleep disorders are serious con- ture if the latter is possible. If clients cannot get up from the
ditions with many negative effects, and depression, inatten- floor by themselves, then family members or caregivers
tion, and fatigue are all risk factors for falls. Both the condi- should be taught how to assist clients to get up from the
tion and its treatment increase risk! Clients on blood-thinning floor. This may be as simple an act as bringing a chair close
medication who are at risk for falls are also at risk for to the client so the client can use the chair to get up indepen-
serious bleeding problems should a fall occur; the physician, dently. Clients at risk for falls who will be home alone for
client, and family or caregiver should all be alerted to extended periods of time would benefit from a wearable
this risk. Medication management guidance for fall preven- home alerting system. If such a system is cost-prohibitive,
tion directed to physicians is available from the American the client should develop the habit of carrying a cell phone
Geriatrics Society. at all times. For clients without cell phones, a landline phone
Home safety modification is an effective intervention for should be left on the floor or a chair seat so that it is within
those who are already at high risk for falls. Ideally an in- reach from the floor should a fall occur. Older clients with
person home safety evaluation is performed by a trained osteoporosis should consider wearing hip protectors. Hip
professional, usually a physical or occupational therapist. If protectors do not reduce the risk of falls but when properly
this is not possible, a home safety survey may be completed fitted and worn may reduce the risk of hip fracture. Adoption
by a reliable source (client, family member, or caregiver). of and adherence to wearing hip protector apparel is typi-
The clinician and client or their responsible decision makers cally low and requires commitment and effort.
The combined aim of balance retraining and fall preven- The role of the vestibular system is to maintain clear
tion is to assist the client to become as active as is safely vision during head motion as well as to orient head and
possible. With improved balance and gait skills, the client trunk in space with respect to gravity when the visual and
achieves higher levels of function and physical activity. With surface references are not sufficient. Horizontal and vertical
attention to and emphasis on fall prevention, safety is main- accelerations, as in riding in a car or an elevator, are
tained, injury is prevented, and the opportunity for improved also detected by the vestibular otolith mechanism as seen
quality of life is preserved. in Figure 22-24.121 The vestibular system is critical for
postural control because it uniquely identifies self-motion
as different from motion in the environment. Recognition
Vestibular System of self-movement as it relates to visual movement can be
Kenda Fuller, PT, NCS disrupted momentarily in a normal individual experiencing
unexpected movement in the peripheral visual environment.
This is a common sensation noticed, for example, when the
OVERVIEW: THE ROLE OF THE VESTIBULAR car next to you moves backward, and you press the brake,
SYSTEM thinking that you are rolling forward. Unless the system
The CNS integrates the information from visual, somatosen- fails, the vestibular system is noticed only when it is stimu-
sory, and vestibular inputs to determine the most appropriate lated beyond the level at which it is typically activated, as
response to maintain stability and homeostasis.119 These in a fast spin or the drop of a roller coaster. The dizziness
three senses play an important role in dynamic equilibrium that occurs in the normal individual when the vestibular
(Figure 22-24). In fact, the somatosensory system is neces- system is overstimulated is reflective of the dizziness that
sary to interpret vestibular information.120 The activity of the occurs when the brain encounters sudden changes or losses
vestibular system must be recognized in order to interpret of input from the vestibular system.122
balance testing. This chapter provides background informa- Disorders of the vestibular system can cause devastating
tion that has been incorporated into the earlier portion on lack of visual stability, loss of balance, and inaccurate sense
balance. of movement. There is an initial loss of trunk and gaze stabil-
ity with vestibular dysfunction that improves as a result of
CNS adaptation. The CNS adaptation is critical to recovery of
Acceleration Tilt function.123 In the course of recovery, the visual or somato-
sensory systems may be chronically used in preference to
the vestibular system, causing abnormal sensory dependence
Anterior-Posterior patterns.123,125 Comorbid dysfunction can affect functional
recovery, especially if it affects the visual or somatosensory
inputs. Prior trauma, either physical or psychological, can
cause maladaptation resulting in responses to intervention
that are inconsistent with typical recovery patterns.126-128
Clinicians are exposed to patients at many different levels
of adaptation, from the ones who show adequate adaptation
Upright
with minimal intervention to those who have recovered only
limited independence after disruption of vestibular system
inputs. Successful intervention is achieved by accurately
analyzing both the missing and the available components of
the system, facilitating adaptation, avoiding excessive sen-
sory substitution, and determining appropriate compensa-
Lateral tory strategies.129 If maladaptation is not understood and
treated properly, it can lead to frustration for the patient and
Lateral Tilt clinician, resulting in less than optimal outcomes.
(Roll)
VESTIBULAR SYSTEM DISORDERS
There are many types of common vestibular disorders. This
chapter cannot provide a total discussion of all these disor-
ders. They have been summarized in Appendix 22-A.
Fore Tilt RECOVERY OF FUNCTION: NEUROSCIENCE

(Pitch) OF THE VESTIBULAR SYSTEM
Recovery of function is related to the mechanism of injury
Occipito-caudal and where the damage is located. In order to understand how
the system recovers, we must first understand how it works.
Figure 22-24 n The otoliths register linear acceleration and The end organ of the vestibular system is basically a me-
static tilt of the head. (From Hain TC, Ramaswany TS, Hillman chanical, fluid-filled system activating afferent signals that
MA: Anatomy and physiology of the normal vestibular system. In travel through the vestibular nerve to the brain stem nu-
Herdman SJ, editor: Vestibular rehabilitation, ed 3, Philadelphia, clei.130,131 Figure 22-25 shows the relative relationship of the
2007, FA Davis.) canals to the otoliths and the cochlea. This demonstrates
Semicircular Vestibular (Reissner’s) membrane

canals Perilymph space
Endolymph
(within membrane) Cochlear
Scala
vestibuli duct
Ampulla
Vestibular nerve
Utricle
(in vestibule) Modiolus
Cochlear nerve
Saccule
(in vestibule)
Oval window Scala

tympani
Round window Cochlea

S Tectorial Basilar Hair Supporting
Cochlear membrane membrane cells cells
L M duct
I Organ of Corti
Figure 22-25 n Components of the vestibular system and cochlea with distribution of neural con-
nections. (From Thibodeau GA: Anatomy and physiology, ed 6, St Louis, 2006, Mosby.)
why there is often a connection between loss of hearing and When there is disruption of signal from one side of the ves-
vestibular dysfunction, especially when the fluid mechanism tibular pathway, it will change the relative input into the CNS,
is part of the impairment. Box 22-1 describes the sensory resulting in a perception of the head rotating toward the intact
components of the vestibular system. At the level of the side when the head is not actually moving (Figure 22-26).
nuclei, the brain stem receives input from the other sensory Initially, as well, there will be a phenomenon of spontaneous
systems related to orientation of the head and body. The nystagmus, a reflex-driven movement of the eyes. In an acute
combined input is further modulated by the cerebellum, asymmetrical vestibular system disruption, the eyes will
providing further calibration. Purkinje cells in the cerebel- move away from the perceived direction of head motion, and
lum provide inhibitory control of the vestibular nuclei. The that movement of the eyes will cause a sensation of dizziness
flocculonodular lobe and medial zone of the cerebellum with the head still, eyes open. The brain quickly identifies this
affect postural control.132 Input continues to the cortex via as an abnormal state and begins CNS recalibration so that the
the vestibular projections (Box 22-2). vestibular system input from each side becomes calibrated
to match the visual and somatosensory system input. The
Recalibration at Rest system is able to determine that despite the uneven signals,
The tonic firing of the vestibular system when the head is in a the head is not really moving.133 There is usually adequate
neutral nonmoving state is symmetrical on both sides of the central adaptation to stop the spontaneous nystagmus in a
system at approximately 50 spikes per second. The brain is lighted environment within 3 days.134 The spontaneous nys-
able to compare this symmetrical resting level tone to the in- tagmus may continue to be active in a dark room, and there
formation coming from the visual and somatosensory sys- may still be a sensation that the head is rotating when the eyes
tems’ feedback about the position and movement of the head. are closed for weeks after the insult. Increasing somatosen-
sory input about stability can help in the central recalibration
process. Input through the joint surfaces by establishing a
stable joint reference can facilitate calibration. This appears to
n POTENTIAL LOCATION OF LESIONS
BOX 22-2
be most effective through mechanical pressure through the
THAT MAY AFFECT THE VESTIBULAR SYSTEM
top of the head or with the use of weights on the shoulders to
n Vestibular end organ and vestibular nerve terminals increase the vertical reference of the spine in a neutral posi-
n Vestibular ganglia and nerve within the internal auditory tion. Figure 22-27 shows how weights are placed on the
canal shoulder to provide somatosensory input. The brain can then
n Cerebellopontine angle match or recalibrate the abnormal perception of head move-
n Brain stem and cerebellum ment induced by the inaccurate vestibular system to the cor-
n Vestibular projections to the cerebral cortex rect reference of the stable somatosensation. It is critical in
the rehabilitation process to achieve accurate CNS recalibra-
From Goodman CC: Pathology: implications for the physical therapist, tion with the head at rest before initiating intervention that
ed 3, Philadelphia, 2008, WB Saunders. requires movement of the head.
Striola
Otoconia
Kinocilium
Otolith
membrane
Receptor cell
Utricular
maculae
Gravity 50 spikes/sec 50 spikes/sec

A
Otolith afferent discharge rate

Left tilt
Ocular
counterroll
10 spikes/sec 90 spikes/sec
B
Right tilt
90 spikes/sec 10 spikes/sec
C
Figure 22-26 n Patterns of excitation and inhibition for the left utricle and saccule when the head is
upright (A), tilted with the left ear 30 degrees down (B), and tilted with the right ear 30 degrees down
(C). The utricle is seen from above and the saccule from the left side. (From Haines DE: Fundamental
neuroscience for basic and clinical applications, ed 3, Philadelphia, 2006, Churchill Livingstone.)
Head Movement and Gaze Stability the head movement, and the gain remains 1:1. Figure 22-28
As the head starts to move, the signal from each part of the shows the neural connections involved in activation of the
vestibular system activates as a result of fluid movement VOR. If the vestibular system does not drive the eyes to the
against the cupula. The direction of movement is determined correct position for stable gaze, the result is vestibular-
by the relative firing pattern of the vestibular system from driven oscillopsia, and again, objects can appear to move as
each side of the head. The vestibular system on the side the head moves. This disorder has significant functional
toward the movement increases in firing, and the side implications and works in conjunction with both smooth
opposite decreases its firing rate. The resulting signal to the pursuits and saccades to interpret the relationship of the
brain stem drives the VOR to move the eyes in opposition to body to the environment (Figure 22-29).
Medial rectus Head movement

muscle
Lateral rectus
muscle
Oculomotor
nucleus
Abducens
nucleus
Horizontal
semicircular
canal
Vestibular
nuclei
Figure 22-28 n Vestibuloocular reflex. When the head is turned
to the right, inertia causes the fluid in the horizontal semicircular
canals to lag behind the head movement. This bends the cupula in
the right semicircular canal in a direction that increases firing in the
right vestibular nerve. The cupula in the left semicircular canal
bends in a direction that decreases the tonic activity in the left
vestibular nerve. Neurons whose activity level increases with this
movement are indicated in solid lines. Neurons whose activity
level decreases are indicated in dotted lines. For simplicity, the
connections of the left vestibular nuclei are not shown. Via connec-
tions between the vestibular nuclei and the nuclei of cranial nerves
III and VI, both eyes move in the direction opposite to the head
turn. (From Lundy-Ekman L: Neuroscience: fundamentals for re-
habilitation, ed 3, Philadelphia, 2007, Saunders.)
reference of head movement and position so that the per-

ceived movement in the environment is properly identified.
The quality of somatosensation in the spine and muscles
of the upper body can contribute to dizziness with head mo-
tion. The vestibular nuclei have the job of integrating so-
matosensory information on its way to the cortex. There
must be adequate input from both systems to distinguish
Figure 22-27 n Use of 5-pound weights on each shoulder to between head-on-body and body-on-head movement. Im-
increase the somatosensory reference allows the vestibular system paired somatosensation, pain, and guarding of movement
to recalibrate to the body reference. Eyes are closed to so that head will disrupt the accuracy of calibration related to head
position is not referenced by vision. movement. The patient who has an abnormal VOR will be
constantly decelerating head movement to less than 2 Hz in
order to prevent blurred vision. That unconscious decelera-
tion by the muscles in the neck can cause stiffness and de-
The VOR is reported as abnormal only if there is loss of crease the sensitivity of the somatosensory mechanisms in
gaze stability, or blurring of target objects with head move- the neck. Two simple tests will alert the therapist to abnor-
ment at 1 to 3 Hz. Rotation or pitch of the head during test- malities of head motion related to the quality of somatosen-
ing of the VOR may cause dizziness because of the differ- sation. Holding the head upright and still, in gravity-neutral
ences in firing patterns from each ear that are not yet position, while the patient rotates in a chair (body on head)
efficiently calibrated. This is especially noted when the head provides information about the somatosensory reference.
is moving in the direction of the abnormal ear. It is impor- The movement may elicit dizziness if the somatosensation is
tant to note that head motion–provoked dizziness can persist impaired. If the patient has been relying on somatosensation
even when gaze stability has normalized. as a primary reference for head position, there may be resis-
The clinician must also be aware that the peripheral vi- tance or guarding against allowing the body to move in a
sual field will appear to move in the opposite direction as direction different from the direction of the head movement.
head movement during these testing procedures. This nor- Rotating in a chair at 1 to 3 Hz and allowing the head to
mal visual phenomenon can cause dizziness in the patient move at the same speed as the body will eliminate somatosen-
with visual dependence or visual motion sensitivity (de- sation reference through the neck receptors and isolate the
scribed later). It is the vestibular system that provides the vestibular system response to rotation of the head. This should
Dynamic Acuity Ranges
Walk
Run
Drive Car 30mph
Sports
0 50 100 (deg/sec)
Pursuit
VOR
Saccadic
Figure 22-29 n Dynamic acuity ranges. (From NeuroCom beta-testing data and analysis (2004-
2008), used by permission.)
be done with the eyes closed to eliminate the sense of visual to gravity is most critical when balancing on unstable sur-
motion. Movement at this speed will cause an increase in diz- faces when vertical and horizontal visual reference is not
ziness even in a normal system, but it should resolve in less than adequate. The vestibular system provides a top-down refer-
10 seconds. If the dizziness persists for longer than 15 seconds, ence for the head and trunk stability in line with gravity
it is considered to indicate abnormal vestibular calibration. while the leg segment is coordinated to maintain surface
reference. Vestibular inputs are critical to determine whether
Head Position Changes the body is swaying or the surface is perturbed.138 The con-
Head position changes in reference to gravity can cause diz- scious perception of verticality used to orient to gravity when
ziness as a function of the vestibular system under certain the support surface is perceived to be unstable is provided by
circumstances. The most common form of head position the vestibular system. Vestibular inputs are used in order to
dizziness in adults is BPV (see Appendix 22-A). In this recognize the changes in angle of the support surface.139-141
condition, debris (otoconia) from the utricle moves into the Surface perturbations or oscillations provide a method to
semicircular canal, and there is suddenly mass in a system examine the ability of the vestibular system to maintain the
that is designed to calculate only fluid pressure changes in head and trunk in gravity-neutral position as the legs move
response to head movement. The added mass causes exces- in reference to the platform movements. When standing
sive deflection of the hair cells in the cupula when the head on a surface that tilts both anteriorly and posteriorly at
is moved into a gravity-dependent position. The otoconia 4 degrees per second, patients with bilateral vestibular loss
move in the direction of gravity through the endolymph, will lose balance and fall.120,140,141 A normal person should
causing a pull on the cupula and increased firing of the hair be able to position a hand-held rod in vertical while standing
cells as if the head were moving quickly in that direction. or sitting on an oscillating surface, irrespective of the angle
Figure 22-30 shows the movement of the otoconia in rela- of the surface, even with eyes closed. Individuals with loss
tionship to head movement in a gravity-dependent state. The of orientation to gravity will orient the hand-held rod with
brain activates the VOR in response to the message that the respect to the angle of the moving surface.142
head is moving quickly, and there is nystagmus based on the It is important to remember that at the same time the
same mechanism as described previously. The nature of this vestibular system is activated in this moving surface condi-
nystagmus reflects the canal in which the debris is floating. tion, the somatosensory system is still providing feedback
As soon as the otoconia come to rest, the pressure on the about the relationship of the head that is provided by ves-
hair cell is gone, and the nystagmus subsides. This takes tibular inputs to the base of support.2 Resulting patterns of
about 20 seconds. There is no nystagmus until the head is muscle activation reflect vestibular and somatosensory inte-
moved into another gravity-dependent position causing the gration to maintain continuous upright postural control. As
otoconia to roll through the canal. a surface rotates to greater degrees, there is more weighting
of the system away from the somatosensory reference to-
Top-down Reference for Postural Control ward the gravity reference.138,143 Patients with noncompen-
The most important role of vestibular information for pos- sated vestibular dysfunction will report lack of awareness of
tural control relates to orientation of the head and trunk in angle changes when standing on an oscillating support sur-
space with respect to gravitational forces.135-137 Orientation face. Therefore, instead of changing the ankle angle to
Posterior
semicircular
canal
A B
Figure 22-30 n The Hallpike-Dix maneuver. A, Starting position with head rotated toward the
side to be tested. B, Lowering the patient’s head backward and to the side allows debris in the pos-
terior canal to fall to its lowest position, activating the canal and causing eye movements and vertigo.
(From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 3, Philadelphia, 2007,
Saunders.)
adjust to the tilt, the torque around the ankle remains locked, muscles in the neck. When the vestibular system function is
holding the leg, trunk, and head at 90 degrees to the surface. missing or inaccurate, there is abnormal muscle activation in
The head then follows the direction of the surface tilt as seen the muscles of the neck.120 One way this function can be
in Figure 22-31. As the surface tilt angle exceeds 8 degrees, tested is by observing head-righting response when a patient
the individual who cannot activate gravitational reference or has vision blocked and is tilted while sitting on a tilt board
adjust the ankle angle will be unable to maintain balance.143 so that the perturbation starts at the surface instead of the
Lateral tilt can activate the system in the same way; with head. In the patient with vestibular dysfunction, when grav-
the head following the direction of the downward tilt, ity vertical reference is lacking, lateral head righting back to
the weight is typically shifted to the downward-most leg. center, or neutral is inadequate. This can be a result of co-
Patients report lack of awareness of the movement gener- contraction of the neck muscles to lock the head into posi-
ated. Figure 22-32 shows the abnormal shift of weight as the tion, with the trunk maintaining the same tilt as the trunk.
patient attempts to orient to the surface rather than gravity. The head stays in reference to the surface in much the same
way as described earlier regarding locking of the ankle
Bottom-up Reference for Postural Control joints. This is evidenced in higher-level activities that re-
The somatosensory system can determine the orientation of quire gravity reference such as tandem walking in visually
the head in reference to the surface through cutaneous, pro- stimulating environments or with vision occluded. As the
prioceptive, pressure, and stretch receptors of the muscles patient tries to use somatosensation to determine head posi-
and joints, primarily related to pressure through the balls of tion instead of gravity reference, the head becomes locked
the feet. Although overreliance on this surface reference can onto the trunk through abnormal muscle activation. The nar-
be destabilizing in some conditions, it contributes to balance row stance limits the contribution of surface reference in the
when the surface is stable or moving slowly (at less than lateral plane, and the patient must take a step out to control
4 degrees per second). At the other end of the spectrum, in the excessive lateral tilt of the trunk and head. Figure 22-33
very fast oscillations the muscle spindles provide stabilizing represents the abnormal and normal views of head righting
information that can contribute to head and trunk stability.144 in both sitting on a tilted surface and standing in tandem. It
It is critical to remember that the vestibular spinal system is the vestibular inputs that should drive the appropriate
also activates the neck muscles in response to head perturba- head righting and resulting postural strategies that are re-
tions and modulates somatosensory-driven activity of quired in these conditions.
A B
Figure 22-32 n A, The patient references her trunk to the plat-
form, shifting weight downhill to the downhill leg. B, The patient
has referenced her trunk and head to gravity, resulting in improved
postural control. (Courtesy Perry Dynamics, Decatur, Ill.)
and the surface, the patient often will report feeling light-
headed or having the sense of floating. When somatosensory
inputs from the neck are reduced, absent, or distorted, the
result is poor spinal segment stabilization. Excessive muscle
activity, including co-contraction of the sternocleidomastoid
(SCM), levator scapulae, upper trapezius, and superficial
neck extensors indicates poor stability, altered afferents, and
recruitment patterns that are ineffective. Nociception from
cervical segments can create “noise” in the postural control
system contributing to dysmetric postural responses and
nausea. Impaired cervical afferents will cause changes in
cadence and length of stride when neck motion is introduced
to gait. Diminished gait measures have been seen on the
DGI in the presence of neck pain. When abnormal somato-
Figure 22-31 n In anterior tilt of the platform, the head and sensation is concurrent with CNS vestibular adaptation, the
trunk follow the reference of the platform rather than maintaining result is less than satisfactory. Because brain pattern learn-
a gravity-neutral position. This is reported as surface reference. ing is task specific and dependent on high repetition, it is not
(Courtesy Perry Dynamics, Decatur, Ill.) helpful to practice poor motor recruitment patterns during
balance retraining.
Having even the slightest touch reference so that the so- Visual Reference for Postural Control
matosensory system can orient the trunk through upper- Orientation of the head in space is possible through predic-
extremity joint position sense is another way to substitute tive control of vision. A stable environment provides visual
somatosensation for vestibular reference.145 The position of vertical and horizontal references for balance. Patients with
the head and trunk can be determined by this touch even vestibular loss are able to substitute vision for vestibular
when the vestibular system function is missing and eyes are reference, even during surface perturbations.146,147 Destabili-
closed. Because the arm stabilizes the trunk more than the zation occurs when the peripheral visual references are mov-
legs do, reaching for a stable surface is a common way to ing slowly or are not in alignment with gravity. When eyes
maintain balance when challenged. The therapist must rec- are tracking something moving in central gaze field, the
ognize when the patient is using this touch reference to background or peripheral visual field will appear to move in
substitute for gravity. This is why allowing a patient to touch the opposite direction. In the patient with lack of gravity
a stable surface during balance training should be quickly reference, when performing diagonal smooth pursuits, pos-
eliminated from intervention to avoid dependence on sur- tural adjustment patterns are activated as if the room were
face reference when attempting to activate the vestibular tilting, because that is the dominant sensory reference being
system. used. The patient is pulled off balance when aligning himself
When the brain is not able to use somatosensation to or herself with the apparent visual vertical. This is most pro-
identify the relationship between appropriate body segments nounced and can be tested easily when a patient is standing
on a compliant surface, tracking a target moving in a figure-

of-eight configuration. Head and trunk sway match the ap-
parent visual field movement instead of actual gravity verti-
cal in the patient who is visually dependent. This test can be
used in a clinical battery to determine the level of visual de-
pendence or substitution related to lack of gravity reference.
Figure 22-34 shows the effect of visual dependence on head
position and the resulting balance responses.
Visual disorders can disrupt balance, cause sensation of diz-
ziness, and lead to limitation of function. Accurate evaluation of
the visual system is critical in differential diagnosis (see Chapter
28 on disorders of vision and visual perception). Disorders of
convergence are common in the vestibular-deficient patient and
can cause delay in the process of adaptation. Sensitivities to
physiological diplopia develop, causing visual motion hypersen-
sitivity during daily activity (Box 22-3). Remember, too, that
abnormal responses in neck muscle activity associated with eye
motion have implications for control of posture and movement.
The smooth pursuit neck torsion test (SPNT) is used to delineate
abnormal cervical afferent influences on oculomotor function
A B from vestibular influences (Box 22-4).
A B
Figure 22-34 n When the visual reference is dominant for head
position, the position of the head changes to match the tilted pe-
ripheral visual reference that results from the eye following the
thumb in a figure-of-8. A, The head tilts off center as the percep-
D tion of the visual field tilts. B, If the vestibular system is dominant,
suppression of the apparent shift in the visual field allows the head
Figure 22-33 n A, When surface reference is used in preference to stay in alignment over the base of support. (Courtesy Ray
to vestibular cue, the head remains in alignment with the surface. Hedenberg, IRB Solutions, Silverthorne, Colo.)
B, As the patient is able to regain vestibular function and gravity
reference, the head remains in alignment with gravity, as a head-
righting response. C, Lack of head-righting responses can cause BOX 22-3 n PHYSIOLOGICAL DOUBLE VISION
excessive use of upper-extremity activity to try to reference to the
surface, with bottom-up firing patterns; hip strategy is activated but n Everything in front of and behind the central focal point
inefficient. Abnormal reference patterns result in loss of balance in is perceived as double.
tandem stance with eyes closed. D, When the vestibular reference n The closer the focal point, the more distance appears to
is restored, the head remains over the feet, so balance is restored. be between the perceived double images.
This is why it is important to observe the angle of the head during n As the central focal point moves in space, the back-
examination of tandem stance or gait. (Courtesy Ray Hedenberg, ground image appears to move.
IRB Solutions, Silverthorne, Colo.)
BOX 22-4 n SMOOTH PURSUIT NECK TORSION n ENVIRONMENTAL CONDITIONS

