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Cerebral Palsy

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Cerebral Palsy

1) Developmental motor speech disorders:

(i) Developmental dysarthria / anarthrias: due to congenital or


genetic causes

• A] Cerebral palsy
• B] Others: Eg Muscular dystrophy, Craniostenosis, Congenital
hydrocephalus etc

(ii) Developmental apraxia of speech (DAS)/Childhood Apraxia


of Speech
2) Acquired motor speech disorders:
• Various etiologies such as vascular pathologies, tumours,
trauma etc. which is acquired in nature
• In children, clinical manifestation of insult to nervous system
falls under 6 major categories:

• Neurosensory Eg; Hearing impairment


• Neurointellectual Eg Intellectual disability
• Neurobehavioral Eg; ADHD
• Seizural Eg Any type of epilepsy
• Neuroperceptual Eg LD, APD etc

• Neuromotor Eg: Cerebral palsy (Sensory-


motor involvement) – sensorymotor
dysfunction.
Cerebral palsy/cerebral dysfunction
• First identified/described by John Little who was
an orthopaedic surgeon. He saw that there was
one more condition which resembled polio but
not actually polio and he called it Little’s disease.
• He found that one way to distinguish this
condition from polio and myopathies was its non
progressive nature.
• Term “Cerebral Palsy” first coined by Phelps
(orthopaedic surgeon).
• In 1947, a group of specialists
(Neurosurgeons, neurologists, orthopaedic
specialists, physiotherapists, occupational
therapists, speech therapists) formed the
AACP (American Academy for Cerebral Palsy)
to form structured programs on diagnosis,
treatment, technological development,
perinatal care of cerebral palsied children.
• In 1977- AACP retermed as AACPDM because
they realized that there were other similar
disorders which came under childhood
disability conditions.
• DM-Developmental medicine means a branch
of medicine that deals with developmental
disabilities of children.
Definition of CP
• Cerebral palsy refers to a nonprogressive CNS deficit.
The lesion may be in a single or multiple locations of
the brain, resulting in definite motor and possible
sensory abnormalities. It occurs as a result of inutero
factors, events at the time of labor and delivery
(congenital CP) or a variety of factors in the early
developing years (acquired CP). In addition to motor
deficit, other associated disorders may be found.

-American Academy for Cerebral Palsy

and Developmental Medicine (1977),


The definition identifies two varieties of CP:
• Congenital (85%)
• Acquired (15%)

• Congenital: (birth to 3-6 months, others say strictly


birth to 15 days) conditions in which etiology is traced
to intrauterine, natal and perinatal factors. Very
common cause in CP.

• Acquired: caused in the post natal period (6 months to


2 years) controversial
• CNS infections, intracranial hemorrhage due to vascular
pathologies (primary and secondary), late onset
hydrocephalus or inadequately treated hydrocephalus,
trauma, neoplastic growths in brain, toxicity, infections
such as meningitis, encephalitis etc.
Sometimes anoxia due to following may lead to acquired
variety
• Strangulation
• High altitude
• Deep pressure
• Hypoglycemia (reduced blood sugar)
• Poisoning

• Genetic: some well documented clients of spastic


paraplegia with autosomal dominant trait.
CP is a static motor deficit with definite motor
nature of the problem which distinguishes
this from other disorders such as MR
syndromes, organic brain deficits, autism or
emotional disorders.
Incidence of CP
• ASHA statistics: 1 to 2 in 1000
• M:F = 1.75 or 2:1
• For reasonable estimates suggest an incidence of
approximately 7:1000 live births and a prevalence of
nearly 500 cases:100,000 population.
Causes for Cerebral Palsy???
Etiologies of CP
I. Congenital
A] Intrauterine factors
1) Prematurity: high risk factor (may or may not be at high risk depending
on GA and BW)
• Small for gestational age (SGA- age of the foetus) and birthweight(BW)
• Appropriate for gestational age (AGA) – preterm with normal
birthweight
• In premature children, if BW is appropriate for GA, lesser risk, but if BW
less and not appropriate for GA, then high risk for acquiring
developmental disabilities including CP.
2) Asphyxia/Respiratory distress syndrome
3) Intracranial haemorrhage (especially MCA)
4) Intrauterine malnutrition
5) Intrinsic developmental disability or abnormal
development
6) Disproportionate CNS maturation
7) Maternal prenatal condition: general health, effects of
drugs, alcohol, tobacco, radiation, environmental
pollutants and chemicals, malnutrition of placenta,
maternal infection (Rubella, toxoplasmosis,
cytomegalovirus, herpes, Syphilis).
B] Perinatal factors
1] Neonatal asphyxia: due to
• Obstetrical accidents (breech presentation, precipitated delivery,
dry delivery, pre term, post term, forceps delivery, caesarian
delivery etc)
• Anaesthesia
• Umbilical cord around neck or other mechanical problems
• Intrinsic pulmonary deficits of the infant (common in premature
babies)

