Essential Neurology 4Th Ed PDF

Essential
Neurology
Dedication
To both Biddies with love
FOURTH EDITION
Essential
Neurology
Iain Wilkinson
BSc, MD, MA, FRCP
Formerly Consultant Neurologist
Addenbrookes Hospital
Cambridge
and
Fellow of Wolfson College
and Associate Lecturer
University of Cambridge Medical School
Graham Lennox
BA FRCP
Consultant Neurologist
Addenbrookes Hospital
Cambridge
and
West Suffolk Hospital
Bury St Edmunds
2005 by Blackwell Publishing Ltd

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permission of the publisher.
First published 1988
Reprinted 1989, 1992
Four Dragons edition 1989
Reprinted 1992
Second edition 1993
Reprinted 1994, 1995, 1996
Four Dragons edition 1993

Reprinted 1994, 1997, 1998
Third edition 1999
Reprinted 2000, 2001, 2002, 2004
Fourth edition 2005
Library of Congress Cataloging-in-Publication Data

Wilkinson, I.M.S.
Essential neurology / Iain Wilkinson, Graham Lennox.4th ed.
p. ; cm.
Includes bibliographical references and index.
ISBN-13: 978-1-4051-1867-5
ISBN-10: 1-4051-1867-9
1. Neurology. 2. Nervous systemDiseases.
[DNLM: 1. Nervous System Diseases. WL 140 W686e 2005]
I. Lennox, Graham. II. Title.
RC346.W55 2005
616.8dc22
2004019636
ISBN-13: 978-1-4051-1867-5
ISBN-10: 1-4051-1867-9
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Contents
Preface to the fourth edition, vii

Preface to the first edition, viii
Acknowledgements, ix
Abbreviations, x
1 Clinical skills, physical signs and anatomy, 1
2 Stroke, 25
3 Brain tumour, 40
4 Head injury, 55
5 Parkinsonism, involuntary movements and ataxia, 67
6 Paraplegia, 83
7 Multiple sclerosis, 99
8 Cranial nerve disorders, 111
9 Nerve root, nerve plexus and peripheral nerve lesions, 137
10 Motor neurone disease, peripheral neuropathy, myasthenia
gravis and muscle disease, 155

11 Unconsciousness, 175
12 Epilepsy, 192
13 Headache and facial pain, 213
14 Dementia, 224
15 Infections of the nervous system, 238
Answers to case histories, 255

Index, 269
v
Preface to
the fourth edition
The three previous editions of this book were written by only

one of us. The addition of a younger and enthusiastic second author has undoubtedly improved the book. It is more accurate
and thoroughly modern. Both authors have been singleminded in their intention to present, clearly and concisely, that
neurology and neurosurgery which is required by medical
students at the time of graduation.
The revision of diagrams, use of colour, clarification of text,
and inclusion of illustrative case histories have all helped, but it
is the authors discipline to adhere closely to the title of the book,
Essential Neurology, which makes them hope that the textbook
remains helpful to its readers.
I.W. and G.L.
vii
Preface to
the first edition
Excellent textbooks of neurology already exist, which deal with

the subject in a detailed and comprehensive manner. This is not
what the majority of clinical medical students require.
In writing this textbook, I have been preoccupied with the
following questions:
Have I kept to basic principles?
Have I made each topic as easy as possible to understand,
both in the text and in the diagrams?
Have I omitted all unnecessary detail?
There is no section in the book specifically dedicated to How
to examine the nervous system. I believe each student has to
learn this by apprenticeship to clinical neurologists in the ward
and in the clinic.
Every effort has been made to ensure that this book lives up to
its name, in setting out clearly all that the student needs to know
about the common neurological and neurosurgical conditions.
I.W.
viii
Acknowledgements
We both want to acknowledge the very great help of Drs N.

Antoun and J. Gillard in preparation of the neuroradiological
images.
ix
Abbreviations
BG
C
CNS
CSF
CT
ECG
EEG
EMG
LMN
MR
NMJ
S
UMN
basal ganglia
cerebellum
central nervous system
cerebrospinal fluid
computerized tomography
electocardiogram
electroencephalogram
electromyogram
lower motor neurone
magnetic resonance
neuromuscular junction
sensation
upper motor neurone
CHAPTER 1
Clinical skills, physical

signs and anatomy
Theres something wrong with my left leg, and Im not walking

properly. In this chapter we are going to use this clinical
problem to remind us:
how important the details of the history are in making a
neurological diagnosis, especially the clear definition of how
the problem has evolved with respect to time;
of the components of the nervous system involved in normal
movement, their anatomy (which isnt complicated), and the
physical signs produced by lesions in the various components;
of the common patterns of neurological malfunction, affecting any part of the body, such as a leg;
of the importance of the patients response to his malfunctioning limb, in defining the size and nature of the whole
illness;
of the reliability of the clinical method in leading us to a
diagnosis or differential diagnosis and management plan;
how important it is to explain things clearly, in language the
patient and relatives can understand.
At the end of the chapter are a few brief case histories, given to
illustrate the principles which are outlined above and itemized
in detail throughout the chapter.
Our response to a patient telling us that his left leg isnt working properly must not just consist of the methodical asking of
many questions, and the ritualistic performance of a complex,
totally inclusive, neurological examination, hoping that the
diagnosis will automatically fall out at the end. Nor must our response be a cursory questioning and non-focused examination,
followed by the performance of a large battery of highly sophisticated imaging and neurophysiological tests, hoping that they
will pinpoint the problem and trigger a management plan.
No, our response should be to listen and think, question and
think, examine and think, all the time trying to match what
the patient is telling us, and the physical signs that we are eliciting, with the common patterns of neurological malfunction
described in this chapter.
1
CHAPTER 1
History
We need all the details about the left leg, all the ways it is
different from normal. If the patient mentions wasting, our
ideas will possibly start to concentrate on lower motor neurone
trouble. We will learn that if he says its stiff, our thoughts will
move to the possibility of an upper motor neurone or extrapyramidal lesion. If he cant feel the temperature of the bath water
properly with the other (right) leg, it is clear that we should
start to think of spinal cord disease. If it makes his walking
unsteady; we may consider a cerebellar problem. Different
adjectives about the leg have definite diagnostic significance.
We will ask the patient to use as many adjectives as he can to
describe the problem.
Details of associated symptoms or illnesses need clarification. Theres nothing wrong with the other leg, but my left hand
isnt quite normal one starts to wonder about a hemiparesis.
My left hands OK, but theres a bit of similar trouble in the
right leg we might consider a lesion in the spinal cord. I had a
four-week episode of loss of vision in my right eye, a couple
of years ago we might wonder about multiple sclerosis.
Mind you, Ive had pills for blood pressure for many years
we might entertain the possibility of cerebrovascular disease.
These and other associated features are important in generating
diagnostic ideas.
Very important indeed in neurological diagnosis is detail
of the mode of onset of the patients symptoms. How has the
left leg problem evolved in terms of time? Lets say the left
leg is not working properly because of a lesion in the right
cerebral hemisphere. There is significant weakness in the left
Years
Benign tumour
Degenerative condition
M/W
Months/weeks
Malignant tumour
Chronic inflammation
2
Weeks/days
Demyelination in CNS
Acute inflammation
Days/hours
Very acute inflammation
Instantaneous
Vascular
Trauma
1
Years
Severity of symptoms
W/D
D/H
I
1
0
Weeks
Duration of symptoms before presentation
2
Fig. 1.1 The patients history

indicates the probable pathology.
CLINICAL SKILLS
leg, slight weakness in the left hand and arm, some loss of
sensation in the left leg and no problem with the visual fields.
This same neurological deficit will be present whatever the
nature of the pathology at this site. If this part of the brain isnt
working there is an inevitability about the nature of the
neurological deficit.
It is the history of the mode of evolution of the neurological
deficit which indicates the nature of the pathology (Fig. 1.1).
Components of the nervous system required for

normal function; their anatomy; physical signs
indicating the presence of a lesion in each
component; and the common patterns in which
things go wrong
The basic components of the nervous system required for
normal movement are shown on the simple diagram below.
Upper motor neurone
Lower motor neurone
Muscle
Basal ganglia
Cerebellum Sensation
Neuromuscular junction
Lesions along the primary motor pathway, UMNLMN

NMJM, are characterized by weakness or paralysis. We will
see that the characteristics of the weakness are different in each
instance; for example, UMN weakness has different characteristics from LMN weakness. Knowledge of these characteristics
is fundamental to clinical neurology.
Normal basal ganglia, cerebellar and sensory function is essential background activity of the nervous system for normal
movement. Lesions in these parts of the nervous system do not
produce weakness or paralysis, but make movement imperfect
because of stiffness, slowness, involuntary movement, clumsiness or lack of adequate feeling.
So we will be questioning and examining for weakness, wasting, stiffness, flaccidity, slowness, clumsiness and loss of feeling
in our patients left leg. This will help to identify in which component of the nervous system the fault lies.
To make more sense of the patients clinical problem, we have to
know the basic anatomy of the neurological components, identified above, in general terms but not in every minute detail.
This is shown on the next three pages.
CHAPTER 1
An upper motor neurone involved in left leg

movement
Cell body in motor cortex of right cerebral
hemisphere
Axon:
descends through right internal capsule
crosses from right to left in the medulla
travels down the spinal cord on the left
side in lateral column
synapses with a lower motor neurone
innervating left leg musculature
A lower motor neurone involved in left leg

movement
Cell body at the lower end of the spinal cord
on the left side
Axon:
leaves the spine within a numbered
spinal nerve
travels through the lumbosacral plexus
descends within a named peripheral nerve
synapses with muscle at neuromuscular
junction
CLINICAL SKILLS
Basal ganglion control of the left leg

The structures involved in extrapyramidal
control of the left side of the body
reside in the basal ganglia and cerebral
peduncle on the right
Basal ganglion function is contralateral
Cerebellar control of the left leg

The left cerebellar hemisphere has two-way
connection with the right cerebral
hemisphere and the left side of the body,
via the cerebellar peduncles, brainstem
and spinal cord
Cerebellar function is ipsilateral
CHAPTER 1
Pain and temperature sensation in the left leg

Third sensory neurone:
cell body in thalamus
axon travels to sensory cortex
Second sensory neurone:
cell body in lumbar spinal cord on the
left
axon crosses to the right and ascends
to thalamus in lateral column of spinal
cord
Dorsal root ganglion cell:
distal axon from the left leg, via
peripheral nerve, lumbosacral plexus
and spinal nerve
proximal axon enters cord via dorsal
root of spinal nerve, and relays with
second sensory neurone
Position sense in the left leg

Third sensory neurone:
cell body in thalamus
axon travels to sensory cortex
Second sensory neurone:
cell body in gracile or cuneate nucleus
on left side of medulla, axon crosses
to the right side of medulla and
ascends to thalamus on the right
Dorsal root ganglion cell:
distal axon from left leg, via peripheral
nerve, lumbosacral plexus and spinal
nerve
proximal axon enters cord, ascends in
posterior column on the left to reach
the second sensory neurone in left
lower medulla
CLINICAL SKILLS
The final piece of anatomical knowledge which is helpful

for understanding the neurological control of the left leg, is a
little more detail about motor and sensory representation in
the brain. The important features to remember here are:
the motor cortex is in front of the central sulcus, and the
sensory cortex is behind it;
the body is represented upside-down in both the motor and
sensory cortex;
the axons of the upper motor neurones in the precentral
motor cortex funnel down to descend in the anterior part of
the internal capsule;
the axons of the 3rd sensory neurone in the thalamus radiate
out through the posterior part of the internal capsule to reach
the postcentral sensory cortex.
Leg
Leg
Postcentral
sensory
cortex
Arm
Face
Arm
Precentral
motor
cortex
Thalamus
Internal capsule
Face
Basal ganglion
Having reviewed the components of the nervous system

involved in normal function of the left leg, and their basic
anatomy, we now need more detail of:
the clinical features of failure in each component;
the common patterns of failure which are met in clinical
practice.
The next section of this chapter reviews these features and
patterns.
CHAPTER 1
Upper motor neurone
Upper motor neurone
Lower motor neurone
X
Muscle
Basal ganglia
Characteristic of upper motor neurone lesions:

no wasting;
increased tone of clasp-knife type;
weakness most evident in anti-gravity muscles;
increased reflexes and clonus;
extensor plantar responses.
X
Contralateral monoparesis
A lesion situated peripherally in
the cerebral hemisphere, i.e.
involving part of the motor
homunculus only, produces
weakness of part of the
contralateral side of the body, e.g.
the contralateral leg. If the lesion
also involves the adjacent sensory
homunculus in the postcentral
gyrus, there may be some sensory
loss in the same part of the body.
Contralateral hemiparesis
Lesions situated deep in the
cerebral hemisphere, in the region
of the internal capsule, are much
more likely to produce weakness
of the whole of the contralateral
side of the body, face, arm and leg.
Because of the funnelling of fibre
pathways in the region of the
internal capsule, such lesions
commonly produce significant
contralateral sensory loss
(hemianaesthesia) and visual loss
(homonymous hemianopia), in
addition to the hemiparesis.
CLINICAL SKILLS
Ipsilateral monoparesis
A unilateral lesion in the spinal
cord below the level of the neck
produces upper motor neurone
weakness in one leg. There may
be posterior column (position
sense) sensory loss in the same
leg, and spinothalamic (pain and
temperature) sensory loss in the
contralateral leg. This is known as
dissociated sensory loss, and the
whole picture is sometimes
referred to as the Brown-Squard
syndrome.
Ipsilateral hemiparesis
A unilateral high cervical cord
lesion will produce a hemiparesis
similar to that which is caused
by a contralateral cerebral
hemisphere lesion, except that the
face cannot be involved in the
hemiparesis, vision will be
normal, and the same dissociation
of sensory loss (referred to above)
may be found below the level of
the lesion.
10
CHAPTER 1
A spinal cord lesion more usually causes upper motor

neurone signs in both legs, often asymmetrically since the
pathology rarely affects both sides of the spinal cord equally.
Paraparesis, if the lesion is at or

below the cervical portion of the
spinal cord.
Tetraparesis or quadriparesis, if
the lesion is in the upper cervical
cord or brainstem.
Lesions anywhere between the midbrain and lower spinal cord

may, in addition, involve ascending sensory pathways and fibre
tracts involving sphincter function. There may therefore be
sensory loss below the level of the lesion, and the possibility of
bladder, bowel and sexual symptoms.
There may be physical signs which indicate the level of the
lesion very accurately:
LMN signs, loss of reflexes, dermatome pain or sensory loss,
at the level of the lesion in the spinal cord;
cerebellar signs or cranial nerve palsies when the lesion is in
the midbrain, pons or medulla.
CLINICAL SKILLS
11
Lower motor neurone
Upper motor neurone
Lower motor neurone
X
Muscle
Basal ganglia
Characteristics of lower motor neurone lesions:

wasting;
fasciculation;
decreased tone (i.e. flaccidity);
weakness;
decreased or absent reflexes;
flexor or absent plantar responses.
X
X
X
Generalized LMN weakness may
result from pathology affecting
the LMNs throughout the spinal
cord and brainstem, as in motor
neurone disease or poliomyelitis.
Generalized limb weakness
(proximal and distal), trunk and
bulbar weakness characterize this
sort of LMN disorder.
Generalized LMN weakness may

also result from widespread
damage to the axons of the LMNs.
This is the nature of peripheral
neuropathy (also called
polyneuropathy). The axons of
the dorsal root sensory neurones
are usually simultaneously
involved. The LMN weakness
and sensory loss tend to be most
marked distally in the limbs.
X
X
X
X X X X
XXX
X
XX
X X
X X
X
X
X
X
12
CHAPTER 1
LMN weakness may be confined

to the distribution of one spinal
root (above) or one individual
peripheral nerve (below). In such
circumstances, the LMN signs
are found only in the muscles
supplied by the particular nerve
root or peripheral nerve in
question. Almost always there is
sensory impairment in the area
supplied by the nerve or nerve
root. Examples of such lesions are
an S1 nerve root syndrome caused
by a prolapsed intervertebral disc,
or a common peroneal nerve
palsy caused by pressure in the
region of the neck of the fibula.
CLINICAL SKILLS
13
Upper motor neurone
Lower motor neurone
X
Muscle
Basal ganglia
The classic disease of the neuromuscular junction is myasthenia

gravis. Characteristics of myasthenia gravis:
uncommon;
no wasting;
tone normal;
weakness;
fatiguability;
reflexes normal;
positive response to anticholinesterase.
The pattern of muscle involvement in this rare disease:
ocular muscles common:
ptosis;
diplopia;
bulbar muscles fairly common:
dysarthria;
dysphagia;
trunk and limb muscles less common:
limb weakness;
trunk weakness;
breathing problems.
More common paralysis due to neuromuscular blockade is
that which is produced by anaesthetists during operative
surgery.
Myasthenia gravis would not be a likely diagnosis in a patient
presenting with left leg malfunction.
14
CHAPTER 1
Muscle
Upper motor neurone
Lower motor neurone
X
Muscle
Basal ganglia
Characteristics of primary muscle disease:

uncommon;
wasting;
no fasciculation;
weakness;
tone normal or reduced;
reflexes normal or reduced.
Proximal muscle weakness typifies most primary muscle
disease. The patient has difficulty in lifting his arms above
shoulder level, and in rising from a chair into the standing
position. He needs to use his arms to help him do this. In the
standing position, the trunk muscle weakness often allows an
abnormal amount of extension of the lumbar spine, so that the
abdomen protrudes forwards. When walking, the abdominal
and pelvic muscle weakness allow downward tilting of the
pelvis when the leg is off the ground. This is known as
Trendelenburg weakness.
A diagnosis of muscle disease would be unlikely in a patient
presenting with unilateral leg malfunction. This is partly
because muscle disease is rare, and partly because it usually
produces bilateral symmetrical weakness.
CLINICAL SKILLS
15
Basal ganglia
Upper motor neurone
Lower motor neurone
Muscle
X
Basal ganglia
Two main syndromes, each with different characteristics:

1. Parkinsons disease:
common;
tremor at rest;
increased tone;
bradykinesia;
flexed posture.
2. Involuntary movements:
uncommon;
involuntary movements at rest and during action;
tone increased, normal or reduced;
normal speed of movement;
all sorts of postural abnormalities.
No weakness in either.
These syndromes may be unilateral and are commonly
asymmetrical, the pathology being in the basal ganglia of the
contralateral cerebral hemisphere.
It is not at all improbable that a patient complaining of left leg
malfunction, and difficulty in walking, might be presenting
with Parkinsons disease.
16
CHAPTER 1
Cerebellum
Upper motor neurone
Lower motor neurone
Muscle
X
Basal ganglia
Characteristics of cerebellar lesions are:

1. Incoordination of muscle activity:
in the head: nystagmus, dysarthria;
in the arms: fingernose ataxia, kinetic tremor, difficulty
with rapid alternating movements (dysdiadochokinesia);
in the legs: heelkneeshin ataxia, gait ataxia, falls.
2. There is no weakness. (Alcohol in large doses impairs
cerebellar function. Intoxicated people show all the features of
muscular incoordination mentioned above, but may be very
strong.)
3. In a unilateral cerebellar lesion, the neurological deficit is
ipsilateral to the side of the lesion. A patient complaining of
malfunction of the left leg due to a left cerebellar lesion would
have heelkneeshin ataxia most marked in the left leg, and gait
ataxia with deviation to the left. There might also be left
arm cerebellar signs, and nystagmus most marked looking to
the left.
CLINICAL SKILLS
17
Sensation
Upper motor neurone
Lower motor neurone
Muscle
X
Basal ganglia
Characteristics of movement in the presence of sensory loss:

ataxia or clumsiness of movement due to loss of sense of position mainly, but also due to loss of touch sensation;
partial compensation by active monitoring of movement by
the eyes;
no weakness.
There are three main clinical syndromes where sensory loss
may play an important role in impairing movement and
function.
Cerebral hemisphere lesions:

impaired accurate movements of
the contralateral limbs because
central registration of limb
position is lost.
18
CHAPTER 1
XXX
X XX
XX
X X
X X
X
X
X
X
Loss of proprioceptive sense in

the legs and feet may occur as a
result of either spinal cord disease
(above) or peripheral neuropathy
(below). The loss of sense of
position gives rise to clumsiness
of leg movement when walking,
unsteadiness, and the need to
watch the feet and floor carefully.
There is marked unsteadiness
and falling when vision cannot
compensate, e.g. in the dark, in
the shower, when washing the
face, when putting clothes over
the head. Rombergs sign (stance
steady with eyes open, but
unsteady with eyes closed) is
positive in such patients.
CLINICAL SKILLS
19
The patients response to his symptoms

Hopefully the nature of the patients physical illness causing
the left leg malfunction will emerge from the history and
examination, carried out against this background knowledge of
common patterns of neurological failure. Just as important, and
to be conducted during the same history and examination, is
an evaluation of the patients response to the illness. How
much is the imperfect function in the left leg bothering him?
What are the practical consequences of having the problem in
his everyday life? What does he think is the matter? Has he
worried about a really serious cause? Is he anticipating recovery
or further disability?
In this section we recognize that the total illness in any patient
is the sum of the physical illness plus the patients psychological
reaction to the physical illness. The latter may be appropriate
and entirely understandable. Sometimes, however, the reaction
is exaggerated for some reason, making the whole illness a
bigger one for the patient, his family and the medical staff
looking after him. Recognition of the two elements of illness,
and the management of both, are particularly appropriate in
patients with neurological disorders.
Patient's total
illness
Patient's
psychological
reaction
Physical
illness
A physical illness with an

appropriate reaction
The same physical illness
with an exaggerated
reaction, producing a
bigger total illness
Total
illness
Physical
illness
Patient's
reaction
Both elements need to be:
Diagnosed
Recognized
Investigated
Understood
Treated
Discussed
20
Diagnosis, explanation and planning

A diagnosis, or differential diagnosis, for the patients left leg
problem is established, and a good feel for the level of patient
concern has been achieved. Tests to confirm the diagnosis often
need to be arranged before the diagnosis is finally established.
Careful explanation of the differential diagnosis, and careful
explanation of the tests to the patient, often with another
family member present, is important. Diagnostic certainty may
be achieved nowadays as a result of sophisticated scanning,
neurophysiological tests and laboratory investigations, but
some patients are apprehensive about such investigations and
others are very apprehensive about what diagnosis may emerge
as a result of them. The need for excellent communication and
patient explanation reaches its height when the final diagnosis
and management plan are discussed with the patient (and
family member). Plenty of opportunity should be given for the
patient and family to express their feelings at this stage.
The following five points are helpful from the communication point of view. The doctor should show in an open and
friendly way, that:
1. there is always enough time;
2. there is always enough concern;
3. enough privacy is always available for the patient to speak
freely and openly;
4. there is always an opportunity to talk to the patients family;
5. he can talk to the patient and family in language they can
easily understand.
Investment of such time and effort with a patient who has a
neurological illness is always worthwhile. The more the patient
and family trust, like and respect the doctor, the greater will be
their confidence in the diagnosis and their compliance with the
management. It is clearly a shame if a bright doctor has established the correct explanation of our patients left leg problem,
but communicates very poorly and establishes little rapport
with the patient. The patient may be far from satisfied, and seek
help elsewhere.
Patience with patients

Time
Concern
Privacy
Relatives
Language
CHAPTER 1
CLINICAL SKILLS
21
Some typical case histories

Lets create a few different neurological scenarios which might
develop from a patient presenting with a malfunctioning left
leg, to show the range of different outcomes.
A semi-retired builder of 68
years, smoker, has noticed
gradually progressive
weakness in the left leg for
68 weeks. Both he and his
wife are worried, mainly
because they have an imminent 4-week trip to visit
their son and family in Australia.
General examination is normal.
Neurological examination reveals mild UMN signs
in the left arm and major UMN signs in the left leg.
A chest X-ray shows a mass at the right hilum and a
CT brain scan shows two mass lesions, one
(apparently producing no problem) in the left frontal
region, and one in the region of the precentral motor
cortex high up in the right fronto-parietal region.

Bronchoscopy confirms that the right hilar lesion is a
bronchial carcinoma.
In discussion, it transpires that the patient had a
strong notion that this was what was wrong from
fairly early on, confirmed for him when he was asked
to have a chest X-ray; that he would like to take
advantage of the transient improvement produced by
large-dose steroids, reducing the oedema around the
brain lesions; and that, although not rich, he could
afford hospital care or urgent flights home from
Australia if required.They would continue with their
planned visit, and cope as best they could when the
inevitable worsening of his condition occurred,
hopefully when they have returned home.
A widow of 63 years, who has been on

treatment for high blood pressure for
some years, developed sudden
weakness of the left leg whilst washing
up at 9 a.m. She fell over and had to call
the doctor by shuffling across the floor
of the house to the telephone. Now, 3
days later, there has been moderate recovery so that
she can walk but she feels far from safe.
Her father had hypertension and died after a stroke.
General examination reveals a BP of 200/100, a
right carotid arterial bruit, a right femoral arterial bruit
and hypertensive retinopathy.
Neurological examination reveals mild UMN signs
in the left arm and major UMN signs in the left leg.
Chest X-ray and ECG both confirm left ventricular
hypertrophy.
Blood tests are all normal.
CT brain scan shows no definite abnormality.