BOX 22-5
TEST (SPNT) THAT MAY CAUSE VISUAL MOTION
HYPERSENSITIVITIES
Test: Smooth pursuits tested in head neutral and then
compared with head rotated right and left Grocery stores—cans and boxes appear to be moving
Findings: Compare gain in three head positions backward when one walks down isle
Considerations: Shows relationship to cervical pain, Airports with moving sidewalks
proprioception, and oculomotor control Malls with open walkways and glass elevators
Disco lights
Escalators, if the “down” and “up” escalators are side by
side
Sensory Substitution for Postural Control Department Stores, especially during holidays or when
When, as noted several times earlier, critical information displays are moving
from one system is absent or inaccurate, the CNS will begin Large “box” stores where the ceiling is unusually high
to rely more heavily on the other systems for necessary ref- Large-format TV and movies, especially IMAX or 3D
erence. Although this is used initially to provide stability Walking outside when the wind blows the tree limbs
during the recalibration process, it can limit adaptation over Driving in rain or snow with windshield wipers active
the course of recovery.125 Visual or somatosensory depen-
dency patterns develop when the patient persistently makes
use of either or both of those systems in preference to ves-
tibular references when the most efficient reference for the sensory references. Avoiding provoking environments such
environmental condition would be the vestibular system. as airports or malls, wearing dark glasses inside, limiting
This phenomenon has been identified in individuals with driving, and using bracing techniques for balance are com-
unilateral vestibular loss, thought to be compensated for. On mon behaviors in this group of patients. Box 22-5 presents
testing there was an average of only 50% use of vestibular conditions that may drive hypersensitivities. These patients
system weighting (trunk in gravity neutral), resulting in have typically lost their jobs and generally underperform in
trunk sway in reference to the surface tilt, when tested on a life roles. Routine vestibular rehabilitation is usually not
rotating (tilting) surface at 8 degrees. Individuals with nor- successful. Unless sensitivities are identified at initial pre-
mal functioning systems showed 100% reliance on gravity sentation, a patient in this category may become recognized
by the time the surface made the 8-degree rotation, with only when several treatment regimens fail. Based on the
minimal head and trunk sway following the surface tilt.120 status of normal test results for individual systems, the pa-
Dependency patterns are typically observed in an indi- tient has often been told that there is nothing wrong and
vidual who does not recover satisfactory adaptation and in- therefore the problem is “in his head.” Indeed, this category
tegration of the sensory systems required for normal balance of patient often has an overlapping psychological disorder
responses in a variety of environmental conditions. Clini- that should be treated concomitantly. Anxiety, depression,
cally, these substitution patterns often manifest as hyperreli- posttraumatic stress disorder, and a history of physical or
ance on vision or somatosensory cues even when the ves- verbal abuse are common comorbidities that will affect in-
tibular system may have adequate potential for recovery. tervention and successful outcomes. It is sometimes possible
When given standard vestibular rehabilitation, these patients to identify the condition within the initial evaluation. These
often do not recover a full return to activity and are left with patients often report abnormal sensations with the head at
functional limitations or symptoms that have a negative im- rest with eyes closed, such as “flickers,” “explosions,” or
pact on their lives. They may experience discomfort every “racing,” that do not reflect vestibular or somatosensory
time they close their eyes or may find it impossible to walk deficits. These patients will often demonstrate fear of the
down an incline in a visually challenging environment. In- testing process but perform better on the higher-level tests of
creased vestibular weighting can improve the ability to ac- balance, such as eyes closed on foam, especially when dis-
complish ADLs and improve balance confidence.148 Inter- tracted by a concurrent mental task. Visual motion hypersen-
ventions are now possible that provide both the therapist and sitivity during oculomotor testing is common, and excessive
the patient with feedback regarding trunk movement during startle is often seen during tests of head righting.
surface perturbations.
Vestibular Contributions to Movement
Inadequate Use of Available Sensory Reference Vestibular system losses can result in motor responses that are
Central maladaptation is a term used for what appears to be larger than necessary for the task, which can predispose a pa-
a condition that limits the use of any of the sensory systems tient to falling. This can be seen in the gait cycle when the
so that each system seems to be inadequate for reference. body’s center of mass moves faster and farther than the indi-
The vestibular, visual, and somatosensory systems may test vidual can control. This movement appears similar to that of
normal, but the individual is not able to use them adequately the patient with cerebellar dysfunction (see Chapter 21), and
for functional activity. Patients report chronic subjective diz- indeed may represent the loss of vestibular input to the Pur-
ziness and have a tendency to develop hypersensitivities to kinje cells in the cerebellum that would normally modulate
stimuli in their environment.149,150 vestibular pathways.120,151 The earlier portion of Chapter 22,
Hypersensitivity patterns result in a patient who is intol- which discusses balance, describes automatic postural re-
erant of typical environmental conditions that include appar- sponses and describes balance testing in detail. It is important
ent visual motion, ground vibrations, or conflict between to understand the role of the vestibular system in movement
disorders related to balance. Patients with vestibular deficits Positional Nystagmus

typically rely primarily on their ankle strategy, which permits Positional nystagmus is induced by a change in head posi-
the head to remain aligned with the body and sustains congru- tion. Nystagmus caused by stimulation of the peripheral
ence between vestibular and somatosensory inputs, with semicircular canals from movement of the otoconia or cana-
the somatosensory system being the dominant reference. Use liths typically lasts up to 30 seconds and then dissipates.
of hip strategy may be modified or limited in the vestibular- Static nystagmus occurs with lesions to the peripheral oto-
deficient patient because when the head is moving in the lith system through connections in the vestibular nuclei and
opposite direction as the COG, vestibular and somatosensory cerebellum. It is provoked with change of head position in
inputs are not congruent.126 relation to gravity and continues as long as the position is
Activities that require use of hip strategy, such as stand- maintained, although it can fluctuate in frequency and am-
ing in tandem or on one leg, can be a problem for clients plitude. Nystagmus caused by central vestibular system
with bilateral vestibular loss or an uncompensated vestibular damage lasts minutes or longer before abating.
lesion.152 However, some patients demonstrate excessive use
of hip strategy on a level surface when an ankle strategy Gaze-Evoked Nystagmus
would suffice. This often is related to a maladaptation or Gaze-evoked nystagmus occurs when clients shift the eyes
somatoform dizziness. from a primary central position to a second location. It is
The vestibulospinal system is responsible for initiation of caused by the inability to maintain stable gaze position, and
muscle activity in the neck in response to perturbations of the eye drifts back toward the center or primary position.
the head as stated earlier. Abnormal integration of the ves- Usually indicative of a CNS problem, it is common in MS,
tibular responses can be seen in excessive co-contraction of brain injury, and congenital lesions.
the muscles of the neck when attempting to stabilize on
unsteady surfaces. Responses to postural perturbations that Video Nystagmography
start at the surface instead of the head may be exaggerated Video nystagmography (VNG) captures eye movements re-
and poorly timed in the patient with insufficient use of ves- lated to vestibular dysfunction using video goggles or elec-
tibular system input. Understanding the critical link between trodes surrounding the orbit of the eye. Oculomotor testing is
the sensory input and motor output of the structures in the performed to determine the ability to move the eye at normal
neck in relation to VSR activity is critical. Comorbid speeds. Abnormal responses can indicate central dysfunction.
dysfunction of the cervical spine can negatively affect the Another component of the VNG is the caloric test, in which
reactions driven through the vestibular and somatosensory warm and cold air are introduced into the external ear canal to
systems. manipulate the fluid in the horizontal canal to isolate the ear
and indicate the relative function on one side compared with
EXAMINATION OF THE VESTIBULAR the other. Central disorders will produce nystagmus patterns
SYSTEM AND DEVELOPMENT OF SPECIFIC that are different from those related to a peripheral lesion.
MOVEMENT DIAGNOSES
Assessment of eye movement control can help diagnose Vestibular-Evoked Myogenic Potential
dysfunction of the peripheral and central vestibular path- Vestibular-evoked myogenic potential (VEMP) is based on
ways. In particular, tests for a specific type of abnormal eye the principle that the saccule is sensitive to sound and re-
movement called nystagmus should be performed in clients sponds in a similar fashion to clicking sounds as it does to
with dizziness and those with known neuropathology in- tilt. A click produced in the ear stimulates the saccule, which
volving these pathways. Nystagmus is involuntary, rhythmic in turn inhibits the synchronous discharges of muscles in the
oscillation of the eyes, with movement in one direction SCM on the same side. The rate of firing or tone of the SCM
clearly faster than movement in the other direction. The is inhibited during the recorded sounds, and this change is
client with nystagmus will also usually report vertigo. There captured using surface electromyography (sEMG). The re-
is more than one type of nystagmus; identification of the sponse in one ear can be compared with the response on the
particular type can direct the clinician toward the area of other side in the same person. VEMP findings are consid-
dysfunction. Visually induced, optokinetic, and end-gaze ered to be abnormal when they are very asymmetrical such
nystagmus should not be confused with conditions having a that one side is more than twice as large as the other, low in
vestibular cause.134,153 amplitude, or absent. It is thought that this inhibition al-
lowed the head to turn reflexively to sound, as was critical
Spontaneous Nystagmus for survival of early humans.131 An abnormal VEMP can
Spontaneous nystagmus results from imbalance in the ves- indicate ipsilateral lesion in the saccule, the inferior vestibu-
tibular signals through their transmission to the oculomotor lar nerve, the lateral vestibular nucleus, or the medial ves-
neurons. This imbalance produces a constant drift of the tibulospinal tract. Conversely, dysfunction of the motor
eyes in one direction interrupted by brief, fast movement neurons of the SCM will cause abnormal findings that are
in the opposite direction. Spontaneous nystagmus occurs not related to vestibular disorder.
after acute vestibular lesions and usually lasts approxi-
mately 24 hours. Peripheral versus central lesions may be Subjective Visual Vertical
distinguished by asking the patient to fix his or her gaze on Subjective visual vertical (SVV) is used to test the degree of
a stable target. Nystagmus from peripheral vestibular lesions ocular torsion present in unilateral lesions. The SVV is
is easily inhibited with visual fixation. Nystagmus caused by tested in absolute darkness or an environment that prevents
central lesions of the brain stem or cerebellum is not easily visual reference to vertical. The patient is asked to orient a
inhibited with visual fixation. rod to gravity, and the degree of off-axis tilt represents
the torsion of the eye that is common in acute unilateral Vestibuloocular Reflex Cancellation
lesions.154 VOR cancellation reflects the ability to synchronize simul-
taneous eye and head movements in the same direction and
Gaze Stability and Vestibuloocular Reflex is associated with the ability of the brain to suppress the
The ability to hold the eyes fixed on a target while the head VOR. This function allows an individual to track an object
is moving is known as gaze stabilization. To test the accu- while moving the head at the same speed. Testing results are
racy of the vestibular system gain, the head is rotated or reported as normal if the eye can remain in the center of the
moved up and down at a rate of about 2 Hz. This is the rate orbit as the head and eyes track an object as it moves across
at which the head moves during typical daily tasks, moving the visual field. If the central integration capabilities are
up to about 3 to 4 Hz with activities such as sports. When an abnormal, the client will not be able to override the reflex
individual is unable to achieve similar clarity of vision at activity and cannot keep the eye and head moving at the
rest and at 2 Hz, it would be expected that the VOR is not same rate in the same direction.
sufficiently calibrated.155 If the image blurs, the gain of the
system is abnormal, meaning that the vestibular system is Gait
unable to move the eyes at the exact speed in the opposite Vestibular control of position for the upper body and head
direction as the head movement. The ratio of eye velocity to appears to be separated from the lower body in gait in a simi-
head velocity is known as the gain of the VOR. The gain of lar pattern as noted during perturbed stance. Head and trunk
an intact VOR is usually equal to one, which means move- stability remain constant throughout the phases of gait, and
ment of the eyes is equal to the movement of the head.156 vestibular inputs appear to be most critical during initiation of
Testing of dynamic visual acuity assesses the acuity that can gait, toe-off, and heel strike. Vestibular information contrib-
be obtained during a specific rate of rotation of the head. It utes to the planned foot trajectory and placement of the foot
can be tested with manual head turn using a Snellen eye to prevent disequilibrium. It is interesting to note that during
chart (the same chart that is used to determine visual acuity, steady-state gait, and even more so with running, vestibular
with normal vision recorded as 20/20) with the patient read- contribution appears to diminish in importance. This may be
ing the smallest line that is comfortable, then having the because running is so highly automated and the trajectory
head moving at 2 Hz in attempts to read the same line.157 remains fairly steady.157
When acuity drops more than three lines, it is clear that the The gait pattern reflected by vestibular dysfunction, or lack
patient will be unable to maintain visual acuity during typi- of integration, involves flat-foot gait with minimal heel strike,
cal daily activity. Quantified dynamic visual acuity can be abnormal foot placement requiring larger-than-normal trunk
recorded as the logarithm of minimal angle of resolution or adjustment. In order to control the position of the trunk, the
LogMAR. This can be tested and quantified by use of equip- base of support is widened. Speed of gait is another indication
ment such as inVision (NeuroCom International). Gaze of vestibular function from the perspective that patients with
stability can also be quantified using the same equipment, bilateral vestibular loss demonstrate a slower self-selected
but the measure is one of function, reporting the head speed speed. Typically, increased double-limb stance time and
that can be obtained while maintaining gaze stability. This is decreased stability at heel strike are present.158 Walking with
a good way to clarify the amount of deceleration that is head turns becomes even more challenging as the vestibular
necessary in order for the patient to maintain proper vision. system is activated and the somatosensory and visual systems
Figure 22-35 shows testing and treatment available using are disadvantaged. Vestibular contributions to stability during
quantified dynamic visual acuity. transitions from sitting to standing, initiation of gait, and
abnormal foot placement can be identified during standard
tests such as the TUG, the Tinetti, and the DGI. Scores
are adversely affected when vestibular system functions are
diminished. The FGA was developed specifically for use
with patients with vestibular disorders.159 For a more com-
plete description of tests of balance, see the earlier portion of
Chapter 22.
Movement diagnoses related to vestibular examination
are presented in Box 22-6.
BOX 22-6n MOVEMENT DIAGNOSES RELATED

TO VESTIBULAR EXAMINATION
Spontaneous nystagmus inhibited by fixation
Sensation of motion with body held still
Instability or dizziness during head motion
Loss of adequate head righting
Head position dizziness
Figure 22-35 n Quantified dynamic visual acuity is possible Dependency patterns
with systems such as inVision. (Courtesy NeuroCom International, Inadequate use of available sensory reference
Clackamas, Ore.)
INTERVENTION position. The client sits back up with the head tucked. Side
tilts of the head, as well as forward and backward move-
Positional Dizziness ments of the head and trunk, are avoided for 24 hours.164
The Hallpike-Dix maneuver is a positional test used to de- Position-provoked dizziness may alternatively be related
termine if otoconia are present in the posterior or anterior to canal sensitivity or abnormal firing through the brain stem
semicircular canal. Figure 22-30 demonstrates the position when BPV is not found to be the cause. In this case, exer-
of the head and movement of the otoconia. A positive re- cises should be done to increase the client’s tolerance to the
sponse is a delayed nystagmus of about 3 to 15 seconds, provoking position(s). This involves having the client per-
which determines potential diagnosis of BPV. form the provoking positions to give the CNS the opportu-
To test, the client is positioned in long sitting on a mat or nity to adjust to the sensation that the position triggers.
plinth such that, when supine, the head and neck extend over Rolling on a bed or spinning in a chair can help adapt when
the upper edge of the surface. The examiner holds the head stimulation to the horizontal mechanism is disrupted. In
of the sitting client between both hands and then rapidly cases of maladaptation causing sensitivity of head position,
moves the client backward and down with the head turned to moving gradually into the position of discomfort while
the side and the neck extended 30 to 45 degrees below the minimizing input from the other sensory systems can be
horizontal position. The head is held in this position for 20 successful. In addition to exercise sessions, incorporating
to 30 seconds. The examiner monitors for symptoms of ver- the provoking positions into daily activities is also important
tigo and observes the eyes for nystagmus.121
When the Hallpike-Dix test position indicates BPV, spe- Adaptation
cific, highly effective procedures can be performed in the Adaptation represents the highest level of recovery in the
clinical setting to remediate the disorder.160 patient with a vestibular dysfunction, and therefore as much
Canalith repositioning is a series of passive movements adaptation as possible should be facilitated for the final out-
designed to move loose debris (otoconia) through the canal come.129,133 As noted earlier, the patient may be at any level
and back into the otolith. The client is first brought down of adaptation when intervention is initiated, and it is critical
into the extended and rotated position that causes the nystag- to be able to recognize the symptoms and behaviors associ-
mus and vertigo (the positive Hallpike-Dix position). The ated with lack of adaptation and those that represent sensory
head is held in that position until the symptoms fade com- substitution. Overdependence on nonvestibular sensory ref-
pletely or for 60 to 90 seconds. The head is maintained in erence must be extinguished. Isolating specific components
extension and then slowly rotated toward the unaffected side of the symptoms reported and understanding how impair-
and kept in that position for an additional 1 to 2 minutes to ments can manifest across several different testing proce-
allow movement of the otoconia through the canal. The cli- dures will guide intervention. Activation of an error signal
ent then rolls so that she or he is side-lying and the head is starts the recovery process, and the environment must be
turned to a 45-degree position relative to the ground. This carefully manipulated to challenge the patient at the right
position often produces more vertigo and nystagmus as the level for the correct impairment. Substitution strategies must
otoconia continue to move through the canal. In the next be eliminated during exercise even if they are still in use
movement, the head is tipped toward the chest and the client during activity. To stimulate the use of vestibular inputs for
is assisted into the sitting position. Figure 22-36 shows the adaptation of the CNS, environments are designed to disad-
sequence of the Epley maneuver. The client must then fol- vantage both vision and somatosensation. Practice can be on
low specific instructions for 24 hours.161 These include unstable or compliant surfaces, with vision either absent or
avoiding forward, backward, or lateral head tilts or bending destabilized by eye and head movements, progressing to
activities. Clients should also sleep with the head elevated to optokinetic stimulation.140,165-168 Central dysfunction will
at least 30 degrees and avoid turning to the involved side.162 negatively affect recovery rate, and knowledge of the degree
When the BPV is within the horizontal canal, dizziness and form of central disorders is important to determine
or vertigo is reported when rolling, especially if the head is prognosis and modify interventions. Psychogenic disorders
elevated on a pillow because that puts the canal in a position will also affect the process of recovery and need to be ad-
perpendicular to the ground. The symptoms are reported dressed with the appropriate professional support.
when the head is turned in either direction, but the side that When the integration of vestibular and somatosensory
triggers the worse symptoms is thought to be the side of the inputs is not congruent even at rest, there is a reported sensa-
dysfunction. tion of movement inside the head when the body is held at
Horizontal canal BPV is tested for in the supine position rest in supported sitting. In this instance, enhancing the so-
with the head held in 30 degrees of flexion to keep the lateral matosensory input by weighting through the spinal column
canal perpendicular to the ground or in the neutral position or having the patient lie on a firm surface should be part of
for ease of positioning. The head is then turned in each di- the initial intervention. This allows the vestibular system to
rection and the eyes observed for horizontal nystagmus. This calibrate using somatosensory input as a reference. This
must be distinguished from static positional nystagmus by activity, known as settling, is a good way to allow the patient
the fact that the nystagmus will fatigue if it is caused by to manage symptoms when they have been exacerbated by
movement of the otoconia but otherwise persists.163 activity. Use of distracting mental tasks pushes the adapta-
The repositioning intervention then begins with the client tion to the subconscious level. Figure 22-27 shows the setup
supine with the head turned toward the more affected ear. of weighting the spine to encourage settling of symptoms.
The head is then turned away from the affected ear and On the other hand, if the use of weights increases the
the client is slowly rolled 360 degrees (essentially staying in sensation of movement, the clinician should suspect abnor-
the same place) until the head is returned to the original mal central sensory weighting of somatosensory inputs.
Figure 22-36 n Epley maneuver: Canalith repositioning maneu-

A ver for the patient with posterior canal benign positional vertigo
(BPV). Figure represents procedure for right-side BPV. Movement
of particles through the canal is shown in each position. (From
Furman JM, Cass SP: Benign paroxysmal positional vertigo.
N Engl J Med 341:1590–1596, 1999. Copyright 1999 Massachu-
setts Medical Society. All rights reserved.)
B
Slow progressive introduction of weights may be necessary
to achieve decreased sensation of movement.
VOR adaptation requires movement of images on the
retina, or retinal slip. Therefore intervention begins with
head movement at the speed that allows stable vision.156,169
Adaptation of the VOR is accomplished by having the pa-
tient move his or her head while trying to maintain gaze
stabilization by keeping a stationary object in clear focus.128
As the system adapts, speed of head movement increases,
with the goal of achieving head movement at 2 Hz without
the object blurring. Initially the client can focus on the
C thumb or a business card held at arm’s length. The activity
is progressed to a higher level of difficulty by adding a back-
ground visual stimulus such as a television set or a visually
complex environment. Gaze stabilization with head turns
while standing on an uneven surface or while walking cre-
ates a higher-level challenge. Many clients have avoided
head movement, so simply turning the head may initially
trigger dizziness. As stated previously, dizziness with head
motion should not be confused with abnormal VOR; in VOR
dysfunction the visual image blurs as the head moves.
As stated previously, the vestibular system provides a
top-down point of reference that the somatosensory system
D uses to prevent the head and trunk from aligning with sur-
face changes. Perturbations in the form of oscillating sur-
faces provide a mechanism to activate the gravity receptors,
and feedback about the alignment of the trunk to gravity
neutral can help to discourage surface dependence when it
should not be the dominant reference.140
Ineffective head-righting responses observed during
sharpened Romberg or standing on a narrow surface
should be treated as such and activated for efficiency start-
ing in a sitting tilt. Activities that require quick changes of
position in a superior or inferior direction, such as a lunge
or going up and down stairs, can be difficult when the
otoliths are damaged. Good program components for oto-
lith stimulation are activities involving up-and-down body
movements. Examples include sitting to standing, seated
bouncing on a Swiss ball, and standing bouncing on a
mini-trampoline, all with eyes closed to eliminate use of
vision for stability. Certain positions or movements of the
head during upright activities can affect balance if just one
part of the vestibular system has abnormal function. If the
otoliths are damaged or hypersensitive, a voluntary lateral
tilt of the head when standing with the eyes closed can
cause destabilization.
A vestibular adaptation program should challenge the
patient at the limit of his or her ability. Clients often choose
to do the easiest exercise and avoid the more difficult exer-

cises if they are not educated about the need to trigger the
symptoms. Conversely, if the challenge is too far above the
ability of the patient, the CNS will fail to adapt and symp-
toms will not decrease.
Elimination of Visual Dependence

When the use of vestibular inputs has been minimized
through self-limitation of head and eye movements to
control dizziness and imbalance, the visual system often
becomes dominant for balance. Testing as identified in
Figure 22-34 can be used for intervention. Another way to
train the client to use vestibular input versus vision is watch-
ing a ball being tossed from hand to hand while standing on
Figure 22-38 n Projecting peripheral movement activates the
a compliant surface (Figure 22-37). This can also be done
optokinetic response. Individuals with visual dependence for bal-
while walking. Clients with visual dependency often report
ance, or visual motion hypersensitivity are not able to adequately
excessive fatigue after activity because of the strain of using
achieve gaze stability of the central object or will lose balance as
vision for postural stability. When these clients are in situa-
they match head position to the peripheral reference. In the patient
tions with excessive visual stimulation, reports of dizziness
with maladapted responses, this stimulus can cause significant
increase. The subtle eye movements associated with viewing
nausea and other abnormal autonomic nervous system (ANS)
a computer monitor cause more fatigue for the individual
responses.
with vestibular disorder. These individuals also often avoid
crowds as in a mall, grocery store, or airport. Attending
church services, which are often characterized by low light-
ing, visual stimulation, and the need to stand with eyes ability to maintain gaze reference when there is movement
closed or read a hymnal while singing challenges the ves- in the visual background.
tibular system. Figure 22-38 shows the use of optokinetic
stimulus to decrease visual dependence and to improve the Gait
Gait with head motion is important for the individual with
vestibular dysfunction. Because head movement causes vi-
sual disturbances and dizziness, the client with a vestibular
disorder will significantly limit head movement while walk-
ing. When visual cues are used predominantly for balance,
the client will try to keep the body in line with vertical and
horizontal visual targets. This will decrease the small, natural
movements typically made during the gait cycle. Patients
with potentially recoverable vestibular function should be
trained to walk with eye and head movements, trunk rotation,
and arm swing. Figure 22-39 shows abnormal placement of
the foot for stability when the individual is unable to use the
head orientation to gravity as the primary reference.
When the vestibular system does not accurately inform
the client about the speed and direction of head movement,
visual cues are used to determine movement speed and di-
rection in relation to nonmoving objects. However, in envi-
ronments with a lot of motion, or when someone approaches
in the opposite direction, determining speed and direction of
self-movement becomes more difficult. Clients often report
dizziness and imbalance in a crowd. Changing visual envi-
ronments can trigger imbalance in the client with visual
dependency. Walking into a darkened room, especially if the
surface is uneven (as in a theater), can often trigger a fall or
stumble. Clients with permanent vestibular loss should be
educated about these potentially high-risk environments and
taught compensatory strategies to ensure safe mobility. If
improvement in vestibular function is anticipated, however,
then progressive exposure to these busy environments is
Figure 22-37 n Keeping the gaze directed at the moving ball needed to prepare the client for real-world mobility.
while standing on a compliant surface eliminates the primary use To increase somatosensory input, clients with a vestibular
of visual targets to stabilize the head and is an effective way to shift disorder often put the whole foot down at once to get better
reference from visual to vestibular. (Courtesy Ray Hedenberg, IRB input on the position of the body relative to the ground. The
Solutions, Silverthorne, Colo.) normal heel-toe weight progression over the ball of the foot
is diminished. This is often seen in conjunction with in-

creased step width while walking. This compensatory strat-
egy is acceptable in clients with permanent loss but should
be discouraged in clients who do not need to be overreliant
on somatosensation for balance control during walking.
Walking on uneven surfaces can be a challenge if the client
is primarily reliant on somatosensory input and has poor
visual-vestibular interaction. This is one reason why walk-
ing indoors is less of a problem than walking outdoors.
Again, clients who are not expected to recover vestibular
function should be educated about these potentially hazard-
ous environments and encouraged to develop compensatory
mechanisms to permit safe mobility. Gait training on pro-
gressively less stable surfaces is appropriate for clients
who need to reduce dependence on somatosensory cues and
improve visual-vestibular interaction.
Substitution
Permanent bilateral vestibular loss, however, requires sub-
stitution of somatosensation and vision to orient to the envi-
ronment. Use of a cane or walking stick to increase use of
somatosensation and allow more time to prepare for the next
step can increase confidence in gait. Control of turns can be
achieved by a quick stop while the head is turning, rapid
saccade to stable target, and then completion of the turn with
a fixed gaze. Figure 22-40 shows the sequence of the spot
A B turn. This process can become second nature and can be
performed on a regular basis to increase stability during
daily tasks.170 Driving can be trained in a safe manner in
reference to head turns and visual references by doing a
slow blink to decrease distracting visual flow.171
Maladaptation
Clinical interactions have an important influence on the
course of maladapted responses. Explaining the psychoso-
matic connections in detail can be the first step in recovery.
This is critical in order to engage the patient who demon-
strates strong avoidance behaviors. Successful outcomes are
possible; however, the process may take longer because the
central modulation of sensory input is compromised, and
therefore adaptation will occur in smaller increments. Proper
referral to someone to assist with the management of the
psychological or psychiatric condition should always be
considered.
C
D
Figure 22-39 n A and B, When the vestibular, visual, and so-
matosensory systems are integrated properly, the movement of the
head does not change the pattern of the step. C and D, When the
vestibular system is not primary for reference, step outs occur dur- A B C
ing head rotations. This demonstrates the foot moving in the direc- Figure 22-40 n The “spot turn” for the patient with bilateral
tion of head turn, causing a staggering gait pattern seen often with vestibular loss. A, When the patient is ready to turn, the front foot
patients with lack of ability to activate primary dominance of is planted to provide somatosensory reference. B, Once the foot is
the vestibular system. (Courtesy Ray Hedenberg, IRB Solutions, planted, the head is turned. C, Visual reference on a nonmoving
Silverthorne, Colo.) target is maintained while the body turns under a stable head.
(Courtesy Ray Hedenberg, IRB Solutions, Silverthorne, Colo.)
FEEDBACK sponse to gravity reference; however, the rate of retention or

Biofeedback can be used in a variety of ways. Feedback carryover may still be related to available plasticity within
about correct postural responses remains the task of the each system.
therapist in the training paradigm. Visual feedback
has been used to supplement center of pressure reference CONCLUSION
for control of weight shifting using vision to supplement This chapter has been divided into two sections owing to the
vestibular integration to somatosensory inputs. Visual specificity and amount of information specific to both areas.
feedback about the head and trunk movement versus Balance was presented first because of the general nature of
total movement on a perturbed surface is available on the this topic and because it lays the foundation for discussion
Proprio 5000 (Figure 22-41). of vestibular problems. Although vestibular disorders have a
Use of the Nintendo Wii Fit has been popular and pro- direct effect on balance, not all balance dysfunction is
vides information about results of weight shifting integrated caused by vestibular problems. With the amount of research
into games that integrate a balance task into an activity that available in both areas and the areas of specialization sepa-
may represent another activity. Training is performed on a rating, the chapter was separated to aid the readers in focus-
nonmoving surface, so somatosensory drive is activated. ing on specific problem areas. The references have been
Patients with visual motion hypersensitivities may find the placed chronologically as appearing in the chapter, but the
games overstimulating at the beginning of the session, but reader will see that the two portions have been clearly iden-
playing them may also be an appropriate method to increase tified to assist in finding materials specific to the topic of
adaptation so that more visual stimulus can be tolerated. interest. The complexity of these two topic areas has grown
Audio feedback has long been used in the patient with as new research and new technology have become available.
vestibular system loss to provide information about sway Outcomes of treatment once thought unrealistic have be-
when surface and visual reference is lacking. It can be used come reality. Many patients who once thought their quality
in many different ways for intervention. of life was permanently diminished and their ability to par-
Vibrotactile feedback using accelerometer or gyroscopic ticipate in meaningful activities had been taken away now
information has emerged in recent years. This has been suc- show tremendous improvement in functional movement.
cessful on many levels, and the modes of input are becoming The effectiveness of therapists working with these patients
less intrusive to those who wear them. The input typically depends on understanding the specificity of the clinical
provides immediate positive changes in performance of a problems and applying interventions that show measurable
task, with behaviors that represent improved postural re- change. The importance of active patient participation can-
not be overemphasized when discussing motor control and
motor learning principles needed to optimize positive
changes in balance and in specific vestibular disorders.
These problems dramatically affect an individual’s quality
of life, and the role occupational and physical therapists play
in providing appropriate interventions has established effi-
cacy in practice.
Acknowledgment
Thanks to Janet Helminski, PT, PhD; Linda Horn, PT, NCS;
Pat Huston, MS, PT; Leslie Allison, PT, PhD, NCS; and
Kenda Fuller, PT, NCS, for their significant contributions to
the development of this chapter. Gratitude is also extended
to Darcy Umphred, PT, PhD, FAPTA, and our families for
their patience and support.
References
Figure 22-41 n Feedback about the position of the trunk in rela- companies this textbook. This online service will, when
tion to the center of the platform, as well as feedback about the available, provide a link for the reader to a Medline abstract
amount of trunk flexion and extension and rotation, can be pro- for the article cited. There are 182 cited references and other
vided to the patient during the perturbations. The patient can also general references for this chapter, with the majority of
receive summary feedback after sessions performed with the eyes those articles being evidence-based citations.
closed. (Proprio 5000, Perry Dynamics, Decauter, Ill.)
CASE STUDY 22-1 n ANDY