• 2] Infection: Eg; sepsis (illness in which the body has a severe


response to bacteria or other germs, symptoms not caused by the
germs themselves. Instead, chemicals the body releases cause the
response), meningitis.

• 3] Intracranial haemorrhage during delivery


4] Hyperbilirubinemia (increased levels of bilirubin in
the blood) : RH incompatibility

5] Blood group incompatibility: ABO groups

6] Fixed motor lesions due to:


CNS insult: hydrocephaly, anencephaly (absence of
skull or large part of brain), encephalocele (bones of
the skull do not close completely, creating a gap
through which cerebral spinal fluid, brain tissue and
the membrane that covers the brain (the meninges)
can protrude into a sac-like formation) etc
• Other causes such as neurofibromatosis (nerve
tissue grows tumors), syndromes
Classification of Cerebral Palsy??
Classification of CP

• 1] Physiologic (Motor): A] By Denhoff


1. Spasticity 5. Ataxia

2. Athetosis 6. Atonia

a) Tension 7. Mixed

b) Nontension 8. Unclassified

c) Dystonia

d) Tremor

4. Rigidity
B] By Hardy
(i) Disorders of muscle tone: extent of tension maintained in muscle group
Hypotonia → Atonia, Flaccidity
Hypertonia →Spasticity, Rigidity and Tension

(ii) Disorders of movement (kinesia)


a) Hyperkinesia (UMN lesion and excessive involuntary movement)
I Dyskinesia: Athetosis (Slow variety), Chorea (Fast variety),
Choreoathetosis

II General variety
Dystonia
Tremors
Myoclonus and spasmodic disorders
Ballismus
b) Hypokinesia: LMN lesion (reduced movements, rare in
CP)

(iii) Disorders of coordination: Ataxia

2]Topographic [involvement of limbs of the body]

• Monoplegia (symptoms are present only in one limb)

• Hemiplegia (one side of the body is affected – usually


spastic and occasionally athetoid)

• Paraplegia (involvement of legs only - spastic and rigid


types usually)
•Triplegia (involvement of three extremities, more often
both legs and one arm (usually spastic))
•Quadriplegia / Tetraplegia (involvement of all four limbs
where legs are most involved (usually spastic with greatest
involvement in arms, usually dyskinetic including athetoid)
•Diplegia (involvement of like parts on either side of the
body i.e. bilateral paralysis; both hands or both legs, arms
are affected to a lesser degree than legs)
•Double hemiplegia (arms are more involved than legs.
Usually spastic in type)
3] Etiological: Genetic, Prenatal, Natal and Post Natal
4] Neuroanatomical: Cortical, subcortical, UMN, LMN, central, peripheral
etc.
5] Supplementary
(i) Psychological status: cognition, degree of MR etc
(ii) Physical status: (assessed by a paediatrician)
• Physical growth evaluation- height, weight etc.
• Developmental level- in different domains like sensory, sp-lang
• Bone age (By biopsy)
• Contractures (permanent fixed postures in body when
spasticity /rigidity is not reduced through physiotherapy)
(iii) Convulsions/seizures
• Grandmal, petitmal, focal etc
(iv) Posture and locomotive behavior pattern
(v) Eye hand behavior pattern
Eye movements
Eye posture
Fixation (eye gaze)
Convergence (a problem in squint eyes)
Prehensory approach (simultaneous eye hand coordination exhibited
on both sides)
Grasp
Manipulation
Hand dominance
(vi) Visual status
a) Sensory: amblyopia (astigmatism-a type of squint where the image
formation of retina is affected), field defects, myopia.
b) Motor:
Conjugate deviations
•Fixation defects (sudden spasm)
•Spasmus fixus (fixation deficit due to sudden spasm in
extra and intraocular muscles)
•Strabismus fixus (fixation deficit due to squint)
Esotropia (type of squint when eyeballs deviate towards
midline)
Exotropia (deviate away from midline)
Hypertropia (deviate towards head)
Hypotropia (deviate towards mouth)
• Nystagmus (involuntary movements of eyeball)
• Pseudopalsy of external eye muscles (appears as
palsy of eyeballs, severe spasticity in and around
eyeballs, but not eyeballs)