Carotid doppler studies show critical stenosis of the
lower end of the right internal carotid artery.
A small stroke, from which she seems to be
recovering satisfactorily, is the diagnosis discussed
with her.The patient is happy to see the
physiotherapist to help recovery. She is worried about
the degree of recovery from the point of view of
driving, which isnt safe in her present car (which has
a manual gear shift). She understands she is
predisposed to future strokes because of her
hypertension and carotid artery disease. She is
prepared to take preventative drug treatment in the
form of aspirin, blood pressure pills and a statin. She
doesnt smoke. She wants to have a good talk to her
doctor son before submitting herself to carotid
endarterectomy, although she understands the
prophylactic value of this operation to her.
22
CHAPTER 1
A golf course groundsman of 58

years gives a history of lack of
proper movement of the left leg,
making his walking slower. It has
been present for 6 months and
has perhaps worsened slightly.
Most of his work is on a tractor so
the left leg problem hasnt really
affected him at work. He feels he must have a nerve
pinched in his left leg somewhere.
Neurological examination reveals a rather fixed
facial expression, tremor of the lightly closed eyes, a
little cogwheel rigidity in the left arm with slow fine
movements in the left fingers. In the left leg there is a
slight rest tremor and moderate rigidity. He walks in a
posture of mild flexion, with reduced arm swinging on
the left and shuffling of the left leg. His walking is a

little slow.
He is profoundly disappointed to hear that he has
Parkinsons disease. He has never had any previous
illness, and somebody in his village has very severe
Parkinsons disease indeed.
Several consultations are required to explain the
nature of Parkinsons disease, the fact that some
people have it mildly and some severely, that effective
treatment exists in the form of tablets, and that a very
pessimistic viewpoint isnt appropriate or helpful to
him.
Gradually hes coming round to the idea and
becoming more optimistic. Levodopa therapy is
producing significant improvement. Literature
produced by the Parkinsons Disease Society has
helped his understanding of the illness.
A woman of 24 years presents

with a 3-week history of
heaviness and dragging of the
left leg. She has had to stop
driving because of left leg
weakness and clumsiness. For a
week she hasnt been able to
tell the temperature of the bath water with the right
leg, though she can with the weak leg. She has
developed a little bladder frequency and urgency. She
has had to stop her job as a riding school instructor.
Three years ago she lost the vision in her left eye for
a few weeks, but it recovered well. Doctors whom she
saw at the time talked about inflammation of the optic
nerve.
She is engaged to be married in a few months
time.
Neurological examination reveals no abnormalities
in the cranial nerves or arms. She has moderate UMN
signs in the left leg, loss of sense of position in the left

foot and toes, and spinothalamic sensory loss (pain
and temperature) throughout the right leg. She drags
her left leg as she walks.
She understands that she now, almost certainly, has
another episode of inflammation, this time on the left
hand side of her spinal cord, similar in nature to the
optic nerve affair 3 years ago.
She accepts the offer of treatment with high-dose
steroids for 3 days, to help to resolve the
inflammation. She is keen to return to work.
The neurologist knows that he has got quite a lot
more work to do for this girl. He has to arrange for the
investigations to confirm his clinical opinion that she
has multiple sclerosis. He will then have to see her
(and her fianc, if she would like it) and explain that
multiple sclerosis is the underlying explanation for the
symptoms. He will have to do his best to help her to
have an appropriate reaction to this information. Both
she and her fianc will need information and support.
CLINICAL SKILLS
23
A man of 46 years, scaffold-erector,

knows that his left foot is weak. It has
been present for a few months. He has
lost spring at the ankle, and the left
foot is weak when taking all his weight
on ladders and scaffold. Hes had back
pain, on and off, for many years like a
lot of his work mates. He doesnt get paid if hes not
working.
General examination is normal except for some
restriction of forward flexion of the lumbar spine.
Neurological examination reveals wasting and
weakness of the left posterior calf muscles (i.e. foot
and toe plantar-flexors), an absent left ankle jerk, and
impaired cutaneous sensation on the sole and lateral
aspect of the left foot.
Scanning confirms the presence of a large

prolapsed intervertebral disc compressing the left S1
nerve root.
He is offered referral to a neurosurgeon.
His concerns are:
Will the operation work (i.e. restore better function
to the left leg)?
Yes more likely than not, but only over several
months, even up to a year.
How much time off work?
Probable minimum of 68 weeks and then light
duties for a further 68 weeks.
Should he be thinking of changing his job?
Not essential, but an excellent idea if a good
opportunity turned up.
A 38-year-old unkempt
alcoholic presents with a left
foot drop so that he cannot lift
up the foot against gravity, and
as he walks there is a double
strike as his left foot hits the
ground, first with the toe and
then with the heel. Hes very frequently intoxicated,
and he cant remember how, or precisely when, the
foot became like this.
General examination reveals alcohol in his breath,
multiple bruises and minor injuries all over his body,
no liver enlargement, but generally poor nutritional
state.
Neurological examination reveals weakness of the

left foot dorsiflexion, left foot eversion, left toe
dorsiflexion and some altered cutaneous sensation
down the lower anterolateral calf and dorsal aspect of
the foot on the left.
A left common peroneal nerve palsy, secondary
either to compression (when intoxicated) or to trauma,
at the neck of the left fibula, is explained as the most
probable diagnosis.Arrangements are made for a
surgical appliance officer to provide a foot-support,
the physiotherapists to assess him, and for
neurophysiological confirmation of the diagnosis.
The patient defaults on all these and further
appointments.
CHAPTER 2
Stroke
Introduction
Stroke causes sudden loss of neurological function by disrupting the blood supply to the brain. It is the biggest cause
of physical disability in developed countries, and a leading
cause of death. It is also common in many developing countries.
The great majority of strokes come on without warning. This
means that for most patients the aims of management are to
limit the damage to the brain, optimize recovery and prevent recurrence. Strategies to prevent strokes are clearly important.
They concentrate on treating the vascular risk factors that
predispose to stroke, such as hypertension, hyperlipidaemia,
diabetes and smoking.
The two principal pathological processes that give rise to
stroke are occlusion of arteries, causing cerebral ischaemia or
infarction, and rupture of arteries, causing intracranial
haemorrhage (Fig. 2.1). Haemorrhage tends to be much more
destructive and dangerous than ischaemic stroke, with higher
mortality rates and a higher incidence of severe neurological
disability in survivors. Ischaemic stroke is much more common,
and has a much wider range of outcomes.
Fig. 2.1 (a) CT scan showing a

right middle cerebral artery
occlusion as a large wedge of low
density; the blocked artery itself
is bright. (b) CT scan showing a
large left internal capsule
haemorrhage.
(a)
(b)
25
26
CHAPTER 2
Cerebral ischaemia and infarction

Reduction in the flow of blood to any part of the brain first
causes ischaemia, a reversible loss of function, and then, if the
reduction is severe or prolonged, infarction with irreversible
cell death. The blood supply to the anterior parts of the brain
(and to the eyes) comes from the two carotid arteries, which
branch in the neck to give rise to the internal carotid arteries;
these branch again in the head to give rise to the anterior and
middle cerebral arteries. The posterior parts of the brain are
supplied by the two vertebral arteries, which join within the
head to form the basilar artery, which in turn gives rise to the
posterior cerebral arteries (Figs 2.2 and 2.3).
The internal carotid and basilar arteries connect at the base of
the brain through the circle of Willis. This anastomosis allows
some cross-flow if one of the supply arteries is occluded, but the
extent of this varies enormously from patient to patient. Beyond
the circle of Willis, the cerebral arteries are best thought of as
end-arteries. Restoration of normal perfusion in tissue made
ischaemic by occlusion of one of these end-arteries cannot rely
on blood reaching the ischaemic area through anastomotic
channels. Recovery of function in the ischaemic tissue depends much more upon lysis or fragmentation of the occluding
thrombo-embolic material.
The usual cause of occlusion of one of the cerebral arteries is
acute thrombus formation at the site of an atheromatous plaque.
The thrombus can occlude the vessel locally or throw off emboli
which block more distal arteries. This process is particularly
common at the origin of the internal carotid artery, but can occur
anywhere from the aorta to the cerebral artery itself. A less common cause of occlusion is embolism from the heart. In younger
patients, dissection of the carotid or vertebral artery (in which a
split forms between the layers of the artery wall, often after
Anterior cerebral
Anterior
communicating
Middle cerebral
Internal carotid
Basilar
Posterior
communicating
Posterior cerebral
Fig. 2.2 Circle of Willis.
STROKE
27
minor neck trauma) can either occlude the vessel or allow a

thrombus to form and embolize distally (Fig. 2.3).
Patients with hypertension or diabetes may occlude smaller
arteries within the brain through a pathological process which
may have more to do with degeneration in the artery wall than
atheroma and thrombosis. This small vessel disease may cause
infarcts a few millimeters in diameter, termed lacunar strokes,
or a more insidious illness with dementia and gait disturbance.
If complete recovery from an ischaemic event takes place
within minutes or hours, it is termed a transient ischaemic
attack (TIA). Where recovery takes longer than 24 hours the
diagnosis is stroke. The pathophysiology of the two conditions,
and the implications for investigation and treatment, are the
same. In both situations, the history and examination help to
establish the cause (with a view to secondary prevention) and
assess the extent of the damage (to plan rehabilitation).
Middle
cerebral
Anterior
cerebral
Anterior
cerebral
Circle of
Willis
Middle
cerebral
Posterior
cerebral
Circle of
Willis
Basilar
Vertebral
Internal
carotid
Fig. 2.3 Arteries supplying the brain.
Posterior
cerebral
Basilar
Cerebellar
Cerebellar
Eye
Internal
carotid
Ophthalmic
Vertebral
Common
carotid
28
CHAPTER 2
Symptoms and signs of the cause of cerebral ischaemia

and infarction
The common conditions which give rise to cerebral ischaemia
and infarction are listed below.
1. Atheroma in either the large neck arteries or the cerebral
arteries close to the brain. There may be a history of other
atheromatous disease:
previous angina pectoris or heart attack;
intermittent claudication of the legs;
previous TIA or stroke.
There may be a history of vascular risk factors:
hypertension;
diabetes;
hyperlipidaemia;
family history of atheromatous disease;
smoking.
Examination may reveal evidence of these risk factors or evidence of atheroma, with bruits over the carotid, subclavian or
femoral arteries, or absent leg pulses.
2. Cardiac disease associated with embolization:
atrial fibrillation;
mural thrombus after myocardial infarction;
aortic or mitral valve disease;
bacterial endocarditis.
Leg
Trunk
Arm
Hand
Face
Speech
Less common conditions

associated with cerebral
ischaemia or infarction
Non-atheromatous arterial
disease
carotid or vertebral
artery dissection
arteritis
subarachnoid
haemorrhage
Unusual cardiac conditions
giving rise to emboli
non-bacterial
endocarditis
atrial myxoma
paradoxical embolism
(via a patent foramen
ovale)
Unusual embolic material
fat
air
Increased blood viscosity or
coagulability
polycythaemia
antiphospholipid
antibodies
perhaps other
thrombophilic disorders
Venous infarction (where
there is impaired perfusion of
brain tissue drained by
thrombosed or infected
veins)
sagittal sinus thrombosis
cortical
thrombophlebitis
Reading
Writing
Arithmetic
Vision
Eye
Cranial
nerve nuclei
Pathways to and
from cerebellum
Ascending and
descending
tracts
Fig. 2.4 Localization of function

within the brain.
STROKE
29
The loss of function that the patient notices, and which may be
apparent on examination, depends on the area of brain tissue
involved in the ischaemic process (Fig. 2.4).
r
erio
nt
Middle
Posterior
Vertebro-basilar
Anterior
Neurological symptoms and signs of cerebral ischaemia

and infarction
The follow suggest middle cerebral artery ischaemia:

loss of use of the contralateral face and arm;
loss of feeling in the contralateral face and arm;
dysphasia;
dyslexia, dysgraphia, dyscalculia.
The following suggests anterior cerebral artery ischaemia:
loss of use and/or feeling in the contralateral leg.
Middle
Post
e
The following suggests posterior cerebral artery ischaemia:

contralateral homonymous hemianopia.
rior
Vertebro-basilar
Involvement of face, arm and leg with or without a homonymous hemianopia suggests:
internal carotid artery occlusion.
The ophthalmic artery arises from the internal carotid artery
just below the circle of Willis (see Fig. 2.3). The following suggests ophthalmic artery ischaemia:
monocular loss of vision.
Combinations of the following suggest vertebrobasilar artery
ischaemia:
double vision (cranial nerves 3, 4 and 6 and connections);
facial numbness (cranial nerve 5);
facial weakness (cranial nerve 7);
vertigo (cranial nerve 8);
dysphagia (cranial nerves 9 and 10);
dysarthria;
ataxia;
loss of use or feeling in both arms or legs.
The following suggest a small but crucially located lacunar
stroke due to small vessel ischaemia:
pure loss of use in contralateral arm and leg;
pure loss of feeling in contralateral arm and leg.
30
CHAPTER 2
Management of cerebral ischaemia and infarction

There are three aspects of management, which are carried out
simultaneously.
Confirmation of diagnosis
ACT brain scan is usually required to exclude with certainty the
alternative diagnosis of intracerebral haemorrhage.
Optimization of recovery
Thrombolytic therapy, for example with tissue plasminogen activator, has been shown to improve outcome if given within 3
hours of symptom onset. The great majority of patients come to
hospital later than this, and studies to determine the role of
thrombolysis in this group are under way. Acute aspirin treatment is of modest but definite benefit, probably because of its
effect on platelet stickiness.
There is also good evidence that outcome is also improved by
management in a dedicated stroke unit. This effect is probably
attributable to a range of factors, including:
careful explanation to the patient and his relatives;
systematic assessment of swallowing, to prevent choking
and aspiration pneumonia, with percutaneous gastrostomy
feeding if necessary;
early mobilization to prevent the secondary problems of
pneumonia, deep vein thrombosis, pulmonary embolism,
pressure sores, frozen shoulder and contractures;
early socialization to help to prevent depression, and help the
acceptance of any disability; and treatment of depression
when this occurs;
management of blood pressure, avoiding over-enthusiastic
treatment of hypertension in the first 2 weeks or so (when
cerebral perfusion of the ischaemic area is dependent upon
blood pressure because of impaired autoregulation), moving
towards active treatment to achieve normal blood pressure
thereafter;
early involvement of physiotherapists, speech therapists
and occupational therapists with a multidisciplinary team
approach;
good liaison with local services for continuing rehabilitation, including attention to the occupational and financial consequences of the event with the help of a social
worker.
Prevention of further similar episodes
This means the identification and treatment of the aetiological
factors already mentioned. The risk of further stroke is significantly diminished by blood pressure reduction, with a thiazide
Large, wedge-shaped, low

density area representing tissue
infarction by a right middle
cerebral artery occlusion.
Recovery from stroke

? Thrombolysis
Aspirin
Stroke unit
Attention to swallowing
Explanation
Mobilization
Socialization
Careful BP management
Paramedical therapists
Rehabilitation
STROKE
Significant stenosis by atheroma

at the origin of the internal carotid
artery.
Stroke prevention
Lower blood pressure with
thiazide ACE inhibitor
Statin
Aspirin
Warfarin for those with a
cardiac source of emboli
Carotid endarterectomy for
ideally selected patients
Identify and treat ischaemic
heart disease and diabetes
Discourage smoking
31
diuretic and an angiotensin-converting enzyme (ACE) inhibitor, even in patients with normal blood pressure, and also
by statin therapy even if the cholesterol level lies within the
normal range.
Investigations should include a full blood count, ESR (if
elevated consider vasculitis, endocarditis, atrial myxoma or
secondary infection), fasting glucose and lipids, and a search
for cardiac sources of emboli. This starts with a careful cardiac
examination, ECG and chest X-ray, supplemented by transthoracic or transoesophageal echocardiography if there is any
suspicion of a cardiac source.
If the stroke is in the carotid territory, and especially if the
patient has made a reasonable recovery and is otherwise
healthy, further investigation should be undertaken to see if
the patient might benefit from carotid endarterectomy. The
patients who benefit most from such surgery are those with
localized atheroma at the origin of the internal carotid artery
in the neck, producing significant (over 70%) stenosis of the
arterial lumen. The severity of stenosis can be established noninvasively by Doppler ultrasound or MR angiography, supplemented if necessary by catheter angiography. The presence or
absence of a carotid bruit is not a reliable guide to the degree of
stenosis. Well-selected patients in first-class surgical hands
achieve a definite reduction in the incidence of subsequent
ipsilateral stroke.
Antiplatelet drugs (e.g. aspirin) reduce the risk of further
strokes (and myocardial infarction). Anticoagulation with warfarin is of particular benefit in atrial fibrillation and where a cardiac source of embolization has been found.
Despite all this, some patients continue to have strokes
and may develop complex disability. Patients with predominantly small vessel disease may go on to suffer cognitive
impairment (so-called multi-infarct dementia, described on
p. 231) or gait disturbance with the marche petits pas (walking
by means of small shuffling steps, which may be out of proportion to the relatively minor abnormalities to be found in
the legs on neurological examination). Patients with multiple
infarcts arising from large vessels tend to accumulate physical
deficits affecting vision, speech, limb movement and balance.
Some degree of pseudobulbar palsy (with slurred speech,
brisk jaw-jerk and emotional lability) is common in such cases
due to the bilateral cerebral hemisphere involvement, affecting
upper motor neurone innervation of the lower cranial nerve
nuclei (see p. 134).
32
CHAPTER 2
Subarachnoid haemorrhage and

intracerebral haemorrhage
Anatomy and pathology
The pathological process here is the sudden release of arterial
blood, either into the subarachnoid space around the brain, or
directly into the substance of the brain. In subarachnoid haemorrhage the bleeding usually comes from a berry aneurysm arising from one of the arteries at the base of the brain, around the
circle of Willis (Fig. 2.5, left). In middle-aged, hypertensive patients, intracerebral haemorrhage tends to occur in the internal
capsule or the pons, because of the rupture of long thin penetrating arteries (Fig. 2.5, right). In older patients, intracerebral
haemorrhages occur more superficially in the cerebral cortex as
a result of cerebral amyloid angiopathy. Arteriovenous malformations of the brain are a rare cause of either subarachnoid or
intracerebral haemorrhage.
Thalamus
Brain covered
by pia
Corpus
striatum
CSF
Bone covered by dura

Middle cerebral artery
bearing an aneurysm
Surface
of brain
Middle cerebral artery
giving rise to the long
thin striate arteries
Micro-aneurysms
on the course of a
striate artery in a
hypertensive
patient
Fig. 2.5 Left: Berry aneurysm, the common cause of subarachnoid haemorrhage. Right: Micro-aneurysms,
the common cause of intracerebral haemorrhage.
STROKE
33
Neurological symptoms and signs
Subarachnoid haemorrhage
Sudden, very severe
headache
Neck stiffness/rigidity
vomiting
loss of consciousness
papilloedema
neurological deficit
Intracerebral haemorrhage
Sudden, severe
Headache
vomiting
papilloedema
The range of consequences of subarachnoid and intracranial

haemorrhage is illustrated in Figs 2.6 and 2.7. Both cause a sudden rise in intracranial pressure, with headache, vomiting and
a decrease in conscious level, which may be followed by the
development of papilloedema.
In subarachnoid haemorrhage the bleeding irritates the
meninges. This causes the characteristic sudden severe
headache (like being hit on the head with a baseball bat) and
neck stiffness; there is often a brief loss of consciousness at the
moment of the bleed. It is the suddenness of onset which helps
to differentiate subarachnoid haemorrhage from the headache
and neck stiffness of infective meningitis, which come on over a
few hours rather than seconds. Migraine can sometimes produce severe headache abruptly but without the severe neck
stiffness of subarachnoid haemorrhage.
An intracerebral bleed in the region of the internal capsule will
cause sudden severe motor, sensory and visual problems on the
contralateral side of the body (hemiplegia, hemianaesthesia
and homonymous hemianopia). In the pons, sudden loss of
motor and sensory functions in all four limbs, associated with
disordered brainstem function, accounts for the extremely high
mortality of haemorrhage in this area.
Bleeding into the ventricular system, whether the initial bleed is
subarachnoid or intracerebral, is of grave prognostic significance. It is frequently found in patients dying within hours of
the bleed.
High blood pressure readings may be found in patients
shortly after subarachnoid or intracerebral haemorrhage, either
as a response to the bleed or because of pre-existing hypertension. Over-enthusiastic lowering of the blood pressure is
not indicated because the damaged brain will have lost its
ability to autoregulate. Low blood pressure therefore leads to
reduced perfusion of the damaged brain tissue.
34
Common to all cases

of subarachnoid
haemorrhage
CHAPTER 2
1 Blood throughout the subarachnoid space,

therefore headache and neck stiffness
2 Raised intracranial pressure, therefore the
possibility of depressed conscious level,
headache, vomiting, papilloedema
No brain damage, so no focal

Spasm of a cerebral artery has

occurred in the vicinity of an
aneurysm, usually a few days
after the initial bleed, causing
focal ischaemia and neurological
deficit
The aneurysm has also bled

directly into adjacent brain
tissue, forming a haematoma
and causing immediate focal
The aneurysm has bled into

adjacent brain tissue, and the
haematoma has burst into the
ventricles. Focal neurological
deficit and coma likely
Fig. 2.6 Diagram to show the

clinical features of subarachnoid
haemorrhage.
STROKE
35
Common to all cases

of intracerebral
haemorrhage
1 Rapid rise in intracranial pressure, therefore

the possibility of depressed conscious level,
headache, vomiting, papilloedema
2 Destruction of brain tissue by the
haematoma producing a focal neurological
deficit, appropriate to the site of the lesion
In the region of the internal

capsule, therefore contralateral
hemiplegia, hemianaesthesia
and homonymous hemianopia
Features of an internal capsular

bleed, plus rupture of blood into
the ventricles with possible
subsequent appearance in the
CSF. Coma and neck stiffness
may result
In the region of the pons,

therefore massive neurological
deficit (cranial nerve palsies,
cerebellar signs and quadriplegia),
plus obstructive hydrocephalus
making coma extremely likely

clinical features of intracerebral
haemorrhage.
36
CHAPTER 2
Management of subarachnoid haemorrhage

Establish the diagnosis
The first investigation for suspected subarachnoid haemorrhage is an urgent CT brain scan. This will confirm the presence
of subarachnoid blood in the great majority of cases. If the
scan is normal, a lumbar puncture should be performed to
look for blood or xanthochromia (degraded blood) in the CSF.
The CSF may be normal if the lumbar puncture is performed
within a few hours or more than 2 weeks after the bleed. A lumbar puncture should not be performed if the subarachnoid
haemorrhage has been complicated by the development of an
intracerebral haematoma, since it may lead to coning (see pp.
424).
Prevent spasm and minimize infarction
The calcium channel blocker nimodipine reduces cerebral
infarction and improves outcome after subarachnoid haemorrhage. Disturbances of sodium homeostasis are common,
and the electrolytes should be monitored regularly.
CT scan showing high density in

the sulci over the right cerebral
hemisphere due to subarachnoid
blood.
Prevent re-bleeding
Specialist neurosurgical advice should be sought. Patients who
have withstood their first bleed well are offered carotid and vertebral angiography within a few days to establish whether or
not a treatable aneurysm is present. Treatment usually involves
either surgically placing a clip over the neck of the aneurysm to
exclude it from the circulation, or packing of the aneurysm with
metal coils, delivered by arterial catheter under radiological
guidance, to cause it to thrombose.
Patients with subarachnoid haemorrhages confined to the
area in front of the upper brainstem (so-called perimesencephalic haemorrhages) rarely have an underlying aneurysm
and have an excellent prognosis without treatment.
Rehabilitation
Many patients who survive subarachnoid haemorrhage (and its
treatment) have significant brain damage. A proportion, often
young, will not be able to return to normal activities. They will
need support from relatives, nurses, physiotherapists, speech
therapists, occupational therapists, psychologists and social
workers, ideally in specialist rehabilitation units.
This has come from the large

middle cerebral aneurysm
arrowed.
STROKE
37
Management of intracerebral haemorrhage
Enhanced CT scan showing high

signal in an arteriovenous
malformation, with bleeding into
the adjacent brain (asterisk) and
subarachnoid space.

A CT brain scan will confirm the diagnosis and steer you away
from thrombolytic therapy which would make the bleeding
worse. The location and extent of the bleeding may give a clue to
the cause and will determine management.
Lesions in the internal capsule
1. The optimal initial treatment is still a matter of research. It
may sometimes be helpful to reduce intracranial pressure, for
example with mannitol or by removing the haematoma.
2. Hypertension should be treated gently at first, and more
vigorously after a few weeks.
3. Rehabilitation: a major and persistent neurological deficit is
to be expected, and all the agencies mentioned under rehabilitation of patients with subarachnoid haemorrhage are likely to be
of value.
Lesions in the pons
The mortality and morbidity of lesions in the pons are such as to
make active treatment of any sort of questionable medical or
ethical merit.
Lesions in the cerebral cortex
If there is a single cortical bleed, especially in a younger patient,
then consideration should be given to a search for an underlying arteriovenous vascular malformation. This can be done
with MR imaging once the haematoma has resolved. Multiple
cortical bleeds in the elderly are usually due to cerebral amyloid
angiopathy and are best treated conservatively. There is a high
risk of both recurrence and subsequent dementia in this latter
group of patients.
CT scan showing a recent right

parietal haemorrhage and low
density from a previous left
frontal bleed, due to amyloid
angiopathy.
The ideal treatment of intracerebral haemorrhage is prophylactic. Intracerebral haemorrhage is one of the major complications
of untreated hypertension. There is good evidence to show that
conscientious treatment of high blood pressure reduces the incidence of intracerebral haemorrhage in hypertensive patients.
38
CHAPTER 2
CASE HISTORIES
Case 1
A 42-year-old teacher has to retire from the classroom
abruptly on account of the most sudden and severe
headache she has ever had in her life. She has to sit
down in the staffroom and summon help on the
phone.The school secretary calls for an ambulance as
soon as she sees the patient, who is normally very
robust and is clearly in extreme pain.
On arrival in the hospital A & E department, the
patient is still in severe pain. Her BP is 160/100 and
she shows marked neck stiffness but no other
abnormalities on general or neurological examination.
A clinical diagnosis of subarachnoid haemorrhage is
made. She is admitted to the medical ward.
a. What investigations would you arrange?
b. How would you proceed now?
Case 2
A 55-year-old publicans wife is known to suffer from
significant hypertension. She is receiving medication
from her GP for this, but is not at all compliant. She
smokes, and partakes of a drink. She is very

overweight.
She suddenly collapses in the kitchen. She is
unrousable except by painful stimuli, is retching and
vomiting, and does not move her left limbs at all.
a. What is the most likely diagnosis?
She is taken to hospital by ambulance. Her physical
signs in A & E are:
obese;
BP 240/140;
no neck stiffness;
no response to verbal command, and no speech;
eyes closed, and deviated to the right when the
eyelids are elevated;
no movement of left limbs to painful stimuli;
withdrawal of right limbs from painful stimuli;
extensor left plantar response.
b. What is the most likely diagnosis?
c. What investigations would you arrange?
d. What would you tell her husband?
(For answers, see p. 255.)
CHAPTER 3
Brain tumour
Introduction
Like malignant neoplasms anywhere else in the body, histologically malignant brain tumours carry a poor prognosis. Histologically benign brain tumours are often difficult to remove.
This may be the result of a lack of clear boundary between
tumour tissue and normal brain substance, e.g. in a low-grade
cerebral astrocytoma, or because the tumour lies very close to
a part of the brain with important functions, e.g. an acoustic
neuroma lying beside the brainstem.
Brain tumours therefore have an unfavourable reputation. It
is frustrating that the improvements in our ability to diagnose
brain tumours (with better imaging and less invasive biopsy
techniques) are only just starting to be accompanied by
improvements in our ability to treat them.
Intracranial compartments
Many of the problems caused by brain tumours arise because
the brain lies within a rigid compartmentalized box (Fig. 3.1).
The falx cerebri runs vertically from the front to the back of the
head. The two compartments on either side each contain a cerebral hemisphere. These are joined together below the front of
the falx by the corpus callosum. At the back of the falx, the tentorium cerebelli runs from side to side. Below it is the third compartment, the posterior fossa. This contains the brainstem and
the cerebellum. The top of the brainstem (i.e. the midbrain) is
continuous with the cerebral hemispheres through a hole in the
tentorium, the tentorial hiatus. The bottom of the brainstem (i.e.
the medulla) leads to the spinal cord through a hole in the floor
of the skull, the foramen magnum.
Figure 3.1 also shows the ventricular system. Cerebrospinal
fluid is produced by the choroid plexus in each of the ventricles.
It passes down through the ventricular system, leaving the
fourth ventricles via the foramina of Luschka and Magendie to
enter the subarachnoid space. It then circulates over the surface
of the brain and spinal cord before being resorbed.
40
BRAIN TUMOUR
41
Lateral aspect of brain
Surface
of brain
Coronal section through brain

(posterior aspect)
Dura
Dura
Surface
of brain
FC
LV
LV
3
Eye
L
TC
Dura
TC
Median section through brain
Dura
Dura
Cerebellum removed to
reveal posterior aspect
of midbrain, cut cerebellar
peduncles, floor of 4th
ventricle, posterior
aspect of medulla and
spinal cord
FC
Shape of ventricles
(viewed from left)
CC
3
TC
FM
4
M
T
C
Dura
(a)
(b)
Flow of CSF
Lateral ventricles which contain

choroid plexus
The rigid frame containing the brain

Dura
Foramina of Munro
3rd ventricle which contains
choroid plexus
FC
Aqueduct
4th ventricle which contains
choroid plexus
TC
Foramina of Luschka and

Magendie
Subarachnoid space
Key
L =
M=
F =
B=
O =
T =
Foramen of Luschka
Foramen of Magendie
Frontal horn
Of lateral ventricle
Body
within each cerebral
Occipital horn
hemisphere
Temporal horn
(c)
LV
FM
FC
CC
TC
=
=
=
=
=
Lateral ventricle
Foramen of Munro
Falx cerebri
Corpus callosum
Tentorium cerebelli
Dura
Interconnected
3 = 3rd ventricle
by aqueduct
4 = 4th ventricle
C = Cisterns, i.e. spaces filled by
CSF in the subarachnoid space
around the base of the brain
Fig. 3.1 (a) Diagrams to show the lateral aspect of, and a median section through, the brain. (b) Diagrams
to show a coronal section through the brain (posterior aspect) and the shape of the ventricles as viewed
from the left. (c) Diagram to show the rigid frame containing the brain.
42
CHAPTER 3
Tentorial herniation, coning and shift

Figure 3.2 shows the influence of mass lesions situated in different parts of the compartmentalized space within the skull.
When a mass lesion is making one cerebral hemisphere too large
for its compartment (Fig. 3.2a):
the supratentorial midline structures (corpus callosum and
3rd ventricle) are pushed towards the opposite side of the
skull below the falx;
the infero-medial part of the cerebral hemisphere is pushed
through the tentorial hiatus (compressing the midbrain);
the whole brainstem is pushed downwards so that the lowermost parts of the cerebellum and medulla oblongata become
impacted in the foramen magnum.
The movement at the tentorial hiatus is known as tentorial
herniation, and the impaction at the foramen magnum is known
as coning of the medulla. They commonly occur simultaneously.
The effects on the patient are:
depression in conscious level (distortion of the reticular formation lying throughout the whole of the brainstem);
an impairment of ipsilateral 3rd nerve function and dilatation of the pupil (tentorial herniation compressing the
midbrain);
interference with the vital functions of respiration and circulation (compression of the medulla oblongata).
A mass lesion situated in the midline (Fig. 3.2b) causes obstruction to the downward flow of CSF through the ventricular
system. Under such circumstances, the ventricles above the site
of obstruction dilate, and both cerebral hemispheres become
too large for their compartments. Bilateral tentorial herniation
and coning are likely to occur with the same dangerous clinical
consequences.
In the presence of a unilateral posterior fossa mass lesion
(Fig. 3.2c), there is movement of the midline posterior fossa
structures to one side. This may compress the 4th ventricle sufficiently to block the downward flow of CSF, resulting in ventricular dilatation above the site of obstruction. There will be
downward movement and compression at the level of the foramen magnum. At the tentorium cerebelli, there may be upward
movement and compression of the midbrain or, if the supratentorial ventricular dilatation becomes very marked there may be
downward herniation bilaterally. Depression of conscious
level, dilated pupils and impaired vital functions may all result
from such a lesion.
Coning
Cause:
usually downward
movement of brainstem
Effects:
conscious level
pupils reaction to light
vital functions including
breathing
Aggravated:
by lumbar puncture
BRAIN TUMOUR
43
Brain shift, secondary to

a unilateral cerebral
hemisphere mass lesion
(a)
Ventricular dilatation and

brain shift secondary to a
midline mass lesion
obstructing the flow of
CSF
(b)
Brain shift and ventricular

dilatation secondary to a
unilateral posterior fossa
mass lesion
(c)
Fig. 3.2 (a) Brain shift secondary to a unilateral cerebral hemisphere mass lesion. (b) Ventricular dilatation
and brain shift secondary to a midline mass lesion obstructing the flow of CSF. (c) Brain shift and
ventricular dilatation secondary to a unilateral posterior fossa mass lesion.
44
Lethal lumbar puncture

Lumbar puncture is dangerous if intracranial pressure is raised
due to a mass lesion. It reduces the CSF pressure below the foramen magnum. This can encourage downward brain shifts with
tentorial herniation and coning of the medulla. This in turn
causes progressive loss of consciousness and impaired control
of breathing, which may be ultimately fatal. Lumbar puncture
should not, therefore, be performed where there is known to be
a mass lesion of sufficient size to cause raised intracranial pressure, or in situations where the possibility of one exists. Examples of these situations include patients with focal deficit (such
as a hemiparesis), patients with papilloedema, and patients
who are in a coma of unknown cause. In all these situations, the
cause should be clarified with brain imaging before a lumbar
puncture is contemplated.
On the other hand, where headache and papilloedema are
due to a general elevation of intracranial pressure without any
mass lesion, e.g. in meningitis and uncomplicated subarachnoid haemorrhage, lumbar puncture is safe and may relieve
symptoms.
False localizing signs

We have seen that a mass lesion in one compartment of the brain
can induce shift and compression in parts of the brain remote
from the primary lesion. Brain tumours that are causing raised
intracranial pressure are known to produce false localizing signs,
which are no more than clinical evidence of these secondary
movements of brain tissue:
the descent of the brainstem may stretch the 6th cranial nerve
to produce a non-localizing lateral rectus palsy:
the ventricular dilatation above midline CSF obstructive
lesions (Fig. 3.2b), or above posterior fossa lesions (Fig. 3.2c),
may produce:
intellectual and behavioural changes suggestive of primary frontal pathology;
an interference with vertical eye movements (which are
programmed in the upper midbrain) because of the dilatation of the posterior part of the 3rd ventricle and aqueduct;
the impairment of conscious level, pupillary dilatation
and depression of vital functions, mentioned already in this
chapter, are the most pressing false localizing signs and
demand immediate action by doctors in charge of the case.
CHAPTER 3
Practical tip
1. Always measure the CSF
pressure at the start of a
lumbar puncture
2. Make sure the patient is
relaxed and not too tightly
curled
3. If the pressure is
unexpectedly elevated
(>25 cm of CSF):
collect the sample in the
manometer
take out the LP needle
secure IV access in case you
need to give mannitol
start neurological
observations at 15-minute
intervals
arrange an urgent brain
scan
consider obtaining
neurosurgical advice
False localizing sings

Sixth nerve palsy
Frontal signs
Impaired up-gaze
Signs of coning
BRAIN TUMOUR
Clinical features of
brain tumours
Raised intracranial pressure
Epilepsy
Evolving neurological
deficit
45
Clinical features
There are three groups of symptoms and signs resulting from
brain tumours: raised intracranial pressure, epilepsy and an
evolving focal neurological deficit.