Andy is a 27-year-old man who sustained a severe closed-head 3. Good static and fair dynamic sitting balance
injury in a skiing accident. He was hospitalized for 2 months 4. Contact guard sit-to-stand
and resided at a long-term care facility for 6 months before 5. Minimal assist static standing balance
cranial surgery for removal of bilateral subdural hygromas and Note: Ambulation goals were temporarily deferred because
revision of a ventriculoperitoneal shunt. After surgery he dem- of the ankle contractures and balance deficits.
onstrated marked improvement and was transferred to a reha- Early treatments included the following:
bilitation unit. His initial physical therapy assessment revealed 1. Standing frame activities for head control, visual
the following impairments, which had a negative effect on pos- tracking, trunk control, reduced lower-extremity
tural control: extensor tone, and heel cord stretching with
1. Oculomotor deficits (difficulty tracking to the right and ultrasound
upward) 2. Neurodevelopmental sequence activities for head and
2. Disorientation trunk control; trunk strengthening; decreased lower-
3. Delayed and slow motor responses extremity extensor tone; balance on all fours, heel-
4. Bilateral ankle plantarflexion contractures (1 to 10 degrees sitting, kneeling
left, 1 to 15 degrees right); limited right shoulder flexion 3. Supine to and from sitting, especially over the
(0 to 100 degrees) and external rotation (0 to 20 degrees) right arm
5. Hypotonic trunk (right, moderate; left, mild), hyper- 4. Sitting balance with upper-extremity functional tasks
tonic (extensor) lower extremities (right, moderate; left, (e.g., putting glasses on and taking them off, taking shirt
mild), hypertonic right upper extremity (mild) off and putting it on, wiping nose with tissue), with
6. Fair head control focus on right visual tracking, right trunk elongation,
7. Poor trunk control with right scapular atrophy, short- and incorporation of right lower-extremity ground
ened right side, strength 32/5 pressure for stability
8. Left upper and lower extremity movement isolated and 5. Transfer training with incorporation of right upper
coordinated but slow, strength 4/5 at shoulder, 41/5 extremity to push up, reach and grasp, and right lower-
elbow, wrist, hand, 4/5 hip and knee, 31/5 ankle, able extremity placing and weight bearing
to place and hold for weight bearing As soon as Andy’s ankle dorsiflexion ROM was near neu-
9. Right upper extremity rests and moves in synergistic pat- tral on the right (was then 0 to 5 degrees on the left), neurode-
tern but can move out of synergy with request or demon- velopmental activities were phased out and standing balance
stration; strength 32/5 at shoulder and 42/5 distally; and pregait activities in the parallel bars were initiated with
coordination is poor; can place and hold for weight moderate assistance. He rapidly progressed to minimal-assis-
bearing if cued but not spontaneously tance gait in the parallel bars but with significant scissoring of
10. Right lower extremity moves in flexor-extensor pattern, the lower extremities. Gait outside the bars was begun with a
grossly 31/5 in hip and knee flexion, 21 hip exten- quad cane on the left, but Andy was not able to organize the
sion, 31/5 knee extension, no isolated ankle move- sequence for cane use and did not use the cane when loss of
ment, cannot place or hold for weight bearing balance occurred, so use of the cane was discontinued. Gait
Functional tests found the following activity limitations: without an assistive device required moderate assistance from
1. Minimum assist supine-to-sit the therapist for balance. A line drawn on the floor provided a
2. Sitting balance, poor visual cue to remind him to keep his feet apart; when walking
3. Moderate assist sit-to-stand without this cue, approximately 25% of his steps were close or
4. Standing balance, unable crossed.
5. Moderate assist transfers At discharge, 2 months after admission, Andy had good
6. Nonambulatory visual tracking; normal ROM with the exception of right
Body system impairment goals were the following: lower-extremity dorsiflexion, which was limited to 0 to
1. Increase ROM to within normal limits throughout 5 degrees; normal tone in the left extremities; mildly
2. Increase trunk tone to normal and strength to 41/5 increased tone in the right extremities with slight extensor
3. Decrease right-sided tone to normal patterning in the leg; good head and trunk control; and
4. Increase spontaneous use, isolated movement, and strength grossly 41/5 throughout. Functionally, he was
strength (41/5) in right extremities independent in bed mobility, wheelchair mobility, and sitting
5. Able to place and bear weight on right lower extremity balance. He required supervision for safety in transfers and
Short-term functional goals were the following: standing activities and minimal to moderate assistance for
1. Independent in all bed mobility indoor ambulation without an assistive device depending on
2. Independent in wheelchair transfers his fatigue level.
CASE STUDY 22-2 n DORIS

Doris is a 73-year-old woman with a long history of Parkinson treatment plan included vestibular exercises for the dizziness
disease who had fallen four times within the 6 months before and balance retraining exercises for instability and falls. The
referral to physical therapy. As a result of her most recent fall, vestibular exercises she was given were designed to provoke
during which she hit her head, Doris had ear pounding, light- her symptoms repeatedly and included head turning in supine
headedness, and headaches. After referral to an otolaryngolo- and sitting (progressed to standing), rolling in bed, rocking in
gist, she was diagnosed with unspecified peripheral vestibular a rocking chair, and sit-to-stand practice. As her dizziness
dysfunction and referred to outpatient therapy. Her therapist subsided, her home program was modified to increase the
found that Doris reported increased lightheadedness and dizzi- number and rate of head movements. To improve her use of
ness, with anterior-posterior head movements, rolling in bed, somatosensory and vestibular inputs, Doris also practiced
sit-to-stand, and the Hallpike-Dix maneuver (worse to the standing on a firm surface with eyes closed (with family
right). Multiple impairments that could be contributing to supervision). In the clinic, Doris did stretching, strengthening,
her instability and falls, as well as symptoms related to and postural extension exercises to address her musculoskele-
the vestibular disorder, were also noted. Doris had mildly tal limitations. For increased use of somatosensory and ves-
decreased ROM in her left ankle, shoulders, and neck; tibular inputs, she practiced standing and weight shifts with
mild left-sided weakness and lack of coordination; marked optokinetic stimulation. By using postural sway biofeedback,
bilateral upper-extremity tremor; and moderately forward- she practiced achieving the midline position, controlled
flexed posture. anterior and left-sided weight shifts at progressively faster
She could not perform an ankle strategy at all and contin- speeds, and ankle strategy. Gait training included starts, stops,
ually used hip strategy; she also used stepping strategy fre- turns, and obstacle avoidance and progressed to community
quently with the least shift or sway. Static postural sway ambulation tasks such as curbs and ramps. As her endurance
tests indicated that Doris had excessive sway when attempt- improved, she also did gait training on the treadmill to
ing to stand still and that she kept her COG slightly posterior increase the gait speed, stride length, hip strength, and use
and to the right of midline. Sway increased tenfold with eyes of vestibular inputs.
closed, indicating poor use of somatosensory inputs for pos- Despite her multiple problems, Doris was able to reduce
tural control. Doris could not perform repeated weight shifts the severity of her impairments and consequently improve her
in either anterior-posterior or medial-lateral directions. Her functional level. Her dizziness resolved completely. Although
limits of stability were severely restricted to less than half she still had excess sway during static standing, she was able
of normal sway range anteriorly, and her movement time to achieve and hold a midline position, and her sway with
was slow. eyes closed reduced by more than half. Doris could shift her
Functional testing revealed that Doris had several disabili- weight in both anterior-posterior and medial-lateral directions
ties. She had to use a walker or have manual assistance to am- at moderate speeds by using ankle strategy without stepping.
bulate and could negotiate level surfaces only. Without her Her limits of stability were expanded from 35% to 80% of
walker or handhold assistance, Doris could stand for less than normal, and she was able to shift her weight much more
30 seconds and take a maximum of 10 steps. For community quickly. Functionally, she could stand without the walker for
ambulation, Doris needed minimum assistance with her walker 8 minutes and walk independently indoors on level surfaces
and could go only short distances. She also required minimum without the walker for short distances. She was independent
assistance with bathing and household tasks. in community ambulation with the walker. At a 3-month
Doris participated in therapy twice a week for 6 weeks follow-up visit, Doris reported that she had experienced no
and also performed a home exercise program daily. Her more falls.
APPENDIX 22-A n Common Vestibular Disorders

Benign Positional Vertigo (BPV)
The otoconia in the otolith can become loose, clump together, aware of the mechanism, it seems to be something that is uncon-
and form densities known as canaliths, which can move into a trollable because it is associated with head movement. Spontane-
semicircular canal and become a cause of vertigo. In cases of ous remissions are common and may reflect an underlying disorder
BPV, the involved side is distinguished by which ear is toward such as hydrops or migraine-induced ischemia. Infections or in-
the ground when the symptoms occur. The critical hallmark of flammations may occur months or years before the onset of BPV.
BPV is that the vertigo usually starts after 5 to 10 seconds and Adverse life events are reported to trigger an event, especially in
resolves or fatigues within 20 to 40 seconds. A less common individuals with an underlying disorder.
variant of BPV, cupulolithiasis reflects the adherence of the oto- Infection
conia to the cupula. The vertigo associated with change of head Acute unilateral vestibulopathy affects the vestibular nerve. Most
position is caused by the direct pressure deflection of the cupula. often the infection is viral in nature and is known as neuronitis or
The vertigo appears with less latency and is often more persis- neuritis. It can also be caused by bacterial infection from a variety
tent, taking up to 60 seconds to resolve after change of head of causes, either as a primary infection or secondary to bacterial
position. meningitis or encephalitis. Vestibular neuritis can be partial, affect-
Benign positional nystagmus or vertigo is a common sequela of ing the superior afferents from the horizontal and anterior semicir-
head concussion, viral labyrinthitis, hydrops, and vascular occlu- cular canals primarily.
sion in the distribution that feeds the inner ear. It can also develop The infection often is preceded by a systemic illness or an upper
without a known external cause and is the most common cause of respiratory tract infection, but it can be an isolated infection affect-
vertigo.172 Other conditions that can cause head position dizziness ing the nerve or labyrinths. This causes an acute, severe dizziness
are described in Appendix Box 22-1. often accompanied by nausea and vomiting. Initial impairment
BPV may involve any semicircular canal, although the posterior may include ocular tilt, skew deviation, or lateropulsion. Recovery
canal is most common because of its relationship to the otoliths reflects central adaptation to loss of input on one side of the ves-
when the person is in the recumbent position. The horizontal canal tibular system.
can also collect otoconia, and the result is horizontal nystagmus Endolymphatic Hydrops
generated with head movement; dizziness often occurs as the head Increased fluid pressure within the labyrinth, known broadly as
is going backward, when the horizontal canal moves into the hydrops, will cause vertigo. The fluid pressure may increase be-
gravity-responsive position. The dizziness is also triggered in the cause the fluid cannot move out of the system through the endo-
head rotated and flexed position. lymphatic sac, which normally absorbs excess fluid. As the fluid
Despite the use of the term benign, the symptoms related to pressure increases, the brain receives abnormal signals from the
positional vertigo are intense and can cause significant disability. cupula of the labyrinth on one side, and the result is the sensation
There is often a strong sense of falling or spinning out of control, of spinning. There is often concurrent low-frequency loss of hear-
even when the individual is lying on a bed. Before the individual is ing related to the fluid pressure in the cochlea. The episode can last
from just a few minutes to a day but usually lasts 2 to 4 hours. The
use of diuretics can control the fluid changes and decrease the
number and intensity of symptoms. Meniere disease is a type of
hydrops that occurs intermittently; the person has normal balance
n ALTERNATIVE CAUSES
APPENDIX BOX 22-1
when not having an episode.
OF HEAD POSITION DIZZINESS AND NATURE
Over time there appears to be a gradual degradation of the
OF DIZZINESS
vestibular system, resulting in symptoms associated with chronic
Unilateral vestibular dysfunction: Dizziness when changing head unilateral vestibular loss. These patients often have a diffuse diz-
position while supine ziness and report imbalance between episodes. Intervention is
Vestibular migraine: Episodic dizziness that can usually be related targeted at adaptation of the abnormal vestibular responses, and
to specific triggers this can improve symptoms, although it cannot remedy the dis-
Cerebellar nodulus: Nystagmus is downbeat without torsion with- ease itself. Traumatic hydrops can be the result of a blow to the
out fatigue or habituation head during a fall or whiplash injury. The mechanism is not fully
Vertebral artery compression: Nystagmus with extreme rotation or understood, but it may be related to damage to the endolymphatic
extension of neck sac during the trauma, resulting in inflammation or scarring that
Central vestibular pathways: Nystagmus without dizziness, usu- limits the regulation of fluid in the sac. Patients often appear
ally unidirectional drunk during an episode and therefore may limit social interac-
Geriatric supine nystagmus: Vertical nystagmus when supine tions or driving for fear of an attack. In some individuals an at-
Perilymph fistula: Episodic dizziness after trauma, increases with tack can include the “crisis of Tumarkin,” resulting in feeling as
Valsalva or head hanging if they have been thrown to the ground without a sensation of
Superior canal dehiscence: Dizziness with loud noises or change dizziness.
of head position Perilymph Fistula
Hypermobile stapes: Unstable at oval window; allows fluid pres- Fistula, or an abnormal communication of the inner and middle ear,
sure changes that cause dizziness with head positions can occur at the round or oval windows. This may have a congeni-
Head extension: Otoliths outside of functional range, causing tal cause or may be related to trauma through the middle ear from
dizziness surgery, blasts, head injury, or even sneezing. The Valsalva maneu-
Orthostatic intolerance: Dizziness when bending or quickly stand- ver can trigger an intense sensation of dizziness owing to increased
ing from sitting, never when lying pressure in the system, and daily activity can produce an almost
Continued
APPENDIX 22-A n Common Vestibular Disorders—cont'd

persistent sense of dizziness that is relieved by recumbent positions Traumatic Brain Injury (TBI)
with minimal head movement. Typical vestibular testing is nondi- TBI can affect the vestibular system in several ways. It can cause
agnostic, but use of pressure in the ear may assist this diagnosis. direct damage to the vestibular end organ (in the temporal bone);
Vestibular rehabilitation for adaptation fails because the system BPV; and, in many cases, disruption of the integration of the ves-
has a persistent fluctuating nature. Successful surgical repair of tibular nuclei (in the brain stem) and cerebellum. Sensorimotor
the fistula produces the stability needed to resume rehabilitation disturbances are common with TBI involving the cerebellum or
efforts. parietal lobe. Visual dysfunction results from damage to brain stem
Superior Semicircular Canal Dehiscence Syndrome areas such as the pontine gaze centers or central damage in the
Dehiscence or thinning of the bone overlying the superior (ante- medial longitudinal fasciculus. Frequently the third, fourth, or sixth
rior) semicircular canal creates a “third mobile window,” and the cranial nerve is damaged, and this affects the ability to move the
effect of change in pressure of the canals appears to be similar to eyes for conjugate gaze. In some extensive TBI cases the brain
the fistula. Loud noise or pressure can cause disequilibrium. Surgi- loses its ability to use any of the three sensory systems accurately.
cal repair usually produces good results. Dizziness and imbalance are prevalent complaints from client with
Vertiginous or Vestibular Migraine TBI because there are often situations in which they cannot acquire
The aura or even the primary symptom of migraine may be dizzi- accurate sensory information.174
ness. Diagnosis is based on the episodic nature, recognition of trig- Each system should be evaluated individually for its function.
gers, history of migraine, and combination of dizziness with the In patients with TBI, the adaptation of the vestibular system occurs
other typical prodromes of migraine including photophobia, nausea, more slowly and with more effort than in other clients with ves-
and vestibulocochlear symptoms of tinnitus and sensitivity to sound. tibular deficits. The client with vestibular problems associated with
The pathophysiology follows that of migraine headache, in which TBI requires significantly more intervention initially, and the out-
there are multiple levels of dysfunction from gene defects that drive comes are less favorable than for other clients experiencing ves-
familial autosomal disorders and an inherited migraine threshold, tibular dysfunction.175
brain stem activity that can trigger vascular responses of dilation Allergies
or restriction, serotonin platelet activity, and spreading neuronal Persons with allergies are often predisposed to episodes of dizzi-
depression. Medical management follows the criteria for migraine. ness. Foods, airborne allergens, and chemicals can trigger dizzi-
Rehabilitation is indicated when avoidance of activity has changed ness in these individuals.
the sensory integration, or when multiple episodes have influenced Metabolic Disorders
the system toward dependency or hypersensitivity patterns.173 Vertigo and dizziness are often reported with metabolic disorders
Vascular Disorders such as diabetes. Autoimmune diseases such as rheumatoid arthri-
Ischemia in the areas of the vestibular system (brain stem, cerebel- tis, lupus, and human immunodeficiency virus infection can also
lum, parietal-insular cortex) can cause dizziness and imbalance. cause symptoms when the disease process damages components of
Vertebral basilar artery insufficiency syndrome, for example, clas- the vestibular system.
sically produces these problems. Ischemia is usually seen in indi- Autoimmune Ear Disease
viduals older than 50 years, but it can also be associated with Autoimmune responses may result in rapid decline of hearing with
bleeding disorders such as leukemia. Migraine headache can cause intermittent symptoms of vertigo, aural pressure, and tinnitus. It is
intermittent dizziness from compromise of blood flow in the areas caused by the deposition of antibody-antigen complex in the capil-
of the vestibular system. laries or basement membranes of inner ear structures. It can appear
Neoplasia in the same manner as hydrops, with fluctuations of hearing loss
Neoplasia can compromise vestibular function when it occurs near and vestibular function. The Western Blot is one of the most widely
any part of the vestibular system. Vestibular schwannoma (com- used diagnostic tools.
monly but mistakenly known as acoustic neuroma) can cause Autonomic Related Vertigo
damage as it slowly grows on the sheath of the vestibular nerve. The autonomic and vestibular systems are physiologically con-
The schwannoma can grow into the pontocerebellar angle and nected, and significant activation of the vestibular system will
cause symptoms typically associated with cerebellar lesions. Me- cause nausea, pallor, sweating, and clamminess. Autonomic dys-
ningiomas (encapsulated tumors found most often deep in the function can contribute to dizziness and is reported in conjunction
brain) growing in the area of the temporal lobe can cause pressure with palpitations, chronic fatigue, sleeping disorders, cold ex-
on the vestibular mechanism. In some cases, damage to the ves- tremities, headaches, gastrointestinal disorders, medication intoler-
tibular nerve occurs as a result of surgical removal of the tumor. ance, and fainting.
Ototoxicity Mitral valve prolapse can be found in patients with such signs
Aminoglycosides, antibiotics used in cases of massive or systemic and symptoms, and disorders of circulation should be considered.
infection, can be ototoxic (causing damage to the vestibular hair Orthostatic concerns should be considered, and often patients are
cells). Although a small percentage of users experience this ad- found to have abnormal tilt-table test results. Often patients will
verse effect, it can affect both sides of the bilateral vestibular ap- have been diagnosed with Meniere disease but do not respond to
paratus and cause significant disability. Often the client does not diuretics and, when observed carefully, have signs as described
begin to experience the symptoms until the medication has been previously, with lightheadedness a component of the dizziness,
used for more than a week. along with vertigo.176
APPENDIX 22-A n Common Vestibular Disorders—cont'd

Mal de Debarquement (Disembarkment Syndrome) have an influence on autonomic regulation, and symptoms such as
The symptoms of continued rocking or a sensation that one has just heart palpitations, fainting spells, and chronic fatigue are reported
gotten off a boat is the hallmark of disembarkment syndrome. In- to a higher degree when there is an additional psychogenic compo-
deed it comes often after a long boat or airplane ride, often when nent. Vertiginous migraineurs have a higher frequency of comorbid
there is turbulence. The vestibular system seems to be activated to anxiety disorders. The common neuroanatomic pathways provide
a high degree, then is unable to calibrate back to normal in refer- focus for interventions. Antivertiginous medications are not effec-
ence to the somatosensory input available.177 tive, and challenging the vestibular pathways can often lead to in-
The sensation is greater when the patient is at rest, and move- creased avoidance behaviors, more dizziness, and actual decline in
ment is actually preferable to standing or sitting. The system re- function.179 Determination that there is a psychogenic or somato-
mains maladapted, and the condition can persist over months and form component of the disorder is often made late in the diagnosis
years, causing significant disability and frustration. Rehabilitation when treatment from the ear, nose, and throat specialist, neurolo-
is directed toward recalibration of the somatosensory and vestibu- gist, or internist has failed.180-182 Appendix Box 22-2 describes the
lar systems. relationship of the disorders.
Somatoform Dizziness
Forty percent of dizzy patients have psychological disorders, and
individuals with psychological disorders report more disability re-
lated to dizziness. There are connections between the locus coeru-
n RELATIONSHIP BETWEEN
APPENDIX BOX 22-2
leus and lateral vestibular nucleus within the brain stem, and both
OTOLOGICAL CONDITIONS AND ANXIETY
nuclei are affected by serotonergic processes. Primary somatoform
disorders, dissociative (conversion) disorders, and anxiety disor- Otogenic: Primary otological conditions that can trigger secondary
ders produce dizziness without organic cause.178 Secondary, reac- anxiety disorders
tive, or comorbid disorders can emerge as a consequence of identi- Psychogenic: Anxiety disorders as primary cause of dizziness
fiable organic dysfunction, but the recovery process is derailed Interactive: Otological conditions that exacerbate preexisting
owing to an underlying psychiatric disorder. Vestibular disorders anxiety
CHAPTER 23 Movement Dysfunction Associated
with Hemiplegia
SUSAN D. RYERSON, PT, DSc

atypical movements After reading this chapter the student or therapist will be able to:
clinical hypertonicity 1. Identify the various types of neurovascular disease.
composite impairments 2. Identify the atypical patterns of movement in clients with residual hemiplegia.
edema 3. Identify significant primary and secondary body system problems (impairments) that
evaluation of movement control interfere with functional movement patterns and limit ability to participate.
goal setting 4. Describe a reeducation intervention strategy for improving functional movement in
hemiplegia clients who have had a stroke.
movement deficits
muscle activation deficits
orthoses
postural control
predictors of recovery
primary impairments
secondary impairments
shoulder pain
shoulder subluxation
significant impairments
standardized evaluations of function
trunk and arm linked movements
trunk control
trunk and leg linked movements
undesirable compensations
OVERVIEW occurs with strokes involving the cerebral hemisphere or

The treatment of hemiplegia from vascular insult is contro- brain stem. A stroke, or cerebrovascular accident (CVA),
versial. Various treatment methods have been devised and results in a sudden, specific neurological deficit and occurs
advocated. Recent scientific theories have changed the focus when a brain blood vessel is either occluded by a clot or
of treatment from one of inhibition of abnormal tone and bursts. It is the suddenness of this neurological deficit—
facilitation of normal movement to reeducation of control occurring over seconds, minutes, hours, or a few days—
and weakness, and functional retraining. In this chapter, that characterizes the disorder as vascular. Although the
pathological conditions, body system problems (impair- motor deficits of hemiplegia may be the most obvious sign
ment), functional limitations, and intervention strategies for of a CVA and a major concern of therapists, other symp-
clients with hemiplegia from stroke are reviewed. Although toms are equally disabling, including sensory dysfunction,
hemiplegia from neurovascular pathological conditions is aphasia or dysarthria, visual field defects, and mental and
the focus of the chapter, therapists can use this information intellectual impairment. The specific combination of these
and apply it to adults with hemiplegia caused by other cen- neurovascular deficits enables a physician to detect both
tral nervous system (CNS) pathological conditions, such as the location and the size of the defect. CVAs can be classi-
tumor (see Chapter 25), trauma (see Chapter 24), multiple fied according to pathological type—thrombosis, embo-
sclerosis (see Chapter 19), and demyelinating diseases (see lism, or hemorrhage—or according to temporal factors,
Chapter 17). Movement components and their relationship such as completed stroke, stroke-in-evolution, or transient
to functional performance are used as the basis for selection ischemic attacks (TIAs).
of therapy techniques and training.
Epidemiology
Definition In the United States, stroke is the third ranking cause of
Hemiplegia, a paralysis of one side of the body, is the clas- death—more than 137,000 people die each year—and is
sic sign of neurovascular disease of the brain. It is one the leading cause of adult disability.1 The National Stroke
of many manifestations of neurovascular disease, and it Association estimates that 795,000 new or recurrent strokes
711
occur each year. The incidence of stroke rises rapidly with Pathoneurological and Pathophysiological Aspects
increasing age: two thirds of all strokes occur in people Classification
older than the age of 65 years; and after the age of 55 years, The pathological processes that result from a CVA can be
the risk of stroke doubles every 10 years. With the over- divided into three groups—thrombotic changes, embolic
50-years age group growing rapidly, more people than ever changes, and hemorrhagic changes.
are at risk. In the United States, the incidence of stroke is Thrombotic Infarction. Atherosclerotic plaques and hy-
greater in men than in women, and it is twice as high in pertension interact to produce cerebrovascular infarcts. These
blacks as in whites. Cerebral infarction (thrombosis or em- plaques form at branchings and curves of the arteries. Plaques
bolism) is the most common form of stroke, accounting for usually form in front of the first major branching of the cere-
70% of all strokes. Hemorrhages account for another 20%, bral arteries. These lesions can be present for 30 years or more
and 10% remain unspecified. Stroke is the largest single and may never become symptomatic. Intermittent blockage
cause of neurological disability. Approximately 4 million may proceed to permanent damage. The process by which a
Americans are dealing with impairments and disabilities thrombus occludes an artery requires several hours and ex-
from a stroke. Of these, 31% require assistance, 20% need plains the division between stroke-in-evolution and completed
help walking, 16% are in long-term care facilities, and 71% stroke.10
are vocationally impaired after 7 years.1 One study reported TIAs are an indication of the presence of thrombotic
that 12% of subjects have complete functional arm recovery disease and are the result of transient ischemia. Although the
and 38% have some dexterity 6 months after stroke. In addi- cause of TIAs has not been definitively established, cerebral
tion, loss of leg movement in the first week after stroke and vasospasm and transient systemic arterial hypotension are
no arm movement at 4 weeks are associated with poor out- thought to be responsible factors.
comes at 6 months.2 Embolic Infarction. The embolus that causes the
The three most commonly recognized risk factors for stroke may come from the heart, from an internal carotid
cerebrovascular disease are hypertension, diabetes mellitus, artery thrombosis, or from an atheromatous plaque of the
and heart disease. The most important of these factors is carotid sinus. It is usually a sign of cardiac disease. The
hypertension.3 Because high blood pressure is the greatest infarction may be of pale, hemorrhagic, or mixed type. The
risk factor for stroke, human characteristics and behaviors branches of the middle cerebral artery are infarcted most
that increase blood pressure, including increased high serum commonly as a result of its direct continuation from the
cholesterol levels, obesity, diabetes mellitus, heavy alcohol internal carotid artery. Collateral blood supply is not estab-
consumption, cocaine use, and cigarette smoking, increase lished with embolic infarctions because of the speed of
the risk of stroke. obstruction formation, so there is less survival of tissue
Ostfeld4 noted that mortality rates for stroke declined, distal to the area of embolic infarct than with thrombotic
slowly at first (from 1900 to 1950) and then more quickly infarct.2
(from 1950 to 1970), with a sharp drop noted around 1974. Hemorrhage. The most common intracranial hemor-
Experts have speculated that the greater use of hypertensive rhages causing stroke are those resulting from hyperten-
drugs in the 1960s and 1970s started this decline, and the sion, ruptured saccular aneurysm, and arteriovenous (AV)
creation of screening and treatment referral centers for high malformation. Massive hemorrhage frequently results
blood pressure may account for the marked decline in the from hypertensive cardiac-renal disease; bleeding into
late 1970s. the brain tissue produces an oval or round mass that dis-
places midline structures. The exact mechanism of hem-
Outcome orrhage is not known. This mass of extravasated blood
The long-term follow-up on the Framingham Heart Study decreases in size over 6 to 8 months.
revealed that long-term stroke survivors, especially those Saccular, or berry, aneurysms are thought to be the result
with only one episode, have a good chance for full func- of defects in the media and elastica that develop over years.
tional recovery.5 For people left with severe neurological This muscular defect plus overstretching of the internal elas-
and functional deficits, studies have demonstrated that reha- tic membrane from blood pressure causes the aneurysm to
bilitation is effective and that it can improve functional abil- develop. Saccular aneurysms are found at branchings of
ity.6,7 It has been demonstrated that age is not a factor in major cerebral arteries, especially the anterior portion of the
determining the outcome of the rehabilitation process.8 Cur- circle of Willis. Averaging 8 to 10 mm in diameter and vari-
rently it is thought that clients should be given an opportu- able in form, these aneurysms rupture at their dome. Sac-
nity to participate in the rehabilitation process, regardless of cular aneurysms are rare in childhood.
age, unless it is medically contraindicated. AV malformations are developmental abnormalities that
The prediction of ultimate functional outcome has been result in a spaghetti-like mass of dilated AV fistulas varying
hampered by the inaccuracy of commonly used predictors in size from a few millimeters in diameter to huge masses
(medical items, income level, intelligence, functional level). located within the brain tissue. Some of these blood vessels
Computed tomography (CT), functional magnetic resonance have extremely thin, abnormally structured walls. Although
imaging, and regional cerebral blood flow studies are used the abnormality is present from birth, symptoms usually
in diagnosis and increasingly as predictors of functional redevelop at ages 10 to 35 years. The hemorrhage of an AV
covery after stroke. Positron emission tomography and malformation presents a pathological picture similar to that
single-photon emission CT are newer techniques that are for the saccular aneurysm. The larger AV malformations
used in research centers to define areas of dysfunctional but frequently occur in the posterior half of the cerebral hemi-
perhaps “salvageable” tissue.2,9 sphere.10
CHAPTER 23 n Movement Dysfunction Associated with Hemiplegia 713
Clinical Findings n Sudden severe headache with no known cause