• (vii) Auditory disorders (e.g. in athetoid due to


kernicterus, the could be high frequency sloping
SNHL)

• (viii) Speech / language and communication


disorders
6] Functional capacity
•Class I: no practical limitation of capacity
•Class II: Mild to moderate limitation of capacity
•Class III: Moderate to severe limitation of capacity
•Class IV: Non ambulatory

7] Therapeutic classification
•Class A: Not requiring treatment
•Class B: Requires minimal therapy
•Class C: Requires team approach
•Class D: Requires long term institutionalization and treatment
Motor Development: Typically
developing Vs Cerebral palsy
Motor development in typically developing children and in children
with cerebral palsy
• Typically developing children
• Based on neural commands from motor areas,
the structures of the body act-i.e. muscles,
tendons and joints
• Various degrees of (a) flexion and extension (b)
cocontraction (c) force physiology possible
(precision, range, speed, force)
Definition of
A] Milestones-motor, sensory, social and adaptive, speech and
language, visual, auditory etc.
B] Critical period (optimum age) : A period during which acquisition
of skills or behaviors are at their optimum. Generally identified as
0-5 years.
C] Sensitive period (periods when a child may learn particular skills
more easily than others) E.g: One word utterance by 1 year

Motor milestones
1] Gross motor development
2] Fine motor development
How does the gross motor and fine
motor development occur??
Neural correlates of milestone acquisition: maturation of
nervous system
1] Synaptic connections
2] Myelination (increase in thickness for faster
conduction)
3] Neurotransmitters (quantity and function change for
faster conduction)
4] Cell morphology (increase in cell size and morphology)
5] Dendritic arborization
Motor development (control) in normal children continued….

•The direction of motor development is always proximodistal (arms


first control-> fingers) and cephalocaudal (head control first->arms-
>legs)
E.g: Initially arms are flexed/adductedabducted/extension
(proximodistal)
•Initially only reflexive motor activities/behaviour (primitive reflexes)
which is later modified into voluntary activities (becoz cortex is not
mature in new borns- movements controlled by lower centres. As
maturation occurs, function taken over by brainstem, midbrain and
then cortex by one to one and a half years)
•REFLEXIVE ACTIVTIES: Brainstem & Spinal cord
•Voluntary activities: Cortex
Jackson’s concept
•Principle of Evolution of nervous system
•Principle of Dissolution of the nervous system

•Dr. Jackson regarded the CNS as an hierarchy in which


each grade controls the grade below and is controlled by
grade above

•Each centre in the lowest grade represents a limited


portion of the organism and each centre in the highest
grade represents the whole of the organism
• Principle of evolution: Because the higher structures
are immature, the lower structures are responsible for
the control of motor actions and as and when the
higher structures mature the function of the lower
centers are controlled by the higher centers.

• Principle of dissolution: When an individual incurs


damage to the cortical structures, the cortical
structure loses its control over the lower structures
and the function of the cortical structures are taken
over by the lower structures.
• Duplex’s symptomatology- explained the same concept
as symptoms

• When disease affects the nervous system, there are two


sets of manifestations, negative and positive: loss of
function and excess of function

• Positive symptoms→ release of functions (excess) of


lower order nervous system (E.g: Primitive reflexes)
• Negative symptoms → loss of function of higher order
nervous system (E.g: Loss of postural reflexes)
Motor Development in children with
Cerebral Palsy
Because of the lesion and topography of disorder affects
the motor movement to various degrees
• difficulty to initiate movement
• total arrest of movements
• stereotyped movements
• involuntary movements
• inco-ordinated movements
• Abnormal posture patterns
• Contractures
• Motor milestones are
a) Delayed and
b)Deviant: Inappropriate acquisition of milestones
• Delay and Deviance α Severity of the disorder.
• Delay and Deviance is seen in both
a) Gross motor E.g: Sitting behavior
b) Fine motor E.g: grasping
• Delayed and deviance: One of the High risk
factors.

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