The cardinal features of raised intracranial pressure are:
headache;
vomiting;
papulloedema;
false localizing signs;
depression of conscious level;
signs of tentorial herniation and coning.
Only two further clinical points need to be made about these
features.
1. The headaches of raised intracranial pressure tend not to be
extremely severe, they do keep on troubling the patient, they are
usually generalized throughout the head, and they tend to be
worse in the mornings when the patient wakes. They sometimes wake the patient earlier than his normal waking time.
They may be made worse by coughing, straining and bending.
Unfortunately, like many things in clinical medicine, none of
these features is absolutely specific.
2. Perfusion of the retina and optic disc may become critical in
the presence of raised intracerebral pressure and papilloedema.
The patient may report transient blurring or loss of vision. Such
visual obscurations should stimulate urgent investigation and
treatment.
Epilepsy
Focal epilepsy, focal epilepsy progressing on to a generalized
tonicclonic seizure, tonicclonic seizures with post-ictal focal
neurological signs, and tonicclonic epilepsy without any apparent focal features may all indicate the presence of a tumour
in the cerebrum. (Focal epilepsy is discussed in detail on
pp. 1968). Epilepsy is not a feature of posterior fossa tumours.
Epilepsy is not commonly caused by tumours, and less than
50% of cerebral tumours produce epilepsy, but the occurrence
of epilepsy in adult life should prompt the possibility of a brain
tumour in the doctors mind.
46
Frontal
(Tend to present late since tumours
here can become quite large before
producing a definite neurological deficit)
Dementia
Alteration of mood
Alteration of behaviour
Incomplete insight
Incontinence
Olfactory or optic
nerve malfunction
CHAPTER 3
Central
(Tend to present early)
Contralateral limb weakness
Contralateral sensory loss
Dysphasia if dominant hemisphere
Parietal
If nonSpatial disorientation dominant
Dressing dyspraxia
hemisphere
Dyslexia
Dysgraphia If dominant
hemisphere
Dyscalculia
Neglect of contralateral limbs
Contralateral homonymous
lower quadrant visual field
defect or neglect (see pp. 11314)
Occipital
hemianopia
Temporal
Dysphasia if dominant hemisphere
Memory impairment
Alteration in mood
Alteration in behaviour
upper quadrant visual
field defect or neglect
(see pp. 11314)
Lateral posterior fossa
e.g. Acoustic neuroma
Cranial nerve palsies 8, 7, 5
Ipsilateral cerebellar deficit
Fig. 3.3 Lateral aspect of the brain, showing the neurological deficits produced by tumours at various
sites.
An evolving focal neurological deficit

The presence of a tumour impairs the function of the part of the
brain in which it resides. The nature of the evolving focal neurological deficit clearly depends on the site of the lesion. Figures
3.3 and 3.4 depict the principal neurological deficits produced
by tumours situated in various parts of the brain. Tumours near
the midline (Fig. 3.4) and in the posterior fossa may produce
marked features of raised intracranial pressure before there are
many localizing signs.
BRAIN TUMOUR
47
Bifrontal and
corpus callosum
Dementia
Gait disturbance
Pituitary gland
Optic chiasm
Hypothalamus
Visual field defects
Appetite or
Wakefulness
Endocrine effects
Cranial nerves 3,
4, 6, 5a
Fig. 3.4 Median section through

the brain, showing the
neurological deficits produced by
tumours at various sites.
Cerebellum
Nystagmus
Dysarthria
Limb and gait
ataxia
Brainstem
Cranial nerve palsies
312, depending on
level of tumour
Cerebellar deficit, due
to impaired inflow or
outflow from the
cerebellum
Long tract, motor
and sensory, deficits
in limbs and trunk
Impaired vital functions
i.e. respiration,
thermo-regulation
and circulation
The length of history of any of the three main features of a

brain tumour is a guide to the nature of the tumour, in terms of
malignancy. This is not foolproof, however. Certainly a long
history suggests a benign or low-grade malignancy tumour.
A short progressive history obviously implies malignancy,
but sometimes a benign tumour can be silent for years only to
produce a short worrying history when the brain and the
intracranial compartments finally become unable to absorb
the presence of the enlarging mass lesion.
48
Common brain tumours

Gliomas are seen to appear in both the benign and malignant
groups of tumours. Astrocytomas are by far the most common
glial tumour; tumours derived from oligodendrocytes, ependyma, neurones, primitive neuroectodermal or other tissues
are much rarer. Unless otherwise specified, the words primary
brain tumour or glioma refer to an astrocytoma in clinical
practice. Gliomas are classified histologically from grade 1
(benign) to grade 4 (the highly malignant glioblastoma multiforme). Benign gliomas are, unfortunately, much less common
than malignant ones and have a tendency to become more
malignant with time.
CHAPTER 3
Benign
Grade 12 gliomas
Meningioma
Pituitary adenoma
Acoustic neuroma
Malignant
Primary
grade 34 gliomas
Secondary
metastatic carcinoma
Meningiomas are nearly always benign. They may arise

from any part of the meninges, over the surface of the brain,
from the falx, or from the tentorium. There is a plane of cleavage
between tumour and brain tissue which makes total removal a
definite possibility, so long as the tumour is reasonably accessible and unattached to dural venous sinuses, e.g. the sagittal
sinus.
Pituitary adenomas produce two principal sets of symptoms:
space-occupying effects and endocrine disturbance. When
the pituitary gland enlarges in the pituitary fossa, it most commonly expands upwards (suprasellar extension) to compress
optic nerves/chiasm/tracts. The classical bitemporal hemianopia resulting from chiasmal compression occurs when the
optic chiasm is right above a pituitary extension which is
directly upwards. The exact position of the optic chiasm, and
the direction of pituitary expansion, do, however, vary from
one case to another, so monocular blindness due to optic nerve
compression and homonymous hemianopia from optic tract
compression are not uncommon in patients with pituitary
adenomas. Lateral expansion of pituitary adenomas may compress structures on the lateral wall of the cavernous sinus
(cranial nerves, 3, 4, 5a and 6), producing double vision and
forehead numbness. Forward and downward expansion of the
adenoma results in enormous expansion of the pituitary fossa
and occasional erosion through bone into the sphenoidal air
sinus.
The endocrine disturbances that accompany the development of a pituitary adenoma are positive if the tumour cells are
secretory (prolactin, growth hormone, etc.), and negative if the
tumour is preventing normal secretion by the rest of the pituitary gland (varying degrees of panhypopituitarism).
The glioma (top) is poorly

differentiated from the
surrounding brain, unlike the
meningioma (bottom).
BRAIN TUMOUR
49
Acoustic neuromas are benign tumours of the Schwann cells

along the course of the acoustic nerve, between the cerebellopontine angle and the internal auditory meatus in the petrous
temporal bone. First and foremost, they produce progressive
unilateral nerve deafness, but by the time of recognition there
may well be associated 5th and 7th nerve dysfunction, unilateral cerebellar signs and evidence of raised intracranial pressure. Early diagnosis is highly desirable since a small tumour
can be treated with radiotherapy or surgery with fewer complications than a large one which has caused brainstem displacement and raised intracranial pressure.
Pituitary adenoma (arrows).
Common malignant tumours in the brain are either gliomas or

metastases, in particular malignant astrocytomas and metastatic
carcinoma. Together these constitute well over 60% of all brain
tumours. The history is usually short, of raised intracranial
pressure, epilepsy or neurological deficit. Not uncommonly, all
three groups of symptoms are present by the time of diagnosis.
It is not uncommon for a primary carcinoma elsewhere in the
body to present with metastatic disease in the brain. If the
metastases are multiple, the differentiation from malignant
glioma is not difficult, but solitary cerebral metastases are quite
common.
Differential diagnosis
Acoustic neuroma (arrow).
Metastases (arrows).
The common presenting symptoms of brain tumours are:

raised intracranial pressure;
epilepsy;
a progressive focal neurological deficit.
Clearly, other mass lesions within the head may produce all
three features. There may be difficulty in differentiating a malignant tumour from an intracerebral abscess when the history is a
short one, and from subdural haematoma when the history is a
little longer.
Other causes of raised intracranial pressure include severe
arterial hypertension, chronic meningitis and so-called benign
intracranial hypertension (BIH). BIH is probably due to reduced
venous drainage of the brain, and mainly affects young obese
women or patients with a predisposition to venous thrombosis.
Epilepsy and focal epilepsy are more usually caused by
epileptogenic scars from previous intracranial disease.
The principal alternative cause of a progressive subacute
focal neurological deficit is an ischaemic stroke, which occasionally, instead of developing with characteristic abruptness,
comes on in a stuttering way.
50
Investigation
Brain imaging
Brain tumours can be detected using CT X-ray scanning or MR
scanning. The choice of technique used will often depend upon
local facilities. CT is cheaper, more widely available and (when
used with contrast enhancement) capable of detecting the
majority of tumours. MR scanning is superior in many ways,
especially in detecting small tumours and tumours in the base
of the skull and the posterior fossa. MR also allows the images to
be presented in a range of planes, which helps with surgical
planning.
Admission to hospital
If the clinical presentation and results of scanning suggest a
brain tumour, admission to hospital for further investigation
will be indicated in most cases, especially if there is progressive
neurological deficit or evidence of raised intracranial pressure.
This will be an extremely stressful experience for the patient and
relatives. Reassurance, sympathy and encouragement together
with adequate explanation are required.
Other tests
The brain imaging procedures mentioned above sometimes
require the support of:
radiological and other imaging techniques elsewhere in the
body if metastatic disease seems likely;
carotid or vertebral angiography if the neurosurgical team
needs this information prior to surgery;
haematological and biochemical investigation if cerebral
abscess, granuloma, metastatic disease or pituitary pathology are under consideration.
(NB Lumbar puncture is contra-indicated in suspected cases of
cerebral tumour.)
CHAPTER 3
BRAIN TUMOUR
Management of brain tumours

Admission to hospital
Scanning
No lumbar puncture
Dexamethasone
Surgery
51
Management
If the patient shows marked features of raised intracranial pressure and scanning displays considerable cerebral oedema,
dexamethasone may be used with significant benefit. The patient will be relieved of unpleasant, and sometimes dangerous,
symptoms and signs, and the intracranial state made much
safer if neurosurgical intervention is to be undertaken.
Radiotherapy
Anticonvulsants
Surgical management
Complete removal
Meningiomas, pituitary tumours not susceptible to medical
treatment, acoustic neuromas and some solitary metastases in
accessible regions of the brain can all be removed completely.
Sometimes, the neurosurgical operation required is long and
difficult if the benign tumour is relatively inaccessible.
Partial removal
Gliomas in the frontal, occipital and temporal poles may be
removed by fairly radical debulking operations. Sometimes,
benign tumours cannot be removed in their entirety because of
tumour position or patient frailty.
Biopsy
If at all possible, the histological nature of any mass lesion in the
brain should be established. What looks like a glioma or metastasis from the clinical and radiological points of view occasionally turns out to be an abscess, a benign tumour or a granuloma.
If the mass lesion is not in a part of the brain where partial removal can be attempted, biopsy by means of a needle through
a burrhole usually establishes the histological diagnosis. The
accuracy and safety of this procedure may be increased by use of
stereotactic surgical techniques. Histological confirmation may
not be mandatory where there is strong collateral evidence of
metastatic disease.
Histological confirmation may be postponed in patients presenting with epilepsy only, in whom a rather small mass lesion
in an inaccessible part of the brain is revealed by a scan. Sequential scanning, initially at short intervals, may be the most reasonable management plan in such patients.
52
Shunting and endoscopic surgery

Midline tumours causing ventricular dilatation are routinely
treated by the insertion of a shunt into the dilated ventricular
system. The shunt tubing is tunnelled under the skin to drain
into the peritoneal cavity. This returns intracranial pressure to
normal, and may completely relieve the patients symptoms.
Sometimes it is possible and desirable to remove the tumour or
treat the hydrocephalus using intracranial endoscopic procedures instead.
Additional forms of treatment

Radiotherapy
Middle-grade gliomas, metastases and incompletely removed
pituitary adenomas are the common intracranial tumours
which are radiosensitive. The posterior fossa malignant tumours of childhood and lymphoma are also sensitive to radiotherapy. Radiotherapy commonly follows partial removal or
biopsy of such lesions, and continues over a few weeks whilst
the preoperative dose of dexamethasone is being gradually
reduced.
Chemotherapy
Chemotherapy can be useful as primary treatment for lymphoma and as adjunctive therapy for oligodendroglioma and
some high-grade astrocytomas.
Anticonvulsants
Control of epilepsy may be an important part of the management of a patient with a supratentorial brain tumour.
Dexamethasone
Taken in large and constant dosage, dexamethasone may be the
most humane treatment of patients with highly malignant
gliomas or metastatic disease. Used in this way, dexamethasone
often allows significant symptomatic relief so that the patient
can return home and enjoy a short period of dignified existence
before the tumour once more shows its presence. At this point,
dexamethasone can be withdrawn and opiates used as
required.
CHAPTER 3
BRAIN TUMOUR
53
Prognosis
The fact that the majority of brain tumours are either malignant
gliomas or metastases, which obviously carry a very poor prognosis, hangs like a cloud over the outlook for patients with the
common brain tumours.
The table below summarizes the outlook for patients with the
common brain tumours. It can be seen that such pessimism is
justified for malignant brain tumours, but not for the less common benign neoplasms.
Tumour
Treatment
Outcome
Meningioma
Surgical removal if possible

and/or radiotherapy if not
Residual disability common

Recurrence rate (1% per year)
Watch and wait or

biopsy and radiotherapy or
partial removal radiotherapy
chemotherapy
Prolonged survival, usually with

residual disability, but recurrence is
very common and often higher grade
Partial removal and radiotherapy

chemotherapy or palliative care
Few patients survive 1 year
Lymphoma
Biopsy and chemotherapy
Improving: median survival about 2 years
Pituitary adenoma
Medical therapy for prolactinoma

or
surgical removal radiotherapy
Excellent
Acoustic neuroma
Watch and wait

or radiotherapy
or surgical removal
Deafness facial weakness are

common; survival is excellent
Glioma
Lower grades
High grade
54
CHAPTER 3
CASE HISTORIES
Case 1
A 65-year-old widow presents with a 6-month history
of unsteadiness. She has started to veer to the left. She
has been well prior to this, apart from a longstanding
hearing problem and surgery for colon cancer 5 years
ago. On examination she has an ataxic gait, slight
leftwards nystagmus, an absent left corneal reflex
and marked left-sided deafness.There is no
papilloedema.
a. What do you think is the cause of her symptoms?
Case 2
A 45-year-old oil company executive returns from
secondment in Nigeria because of ill health. Over the
last 3 months he has become slow and erratic, making

frequent mistakes at work and getting lost on his way
home. His memory has become poor and he has had
difficulty in finding his words. His appetite has faded
and he has lost weight. In the last 2 weeks he has
become unsteady on his feet and incontinent of
urine.
On examination he is thin. He has no fever but his
axillary and inguinal lymph nodes are slightly
enlarged. He is drowsy. He had bilateral papilloedema.
He has difficulty cooperating with a neurological
examination and becomes increasingly irritated when
you persist.
a. What do you think is the cause of his symptoms?
CHAPTER 4
Head injury
The causes
Road traffic accidents involving car drivers, car passengers,
motorcycle drivers and pillion-seat riders, cyclists, pedestrians
and runners constitute the single greatest cause of head injury
in Western society. Compulsory speed limits, car seat-belts,
motorcycle and cycle helmets, stricter control over driving after
drinking alcohol, and clothing to make cyclists and runners
more visible have all proved helpful in preventing road accidents, yet road accidents are still responsible for more head
injuries than any other source.
Accidents at work account for a significant number of head
injuries, despite the greater use of protective headgear. Sport,
especially boxing and horse riding, constitutes a further source
for head injury, prevented to some extent by the increasing use
of protective headgear. Accidents around the home account for
an unfortunate number of head injuries, especially in young
children who are unable to take proper precautions with open
windows, ladders, stairs and bunk-beds. Child abuse has to be
remembered as a possible cause of head injury.
It is a sad fact that head injury, trivial and severe, affects
young people in significant numbers. Approximately 50% of
patients admitted to hospital on account of head injury in the
UK are under the age of 20 years. Accidents in the home, sports
accidents, and accidents involving motorcycles, cars and alcohol, account for this emphasis on youth.
The outcome from head trauma depends upon the age
and pre-existing health of the patient at the time of injury,
and upon the type and severity of the primary injury to the brain.
In the care of the individual patient, there is little that
the doctor can do about these factors. They have had their influence by the time the patient is delivered into his hands. Outcome
does also depend, however, upon minimizing the harm done by
secondary insults to the brain after the injury. Prevention of
secondary brain injury is the direct responsibility of those caring
for the patient. It is one of the main messages of this chapter.
55
56
The effect in pathological terms

Primary brain injury
The brain is an organ of relatively soft consistency contained in
a rigid, compartmentalized, unyielding box which has a rough
and irregular bottom. Sudden acceleration, deceleration or rotation certainly allow movement of the brain within the skull. If
sudden and massive enough, such movement will cause tearing
of nerve fibres and petechial haemorrhages within the white
matter, and contusions and lacerations of the cortex, especially
over the base of the brain.
If the trauma has been severe, the diffuse damage to the brain
described above may cause generalized brain swelling, just like
the swelling that occurs with injury to any other organ. The
brain, however, is contained within a rigid box, unlike other organs of the body. Diffuse brain swelling may result in the brain
becoming too large in volume for the space allocated to it. As
discussed in Chapter 3 (p. 42), this may lead to tentorial herniation, midbrain compression, impaction of the lower medulla
and cerebellar hemispheres in the foramen magnum, secondary
brainstem pathology and death (Fig. 4.1).
Severe rotational head injuries may cause primary brainstem
injury, which may itself be fatal.
Secondary brain injury

In the circumstances of head injury, the diffusely damaged
brain is extremely vulnerable to four other insults, all of which
produce further brain swelling (Fig. 4.1). By causing further
swelling, each of the four insults listed below tend to encourage
the downward cascade towards brainstem failure and death:
1. Arterial hypotension from blood loss at the time of injury,
possibly from the scalp but more probably from an associated
injury elsewhere in the body. Hypotension and hypoxia, in
isolation but especially in combination, will cause hypoxic
ischaemic brain damage with swelling.
2. Arterial hypoxia because of airway obstruction, associated
chest injury or an epileptic fit.
3. Infection head injuries in which skull fracture has occurred may allow organisms to enter the skull via an open
wound, or from the ear or nose. Infection causes inflammatory
oedema.
4. Intracranial haematoma the force of injury may have torn a
blood vessel inside the skull (either in the brain substance or in
the meninges) so that a haematoma forms. This causes further
compression of the brain by taking up volume within the rigid
skull.
CHAPTER 4
HEAD INJURY
57
Primary injury
Mild
Moderate
Severe
Lethal
Death
Minimal
diffuse
damage
Sufficient
diffuse
damage
to cause
generalized
brain swelling
Severe
diffuse
damage
and brain
swelling,
tentorial
herniation
and coning
Brainstem
failure
secondary
to herniation
and coning
Hypotension
Hypoxia
Infection
Haematoma
The four main additional insults, any of which may
produce further brain swelling, and push the
patient in the direction of secondary brainstem
damage and death
Fig. 4.1 A summary of the pathological effects that may occur as a consequence of head injury.
58
The effect in clinical terms

The clinical instrument that is used to monitor the effects of
head injury on patients requiring admission to hospital is the
neurological observation chart created by competent, trained
nursing staff.
This chart includes the patients responsiveness using the
Glasgow Coma Scale (GCS), recording the patients best eye,
verbal and motor responses, together with his vital signs of
pupil size and reactivity, blood pressure, pulse, respiratory rate
and temperature. The observations are made at intervals appropriate to the patients clinical condition every 15 minutes in
critically ill patients.
The Glasgow Coma Scale is described and illustrated in
Chapter 11 (p. 184, Fig. 11.3). The changes in the charted observations of best eye, verbal and motor responses are used to
signal changes in the patients brain condition with great sensitivity. The vital signs of pupil size and reactivity, blood pressure,
pulse, respiratory rate and temperature are, essentially, indicators of brainstem function.
Figure 4.2 shows the clinical states that accompany mild,
moderate and severe degrees of diffuse cerebral damage after
head injury. The adverse influence of hypotension, hypoxia, infection and intracranial haematoma will become apparent in
the patients chart observations, possibly accompanied by an
epileptic fit. (An epileptic fit may be the cause or effect of a deteriorating intracranial situation, undesirable because of the
associated anoxia and effects on intracranial pressure.) The appearance of abnormal brainstem signs indicates that the additional insult is adversely affecting the brain very seriously.
Figure 4.2 also shows the clinical clues, and definite signs,
that confirm the presence of one or more of the four additional
insults. Ideally, the clues should make the doctor so alert to the
possibility of the insult becoming operational that the problem
is rectified before the patient starts to decline significantly in his
Glasgow Coma Scale observations, and certainly before evidence of impaired brainstem function appears. This is what is
meant by the prevention of secondary brain injury.
CHAPTER 4
HEAD INJURY
59
Primary injury
Mild
Concussion,
or concussion
plus some
retrograde
and posttraumatic
amnesia
Moderate
Persistent
coma after
the accident
with fairly
good scores
on the
Glasgow
Coma Scale,
and no signs
of brainstem
malfunction
Severe
Persistent coma
after the
accident, poor
scores on the
Glasgow Coma
Scale, and
evidence of
failing brainstem
function

The four additional insults will show their adverse influence
on the brain by :
declining performance in Glasgow Coma Scale observations
an epileptic fit
impaired brainstem function observations
Hypotension
Hypoxia
Infection
Haematoma
Clinical clues
Large scalp laceration
i.e. external blood loss
Associated major
injury to chest,
abdomen, pelvis,
limbs, i.e. external
and internal blood
loss
History that the
patient was propped
upright after injury
with known blood
loss and probable
hypotension
Patient found face

down, unconscious
Upper airways
obstruction whilst
unconscious
Severe associated
facial injury
Aspiration of blood
or vomit into
trachea
Prolonged epileptic
fit
Associated injury to
chest wall or lungs
Respiratory
depression by
alcohol or drugs
Open scalp wound

over skull fracture
Leakage of CSF from
scalp wound, nose
or ear
Inadequate inspection,
cleaning, or
debridement of
open scalp wound
over fracture site
Skull fracture found
on CT scan in
region of wound,
nose or ear
Intracranial air seen
on CT scan
Factors known to be
associated with the
development of an
intracranial
haematoma, whether
extradural, subdural
or intracerebral :
Skull fracture
Impaired conscious
level (even
disorientation) i.e.
a fully orientated
patient with no
skull fracture is
very unlikely to
develop a
haematoma
Noisy obstructed
breathing
Abnormal chest
movement
Abnormal respiratory
rate
Abnormal chest
X-ray
Abnormal blood
gases
Purulent discharge
from scalp wound
Proved infection
of CSF
CT scan
Clinical evidence
Development of shock
Low blood pressure
Rapid pulse
Sweating
Fig. 4.2 A summary of the clinical effects of head injury.
60
Management
The first question, near or at the site of the head injury, is
whether there is any indication for assessment in the hospital
emergency department.
Indications for hospital assessment after head injury

Any loss of consciousness, amnesia or fall in the Glasgow
Coma Scale at any time.
Any focal neurological symptom or sign.
Suspicion of skull fracture or penetrating injury.
Seizure, vomiting or persistent headache.
Current drug or alcohol intoxication (making assessment
unreliable).
High-energy head injury (e.g. pedestrian struck by car;
thrown from moving vehicle; fall downstairs; fall of 1 metre
or more onto head).
Coagulopathy (history of bleeding or clotting disorder or
anticoagulated).
Previous neurosurgery.
Suspicion of non-accidental injury.
Age 65 years or more.
Once in hospital, patients may require management in the
emergency department, in the trauma ward of the district general hospital, or in a neurosurgical centre (Fig. 4.3). In all these
places, and during transfer from one to another, management is
directed towards prevention and treatment of the four serious
insults causing secondary brain injury (see Fig. 4.2).
In the emergency department the patient must be assessed,
Glasgow Coma Scale observations initiated (see p. 184), and if
necessary the patient must be resuscitated. Then it has to be
established whether there is any indication for a CT brain
scan and/or neurosurgical advice.
Indications for CT brain scan after head injury
GCS less than 13 at any time, or 1314 at 2 hours after injury.