The focal neurological deficit resulting from a stroke, n Sudden trouble seeing with one or both eyes
whether embolic, thrombotic, or hemorrhagic, is a reflection Anticoagulant drugs are used to prevent TIAs and may
of the size and location of the lesion and the amount of col- stop a stroke-in-evolution. Before anticoagulant drugs are
lateral blood flow. Unilateral neurological deficits result used, an accurate differential diagnosis is necessary because
from interruption of the carotid vascular system, and bilat- of the danger of excessive bleeding if hemorrhage is present.
eral neurological deficits result from interruption of the Heparin is often used in the early stage of the stroke, and
vascular supply to the basilar system. Clinical syndromes warfarin (Coumadin) or dabigatran (Pradaxa) is commonly
resulting from occlusion or hemorrhage in the cerebral cir- used in the months after the stroke. Cerebral edema, if present,
culation vary from partial to complete. Signs of hemorrhage is managed pharmacologically during the first few days.
may be more variable as a result of the effect of extension to Antiplatelet drugs such as aspirin, dipyridamole (Persantine),
surrounding brain tissue and the possible rise in intracranial and sulfinpyrazone (Anturane) are used to prevent clotting
pressure. Table 23-1 summarizes the clinical symptoms and by decreasing platelet “stickiness.”10
the anatomical structures involved according to specific ar- Surgical treatment (thromboendarterectomy or graft-
terial involvement. ing) is used when TIAs are the result of arterial plaques.
The frequencies of the three types of cerebrovascular Areas accessible to and suitable for surgery include the
disease—thrombosis, embolism, and hemorrhage—vary ac- carotid sinus and the common carotid, innominate, and
cording to whether they were taken from a clinical study or subclavian arteries. Although both surgery and anticoagu-
from an autopsy study, but they rank in the order presented lant therapy are used for TIAs, Adams and Victor10 exten-
in this section. Ischemic strokes, thrombotic or embolic, ac- sively reviewed the wide divergence of opinions. For cli-
count for 80% of strokes, and hemorrhagic strokes account ents who have had a stroke yet recovered quickly and
for 20%.11 The clinical symptoms and laboratory findings well, medical care focuses on prevention. Prevention usu-
for each type are condensed in Table 23-2. ally includes maintaining blood pressure and blood flow,
monitoring hypotensive agents (if given), and avoiding
Medical Management and Pharmacological oversedation, especially for sleep, to prevent cerebral
Considerations ischemia.
Embolic Infarction. Management of embolic infarction
Acute Medical Care is similar to that of thrombotic infarction. The primary em-
Thrombosis and Transient Ischemic Attacks. Al- phasis is on prevention. Long-term anticoagulant therapy is
though infarcted tissue cannot at present be restored, medi- effective in preventing embolic infarction in clients with
cal management of the acute stroke from thrombosis or TIA cardiac problems such as atrial fibrillation, myocardial in-
is geared toward improving the cerebral circulation as farction, and valve prostheses. The diagnostic use of CT is
quickly as possible to prevent ischemic tissue from becom- important in anticoagulant therapy to rule out hemorrhage
ing infarcted tissue. Cells that have 80% to 100% ischemia after the infarct.
will die in a few minutes because they cannot produce en- Hypertensive Hemorrhage. Medical procedures for
ergy, specifically adenosine triphosphate. This energy fail- hypertensive hemorrhage parallel those for thrombosis and
ure results in an activation of calcium, which causes a chain embolism. Surgical removal of the clot and lowering of the
reaction resulting in cell death.1 Around this area of infarc- systemic blood pressure to decrease hemorrhage have gen-
tion is a transitional area where the blood flow is decreased erally not been helpful. Again, the preventive use of antihy-
50% to 80%. Cells in the transitional area are not irrevers- pertensive drugs in clients with essential hypertension is the
ibly damaged.12,13 soundest medical management available.10
One of the newer drugs available for immediate stroke Ruptured Aneurysm. Comatose clients are not good
treatment is tissue plasminogen activator (t-PA) (see candidates for surgery. However, if the client survives the
Chapter 36). It is approved for use within 3 hours of first few days and if the state of consciousness improves,
symptom onset but is most effective if used within the first surgical intervention, whether extracranial or intracra-
90 to 180 minutes. Recent studies indicate that 42% of nial, is the treatment of choice. Medical treatment con-
patients who have sustained a stroke wait 24 hours before sists of lowering arterial blood pressures. Bed rest for
getting care, with the average being 13 hours.13 The im- 4 to 6 weeks with all forms of exertion avoided is pre-
portance of community-wide programs to increase aware- scribed. Antiseizure medication may be used. Often a
ness of symptoms and effectiveness of emergency medical systemic antifibrinolysin is given to impede lysis of the
responses is immense for this drug’s usage. The American clot at the site of rupture. Vasospasm, resulting in severe
Heart Association and the National Stroke Association are motor dysfunction, occurs with the use of drugs such
creating community campaigns to increase awareness of as reserpine (Serpasil) and kanamycin (Kantrex) (see
the medical emergency nature of stroke symptoms. These Chapter 36).
campaigns encourage people to call 911 immediately Regardless of the cause of the stroke, comatose clients
when any of the following warning signs occur: are managed by (1) treatment of shock; (2) maintenance of
n Sudden numbness or weakness of the face, arm, or clear airway and oxygen flow; (3) measurement of arterial
leg, especially on one side of the body blood gases, blood analysis, CT, and spinal tap; (4) control
n Sudden confusion or trouble speaking or understanding of seizures; and (5) gastric tube feeding (if coma is pro-
n Sudden trouble walking, dizziness, loss of balance or longed). Hypertensive hemorrhage is one of the most com-
coordination mon vascular causes of coma.14
TABLE 23-1 n CLINICAL SYMPTOMS OF VASCULAR LESIONS
AFFECTED VESSEL CLINICAL SYMPTOMS STRUCTURES INVOLVED

Middle cerebral artery Contralateral paralysis and sensory deficit Somatic motor area
Motor speech impairment Broca area (dominant hemisphere)
“Central” aphasia, anomia, jargon speech Parieto-occipital cortex (dominant hemisphere)
Unilateral neglect, apraxia, impaired ability to Parietal lobe (nondominant hemisphere)
judge distance
Homonymous hemianopia
Loss of conjugate gaze to opposite side Optic radiation deep to second temporal
convolution
Avoidance reaction of opposite limbs Frontal controversive field
Pure motor hemiplegia Parietal lobe
Limb—kinetic apraxia Upper portion of posterior limb of internal
capsule
Premotor or parietal cortex
Anterior cerebral artery Paralysis—lower extremity Motor area—leg
Paresis in opposite arm Arm area of cortex
Cortical sensory loss
Urinary incontinence Posteromedial aspect of superior frontal gyrus
Medial surface of posterior frontal lobe
Contralateral grasp reflex, sucking reflex Uncertain
Lack of spontaneity, motor inaction, echolalia Uncertain
Perseveration and amnesia
Posterior cerebral artery
Peripheral area Homonymous hemianopia Calcarine cortex or optic radiation
Bilateral homonymous hemianopia, cortical Bilateral occipital lobe
blindness, inability to perceive objects not
centrally located, ocular apraxia
Memory defect Inferomedial portions of temporal lobe
Topographical disorientation Nondominant calcarine and lingual gyri
Central area Thalamic syndrome Posteroventral nucleus of thalamus
Weber syndrome Cranial nerve III and cerebral peduncle
Contralateral hemiplegia Cerebral peduncle
Paresis of vertical eye movements, sluggish Supranuclear fibers to cranial nerve III
pupillary response to light
Contralateral ataxia or postural tremor
Internal carotid artery Variable signs according to degree and site of Uncertain
occlusion—middle cerebral, anterior cere-
bral, posterior cerebral territory
Basilar artery Ataxia Middle and superior cerebellar peduncle
Superior cerebellar artery Dizziness, nausea, vomiting, horizontal Vestibular nucleus
nystagmus
Horner syndrome on opposite side, decreased Descending sympathetic fibers
pain and thermal sensation Spinal thalamic tract
Decreased touch, vibration, position sense of Medial lemniscus
lower extremity greater than that of upper
extremity
Nystagmus, vertigo, nausea, vomiting Vestibular nerve
Anterior inferior Facial paralysis on same side Cranial nerve VII
cerebellar artery Tinnitus Auditory nerve, lower cochlear nucleus
Ataxia Middle cerebral peduncle
Impaired facial sensation on same side Fifth cranial nerve nucleus
Decreased pain and thermal sensation on Spinal thalamic tract
opposite side
Complete basilar Bilateral long tract signs with cerebellar and
syndrome cranial nerve abnormalities
Coma
Quadriplegia
TABLE 23-1 n CLINICAL SYMPTOMS OF VASCULAR LESIONS—cont’d
AFFECTED VESSEL CLINICAL SYMPTOMS STRUCTURES INVOLVED

Pseudobulbar palsy
Cranial nerve abnormalities
Vertebral artery Decreased pain and temperature on opposite Spinal thalamic tract
side
Sensory loss from a tactile and proprioceptive Medial lemniscus
Hemiparesis of arm and leg Pyramidal tract
Facial pain and numbness on same side Descending tract and fifth cranial nucleus
Horner syndrome, ptosis, decreased sweating Descending sympathetic tract
Ataxia Spinal cerebellar tract
Paralysis of tongue Cranial nerve XII
Weakness of vocal cord, decreased gag Cranial nerves IX and X
Hiccups Uncertain
Modified from Adams RD, Victor M: Principles of neurology, New York, 1981, McGraw-Hill.
n CLINICAL SYMPTOMS AND LABORATORY FINDINGS FOR NEUROVASCULAR DISEASE—

TABLE 23-2
RUPTURED SACCULAR ANEURYSM
DISEASE TYPE CLINICAL PICTURE LABORATORY FINDINGS

THROMBOSIS Extremely variable Cerebrospinal fluid pressure is normal
Preceded by a prodromal episode Cerebrospinal fluid is clear
Uneven progression Electroencephalogram: limited differential
Onset develops within minutes or hours or over days diagnostic value
(“thrombus in evolution”) Skull radiographs not helpful
60% occur during sleep—patient awakens unaware of Arteriography is definitive procedure;
problem, rises, and falls to floor demonstrates site of collateral flow
Usually no headache, but may occur in mild form CT scan helpful in chronic state when
Hypertension, diabetes, or vascular disease elsewhere in cavitation has occurred
body
TIA Linked to atherosclerotic thrombosis Usually none
Preceded or accompanied by stroke
Occur by themselves
Last 2-30 min
A few attacks or hundreds are experienced
Normal neurological examination findings between attacks
If transient symptoms are present on awakening, may indi-
cate future stroke
EMBOLISM Extremely variable
Cardiac Occurs extremely rapidly—seconds or minutes Generally same as for thrombosis except
for the following:
Noncardiac There are no warnings If embolism causes a large hemorrhagic
Atherosclerosis Branches of middle cerebral artery are involved most infarct, cerebrospinal fluid will be
Pulmonary frequently; large embolus will block internal carotid ar- bloody
thrombosis tery or stem of middle cerebral artery 30% of embolic strokes produce small
Fat, tumor, air If embolus is in basilar system, deep coma and total hemorrhagic infarct without bloody
paralysis may result cerebrospinal fluid
Often a manifestation of heart disease, including atrial
fibrillation and myocardial infarction
Headache
As embolus passes through artery, client may have neuro-
logical deficits that resolve as embolus breaks and passes
into small artery supplying small or silent
brain area
Continued
TABLE 23-2 n CLINICAL SYMPTOMS AND LABORATORY FINDINGS FOR NEUROVASCULAR DISEASE—
RUPTURED SACCULAR ANEURYSM—cont’d
DISEASE TYPE CLINICAL PICTURE LABORATORY FINDINGS

HEMORRHAGE
Hypertensive Severe headache CT scan can detect hemorrhages larger
hemorrhage Vomiting at onset than 1.5 cm in cerebral and cerebellar
Blood pressure .170/90; usually from “essential” hyper- hemispheres; it is diagnostically superior
tension but can be from other types to arteriography; it is especially helpful
Abrupt onset, usually during day, not in sleep in diagnosing small hemorrhages that do
Gradually evolves over hours or days according to speed not spill blood into cerebrospinal fluid;
of bleeding with massive hemorrhage and increased
No recurrence of bleeding pressure, cerebrospinal fluid is grossly
Frequency in blacks with hypertensive hemorrhage is bloody; lumbar puncture is necessary
greater than frequency in whites when CT scan is not available
Hemorrhaged blood absorbs slowly—rapid improvement Radiographs occasionally show midline
of symptoms is not usual shift (this is not true with infarction)
If massive hemorrhage occurs, client may survive a few Electroencephalogram shows no typical
hours or days as a result of brain stem compression pattern, but high voltage and slow waves
are most common with
hemorrhage
Urinary changes may reflect renal disease
Ruptured saccular Asymptomatic before rupture CT scan detects localized blood in hydro-
aneurysm With rupture, blood spills under high pressure into sub- cephalus if present
arachnoid space Cerebrospinal fluid is extremely bloody
Excruciating headache with loss of consciousness Radiographs are usually negative
Headache without loss of consciousness Carotid and vertebral arteriography is per-
Sudden loss of consciousness formed only when diagnosis is certain
Decerebrate rigidity with coma
If severe—persistent deep coma with respiratory arrest,
circulatory collapse leading to death; death can occur
within 5 minutes
If mild—consciousness regained within hours then confu-
sion, amnesia, headache, stiff neck, drowsiness
Hemiplegia, paresis, homonymous hemianopia, or aphasia
usually absent
Modified from Adams RD, Victor M: Principles of neurology, New York, 1981, McGraw-Hill.
CT, Computed tomography; TIA, transient ischemic attack.
Medical Management of Associated Problems action on the gamma motor neurons. This form of drug is
Spasticity. Spasticity and its treatment constitute a used widely to treat spasticity, although the greatest disad-
major medical problem after stroke because clients com- vantage of centrally acting drugs is that they depress the en-
plain about it, it may fluctuate, and it does not respond to tire CNS. Drowsiness and anxiety are common side effects.
one fixed treatment. The relationship between spasticity and Peripherally acting drugs are used to block a specific link
movement after stroke is an area of continued interest for in the gamma group. Procaine blocks selectively inhibit the
researchers. Recent studies have refuted the earlier belief small gamma motor fibers, resulting in a relaxation of intra-
that spasticity was inversely related to voluntary move- fusal fibers. The effect of procaine blocks is transient. Intra-
ment.15,16 Although therapists are more hesitant to treat muscular neurolysis with the injection of 5% to 7% phenol
spasticity now, physicians continue to treat it aggressively. has been used to destroy the small intramuscular mixed nerve
Various pharmacological, surgical, and physical means are branches.17 Phenol blocks relieve hypertonicity and improve
used to decrease spasticity. The pharmacological and surgi- function, especially when followed by an intensive course of
cal means are examined here, and therapy management is therapy.18 It can provide relief for 2 to 12 months, and the
discussed later. effects have been documented to last as long as 3 years.17,18
Two types of drugs are used to counter the effects of spas- Disadvantages of phenol use include its toxicity to tissue and
ticity: centrally acting and peripherally acting agents. Cen- the complications of pain that occasionally result.
trally acting drugs, such as diazepam, have been used to Botulinum toxin type A (Botox) is also used to decrease
depress the lateral reticular formation and thus its facilitatory the effects of hypertonicity on functional movement in
hemiplegia.19-21 Local injection of the toxin into spastic by subjects without hemiplegia.26 The decreased respiratory
muscles produces selective weakness by interfering with the output and the increased oxygen demand that result from
uptake of acetylcholine by the motor end plate. The effect of atypical movement patterns are responsible for early fatigue
the toxin is temporary, depends on the amount injected, and in persons with hemiplegia. Treatment objectives and tech-
is associated with minimal side effects. Repeat injections niques must reflect the understanding of this respiratory
are recommended no sooner than 12 to 14 weeks to avoid problem. For clients who walk at velocities greater than
antibody formation to the toxin. Researchers report positive 0.48 m/s, a gain in walking capacity is associated with an
functional results when botulinum toxin A injections are increased peak Vo2. Research exploring the role of exercise
followed by intensive muscle reeducation and appropriate after stroke indicated that gains in respiratory fitness were
splinting.22 associated with increased walking capacity. In clinical prac-
Dantrolene sodium is used to interrupt the excitation-con- tice, therapists should remember to include standard respira-
traction mechanism of skeletal muscles. Trials have shown tory measures and functions to evaluate the efficacy of treat-
that it has reduced spasticity in 60% to 80% of clients while ment techniques.27
improving function in 40% of these clients. The side effects— Cardiovascular Health. In the chronic stage of recov-
drowsiness, weakness, and fatigue—can be decreased through ery, clients may have significant cardiovascular decon-
titration of dosage. Serious side effects, including hepatotox- ditioning with half the fitness levels of age-matched con-
icity, precipitation of seizures, and lymphocytic lymphoma, trols. This decrease in fitness affects the performance of
have been reported when the drug has been used in high doses daily activities and adds to these clients’ morbidity and
over a long time.17 mortality risk. This decreased fitness results in part from
Baclofen, in pill form, is used as a skeletal muscle relax- decreased mobility of the leg, muscular atrophy, altered
ant to decrease spasticity. It can now be delivered intrathe- muscle physiology, increased muscular fat, and altered
cally into the spinal cord with a pump that is surgically in- peripheral blood flow.28,29
serted into the body. It relieves spasticity with a small Fractures. If the hemiplegic client has severe extremity
amount of medication (10 mg/20 mL, 10 mg/5 mL). Intra- or trunk weakness and relies heavily on the nonparetic ex-
thecal baclofen has had dramatic results in cases of severe tremities for function, poor balance and falls are possible.
spasticity because it acts directly on the affected muscles After a stroke the risk of hip fracture is greatest in the first
instead of circulating in the blood. It is used for extremity year of recovery. Eighty percent of hip fractures occur on
spasticity that interferes with the ability to assume func- the paretic side and are the result of bone loss or falls. In
tional positions in patients with severe stroke, multiple addition, other common fracture sites are the humerus and
sclerosis, head injury, and cerebral palsy.23 wrist.30
The surgical treatment of spasticity through tenotomy or Therapy intervention for a hip fracture with a hemiplegia
neurectomy is considered when all other treatments fail, and is complicated by increased difficulty sustaining a symmetri-
it is used to correct deformity, especially of a hand or foot. cal trunk posture over the fractured hip, decreased strength in
A peripheral nerve block is often used as a diagnostic tool to the leg, pain, and spasticity. In addition to the loss of balance
evaluate the effect of surgical treatment. If anatomical or and protective mechanisms, the development of osteoporosis
functional gains are made through a temporary nerve block, from disuse is a limiting factor for functional recovery after
consideration is given to surgical release. The surgical treat- a fracture.31
ment of spasticity does not necessarily result in increased Thrombophlebitis. Thrombophlebitis may occur in the
movement control and, with the increased understanding of early stages of rehabilitation. Vascular changes are often
the causes of spasticity, does not seem appropriate in stroke. premorbid. Deep vein thrombosis is caused by altered blood
Seizures. The highest risk for seizure after a stroke is flow, damage to the vessel wall, and changes in blood co-
immediately afterward; 57% of seizures occur in the first agulation times. The vascular changes are aggravated by the
week and 88% occur within the first year.24 Seizures after inactivity and dependent postures of the weak extremities.
thrombotic and embolic stroke are usually of early onset, Deep vein thrombosis is many times more common in the
whereas seizures after hemorrhagic stroke are of late weak leg.32
onset. The management of seizures after stroke is usually Complex Regional Pain Syndrome. Formerly known
with antiseizure medication. Commonly used drugs include as reflex sympathetic dystrophy, complex regional pain
phenytoin (Dilantin), carbamazepine (Tegretol), gabapentin syndrome is a chronic pain condition affecting the paretic
(Neurontin), and divalproex (Depakote).25 Side effects that arm or leg. The extremity pain is reported as intense and
interfere with movement therapy include drowsiness, ataxia, burning and may be accompanied by swelling and red-
distractibility, and poor memory. ness. It leads to changes in bone and skin and, if left un-
Respiratory Involvement. Fatigue is a major problem treated, becomes debilitating. Medical treatment includes
for the person with hemiplegia. This fatigability, which in- the use of chemical sympathetic blocks and oral or intra-
terferes with everyday life processes and active rehabilita- muscular corticosteroids. The use of blocks and cortico-
tion, is attributed to respiratory insufficiency resulting from steroids often stops the burning pain. The length of time
paralysis of one side of the thorax. Haas and colleagues26 of the relief varies from client to client. Adverse reactions
studied respiratory function in hemiplegia and found de- from blocks and corticosteroids occur about 20% of the
creased lung volume and mechanical performance of the time33,34 (see Chapter 32).
thorax to be significant factors, in addition to abnormal pul- Pain. The pharmacological management of joint pain
monary diffusing capacity. Clients with hemiplegia con- after stroke (usually shoulder pain) includes the local injec-
sume 50% more oxygen while walking slowly (regardless of tion of corticosteroids. (For additional information regard-
the presence or absence of orthotic devices) than that used ing pain and its management, see Chapter 32.)
Sequential Stages of Recovery from Acute continue for months or years.38,39 Measuring recovery is dif-
to Adaptive Phase ficult because the definition of “successful” or “complete”
recovery varies greatly. Duncan reports that if recovery is
Evolution of Recovery Process defined at the disability level (Barthel score greater than 90),
The evolution of the recovery process from onset to the 57% of stroke survivors have a complete recovery. However,
return to community life can be divided into three stages— if impairments are measured, less than 37% recover fully. And
acute, active (rehabilitation), and adaptation to personal if recovery is related to prior physical functioning, less than
environment. 25% are considered completely recovered.40
The acute state involves the stroke-in-evolution, the The initial functional gains after the stroke are attrib-
completed stroke, or the TIA and the decision whether to uted to reduction of cerebral edema, absorption of dam-
hospitalize. aged tissue, and improved local vascular flow. However,
The stroke-in-evolution develops gradually with distinct these factors do not play a role in long-term functional
demarcation of the damaged area over 6 to 24 hours. Throm- recovery. The brain damage that results from a stroke is
bosis, the most common cause of stroke, results first in thought to be circumvented rather than “repaired” during
ischemia and finally in infarction. Its gradual onset has led the process of functional recovery. The CNS reacts to
researchers to believe that a “cure” may be found for this injury with a variety of potentially reparative morpho-
type of stroke. If ischemic tissue can be treated and saved logical processes. Two mechanisms underlying functional
before infarction occurs, the neurological damage may be recovery after stroke are collateral sprouting and the un-
reversible. Small hemorrhages also may become a stroke-in- masking of neuropathways: regeneration and reorganiza-
evolution by effusing blood along nerve pathways and by tion.38 Research continues to provide important insights
attracting fluid.35 A completed stroke has a sudden onset and into the fundamental capabilities of the brain to respond
produces distinct, nonprogressive symptoms and damage to damage. Methods of intervention that use the environ-
within minutes or hours. In contrast, the TIA has a brief ment and help the client learn lead to long-term improved
duration of neurological deficit and spontaneous resolution recovery.
with no residual signs. TIAs vary in number and duration. The CNS has some predictable traits in response to in-
The physician decides the extent of hospitalization. The jury. Twitchell, in his classic study, first documented the
trend to hospitalize is more common today than years ago.36 initial loss of voluntary function.41 Although paralysis with
However, a mild stroke or TIA may produce minimal physi- flaccidity initially exists, there is seldom, if ever, total pa-
cal and mental symptoms, and the person may not even seek ralysis. He reported both an increase in deep tendon re-
medical help. Cost-containment measures in hospitals and flexes after 48 hours and the emergence of synergistic
managed care have led to decreased lengths of stay and the patterns of movement.41 The synergistic movement pat-
development of critical pathway plans to deliver services terns of the upper extremity and lower extremity have been
more efficiently. Critical pathways are plans that describe described in detail by many.42-44 Verbal description of a
the duration and extent of services after a stroke. The inpa- visual phenomenon often leads to differences in written
tient length of stay for acute stroke is currently 2 to 4 days. and spoken communication, yet the visual array or behav-
After the inpatient stay, the client follows one of four path- ioral patterns may be exactly the same.45 Synergistic pat-
ways: he or she returns home with or without home care terns may not be the same as movement combinations
services, goes to a rehabilitation hospital for a 2- to 4-week necessary for function. Although it is stated that the leg
stay, goes to a subacute facility to become strong enough for recovers more quickly or better than the arm, a leg that is
the rehabilitation regimen, or goes to a long-term care facil- bound by an extensor synergy and that is as “rigid as a pil-
ity for rehabilitation or maintenance care. lar” during gait has not recovered more quickly and has no
Once the stroke is completed, the clinical symptoms begin better function than an arm that is flexed and held across
to decrease in severity. A person with a stroke caused by an the chest and that can only grasp in a gross pattern with no
embolic episode may have symptoms that reverse completely ability to release.
in a few days; more frequently, however, improvement takes Although studies are investigating the exact nature of the
place very slowly with a marked deficit. The fatality rate is relationship between voluntary movement and spasticity,
high within the first day but decreases substantially in the clinical evidence demonstrates that as voluntary function
following months of recovery.36 Evidence from efficacy stud- increases, the dependence on synergistic movement de-
ies of rehabilitation programs that aim at improving func- creases.16 With the knowledge that the CNS is capable of
tional performance is limited. Studies by Bamford and col- reacting to injury with a variety of morphological processes,
leagues37 indicate that early rehabilitation intervention we should no longer view the effect of a stroke as a fixed
reduces disability and improves compensatory strategies. event. Because the brain immediately institutes neuromech-
The Framingham Heart Study has revealed that long-term anisms that reconstitute typical functions, therapy interven-
stroke survivors have a good chance of returning to indepen- tions should emphasize use of movement patterns on the
dent living. The greatest deficit in persons with hemiplegia affected side to maximize return and to help the client
who have recovered basic motor skills and who have re- achieve the highest level of function.
turned home is in the psychosocial and environmental areas.5
Predictors of Recovery
Recovery of Motor Function Research in motor recovery shows that although motor re-
Recovery of motor function after a stroke was thought his- covery may continue after 6 months, the functional status
torically to be complete 3 to 6 months after onset. More recent usually remains constant, and that 86% of the variance in
research has shown that functional recovery from a stroke can 6-month recovery is predictable at 1 month.46
In one study, although 58% of the patients regained inde- Impairments Contributing to Activity and
pendence in activities of daily living (ADLs) and 82% Participation Limitations
learned to walk, 30% to 60% of patients had no arm func- Clients with hemiplegia from stroke have movement prob-
tion.47 Initial return of movement in the first 2 weeks is one lems—impairments—that lead to activity and participation
indicator of the possibility of full arm recovery. But failure limitations. These movement problems manifest themselves
to recover grip strength before 24 days was correlated with as loss of movement in the trunk and extremities, atypical
no recovery of arm function at 3 months.47 In another study patterns of movement, and involuntary nonpurposeful move-
that used the modified Rankin scale as the outcome measure, ments of the affected side that lead to compensatory func-
half of the patients recovered within 18 months with the tional strategies. These impairments interfere with normal
greatest amount of recovery present at the 6-month mark. functional movements and may lead to loss of independence
Predictors of recovery in this group included stroke severity, in daily life.
no previous ischemic stroke, peripheral artery disease, or Impairments are the signs, symptoms, and physical find-
diabetes.48 ings that relate to a specific disease pathology. Schenkman and
One problem inherent in prognostic research is the lack Butler were the first to apply a model of impairments to neu-
of a movement-based classification system. The clinical rological physical therapy practice. Ryerson and Levit, using a
“predictors” in regression models are assumed to be static, similar format, specifically defined the impairment categories
whereas in fact they may change over time. Another prob- as primary, secondary, and composite54,55 (Box 23-1).
lem is that there may be a lack of accuracy because of dif- Primary Impairments. Primary impairments are physi-
ferences in researchers’ objectives.49 cal findings that are associated with the specific brain lesion.
As clinicians we can help minimize the problems in re- The primary impairments of stroke that relate to functional
search methods by precisely formulating functional goals, recovery of movement include changes in strength, changes in
stating movement components and significant impairments muscle tone, muscle activation or control changes (sequenc-
that interfere with functional performance, and following a ing, firing, initiation), and changes in sensation. Cognitive and
model when making clinical decisions to postulate cause perceptual, emotional, and speech and language changes are
and effect during intervention. also primary impairments that have an effect on function but
are less of a focus of this chapter.
Classification of Atypical Movement Patterns Secondary Impairments. Secondary impairments in-
Although the Guide to Physical Therapist Practice groups volve systems of the body other than the neurological sys-
patients with neurological dysfunction according to path- tem. They occur as a consequence of the stroke or because
ological condition, therapy intervention rarely is directed
by the diagnosis of stroke and resultant hemiplegia.50
The World Health Organization (WHO) classification
system, the International Classification of Function n IMPAIRMENTS THAT INTERFERE
BOX 23-1
(ICF-2), provides a structure that allows us to evaluate by WITH FUNCTIONAL MOVEMENT
health condition, impairment, or activity or participation
PRIMARY IMPAIRMENTS
limitation.51,52
Changes in muscle strength
Impairment-related classification systems for stroke are
n Paralysis or weakness
just beginning to be researched.53 Currently, atypical move-
Changes in muscle tone
ment patterns in stroke are classified according to type of
n Hypotonicity
lesion (embolism, thrombosis, TIA) or side of weakness.
n Spasticity
The classification models make it easier for therapists to
n Clinical hypertonicity
identify and define the focus of their intervention for the
Changes in muscle activation
neurological patient. These models help us organize our in-
n Inappropriate initiation
terventions into two categories: (1) interventions that aim at
n Difficulty sequencing
improving relevant impairments that contribute to functional
n Inappropriate timing of firing
limitations and disability and (2) interventions that focus on
Changes in sensation
the activity or participation limitations. The treatment inter-
n Awareness
ventions in this chapter try to relate limitations in activities
n Interpretation
to relevant underlying impairments.
Although the main focus of this chapter is the evalua- SECONDARY IMPAIRMENTS
tion and treatment of activity limitations and impairments Changes in alignment and mobility
resulting from a loss of movement control, a stroke may Changes in muscle and soft tissue length
result in damage to other systems that affects the client’s Pain
ability to perform functional skills. There may be defi- Edema
ciencies in sensory processing (vision, somesthetic sensa- COMPOSITE IMPAIRMENTS
tion, and vestibular systems) and disorders of cognitive Movement deficits
integration (arousal and attention, awareness of disability, Atypical movements
memory, problem solving, and learning), which all have a Undesirable compensations
large impact on functional retraining. Depression and,
most important, problems of language and communication Modified from Ryerson S, Levit K: Functional movement reeducation: a
also affect the client’s ability to participate in a therapy contemporary model for stroke rehabilitation, New York, 1997, Churchill
program. Livingstone.
of other medical and environmental influences, such as a the hip and knee flex. The hip and knee flexion combined
fall, pneumonia, or phlebitis. As they develop, they influ- with a tendency to place more weight on the stronger leg
ence one another and the primary impairments. Secondary places the ankle in plantarflexion, and no weight is borne on
impairments influence the client’s level of disability by con- the heel. As the client learns to walk, either the knee flexes
tributing additional physical problems. There are four major because of weakness or the patient compensates and “locks”
categories of secondary impairments: orthopedic changes in the knee in extension.
alignment and mobility, changes in muscle and soft tissue Over time, the heavy arm pulls the upper body into flex-
length, pain, and edema. ion, creating an appearance of a low shoulder. To stand and
Composite Impairments. Composite impairments are walk, a compensatory shift of the upper body onto a cane
the combined effects of the primary and secondary impair- helps the client balance. This overshifting of the upper body
ments, motor recovery, treatment, and behavioral factors. also makes it easier for these clients to initiate stepping with
Movement deficits are the missing pieces of movement control the use of pelvic elevation (Figures 23-1 and 23-2).
that the client needs to move normally. Atypical movements Atypical movement patterns are found in clients with
are movements that deviate from normal coordinated move- unbalanced muscle return and deficits in muscle activation.
ment. Undesirable compensations are alternative, severely These clients have difficulty organizing and sequencing
one-sided strategies used to perform a functional activity muscle return, quieting muscles after active firing, and grad-
because of loss of normal movement patterns. ing strength of contractions. Clients with unbalanced return
can be further divided into two subcategories: (1) those with
Patterns of Recovery greater weakness, that is, unbalanced return, with secondary
In the 1970s, “neurophysiological” theories and approaches
changed therapy treatment for adults with CNS lesions. The
founders of these approaches described positions and pat-
terns of trunk and extremity movement.42-44 These patterns
were described in terms of spastic synergies, reflexive pat-
terns, and position. Extremity movements were described as
patterns of flexor or extensor synergies, arm and leg patterns
were changeable according to the influence of tonic reflexes,
and trunk position was always short on the affected side
with scapular and pelvic retraction. The intervention tech-
niques followed the descriptions and understanding of the
movement problems. As knowledge from orthopedics, man-
ual therapy, and motor control grew, therapists looked more
closely at movement patterns and body position in clients
with hemiplegia and expanded the categories. As early as
1982, new descriptions emerged that combined synergistic
A B
patterns and biomechanical influences on the musculoskel-
etal systems.56 Today, descriptions of position and patterns
of movement follow the impairment categories. The com-
posite impairment category used in this chapter has three
generalized movement patterns that create one model of
classification: (1) movement deficits, (2) atypical move-
ments, and (3) undesirable compensatory patterns.54
Movement deficits result from severe weakness or paraly-
sis with either gradual, balanced return or no significant re-
turn. Functional movement patterns and levels of indepen-
dence are based on the distribution and amount of return:
trunk control greater than extremity control, extremity control
greater than trunk control, distal extremity return greater than
proximal extremity return or vice versa, and arm control
greater than leg control or vice versa. These clients do not C D
have problems with spasticity but, when weakness is severe,
have long-term problems with the secondary impairments of Figure 23-1 n A, Client with right hemiplegia. Movement defi-
muscle shortening and loss of joint range. cit: paralysis; client was unable to move arm or leg in standing or
In the acute stage, the arm hangs by the side, the humerus sitting. B, Client uses cane and tries to shift to the right as he gets
is internally rotated, the elbow is extended, and the forearm ready to step forward with the left leg. Note how the heavy weight
is pronated. Inferior shoulder subluxation is common. The of the right arm pulls the upper body into forward flexion and rotation
trunk is weak, the ribs flare, and posture is impaired, with a left. C, Client prepares to step forward with the right leg. Note that
convex lateral curve seen on the affected side. (Appearances his attendant has corrected his upper-body position. D, Client leans
of lateral trunk flexion with the concavity on the affected heavily onto cane (his upper body translates laterally to the left) to
side exist with compensatory upper- and lower-trunk move- lessen weight on the right leg. He will accomplish the “step” by
ments.) In standing, the client has problems recruiting rotating his upper body to the left, a compensation for the loss of
strength on the affected leg. The pelvis lists downward, and leg control in standing.
Figure 23-2 n A, Client with right hemiplegia. Movement deficit: weakness; client is able to walk
with a brace and does not need a cane. B, During stance, his upper body moves laterally to the right
and his right femur internally rotates as his knee hyperextends. C, He has enough trunk control to
stand and balance and sufficient leg control to lift the leg with knee flexion.
problems of muscle shortening and poor alignment, and These clients demonstrate trunk instability, excessive ex-
(2) those with greater return, with more problems of hyper- tremity movement, and overshooting of distal targets. Volun-
tonicity in the arm and leg. tary extremity movements are usually present but uncoordi-
These clients move and function with patterns that nated (see Chapter 21).
were formerly described as “spastic” or “synergistic.” Undesirable compensatory patterns are patterns of func-
They have either anterior or superior shoulder sublux- tion that may arise from either of the two previously de-
ations, which determine the possibilities for fractionated scribed movement categories. Compensations are alterna-
movement in the arm. Common atypical leg movement tive movements or movement substitutions used to
patterns used for walking include swing—proximal initia- circumvent the challenge to the impaired side during daily
tion patterns of pelvic hiking or rotation toward the activities. Although compensatory movements may be nec-
affected side, hip flexion with internal rotation and knee essary and desirable to achieve the highest level of activity
extension, or pelvic posterior tilting with hip abduction performance when there is no ability for recovery to occur,
and knee flexion—and stance—contact with ground via some may be more desirable than others. Undesirable com-
toe strike or foot flat, loss of hip extension, and using pensatory patterns are noticeably one sided; they rely on
excessive forward trunk flexion to initiate forward pro- movements of the uninvolved arm and leg and are accompa-
gression instead of moving the shank and lower leg over nied by asymmetrical postural trunk movements. They lead
the foot. to unsafe patterns, or to secondary impairments, or contrib-
Regardless of the proximal trunk and extremity patterns, ute to strategies that may have the potential to block or
the ankle-foot and wrist-hand patterns are predictable on the hinder future motor recovery. These undesirable compensa-
basis of the amount of distal return and the effects of proxi- tory patterns create “learned nonuse” of the affected arm and
mal alignment. With weakness, the ankle plantarflexes and leg and foster asymmetrical postural patterns. Recent re-
the wrist flexes. The foot or hand rotates on the ankle or search findings indicate that limiting compensatory trunk
wrist according to the pattern of and amount of return of movements may actually increase the performance of arm-
proximal movements. Finger and toe patterns (curling or reaching activities.
fisting, clawing) follow biomechanical rules of compensa- Patients who come into therapy with strongly established
tion or correlation (Figure 23-3). undesirable compensatory patterns do not respond quickly
Although the main movement problems of stroke occur to any type of intervention. Although therapists may be
because of weakness and atypical muscle activation patterns tempted to train a one-sided pattern in early rehabilitation to
(e.g., sequencing, initiation), other movement disturbances, quickly meet a stated goal, the long-term effects of learned
such as ataxia, may occur. In clients with ataxia, the main nonuse of one side of the body include increased severity of
movement problem is one of wide swings of tone and muscle secondary impairments and poor balance with an increased
activation disturbances with fewer problems of weakness. chance of falls (Figure 23-4).
Figure 23-3 n A, Client with right hemiplegia. Movement deficit: loss of control of firing patterns,
timing, and sequencing. B and C, Client walking.
EVALUATION PROCEDURES
Evaluation is a process of collecting information to establish
a baseline level of performance to plan interventions and to
document progress. This section reviews medical evalua-
tions, standardized evaluations of functional performance
(disability scales), evaluation of motor function and balance,
and evaluation of secondary impairments that interfere with
motor performance.
Medical Evaluation
After or during the evolution of a stroke, a thorough medical
examination is conducted. All systems are surveyed, with
emphasis placed on the level of consciousness; mental,
affective, and emotional states; communication; cranial
nerves; perceptual ability; sensation; and motor function.
The National Institutes of Health (NIH) Stroke Scale is often
used to evaluate the level of these common impairments
poststroke.57
Levels of Consciousness
Scales of varying types are used to measure the client’s level
of consciousness, to assess the initial severity of brain dam-
age, and to prognosticate recovery curves. The Glasgow
Coma Scale, devised by Teasdale and Jennett in collabora-
tion with Plum,58 has been used for nontraumatic comas
caused by stroke, head injury, and cardiac disease. This
scale records motor responses to pain, verbal responses to
Figure 23-4 n Client with right hemiplegia. Severe compensa- auditory and visual clues, and eye opening. It assigns nu-
tory patterns. She walks with a quad cane and standby assistance. merical values according to graded scales. Plum and
Pelvis rotates to the right, upper body rotates to the left, hip flexes, Caronna59 and Levy and colleagues60 have also established
and knee hyperextends. There is strong lateral translation of upper criteria for correlating clinical signs of coma with prognosis.
body to the left (to the stable cane). The standard descriptions of level of consciousness—
normal, semistupor, stupor, deep stupor, semicoma, coma,
deep coma—are categorized by objective medical data but
often leave a gap in the understanding of how the client
functions in life.58 This gap was closed by the creation of a
scale, Levels of Cognitive Functioning, at Rancho Los BOX 23-2n PERCEPTUAL DEFICITS IN
Amigos Hospital. This behavioral rating scale is not a test of CENTRAL NERVOUS SYSTEM DYSFUNCTION
cognitive skill but an observational rating of the client’s abil-
ity to process information61 (see Chapter 24). LEFT HEMIPARESIS: RIGHT HEMISPHERE—
GENERAL SPATIAL-GLOBAL DEFICITS
Mental, Emotional, and Affective States Visual-perceptual deficits
The history portion of the neurological evaluation leads to n Hand-eye coordination
an assessment of the mental, emotional, and affective states. n Figure-ground discrimination
The client’s ability to describe the illness gives information n Spatial relationships
on memory, orientation to time and place, the ability to ex- n Position in space
press ideas, and judgment. If the examiner suspects a par- n Form constancy
ticular problem, a more thorough review is undertaken of the Behavioral and intellectual deficits
higher cortical function: serial subtraction, repetition of dig- n Poor judgment, unrealistic behavior
its, and recall of objects or names. Clients with right hemi- n Denial of disability
plegia may be cautious and disorganized in solving a given n Inability to abstract
task, and clients with left hemiplegia tend to be fast and n Rigidity of thought
impulsive and seemingly unaware of the deficits present. n Disturbances in body image and body scheme
These different response patterns stem from hemispheric n Impairment of ability to self-correct
involvement and prior hemispheric specialization. n Difficulty retaining information
Loss of emotional control often exists after a stroke. Cry- n Distortion of time concepts
ing is a common problem. Although excessive, inappropri- n Tendency to see the whole and not individual steps
ate, or uncontrollable crying is usually a result of brain n Affect lability
damage and a sign of emotional lability, crying can also be n Feelings of persecution
an expression of sadness as a result of depression. This dif- n Irritability, confusion
ference is distinguishable by the ease with which the crying n Distraction by verbalization
can be stopped. Other signs of emotional lability in persons n Short attention span
with hemiplegia from stroke include inappropriate laughter n Appearance of lethargy
or anger. n Fluctuation in performance
n Disturbances in relative size and distance of objects
Communication RIGHT HEMIPARESIS: LEFT HEMISPHERE—
A general evaluation of communication disorders is noted GENERAL LANGUAGE AND TEMPORAL ORDERING
while taking the history. Cerebral disorder resulting from DEFICITS
infarct or hemorrhage can produce a loss of production or Apraxia
comprehension of the spoken word, the written word, or n Motor
both. The therapist should be familiar with all types of com- n Ideational
munication disorders and with alternate modes of communi- Behavioral and intellectual deficits
cation to establish a good client relationship. n Difficulty initiating tasks
n Sequencing deficits
Cranial Nerves and Reflexes n Processing delays
Thorough cranial nerve evaluation is necessary in hemiple- n Directionality deficits
gia because a deficit of a particular cranial nerve helps to n Low frustration levels
determine the exact size and location of the infarct or hem- n Verbal and manual perseveration
orrhage. In hemiplegia, it is imperative to check for visual n Rapid performance of movement or activity
field deficits, pupil signs, ocular movements, facial sensa- n Compulsive behavior
tion and weakness, labyrinthine and auditory function, and n Extreme distractibility
laryngeal and pharyngeal function.
Standard areas of reflex testing include the triceps, biceps,
supinator, quadriceps, and gastrocnemius muscles. Accord-
ing to Adams,10 there are four plantar reflex responses:
(1) avoidance–quick, (2) spinal flexion–slow, (3) Babinski– Perceptual retraining without standardized norms for the
toe grasp, and (4) positive support. deficit is at best difficult. The soundest course currently
available appears to be one that relates perceptual and motor
Perception learning rather than retraining perception in isolation (see
Perceptual deficits in clients with hemiplegia are complex Chapters 4 and 14).
and intimately linked to the sensorimotor deficit. Sensory
integration theory has begun to establish normative values Sensation
and objective data for testing and documenting perceptual Traditional sensory testing is used to assess sensory deficits in
deficits in children. Currently, norms and testing procedures the adult with hemiplegia: light touch, deep pressure, kines-
for adults have not been standardized, but perceptual deficits thesia, proprioception, pain, temperature, graphesthesia, two-
have been identified in clients with hemiplegia. Common point discrimination, appreciation of texture and size, and
perceptual deficits found in left and right brain damage are vibration. A comparison of the differences in the two sides of
listed in Box 23-2. the body and qualitative and quantitative measurements are
important features of sensory testing. Sensory testing is diffi- The Berg Balance Scale is easy to administer, takes 5 to
cult because it relies on the client’s interpretation of the sensa- 10 minutes, and has norms specific to clients who have had
tion, the client’s general awareness and suggestibility, and the a stroke.70,71
client’s ability to communicate a response to each test item. The Balance Evaluation Systems Test (BESTest and
The presence and quality of sensory loss must be consid- Mini-BESTest) is a balance evaluation that helps clinicians
ered during the process of reeducating motor control. Al- identify the impaired underlying system that is contributing
though Sherrington established the principle of interdepen- to poor dynamic balance. The mini-BESTest is composed of
dence of sensation and movement, current researchers have 14 items and can be administered in 10 to 15 minutes.72,73
refined the concept and hypothesize that sensation modifies The Postural Assessment Scale for Stroke is a clinical
continuing movement by providing feed-forward informa- balance measure that has been found to have better psycho-
tion, feedback, and corollary discharge. They have provided metric characteristics than the Berg Balance Scale or the
evidence that sensation is not an absolute prerequisite for balance subtest of the Fugl-Meyer test for people with se-
movement.62 vere stroke during the acute recovery phase. It has excellent
Evaluation of motor function includes both standardized reliability and validity and is easy to perform.74,75
evaluation of functional performance and evaluation of The Functional Reach Test provides a measure of balance
movement control. Manual muscle testing, although used by in standing. It measures control only during anterior (for-
physicians to determine a general level of strength, is not ward reach) weight shifts. Reliability is high, and the test is
widely used by therapists to measure strength in individuals fast and easy to perform.76
with CNS dysfunction because of the insensitivity of the test The Wolf Motor Function Test is used to measure upper-
to loss of trunk and limb linked control. New measures of extremity movements and functional tasks. It is a timed test,
manual muscle testing for stroke are now beginning to be has been tested for reliability and validity, and is the assess-
investigated. ment used in constraint-induced treatment studies.77
The Trunk Impairment Scale measures trunk movement
Standardized Evaluations patterns and dynamic and static sitting balance. It has high
test-retest reliability and excellent concurrent validity.78
Functional Performance
During the initial interview the therapist and the client to- Gait
gether form a list of limitations and relate them to the cli- The evaluation of gait patterns includes the assessment of
ent’s goals and needs. The client can state his or her per- gait speed, a description of gait deviations, and, ideally, the
ceived functional limitations, or the therapist can ask the assignment of a value representing the efficiency of ambula-
client to perform tasks. Commonly used standardized tests tion.79 Therapists are encouraged to measure gait speed
and scales for activity and participation limitations are listed throughout the rehabilitation process to quantitatively iden-
here. Additional information can be found in Chapter 8. tify improvement in walking ability.80,81
The 5-meter walk test is responsive to changes in the
Scales acute phase of recovery especially the first 5 weeks post-
The Barthel Index is one of the oldest measures of disabil- stroke. The 10-meter walk test has excellent reliability in
ity.63 It has excellent validity and reliability and is simple to the chronic recovery phase and is correlated with walking
use, but it does not discriminate at higher levels of activity. parameters and endurance.82
The Motor Assessment Scale (MAS) comes from the in- The 2-, 6-, and 10-minute walk tests are measures of
tervention theory of Carr and Shepherd.64 Its reliability is walking endurance with high reliability and validity.83
high, it is simple to administer, and it takes only 15 minutes The Functional Ambulation Profile is a system that at-
to perform. Although it mainly evaluates mobility skills, tempts to relate the temporal aspects of gait to neuromuscu-
there is an arm and hand function section. The tests of arm lar and cardiovascular functioning and converts this relation-
function include movement patterns without tasks, and the ship to a single numerical score.84
hand function section uses object manipulation. The Timed Up-and-Go Test measures (in seconds) the abil-
The Functional Independence Measure (FIM) is com- ity to rise from a chair, walk 3 meters, turn, walk, and return
monly used in rehabilitation centers, takes 45 minutes to to a seated position. It is frequently used in geriatric popula-
perform, and measures ADLs, mobility, cognition, and com- tions, but there is no validity testing for people poststroke.85
munication.65,66 It has good to excellent reliability. The temporal characteristics of gait—step time, cycle
The Rivermead Mobility Index measures common mobil- time, step length, and stride length—can be measured with
ity functions, takes 5 minutes to perform, and has been a piece of chalk and a stopwatch or with more sophisticated
tested for reliability and validity.67 equipment such as a gait analyzer. These parameters provide
The Assessment of Motor and Process Skills (AMPS) is a an objective measurement of performance and a baseline
standardized test that measures task-performance abilities from which the efficacy of treatment procedures and client
and efficiency during instrumental ADLs (IADLs).68 progress can be assessed.
Gait deviations in persons with hemiplegia have been
Tests of Motor Function and Balance described according to their biomechanical and kinesiologi-
The Fugl-Meyer Assessment is a measure of extremity im- cal abnormalities and in terms of the loss of centrally pro-
pairment severity. It is weighted, with more items measuring grammed motor control mechanisms.86,87
arm movement than leg movement. The test factors in re- Perry87 described common problems of the hemiplegic
flexes and sensation and has good validity and reliability. It person’s gait as loss of controlled movement into plan-
requires from 45 minutes to 1 hour to perform.69 tarflexion at heel strike, loss of ankle movement from heel
strike to midstance (resulting in loss of trunk balance and functional performance.90-92 Motor weakness is present in
forward momentum for pushoff), and loss of the normal 75% to 80% of clients after a stroke. There appears to be no
combination of movement patterns at the end of stance (hip difference in clients with left- or right-sided hemiplegia in
extension, knee flexion, and ankle extension) and at the end terms of frequency or severity of weakness.93 In contrast to
of swing (hip flexion with knee extension and ankle flexion). these studies, Landau and Sahrmann94 investigated the de-
Knutsson and Richards86 classified the motor control gree of functional impairment in strength that was a result of
problems of the hemiplegic gait into three descriptive types. deficits in the contractile element of the affected muscles.
Type I is characterized by inappropriate activation of the calf Their findings from comparisons of maximal tetanic con-
muscles early in the gait cycle with corresponding low mus- traction of the anterior tibialis muscle suggest that maximal
cular activity in anterior compartment muscles. In the type I voluntary muscle strength was not impaired. Although re-
activation pattern, the calf musculature is activated before the cent research has moved weakness back into the impairment
center of gravity passes over the base of support. This thrusts list, there is much more to be learned about the nature of
the tibia backward instead of propelling the body forward in weakness in CNS dysfunction.
a pushoff as normally occurs. The client with hemiplegia The objective assessment of active movement in hemi-
compensates for the backward thrust of the tibia by anteriorly plegia is commonly documented by therapists through the
tilting the pelvis or flexing forward at the hip. Type II con- use of the Fugl-Meyer assessment scale, derived from syn-
sists of an absence of or severe decrease in electromyo- ergistic stages as outlined by Brunnstrom,43 and is similar to
graphic activity in two or more muscle groups of the involved a version of Bobath’s long evaluation form, which gradually
lower extremity. This pattern of markedly decreased muscu- builds series of selective or fractionated movement in the
lar activity results in the adoption of compensatory mecha- arm, trunk, and leg.95
nisms to gain stability. Type III activation patterns consist of When clinically assessing weakness and control of active
abnormal coactivation of several limb muscles with normal movement patterns, the therapist analyzes and identifies the
or increased muscular activity levels in the muscle groups of client’s patterns of posture and movement in the trunk and
the involved side. This type of pattern results in a disruption extremities by position (supine, side lying, sitting, and
of the sequential flow of motor activity. standing) and in linked combinations. Active movement
The Stroke Impact Scale (SIS) and the short version, the control is evaluated in individual muscles, movement com-
Stroke Impact Scale–16 (SIS-16) are measures with high ponents, and movement sequences.54 Verbal directions or
reliability and validity for people poststroke. The long ver- demonstrations may be necessary to help the client under-
sion assesses eight domains: strength, emotion, hand func- stand what is desired. In this phase of the evaluation, the
tion, memory, physical function and mobility, communica- therapist should not physically assist the client’s movement
tion, ADLs, and social participation. The SIS-16 includes but should be prepared to prevent loss of balance.
most of the original items in the SIS physical function and While evaluating force production or weakness in all
mobility domain.88,89 these categories, the therapist gathers information about se-
quencing movements in increasingly complex patterns, tim-
Evaluation of Movement Control ing of muscle firing, and speed of movement. Muscle activa-
After the standardized testing has been performed, the thera- tion deficits in these categories may explain why some
pist continues to a subjective evaluation of movement com- clients with minimal weakness do not regain spontaneous
ponents to gather information to answer the question “why” functional use of the extremities.54
it is difficult for the client to perform specific movements or
tasks. Assisted Movement
Clients who have sustained a stroke have difficulty mov- After the evaluation of active movement, therapists use their
ing the trunk and the arm and leg on the affected side be- hands while retesting the movements to gain additional in-
cause of the presence of primary and secondary impair- formation about the relationships between impairments.
ments. Objective standardized measures for the primary Whereas the use of handling must be judicious, handling is
impairments are few; standard muscle testing has been ques- used during an assessment for the following purposes:
tioned for CNS deficits because of the numerous degrees of 1. To correct alignment to gather additional information
freedom available and the discrepancy in functional strength about strength, control, and orthopedic impairments
on the basis of the increasing degrees of difficulty of con- (Figure 23-5)
trolling linked trunk and extremity patterns as the body 2. To limit degrees of freedom of one of the joints to as-
moves from function in supine to function in sitting to func- sess relationships between intralimb segments
tion in standing. 3. To assist the movement of a weak muscle
4. To block or stabilize a joint to assess the performance
Active Movement and Strength of a weaker muscle group or to limit the degrees of
When active movement patterns in the trunk and extremities freedom of an intralimb segment54 (Figure 23-6)
are assessed, the therapist measures both strength and con- Example
trol. Paralysis, weakness, and imbalanced return are deter- Step 1. Assessment of forward reach in sitting by client with
minants of strength. Initiation pattern, sequencing, and left hemiplegia. Active movement patterns on left: client
control of firing patterns are indicators of control. Weakness initiates movement proximally; shoulder flexes to 60 de-
and paralysis after stroke have been largely ignored because grees, with internal humeral rotation; abducted, down-
of a lingering focus on spasticity. Some recent studies have wardly rotated scapula elevates during the movement;
shown that muscle weakness is, in fact, present and inter- elbow flexes, forearm supinates to 10 degrees; wrist
feres with the ability to generate enough force to achieve remains in flexion and radial deviation. Client leans trunk
and use of elbow flexors to lift arm. Clinical judgment or