Suspected skull fracture or penetrating head injury (see box).
Post-traumatic epileptic seizure.
Focal neurological deficit.
More than one episode of vomiting (except perhaps in
children).
Amnesia for more than 30 minutes of events prior to impact.
Any loss of consciousness or amnesia if also:
aged 65 years or older;
coagulopathy;
high-energy head injury.
CHAPTER 4
A Airway
Clear airway, with cervical
spine control until cervical
injury is confidently excluded
B Breathing
Assess ventilation and chest
movement. Arterial blood
gases
C Circulation
Assess likelihood of blood
loss. Monitor BP and P
frequently. Establish IV line
D Dysfunction of CNS
Assess by Glasgow Coma
Scale at frequent intervals
E Exposure
Identify all injuries, head to
toe, front and back
Reasons to suspect a skull

fracture or penetrating
head injury
Clear fluid (CSF) running
from nose
Blood or clear fluid running
from ear(s)
Bruising around eye(s) with
no eye trauma (panda eyes)
Bruising behind ear(s)
(Battles sign)
New unilateral deafness
Significant visible scalp or
skull wound
HEAD INJURY
61
The patient is first seen in hospital emergency department or

general practice surgery
Trivial
Mild
Moderate
Moderate
Severe
Admit for observation and

care in a trauma ward with
experience of head injuries, in
a district general hospital
CT scan and
neurosurgical advice
Admit for highly specialized

observation and care in a
neurosurgical ward with
intensive care facilities
Home
Home
Home
(either direct, via district
general hospital or
rehabilitation unit)
Fig. 4.3 A logistic plan showing the management of patients with

head injury.
Reasons to talk to a neurosurgeon

New and potentially significant abnormality on CT brain
scan.
Persisting coma (GCS of 8 or less) after initial resuscitation.
Unexplained confusion (for more than 4 hours).
Falling GCS (especially falling motor score).
Progressive focal neurological deficit.
Epileptic seizure without full recovery.
Penetrating injury.
CSF leak.
Whether in the emergency department, the CT scan suite,
the trauma ward of the district general hospital, the ambulance
or the neurosurgical centre, the same vigilant care of the
patient with respect to GCS observations, blood pressure,
oxygenation and avoidance of infection must be maintained.
62
It is important to:
continue regular competent neurological observations at
intervals appropriate to the patients condition (half-hourly,
hourly, 2-hourly, 4-hourly);
remember the head injury may be non-accidental, especially
if there is any uncertainty about its mechanism (see box);
remember that there are pharmacological dangers. If the
patient smells of alcohol, the doctor may recognize that
this could be a contributory factor towards the patients
depressed state of consciousness, but he will be unwise
to attribute the whole clinical picture to alcohol if a head
injury is present, especially if a skull fracture is present.
Do not complicate the state of consciousness, or the assessment of it, by the use of strong, CNS depressant, analgesics;
by the overuse of intravenous, CNS depressant, drugs to
control an individual epileptic fit; by permitting any procedures requiring general anaesthesia, which can possibly
wait for 2 or 3 days; or by the use of mydriatic drugs to look at
the fundi;
continue to anticipate, prevent, detect early, and treat energetically any of the four serious insults which may further
damage the brain and cause further brain swelling (see Fig.
4.2, p. 59).
If the patient requires admission to the neurosurgical centre
great attention should be paid to the maintenance of normal
intracranial pressure and perfusion of the brain. This often
involves the use of intracranial pressure monitors, intermittent
intravenous administration of mannitol and neurosurgery
(e.g. to evacuate an extradural haematoma). The routine care of
the unconscious patient (see Chapter 11, p. 187) is established.
After-care
In all but the most trivial head injuries, the patient will benefit
from some after-care. This may not amount to more than simple explanation and reassurance regarding a period of amnesia,
headaches, uncertainty about a skull fracture, timing of return
to work, etc. Without the opportunity to discuss such matters,
unnecessary apprehensions may persist in the mind of the
patient and his family.
At the other end of the scale, somebody recovering from a
major head injury (often associated with other injuries) may
need a great deal of further medical and paramedical care for
months after the head injury. This may mean a considerable
period in hospital or in a rehabilitation unit whilst recovery
from the intellectual, psychological, neurological and orthopaedic deficits gradually occurs.
CHAPTER 4
Head injury may result from:

stroke
cerebral haemorrhage
epilepsy
cardiac dysrhythmia
alcoholic intoxication
non-accidental injury to
children
HEAD INJURY
Initial features:
level of consciousness
skull fracture
focal neurological signs
Secondary features:
epilepsy
intracranial haematoma
meningitis
Duration:
of coma
of post-traumatic amnesia
of stay in hospital
Persisting deficits:
intellectual
psychological
focal neurological
63
The consequences
How severe a head injury was it, doctor?
Several factors, shown in the adjacent margin, must be borne in
mind when formulating a reply to this enquiry.
With regard to the duration of coma and post-traumatic amnesia, confusion may arise. The duration of post-traumatic amnesia refers to the period after the accident until the time that the
patient regains ongoing memory. The patient often describes
the latter as the time he woke up after the accident. He means the
time he recovered his memory, not the time he recovered consciousness. The time he says he woke up is often long after the
time that observers have noted return of consciousness (eyes
open and paying attention, speaking and using his limbs
purposefully).
The duration of hospital stay must also be clarified. If there
was associated (orthopaedic) injury, the patient may have been
hospitalized long after the time for discharge purely on head
injury grounds.
Post-concussion syndrome
Headache
Dizziness
Impaired concentration
Impaired memory
Fatigue
Anxiety
Depression
Irritability
Indecisiveness
Impaired self-confidence
Lack of drive
Impaired libido
This usually follows minor concussive head injury or less, with

post-traumatic amnesic periods lasting a few minutes, rather
than major head injury. It consists of a remarkably stereotyped
set of symptoms, as shown alongside, unaccompanied by abnormal neurological signs.
Controversy exists as to the cause of the post-concussion
syndrome. On the one hand, it is accepted that concussive
head injury can produce diffuse minor brain damage. (If
repeated, this may accumulate to the punch-drunk state,
known as post-traumatic encephalopathy, seen in some boxers
towards the end of their careers.) On the other hand, the
syndrome can be seen in some patients suffering head injuries
at work, where the evidence of concussion or amnesia is very
minimal and where litigation and financial compensation are
paramount.
The syndrome can be very disabling for months, even years,
after a minor head injury. Despite our lack of understanding of
its precise nature, the syndrome has to be recognized as a definite entity responsible for considerable morbidity after head injury in some patients.
64
CHAPTER 4
Post-traumatic epilepsy
Patients surviving head injury may have developed an
epileptogenic scar in the brain, which may subsequently give
rise to focal or secondarily generalized epileptic attacks. Posttraumatic epilepsy shows its presence within a year of the
accident in about 50% of patients who are going to develop this
late complication of their head injury. In the rest, it may not
occur for several years.
There are certain features of the head injury which make posttraumatic epilepsy more likely:
post-traumatic amnesia lasting more than 24 hours;
focal neurological signs during the week after the head
injury;
epilepsy during the week after the head injury;
depressed skull fracture;
dural tear;
intracranial haematoma.
These risk factors enable fairly accurate prediction of the risk
of epilepsy in a patient after head injury, and are valuable when
advising patients about prophylactic anticonvulsants and
driving.
Chronic subdural haematoma

In elderly patients who have suffered trivial head injury, sometimes so trivial that it is not clearly remembered, blood may
start to collect in the subdural space. This is not sudden, severe
arterial bleeding, but a process that evolves over several weeks,
a gradual accumulation and liquefaction of blood. The blood
accumulates over the convexity of the brain, with gradual elevation of intracranial pressure, shift of midline structures and
eventual tentorial herniation and coning.
The clinical picture is of subacute fluctuating drowsiness and
confusion, often associated with headache, in which focal neurological signs appear late. Any elderly patient presenting in
this way is a candidate for a chronic subdural haematoma, with
or without a history of head injury. Chronic alcoholics and patients on anticoagulants have an increased risk of chronic subdural haematoma.
Chronic subdural haematomas may occur bilaterally, and
may be hard to visualize on conventional CT scans if the altered
blood is isodense with brain tissue. They are treated by evacuation of the blood through burrholes, with good results.
Bilateral subdural haematomas.
HEAD INJURY
Outcome from severe

head injury
50% mortality
Disability common in
survivors
Often young people
65
Outcome from severe head injury

Mortality from severe head injury (coma lasting more than 6
hours) is of the order of 50%. Those who survive are likely to
have deficits in some or all of the following areas, depending on
which parts of the brain have been most damaged:
intellectual function;
mood, behaviour, personality;
speech and communication;
vision;
motor and sensory function in the limbs;
post-traumatic epilepsy.
Most of a patients recovery will have occurred within 6
months of the injury, though further slower improvement may
occur in the next 1218 months.
Patients displaying deficits in all areas constitute what the
general public recognize as a brain-damaged person, often unable to live independently. Commonly, the patients are young at
the time of their accident, and many years of life may lie ahead
of them. In general, provision for such patients is inadequate in
terms of chronic young sick units, where their ongoing care may
be successfully managed in some sort of shared care system
with the patients relatives.
Compensation and medico-legal aspects

Because road traffic accidents and accidents at work cause a
large percentage of head injuries, compensation for suffering,
disability, loss of earnings, restriction in recreational pursuits,
etc. is very commonly sought in the months and years after the
accident. This occurs after both minor and major injuries, and it
is unusual for a case to be settled before 2 years have elapsed
after the accident.
Though this activity is entirely reasonable, it is unsettling for
the patient and his family. It tends to perpetuate the accident
and its effects in their minds longer than would otherwise be the
case.
66
CHAPTER 4
CASE HISTORIES
Case 1
A 12-year-old boy has been involved in an accident
whilst riding his bicycle.All the details are not clear,
but the accident happened at traffic lights, the boy was
not wearing a helmet, he was struck by a car and he
lost consciousness transiently though he was
subsequently able to talk reasonably coherently. He
was not able to walk into the ambulance which
brought him to the emergency department.
On examination he is orientated in person but not
in time or place.There is some bruising and swelling
above his left ear, though no scalp laceration.There are
no focal neurological signs.There are no signs of injury
to any other part of his body.
a. What management would you arrange?
Ninety minutes later, some 3 hours after the accident,
the neurological observations start to show
deterioration. He will only open his eyes to strong
painful stimuli and his speech is reduced to mumbled

incomprehensible sounds. His limb responses are
variable but he can localize painful stimuli with each
hand.Vital functions and pupil reactions remain
normal.
b. What is your management now?
Case 2
A dishevelled middle-aged man is brought to the
emergency department by ambulance. He has been
found unconscious on the pavement outside a public
house. He smells strongly of alcohol. He is bleeding
from a large left-sided scalp wound. He is deeply
unconscious with a Glasgow Coma Scale of 8 (E2 V2
M4) but no focal neurological deficit.
a. What are the possible reasons for his state?
(For answers, see pp. 2567.)
CHAPTER 5
Parkinsonism,
involuntary movements
and ataxia
Introduction
An elderly man comes to see you to report a 6-month history
of progressive dragging of his left leg and difficulty using his
left hand for fiddly tasks. He is worried that he has a brain tumour. You find no weakness or spasticity but note that he stoops
and shuffles a little when he walks and cannot wiggle the left
fingers or open and close the left hand rapidly. You are able to
tell him that he has the entirely happier diagnosis of Parkinsons
disease.
What this case emphasizes is the principle outlined in
Chapter 1 that normal, smooth, well-coordinated movements
rely not just on the integrity of the primary motor pathway
(upper motor neuronelower motor neuroneneuromuscular
junctionmuscle). They also require normal inputs to this
pathway from the basal ganglia, cerebellum and sensory pathways, as shown in Fig. 5.1. It also illustrates the fact that many
of the disorders of movement and coordination that we will
discuss in this chapter can be diagnosed by history-taking and
examination, without sophisticated investigations.
Upper motor neurone
Lower motor neurone
Muscle
Basal ganglia
Fig. 5.1 Diagram to show the basic components of the nervous system required for normal movement.
67
68
CHAPTER 5
Tremor
Mainly at rest (often

suppressed by movement)
Mainly on sustained posture

(e.g. outstretched hands)
Mainly on movement (but

also with sustained posture)
Parkinson's disease
Physiological tremor
Anxiety
Thyrotoxicosis
Essential tremor
Drugs (including salbutamol,
lithium, valproate, etc.)
Cerebellar disease
Fig. 5.2 Classification of tremor.
Tremor
Tremor is a rhythmic (or fairly rhythmic) to and fro movement
of a part of the body. It can range in severity from something that
the patient can feel but you cannot see, through to something
that causes wild involuntary movements that prevent any
useful limb function. A classification of the common forms of
tremor is shown in Fig. 5.2.
The important differentiating feature of Parkinsons disease
tremor is the fact that it is most evident at rest. It is reduced
or abolished by voluntary movement. Other core features of
Parkinsons disease, such as bradykinesia and rigidity, are usually also present to some degree and point to the correct diagnosis.
Patients with disease in the cerebellum and its brainstem
connections commonly have clumsy, uncoordinated movements of their limbs. The incoordination is present throughout
the proximal and distal limb muscles, leading to largeamplitude, chaotic shaking of the limb. This is most marked on
attempted movement, and is therefore termed kinetic tremor,
although it is almost always also present on sustained posture.
Much the most common cause of severe kinetic tremor is
multiple sclerosis.
Patients with cerebellar degenerations may have a milder
kinetic tremor, where the involuntary of oscillations appear as
the limb approaches its intended target, a phenomenon sometimes referred to as intention tremor.
In both situations, there are likely to be additional signs of
cerebellar or brainstem dysfunction, such as dysarthria, gait
ataxia, limb ataxia with past pointing and dysdiadochokinesia,
and nystagmus.
PARKINSONISM, INVOLUNTARY MOVEMENTS AND ATAXIA
Examining for tremor

Look for tremor at rest:
hands in lap
patient distracted (Close
your eyes and count
backwards)
Look for postural tremor:
arms outstretched
hands slowly brought in
towards the face
Look for kinetic tremor:
Touch my finger, then your
chin
If you cannot see the tremor:
listen to the affect muscles
with a stethoscope
69
We all have some degree of tremor on sustained posture,

termed physiological tremor. This is faster than parkinsonian or
cerebellar tremor, absent at rest, and most evident in the hands.
Most of the predominantly postural tremors are due to an exaggeration of this phenomenon.
Postural tremors are made more obvious when circulating
catecholamines are increased, for example by anxiety, and by
sympathomimetic drugs like salbutamol. Conversely, they are
suppressed to some extent by beta-adrenergic blockade and
sometimes by alcohol. The mechanisms of these effects are not
understood. They are often exploited by people who require a
steady hand, such as surgeons, snooker players or musicians,
who may take propranolol or alcohol on important occasions.
Reliance on alcohol to treat tremor can lead to dependency and
liver disease.
The most disabling postural tremor is familial essential
tremor, which tends to be dominantly inherited. A more jerky,
asymmetrical variant of essential tremor is seen in patients with
a personal or family history of dystonia, and is referred to as
dystonic tremor; it is less responsive to beta-blockers or alcohol.
Parkinsons disease
Parkinsons disease is rare before the age of 40, but becomes increasing common with age, and affects 12% of people over 65
years old. Several genes causing familial Parkinsons disease
have been identified, and other genes probably contribute to the
risk of developing the disease in those with no family history.
Environmental factors that increase the risk of developing
Parkinsons disease include working with pesticides; drinking
coffee and smoking cigarettes both reduce the risk.
The symptoms and signs of Parkinsons disease reflect a
highly selective pattern of degeneration in the brain. The worst
damage occurs in the dopamine-producing neurones of the
substantia nigra, and this accounts for many of the abnormalities of movement, referred to as parkinsonism. These neurones
project to the corpus striatum via the nigrostriatal pathway. The
consequence of loss of neurones in the substantia nigra is
dopamine deficiency in the corpus striatum. This may be unilateral, asymmetrical or symmetrical.
The noradrenaline- and 5HT-producing neurones in the
brainstem are also affected, and this may explain the high incidence of depression in Parkinsons disease. The neurones that
deliver acetylcholine to the cerebral cortex are affected as well;
this, together with involvement of the cortial neurones themselves, contributes to cognitive symptoms. In all these locations
the neurones degenerate in a characteristic way, forming
clumps of protein called Lewy bodies.
70
The speed and extent of this process vary considerably

between individuals. Some patients become disabled by impaired movement and then dementia within a few years. Others
have a barely progressive tremor that may require little or
no treatment for a decade or more. Most patients fall between
these extremes, with symptoms that become slightly more
troublesome (and require increasingly complex treatment) with
each passing year. With treatment, people with Parkinsons
disease now have a normal life expectancy, unless they go on
to develop dementia. This is much harder to treat and still
shortens life.
Features of Parkinsons disease

There is often a delay in diagnosing Parkinsons disease. The
early symptoms of Parkinsons disease can be vague (see box).
The signs are usually asymmetrical, leading to confusion with
strokes and tumours.
The core features of Parkinsons disease are bradykinesia and
rigidity. Bradykinesia is a lack of spontaneous movements
(often most noticeable as reduced blinking, lack of facial expression and reduced arm swing when walking), and a slowing of
movements, especially fine repetitive ones. Rigidity is an increase in tone throughout the full range of movement, unlike
spasticity which builds up and then gives way. Rigidity may
have a constant resistance (like bending a lead pipe) or a juddering feel (like turning a cogwheel against a ratchet).
The rest tremor of Parkinsons disease (in the limbs, the jaw
or the lightly closed eyes) is highly characteristic, but 50% of
patients do not have it at presentation and 20% never get it.
Gait disturbance is usually mild in the first few years. Patients
may then develop difficulty in starting to walk or in stopping
(festination) or may abruptly freeze in doorways or crowds.
Eventually most patients have falls. The falling is partly a
consequence of slow, stiff muscles or freezing, but partly also
due to a failure of more complex postural righting reflexes
or orthostatic hypotension. It has a very bad effect on quality
of life.
Non-motor symptoms are often equally distressing, especially depression, dementia and disturbed sleep. Depression has
a prevalence of 30% and should be actively sought and
treated. Dementia is unusual before 70 years but affects many
patients thereafter. Losing the thread of sentences and memory
loss are followed by periods of confusion, often accompanied
by visual hallucinations (see p. 230). Sleep may be disrupted for
several reasons. The most worrying is REM sleep behavioural
disturbance, in which patients act out their vivid dreams.
CHAPTER 5
Early symptoms of
Parkinsons disease
Commonly non-specific at
first:
aches and pains
disturbed sleep
anxiety and depression
slower dressing
slower walking
Later more specific:
tremor
difficulty turning in bed
stooping or shuffling
softer speech
spidery handwriting
Main signs of
Parkinsons disease
Rigidity
Bradykinesia
Tremor
Gait disturbance
Stooped posture
Causes of reduced
facial expression
Parkinsonism
Depression
Severe facial weakness
Severe hypothyroidism
71
Management of patients with Parkinsons disease

The management of patients with Parkinsons disease requires
patience and persistence. The patients concerns and expectations may be very different from yours. It may take time to reach
a shared understanding and objectives. Specialist nurses and
patient groups like the Parkinsons Disease Society are a huge
help with this. Non-drug treatments should be sought where
possible, and advice from speech therapists, physiotherapists,
occupational therapists and dieticians is often required.
Drug schedules should be altered gradually. Side-effects
from the drugs are common, making regular consultations
important. As the illness evolves, drug schedules can become
quite complicated, needing clear explanation and written
confirmation.
The mainstay of drug treatment is to boost dopaminergic
activity in the nigrostriatal pathway, either by giving levodopa
which can be turned into dopamine within the remaining neurones in the substantia nigra or by giving dopamine agonists
which mimic the effect of dopamine in the striatum (Fig. 5.3).
Less potent benefits can be obtained from drugs which
inhibit the metabolism of dopamine by monoamine oxidase
type B and catechol-O-methyl transferase, and from drugs that
modify other neurotransmitters in the striatum such as amantadine and anticholinergics.
Levodopa treatment was developed in the 1960s and remains
the most powerful treatment for Parkinsons disease. Levodopa
Other drugs benefit Parkinson's disease by inhibiting
the unopposed action of other neurotransmitters in the
corpus striatum, e.g. anticholinergics, glutamate antagonists
Corpus striatum
L-dopa is metabolized to dopamine and
thereby elevates brain dopamine levels,
without similar elevation elsewhere
when given with peripheral inhibitors
(such as dopa decarboxylase and COMT
inhibitors)
NIGROSTRIATAL
PATHWAY
Dopamine
Dopamine agonists simulate

dopamine action directly in
the brain
MAO type B inhibitors inhibit
the metabolism of released dopamine
thereby prolonging its action
Substantia nigra
Clearly drugs which either interfere with the biosynthesis

of dopamine (tetrabenazine) or which block
dopamine receptors (phenothiazines, metoclopramide)
can induce parkinsonism or aggravate it
Fig. 5.3 Diagram to show the substantia nigra in the midbrain, and the nigrostriatal pathway.
72
is absorbed from the gut, crosses the bloodbrain barrier and is

converted into dopamine by the enzyme dopa-decarboxylase.
It is given with a dopa-decarboxylase inhibitor which does not
cross into the brain. This prevents the levodopa from being converted into dopamine in the body (where it would cause nausea
and vomiting) without interfering with the production of
dopamine in the brain.
Most patients initially respond brilliantly to levodopa
therapy. There are three subsequent problems:
Wearing off: the response becomes shorter and less marked.
Dyskinesia: each dose produces involuntary chorea
movements.
Onoff effect: the transition between lack of response (off) and
response (on) becomes rapid.
The oral dopamine agonists are not as potent as levodopa in
alleviating parkinsonism, but are less prone to cause dyskinesia
and fluctuation. Young and mildly affected patients are sometimes started therefore on a dopamine agonist initially, in the
hope of postponing levodopa therapy and its complications for
a while. Alternatively the agonist drugs can be introduced later,
to reduce the patients reliance on levodopa after these complications have begun. It is not clear whether one strategy is better
than the other. Dopamine agonists often cause their own
adverse effects in older patients, especially hallucinations.
Selegiline and entacapone prolong the duration of action of
levodopa, and have a limited role in smoothing fluctuations.
Amantadine can be very helpful in suppressing dyskinesia.
There are two strategies for very refractory fluctuation or
dyskinesia:
Apomorphine infusion: this parenteral dopamine agonist can
be infused subcutaneously to achieve stable control. This is
tricky and requires specialist medical and nursing input.
Surgical treatments: these are aimed at inhibiting overactive
parts of the basal ganglia circuitry, either with a stereotactic
lesion or by implanting an electrode with an impulse generator. Targets for these operations include the internal globus
pallidus within the striatum (to reduce dyskinesia) and the
tiny subthalamic nucleus (to reduce the parkinsonism itself).
Procedures to graft the striatum with fetal substantia nigra
cells or stem cells remain experimental.
Anticholinergic drugs are useful for treating tremor, but have
much less effect on the other aspects of parkinsonism, and often
seriously aggravate memory problems and hallucinations.
Conversely, memory problems and hallucinations often respond to cholinergic therapy with drugs like donepezil and rivastigmine. These inhibit the cholinesterase enzyme that beaks
down acetylcholine. They do not aggravate the parkinsonism.
An alternative way of suppressing hallucinations (but not
CHAPTER 5
Drugs for Parkinsons disease

Levodopa with dopa
decarboxylase inhibitor:
cobeneldopa (Madopar)
cocareldopa (Sinemet)
Dopamine agonists:
cabergoline
pergolide
ropinirole
premipexole
bromocriptine
apomorphine
MAO-B inhibitor:
selegiline
COMT inhibitor:
entacapone
Glutamate antagonist:
amantadine
Anticholinergics
Cholinergics:
rivastigmine
donepezil
Atypical neuroleptics:
quetiapine
clozapine
Antidepressants
The main side-effects or

overdose phenomena when
enhancing the failing
nigrostriatal pathway
Dopamine in the gut:
nausea
vomiting
Dopamine in the striatum:
dyskinesia
Dopamine elsewhere in the
brain:
postural hypotension
confusion/hallucinations
73
memory problems) is to use atypical neuroleptic drugs such as

quetiapine or clozapine. Conventional neuroleptics (such as
haloperidol or chlorpromazine) cannot be used in Parkinsons
disease because they exacerbate parkinsonism, occasionally to
a fatal degree.
Other causes of parkinsonism

The substantia nigra, nigrostriatal pathway and corpus
striatum may be involved in
diffuse cerebral pathology:
viral encephalitis
cerebrovascular disease
severe head injury
Parkinsonian signs may feature
amongst other neurological
deficits subsequently
There are rarer patterns of
neuronal loss from the CNS
which involve nigral and striatal
neurones:
progressive supranuclear palsy
(SteeleRichardson syndrome)
multisystem CNS degeneration
with autonomic failure
(ShyDrager syndrome)
Parkinsonism can result from other problems in the nigrostriatal pathway (Fig. 5.4). The commonest is drug-induced parkinsonism, due to drugs which block striatal dopamine receptors,
such as antipsychotics or antiemetics. Vascular parkinsonism
is due to multiple small infarcts in the basal ganglia and
often causes more trouble with walking than the upper limbs.
Other neurodegenerative diseases can cause parkinsonism together with additional features. These include multiple system
atrophy where there may be any combination of parkinsonism,
cerebellar ataxia and autonomic failure, and progressive
supranuclear palsy where there is usually a severe disturbance of
vertical eye movements. Clinical pointers to these other causes
include:
symmetrical bradykinesia and rigidity, without tremor;
lack of response to levodopa therapy;
poor balance and falls in the first 2 years of the illness.
Biosynthetic
pathway to form
dopamine
Drugs which interfere with

dopamine synthesis and release
by the pre-synaptic terminal of
the nigrostriatal axon, A, or
which block dopamine uptake by
the receptors on the surface of
the corpus striatal neurone, B,
may induce parkinsonism
A
e.g.
reserpine
tetrabenazine
Fig. 5.4 Parkinsonism other than idiopathic Parkinsons disease.
B
e.g.
chlorpromazine
haloperidol
metoclopramide
74
CHAPTER 5
Involuntary movements
The main categories of involuntary movements are tremor,
chorea and dystonia, tics, and myoclonus.
Chorea and dystonia

Chorea refers to fidgety momentary movements which flit randomly around the body. They resemble fragments of purposeful acts or gestures. If you find yourself responding to the
movements then you are likely to be dealing with chorea. Dystonia refers to slower involuntary movements that give rise to
repetitive twisting or fixed abnormal postures.
There is an overlap between the two movement disorders,
in both their clinical manifestations and their causes. Both
are due to dysfunction in the basal ganglia. Many patients
will have mainly chorea with a little dystonia, or vice versa.
The term athetosis refers to slow writhing movements that
are halfway between chorea and dystonia, but is now largely
reserved for a form of cerebral palsy where these movements
predominate.
Hemichorea and hemiballismus

If chorea affects just one side of the body it is termed hemichorea, or hemiballismus if the movements are wild and large
scale. This is usually due to a small infarct in the subthalamic
nucleus on the opposite side of the brain. Although the movements usually subside within weeks, they can be exhausting
and can be suppressed with dopamine-blocking drugs (like
haloperidol).
Huntingtons disease
The most serious cause of chorea is Huntingtons disease. This
is inherited as an autosomal dominant disorder, and is one of
several neurodegenerative diseases that are due to an expansion in a run of repeated CAG nucleotide triplets within a gene.
It typically starts with chorea, often accompanied by impulsive or erratic behaviour, and progresses relentlessly to cause
dystonia and parkinsonism together with dementia. Depression and suicide are common, but most patients ultimately
succumb to the complications of immobility. People who are
at risk of developing Huntingtons disease because of their
family history can, if they wish, undergo predictive genetic
testing. This process is surrounded by emotional issues and
requires very careful genetic counselling.
Causes of chorea
Drugs:
levodopa in Parkinsons
patients
oral contraceptive pill
many psychiatric drugs
Vascular disease of the basal
ganglia:
atheroma
systemic lupus
erythematosus
Degenerative diseases:
Huntingtons disease
Post-infectious:
Sydenhams chorea
Other causes:
thyrotoxicosis
Trinucleotide repeat diseases