hypothesis 2: Forearm, wrist, and hand position prevents
distal initiation and biases shoulder in internal rotation,
thus blocking use of elbow extensors.
Step 3. Test hypothesis 1: Therapist uses her or his hands to
externally rotate the humerus to neutral and asks client
to reach again. Result: Client activates elbow extension
halfway through range with shoulder forward flexion
and places wrist and hand on table. Clinical intervention
implication: Increased control of humeral external rota-
tion and increased control of accompanying scapular
pattern are important intervention goals to regain for-
ward reach of arm. Retrain trunk, scapular, and humeral
A B movement patterns, with emphasis on shoulder external
rotation and scapular upward rotation. Assess secondary
Figure 23-5 n A, Client with right hemiplegia trying to perform impairments of pectoral and rotator cuff tightness (rota-
an upper-body–initiated lateral weight shift to the left. Note that tor cuff is shortened if scapula is in an abducted posi-
the spine is straight and the right hip is off the surface. B, Therapist tion). If result is unchanged, test hypothesis 2: Therapist
uses her hands to correct and stabilize the lower trunk as the client supports wrist and hand with wrist splint or with his or
initiates the upper-body lateral movement to the left. The therapist her hand and asks client to reach again. Result: Client
gains information about trunk and hip control and secondary im- activates elbow extension and places the wrist and hand
pairments of trunk muscle tightness. Note that the spine is begin- on the table. Clinical intervention implication: Preven-
ning to curve as the client uses eccentric activity of the right lateral tion or blocking of wrist flexion limits the degrees of
musculature to control the movement. Active stretching of the right freedom, changes the internal rotation moment on the
quadratus lumborum and latissimus occurs if tight muscles are distal portion of the lever arm, and allows use of existing
present. elbow extensors. Use small wrist splint during indepen-
dent practice or use object to assist or preset distal seg-
ment during practice.
Tone
The evaluation of extremity tone, sometimes referred to as
spasticity, continues to be an integral part of poststroke
movement research and is part of the physician’s neuro-
logical examination. Over the years, leading physiologists
have split into two camps over the definition of tone. Dur-
ing the beginning of the 20th century, tone was thought of
as postural reflexes. In the 1950s the concept of tone was
thought of as a state of light excitation or a state of pre-
paredness.96 Granit97 later encouraged us to think of the
relatedness of both these views. He believed that the same
spinal organization is mobilized by the basal ganglia to
produce both manifestations of tone: a state of prepared-
ness and the postural reflexes.97 In the 1980s, scientists
challenged the concept that what led to a spastic movement
A B pattern was hypertonicity resulting from an exaggerated
Figure 23-6 n A, Client with right hemiplegia moving from sit- stretch reflex.98,99 A new construct emerged in the follow-
ting to standing. Note the tendency to use the left leg more than the ing years that acknowledged the contribution of both neu-
right, the left rotation of the upper body, and the position of the ral and nonneural elements to the phenomenon of “spastic-
right arm. B, Therapist uses her hands to stabilize the lower leg and ity.” This newer concept of spasticity explains why the
to assist lower-leg movements as the client initiates sit to stand. stretch reflex or tendon tap response (performed in a pas-
Note the change in upper-body position and the decrease in arm sive condition—during rest) is an “epiphenomenon and is
posturing. not the cause of the “spastic movement problem” that inter-
feres with movement.”100 Although the Modified Ashworth
Scale is an objective measure of spasticity caused by the
stretch reflex,101 it is not a measure of the functional prob-
lem that interferes with skilled movement. It is heartening
forward to assist with task but cannot reach arm forward to hear such discussions occurring among physiologists
to place it on table. because therapists are also questioned about their notations
Step 2. Clinical judgment or hypothesis 1: Weakness of of and changes in tone and they often have no objectively
scapula and humeral external rotators prevents antigrav- derived standard clinical system for measurement. The
ity use of elbow extensors during forward reach. Supina- debate over tone continues, but clients with CNS dysfunc-
tion of forearm comes from strong proximal initiation tion clinically display changes of muscle tone that result in
longer rehabilitation stays and problematic secondary im- establishment of antigravity control. Transition, the second
pairments.102 phase, represents the point in the functional activity at which
The response of a spastic muscle to stretch differs during there is a switch in the muscle groups that provide antigrav-
passive and active movements, leading some to question the ity control. The third phase is the completion of the activity,
usefulness of the classic numerical test of spasticity, the involving a final weight shift and the ability to maintain
Ashworth Scale. The Ashworth Scale rates the severity of postural control.54
tone from 1 to 5.103 If assistive devices are used, the following questions
The first noticeable change in tone is the change from should be asked: Is the device always used? If not, when is
the premorbid state. Clients in the acute phase of hemiple- it used? How is the device used? Could the device be used
gia exhibit, for varying periods of time, a lower than nor- another way that would foster trunk symmetry and allow
mal tonal state. Clients with paralysis of the extremities activity of the affected extremities?
exhibit low tone or hypotonicity. The extremities feel like
“dead weight” as the therapist moves them. As neuromus-
Evaluation of Secondary Impairments
cular return slowly begins, the extremities feel heavy,
but some “following” of passive movement patterns is Loss of Joint Range and Muscle Shortening
detected. In hemiplegia, loss of joint range is caused by muscle short-
As the client becomes more active, he or she uses all ening from poor alignment that is the result of weakness or
available movement patterns. Ryerson and Levit have de- muscle activation problems. Loss of alignment occurs early
scribed three specific situations, which in reality have in recovery, whereas muscle shortening and loss of range
overlap, wherein tone increases (see page 23 for a de- occur over time. When measuring joint range of motion and
tailed discussion).54 This increased tone, or clinical hyper- muscle shortening, the therapist must remember to consider
tonicity, occurs in the arm and leg if the client’s trunk the functional consequences of two-joint (multijoint) muscle
control is less than the demand of the task, if altered joint tightness.
alignment increases the tension of the muscle, or if the Example 1. In sitting (knee bent), the client has ankle
voluntary movement pattern of the extremity is unbalanced joint dorsiflexion range from 0 to 10 degrees; but in stand-
and disorganized.54,104 ing (knee and hip straight), ankle joint dorsiflexion range is
One clinical description of increased extremity tone 220 degrees. This functional loss of ankle range causes
put forth in the 1970s is still somewhat useful today: se- significant problems for standing and walking. Loss of an-
vere hypertonicity makes coordinated movements impos- kle joint range in standing may be the result of gastrocne-
sible; moderate hypertonicity allows movements that are mius and soleus, tensor fasciae latae, or hamstring muscle
characterized by great effort, slow velocity, and abnormal tightness (Figure 23-7).
coordination; slight hypertonicity allows gross movement Range-of-motion measurements should be documented
patterns to occur with smooth coordination, but com- in terms of functional position. Extremity muscles that
bined, selective movement patterns are uncoordinated or cross multiple joints are the most common groups to
impossible.105 shorten and limit joint range in hemiplegia. Muscle shifting
(changes in the resting position of muscle bellies and ten-
Equilibrium and Protective Reactions dons) occurs with prolonged changes in alignment and loss
Equilibrium reactions help us to maintain or regain balance of joint range.
by keeping the center of gravity within the base of support. Example 2. Long-standing wrist flexion may cause the
Equilibrium reactions are often referred to as the body’s ulnar wrist extensor to slip volarly and function as a wrist
“first line of defense” against falling. They occur when the flexor. Similarly, a position of knee flexion with ankle plan-
body has a chance of winning the battle against gravity. If tarflexion and subtalar varus may lead to lateral shifting of
equilibrium reactions cannot preserve balance, the second the anterior tibial muscle belly. As the muscle shifts later-
line of defense emerges: protective reactions. One of the ally, the tension increases distally and foot supination be-
best known protective responses in the arm is the “parachute comes more pronounced.
reaction.” Protective responses in the leg in standing posi-
tions include hopping and stepping. Pain
When assessing equilibrium or balance reactions in cli- Two commonly used standardized pain measurement scales
ents with hemiplegia, the therapist remembers the distinc- are the visual analog pain rating scale and the McGill Pain
tion between equilibrium reactions and protective reactions. Questionnaire.106,107 These scales focus primarily on the in-
Equilibrium reactions should be assessed while slowly tensity of pain but provide an objective measure of interven-
moving either the limb or trunk away from the base of sup- tion effectiveness. For an in-depth discussion of the topic of
port. The amount of control in the trunk and supporting pain management, see Chapter 32.
limb, the size of the base of support, and the available range The presence of pain in hemiplegia is devastating for the
of motion as well as the evaluator’s handling skills affect client and makes movement reeducation difficult. Shoulder
the response (see Chapter 22). pain is the most frequent pain complaint after stroke.11,108
Pain must be evaluated specifically and should not be al-
Descriptive Analysis of Functional Activities lowed to occur during intervention; the “no pain, no gain”
When evaluating functional activities, the therapist assesses message that is sometimes used in sports or orthopedic in-
three phases of the movement pattern. The first phase is the tervention should not be used in neurorehabilitation. Pain is
initiation of the act, which includes the body segment initi- an indicator that joint alignment or movements are incorrect.
ating the movement, the direction of movement, and the See Box 23-3 for general questions.
Figure 23-7 n A, Client with right hemiplegia with limited range in hamstring, tensor fasciae la-
tae, and gastrocnemius and soleus muscles. B, Client has sufficient range at ankle to keep foot on the
floor in sitting and as she initiates the rise to standing. C, As she stands and reaches the limit of range
of these two muscle groups, her body compensates. The pelvis rotates right, and the tight medial
hamstring adducts and internally rotates the femur and pulls the knee into extension as its medial
insertion becomes more anterior to the joint. D, As the knee extends more, the calcaneus moves into
equinus and varus. The foot supinates as a result of calcaneal varus and external tibial rotation from
the tight tensor fasciae latae.
Motor Evaluation Forms BOX 23-3n QUESTIONS FOR SUBJECTIVE

The foregoing information, once gathered, can be placed on EVALUATION OF PAIN
an evaluation form in many ways. Every medical institution Location: Where is the pain? Pinpoint the location.
seems to have its own evaluation form and its own system of Type: What does it feel like?
recording data. Active movement at the shoulder joint may n Pins and needles
be described in one institution in terms of percentages of n Sharp and stabbing
synergistic stages, at another institution by a narrative of n Aching
degrees and planes of movement, and at still another by n Dull
functional outcomes of shoulder movement. At one hospital n Pulling
the documentation of pain may be descriptive, and at an- Occurrence: When does the pain occur?
other it may be numerical. It is important to keep in mind the n At rest
substance of the evaluative material, not the form in which n During movement
it is described. A detailed motor evaluation form is neces- o Range-of-motion exercises
sary for the establishment of realistic goals and for subse- o Weight-bearing exercises
quent treatment planning, but the specific form depends both o A specific part of the movement
on the needs of the specific clinical setting and on the clini-
cian’s choice.
Recognizing Needs cannot rise to stand. Common primary impairments include

The information obtained from the total evaluation provides paralysis or weakness of the left arm and leg, a loss of
the basis for answers to the following questions: muscle activation resulting in an inability to coordinate
n What activities are possible? trunk and lower-body movements to allow forward weight
n What activities are not possible? shifts in sitting and/or to perform transfers from bed to chair
n How do the movement impairments and secondary and/or inability to sequence forward arm movements, and
impairments relate to activity performance? impaired proprioception in the extremities. Secondary im-
By understanding the impairments and their relationship pairments that begin to appear by the end of the acute recov-
to activity limitations, the therapist can answer the following ery phase might include trunk asymmetries (lateral trunk
question: What significant movement components are miss- flexion with a left spinal convexity; inferior shoulder sub-
ing? The answer to this question becomes a hypothesis for luxation; loss of ankle joint dorsiflexion range; muscle
intervention planning. How the possible is accomplished shortening in the pectoralis, wrist flexors, and gastrocne-
and why the impossible exists provide logical suggestions mius; and edema in the hand and foot.
for selection of intervention techniques. In the rehabilitation phase of care, the primary impair-
Therapy intervention occurs at either the level of activity ments of weakness and loss of muscle activation pattern
limitation or the level of movement-related primary and (motor control) begin to improve. As the client performs
secondary impairments. The process of establishing goals tasks that exceed his or her level of trunk strength and control,
and selecting activities for intervention begins with clinical clinical hypertonicity in the arm and leg may increase as
decision making or problem solving. a strategy for maintaining balance or reinforcing trunk
control. Secondary impairments of shoulder pain in-
CLINICAL DECISION MAKING crease, along with increasing instances and degrees of
AND PROBLEM SOLVING muscle tightness and continued loss of trunk-extremity
Problem solving is a process of gathering and analyzing alignment.
evaluation information from movement analysis, organizing
and reflecting on this information to develop hypotheses for Developing Hypotheses for Significant
causal relationships between activity limitations and signifi- Impairments
cant impairments, and establishing and prioritizing goals for The process of motor performance evaluation results in a list
therapeutic intervention. The problem-solving process is of activities and related impairments. However, not all these
also used to hypothesize how the movement problems of the impairments directly relate to each activity limitation of the
trunk, arm, and leg are interrelated and how these problems client. The therapist, using clinical judgment, hypothesizes
relate to the ability to perform tasks. Movement control a causal relationship between frequently occurring impair-
deficits, secondary impairments, and compensatory or atyp- ments and activity limitation. These impairments, called
ical movement patterns should be identified in relation to significant impairments, are the ones that must be changed
each significant activity limitation. for measurable changes in movement and function to oc-
cur.54 The other impairments are not forgotten but are re-
Analyzing Evaluation Material evaluated later as improvement begins and new activity
The relationship between activity performance and primary goals are chosen. The significant impairments are often used
and secondary impairments in stroke has not been reas the focus of short- and long-term goals because they are
searched. Therefore, this relationship, which is the basis for the underlying building blocks of the selected activity goal.
therapy intervention, must be derived from clinical experi- Because functional movement depends on the linkage of
ence and judgment. Clinical reflection guides the evaluation trunk and extremity movements, the therapist develops hy-
process—what should be evaluated and how. As a result of potheses between impairments in the extremities and spe-
the evaluation, the therapist has a list of functional skills that cific levels of trunk control to set goals that result in im-
are difficult or impossible for the client to perform and a list proved activity performance. If weakness and control deficits
of primary and secondary impairments that relate to the at- of the trunk, arm, and leg are treated separately, the client
tempted performance of that task. The therapist analyzes may see improvement in the impairments but not see a
this information with the goal of identifying common im- change in function (Box 23-4). (See Chapters 4 and 9 for
pairments in categories of tasks: Which primary impair- further discussion.)
ments are major impediments in each task analyzed? Are
there secondary impairments that interfere with the client’s Goal Setting
ability to perform specific critical movement components? Once the therapist reviews the desired functional goal and
What is the level of trunk-extremity control during task per- identifies the underlying significant impairments, the inter-
formance in each functional position evaluated? While ana- vention plan is created and discussed with the client.
lyzing the evaluation material, the therapist pays attention to
all significant factors that limit performance of tasks, includ- Functional Goals
ing environmental, cognitive, perceptual, and emotional Functional goals are based on the needs and desires of the
barriers. client and on the functional impairments that were identified
by the therapist during the initial assessment. Functional
Example goals should represent a significant change in the patient’s
A patient after an acute stroke with left hemiplegia cannot level of independence, be practical, and reflect improvement
perform morning daily care activities at the sink while sit- in a specific activity limitation. They state the desired func-
ting in a wheelchair, cannot transfer from bed to chair, and tion and the expected level of performance.54
n MOVEMENT COMPONENT
BOX 23-4 n COMPONENT GOALS IN
BOX 23-5
CONTROL MODEL OF POSTURAL CONTROL FUNCTIONAL TRAINING
Postural tone and stability Component goal (power): Restore strength in trunk and
Trunk control extremity patterns (individual muscles, components,
Level I: Basic movement components sequences)
Upper-body– and lower-body–initiated movement Component goal (structure): Minimize or eliminate second-
Anterior ary impairments
Posterior Component goal (control): Reeducate patterns of control
Lateral (sequencing and timing)
Level II: Coordinated trunk and extremity patterns
Level III: Power production
Equilibrium and protection
Choosing Intervention Techniques
Once the problem-solving process of goal setting is finished,
therapists can select specific intervention techniques and
Example. Client will stand independently and safely activities. Therapists have many techniques to choose from
while performing self-care activities at the bathroom sink. to meet their goals. After a stroke, most clients will not fully
regain normal movement patterns regardless of the type of
Long-Term Goals intervention they receive.
A long-term goal should reflect a major improvement in a Controversy exists as to the means of increasing func-
primary or secondary impairment or an increase in level of tional mobility and performance in clients who have had a
performance of an existing skill. The accomplishment of a stroke. One school of thought teaches compensatory pat-
long-term goal brings the client closer to the functional goal. terns or hopes for some use of the affected side through
The time it takes to accomplish a long-term goal varies tre- task-specific practice without direct intervention for the
mendously depending on the frequency of treatment and the neurological impairments. The other prevalent practice pat-
length of time after stroke. The therapist may set many tern is to increase functional movement patterns on the af-
short-term goals to achieve one long-term goal. Long-term fected side to help achieve an activity goal by increasing
goals may be stated in functional terms, but they usually control and strength of movement sequences of the trunk
reflect a change in a primary impairment: an increase in and limb through specific levels of reeducation.54,109-111
strength, movement control, or balance.54 A combination of these two practices may be useful:
Examples impairment-based intervention strategies to reeducate
Functional Goal. Client will be able to perform meal movement and training strategies to foster desirable com-
preparation activities in the kitchen safely (while standing). pensations—a functional reeducation strategy. This type of
Long-Term Goals intervention includes strengthening trunk and extremity
1. Client will perform upper-body–initiated movement (lat- linked patterns of movement, minimizing or eliminating
eral and rotational) while standing, supporting hips secondary impairments that interfere with regaining con-
against a kitchen counter. trol, teaching appropriate compensations, and training the
2. Client will safely stand near the kitchen counter and client to practice functional movement patterns in the con-
maintain balance during far-reach movements of the un- text of daily tasks54,112 (Box 23-6). Research findings sup-
involved arm. port a link between the trunk and upper extremity and the
trunk and lower extremity during reaching activities.113,114
Short-Term Goals One result of this research has been to design treatment
A realistic short-term goal should be achievable quickly and interventions that restrain trunk movements during forward
should be based on the result of the patient’s response to reach retraining to increase control of elbow extension
handling during the evaluation of movement. Short-term movement in the paretic arm.115,116
goals should directly relate to the accomplishment of the For reeducation to be effective, therapists must allow the
long-term goal. There are multiple short-term goals that re- patient to initiate the active trunk and extremity pattern,
late to one long-term goal. Short-term goals are compiled
from the list of relevant secondary impairments or desired
increases of strength or movement control. These goals are
measurable but do not in and of themselves result in a func- BOX 23-6n REEDUCATION STRATEGY FOR
tional change.54 INTERVENTION
When stated in terms of movement control rather than Reeducating basic trunk movement components
functional performance, these goals include the reestablish- Linking coordinated trunk and extremity patterns
ment of generalized movement patterns that link movement n Weight bearing
patterns of the trunk and extremities (Box 23-5). n Movements in space
Example. Decrease tight hamstring and gastrocnemius Preventing, minimizing, eliminating secondary impairments
muscles in standing position to allow the foot to remain flat Teaching appropriate compensations
on the floor during assisted and independent upper-body Teaching independent practice routines
movements while standing (e.g., forward reach beyond
arm’s length).
must move from assisted practice to independent practice recovery of movement increases, therapists must critically
with the assistance of appropriately selected objects or ver- analyze research findings and judiciously integrate them
bal cues, and must teach the patient appropriately staged with their clinical experience and judgment.
practice patterns. Studies based on the “learned nonuse”
phenomenon described by Taub have shown that when pa- COMMON IMPAIRMENTS AND
tients are encouraged to use the affected arm rather than re- INTERVENTION SUGGESTIONS
ceiving pessimistic messages about its potential, movement
and functional use, even if limited, are possible.117,118 Weakness and Loss of Control
Regardless of intervention type used, task-performance Diminished muscle strength, either paralysis or weakness, is
practice or a reeducation strategy, there comes a time in the an important category of impairment in hemiplegia. A para-
recovery process when therapists help the client select prac- lyzed muscle is unable to contract to produce enough force
tical compensatory strategies. Compensatory strategies are for movement. A weak muscle contracts insufficiently for
taught when the client needs to function independently and joint or body segment movement or to allow functional per-
cannot yet use the affected arm because of insufficient formance.90-92 In a client who has sustained a severe, acute
recovery or the severity of damage. To be appropriate, the stroke, the paralysis or weakness affects the majority of
strategy should incorporate the use of the involved extremi- muscles and results in a loss of functional movement in the
ties and use appropriate trunk movement patterns to maxi- face, trunk, arm, and leg. In clients who have had less severe
mize future return of movement. Undesirable compensa- strokes, some muscle groups are weak and produce move-
tions are patterns that are so asymmetrical that they fail to ment, whereas other muscles are paralyzed and cannot be
incorporate available movements of the affected trunk and activated.119
extremities (Figure 23-8). Weakness from stroke differs from generalized weakness
Although current literature generally applauds function- and orthopedic weakness: it involves one entire side of the
based techniques, therapists in clinical settings use hands- body and includes the trunk and extremities. After a stroke,
on approaches to increase muscle strength and control and the trunk, perhaps because of its bilateral cortical innerva-
to decrease impairments that block the emergence of new tion, does not display the degree of weakness found in the
functional patterns.54 As research in movement science and extremities. As a result of differences in testing methods,
Figure 23-8 n A, Client with left hemiplegia using his right side to move to sitting and not incor-
porating movement of the left side—an undesirable compensation. B to D, Client moving to sitting
while using as much control as possible on the left side to assist the movement to sitting.
position, and design, there is no consensus on the degree of is influenced by the input from the corticospinal tracts,
trunk muscle weakness after a stroke: some research find- the vestibular system, the alpha and gamma systems, and
ings indicate a loss of lateral paretic-side trunk strength,120 peripheral-tactile and proprioceptive receptors.138 Normal
others report no significant difference in lateral trunk postural tone allows a constant interplay among the various
strength,121,122 and others find slight weakness in the trunk muscle groups in the body and imparts a constant readiness
extensors.123,124 However, it is clear that weakness in the to move and to react to changes in the environment (internal
extremities interferes with functional use in either weight and external). It provides an ability to adjust automatically
bearing or movement in space.54,125 and continuously to movements. These adjustments provide
the proximal fixation necessary to hold a given posture
Model of Postural Control against gravity while allowing voluntary and selective move-
Trunk control allows the body to remain upright, to adjust to ments to be superimposed without conscious or excessive
weight shift, to control movements against the constant pull effort.
of gravity, and to change and control body position for bal- Trunk Control. Trunk control can be divided into levels
ance and function. Therapy based on neurophysiological of increasing complexity. The first level of trunk control is
models stressed facilitation of trunk rotation to gain trunk the ability to perform the basic movement components.
control. Newer clinical models of postural and trunk control Trunk strength and control at this level provide a base that
began appearing around 1990.104,126,127 Information from allows extremity movement to be combined and used for
motor control science has resulted in revision of therapists’ function. Retraining strength and control of basic trunk
thoughts on trunk control.128-130 movements in the three cardinal planes is a prerequisite for
In a movement component model of postural control, the coordination of trunk and extremity patterns for tasks.
trunk control has levels of increasing difficulty131 (see Box Trunk movements in sitting are initiated from the upper
23-4). Trunk control not only helps us remain upright but trunk or the lower trunk according to the demands of the
also allows weight transfer to free an arm or leg for function. task. In standing, functional trunk movements are initiated
For some functional movements, such as sitting, trunk con- from the upper trunk (if the head or arm is initiating a task)
trol keeps the upper and lower trunk stable during weight or the lower extremity. The two initiation patterns result in
shifting and balance. For other tasks, such as reaching for- different spinal patterns, different types of muscular activity,
ward beyond the length of the arm, the upper trunk is stable and changes in the distribution of weight54 (Tables 23-3 and
and adjusts to the lower-body–initiated anterior weight 23-4). These basic movement patterns allow the body to be
shift.54 positioned for functional use.
Additional postural control models, based on a develop- The second level of trunk control, coordination of trunk
mental or systems model, are well documented.105,132-134 and extremity patterns, may best be explained through the
Research in the field of postural control shows that the level concept of anticipatory postural control. Anticipatory con-
of trunk control and trunk strength correlates with sitting trol allows the coordinated linkage of trunk and extremity
balance, that extremity function correlates with trunk con- patterns before the activation of extremity movements: it
trol, and that loss of trunk strength occurs in all planes.135-137 allows us to sit and reach beyond arm’s length without fall-
Postural Tone and Stability. Clients with hemiplegia ing forward or to step forward with one leg as walking is
frequently have alterations in both muscle tone and postural initiated. Researchers have identified altered anticipatory
tone. Postural tone refers to the overall state of tension in the postural responses in people after stroke in both sitting and
body musculature. Postural tone is tone that is “high” standing positions. The pattern of anticipatory responses
enough to keep the body from collapsing into gravity but poststroke appears to be preserved, but the timing of the
“low” enough to allow the body to move against gravity. It response is slowed.139,140
TABLE 23-3 n UPPER-BODY–INITIATED WEIGHT SHIFT PATTERN: SITTING
WEIGHT SHIFT SPINAL PATTERN MUSCLE ACTIVITY

Anterior movement—reach down to floor Flexes Eccentric extensor activity
Posterior—to sit back up Extends Concentric extensor activity
Lateral—reach sideways and down to right Laterally flexes with concavity on right Eccentric lateral activity on left
Lateral—comes back up to middle Spine moves back to neutral Concentric lateral activity on left
TABLE 23-4 n LOWER-BODY–INITIATED WEIGHT SHIFT PATTERN: SITTING
WEIGHT SHIFT SPINAL PATTERN MUSCLE ACTIVITY