Many genes have stretches of
DNA where the same three
nucleotides are repeated over
and over again (e.g.
CAGCAGCAG etc.). Several
neurological diseases are due
to expansions of these
sequences, with an excessive
number of repeats. Examples
include:
Huntingtons disease
several dominantly
inherited cerebellar ataxias
Friedreichs ataxia
one form of motor neurone
disease
myotonic dystrophy
fragile X mental retardation
These expansions have
unusual features. The
expansions are unstable and
can increase in size from one
generation to the next, leading
to an earlier age of onset
(termed anticipation). This
tendency may exclusively
affect expansions transmitted
by mothers or fathers. The
ways in which the extra
nucleotides cause disease is a
matter of intense current
research, but may involve
changes in the expression of
the gene and the folding of the
protein product.
75
Sydenhams chorea
This used to be a common sequel to streptococcal infection
in young people, along with rheumatic fever. It is now rare in
developed countries but remains common elsewhere. Other
post-streptococcal movement disorders are described in
Chapter 15 (p. 253).
Drug-induced movement disorders

We have seen already how tremor and parkinsonism can be induced by drugs. Drugs can also cause all the other movement
disorders, especially chorea and dystonia. Drugs which block
dopamine receptors are the main culprit. They can cause acute
dystonia (for example, when intravenous metoclopramide is
given to a young person for nausea), with spasms of the face,
neck and arms and a phenomenon called an oculogyric crisis
where the eyes roll up involuntarily. Long-term use of these
drugs (for example, when neuroleptic drugs are used for depression or schizophrenia) can cause complex involuntary
chewing and grimacing movements in the face called tardive
dyskinesia, which persist after the causative drug is
withdrawn.
Focal dystonia
Dystonia can affect one localized area of the body. Common
forms of focal dystonia include blepharospasm, where there are
repetitive spasms of eye closure that can seriously interfere with
vision; torticollis, where the head pulls painfully to one side and
may also shake; and writers cramp, where the forearm cramps
up and causes the hand to take on a painful twisted posture
when writing is attempted. These conditions can be treated by
weakening the overactive muscles with regular botulinum
toxin injections.
76
Other forms of dystonia

Dystonia can affect larger areas of the body, such as the neck
and one arm, segmental dystonia, the trunk muscles, axial dystonia, or the whole of the body, generalized dystonia. Generalized
dystonia usually begins in childhood and often has a genetic
basis. It can be treated to a very limited extent with drugs.
There is increasing interest in treatment with brain stimulator
operations.
Wilsons disease
This is a very rare metabolic disorder characterized by the accumulation of copper in various organs of the body, especially the
brain, liver and cornea. It is inherited in an autosomal recessive
fashion, and is due to mutations in a gene for ATP-dependent
copper-transporting protein.
It is a disease of children and young adults. In the brain, it
chiefly affects basal ganglia function, giving rise to all sorts
of movement disorders including tremor, chorea, dystonia
and parkinsonism. It can also cause behavioural disturbance,
psychosis or dementia. In the liver it may cause cirrhosis and
failure. In the cornea it is visible (with a slit lamp) peripherally
as a brownish KayserFleischer ring. Looking for this and a
low serum caeruloplasmin level are ways of screening for the
disease.
The importance of Wilsons disease is that it can be treated
with copper-chelating drugs (like penicillamine) if diagnosed
early, when brain and liver changes are reversible.
Tics
Tics are stereotyped movements that can be momentary or more
complex and prolonged. They differ from chorea in that they
can be suppressed for a while by an effort of will. Simple tics,
like blinking or grimacing or shrugging repeatedly, are very
common in children, especially boys aged 710 years. In a small
minority these persist into adult life. A wider range of tics, producing noises as well as movements, is suggestive of Gilles de la
Tourette syndrome.
CHAPTER 5
Gilles de la Tourette syndrome

Georges Gilles de la Tourette
was a nineteenth-century
French neurologist who came
across the disorder while
attempting to classify chorea.
He went on to have a
distinguished career, survived
being shot by the husband of a
patient, and died of
neurosyphilis. Gilles de la
Tourette syndrome begins in
children and teenagers with:
multiple motor tics, with a
gradually evolving
repertoire of movements;
phonic tics, commonly
sniffs and grunts, rarely
repetitive speech (echolalia)
or swearing (coprolalia);
obsessivecompulsive
disorder, such as repeated
checking or complex rituals.
Mild forms are common in the
general population, and very
common in people with
learning disability. The tics
respond to dopamineblocking drugs. The
obsessivecompulsive
disorder (which is often more
disabling) may improve with
selective serotonin reuptake
inhibitors (like fluoxetine) or
behavioural therapy. It is
possible that some cases may
be caused or exacerbated by
autoimmune responses to
streptococcal infection
analogous to Sydenhams
chorea.
77
Myoclonus
Myoclonus produces sudden, shock-like jerks. It is a normal
phenomenon in most children and many adults as they are
falling off to sleep. It also occurs in a wide range of disease
states, and can be due to dysfunction in the cerebral cortex, basal
ganglia, brainstem or spinal cord.
Myoclonus as part of general medicine:
hepatic encephalopathy (liver flap);
renal failure;
carbon dioxide retention.
Myoclonus as part of degenerations of the cerebral cortex:
Alzheimers disease;
Lewy body dementia;
CreutzfeldtJakob disease.
Myoclonus as part of epilepsy:
juvenile myoclonic epilepsy (where there are jerks in the
morning: messy breakfast syndrome);
severe infantile epilepsies.
Myoclonus due to basal ganglia disease:
jerking on attempted movement (action myoclonus) after
anoxia due to cardiorespiratory arrest or carbon monoxide
poisoning.
Myoclonus due to brainstem disease:
exaggerated jerks in response to sudden noise (startle
myoclonus) in rare metabolic and degenerative disorders.
78
CHAPTER 5
Cerebellar ataxia
Figure 5.5 is a grossly oversimplified representation of the
cerebellum. The function of the cerebellum is to coordinate
agonist, antagonist and synergist muscle activity in the performance of learned movements, and to maintain body equilibrium whilst such movements are being executed. Using a
massive amount of input from proprioceptors throughout the
body, from the inner ear and from the cerebral hemispheres, a
complex subconscious computation occurs within the cerebellum. The product of this process largely re-enters the CNS
through the superior peduncle and ensures a smooth and orderly
sequence of muscular contraction, characteristic of voluntary
skilled movement.
In man, the function of the cerebellum is seen at its best in
athletes, sportsmen, gymnasts and ballet dancers, and at its
worst during states of alcoholic intoxication when all the features of cerebellar malfunction appear. A concern of patients
with organic cerebellar disease is that people will think they are
drunk.
Midbrain
Pons
Cerebellum
Medulla
Cerebro-cerebellar input, via

pons and middle cerebellar
peduncle
Outflow from
the cerebellum,
via superior
cerebellar
peduncle
Localization of lesions
From Fig. 5.5 it is clear that patients may show defective cerebellar function if they have lesions in the cerebellum itself, in the
cerebellar peduncles, or in the midbrain, pons or medulla. The
rest of the CNS will lack the benefit of correct cerebellar function
whether the pathology is in the cerebellum itself, or in its incoming and outflowing connections. Localization of the lesion may
be possible on the basis of the clinical signs.
Midline cerebellar lesions predominantly interfere with the
maintenance of body equilibrium, producing gait and stance
ataxia, without too much ataxia of limb movement.
Lesions in the superior cerebellar peduncle, along the course of
one of the chief outflow tracts from the dentate nucleus in the
cerebellum to the red nucleus in the midbrain, classically produce a very marked kinetic tremor, as mentioned at the beginning of this chapter.
Lesions in the midbrain, pons and medulla, which are causing
cerebellar deficits by interfering with inflow or outflow pathways to or from the cerebellum, may also cause other brainstem signs, e.g. cranial nerve palsies, and/or long tract signs
(upper motor neurone or sensory) in the limbs.
Vestibulo-cerebellar
input, via inferior
cerebellar peduncle
Spino-cerebellar input
(proprioception), via inferior
cerebellar peduncle
Fig. 5.5 Highly simplified

diagrammatic representation of
the brainstem and cerebellum as
viewed from the left.
79
Clinical signs of cerebellar dysfunction

The common, important clinical signs of cerebellar dysfunction
are listed below.
Nystagmus.
Dysarthria: the muscles of voice production and speech lack
coordination so that sudden irregular changes in volume and
timing occur, i.e. scanning or staccato speech.
Upper limbs: ataxia and intention tremor, best seen in movement
directed towards a restricted target, e.g. the fingernose test;
dysdiadochokinesia, i.e. slow, inaccurate, rapid alternating
movements.
Lower limbs: ataxia, best seen in the heelkneeshin test.
Gait and stance ataxia, especially if the patient is asked to walk
heel to toe, or to stand still on one leg.
Hypotonia, though a feature of cerebellar lesions, is not very
useful in clinical practice.
Cerebellar representation is ipsilateral, so a left cerebellar hemisphere lesion will produce nystagmus which is of greater
amplitude when the patient looks to the left, ataxia which is
more evident in the left limbs, and a tendency to deviate or fall to
the left when standing or walking.
To date, it has not been possible to improve defective cerebellar function pharmacologically.
Causes of cerebellar malfunction

The common causes of cerebellar malfunction are:
cerebrovascular disease;
multiple sclerosis;
drugs, especially anticonvulsant intoxication;
alcohol, acute intoxication.
Rarer cerebellar lesions include:
posterior fossa tumours;
cerebellar abscess, usually secondary to otitis media;
cerebellar degeneration, either hereditary (e.g. Friedreichs
ataxia and autosomal dominant cerebellar ataxia), alcohol
induced, or paraneoplastic;
ArnoldChiari malformation (the cerebellum and medulla
are unusually low in relation to the foramen magnum);
hypothyroidism.
80
Sensory ataxia
Since proprioception is such an important input to the cerebellum for normal movement, it is not surprising that loss of proprioception may cause ataxia, and that this ataxia may resemble
cerebellar ataxia.
Pronounced loss of touch sensation, particularly in the hands
and feet, seriously interferes with fine manipulative skills in the
hands, and with standing and walking in the case of the feet.
In the presence of such sensory loss, the patient compensates
by using his eyes to monitor movement of the hands or feet. This
may be partially successful. An important clue that a patients
impaired movement is due to sensory loss is that his clumsiness
and unsteadiness are worse in the dark, or at other times when
his eyes are closed, e.g. washing his face, having a shower,
whilst putting clothes over his head in dressing.
Signs of sensory ataxia

In the hands
Pseudoathetosis: the patient is unable to keep his fingers
still in the outstretched position. Because of the lack of feedback on hand and finger position, curious postures develop
in the outstretched fingers and hands when the eyes are
closed.
Clumsiness of finger movement, e.g. when turning over the
pages of a book singly, and when manipulating small objects
in the hands, made much worse by eye closure. Shirt and
pyjama top buttons, which cannot be seen, present more
difficulty than other buttons.
Difficulty in recognizing objects placed in the hands when
the patients eyes are closed, and difficulty in selecting familiar articles from pockets and handbags without the use of the
eyes.
Loss of touch and joint position sense in the fingers.
In the legs
Marked and unequivocal Rombergism. The patient immediately becomes hopelessly unsteady in the standing position
when the eyes are closed.
As the patient walks, he is obviously looking at the ground
and at his feet.
Loss of touch and joint position sense in the feet and toes.
CHAPTER 5
81
Causes of sensory ataxia

These are shown in Fig. 5.6:
Peripheral neuropathy
Spinal cord disease
Cerebral hemisphere lesions
X
X X
X X
X
2
X X
X X
X X
X
X
X
X
Fig. 5.6 Sensory deficits causing

sensory ataxia.
Ataxia in vestibular disease

Again, because vestibular inputs are vital to cerebellar function,
disorders of the vestibular system can produce ataxia, especially of gait. This cause of unsteadiness can usually be recognized by the presence of prominent vestibular symptoms and
signs like vertigo and rotatory nystagmus (see pp. 1268), and
by the absence of other cerebellar, brainstem and sensory signs.
82
CHAPTER 5
CASE HISTORIES
Case 1
A 75-year-old woman notices that she can no longer
deal the cards at her bridge club because her hands
have become clumsy and slow. Her handwriting has
become spidery and small. She cannot roll over in bed.
She shuffles when she walks.
She lives with her husband who is in good health.
She has never smoked. Her parents both lived into
their eighties without anything similar, and her sister is
alive and well. She is on medication for hypertension
and a hiatus hernia.
On examination she walks with a flexed posture,a
shuffling gait and no arm swing.She has moderate
bradykinesia and rigidity in both arms.There is no tremor
or cerebellar deficit.Her eye movements are normal for
her age.Her pulse and blood pressure are normal.
a. What part of the history would you most like to
clarify?
because of a slowly increasing tendency to fall, but

now he topples over if he is jostled in the corridor and
has to stay close to the wall for support. His speech is a
little slurred, especially when he is tired. He has no
headaches or weakness.
He has no past medical history or family history of
similar problems. He is the oldest of four children. He
does not consume alcohol or drugs.
On examination he walks on a broad base, lurching
from side to side. He cannot walk heel to toe or stand
with his feet together. He has mild fingernose and
heelkneeshin ataxia and performs alternating
movements slowly and awkwardly. His speech is
slurred.There is no nystagmus. Both optic discs are
pale.All of his reflexes are absent. His plantar
responses are extensor. He cannot feel the vibration of
a tuning fork in his feet.
a. Where in the nervous system does the problem lie?
b. What do you think is the cause of his problems?
c. What are the issues for his parents?
Case 2
A 16-year-old boy comes to see you about his balance.
He has avoided running and football for 2 years
CHAPTER 6
Paraplegia
Anatomical considerations
Dura
Spinal cord
NB Lesions in the cervical or thoracic

spine cause UMN signs in legs
C1
T1
C1
C2
C3
C4
C5
C6
C7
C8
T1
T2
T3
T4
T5
T6
T7
T8
T9
T10
T11
T12
L1
NB Lesions in the lumbosacral spine
cause LMN signs in legs
L1
L2
L3
Figure 6.1 shows the relationship of the spinal cord, dura,

spinal nerves and vertebrae to each other. The important points
to note are:
the spinal cord terminates at the level of the L1 vertebra. Any
disease process below the level of this vertebra may cause
neurological problems, but it will do so by interfering with
function in the cauda equina not in the spinal cord;
because the vertebral column is so much longer than the
spinal cord, there is a progressive slip in the numerical value
of the vertebra with the numerical value of the spinal cord at
that level, e.g.
C7 vertebra corresponds to T1 cord
T10 vertebra corresponds to T12 cord
L1 vertebra corresponds to S1 cord;
the dural lining of the bony spinal canal runs right down to
the sacrum, housing the cauda equina below the level of the
spinal cord at L1;
the vertebrae become progressively more massive because of
the increasing weight-bearing load put upon them;
any lesion of the spine in the cervical and thoracic region, as
far down as the 10th thoracic vertebra, may result in upper
motor neurone signs in the legs;
lesions in the lumbosacral spine may result in lower motor
neurone signs in the legs.
L4
L5
Sacrum
S1
S2
S3
S4
S5
Co
Coccyx
Fig. 6.1 Diagram to show the relationship of the spinal cord, dura
and spinal nerves to the vertebrae. Co, coccygeal.
83
84
CHAPTER 6
Lateral corticospinal
or pyramidal tract
Right
Left
Posterior column
Right
From left leg
To left leg
Very important, but anatomically

poorly defined pathways
Lateral spinothalamic tract
Right
Left
Left
From left leg
Right
Left
Bladder, bowel and sexual function
Fig. 6.2 Diagram to show the spinal cord, the important tracts and their relationship to the left leg.
Figure 6.2 shows those tracts in the spinal cord which are
important from the clinical point of view:
the UMN pathway or pyramidal tract from the right hemisphere crosses from right to left in the lower medulla and
innervates lower motor neurones in the left ventral horn.
Axons from these lower motor neurones in turn innervate
muscles in the left arm, trunk and leg;
the posterior column contains ascending sensory axons
carrying proprioception and vibration sense from the left
side of the body. These are axons of dorsal root ganglion cells
situated beside the left-hand side of the spinal cord. After
relay and crossing to the other side in the medulla, this pathway gains the right thalamus and right sensory cortex;
the lateral spinothalamic tract consists of sensory axons carrying pain and temperature sense from the left side of the
body. These are axons of neurones situated in the left posterior horn of the spinal cord, which cross to the right and ascend
as the spinothalamic tract to gain the right thalamus and right
sensory cortex;
ascending and descending pathways subserving bladder,
bowel and sexual function.
PARAPLEGIA
Neurological parts:
85
Parts of vertebra:
Spine
Spinal cord
Cerebrospinal
fluid
Spinal canal lined

by dura
Lamina
Dorsal root
and ganglion
Intervertebral
facet joint
Ventral root
Pedicle
Intervertebral
foramen
Spinal nerve
Body
Fig. 6.3 Superior aspect of a cervical vertebra, showing the spinal cord, the nerve roots and the spinal
nerves.
Figure 6.3 shows the upper aspect of a cervical vertebra,

noting the bony spinal canal, lined by dura, in which the spinal
cord lies. Four points are important from the clinical point of
view:
some individuals have wide spinal canals, some have narrow
spinal canals. People with constitutionally narrow canals are
more vulnerable to cord compression by any mass lesion
within the canal;
the vulnerability of the spinal nerve, in or near the intervertebral foramen, (i) to the presence of a posterolateral intervertebral disc protrusion and (ii) to osteoarthritic enlargement of
the intervertebral facet joint;
the vulnerability of the spinal cord, in the spinal canal, to a
large posterior intervertebral disc protrusion;
below the first lumbar vertebra a constitutionally narrow
canal will predispose to cauda equina compression (see
Fig. 6.1).
86
CHAPTER 6
Clinical considerations
The clinical picture of a patient presenting with a lesion in the
spinal cord is a composite of tract signs and segmental signs, as
shown in Fig. 6.4.
Tract symptoms
and signs
Tract signs
A complete lesion, affecting all parts of the cord at one level
(Fig. 6.5), will give rise to:
bilateral upper motor neurone paralysis of the part of the
body below the level of the lesion;
bilateral loss of all modalities of sensation below the level of
the lesion;
complete loss of all bladder, bowel and sexual function.
It is more frequent for lesions to be incomplete, however, and
this may be in two ways.
1. The lesion may be affecting all parts of the spinal cord at one
level (Fig. 6.5a), but not completely stopping all function in the
descending and ascending tracts. In this case there is:
bilateral weakness, but not complete paralysis, below the
level of the lesion;
impaired sensory function, but not complete loss;
defective bladder, bowel and sexual function, rather than
complete lack of function.
2. At the level of the lesion, function in one part of the cord may
be more affected than elsewhere, for instance:
just one side of the spinal cord may be affected at the site
of the lesion (Fig. 6.5b), the so-called Brown-Sqard
syndrome;
the lesion may be interfering with function in the posterior
columns, with little effect on other parts of the cord (Fig.
6.5c);
the anterior and lateral parts of the cord may be damaged,
with relative sparing of posterior column function (Fig.
6.5d).
The level of the lesion in the spinal cord may be deduced
by finding the upper limit of the physical signs due to tract
malfunction when examining the patient. For instance, in
a patient with clear upper motor neurone signs in the legs,
the presence of upper motor neurone signs in the arms is good
evidence that the lesion is above C5. If the arms and hands are
completely normal on examination, a spinal cord lesion below
T1 is more likely.
Segmental
symptoms
and signs
Lesion
Tract symptoms
and signs
Fig. 6.4 Diagram to show that

the clinical phenomena generated
by a spinal cord lesion are a
composite of tract and segmental
features.
PARAPLEGIA
87
Right
Left
Complete spinal cord lesion
Right-sided spinal cord lesion
No downward or upward
transmission of impulses
No neurotransmission in :
Right pyramidal tract
\ UMN signs right leg
Right posterior column
\ position and vibration
sense loss right leg
Right
Left
Right spinothalamic tract

\ pain and temperature
sense loss left leg
Effect upon bladder
variable, probably just
intact
(a)
(b)
Posterior column spinal

cord lesion
Anterolateral column
spinal cord lesion
No neurotransmission in
either posterior column
\ position and vibration
sense loss in both legs
No neurotransmission in :
Bladder probably intact
Either spinothalamic tracts

\ pain and temperature
sense loss both legs
Either pyramidal tracts

\ UMN signs both legs
Tracts to bladder, bowel etc.

\ incontinence, retention,
constipation
(c)
(d)
Fig. 6.5 Various spinal cord lesions and their tract signs. (a) A complete spinal cord lesion. (b) A right-sided
spinal cord lesion. (c) A posterior spinal cord lesion. (d) An anterolateral spinal cord lesion.
88
Segmental signs
In addition to interfering with function in the ascending and descending tracts, a spinal cord lesion may disturb sensory input,
reflex activity and lower motor neurone outflow at the level
of the lesion. These segmental features may be unilateral or bilateral, depending on the nature of the causative pathology.
Chief amongst the segmental symptoms and signs are:
pain in the spine at the level of the lesion (caused by the
pathological causative process);
pain, paraesthesiae or sensory loss in the relevant dermatome (caused by involvement of the dorsal nerve root, or
dorsal horn, in the lesion);
lower motor neurone signs in the relevant myotome (caused
by involvement of the ventral nerve root, or ventral horn, in
the lesion);
loss of deep tendon reflexes, if reflex arcs which can be
assessed clinically are present at the relevant level. (A lesion
at C5/6 may show itself in this way by loss of the biceps or
supinator jerks. A lesion at C2/3 will not cause loss of deep
tendon reflexes on clinical examination.)
A common example of the value of segmental symptoms
and signs in assessing the level of a spinal cord lesion is shown
in Fig. 6.6.
Knowledge of all dermatomes, myotomes and reflex arc
segmental values is not essential to practise clinical neurology,
but some are vital. The essential requirements are shown in
Fig. 6.7.
Before proceeding to consider the causes of paraplegia in
the next section, two further, rather obvious, points should be
noted.
Paraplegia is more common than tetraplegia. This is simply a
reflection of the fact that there is a much greater length of
spinal cord, vulnerable to various diseases, involved in leg
innervation than in arm innervation, as shown in Fig. 6.1.
At the beginning of this section, and in Fig. 6.4, it was stated
that patients with spinal cord lesions present with a composite picture of tract and segmental signs. This is the truth,
but not the whole truth. It would be more accurate to say that
such patients present with the features of their spinal cord
lesion (tract and segmental), and with the features of the
cause of their spinal cord lesion. At the same time as we are
assessing the site and severity of the spinal cord lesion in a
patient, we should be looking for clinical clues of the cause of
the lesion.
CHAPTER 6
PARAPLEGIA
89
Left arm normal

Hesitancy of micturition
Pain and temperature
sensory loss, left leg
Pain in neck
Pain and
numbness
Thin weak deltoid
and biceps
Right leg
UMN weakness
Position and vibration
sensory loss
Absent biceps and

supinator jerks
Fig. 6.6 The segmental and tract symptoms and signs of a right-sided C5/6 spinal cord lesion.
Xiphisternum T6
Umbilicus T9
Anterior
Symphysis pubis T12
Posterior
T2
S3/4/5
C5
L2/3
T1
S2
C6
C8
Shoulder abduction
Elbow flexion
Elbow extension
Finger extension
Finger flexion
Small hand muscles
(e.g. finger abduction)
C5
C5/6
C7/8
C7/8
C7/8
Hip flexion
Knee extension
Foot/toe dorsiflexion
Foot/toe plantar flexion
Knee flexion
Hip extension
L2/3
L3/4
L4/5
S1/2
L5/S1
L5/S1
Biceps jerk
Supinator jerk
Triceps jerk
C5/6
C5/6
C7/8
Knee jerk
Ankle jerk
L3/4
S1/2
T1
L4/5
C7
S1
S1
L5
Fig. 6.7 The important dermatomes, myotomes and reflex arc segmental values, with which a student
should be conversant.
90
Causes of paraplegia
The four common causes of spinal cord dysfunction are illustrated in Fig. 6.8: trauma, multiple sclerosis, malignant disease
and spondylotic degenerative disease of the spine.
Trauma
Road traffic accidents involving motorcycles and cars are the
commonest cause, followed by domestic falls, accidents at work
and accidents in sport. The order of frequency of injury in terms
of neurological level is cervical, then thoracic, then lumbar. Initial care at the site of the accident is vitally important, ensuring
that neurological damage is not incurred or increased by clumsy inexperienced movement of the patient at this stage. Unless
the life of the patient is in jeopardy by leaving him at the site of
the accident, one person should not attempt to move the patient.
He should await the arrival of four or five other people,
hopefully with a medical or paramedical person in attendance.
Movement of the patient suspected of spinal trauma should be
slow and careful, with or without the aid of adequate machinery
to cut the patient out of distorted vehicles. It should be carried
out by several people able to support different parts of the body,
so that the patient is moved all in one piece.
Multiple sclerosis
An episode of paraplegia in a patient with multiple sclerosis
usually evolves over the period of 12 weeks, and recovers in
a couple of months, as with other episodes of demyelination
elsewhere in the CNS. Occasionally, however, the paraplegia
may evolve slowly and insidiously (see Chapter 7). Spinal cord
lesions due to multiple sclerosis are usually asymmetrical and
incomplete.
Malignant disease of the spine

Secondary deposits of carcinoma are the main type of trouble in
this category: from prostate, lung, breast and kidney. Often,
plain X-rays of the spine will show vertebral deposits within
and beside the spine, but sometimes the tumour deposit is primarily meningeal with little bony change. Steroid treatment,
surgical decompression, radiotherapy or chemotherapy can
sometimes make a lot of difference to the disability suffered by
such patients, even though their long-term prognosis is poor.
CHAPTER 6
Causes of spinal cord disease

Common
Trauma
Multiple sclerosis
Malignant disease
Spondylosis
Rare
Infarction
Transverse myelitis
Bends in divers
Vitamin B12 deficiency
Syringomyelia
Motor neurone disease
Intrinsic cord glioma
Radiation myelopathy
Arteriovenous malformation
Extradural abscess
Prolapsed intervertebral
thoracic disc
Neurofibroma
Meningioma
Atlanto-axial subluxation in
rheumatold arthritis
PARAPLEGIA
91
Clinical clues of the common causes of a spinal cord lesion

These clues are fallible; MR scan and other imaging
techniques should be overused rather than underused,
to establish diagnostic certainty
Multiple sclerosis
Evidence of dissemination of lesions in time and space
throughout the CNS is essential for the diagnosis of
multiple sclerosis. This evidence may lie in the history, on
examination, or in the result of investigation, e.g. a delayed
visual evoked potential
Spinal cord
Trauma
This is usually evident, but may be missed in patients:
who have previous neurological disease, e.g. multiple sclerosis
who are unconscious because of an associated head injury
or alcohol
who have other serious injuries which distract medical
attention away from the CNS
if a full neurological examination has been omitted after
trauma to the head, neck or spine
Meninges
Malignant tumour
Malignant disease causing spinal cord compression is
usually metastatic, in the spine and/or the meninges. Clinical
evidence of a primary malignant tumour, or of metastatic
disease elsewhere, will suggest this cause
Vertebrae, intervertebral discs and ligaments
Spondylotic myelopathy
Spondylotic myelopathy in the cervical region is rare under
the age of 50. Segmental symptoms and signs in the arms
are common in this condition
Fig. 6.8 The common causes of paraplegia.
Spondylotic myelopathy
Patients with central posterior intervertebral disc prolapse between C4 and T1, with or without consitutionally narrow canals,
make up the majority of this group. The cord compression may
be at more than one level. The myelopathy may be compressive
or ischaemic in nature (the latter due to interference with
arterial supply and venous drainage of the cord in the presence
of multiple-level disc degenerative disease in the neck). Decompressive surgery is aimed at preventing further deterioration in
the patient, rather than guaranteeing improvement.
92
CHAPTER 6
Management of recently developed

undiagnosed paraplegia
Four principles underly the management of patients with
evolving paraplegia.
1. Get on with it!
2. Care of the patient to prevent unnecessary complications.
3. Establish the diagnosis.
4. Treat the specific cause.
Get on with it!