Posterior weight shift Flexion Concentric flexor activity
Anterior weight shift Extension Concentric extension activity
Lateral weight shift to right Lateral flexion—convexity to right Eccentric lateral activity on right
Concentric lateral activity on left
This level of trunk control allows the trunk to remain motion in the arm, or to strengthen movement sequences in
stable yet adapt to movement of the arms and legs. There are the arm. It is not used to inhibit tone (Figure 23-10). The
two different ways this happens: trunk movements occur as muscles of the arm are linked with trunk weight shifts dur-
postural adjustments to extremity movement around mid- ing active weight bearing.54 Table 23-5 presents the linked
line, or trunk movements can precede voluntary movements trunk and arm muscle activity during active weight bearing
to help extend the reach of the extremities. These coordi- for one functional task.
nated movements can occur in supine, sitting, or standing The ability to support body weight on both legs for sta-
positions as demonstrated in the following three examples. bility and movement control is important in sitting, stand-
1. While sitting, the client reaches down or sideways to the ing, and walking retraining. Movements of the trunk in
floor to pick up an object. As the arm reaches down, the sitting and standing occur with constant changes of muscle
upper body initiates the anterior weight shift. To extend activity in the legs as part of the base of support, to adjust
the reach of the arm, the lower body provides stability yet to demands of weight shifts, and to increase activity levels
adjusts and adapts. of leg muscles to initiate standing weight shifts. Loss of
2. When the client lifts up a leg to tie a shoe, the lower body control of weight bearing on both legs or on one leg has an
initiates a posterior weight shift. The upper body adjusts immediate effect on balance. Problems of weight-bearing
to the weight shift and to the demands of the arm and control of the leg may exist because of weakness; because
hand as they tie the shoe. of muscle shortening in the pelvis, hip, knee, or ankle; or
3. In standing, upper-trunk movements occur as postural because of posturing. When the leg cannot actively support
adjustments as the legs initiate both the stance phase body weight, undesirable asymmetrical compensations re-
(forward weight shift) and swing phase (stepping) of sult. A significant and often overlooked prerequisite for
walking. active control of the leg in weight bearing is a stable,
The third level of trunk control allows strength and stabil- aligned upper body. The use of forearm or extended-arm
ity for power production from the arm or leg. The movement weight bearing in standing provides external stability to
and control of the trunk are used to support power produc- the upper body while allowing the therapist to reeducate
tion in the extremities for propulsive activities such as stair control of bilateral or unilateral weight-bearing move-
climbing, jumping, running, throwing, hitting, and rowing. ments in the leg.
The entire model is summarized in Box 23-4.
Muscle Activation (Motor Control) Deficits
Extremity Weakness Common muscle activation deficits include improper initia-
Weakness in the arm and the leg results in ineffective and tion, the inability to grade timing and force production, and
inefficient functional patterns in daily life. Intervention for the inability to sequence muscles for task performance.
weakness in the extremities includes reeducation of move-
ments in space, reeducation of weight-bearing movements, Improper Initiation
and training of and appropriate initiation and sequencing of Improper initiation of movement occurs when the
movement. Most clients with hemiplegia regain enough client attempts to move the arm or leg in space and sub-
control in the leg to stand and walk, but those same patients stitutes the stronger proximal muscles for weaker distal
may not be able to use the arm for any purpose. Today the muscles.54
concept of “learned nonuse” may help therapists understand Example 1. If we ask a client to lift a hemiplegic arm
why the discrepancy between arm and leg recovery exists. and reach forward for an object, she or he often initiates the
Wolf and colleagues118 conclude from studies of hemiplegic movement proximally instead of distally with the hand and
patients that learned nonuse does exist in some patients who forearm, using the stronger elevators and abductors instead
have had a stroke and suggest a program of “forced use” of the weaker hand and forearm muscles.
training. Although the research training model may not be This is also seen during walking.
directly transferable to the clinic, this study points out the Example 2. The client initiates the swing phase of gait
benefits of incorporating the use of the affected side in inter- proximally instead of distally with the foot, using the stron-
vention strategies. ger pelvic elevators or rotators instead of the weaker ankle
Distal reeducation is an important component of early and foot muscles.
reeducation that has been neglected by therapists because of
a previous belief that proximal return comes before distal. Inappropriate Muscle Selection
Distal reeducation trains the client to be able to initiate Inappropriate muscle selection for the task occurs when the
movements from the hand or foot, instead of the common client substitutes a strong muscle group for a paralyzed
proximal initiation patterns seen during attempted reach or muscle although it is inappropriate for the function.
stepping (Figure 23-9). Example. When the hamstrings are weak, the client may
Weight bearing on either the forearm or the extended arm use the quadriceps to lift the leg up a step. This results in
is used as a postural assist during transition activities such strong overshifting in the trunk and makes balance precari-
as side lying to sitting, or as a means of supporting the ous.
weight of the upper trunk in sitting or standing, and is used
to stabilize objects during task performance. The activity of Inappropriate Sequencing
accepting weight through the arm is not passive but ex- Inappropriate sequencing includes improper initiation and
tremely active and dynamic. Forearm weight bearing in sit- excessive co-contraction. Excessive co-contraction occurs
ting or standing is used to activate trunk movements, to re- when the client activates too many muscles either at the
establish scapulohumeral rhythm, to maintain range of same time or out of sequence for the task.
Figure 23-9 n A to C, Client with left hemiplegia. Therapist assists movements of forearm, wrist,
and hand as client practices increasing distal arm control. D to F, Therapist introduces object and
assists client as he learns to control the object and the movement. G, Independent practice. H, Client
uses same movements with a similar object.
Excessive Force Production Spasticity

Excessive force production occurs when the patient acti- There is considerable debate in the academic and clinical
vates muscles with inappropriate effort during voluntary therapy community over the clinical relevance of spasticity
movement. When force is excessive, the movement pattern and the need to address it in treatment.90,141 Characteristics
is slow and the extremity feels stiff. Frequently, these mus- of a spastic muscle include increased velocity-dependent
cles easily fatigue and the extremity slowly falls back to the resistance to stretch, a clasp-knife phenomenon, and hy-
starting position. Therapists often label this movement a peractive tendon responses. It is important to remember
“spastic” pattern and intervene with inhibition techniques. that spasticity is the increased tension in a muscle when it
However, if this pattern is not from spasticity but from a is passively stretched. The type of high tone that occurs
muscle activation pattern, the intervention should focus on with active, voluntary movement is a different phenome-
reeducation of control, not inhibition.54 non: it is the result of both neural and nonneural changes
in the muscle. As described earlier in this chapter, Dietz100
Hypotonicity recommended that we assign different names to these two
Hypotonic muscles offer no resistance to passive movement different phenomena of increased tone: he suggested
and are clinically associated with paralysis and weakness. “spasticity” and “spastic movement disorder.” Clinicians
Clients who have had a severe stroke may have no visible realize that although movement interventions may not
movement in the extremities; the extremities display no re- change spasticity, if the “spastic movement disorder” is
sistance to passive movement and feel heavy. As strength and left untreated the task of muscle reeducation becomes
control in the muscles of the trunk and extremities increase, more difficult. Additional, secondary problems such as
the hypotonicity lessens. If weakness persists, the secondary joint dysfunction, pain, and undesirable compensatory
impairments of poor joint alignment, muscle and soft tissue movements may result. I suggest renaming Dietz’s second
tightness, and eventually joint deformity or contracture will category of spastic movement disorder to clinical
be more predominant than problems of spasticity. hypertonicity.
clinical hypertonicity: (1) increased tone as a result of

proximal instability, either insufficient trunk control for the
task or instability of proximal limb musculature (e.g., hip
weakness), (2) increased tension on a two-joint muscle
caused by poor joint alignment and the resultant shortening
or shifting of muscles, and (3) increased tone that is volun-
tarily produced during attempts at active movement, espe-
cially in the extremities.54
These situations are divided into three groups for ease of
description, but, in reality, overlap occurs between groups.
Insufficient postural stability and control in the trunk is the
first explanation for arm and leg clinical hypertonicity. The
extremity patterns of arm flexion or leg extension occur as
an atypical balance strategy when the body is unstable.
Example 1. If a client has sufficient trunk control in sit-
ting, the arm and leg display normal resting positions. How-
ever, during the rise to stand, the arm postures in flexion.
The arm postures most obviously during the transition phase
of the stand when the hips are off the surface and the center
of gravity of the body is behind the feet, the new base of
support.
Treatment of the increased tone, in this case, would not
be directed at the arm. Rather, intervention would focus
on increasing stability of the upper body during lower-
trunk–initiated sit-to-stand patterns and on increasing
strength and control of the leg in weight bearing. As
the trunk and leg gain more control, the arm posturing
decreases (see Figure 23-6).
Figure 23-10 n Client performs lower-body–initiated posterior A second situation in which clinical hypertonicity exists
movement during forearm weight bearing at a table. The therapist is when muscle tension increases in a two-joint muscle be-
uses her hands to stabilize the humerus, and as the patient moves cause of changes in alignment of one of the joints.
back, the therapist’s left hand slowly stretches or releases tight Example of Second Situation. In sitting, tightness in
tissue in the rotator cuff. the gastrocnemius muscle across the knee may not affect the
ability to keep the heel on the floor. However, as the client
extends the knee, the limit of tightness in the gastrocnemius
TABLE 23-5 n TRUNK AND ARM–LINKED is reached and the distal end of the tendon shortens and the
MOVEMENTS IN FOREARM WEIGHT BEARING ankle plantarflexes. When gastrocnemius tightness pulls the
calcaneus into equinus, it also moves the calcaneus into
FUNCTIONAL TASK varus because of the position of its insertion on the susten-
Sit at a table with both forearms supported on the table. Keep- taculum tali. The resultant position, ankle plantarflexion and
ing both arms on the table, move forward toward the table, foot supination, has been labeled a “spastic foot position”
and then move body back away from the table. (see Figure 23-7).
TRUNK AND BODY MOVES BODY MOVES However, after lengthening of the gastrocnemius across
ARM LINK FORWARD BACK the ankle and knee in standing and correction of the calca-
Spine Extends Flexes neal position, the patient can stand and keep the foot on the
floor.
Scapula Adducts and Abducts and
Example 2 of Second Situation of Clinical Hyperto-
depresses elevates
nicity. In an anterior shoulder subluxation, the anterior
Glenohumeral Less flexion More flexion
movement of the humeral head increases tension on the bi-
joint
ceps proximally, resulting in elbow flexion. As the tension
Elbow Flexes Extends
increases, the forearm begins to supinate. As the humeral
head and scapula are repositioned, the tension on the biceps
is diminished. If the therapist holds this proximal correction,
the elbow and forearm posturing activity stops; the forearm
Clinical Hypertonicity slowly pronates and then the elbow extends.
Clinical hypertonicity can be separated from spasticity, in For the posturing to permanently stop, therapy interven-
part, because clinical hypertonicity responds to movement tion must help increase strength and control in linked trunk
interventions. Research findings have helped clinicians real- and scapulohumeral patterns to prevent the malalignment.
ize that spasticity is not the major problem in hemiplegia. The third situation, inappropriate voluntary muscle acti-
However, for interventions to be successful, it is important vation, occurs when the client is trying to move the arm or
to understand the situations in which hypertonicity occurs. leg. The client uses the muscles that have returning strength
At least three different situations result in an increase in in the only way he or she knows how. Historically, these
patterns were labeled “synergistic or spastic.” This label re- lower contour of the shoulder on the hemiplegic side with
sulted in intervention strategies of inhibition. If these pat- shortening of the trunk and concavity of the spine. The
terns are thought of as unbalanced or inappropriately initi- heavy weight of the weak arm pulls the upper quadrant into
ated, or inappropriately sequenced, therapy intervention is excessive forward flexion. In this position, the scapula ele-
more appropriately directed. The abnormally extended or vates and tips forward on a flexed, rotated thoracic spine
flexed leg movement changes when the patient learns new (Figure 23-11).
activation patterns or strengthens weaker muscle sequences. Another compensatory pattern is excessive spinal flexion
Often, these are “learned” patterns and are difficult to throughout the spine, the convexity on the weak side, and
change. Early reeducation should include training in these spinal rotation toward the affected side. Clients with this
skills of controlling sequencing, intensity, and duration of asymmetry usually shift weight onto the stronger hip. This
firing. pattern viewed from the front or rear gives the appearance of
The underlying cause of each of these situations is weak-
ness or loss of control. Therefore, for changes in clinical
hypertonicity to last, treatment interventions must address
the underlying causes. In each of these situations, the cause
is weakness or loss of control of activation patterns.
In this model, generalized inhibition is inappropriate be-
cause it does not focus on the underlying cause. Intervention
techniques that focus on global inhibition of extremity
tone—maximal elongation, vibration, biofeedback, cold, or
relaxation—rarely result in a permanent change in the tone.
The temporary decrease of clinical hypertonicity that occurs
with any of these methods does not by itself directly lead to
an increase in function. If used, they must be immediately
followed by therapeutic exercise to create a learning envi-
ronment that improves motor performance.142,143
Toe Posturing
There are two patterns of toe posturing: toe clawing and toe
curling. Toe clawing, metatarsal hyperextension with pha-
langeal flexion, is a result of loss of alignment; and toe curl-
ing, metatarsal and phalangeal flexion, is a response to insta-
bility of the trunk and leg during standing, that is, part of a
balance response.54
Toe curling and toe clawing interfere with comfort during
standing and walking. Problems of blistering on pads of the
toes and on the top of the proximal interphalangeal joint and
toe pain occur in the intermediate and long-term stage of
hemiplegia as the result of the toes rubbing on the tops of the
shoes and digging into shoe soles. Relief from pressure and
pain on the toe pads (tips) comes with use of commercially
available “hammertoe crest pads” available from distributors
(e.g., AliMed) or from medical pharmacies.
Loss of Alignment
Muscle weakness or atypical anticipatory control in the
trunk leads to atypical alignment patterns in the trunk and Figure 23-11 n A, Client with right hemiplegia. Contour of right
shoulder and pelvic girdle. This loss of alignment creates an shoulder appears lower and longer than on the left. Pelvis lists
atypical starting position for functional movement, inter- downward on the right. B, Therapist lifts the client’s upper body up
feres with muscle activation patterns, and limits weight out of forward flexion and corrects the position of the glenohu-
transfer between extremities. Loss of alignment in the trunk meral joint. Note that the contour of the right shoulder is now
in sitting and standing is analyzed and incorporated into higher and shorter than the left shoulder contour. The trunk is later-
intervention goals to reeducate functional trunk and limb ally flexed with the convexity on the right. These movement com-
coordinated movements. The commonly described pattern ponents, convexity of a lateral curve, high shoulder, and low pelvis,
of trunk shortening (lateral flexion with the concavity) on are compatible. C, Client’s arms are supported symmetrically by a
the affected side is only one of the possible alignment prob- table. Note the convexity of the curve on the right and the low
lems. More routinely, weakness of the trunk on one side pelvis on the right. D, Same client moving forward and down with
results in a flaring of the rib cage and lateral flexion of the an upper-body anterior weight shift. This position allows the thera-
spine with the convexity on the affected side. The “appear- pist to evaluate the position of the trunk. Note the tendency to avoid
ance” of shortening on the side comes from a number of weight on the right hip. The trunk is laterally flexed with the con-
compensatory adjustments to balance or as a result of the vexity on the right, and the right shoulder is higher than the left
heavy weight of a weak arm. Often therapists confuse the shoulder.
a low shoulder. This pattern of rotation to the weak side does