Reversibility is not a conspicuous characteristic of damage
to the CNS. It is important to try to establish the diagnosis and
treat spinal cord disease whilst the clinical deficit is minor.
Recovery from complete cord lesions is slow and imperfect.
Hours may make a difference to the outcome of a patient with
cord compression.
Care of the patient to prevent unnecessary complications

The parts of the body rendered weak, numb or functionless by
the spinal cord lesion need care. Nurses and physiotherapists
are the usual people to provide this.
Skin
Frequent inspection.
Frequent relief of pressure (by turning).
Prevention and vigorous treatment of any damage.
Weak or paralysed limbs
Frequent passive movement and stockings to prevent
venous stagnation, thrombosis and pulmonary embolism.
Frequent passive movement to prevent joint stiffness and
contracture, without overstretching.
Exercise of non-paralysed muscles.
Non-functioning bladder and bowels
Catheterization.
Adequate fluids.
Dietary fibre regulation.
Laxatives.
Suppositories.
Enemas.
Tumour at the foramen magnum

(asterisk) causing high spinal cord
compression.
PARAPLEGIA
93

Foremost in establishing the diagnosis is an MR scan of the
spine and sometimes other imaging techniques. These investigations will reveal cord-compressing pathology and the need
for neurosurgical intervention. Areas of demyelination can also
be visualized within the cord.
If no compressive or intrinsic cord lesion is demonstrated by
scanning, other investigations may be helpful:
CSF analysis, visual evoked potentials, MR scan of the
brain multiple sclerosis;
EMG studies motor neurone disease;
haematological tests and serum vitamin B12 estimation
subacute combined degeneration of the cord.
Treat the specific cause
High signal in the upper cervical

cord due to multiple sclerosis
(arrow).
Low cervical cord compression

due to prolapsed discs (arrows).
Trauma: intravenous steroids, restoration of alignment and

stabilization by operative and non-operative means.
Multiple sclerosis: consider use of high dose methylprednisolone.
Malignant disease: surgical decompression, steroids, radiotherapy, chemotherapy.
Spondylotic myelopathy: surgical decompression.
Infarction: nil.
Bends: hyperbaric chamber.
Subacute combined degeneration of the cord: vitamin B12
injections.
Syringomyelia: consider surgery.
Motor neurone disease: riluzole.
Benign spinal cord tumours: consider surgery.
Radiation myelopathy: nil.
Arteriovenous malformation: embolization or surgery, which
may be difficult.
Extradural abscess: surgery and antibiotics.
Thoracic disc: surgery, which may be difficult.
Spinal neurofibroma and meningioma: surgery.
Atlanto-axial subluxation in rheumatoid arthritis: consider
surgery, which is difficult.
94
CHAPTER 6
Management of chronic diagnosed paraplegia

From whatever cause, there is a group of patients who have become severely paraplegic, and will remain so on a long-term
basis. Their mobility is going to rely heavily on a wheelchair.
Multiple sclerosis accounts for the largest number of such
patients in the UK, many of whom are young with much of
their lives still ahead. Such patients benefit from education,
encouragement and the expertise of nurses, physiotherapists,
dietitians, social workers, occupational therapists, housing
departments, industrial rehabilitation units, psychologists and
doctors. They also need the emotional support of their family
and friends. They have to come to terms with a major disability
and believe in their value despite the loss of normal function in
the lower half of their body.
Attention should be given to the following:
1. Patient education about the level of cord involvement:
what does and does not work.
2. The loss of motor function:
wheelchair acceptance and wheelchair skills;
transfers on and off the wheelchair;
physiotherapy: passive to prevent joint contractures;
active to strengthen non-paralysed muscles;
drugs to reduce spasticity: baclofen, dantrolene,
tizanidine.
3. Sensory loss:
care of skin;
guarding against hot, hard or sharp objects;
taking the weight of the body off the seat of the wheelchair routinely every 15 or 20 minutes.
4. Bladder:
reflex bladder emptying, condom drainage;
intermittent self-catheterization, indwelling catheter;
cholinergic or anticholinergic drugs as necessary;
alertness to urinary tract infection.
5. Bowel:
regularity of diet;
laxatives and suppositories.
6. Sexual function:
often an area of great disappointment;
normal sexual enjoyment, male ejaculation, orgasm,
motor skills for intercourse, all lacking;
fertility often unimpaired in either sex, though seminal
emission in males will require either vibrator stimulation
of the fraenum of the penis or electro-ejaculation;
counselling of patient and spouse helps adjustment.
There are lots of ways

of helping patients with
'incurable paraplegia'
PARAPLEGIA
95
7. Weight and calories:

wheelchair life probably halves the patients calorie
requirements. It is very easy, and counterproductive, for
paraplegic patients to gain weight. Eating and drinking
are enjoyable activities still left open to them. Heaviness
is difficult for their mobility, and bad for the weightbearing pressure areas.
8. Psychological aspects:
disappointment, depression, shame, resentment, anger
and a sense of an altered role in the family are some of the
natural feelings that paraplegic patients experience.
9. Family support:
the presence or absence of this makes a very great
difference to the ease of life of a paraplegic patient.
10. Employment:
the patients self-esteem may be much higher if he can
still continue his previous work, or if he can be retrained
to obtain new work.
11. House adaptation:
this is almost inevitable and very helpful. Living on the
ground floor, with modifications for a wheelchair life, is
important.
12. Car adaptation:
conversion of the controls to arm and hand use may give
a great deal of independence.
13. Financial advice:
this will often be needed, especially if the patient is not
going to be able to work. Medical social workers are
conversant with house conversion and attendance and
mobility allowances.
14. Recreational activity and holidays:
should be actively pursued.
15. Legal advice:
this may be required if the paraplegia was the result of an
accident, or if the patients paraplegia leads to marriage
disintegration, which sometimes happens.
16. Respite care:
this may be appropriate to help the patient and/or his
relatives. It can be arranged in several different ways, for
example:
admission to a young chronic sick unit for 12 weeks,
on a planned, infrequent, regular basis;
a care attendant lives at the patients home for 12
weeks, whilst the relatives take a holiday.
96
Syringomyelia
It is worth devoting a short section of this chapter to
syringomyelia because the illness is a neurological classic. It
brings together much of what we have learnt about cord lesions,
and is grossly over-represented in the clinical part of medical
professional examinations. It is a rare condition.
The symptoms and signs are due to an intramedullary (within the spinal cord), fluid-filled cavity extending over several
segments of the spinal cord (Fig. 6.9a). The cavity, or syrinx, is
most evident in the cervical and upper thoracic cord. There may
be an associated ArnoldChiari malformation at the level of the
foramen magnum, in which the medulla and the lowermost
parts of the cerebellum are below the level of the foramen
magnum. There may be an associated kyphoscoliosis. These
associated congenital anomalies suggest that syringomyelia is
itself the consequence of malformation of this part of the CNS.
The cavity, and consequent neurological deficit, tend to get
larger, very slowly, with the passage of time. This deterioration
may occur as sudden exacerbations, between which long
stationary periods occur.
The symptoms and signs are the direct consequence of a
lesion that extends over several segments within the substance
of the cord. There is a combination of segmental and tract signs,
as shown in Fig. 6.9b.
Over the length of the cord affected by the syrinx there are
segmental symptoms and signs. These are found mainly in the
upper limbs, since the syrinx is in the cervical and upper dorsal
part of the cord.
Pain sometimes, but usually transient at the time of an
exacerbation.
Sensory loss which affects pain and temperature, and often
leaves the posterior column function intact. Burns and
poorly healed sores over the skin of the arms are common
because of the anaesthesia. The sensory loss of pain and temperature with preserved proprioceptive sense is known as
dissociated sensory loss.
Areflexia, due to the interruption of the monosynaptic stretch
reflex within the cord.
Lower motor neurone signs of wasting and weakness.
In the legs, below the level of the syrinx, there may be motor
or sensory signs due to descending or ascending tract involvement by the syrinx. Most common of such signs are upper motor
neurone weakness, with increased tone, increased reflexes and
extensor plantar responses.
CHAPTER 6
The only place where

syringomyelia is common is in
the clinical part of Finals, and
other exams
Low signal cavity within the

upper cervical cord, with mild
ArnoldChiari malformation.
Spine
Kyphoscoliosis
Arms
Painless skin lesions
Dissociated sensory loss
Areflexia
Weakness and wasting
Legs
Spastic paraparesis
Plus or minus
Brainstem signs
Cerebellar signs
Charcot joints
PARAPLEGIA
97
(a)
(b) Segmental signs
Midbrain
Pons
Medulla
Cervical
cord
Posterior column
Tract signs
Thoracic
cord
Pyramidal tract
Lumbosacral
cord
Early syrinx
confined to
cervical and
upper thoracic
cord
Late syrinx
extending into the
medulla (syringobulbia),
and well down into the
spinal cord
Spinothalamic tract
Fig. 6.9 Diagram to show the main features of syringomyelia. (a) The extent of the cavity in the early and
late stages. (b) Segmental and tract signs.
Figure 6.9 is drawn symmetrically, but commonly the clinical

expression of syringomyelia is asymmetrical.
Extension of the syrinx into the medulla (syringobulbia), or
medullary compression due to the associated ArnoldChiari
malformation, may result in cerebellar and bulbar signs.
The loss of pain sensation in the arms may lead to the
development of gross joint disorganization and osteoarthritic change (described by Charcot) in the upper limbs.
There has been a good deal of theorizing about the mechanisms responsible for the cavity formation within the spinal
cord in syringomyelia. No one hypothesis yet stands firm.
Certainly, a hydrodynamic abnormality in the region of
the foramen magnum may exist in those patients with an
ArnoldChiari malformation, and further deterioration may be
prevented by surgical decompression of the lower medullary
region by removal of the posterior margin of the foramen
magnum.
98
CHAPTER 6
CASE HISTORIES
Case 1
A 63-year-old farmer is admitted in the night with a
3-day history of back pain and weakness in both legs.
The admitting doctor notes that weakness is
particularly severe in the iliopsoas, hamstrings and
tibialis anterior, the lower limb reflexes are normal
and the plantar responses are extensor.
Your colleague arranges for the patient to have an
MR scan of his lumbosacral spine early the next
morning and goes off duty.You are telephoned by the
radiologist who informs you that the scan is normal.
a. What should you do now?
Case 2
A 55-year-old instrument maker gradually develops
numbness and tingling in the ulnar aspects of both
hands, which worsens over several weeks. He reports

that bending his head to look down at his lathe gives
him a tingling sensation down both arms. He has
started having to hurry to pass urine.
He is a recent ex-smoker with a past history of
asthma. He takes an inhaler but no other
medication.
On examination of the upper limbs he has normal
muscle bulk, tone and power, the biceps and supinator
reflexes are absent, the triceps reflexes are brisk, and
there is diffuse impairment of light touch and pain
appreciation in the hands. In the lower limbs there is
increased tone, with no wasting or weakness but brisk
jerks and extensor plantar responses.
a. Where in the nervous system does the problem lie?
b. What is the most likely cause?
CHAPTER 7
Multiple sclerosis
General comments
Multiple sclerosis
Common in UK
Not usually severely
disabling
Sufficiently common to be
responsible for a significant
number of young
chronically neurologically
disabled people
After stroke, Parkinsons disease and multiple sclerosis are the

two commonest physically disabling diseases of the CNS in the
UK. Multiple sclerosis affects young people, however, usually
presenting between the ages of 20 and 40 years, which is
quite different from stroke and Parkinsons disease, which are
unusual conditions in patients under 45.
Though potentially a very severe disease, multiple sclerosis
does not inevitably lead to disability, wheelchair life, or
worse. As with many crippling diseases, the common image
of multiple sclerosis is worse than it usually proves to be in
practice. This severe image of the disease is not helped by
charities (some of which do noble work for research and
welfare) who appeal to the public by presenting the illness as a
crippler in print, in illustration, or in person. A more correct
image of the disease has resulted from a greater frankness
between patients and neurologists, so that now it is not only
patients who have the disease very severely who know their
diagnosis. Nowadays, most patients who are suffering from
multiple sclerosis know that this is what is wrong with them,
and the majority of these patients will be ambulant, working
and playing a full role in society. The poor image of the disease
leads to considerable anxiety in young people who develop any
visual or sensory symptoms from whatever cause, especially
if they have some medical knowledge. Most neurologists will
see one or more such patients a week, and will have the pleasure
of being able to reassure the patients that their symptoms are not
indicative of multiple sclerosis.
On the other hand, the disease is common, and enough
patients have the disease severely to make multiple sclerosis
one of the commonest causes of major neurological disability
amongst people under the age of 50 years. Provision for young
patients with major neurological disability is not good in this or
many other developed countries. There is still plenty that can
be done to help patients, and often this is best supervised and
coordinated by their doctor.
99
100
CHAPTER 7
The lesion
The classical lesion of multiple sclerosis is a plaque of demyelination in the CNS (Fig. 7.1). This means:
1. The lesion is in the CNS, not the peripheral nervous system,
i.e. in the cerebrum, brainstem, cerebellum or spinal cord. It
must be remembered that the optic nerve is an outgrowth from
the CNS embryologically. This explains why multiple sclerosis
frequently involves the optic nerves, whereas lesions in the
other cranial nerves, spinal nerves and peripheral nerves in the
limbs do not occur.
2. In the lesion the main insult is to the myelin sheaths with relative sparing of the axon. Saltatory conduction (from node to
node along myelinated nerve fibres) requires healthy myelin
sheaths. It cannot occur along the nerve fibres through a plaque
of demyelination, and non-saltatory conduction is very slow
and inefficient. Neurotransmission is accordingly impaired,
depending upon the size of the lesion, for plaques vary considerably in size.
Clinically, the lesion evolves over a few days, lasts for a few
days or weeks and gradually settles, as shown in Fig. 7.2. Vision
in one eye may deteriorate and improve in this way, or the
power in one leg may follow the same pattern. Clearly, the nature of the neurological deficit depends on the site of the plaque
of demyelination (in the optic nerve or the pyramidal tract in the
spinal cord, in the examples given here).
The evolving pathological lesion underlying the clinical
episode is summarized in Fig. 7.2.
Fig. 7.1 An obvious established

plaque of demyelination in the
myelinated nerve fibres of an
optic nerve.
Vision in the affected eye
MULTIPLE SCLEROSIS
101
No
function
Half
function
(1)
Full
function
March
Eye
(2)
April
May
Optic nerve
June
Brain
Acute destruction of myelin

Inflammatory cells and oedema
Acute stage
(1)
Major clinical deficit
No conduction through acutely

demyelinated axons
Impaired conduction through
adjacent myelinated axons
Plaque of demyelinated axons and

gliosis
Chronic stage
(2)
No, or very slow, conduction through

demyelinated axons
Normal conduction through adjacent
healthy myelinated axons
Little or no clinical deficit depending
on the size of the plaque
Impaired conduction may be detected by
sophisticated neurophysiological tests
Fig. 7.2 Diagram to show an episode of demyelination. The optic nerve has been taken as an example in
this instance.
102
CHAPTER 7
Dissemination of lesions in time and place

Multiple sclerosis is caused by the occurrence of lesions just described in different parts of the CNS, occurring at different times
in a persons life. This dissemination of lesions in time and place
remains the classical and diagnostic characteristic of multiple
sclerosis from the clinical point of view.
Lesions may occur anywhere in the CNS. Individual plaques
vary in size. One way in which a permanent ongoing disability
may evolve in a patient with multiple sclerosis is illustrated in
Fig. 7.3, and the common sites of lesions which may occur irregularly during the patients life are illustrated in Fig. 7.4.
Another cause for the development of a neurological deficit
may be axonal damage occurring in the wake of the primary inflammatory myelin pathology. Evidence is accumulating to
suggest that the axons do not escape completely unscathed in
the CNS of patients with multiple sclerosis.
The number of lesions that show themselves as attacks or
relapses in the clinical history of a patient with multiple sclerosis is much less than the number of lesions that can be found in
the patients CNS post mortem. This agrees with the fact that
MR scanning of the brain and spinal cord at the time of the first
clinical episode of demyelination frequently shows the presence of plaques elsewhere in the CNS, especially in the periventricular white matter. Furthermore, it is not uncommon to
be able to detect lesions in optic, auditory, sensory and motor
CNS pathways by electrical neurophysiological techniques in
patients with multiple sclerosis. Often, there are no symptoms
or signs accompanying such lesions, indicating unsuspected
subclinical involvement of various parts of the CNS.
2003
Right
Left
2000
No
function
1997
Right leg
2007
Half
function
Full
function
1995
2000
2005
2010
Fig. 7.3 The establishment of a neurological deficit in the right leg by episodes of demyelination along the
course of the corticospinal tract over a period of 15 years in a patient with multiple sclerosis.
MULTIPLE SCLEROSIS
103
Common clinical expressions of multiple sclerosis

Periventricular
white matter
Optic
nerve
Midbrain
Pons
Medulla
This section describes what occurs during individual episodes

of demyelination in different parts of the CNS. It also describes
the common neurological deficits that characterize a patient
who has multiple sclerosis moderately severely. Figure 7.4
shows the common sites for plaques of demyelination.
Periventricular white matter

Lesions are very common in this part of the brain. They are seen
early in the disease in patients studied by MR brain scanning,
and are always found post mortem. They do not give rise to
definite symptomatology, however.
Spinal cord
Optic nerve
common sites at which plaques
occur in the CNS of patients with
multiple sclerosis.
Tell-tale signs of previous

optic neuritis
Slightly impaired acuity
Slightly impaired colour
vision
Mild afferent pupillary
defect
Slightly pale optic disc
Delay in the visual evoked
potential
Optic neuritis is a common and typical manifestation of multiple sclerosis. If the lesion is in the optic nerve between the globe
of the eye and the optic chiasm, it is sometimes called retrobulbar (behind the globe of the eye) neuritis. If it is right at the front
of the optic nerve, the lesion itself is visible with an ophthalmoscope, and is sometimes called papillitis (inflammation of the
optic disc). The effect on vision is the same whether the lesion is
anterior or posterior in the optic nerve. If anterior, the optic disc
is visibly red and swollen, with exudates and haemorrhages. If
posterior, the appearance of the optic disc is normal at the time
of active neuritis. Asection of the optic nerve is acutely inflamed
in all instances of optic neuritis, so that pain in the orbit on eye
movement is a common symptom.
The effect on vision in the affected eye is to reduce acuity, and
cause blurring, and this most commonly affects central vision.
The patient develops a central scotoma of variable size and
density. Colour vision becomes faded, even to a point of fairly
uniform greyness. In severe optic neuritis, vision may be lost
except for a rim of preserved peripheral vision, or may be lost
altogether. At this stage, there is a diminished pupil reaction
to direct light with a normal consensual response (often called
an afferent pupillary defect).
After days or weeks, recovery commences. Recovery from
optic neuritis is characteristically very good, taking 48 weeks
to occur. Five years later, the patient often has difficulty remembering which eye was affected. Occasionally, recovery is slow
and incomplete.
104
CHAPTER 7
Midbrain, pons and medulla

Here episodes of demyelination may cause:
double vision due to individual cranial nerve dysfunction
within the midbrain or pons, or more commonly due to
a lesion in the fibre pathways that maintain conjugate
movement of the eyes. Lesions of the medial longitudinal
fasciculus cause an internuclear ophthalmoplegia, in
which there is failure of movement of the adducting eye with
preserved movement of the abducting eye, on attempted
conjugate deviation of the eyes to one side (see Chapter 8,
p. 117);
facial numbness (cranial nerve 5 within the pons);
facial weakness (cranial nerve 7 within the pons);
vertigo, nausea, vomiting, ataxia (cranial nerve 8 within the
pons);
dysarthria and occasional dysphagia (cranial nerves 9, 10
and 12 within the medulla);
cerebellar dysfunction due to lesions on fibre pathways passing in and out of the cerebellum in the cerebellar peduncles,
hence nystagmus, dysarthria, ataxia of limbs and gait;
motor deficits of upper motor neurone type in any of the four
limbs;
sensory deficits, spinothalamic or posterior column in type,
in any of the four limbs.
3
4
567
8
9
10
12
Spinal cord
Lower motor neurone and segmental signs are unusual in
multiple sclerosis. Episodes of demyelination in the spinal
cord cause fibre tract (upper motor neurone, posterior column,
spinothalamic and autonomic) symptoms and signs below the
level of the lesion. Since the length of the fibre tracts in the spinal
cord are physically longer for leg function than for arm function, there is a greater likelihood of plaques in the spinal cord
interfering with the legs than the arms. Episodes of spinal cord
demyelination may cause:
heaviness, dragging or weakness of the arms, trunk or legs;
loss of pain and temperature sensation in the arms, trunk or
legs;
tingling, numbness, sense of coldness, sense of skin wetness,
sense of skin tightness, or a sensation like that which follows
a local anaesthetic or a nettle-sting, in the arms, trunk or legs;
clumsiness of a hand due to loss of position sense and stereognosis;
bladder, bowel or sexual malfunction.
Posterior column
Autonomic
tracts
Pyramidal
tract
Spinothalamic
tract
MULTIPLE SCLEROSIS
105
In a patient with established multiple sclerosis, who has suffered

multiple episodes of demyelination throughout the CNS
(Fig. 7.5), the accumulated ongoing neurological deficit is likely
to consist of:
asymmetrical optic pallor without a major defect in visual
acuity;
a cerebellar deficit causing nystagmus, dysarthria and arm
ataxia;
an upper motor neurone deficit, mild in the arms, moderate
in the trunk and most evident in the legs. The weakness of the
legs often does not allow ataxia to reveal itself in leg movement and walking;
impaired sexual, bladder and bowel function;
a variable amount and variety of sensory loss, more evident
in the legs and lower trunk than in the arms.
Doctors probably tend to overfocus on the specific neurological disabilities in a patient with multiple sclerosis. The
orientation of the patient and family may be less specific,
and more concerned with general lack of mobility and vitality, less robust physical health, and the patients limited
social roles.
1987 Transient loss of

vision in left eye
1997 Numbness and

weakness in both legs
with some bladder
disturbance
1998 Double vision

and unsteadiness
2000 Weak legs again with

incomplete recovery, leg
numbness
2004 Transient loss of vision

in right eye
2007 Further increase in leg

weakness with unsteadiness,
ataxia of arms, dysarthria and
nystagmus
Fig. 7.5 Diagram to show the classical dissemination of lesions in time and space, and the accumulation of
a neurological deficit, in a patient who has multiple sclerosis moderately severely.
106
CHAPTER 7
Diagnosis
There is no specific laboratory test that confirms the presence of
multiple sclerosis. The diagnosis is a clinical one, based upon
the occurrence of lesions in the CNS which are disseminated in
time and place. The presence of subclinical lesions in the CNS
may be detected by:
various clinical neurophysiological techniques. Such techniques essentially measure conduction in a CNS pathway,
detecting any delay in neurotransmission by comparison
with normal control data. The visual evoked potential is the
one most commonly used;
imaging techniques. Frequently MR brain scanning reveals
multiple lesions, especially in the periventricular regions.
The inflammatory nature of the demyelinating lesion may result in an elevated lymphocyte count and globulin content in
the CSF. These changes also lack specificity. Immunoelectrophoretic demonstration of oligoclonal bands in the CSF globulin has come closest to becoming a diagnostic feature of
multiple sclerosis, but it is not specific, producing both falsepositive and false-negative results (Fig. 7.6).
Slow
CMCV
MR scan showing multiple areas

of high signal in the white matter
due to multiple sclerosis.
Magnetic
resonance imaging
of multiple lesions
SSEP
delay
VEP delay
Eye
AEP
delay
Ear
Elevated cell
count and
oligoclonal bands
in CSF
Limbs

abnormal investigations in
patients with multiple sclerosis.
None is specific. MR scanning
is used. CSF abnormalities are
found, especially the presence
of oligoclonal bands in the CSF
globulin. AEP, auditory evoked
potential from ear to temporal
cortex; CMCV, central motor
conduction velocity from
motor cortex to limbs; SSEP,
somatosensory evoked potential
from limbs to sensory cortex; VEP,
visual evoked potential from eye
to occipital cortex.
MULTIPLE SCLEROSIS
107
Aetiology
The cause of multiple sclerosis remains unknown. There
appears to be an interaction of environmental factors with some
form of genetically determined patient susceptibility.
The evidence for genetic susceptibility is as follows:
multiple sclerosis is more common in females than males,
ratio 1.5 : 1;
there is a firm association of multiple sclerosis with certain
HLA types, particularly DR2;
there is an increased incidence of multiple sclerosis in close
relatives;
in multiple sclerosis patients who have a twin, identical
co-twins are more likely to develop it than non-identical
twins.
The evidence for an environmental factor is as follows:
multiple sclerosis is more common in temperate than in
equatorial parts of the world. Migrants moving from highrisk to low-risk areas (e.g. from northern Europe to
Israel) under the age of puberty acquire low risk, and vice
versa;
IgG levels are higher in the CSF of patients with multiple
sclerosis. Antibodies to measles virus, and to some other
viruses, are higher in the CSF of patients with multiple
sclerosis.
Management
Mild or early cases
1. Inform the patient and family of the diagnosis.
2. Educate the patient and family about multiple sclerosis.
3. Dispel the concept of inevitable progression to major disability. Make explanatory literature available.
4. Encourage normal attitudes to life, and normal activities.
(This advice should be given initially by the consultant neurologist, and two interviews at an interval will nearly always be
needed. Subsequent counselling and support by a specialist
nurse or the family doctor may be very valuable, depending on
the patients reaction to the problem.)
108
More serious cases

1. Continued education about the nature of multiple sclerosis.
2. Continued support over the disappointment and uncertainty of having multiple sclerosis.
3. Attention to individual symptoms:
vision, rarely a major problem. Low visual acuity aids may
prove helpful in the minority of patients who need them;
cerebellar deficit, difficult to help pharmacologically;
paraplegia all the problems attendant upon chronic paraplegia (see Chapter 6, pp. 94 and 95) may occur, and require attention;
pain may arise from faulty transmission of sensation and
may respond to antidepressants (e.g. amitriptyline) or
anticonvulsants (e.g. gabapentin);
fatigue common and hard to treat, but may respond to
antidepressants (e.g. fluoxetine) or yoga.
4. Help from nurses, physiotherapists, occupational therapists,
speech therapists and medical social workers, as required.
5. Attention to psychological reactions occurring in the patient
or family. Encourage all activities which the patient enjoys and
are still possible.
6. Respite care arrangements, as required.
All cases of multiple sclerosis

1. Several immunomodulatory drugs (azathioprine, betainterferon, copaxone, mitoxantrone, etc.) reduce the incidence
of relapses somewhat in ambulatory patients with relapsing
and remitting multiple sclerosis. They have a much more
questionable effect on the development of disability.
2. Corticosteroids, often in the form of high-dose intravenous
methyl-prednisolone over 3 days, reduce the duration and
severity of individual episodes of demyelination, without influencing the final outcome.
3. Dietary exclusions and most supplements are of no proven
advantage. Fish oil supplements may be of benefit. The main
dietary requirement is the avoidance of obesity in the enforced
sedentary state.
CHAPTER 7
MULTIPLE SCLEROSIS
109
CASE HISTORIES
Case 1
A 37-year-old man presents with double vision, right
facial numbness and a clumsy right arm. His symptoms
began over the course of a weekend and are starting
to improve 3 weeks later. He had an episode of the
same symptoms 4 years ago which took 2 months to
clear up. His sister has MS.
Examination reveals a right internuclear
ophthalmoplegia (i.e. when he looks to the left, the
right eye does not adduct and the left eye shows
nystagmus), right trigeminal numbness and rightsided limb ataxia.
b. What treatment should he have?
first 15 years, beginning with left optic neuritis. Over

the last 5 years her disability has steadily progressed.
She is now wheelchair-bound and catheterized. She
takes baclofen for leg cramps.
She comes to see you because she is very worried
about the slowly increasing tingling and weakness in
her hands, which is making it difficult for her to do up
buttons or hold a pen. She says that losing the use of
her hands would be the final straw. Examination
reveals wasting and weakness of the first dorsal
interosseus, lumbrical and adductor digiti minimi
muscles; the rest of her hand and forearm muscles
are reasonably strong. Her reflexes are all
brisk.
a. What is the cause of this problem?
b. What would you advise?
Case 2
A 48-year-old woman has had clinically definite MS for
more than 20 years. She had about ten relapses in the
CHAPTER 8
Cranial nerve disorders
Introduction
Nuclei, intermedullary nerve fibre

pathways, cranial nerve, sensory ganglion
and the three main branches of the
trigeminal nerve (motor in dark grey;
sensory in green)
Cerebrum
3
4
5
8
7
6
Cerebellum
Disorders of the cranial nerves usually produce clear abnormalities, apparent to both patient and doctor alike. The specialists
who become involved in the management of patients with
cranial nerve problems are neurologists, neurosurgeons,
ophthalmologists (cranial nerves 24, 6), dentists (cranial nerve
5) and ENT surgeons (cranial nerves 1, 5, 710, 12).
Cranial nerves 1, 2 and 11 are a little different from the others.
Nerves 1 and 2 are highly specialized extensions of the brain, for
smell and sight, in the anterior cranial fossa and suprasellar region. Nerve 11 largely originates from the cervical spinal cord,
rises into the posterior fossa only to exit it again very quickly, to
supply muscles of the neck and shoulder.
It is useful to remember that the other cranial nerves (310
and 12; Fig. 8.1) can be damaged at three different points along
their paths. The lesion may affect the nucleus of the cranial
nerve within the brainstem, where its cell bodies lie. Alternatively, the lesion may damage the axons travelling to or from the
nucleus but still within the brainstem. In both these situations
there is commonly damage to nearby pathways running
through the brainstem, so that in addition to the cranial nerve
palsy, the patient will often have weakness, sensory loss or
ataxia in the limbs. Finally the lesion may affect the nerve itself
outside the brainstem as it passes to or from the structure which
it supplies. This causes either an isolated cranial nerve palsy, or
a cluster of palsies arising from adjacent nerves. Examples of
these clusters include malfunction of 5, 7 and 8 caused by an
acoustic neuroma in the cerebellopontine angle, or malfunction
of 9, 10 and 11 due to malignancy infiltrating the skull base.
10
12
Spinal
cord
Fig. 8.1 Lateral aspect of the brainstem, and cranial nerves 310 and
12 (seen from the left).
111
112
CHAPTER 8
Olfactory (1) nerve (Fig. 8.2)

Patients who have impaired olfactory function complain that
they are unable to smell and that all their food tastes the same.
This reflects the fact that appreciation of the subtleties of flavour
(beyond the simple sweet, salt, acid, meaty and bitter tastes) is
achieved by aromatic stimulation of the olfactory nerves in the
nose. This is why wine tasters sniff and slurp.
The commonest cause of this loss is nasal obstruction by infective or allergic oedema of the nasal mucosa. Olfactory nerve
function declines with age and with some neurodegenerative
diseases. Olfactory nerve lesions are not common. They may result from head injury, either involving fracture in the anterior
fossa floor, or as a result of damage to the nerves on the anterior
fossa floor at the time of impact of the head injury. Sometimes,
the olfactory nerves stop working on a permanent basis for no
apparent reason, i.e. idiopathic anosmia. Very occasionally, a tumour arising from the floor of the anterior fossa (e.g. meningioma) may cause unilateral or bilateral loss of olfactory function.
Anterior cranial
fossa
Nasal cavity
Fig. 8.2 Olfactory nerve and bulb

on the floor of the anterior cranial
fossa, and olfactory nerve bundles
penetrating the thin cribiform
plate to innervate the mucosa in
the roof of the nasal cavity.
Optic (2) nerve, chiasm and radiation (Fig. 8.3)

Figure 8.3 shows the anatomical basis of the three common
neurological patterns of visual loss: monocular blindness,
bitemporal hemianopia and homonymous hemianopia.
Visual
field
Eye
Ipsilateral
monocular
blindness
Optic nerve
Optic chiasm
Optic tract
Bitemporal
hemianopia
Lateral
geniculate
body
Optic
radiation
Visual cortex
Contralateral
homonymous
hemianopia
Fig. 8.3 The anatomy of the visual

pathways, and the three common
types of lesion occurring therein.
CRANIAL NERVE DISORDERS
When recording visual field

defects, the convention is to
show the field from the left eye
on the left, and the right eye on
the right, as if the fields were
projecting out of the patients
eyes and down onto the page
Left
Left
Right
Right
113
Monocular blindness
Monocular visual disturbances occur transiently in the prodromal phase of migraine (see pp. 21415), or as a consequence
of thrombo-embolism in the ophthalmic artery, as a result of ipsilateral carotid artery atheromatous disease or embolism from
the heart. Transient visual loss due to embolization often commences like a curtain descending over the vision. Infarction of
the optic nerve or retina, with permanent monocular visual loss,
is relatively uncommon in patients with thrombo-embolic disease, though common in untreated patients with giant cell
arteritis (see p. 218). Monocular visual loss occurs in patients
with optic neuritis as part of multiple sclerosis (see p. 103).
Rarely, impairment of vision in both eyes occurs as a result of
bilateral simultaneous optic nerve disease:
bilateral optic neuritis due to multiple sclerosis;
methanol poisoning;
Lebers hereditary optic neuropathy;
tobaccoalcohol amblyopia;
longstanding papilloedema due to untreated intracranial
hypertension.
Bitemporal hemianopia
Bitemporal hemianopia due to optic chiasm compression by a
pituitary adenoma growing upwards out of the pituitary fossa
is the most classical situation to be considered here (Chapter 3,
see pp. 478). Like most classical syndromes, it is rather unusual in every typical detail because:
the pituitary tumour does not always grow directly upwards
in the midline, so that asymmetrical compression of one optic
nerve or one optic tract may occur;
the precise relationship of pituitary gland and optic chiasm
varies from person to person. If the optic chiasm is posteriorly situated, pituitary adenomas are more likely to compress
the optic nerves. If the optic chiasm is well forward, optic
tract compression is more likely;
not all suprasellar lesions compressing the optic chiasm are
pituitary adenomas. Craniopharyngiomas, meningiomas
and large internal carotid artery aneurysms are alternative,
rare, slowly evolving lesions in this vicinity.
114
Homonymous hemianopia
Homonymous hemianopia, for example due to posterior cerebral artery occlusion, may or may not be noticed by the
patient. If central vision is spared, the patient may become
aware of the field defect only by bumping into things on the
affected side, either with his body, or occasionally with his car!
If the homonymous field defect involves central vision on the
affected side, the patient usually complains that he can see
only half of what he is looking at, which is very noticeable
when reading.
Though posterior cerebral artery occlusion and infarction
of the occipital cortex is the commonest cerebral hemisphere
lesion causing permanent visual loss, other hemisphere lesions
do cause visual problems:
an infarct or haematoma in the region of the internal
capsule may cause a contralateral homonymous hemianopia, due to involvement of optic tract fibres in the posterior limb of the internal capsule. Contralateral hemiplegia and
hemianaesthesia are commonly associated with the visual
field defect in patients with lesions in this site;
vascular lesions, abscesses and tumours situated in the posterior half of the cerebral hemisphere, affecting the optic radiation (between internal capsule and occipital cortex), may
cause incomplete or partial homonymous hemianopia. Lesions in the temporal region, affecting the lower parts of the
optic radiation, cause homonymous visual field loss in the
contralateral upper quadrant. Similarly, by disturbing function in the upper parts of the optic radiation, lesions in the
parietal region tend to cause contralateral homonymous
lower quadrant field defects.
More subtle dysfunction in the visual pathways may cause
difficulty in attending to stimuli in one half of the visual field, effectively a lesser form of contralateral homonymous hemianopia. In this situation the patient can actually see in each half
of the visual field when it is tested on its own. When both halffields are tested simultaneously, for example by the examiner
wiggling her fingers to either side of a patient who has both
eyes open, the patient consistently notices the finger movements on the normal side and ignores the movements on the
affected side. This phenomenon, which is common after
strokes, is referred to as visual inattention or visual neglect.
CHAPTER 8
Left
Right
Left
Right
Left
Right
115
Third, fourth and sixth cranial nerves

Some modification of the primary motor pathway for voluntary
movement (Fig. 8.4) is necessary in the case of eye movement to
enable simultaneous movement of the two eyes together, i.e.
conjugate movement. This is shown in Fig. 8.5. The centres and
pathways which integrate 3rd, 4th and 6th nerve function lie in
the midbrain and pons.
Upper motor neurone
Lower motor neurone

primary motor pathway.
Muscle
Brainstem
Cerebrum
Cavernous sinus and orbit
X
X
X
Right
eye
muscles
Upper motor neurone
Lower motor neurone

in cranial nerves 3, 4 and 6
Left
eye
muscles
Centres and pathways

for conjugate gaze,
and cranial nerve nuclei
3, 4 and 6, in midbrain
and pons
Supranuclear
gaze palsies
Gaze palsies
Internuclear
ophthalmoplegia
Cranial nerve
palsies 3, 4 and 6
Myasthenia
gravis and
myopathy
Nuclear cranial
nerve palsies
3, 4 and 6
Fig. 8.5 Diagram to show the parts of the nervous system involved in eye movement, and the type of eye
movement disorder that results from lesions in each part.
116
CHAPTER 8
Supranuclear gaze palsy

Site of lesion: cerebral hemisphere.
Common.
Common causes:
massive stroke;
severe head injury.
Movement of the eyes to the left is initiated by the right cerebral hemisphere, just like all motor, sensory and visual functions involving the left-hand side of the body. Each cerebral
hemisphere has a centre in the frontal region, involved in
conjugate deviation of the eyes to the opposite side. Patients
with an acute major cerebral hemisphere lesion are unable to
deviate their eyes towards the contralateral side. This is the
commonest form of supranuclear gaze palsy (right cerebral
hemisphere lesion, and paralysis of conjugate gaze to the left
in the diagram).
The centres for conjugate gaze in the brainstem and the cranial nerves are intact. If the brainstem is stimulated reflexly to
induce conjugate eye movement, either by caloric stimulation of the ears, or by rapid dolls head movement of the head
from side to side, perfectly normal responses will occur.
Paralysis of voluntary conjugate gaze, with preserved reflex
conjugate eye movement, is the hallmark of supranuclear
gaze palsy.
Supranuclear vertical gaze palsy, i.e. loss of the ability to look
up or down voluntarily, is occasionally seen in neurodegenerative diseases.
Gaze palsy
Eyes deviated to the right

because of conjugate gaze
palsy to the left
At the midbrain level

Uncommon.
The programming of the 3rd and 4th cranial nerve nuclei
for conjugate vertical eye movement, and for convergence
of the two eyes, occurs in centres in the midbrain. The
paralysis of voluntary and reflex eye movement which
occurs with lesions in this region is known as Parinauds
syndrome.
At the pontine level
Uncommon.
Conjugation of the two eyes in horizontal eye movements is
achieved by an ipsilateral pontine gaze centre, as shown in
Fig. 8.6. A lesion in the lateral pontine region (on the right in
the diagram) will cause voluntary and reflex paralysis of conjugate gaze towards the side of the lesion.
Eyes won't move up or down

in the vertical plane
Eyes won't converge
There may be associated ptosis
and pupil abnormality
Eyes deviated to the left

because of conjugate gaze
palsy to the right
117
L
3
Midbrain
Fig. 8.6 Brainstem centres and

pathways for conjugate
horizontal movement. Voluntary
gaze to the left is initiated in the
right cerebral hemisphere. A
descending pathway from the
right cerebral hemisphere
innervates the left pontine gaze
centre. From there, impulses pass
directly to the left 6th nerve
nucleus to abduct the left eye, and
(via the medial longitudinal
fasciculus) to the right 3rd nerve
nucleus to adduct the right eye.
Left
pontine
gaze
centre
Pons
6
Medulla
Internuclear ophthalmoplegia
R
nystagmus in the
abducting eye
Paralysis of right eye

adduction with normal
convergence
Site of lesion: midbrain/pons (Fig. 8.6).

Common.
Common cause: multiple sclerosis.
A lesion between the 3rd nerve nucleus in the midbrain and
the 6th nerve nucleus in the pons an internuclear lesion on
the course of the medial longitudinal fasciculus (on the right
side in the diagram):
does not interfere with activation of the left 6th nerve nucleus
in the pons from the left pontine gaze centre, so that abduction of the left eye is normal (except for some nystagmus
which is difficult to explain);
does interfere with activation of the right 3rd nerve nucleus
in the midbrain from the left pontine gaze centre, so that
adduction of the right eye may be slow, incomplete or
paralysed;
does not interfere with activation of either 3rd nerve nucleus
by the midbrain convergence coordinating centres, so that
convergence of the eyes is normal.
118
CHAPTER 8
Before considering 3rd, 4th and 6th nerve palsies in detail, it is

worth remembering the individual action of each of the eye
muscles, and their innervation (Fig. 8.7).
Primary position
Looking up in the
abducted position
superior rectus
3rd nerve
Looking down in the

abducted position
inferior rectus
3rd nerve
Abducting
lateral rectus
6th nerve
Adducting
medial rectus
3rd nerve
Looking up in the
adducted position
inferior oblique
3rd nerve
Looking down in the

adducted position
superior oblique
4th nerve
Furthermore, we have to remember that:

the eyelid is kept up by levator palpebrae superioris which
has two sources of innervation, minor from the sympathetic
nervous system, major from the 3rd cranial nerve;
pupillary dilatation is activated by the sympathetic nervous
system, and is adrenergic;
pupillary constriction is mediated through the parasympathetic component of the 3rd cranial nerve, and is cholinergic.
Fig. 8.7 Normal eye movements

in terms of which muscle and
which nerve effect them. Diagram
shows the right eye viewed from
the front.
119
Third nerve palsy

Common.
Common causes:
posterior communicating artery aneurysm (painful);
mononeuritis in diabetes (pupil usually normal);
pathology beside the cavernous sinus, in the superior orbital
fissure or in the orbit (adjacent nerves commonly involved,
e.g. 4, 6, 5a, and 2 if in the orbit).
The parasympathetic innervation of the eye is supplied by
the 3rd nerve.
The diagram shows a complete right 3rd nerve palsy. The
lesion can be incomplete of course, in terms of ptosis, pupil
dilatation or weakness of eye movement.
Complete ptosis
Eye is deviated 'down and out'

in the primary position
Dilated, non-reactive pupil
Normal abduction
Fourth nerve palsy

Uncommon.
Common cause; trauma
affecting the orbit.
Rotation of globe on
attempted down-gaze
No
Incomplete depression in the

adducted position (right eye in
this diagram)
Compensatory head tilt towards

the opposite shoulder may be
present, to obtain single vision
whilst looking forward
No other movement
There may be some inturning of the

eye and double vision in the
primary position (because of
weakness of right eye abduction
in this diagram)
There may be compensatory head
turning (to the right in this case)
to obtain single vision whilst
looking forward
No abduction of the eye
Some torsion of the eye in the orbit
No
Sixth nerve palsy

Common.
Common causes:
as a false localizing sign in patients with raised intracranial
pressure;
multiple sclerosis and small cerebrovascular lesions within
the pons;
pathology beside the cavernous sinus, in the superior orbital
fissure or orbit (adjacent nerves commonly involved, e.g. 3,
4, 5a, and 2 if in the orbit).
120
Myasthenia gravis
Uncommon.
Ocular involvement common in myasthenia gravis.
Myasthenia should be considered in any unexplained
ophthalmoplegia, even if it looks like a 4th, 6th or partial
3rd nerve palsy (see pp. 119 and 1646).
CHAPTER 8
Ptosis
Eye movement abnormality which
doesn't necessarily match gaze palsy,
internuclear ophthalmoplegia,
or cranial nerve palsy
Variability
Fatiguability
Normal pupils
Myopathy
Graves disease is the only common myopathy to involve eye
muscles.
The patient may be hyperthyroid, euthyroid or hypothyroid.
Inflammatory swelling of the external ocular muscles within
the orbit, often leading to fibrosis, is responsible.
Involvement of the external ocular muscles in other forms of
myopathy occurs, but is exceedingly rare.
Concomitant squint
Very common.
Caused by dissimilar visual acuity and refractive properties
in the two eyes from an early age.
Proper binocular fixation has never been established.
Known as amblyopia.
Fixation is by the better-seeing eye; the image from the
amblyopic eye is suppressed, so there is no complaint of
double vision.
Horners syndrome
Uncommon.
Caused by loss of sympathetic innervation to the eye.
The sympathetic supply to the face and eye is derived
from the hypothalamic region, descends ipsilaterally
through the brainstem and cervical cord, and reaches
the sympathetic chain via the motor root of T1. From the
superior cervical sympathetic ganglion, the fibres pass along
the outer sheath of the common carotid artery. Fibres to
the eye travel via the internal carotid artery and its ophthalmic branch. Fibres to the face travel with the external
carotid artery.
Often asymmetrical
Sometimes unilateral
Proptosis
Lid retraction
Lid lag
Ophthalmoplegia in any direction
Normal pupils
Non-paralytic, each eye possessing

a full range of movement when
tested individually with the other
eye covered
When one eye is covered, the other
fixes. Alternate covering of each
eye shows a refixation movement
in each eye very clearly
Minor degree of ptosis

Small pupil
Enophthalmos
Loss of sweating on the affected
side of the face
Indoors
R
After 1 min of bright sunshine

R
121
HolmesAdie syndrome
Uncommon.
Often unilateral.
An interesting curiosity of no sinister significance.
Very slow pupillary reaction to light, myotonic pupil (left eye
in diagram).
Absent deep tendon reflexes in the limbs is a common accompaniment, especially knee and ankle jerks.
Site of pathology uncertain.
After 30 min of bright sunshine
ArgyllRobertson pupil
Very uncommon.
A sign of tertiary syphilis.
Site of pathology uncertain.
Small, unequal, not round, irregular

No reaction to light
Normal reaction to accommodation
Proptosis
Resistance to backward movement
of the globe in the orbit
Palpable orbital mass
Globe displacement in the orbit by
the mass
Distortion of the eyelid
Mechanical limitation of eye
movement in the orbit
Possible impairment of vision in the
affected eye
Orbital mass lesions

Uncommon.
Causes:
benign tumours;
malignant tumours, primary or secondary;
extension of inflammatory pathology from the paranasal
sinuses;
non-neoplastic inflammatory infiltrate at the back of the
orbit, so-called pseudotumour.
CT scanning of the orbits is the most helpful investigation.
122
CHAPTER 8
Trigeminal (5) nerve

Sensory loss in the face is very noticeable, as a visit to the dentist
which requires a local anaesthetic will remind us. Sensory loss
affecting the cornea can lead unwittingly to serious corneal
damage. Pain in the face is very intrusive.
Figures 8.8 and 8.9 demonstrate the relevant clinical anatomical features of the trigeminal nerve. The following points
are worth noting:
the upper border of sensory loss in a trigeminal nerve lesion
lies between the ear and the vertex, and the lower border is
above the angle of the jaw. Patients with non-organic sensory
loss on the face tend to have the junction of forehead and
scalp as the upper border, and the angle of the jaw as the
lower border;
the corneal reflex requires corneal, not scleral, stimulation,
and the response (mediated through the 7th cranial nerve) is
to blink bilaterally. It can therefore be tested in the presence of
an ipsilateral 7th nerve lesion;
the jaw-jerk, like any other stretch reflex, is exaggerated in the
presence of an upper motor neurone lesion. In the case of the
jaw-jerk, the lesion must be above the level of the trigeminal
motor nucleus in the pons. In patients with upper motor neurone signs in all four limbs, an exaggerated jaw-jerk is sometimes helpful in suggesting that the lesion is above the pons,
rather than between the pons and the mid-cervical region of
the spinal cord;
pathology in the cavernous sinus affects only the ophthalmic
and maxillary branches of the trigeminal nerve, as the
mandibular branch has dived through the foramen ovale, behind the cavernous sinus. Similarly, orbital pathology affects
only the ophthalmic branch, since the maxillary branch has
exited the skull through the foramen rotundum posterior to
the orbit.
Figure 8.9 gives information about the diseases that may
affect the trigeminal nerve. There are really only two common
ones: trigeminal neuralgia and herpes zoster.
Trigeminal neuralgia is described in Chapter 13 (see p. 219), and is
the most common disease affecting the trigeminal nerve. It can
be caused by irritation of the nerve as it enters the brainstem (for
example by an adjacent blood vessel) or within the brainstem
itself (rarely; for example by multiple sclerosis). Presumably
abnormal paroxysmal discharges within the nerve give rise to
the lancinating pain.
5a
C2 + 3
5b
5c
C2 + 3
Fig. 8.8 Areas of the skin

supplied by each branch of the
trigeminal nerve and the 2nd and
3rd cervical dermatomes.
123
Midbrain
5a Ophthalmic
Trigeminal
ganglion
Cerebellar
peduncles
Pons
5b
Maxillary
Medulla
5c
Mandibular, with motor
branches to masseter, temporal
and pterygoid muscles

trigeminal nerve, and the diseases
that may affect it.
Orbital and
cavernous sinus
region
Ganglion
Cerebellopontine
angle, subarachnoid
space and skull base
Brainstem
Tumours
Aneurysms
AV fistula
Pseudotumour
Acoustic neuroma
Basal meningitis
Malignant infiltration
from nasopharynx
Trigeminal neuralgia
Cerebrovascular
disease
Multiple sclerosis
Brainstem
tumours
Herpes
zoster
Herpes zoster (shingles) affecting the trigeminal nerve is also

mentioned in Chapter 13 (see p. 220) and in Chapter 15 (see
p. 239). Though the virus is in the trigeminal ganglion, clinical
involvement is most usually confined to the skin and cornea
supplied by the ophthalmic branch. The painful vesicular rash,
sometimes preceded by pain for a few days and sometimes followed by pain for ever, is similar to shingles elsewhere in the
body. The involvement of the cornea, however, makes urgent
ophthalmic referral essential, and the use of local, oral, or parenteral antiviral agents (like aciclovir) important. Parenteral
administration is especially likely if there is any evidence of
immunosuppression in the patient.
124
Facial (7) nerve

Figure 8.10 shows the peripheral distribution of the facial nerve.
The nerve leaves the pons in the cerebellopontine angle. It provides autonomic efferent fibres to lacrimal and salivary glands,
collects afferent taste fibres from the anterior two-thirds of the
tongue, and provides the innervation of the stapedius muscle in
the ear, before emerging from the stylomastoid foramen behind
and below the ear to innervate the facial muscles as shown in
Fig. 8.10.
Proximal lesions of the facial nerve produce, therefore, in addition to weakness of all the ipsilateral facial muscles, an alteration of secretion in the ipsilateral lacrimal and salivary glands,
impairment of taste perception on the anterior two-thirds of the
tongue, and hyperacusis (sounds heard abnormally loudly) in
the ear on the side of the lesion. If the lesion has been complete,
with Wallerian axonal degeneration distal to the site of the
lesion, recovery is rarely complete and re-innervation is often
incorrect. Axons, which used to supply the lower part of the
face, may regrow along Schwann tubes which lead to the upper
part of the face, and vice versa. Patients in whom this has happened are unable to contract part of their facial muscles in isolation. When they close their eyes vigorously, there is retraction of
the corner of the mouth on the affected side. When they contract
mouth muscles as in whistling, there is eye muscle contraction
and possible closure on the side of the lesion. Sometimes, axons
that used to supply the salivary glands find their way to the
lacrimal glands. In such patients, tears may form excessively in
the eye of the affected side at mealtimes.
Bells palsy
The common disease of the facial nerve is Bells palsy. The cause
of this condition is not certain, although there is some evidence
to suggest inflammation due to reactivation of herpes simplex
virus within the nerve ganglion in many cases. The lesion is usually proximal enough to have effects on taste and hearing. After
some aching around the ear, the facial weakness develops quite
quickly within 24 hours. It affects all the facial muscles including the forehead, which distinguishes it from supranuclear facial weakness (for example due to a stroke) where the forehead
is spared. The patient is usually very concerned by the facial
appearance. Drainage of tears from the eye may be disturbed on
the affected side because the eyelids lose close apposition with
the globe of the eye, so the eye waters. The cornea may be vulnerable because of impaired eye closure. Speaking, eating and
drinking may be difficult because of the weakness around the
mouth.
CHAPTER 8
125
Internal auditory meatus
Stylomastoid foramen
Lacrimal
glands
Nerve
leaves
pons in
cerebellopontine
angle
Stapedius
muscle
Facial
muscles
Geniculate
ganglion
Petrous temporal bone,

containing inner and
middle ear
Salivary Taste in anterior

glands
two-thirds of
tongue
Fig. 8.10 The peripheral distribution of the facial nerve to the muscles of the face, and a highly
diagrammatic representation of the proximal part of the facial nerve within the petrous temporal bone.
The risk of poor recovery is increased if the facial paralysis is

complete, if there is hyperacusis or loss of taste, if the patient is
pregnant or elderly, and if the nerve is electrically inexcitable (if
neurophysiological studies are done).
Care of the eye, encouragement and facial exercises in the
mirror are all that can be offered in the way of treatment in the
acute stage, unless the patient is seen within 72 hours of onset
when a short course of steroids may improve the patients
prospects for recovery.
Rarer causes of facial palsy

Herpes zoster affecting the geniculate ganglion, which lies on
the course of the facial nerve. Vesicles may appear in the external auditory meatus or soft palate to indicate this cause of
the facial palsy. This is known as the RamsayHunt syndrome and behaves like an idiopathic Bells palsy from the
point of view of recovery.
Trauma, fractures involving the petrous temporal bone.
Middle ear infection, acute or chronic.
Diabetes mellitus.
Sarcoidosis.
Acoustic neuroma, either before or after its removal from the
cerebellopontine angle.
Surgery in the ear and parotid gland region.
Lyme disease.
Pregnancy increases the risk of Bells palsy (as Bell himself
recognized).
126
CHAPTER 8
Cochlear
nerve
Semicircular
canals
The cortex most dedicated to

auditory and vestibular function
is in the superior temporal gyrus
Cerebrum
Vestibular
nerve
Cochlear and vestibular

nuclei in the pons, relaying
input from the 8th nerve
to cerebrum, cerebellum
and spinal cord
Cerebellum
Cochlea
Eighth, cochleovestibular nerve
External, middle and

inner ear in the petrous
temporal bone
Fig. 8.11 The left-hand side of the diagram shows detail of the inner ear in the petrous temporal bone. The
right-hand side of the diagram shows the central connections of the 8th nerve.
Cochleo-vestibular (8) nerve

Figure 8.11 reminds us of the extremely delicate structure of the
cochlea and labyrinth within the petrous temporal bone, of the
radiation of incoming information from the inner ear throughout the CNS, and of the localization of auditory and vestibular
functions in the posterior part of the superior temporal gyrus in
the cerebral hemisphere.
The common symptoms and signs found in patients with
cochleo-vestibular disorders, and the common tests used to
evaluate them, appear below.
Symptoms
Signs
Tests
Deafness
Tinnitus
Vertigo
Loss of balance
Deafness of sensorineural type

Nystagmus
Positional nystagmus
Ataxia of gait
Audiometry
Auditory evoked potentials
Caloric responses
Electronystagmography
127
Common causes of deafness and loss of balance
Fig. 8.12 The main diseases

affecting hearing, balance, or
both.
The top section of Fig. 8.12 demonstrates that the common

causes of deafness are in the external, middle or inner ear.
Acoustic neuroma is an occasional cause of slowly progressive
unilateral nerve deafness. Ideally, it should be diagnosed and
treated at this stage, before it has caused other evidence of
a cerebellopontine space-occupying lesion (i.e. 5th and 7th
cranial nerve palsy, ipsilateral cerebellar signs, and raised
intracranial pressure). The tumour is a benign one, derived
from the Schwann cells on the 8th nerve.
The main diseases affecting hearing:

8th nerve
Acoustic neuroma
Cochlea
Presbyacusis
Acoustic trauma
External and middle ear
Wax
Otitis media
Trauma
Otosclerosis
The main disease affecting hearing and balance:

Cochlea and labyrinth
Mnire's disease
The main diseases affecting balance:
Labyrinth
Benign paroxysmal
positional vertigo
Acute vestibular failure
(acute labyrinthitis or
vestibular neuronitis
Drugs (e.g. streptomycin)
Brainstem
Vascular disease
Demyelination
Drugs (e.g. anticonvulsants)
128
Mnires disease is depicted in the central section of Fig. 8.12. It

is probably due to a lesion in the endolymph in both the cochlea
and the labyrinth. It therefore causes auditory and vestibular
symptoms. The typical patient is middle-aged with a history of
unilateral deafness and tinnitus, followed by episodes of very
severe vertigo, vomiting and ataxia lasting hours. It is not easy
to treat.
The common diseases to affect balance without hearing loss are
shown in the lower part of Fig. 8.12. It can be seen that the lesion
is likely to be central in the brainstem, or peripheral in the
labyrinth.
Episodes of ischaemia or infarction in the brainstem, or
episodes of demyelination in patients with multiple sclerosis, are
the common structural lesions in the brainstem to disturb balance. Such lesions commonly produce other neurological signs
(cranial nerve, cerebellar or long tract in the limbs).
The diagnosis of benign paroxysmal positional vertigo is
suggested by the occurrence of intermittent transient vertigo
lasting less than 30 seconds strongly related to putting the head
into a specific position. Turning over in bed, lying down in bed
and looking upwards are common precipitants. No abnormalities are found on examination except for definite positional
nystagmus. Spontaneous resolution of the problem occurs after
a few months.
Perhaps the commonest type of severe vertigo is due to
sudden vestibular failure. This denotes the sudden occurrence
of rotatory vertigo, gait ataxia, vomiting and the need to stay
in bed. Lateralized nystagmus and gait ataxia are the two
abnormal physical signs. The incapacity lasts very severely
for a few days and then gradually resolves over 46 weeks.
Head movement aggravates the symptoms so the patient keeps
still in bed in the acute stage, and walks with his head rather set
on his shoulders in the convalescent stage. The underlying
pathology is not certain. The problem may follow an upper respiratory infection and occasionally occurs in epidemics, hence
the use of the diagnostic terms acute labyrinthitis or vestibular
neuronitis.
Drugs that impair balance include:
amino-glycoside antibiotics, such as streptomycin and gentamicin, which may permanently impair vestibular function
if toxic blood levels are allowed to accumulate;
anticonvulsants, barbiturates and alcohol which impair the
function of the brainstem/cerebellum whilst blood levels are
too high.
CHAPTER 8
129
Spinal accessory (11) nerve