not occur acutely but develops over time in clients who sit
more than they stand or walk.
Lateral translation of the thoracic spine from the hyper-
mobile point of T10 occurs as a means of balancing when
trunk weakness results in a lateral curve, with the convex-
ity on the affected side. This asymmetry is common in
sitting when the client is encouraged to stand by pushing
up with the good arm without putting any weight on the
affected leg. It occurs in standing when quad canes or
hemiwalkers are used before standing control on both legs
is reeducated. The stable external cane acts as a “third
stable leg,” and the “long, weak” side translates laterally as
the unaffected arm pushes down into the cane for stability.
This pattern creates a “skinfold” on the affected side that
has been confused with shortening of the affected side (see
Figures 23-1 and 23-4).
In standing, because the need for leg stability and move-
ment control is much greater than in sitting, the trunk
compensates to accommodate the demands of the leg. Of-
ten the upper-body and lower-body patterns are opposite
one another (i.e., counterrotational). If the leg is in a posi-
tion of ankle plantarflexion and knee extension, the hip
flexes with pelvic rotation toward the affected side. The A B
upper body then counterrotates to provide an equal and Figure 23-12 n A, Client with left hemiplegia and severe weak-
opposite balance pattern to allow the client to stand. The ness. The left arm and hand are in shoulder internal rotation, elbow
opposite may also occur: if the pelvis and hip rotate toward extension, and forearm pronation. The left wrist flexes, and the
the unaffected side because of learned compensatory swing hand pronates and radially deviates on the wrist. B, Client with left
and/or stance patterns, the upper body rotates toward the hemiplegia with atypical movements. The left arm is positioned in
affected side to provide a counterrotation for balance (see shoulder internal rotation and elbow flexion. As the elbow flexes,
Figure 23-4). the forearm begins to supinate on the internally rotated humerus.
The atypical alignment pattern in one client may be The wrist flexes and radially deviates with finger flexion.
different in sitting and standing as a result of the pattern
of loss of control in the leg. In sitting, the hip is in flexion
and provides support, a base, for the upper and lower
trunk. Weakness in the knee and lower leg is not as critical
to sitting balance and function as it is to standing func- Example
tions. In standing, the hip demand is one of neutral exten- A client has a shoulder subluxation resulting in humeral in-
sion to support the trunk; and complex combinations of ternal rotation. This tendency for internal rotation places
knee, ankle, and foot movements are necessary for func- tension on the biceps tendon, resulting in beginning elbow
tional activities. flexion and forearm supination. With unbalanced return and
Shoulder girdle asymmetries are described in the sections weakness of arm muscles, the emerging biceps activity pre-
on shoulder subluxation, and pelvic girdle and leg asymme- dominates and reinforces a posturing pattern of shoulder
tries are described in the sections on standing and walking. internal rotation, elbow flexion with forearm supination
Alignment problems in the distal extremity segments are (Figure 23-12, B).
related to loss of movement control and proximal alignment The weakness pattern of ankle plantarflexion and calca-
changes. Patterns in the distal arm and distal leg are strik- neal equinovarus biases return in the anterior and posterior
ingly similar. When the midjoint, elbow or knee, is extended, tibialis. During movements of the leg in space with hip and
the proximal rotational alignment asymmetry translates knee flexion, this distal supination pattern pulls the tibia into
down the lower segment into the hand or foot. Shoulder in- external rotation. To place the supinated foot on the ground,
ternal rotation translates across an extended elbow, causing the client compensates proximally by rotating the pelvis and
the forearm to pronate and the hand to fall into carpal prona- femur, as a unit, toward the unaffected side. This incompat-
tion (often confused with ulnar deviation) (Figure 23-12, A). ible tibial external rotation and femoral internal rotation re-
Similarly, with knee extension, hip internal rotation asym- sult in knee hyperextension.
metries translate across the knee and cause tibial internal Muscle and tissue tightness is a common result of align-
rotation and midfoot pronation. However, as the midjoints ment problems. Techniques of lengthening muscle and tis-
gain flexion activity, the proximal pattern no longer dictates sue tightness must be balanced with muscle reeducation.
distal asymmetries. The distal weight-bearing pattern or ac- Isolated stretching does not result in a lasting improvement
tive initiation pattern causes a distal rotation that may be in range and may decrease functional ability if not combined
opposite to the proximal rotational pattern. When this occurs, with activities designed to increase control. In hemiplegia,
the midjoint may posture with a result of incompatible intra- slow stretching in functional positions through active weight
limb alignment. shifting (i.e., functional stretching) is more effective than
“orthopedic” stretching because it reeducates the weakness If the client reports joint pain, the therapist should lower
that underlies the loss of muscle length (Figure 23-13). the humerus immediately, reestablish the mobility of the
Because weakness is the underlying cause of loss of scapula, reseat the humerus if necessary, and maintain ap-
alignment and joint range, in the acute phase the joints are propriate humeral rotation while moving the arm up again.
hypermobile. Over time the tissues tighten around some Trunk movements in forearm weight bearing are used to
joints, and therapists often confuse that “feel” with joint teach a self-ranging practice routine that ensures scapulo-
hypomobility. Joint mobilization techniques are rarely humeral rhythm.
needed because the weakness in hemiplegia renders the
joints hypermobile. It is important to avoid excessive mobil- Muscle and Tendon Pain
ity in the intricate joints of the hand and foot. Tightness When a shortened or posturing muscle is stretched too
around a joint may indicate the need for lengthening exer- quickly or beyond available length, a strong “pulling” type
cises, but the joint almost never requires mobilization. of pain is often reported in the region of the muscle belly
being stretched. If the amount of stretch is decreased a few
Pain degrees, the reported pain subsides.
In the client with hemiplegia, arm pain can be caused by an If the inappropriate stretching is not stopped, muscle pain
imbalance of muscles, improper movement patterns, joint progresses to tendon pain. Proximal biceps tendonitis, distal
dysfunction, improper weight-bearing patterns, and muscle biceps tendonitis radiating into the forearm, and wrist flexor
shortening, or it may be related to diminished sensation and tendonitis are most common. The usual cause of tendonitis
sensory interpretation. Although evidence-based approaches is improper weight bearing, with an inactive trunk and
should be used to manage shoulder pain after stroke, sys- “hanging” on the arm with forced elbow extension and
tematic reviews show that there are few rigorous studies that shoulder internal rotation. The treatment of tendonitis is rest
can be used to guide treatment.144 and modalities (i.e., heat, ultrasound, or electrical stimula-
tion) or injection of corticosteroids. When movement reedu-
Joint Pain cation is restarted, it is important to avoid the “exercise” that
Joint pain is caused by poor shoulder joint mechanics during caused the pain and to create a new intervention plan.
movement. Two common alignment problems are loss of
scapular and humeral rhythm and insufficient humeral exter- Complex Regional Pain Syndrome—Shoulder-Hand
nal rotation.108,145 With a shoulder subluxation, the humeral Syndrome
head is not seated in the fossa and passive movements of the One type of complex regional pain occurs in the shoulder
shoulder will not occur with scapulohumeral rhythm. At 60 and hand. It begins with tenderness and swelling of the hand
to 90 degrees of forward flexion, impingement of the cap- and diffuse aching pain from altered sensitivity in the shoul-
sule will occur and the client will report sharp pain on the der and entire arm.146 This pain interferes with the reeduca-
superior aspect of the shoulder joint. The pain ceases when tion of movement patterns and causes a general desire on the
the arm is lowered. The subluxation and loss of scapulo- part of the client to “protect” the arm by not moving it. Lim-
humeral rhythm result from loss of trunk and arm move- ited shoulder, wrist, and finger range of motion soon occurs.
ments or muscle tightness from either persistent arm postur- The second stage includes further loss of shoulder and
ing or weakness. hand range of motion, severe edema, and loss of skin elastic-
ity. This is followed by the third stage, which includes de-
mineralization of bone, severe soft tissue deformity, and
joint contracture.146,147
Not every edematous hemiplegic hand leads to shoulder-
A B hand complex regional pain syndrome. Hand edema results
from an upper extremity that remains dependent and that
does not move for long periods of time. It is essential to
teach the person with hemiplegia how to properly care for
the hand and to give the responsibility for the care of the
hand and arm to the client.
Ryerson and Levit54 propose five steps for intervention for
severe or chronic arm pain: (1) eliminate pain from interven-
tion or the home program, (2) desensitize the arm and hand to
touch, (3) eliminate hand edema, (4) introduce pain-free arm
movements by reestablishing scapular mobility, and (5) begin-
ning with guided arm movements below 60 degrees, gradually
increase the variety and complexity of arm movements.
Figure 23-13 n A, Client with right hemiplegia practicing home Edema
program. During standing forearm weight bearing (providing upper- Edema in the hand and foot is another common secondary
body stability), she initiates a lower-extremity forward-backward impairment that develops as a consequence of loss of move-
movement. As she moves her hips and lower leg forward, she thinks ment control and hospitalization factors such as intravenous
of keeping her knee straight and stretching her calf. B, As she moves infiltrates and limb positioning. Edema limits joint range
her hips backward, she may feel a stretch in the back of her thigh, on and tissue mobility. The edematous fluid places the skin on
the lateral aspect of her trunk, or under her axilla. stretch and acts as an interstitial “glue” that bonds the skin,
fascial tissue, muscle tissue, and tendons. Hand edema is Anterior Subluxation
associated with the development of shoulder-hand syn- Anterior subluxation occurs when the humeral head sepa-
drome. Foot edema is as common as hand edema, limits rates anteriorly from the glenoid fossa. Anterior shoulder
ankle joint dorsiflexion range, and is often ignored during subluxation occurs when the downwardly rotated scapula
intervention programs. Edema begins on the volar surface of elevates and tilts forward on the rib cage and the humerus
the hand and foot, progresses dorsally, and then continues hyperextends with internal rotation. In an anterior sublux-
proximally across the wrist or ankle. ation, as tension increases on the proximal biceps tendon,
Edema interferes with the retraining of functional move- the elbow flexes and the forearm supinates. This subluxation
ment patterns by preventing the smooth glide of tissues. It is found in clients with atypical patterns of return and trunk
must be eliminated before active reeducation begins. rotational asymmetries.54
Edema has defined stages. When the involved tissue feels
soft and fluid, the condition responds to retrograde massage Superior Subluxation
and elevation. When the tissue is gelatinous and pitting, the A superior subluxation occurs when the humeral head
edematous fluid cannot be physically expressed. At this lodges under the coracoid process in a position of internal
stage, it begins to adhere to underlying tissues. The edema rotation and slight abduction. The humeral head is “locked”
must be softened and liquefied through transtissue massage. in this position so that every movement of the humerus is
The last stage of edema is characterized by hard, lumpy tis- accompanied by scapular movement. The scapular position
sue that does not “pit” in response to manual pressure. This in this subluxation is one of abduction, elevation, and neu-
stage of edema requires gentle bilateral compression to tral rotation. The forearm adducts across the body as the
break up the hard, solid areas into regions of softness. The humeral abduction and elbow flexion increase. A superior
soft regions then act as open spaces into which fluid released subluxation occurs in clients with inappropriate muscle fir-
by massage of hard tissue is directed. The goal is to reverse ing and co-contraction.
the process of hardening—from hard, to pitting, to soft and Subluxation is not painful but results in changes in mus-
fluid. In the pitting and hard stages, when the edematous cle length-tension relationships, muscle shortening, and
tissue is not fluid, elevation, elastic gloves, bandaging, and permanent stretch of the joint capsule. If a subluxation
retrograde massage are not effective. When edematous tis- exists, the therapist reduces the subluxation by correcting
sue is soft and fluid, active and active assistive extremity trunk, scapula, and humeral alignment patterns before at-
movement patterns produce muscular contractions that as- tempting to reeducate arm movement patterns. A discussion
sist venous and lymphatic return of the fluid.54 of these subluxations, accompanying trunk movement pat-
terns, and intervention suggestions can be found in therapy
Shoulder Subluxation literature.54 As the client learns to move the arm in patterns
Shoulder subluxation occurs when any of the biomechanical of functional coordination, subluxation and associated arm
factors contributing to glenohumeral joint stability are inter- posturing decrease.
rupted. In persons with hemiplegia, subluxation is related to Prevention of subluxation requires (1) proper assessment
a change in the angle of the glenoid fossa occurring because of secondary alignment problems (rib cage, scapular, hu-
of muscle weakness. In the frontal plane the scapula is nor- meral position), (2) early reeducation of trunk and arm
mally held at an angle of 40 degrees. When the slope of the linked patterns in sitting and standing, and (3) prevention of
glenoid fossa becomes less oblique (and more vertical), the shoulder capsule stretch, including support and positioning
humerus will “slide” down and out of the fossa.148 Ryerson as the client sits, stands, and practices walking.
and Levit149 first described three types of subluxation in
clients with hemiplegia: inferior, anterior, and superior. FUNCTIONAL ACTIVITIES
Functional mobility movement analysis, intervention tech-
Inferior Subluxation niques, unilateral compensatory strategies, and suggestions
The most common type of subluxation is an inferior sublux- for task practice are documented in therapy literature.151-153
ation. It occurs in clients with severe weakness and it is In this section, representative mobility skills are selected in
present in the acute stage. Weakness and the weight of a three functional positions: supine, sitting, and standing. For
heavy arm result in downward rotation of the scapula. each task selected, the focus is on the basic trunk and ex-
Downward rotation orients the glenoid fossa vertically, the tremity control patterns used, significant impairments in
unlocking mechanism of the capsule is lost, and the humerus addition to weakness that make it difficult for the client to
subluxates inferiorly with internal rotation. As the humerus perform the task, and observations from the clinic that relate
internally rotates, the bicipital tuberosity rolls anteriorly; to intervention and practice. Detailed descriptions of each
this anterior prominence is often confused with an anterior trunk pattern and trunk and extremity linked pattern can be
subluxation.54 As subluxation occurs, the shoulder capsule is found in the literature.54
vulnerable to stretch, especially when the humerus is depen-
dent and resting by the side of the body. In this position the Supine
capsule is taut superiorly, so any downward distraction of
the humerus will place an immediate stretch on the upper Rolling
part of the capsule. The coracohumeral ligament reinforces Basic trunk movement patterns for rolling include (1) upper-
the superior portion of the capsule, which is crucial for trunk flexion and rotation initiation, (2) lower-trunk exten-
shoulder stability. Jenson150 has discussed the implications sion and rotation initiation, and (3) symmetrical (log-rolling)
of rupture of this ligament as a result of forced abnormal lateral flexion initiation. These patterns link the trunk with
passive motion as a cause of shoulder pain in subluxation. either the arm or leg during the roll.
Trunk and Extremity Linked Patterns. The upper- The family is educated to understand the nature of the
trunk flexion-rotation initiation pattern links upper-trunk loss of movement and sensation and the effects of these
flexion-rotation with arm reach across the body. Active as- losses on body awareness and early bed mobility. Family
sistive patterns, with the client holding both hands together members are encouraged to sit with, visit, talk to, feed, and
for a bilateral arm reach, are encouraged when strength is touch the person from the client’s affected side. They are
insufficient to lift the arm against gravity or, through thera- instructed in simple movements such as rolling to promote
pist handling, when arm muscle weakness results in such a symmetry, midline control, and activation of trunk and ex-
heavy feeling that the patient cannot control the extremity tremity muscles.
with the unaffected hand. If the therapist assists the arm, the
goal of practice is for the client to initiate the active anti- Feeding and Swallowing
gravity trunk pattern. Although detailed facilitation and inhibition of oral and
A lower-trunk extension-rotation initiation pattern is neck muscle movement for feeding and articulated language
coordinated with either a leg-reach pattern or a flexed-leg are a specialty of speech pathologists, the movement thera-
“push” pattern. Active assistive patterns can be implemented pist activates upper-body control to prepare for more auto-
through therapist handling to help train the sequencing or to matic chew and swallow.
grade the firing patterns of the leg when it is pushing into the Basic trunk patterns to be reeducated include (1) lower-
bed. As the lower body moves from supine toward side body anterior and posterior movement control to move to-
lying, the upper body and arm follow the movement. The ward a table and back into a chair and (2) upper-body ante-
client is encouraged to practice independently with a focus rior and posterior and lateral movement control to provide
on the sequence. During independent practice, the therapist control for head and arm movements.
may provide verbal cues to help the client time the move-
ment of the upper body. Impairments that Interfere
The symmetrical lateral flexion initiation pattern is Oral problems include the following:
known as “log rolling.” In this pattern, the trunk does not n Forward head, poor lip closure, loss of saliva and food
rotate but is active in a coordinated fashion with the arm and n Facial asymmetry during function greater than at rest
leg on the same side; the arm and leg “reach or push” on the n Inability to swallow
leading side. In rolling from supine to side lying, the trunk n Inability to chew
flexors initiate the antigravity movement, and when rolling n Inability to lateralize foods
from side lying to supine, the trunk flexors are the antigrav- n Inability to take liquids from cup or spoon
ity movement initiators. n Muscle weakness
Impairments that Interfere. Shoulder joint pain may Central problems are as follows:
occur when the client rolls onto the affected side. Pain oc- n Poor postural control
curs if the shoulder is trapped under the trunk as the client n Inability to feed self
moves to side lying or when the humeral-scapular alignment Compensations include the following:
causes the shoulder capsule to be impinged. If pain occurs n Use of gravity—head and neck extension
during the roll, the therapist should teach the client to stop, n Chewing on one side only
roll back a few degrees, adjust the position of the arm away n Using the hand to place food in the mouth
from the trunk, and then continue the roll. Therapists should n Using the hand to pull food from the cheek
teach their clients how to avoid shoulder pain during all ac- n Using thicker food than liquids
tivities, especially during rolling or when lying on the more
affected side. Clinical Observations
In rehabilitative or outpatient care, muscle tightness in Excessive drooling occurs with loss of head control and a
the latissimus, quadratus lumborum, biceps, or tensor fas- decreased ability to automatically close the mouth and swal-
ciae latae may limit trunk rotation or trunk and extremity low. If the client tries to lift the head from a flexed position
linked movements. and the cervical spine remains flexed, the head may jut for-
Clinical Observations. Weakness in the extremities is ward into a position of axial extension. As a result of the
a significant factor during rolling because the arm and the biomechanics of the forward head position, the jaw opens,
leg assist the trunk initiation patterns. Rotational patterns automatic swallowing becomes difficult, and saliva runs out
are difficult in the acute stage because they require an in- of the open mouth.
tegration and sequencing of flexor and extensor muscle Drooling from one side of the mouth is annoying and
patterns. Symmetrical rolling may be an easier indepen- embarrassing. The client may not be able to maintain lip
dent pattern to train. Therapists should incorporate active closure and, in addition, may not feel the saliva running out
assistive strategies and extremity strengthening in the or may not identify a need to swallow. Drooling lessens as
early recovery period. Clients have an easier time rolling upper-body control increases.
to the affected side because they use the strength of the In the majority of cases, swallowing problems are tran-
unaffected side to initiate the roll. But they may not want sient in persons with hemiplegia. After the initial insult,
to stay on that side because of shoulder pain, instability of many clients exhibit a decreased gag reflex. In acute care
the hip, or decreased sensation and the fear that ensues. settings, where liquid diets are often routinely given to per-
The client may prefer rolling to the unaffected side be- sons with hemiplegia, education of hospital staff regarding
cause it is easier to rest on, but initiating the movement is the merits of using thicker foods should be considered.
difficult because of loss of control on the affected, leading Thicker, chopped food is easier to swallow than soft food.
side. Soft food is easier to swallow than liquids. Liquids with
distinct taste or texture are easier to swallow than water. Spe- Impairments that Interfere
cific feeding programs are noted in Chapters 9, 11, and 12. Changes in alignment of the arm resulting from weakness
and muscle shortening affect the position of the thoracic
Sitting spine and rib cage. The weight of an extremely weak arm
Function in sitting is based on the ability to maintain the pulls the upper trunk into forward flexion; an increase of
trunk in an upright position, to automatically adjust the flexor tone in the arm influences scapular and rib cage
trunk when the arms or one leg moves around midline, and positions.
to follow movements of the arm and leg as they extend their Shoulder subluxation results in muscle shortening
reach. Control in sitting is also used to help change position, (biceps, pectorals, latissimus, subscapularis), alters the
such as moving from sitting to standing, or lying down. The line of muscle pull, and interferes with scapulohumeral
reestablishment of control in sitting for function is an impor- rhythm. Muscle shortening contributes to loss of upper-
tant early goal in rehabilitation care. body alignment and interferes with reestablishing arm and
Basic trunk movement patterns include the following: trunk control.
1. Anterior, posterior, and lateral upper-body–initiated Loss of trunk alignment as a result of extremity weakness
movements. Upper-body movements are easier to re- and loss of trunk control creates an atypical starting position
train than lower-body movements because the base of for movement and can become an undesirable compensation.
support (contact of the buttocks and thighs) remains
on the surface. Clinical Observations
2. Anterior and posterior lower-body–initiated move- Alignment changes in the arm influence strength and control
ments. With lower-body–initiated movement, the upper of the upper body. Therefore intervention techniques to re-
body needs to be stable yet adjust to and follow the store alignment and control of the arm in relation to the
movement of the lower body. The reeducation of upper- upper trunk must be included in the list of short-term goals
body control allows the therapist to begin retraining to achieve the functional goal of safe, independent task per-
lower-body control. formance in sitting.
3. Lateral lower-body–initiated movements. These move- Active control of the pelvis in a neutral position is neces-
ments are more difficult to reeducated than upper-body sary for the reeducation of lower-body lateral and rotational
movements because as the movement begins, the base weight shifts. Pelvic position influences leg position. If the
of support narrows. pelvis is held in a posterior tilt, the leg initially tends to
4. Rotational movements. In sitting, upper-body rota- abduct; and if it is held in an anterior tilt, the leg initially
tional movements are easier to perform than lower- adducts.
body rotational patterns for the reason noted previ- Clients with poor hip control do not regain functional
ously. trunk patterns while sitting until they can activate and
strengthen hip muscles for stability during weight shifts.
Trunk and Arm Linked Patterns (Representative Weakness of the hip joint results in a desire to shift weight
Examples) to the stronger side, thus creating a spinal or pelvic asym-
Postural adjustments to arm movements around midline re- metry. Clients who push to the affected side in sitting need
quire the trunk to be upright, to be active, and to perform strength from the weak leg for stability as a prerequisite for
small adjustments. When the hand functions in front of the midline control of the trunk.
body, the trunk adjusts with small posterior weight shifts Lower-body–initiated lateral weight shift patterns are dif-
and increased flexor control, whereas as the hand(s) move to ficult to train because they require a narrowing of the base
function behind the body, the trunk adjusts with a small of support. Forearm weight-bearing movement patterns are
anterior weight shift. used to increase the base of support to allow practice of
The trunk moves with an arm to extend reach. If the reach these patterns, which are needed for functional activities
is forward and down to the floor, as if to reach a shoe, the such as scooting, toileting, and lifting one leg off the sur-
upper body initiates an anterior weight shift and the spine face. This movement is difficult to practice without upper-
moves into flexion with control from eccentric contraction body stability (external or internal).
of the spinal extensors. If the reach is forward as if to grab
an object on the far side of a table, the lower body initiates Transfers
an anterior weight shift as the upper body remains stable and Transfers in the half-stand, pivot pattern require upper-
adjusts to the demands of the arm movement. body control over the lower body and combined trunk and
leg control patterns. The squat, pivot position is trained
Trunk and Leg Linked Patterns (Representative when leg strength and control are weak and the goal is to
Examples) train the client to use the affected leg. Transfers involve
Small trunk adjustments occur with leg movements around interim patterns that are trained before safe standing is
midline. If the feet move back under the hips, the trunk ad- possible.
justs with a small amount of anterior weight shift. When one The client practices transfers to different objects (chair,
foot is lifted up to slide into a slipper, the lower body adjusts bed, toilet) to either side. This promotes symmetry, encour-
with a small lateral weight shift. Upper-trunk stability al- ages the use of the affected leg, and allows practice with
lows lower-trunk–initiated patterns when rising to stand. As varying environmental constraints. Transfers to the unaf-
the legs extend and the buttocks lift off the chair, trunk ad- fected side have the advantage of being familiar to hospital
justments accompany the changing leg pattern to control staff because they are the “traditional” textbook way of trans-
trunk position over the legs. ferring the person with hemiplegia. Nevertheless, transfers to
the affected side need to be trained by therapists to allow to move the upper trunk and arm in all planes with appropriate
function in either direction. leg responses and the ability to respond and adjust to weight
transfer to each leg and to provide postural stability for move-
Sitting to Standing ments of each leg in space. Lower-extremity control in stand-
Moving from sitting to standing is an important skill to re- ing has a weight-bearing component and a movement in space
train early after a stroke because it is used many times a day component. As a prerequisite for reeducation of these move-
during functional activities. In a study investigating the rela- ments, the upper body must have enough strength and control
tionship between sitting to standing and walking, Chou and to provide stability and postural adjustments for movements of
colleagues154 found that a critical component of sitting to the leg.
standing was vertical force displacement, the amount of Basic trunk movements to be reeducated include the
weight transferred down into the floor. Those who had a following:
maximal vertical force difference of less than 30% body 1. Upper-body–initiated anterior, posterior, lateral,
weight between both legs displayed faster walking speeds and rotational patterns with critical corresponding
and more typical gait parameters. adjustments in the leg (either hip, knee, or ankle
Two initiation patterns are commonly used, with or with- strategies).
out the use of momentum, to train sitting to standing. A 2. Control of the upper body over the lower trunk during
lower-body–initiated anterior weight transfer occurs with a lower-extremity–initiated weight-bearing movements.
straight spine as the shoulders move forward. Therapists 3. Linked trunk and leg patterns during movements of
should emphasize the forward weight shift component of the leg in space. These are easiest when the leg moves
this pattern rather than the anterior pelvic tilt component; around midline and increase in difficulty as movement
the requirement of sitting to standing is a forward shift of the in space increases in amplitude or speed.
upper body and shoulders. An anterior pelvic tilt usually 4. Increased upper-body control to support power pro-
results in a backward movement of the shoulders. The conduction of the arms for pushing, pulling, or lifting
fusion over this movement occurs because clients often sit in objects and increased lower-body control to support
a position of flexion with a posterior pelvic tilt. To come to power production of the legs for jumping, running,
upright, they must extend the spine and move the pelvis to and stair climbing.
neutral. Although individuals with a tendency for lumbar
extension may have an anterior pelvic tilt as they shift for- Trunk and Arm Linked Patterns
ward, the anterior pelvic tilt is not as important a component Trunk and arm linked patterns include the following:
as is a forward weight shift. 1. Upper-body–initiated flexion movements that occur
An upper-body–initiated anterior weight transfer during with forward and downward arm-reach patterns
sitting to standing requires control in spinal flexion. This 2. Upper-body–initiated extension that occurs when the
pattern keeps body weight over the feet, the new base of arm reaches up or up and back
support, but does not link the extension of the legs with the 3. Upper-body–initiated lateral flexion when the arm
lower trunk. The demand on the trunk from liftoff to stand- reaches down and to one side
ing is greater than in the previous pattern because of the 4. Upper-body flexion and rotation when the arm reaches
need to move the spine from flexion to upright neutral. In the down and to one side.
previous pattern the spine starts and remains in a neutral 5. Upper-body extension and rotation when the arm
position through to standing. The upper-body–initiated pat- reaches up and back to one side
tern is used in rehabilitative and extended care centers be-
cause it allows caregivers to keep weight firmly over the Trunk and Leg Linked Patterns in
feet, thus allowing a safe, maximal-assistance transfer. Weight Bearing
During transfer and sitting-to-standing training, tech- Control of the upper and lower trunk during unilateral
niques of directing manual pressure from the top of the knee stance on either leg is one of the most difficult patterns to
through the tibia into the foot help the client remember to retrain. Control of the trunk in unilateral stance is linked
keep weight on both feet and increase the dorsiflexion with the need for abduction control on the stance leg. In
movement at the ankle. Full standing should not be at- clients with hemiplegia, the complicated control demands
tempted if loss of control in the leg results in nonuse. If the for leg and trunk control in standing combined with the
client cannot activate leg muscles in a weight-bearing posi- presence of weakness and control problems result in loss
tion in attempts to stand, the standing position will be pre- of alignment in multiple joints and undesirable compensa-
carious with undesirable trunk compensatory patterns. tory patterns.
Trunk and Leg Linked Patterns as the Leg

Standing Moves in Space
Standing Control When the leg moves in space in small ranges, the movement
Control in standing is a difficult early goal to achieve because of the upper trunk is small and occurs as a postural adjust-
not only is there a need for trunk and leg coordinated movement. The movement pattern of the femur and pelvis has a
ments, but there is also a prerequisite need for upper-body linked rhythm similar to that of scapulohumeral rhythm: the
(trunk and arm) control over the lower body. Control of basic first 30 to 45 degrees of hip flexion occurs with no pelvic
movement patterns in standing is divided into upper-body– movements; from 45 to 90 degrees the pelvis flexes (poste-
initiated control patterns and lower-extremity–initiated control riorly tilts) with the flexing hip; with continued hip flexion
patterns.134 Upper-body control in standing includes the ability the upper trunk flexes. This pelvic-hip relationship occurs in
the other planes of movement as well and is seen during the Clinical Observations
following functional movements: In the acute phase, therapists can help the client practice
1. Pelvic and lower trunk flexion occurs when the leg standing with the hips and shoulders back against a wall
reaches forward and up; stepping up. to provide support for the trunk and pelvis while creating
2. Pelvic and trunk extension occurs when the leg reaches a safe situation for practicing active self-initiated leg
back. weight-bearing movements. The client can slide down the
3. Pelvic elevation or depression with trunk lateral flex- wall, activating eccentric control in the legs, and then
ion occurs when the leg moves laterally. slide back up, activating concentric control. By using the
wall to assist the stand, the therapist frees his or her hands
Impairments that Interfere to help correct leg alignment problems and lets the client
In standing, loss of alignment in the upper body on the practice the initiation of movement early, independently,
hemiplegic side may result in undesirable compensatory and safely.
patterns that interfere with functional standing movements The client can practice controlled lateral weight transfer
and balance. These patterns include (1) forward flexion of with appropriate trunk activity in this position. Whereas one
the upper trunk, (2) upper-body rotation toward the af- study concluded that there is no relationship between lateral
fected side, and (3) upper-body rotation away from the weight shift and walking, therapists should not conclude that
affected side. unilateral weight acceptance is inappropriate functional
Ankle range may decrease within a few days after stroke training.155 What may be more important than the lateral
and needs to be minimized to allow early standing functions. weight transfer over the leg is learning to depress the foot
Loss of ankle joint dorsiflexion range interferes with the into the floor, as it equalizes weight between the two legs.156
ability of the body to recruit ankle strategies, and limited Upper-extremity forearm or extended-arm weight bear-
ankle joint dorsiflexion range is one cause of knee hyperex- ing provides upper-trunk stability for lower-extremity–
tension in standing. initiated practice. This practice pattern also allows a
Loss of knee control during standing may result from leg means of self-ranging for the ankle, knee, hip, and pelvis.
weakness or loss of intralimb sequencing. Loss of knee con- This position is used not to inhibit tone in the extremities
trol is also influenced by the position and movement control but to activate and strengthen the trunk and legs in linked
of the hip and ankle joints. Initially the knee flexes as more patterns.
weight is shifted to the unaffected side, and the pelvis lists
downward. If the pelvic position is not corrected (leveled) Walking
and the client actively straightens the knee, a compensatory Independent, functional, and safe walking is difficult to re-
pelvic rotation (toward the affected side) may occur. Be- train in the early phases of intervention because it requires
cause of the instability of a weak, flexing leg, the client may refined degrees of trunk and extremity control. It requires an
learn to “lock” the knee in hyperextension as a means of advanced level of trunk control, linked trunk and leg move-
gaining stability (Figure 23-14). ments, and enough strength and control in the leg to support
body weight, to move the multiple joints of the leg in com-
plex patterns, and to control speed, momentum, and balance.
Walking patterns in clients who have experienced stroke are
characterized by slow speed, uneven step and stride lengths,
impaired balance with resulting arm and leg posturing, and
A B reliance on adaptive equipment.
In the current health care environment with the emphasis
on limited therapy visits, therapists are confronted with ma-
jor intervention dilemmas: Should they force the client to
walk without minimal prerequisites? Should they allow un-
desirable compensations although they predict future sec-
ondary problems? Should they use the benefits of the large
health care systems to divide responsibility for continued
gait training among therapy divisions (inpatient, rehabilita-
tion, home care, outpatient)?
Prerequisites for functional, safe walking include the
following:
n Upper-body control to support leg movements in uni-
lateral stance and during swing
n Lower-trunk control to prevent atypical pelvic pat-
terns
n Strength and control of the leg to initiate weight shifts
n Strength and control of the leg to move in space
Figure 23-14 n A, Client with left hemiplegia with knee hyper- Because gait is the most extensively studied, analyzed,
extension wearing a lightweight prefabricated posterior leaf-spring and discussed in terms of intervention, this section describes
brace that does not control his knee hyperextension. B, A solid the prerequisites for walking training and common impair-
ankle brace with foot control that decreases knee hyperextension ments that interfere with walking.157-159 Common impair-
by providing distal stability. ments that interfere with walking are separated into three
divisions of the walking cycle: (1) forward progression, (2)

single- and double-limb support, and (3) swing.160,161
Impairments that interfere with functional walking are
summarized in Box 23-7.
Research on and equipment for partial–body-weight sup-
ported treadmill walking training with or without robotic
assistance have increased over the past 10 years.162-166 In a
2004 review of randomized controlled studies, there was
strong evidence for poststroke treadmill training with or
without body-weight support167 (Figure 23-15). Task speci-
ficity, speed, intensity, and symmetry of practice in this type
of equipment were thought to contribute to improved over-
ground walking performance.162 However, the most recent
Cochrane review reports no statistically significant effect of
treadmill training with or without body-weight support.168 A
n SUMMARY OF SIGNIFICANT
BOX 23-7
FUNCTIONAL IMPAIRMENTS
FORWARD PROGRESSION—HEEL STRIKE TO
MIDSTANCE A B
Poor trunk control Figure 23-15 n A and B, Client with right hemiplegia walking
n Loss of alignment of upper trunk over lower trunk on a treadmill with partial body weight support.
n Loss of control of upper trunk as leg initiates weight
shift forward
Lack of proper initiation pattern and direction
n Excessive forward trunk flexion 2011 randomized controlled trial comparing body-weight–
n Excessive lateral weight shift supported locomotor training with a therapist-supervised
Insufficient ankle joint dorsiflexion range home progressive exercise and balance program reports
n Muscle tightness improvements with both training methods.169
n Loss of control
n Edema

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UMPHRED’S

Darcy A. Umphred, PT, PhD, FAPTA

Gordon U. Burton, PhD, OTR/L

Rolando T. Lazaro, PT, PhD, DPT, GCS

Margaret L. Roller, PT, MS, DPT

UMPHRED’S NEUROLOGICAL REHABILITATION  ISBN: 978-0-323-07586-2

Copyright © 2013 by Mosby, an imprint of Elsevier Inc.

Library of Congress Cataloging-in-Publication Data

Executive Content Strategist: Kathy Falk

Printed in the United States of America

Last digit is the print number: 9 8 7 6 5 4 3 2 1

Foundations for Clinical Practice

CHAPTER 1 Foundations for Clinical Practice*

KEY TERMS OBJECTIVES

P hysical therapists (PTs), occupational therapists (OTs),

Body structures Quality of life and

Phase 1 Risk factors

​n ​ENABLEMENT AND DISABLEMENT MODELS WIDELY ACCEPTED THROUGHOUT THE

ICF model, Health condition Body function and Activity Participation

Conceptual Frameworks for Client/Provider Statistical Model

Figure 1-4 ​n ​Transdisciplinary model for delivery paradigms.

Pre-Disease Disease Activities Participation in Life

Ev L EvL-A LI-A L-M Impairment Activity Disabilities/

Examination Eval/Diagnosis Prognosis Intervention

Figure 1-7 ​n ​Concept of clinical mapping.

Figure 1-9 ​n ​Reverting to more comfortable behavior patterns

BOX 1-1 ​n ​COGNITIVE AREA QUESTIONS FOR CLIENT PROFILE

A. Sensory input: awareness level

BOX 1-2 ​n ​AFFECTIVE AREA QUESTIONS FOR CLIENT PROFILE

A. Level of adjustment or stage of adjustment to the health condition

BOX 1-3 ​n ​SENSORIMOTOR AREA QUESTIONS FOR CLIENT PROFILE

A. Level of motor performance with respect to performance

KEY TERMS OBJECTIVES

I n learning to cope with the often chronic nature of their

included in various wellness models are physical, spiritual, Wellness

A WELLNESS PARADIGM prevention approach typically results in a change in an indi-

Biological & General

Psychological Social and

TABLE 2-3 ​n ​SAMPLE ITEMS FROM PERCEPTUAL MEASUREMENT TOOLS

INSTRUMENT PERCEPTUAL CONSTRUCT SAMPLE ITEMS (RESPONSES)

Perceived Wellness Survey

1. I am always optimistic about my future. 1 2 3 4 5 6

CASE STUDY 2-1 n WELLNESS INTERVENTION CONCEPTS IN NEUROREHABILITATION

CASE STUDY 2-2

In both cases the therapist functions as the movement spe- References

KEY TERMS OBJECTIVES

A s physical and occupational therapists assume greater

all fours, and then upright (cruising and ambulation). Adolph

Motor skills that children display during this period

Figure 3-9 ​n ​Functional development of prehension. A, Early prehension while

erally focused on leisure activities or elite athletic competi-

B Middle Adulthood (40 to 59 Years)

adults who maintain more active lifestyles are more likely

Figure 3-13 ​n ​Three individuals coming to stand using differ-

Figure 3-14 ​n ​Age-appropriate self-select func-

Extensor Control individual in a standing position were looking up at the

Figure 3-21, A through C, shows the same child at 1 week of

UMPHRED’S NEUROLOGICAL REHABILITATION ISBN: 978-0-323-07586-2

n ENABLEMENT AND DISABLEMENT MODELS WIDELY ACCEPTED THROUGHOUT THE

Figure 1-4 n Transdisciplinary model for delivery paradigms.

Figure 1-7 n Concept of clinical mapping.

Figure 1-9 n Reverting to more comfortable behavior patterns

BOX 1-1 n COGNITIVE AREA QUESTIONS FOR CLIENT PROFILE

BOX 1-2 n AFFECTIVE AREA QUESTIONS FOR CLIENT PROFILE

BOX 1-3 n SENSORIMOTOR AREA QUESTIONS FOR CLIENT PROFILE

TABLE 2-3 n SAMPLE ITEMS FROM PERCEPTUAL MEASUREMENT TOOLS

Figure 3-9 n Functional development of prehension. A, Early prehension while

Figure 3-13 n Three individuals coming to stand using differ-

Figure 3-14 n Age-appropriate self-select func-

Figure 3-22 n A 1-month-old child, when supported in a vertical

TABLE 4-1 n THEORIES OF MOTOR CONTROL

n COMPONENTS OF MOTOR CONTROL: BODY SYSTEM PROCESSES INVOLVED IN MOTOR

Figure 4-7 n Concepts important to long-term learning.

Figure 4-8 n Classification and anatomical locations of cortical map.

BOX 4-1 n NEUROPLASTICITY PRINCIPLES TRANSLATED TO GUIDE CLINICAL PRACTICE

BOX 4-1 n NEUROPLASTICITY PRINCIPLES TRANSLATED TO GUIDE CLINICAL PRACTICE—cont’d

BOX 4-1 n NEUROPLASTICITY PRINCIPLES TRANSLATED TO GUIDE CLINICAL PRACTICE—cont’d

CASE STUDY 5-1—cont’d

CASE STUDY 5-1—cont’d

CASE STUDY 5-1—cont’d

True BOX 6-2n QUESTIONS TO HELP GAIN INSIGHT

Figure 7-4 n Self-administered questionnaire to collect medical history information. (Modified

BOX 7-1 n REVIEW OF SYSTEMS CHECKLISTS

TABLE 7-2 INKING PAIN PATTERNS

TABLE 7-6 ESTING PULSE RATE IN BEATS

TABLE 7-8 WELLS” CRITERIA FOR