This nerve arises from the upper segments of the cervical spinal
cord, ascends into the skull through the foramen magnum, only
to exit the skull again with the 9th and 10th cranial nerves
through the jugular foramen. The nerve then travels down the
side of the neck to supply the sternomastoid muscle, and then
crosses the posterior triangle of the neck quite superficially to
supply the upper parts of the trapezius muscle.
Lesions of this nerve are uncommon. It is very vulnerable to
surgical trauma in the posterior triangle of the neck. Loss of
function in the upper part of the trapezius muscle produces a
significant disability in the shoulder region. The scapula and
shoulder sag downwards and outwards in the resting position.
Arm elevation is impaired because of poor scapular stability
and rotation, as illustrated in Fig. 8.13.
(a)
(b)
(c)
A patient, seen from behind, with a right-sided accessory nerve palsy: (a) at rest, (b) attempting to
lift his arms to the horizontal position, and (c) attempting to lift his arms as high as possible
(the scapulae are shown in green)
The first half of the shoulder abduction requires good scapula stabilization by the trapezius (and
other muscles), so that deltoid muscle contraction can take the arm to the horizontal position
The second half of the shoulder abduction requires elevation of the shoulder and scapula rotation
through almost 90 by the trapezius (and other muscles)
Fig. 8.13 The importance of the trapezius muscle in arm elevation.
130
CHAPTER 8
Glossopharyngeal (9), vagus (10) and hypoglossal

(12) nerves
These three lower cranial nerves are considered in one section of
this chapter for two reasons.
1. Together they innervate the mouth and throat for normal
speech and swallowing.
2. They are commonly involved in disease processes together,
to give rise to the clinical picture of bulbar palsy.
More specifically, the glossopharyngeal nerve supplies
the palate and pharynx, the vagus nerve supplies the pharynx
and larynx, and the hypoglossal nerve supplies the tongue.
Taste perception in the posterior third of the tongue is a function
of the glossopharyngeal nerve. Both the glossopharyngeal
and vagus nerves (especially the latter) have an enormous
autonomic function, as shown in Fig. 8.14. Innervation of the
vocal cords by the long, thin, recurrent laryngeal nerves (from
the vagus) exposes them to possible damage as far down as the
subclavian artery on the right, and the arch of the aorta on the
left.
Midbrain
Pons
Medulla
9
Spinal cord
10
Palate
Larynx
Pharynx
Tongue
12
+ vast autonomic
innervation of
salivary glands
heart
great vessels in
abdomen
neck
thorax
Fig. 8.14 Highly diagrammatic

representation of cranial nerves 9,
10 and 12.
131
Bulbar palsy
When there is bilateral impairment of function in the 9th, 10th
and 12th cranial nerves, the clinical syndrome of bulbar palsy
evolves. The features of bulbar palsy are:
dysarthria;
dysphagia, often with choking episodes and/or nasal regurgitation of fluids;
dysphonia and poor cough, because of weak vocal cords;
susceptibility to aspiration pneumonia.
Even though the vagus nerve has a vast and important autonomic role, it is uncommon for autonomic abnormalities to feature in the diseases discussed in this section.
Common conditions affecting 9th, 10th and

12th nerve function
Figure 8.15 illustrates the common conditions which affect 9th,
10th and 12th nerve function. The use of the word common is
relative, since none of the conditions are very common.
Palate
Larynx
Pharynx
Tongue
Fig. 8.15 Common conditions

that affect cranial nerves 9, 10, and
12. *Because these conditions
involve 9th, 10th and 12th nerve
function bilaterally, they are the
common causes of bulbar palsy.
Motor neurone disease*

Cerebrovascular disease
Syringobulbia
Erosive tumours of the
skull base
GuillainBarr syndrome*
Recurrent laryngeal nerve
palsy
Myasthenia gravis*
132

When motor neurone disease is causing loss of motor neurones
from the lower cranial motor nuclei in the medulla, the bulbar
palsy can eventually lead to extreme difficulty in speech
(anarthria) and swallowing. Inanition and aspiration pneumonia are commonly responsible for such patients deaths. The
tongue is small, weak or immobile, and fasciculating (Chapter
10, see pp. 1557).
Infarction of the lateral medulla
Infarction of the lateral medulla, following posterior inferior
cerebellar artery occlusion, is one of the most dramatic
cerebrovascular syndromes to involve speech and swallowing.
Ipsilateral trigeminal, vestibular, glossopharyngeal and vagal
nuclei may be involved, along with cerebellar and spinothalamic fibre tracts in the lateral medulla.
GuillainBarr syndrome
Patients with GuillainBarr syndrome, acute, post-infectious
polyneuropathy (Chapter 10, see p. 163), may need ventilation via an endotracheal tube or cuffed tracheostomy tube.
This may be necessary either because of neuropathic weakness of the chest wall and diaphragm, or because of bulbar
palsy secondary to lower cranial nerve involvement in the
neuropathy.
Recurrent laryngeal nerve palsy
The recurrent laryngeal nerves are vulnerable to damage in the
neck and mediastinum, e.g. aortic aneurysm, malignant chest
tumours, malignant glands and surgery in the neck (especially
in the region of the thyroid gland). A unilateral vocal cord palsy
due to a unilateral nerve lesion produces little disability other
than slight hoarseness. Bilateral vocal cord paralysis is much
more disabling, with marked hoarseness of the voice, a weak
bovine cough (because the cords cannot be strongly adducted)
and respiratory stridor.
Myasthenia gravis
Bulbar muscle involvement in myasthenia gravis is quite
common in this rare condition. The fatiguability of muscle function, which typifies myasthenia, is frequently very noticeable
in the patients speech and swallowing (Chapter 10, see pp.
1646).
CHAPTER 8
133
Dysarthria
The speech disturbance in patients with dysarthria is a purely
mechanical one caused by defective movement of the lips,
tongue, palate, pharynx and larynx. Clear pronunciation of
words is impaired due to the presence of a neuromuscular
lesion.
Speaking is a complex motor function. Like complex movement of other parts of the body, normal speech requires the integrity of basic components of the nervous system, mentioned
in Chapter 1, and illustrated again in Fig. 8.16. There are characteristic features of the speech when there is a lesion in each element of the nervous system identified in Fig. 8.16. These are the
different types of dysarthria.
Slurred speech
Upper motor neurone
Lower motor neurone
Muscle
Basal ganglia
Fig. 8.16 Basic components of the nervous system required for normal movement.
5
Jaw muscles
7
Facial muscles
Larynx muscles
Fig. 8.17 The upper and lower

motor neurones involved in
speech.
Pharynx muscles
10
10
12
12
Tongue muscles
134
CHAPTER 8
It is important to remember the availability of communication aids for patients with severe dysarthria. These may be quite
simple picture or symbol charts, alphabet cards or word charts.
More high tech portable communication aids that incorporate
keyboards and speech synthesizers are also very valuable for
some patients.
Upper motor neurone lesions

The upper motor neurones involved in speech have their cell
bodies at the lower end of the precentral (motor) gyrus in each
cerebral hemisphere. From the motor cortex, the axons of these
cells descend via the internal capsule to the contralateral cranial
nerve nuclei 5, 7, 9, 10 and 12, as shown in Fig. 8.17.
A unilateral lesion does not usually produce a major problem
of speech pronunciation. There is some slurring of speech due to
facial weakness in the presence of a hemiparesis.
Bilateral upper motor neurone lesions, on the other hand,
nearly always produce a significant speech disturbance. Weakness of the muscles supplied by cranial nerves 512 is known as
bulbar palsy if the lesion is lower motor neurone in type (see the
next section in this chapter). It is known as pseudobulbar palsy
if the weakness is upper motor neurone in type. Patients who
have bilateral upper motor neurone weakness of their lips, jaw,
tongue, palate, pharynx and larynx, i.e. patients with pseudobulbar palsy, have a characteristic speech disturbance, known
as a spastic dysarthria. The speech is slow, indistinct, laboured
and stiff. Muscle wasting is not present, the jaw-jerk is increased, and there may be associated emotional lability. The patient is likely to be suffering from bilateral cerebral hemisphere
cerebrovascular disease, motor neurone disease or serious
multiple sclerosis.
Lower motor neurone lesions, and lesions in
the neuromuscular junction and muscles
The lower motor neurones involved in speech have their cell
bodies in the pons and medulla (Fig. 8.17), and their axons
travel out to the muscles of the jaw, lips, tongue, palate, pharynx
and larynx in cranial nerves 512.
Asingle unilateral cranial nerve lesion does not usually produce
a disturbance of speech, except in the case of cranial nerve 7. A
severe unilateral facial palsy does cause some slurring of speech.
Multiple unilateral cranial nerve lesions are very rare.
Bilateral weakness of the bulbar muscles, whether produced
by pathology in the lower motor neurones, neuromuscular
junction or muscles, is known as bulbar palsy. One of the predominant features of bulbar palsy is the disturbance of speech.
The other main features are difficulty in swallowing and incom-
BG
Speech is slow, indistinct,

laboured and stiff in patients
with pseudobulbar palsy
BG
Speech is quiet, indistinct

and nasal in patients with
bulbar palsy
135
petence of the larynx leading to aspiration pneumonia. The

speech is quiet, indistinct, with a nasal quality if the palate is
weak, poor gutterals if the pharynx is weak, and poor labials if
the lips are weak. (Such a dysarthria may be rehearsed if one
tries to talk without moving lips, palate, throat and tongue.)
Motor neurone disease, GuillainBarr syndrome and myasthenia gravis all cause bulbar palsy due to lesions in the cranial
nerve nuclei, cranial nerve axons and neuromuscular junctional regions of the bulbar muscles, respectively (see Chapter 10
and Fig. 8.15).
BG
Parkinsonian patients
have quiet, indistinct,
monotonous speech
BG
Speech is slurred, and

irregular in volume
and timing
Basal ganglion lesions

The bradykinesia of Parkinsons disease causes the characteristic dysarthria of this condition. The speed and amplitude of
movements are reduced. Speech is quiet and indistinct, and
lacks up and down modulation. A monotonous voice from a
fixed face, both voice and face lacking lively expression, is the
typical state of affairs in Parkinsons disease.
Patients with chorea may have sudden interference of their
speech if a sudden involuntary movement occurs in their respiratory, laryngeal, mouth or facial muscles.
Cerebellar lesions
As already mentioned, the dysarthria of patients with cerebellar
disease often embarrasses them because their speech sounds as
if they are drunk. There is poor coordination of muscular action,
of agonists, antagonists and synergists. There is ataxia of the
speaking musculature, very similar to the limb ataxia seen in
patients with cerebellar lesions. Speech is irregular, in both volume and timing. It is referred to as a scanning or staccato
dysarthria.
Drugs that affect cerebellar function (alcohol, anticonvulsants), multiple sclerosis, cerebrovascular disease and posterior
fossa tumours are some of the more common causes of cerebellar malfunction.
136
CHAPTER 8
CASE HISTORIES
Can you identify the most likely problem in each of
these brief histories, and suggest a treatment?
a. I could see perfectly well last week.The right eye is
still OK but the left one is getting worse every day.
I cant see colours with it and I cant read small
print. It hurts a bit when I look to the side.
b. I see two of everything, side by side, but only when
I look to the right.Apart from that, Im fine.
c. Im getting terrible pains on the left of my face.
I darent touch it but its just at the corner of my
mouth, going down into my chin. Its so sharp,
it makes me jump.
d. Ive had an ache behind my ear for a couple of
days but this problem started yesterday. Im
embarrassed, I look so awful. I cant close my left

eye, it keeps running. My mouth is all over to the
right, Im slurring my words, and making a real
mess when I drink.
e. I keep bumping into doorways and last week
I drove into a parked car: I just didnt realize it
was there. I think my eyesight is perfect but the
optician said I needed to see a doctor straight
away.
f. I keep getting terribly dizzy. Rolling over in bed is
the worst: everything spins and I feel sick. Its the
same when I turn my head to cross the road. I am
too frightened to go out.
CHAPTER 9
Nerve root, nerve plexus

and peripheral nerve
lesions
Introduction
In this chapter, we are considering focal pathology in the
peripheral nervous system. This means a study of the effect of
lesions between the spinal cord and the distal connections of the
peripheral nerves with skin, joints and muscles (as shown in
Fig. 9.1). We shall become familiar with focal disease affecting
nerve roots and spinal nerves, nerve plexuses and individual
peripheral nerves. Focal disease infers a single localized lesion,
affecting one nerve root or one peripheral nerve. Diffuse or generalized diseases affecting these parts of the nervous system,
e.g. a peripheral neuropathy affecting all the peripheral nerves
throughout the body, are the subject of Chapter 10.
Focal lesions of the lower cervical and lower lumbar nerve
roots are common, as are certain individual peripheral nerve lesions in the limbs. Accurate recognition of these clinical syndromes depends on some basic neuro-anatomical knowledge.
This is not formidably complicated but possession of a few hard
anatomical facts is inescapable.
Spinal cord
Skin, joints, etc.

Nerve roots
and spinal
nerve
Nerve plexus
Peripheral nerve
(broken lines
indicate length)
Muscle
Fig. 9.1 Schematic diagram of the peripheral nervous system.
137
138
Nerve root lesions

Figure 9.2 is a representation of the position of the nerve roots
and spinal nerve in relation to skeletal structures. The precise
position of the union of the ventral and dorsal nerve roots, to
form the spinal nerve, in the intervertebral foramen is a little
variable. This is why a consideration of the clinical problems
affecting nerve roots embraces those affecting the spinal
nerve. A nerve root lesion, or radiculopathy, suggests a lesion
involving the dorsal and ventral nerve roots and/or the spinal
nerve.
The common syndromes associated with pathology of the
nerve roots and spinal nerves are:
prolapsed intervertebral disc;
herpes zoster;
metastatic disease in the spine.
Less common is the compression of these structures by a
neurofibroma.
Prolapsed intervertebral disc

When the central, softer material, nucleus pulposus, of an intervertebral disc protrudes through a tear in the outer skin, annulus fibrosus, the situation is known as a prolapsed intervertebral
disc. This is by far the most common pathology to affect nerve
roots and spinal nerves. The susceptibility of these nerve
elements to disc prolapses, which are most commonly posterolateral in or near the intervertebral foramen, is well shown in
Fig. 9.2.
The typical clinical features of a prolapsed intervertebral disc,
regardless of the level, are:
1. Skeletal:
pain, tenderness and limitation in the range of movement
in the affected area of the spine;
reduced straight leg raising on the side of the lesion, in the
case of lumbar disc prolapses.
2. Neurological:
pain, sensory symptoms and sensory loss in the dermatome of the affected nerve root;
lower motor neurone signs (weakness and wasting) in the
myotome of the affected nerve root;
loss of tendon reflexes of the appropriate segmental value;
since most disc prolapses are posterolateral, these neurological features are almost always unilateral.
CHAPTER 9
PERIPHERAL NERVOUS SYSTEM
139
Spinous process
Spinal cord
Lamina
Dorsal root and ganglion
Ventral root
Intervertebral facet joint
Pedicle
Body of vertebrae, separated
from each other by
intervertebral discs
Spinal nerve passing through
the intervertebral foramen
(a)
(b)
Fig. 9.2 Diagrams showing the superior aspect of a cervical vertebra, and the lateral aspect of the lumbar
spine. Disc prolapse in the cervical region can cause cord and/or spinal nerve root compression (scan a).
Disc prolapse in the lumbar region (scan b) can cause nerve root compression, but the spinal cord ends
alongside L1 (asterisk) and is unaffected. In either region, additional degenerative changes in the facet
joints may aggravate the problem.
140
CHAPTER 9
Prolapsed intervertebral discs are most common between C4

and T1 in the cervical spine and between L3 and S1 in the lumbosacral spine. In the cervical region, there is not a great discrepancy between the level of the cervical spinal cord segment
and the cervical vertebra of the same number, i.e. the C5 segment of spinal cord, the C5 nerve roots and the C4/5 intervertebral foramen, through which the C5 spinal nerve passes, are all
at much the same level (see Fig. 6.1, p. 83). If the patient presents
with a C5 neurological deficit, therefore, it is very likely that it
will be a C4/5 intervertebral disc prolapse.
Figures 6.1 and 9.3 show that this is not the case in the lumbar
region. The lower end of the spinal cord is at the level of the L1
vertebra. All the lumbar and sacral nerve roots have to descend
Common nerve roots to be

compressed by prolapsed
intervertebral discs:
In the arm C5 In the leg L4
C6
L5
C7
S1
C8
Spinal cord
Vertebral body
Theca, i.e. spinal

dura mater
Lumbosacral nerve roots

forming the cauda equina,
in the subarachnoid space,
within the theca
Intervertebral
disc
Spinal nerve, with dural sleeve,

leaving the spinal canal via an
intervertebral foramen
Sacrum
Fig. 9.3 Posterior view of the

cauda equina. NB The pedicles,
laminae and spinous processes of
the vertebrae, and the posterior
half of the theca, have been
removed.
141
over a considerable length to reach the particular intervertebral

foramen through which they exit the spinal canal. These nerve
roots form the cauda equina, lying within the theca. Each nerve
root passes laterally, within a sheath of dura, at the level at
which it passes through the intervertebral foramen. Posterolateral disc prolapses are likely to compress the emerging spinal
nerve within the intervertebral foramen, e.g. an L4/5 disc
prolapse will compress the emerging L4 root. More medially
situated disc prolapses in the lumbar region may compress
nerve roots of lower numerical value, which are going to exit
the spinal canal lower down. This is more likely to happen if the
patient has a constitutionally narrow spinal canal. (Some individuals have wide capacious spinal canals, others have short
stubby pedicles and laminae to give a small cross-sectional area
for the cauda equina.) It cannot be assumed therefore that an L5
root syndrome is the consequence of an L5/S1 disc prolapse; the
trouble may be higher up. A more centrally prolapsed lumbar
disc may produce bilateral leg symptoms and signs, involving
more than one segment, often associated with sphincter malfunction due to lower sacral nerve root compression.
Figures 9.4 and 9.5 show the segmental value of the movements, reflexes and skin sensation most frequently involved in
cervical and lumbar disc disease. From these diagrams, the area
of pain and sensory malfunction, the location of weakness and
wasting, and the impaired deep tendon reflexes can all be identified for any single nerve root syndrome. (Note that Figs 9.4 and
9.5 indicate weak movements, not the actual site of the weak
and wasted muscles, which are of course proximal to the joints
being moved.)
C7
T1
C7
C8
C7
C8
C8
C6
C5/6
C5
C7/8
C5
T1
Biceps jerk C5/6

Triceps jerk C7/8
T2
Supinator jerk C5/6
Fig. 9.4 Segmental nerve supply to the upper limb, in terms of movements, tendon reflexes and skin
sensation.
142
CHAPTER 9
L2/3
L4/5/S1
L5/S1
L3/4
L4/5
S1/2
Knee jerk L3/4

(NB no jerk for L5)
Ankle jerk S1/2
L2/3
S2
L4/5
S1
There are four main intervertebral disc disease syndromes.

1. The single, acute disc prolapse which is sudden, often related
to unusually heavy lifting or exertion, painful and very incapacitating, often associated with symptoms and signs of nerve
root compression, whether it affects the cervical or lumbar
region.
2. More gradually evolving, multiple-level disc herniation in
association with osteo-arthritis of the spine. Disc degeneration
is associated with osteophyte formation, not just in the main
intervertebral joint between body and body, but also in the
Fig. 9.5 Segmental nerve supply

to the lower limb, in terms of
movements, tendon reflexes and
skin sensation.
143
intervertebral facet joints. Figure 9.2 shows how osteoarthritic changes in the intervertebral facet joint may further
encroach upon the space available for the emerging spinal
nerve in the intervertebral foramen. This is the nature of nerve
root involvement in cervical and lumbar spondylosis.
3. Cervical myelopathy (Chapter 6, see p. 91) when 1, or more
commonly 2 above, causes spinal cord compression in the cervical region. This is more likely in patients with a constitutionally
narrow spinal canal.
4. Cauda equina compression at several levels due to lumbar
disc disease and spondylosis, often in association with a constitutionally narrow canal, may produce few or no neurological
problems when the patient is at rest. The patient may develop
sensory loss in the legs or weakness on exercise. This syndrome
is not common, its mechanism is ill-understood, and it tends to
be known as intermittent claudication of the cauda equina.
Disc disease is best confirmed by MR scanning of the spine at
the appropriate level.
Most acute prolapsed discs settle spontaneously with analgesics. Patients with marked signs of nerve root compression,
with persistent symptoms or with recurrent symptoms, are
probably best treated by microsurgical removal of the prolapsed material.
Cervical and lumbar spondylosis are difficult to treat satisfactorily, even when there are features of nerve root compression.
Conservative treatment, analgesics, advice about bodyweight
and exercise, and the use of collars and spinal supports are the
more usual recommendations.
Symptomatic cauda equina compression is usually helped
by surgery. The benefit of surgical treatment for spinal cord
compression in the cervical region is less well proven.
Herpes zoster
Any sensory or dorsal root ganglion along the entire length of
the neuraxis may be the site of active herpes zoster infection.
The painful vesicular eruption of shingles of dermatome distribution is well known. Pain may precede the eruption by a few
days, secondary infection of the vesicles easily occurs, and pain
may occasionally follow the rash on a long-term basis (postherpetic neuralgia). The dermatome distribution of the shingles
rash is one of the most dramatic living neuro-anatomical lessons to witness.
The healing of shingles is probably not accelerated by the topical application of antiviral agents. In immunocompromised
patients aciclovir should be given systemically. It is not clear
that antiviral treatment prevents post-herpetic neuralgia.
144
Spinal tumours
Pain in the spine and nerve-root pain may indicate the presence
of metastatic malignant disease in the spine. More occasionally,
such pains may be due to a benign tumour such as a neurofibroma. The root pain may be either unilateral or bilateral. It is
known as girdle pain when affecting the trunk, i.e. between T3
and L2. Segmental neurological signs in the form of lower motor
neurone weakness, deep tendon reflex loss, and dermatome
sensory abnormality may be evident, but the reason for early
diagnosis and management is to prevent spinal cord compression, i.e. motor, sensory and sphincter loss below the level
of the lesion (Chapter 6, see pp. 902).
Brachial and lumbosacral plexus lesions

Lesions of these two nerve plexuses are not common, so they
will be dealt with briefly. Of the two, brachial plexus lesions are
the more common. In both instances, pain is a common symptom, together with sensory, motor and deep tendon reflex loss in
the affected limb.
Spinal nerves from C5 to T1 contribute to the brachial plexus,
which runs from the lower cervical spine to the axilla, under the
clavicle, and over the first rib and lung apex. Lesions of the
brachial plexus are indicated in Fig. 9.6.
Spinal nerves from L2 to S2 form the lumbosacral plexus,
which runs downwards in the region of the iliopsoas muscle,
over the pelvic brim to the lateral wall of the pelvis. The common pathology to affect this plexus is malignant disease, especially gynaecological cancer in women.
CHAPTER 9
Trauma
Often very extensive damage
Usually a young man after a
motorcycle injury
Disappointing recovery
Malignancy
Particularly apical lung cancer
involving the lower elements of
the plexus, known as the
Pancoast tumour
As a consequence of metastases
or of radiotherapy for breast
cancer
Cervical rib
Lower elements of the plexus (C8, T1)
are compressed as they pass over
the rib to reach the axilla
There may be associated vascular
insufficiency in the hand, due to
subclavian artery compression
The 'rib' may be bone, or a fibrous
band running from the transverse
process of C7 vertebra
More common in women
Symptoms aggravated by carrying
anything heavy
Brachial neuritis
Uncommon patchy lesion of brachial
plexus causing initial pain, followed
by weakness, wasting, reflex and
some sensory loss
Good prognosis
Fig. 9.6 Lesions of the brachial plexus.
145
146
Peripheral nerve lesions

Individual peripheral nerves in the limbs may be damaged by
any of five mechanisms.
1. Trauma: in wounds created by sharp objects such as knives or
glass (e.g. median or ulnar nerve at the wrist), by inaccurate localization of intramuscular injections (e.g. sciatic nerve in the
buttock), or by the trauma of bone fractures (e.g. radial nerve in
association with a midshaft fracture of the humerus).
2. Acute compression: in which pressure from a hard object is exerted on a nerve. This may occur during sleep, anaesthesia or
coma in which there is no change in the position of the body to
relieve the compression (e.g. radial nerve compression against
the posterior aspect of the humerus, common peroneal nerve
against the lateral aspect of the neck of the fibula).
3. Iatrogenically: following prolonged tourniquet application
(e.g. radial nerve in the arm), or as a result of an ill-fitting plaster
cast (e.g. common peroneal nerve in the leg).
4. Chronic compression: so-called entrapment neuropathy,
which occurs where nerves pass through confined spaces
bounded by rigid anatomical structures, especially near to
joints (e.g. ulnar nerve at the elbow or median nerve at the
wrist).
5. As part of the clinical picture of multifocal neuropathy. There
are some conditions that can produce discrete focal lesions in individual nerves, so that the patient presents with more than one
nerve palsy either simultaneously or consecutively (e.g. leprosy, diabetes and vasculitis).
The speed and degree of recovery from injury or compression
obviously depends on the state of the damaged nerve. No recovery will occur if the nerve is severed, unless it is painstakingly stitched together soon after injury by surgery. Damage
which has injured the nerve sufficiently to cause axonal destruction will require regrowth of axons distally from the site of
injury, a process which tends to be slow and incompletely efficient. Damage which has left the axons intact, and has only
injured the myelin sheaths within the nerve, recovers well.
Schwann cells reconstitute myelin quickly around intact axons.
Some peripheral nerve palsies are more common than others.
Figures 9.7 and 9.8 show the common and uncommon nerve lesions in the upper and lower limbs, respectively. Brief notes
about the uncommon nerve palsies are shown. The remainder
of this chapter deals with the three common nerve palsies in the
upper limbs and the two common ones in the legs.
CHAPTER 9
Common
147
Uncommon
Long thoracic nerve
Paralysis of serratus anterior
Winging of the scapula when
arms held forward
Radial nerve
Mid humerus
Acute compression
or trauma
Axillary or circumflex nerve
Damaged by shoulder dislocation
Weak deltoid, i.e. shoulder
abduction
Sensory loss just below shoulder
Ulnar nerve
Elbow
Chronic compression
Median nerve
Wrist
Chronic compression
Musculocutaneous nerve
Damaged by fracture of humerus
Weak biceps, i.e. elbow flexion
Sensory loss down lateral
forearm
Absent biceps jerk
Posterior interosseous nerve

Site of occasional chronic
compression
Like radial nerve palsy, except
brachioradialis and wrist
extensors intact, and no
sensory loss
Deep palmar branch of ulnar nerve

Site of occasional chronic
compression
Like ulnar nerve palsy, except
little finger abduction intact,
and no sensory loss
Fig. 9.7 Peripheral nerve palsies of the upper limb.
148
CHAPTER 9
Common
Uncommon
Obturator nerve
Damaged by trauma in labour
and by pelvic cancer
Weak hip adduction
Sensory loss down inner aspect
of thigh
Lateral cutaneous
nerve of thigh
Inguinal ligament
Chronic compression
Sciatic nerve
Damaged by acute compression
during coma, by misplaced
intramuscular injections, and
by hip dislocation and surgery
Weak knee flexion, and all muscles
below the knee
Sensory loss throughout foot
and over lateral calf
Absent ankle jerk
Common peroneal
nerve
Neck of fibula
Trauma, acute or
chronic compression
Femoral nerve
Damaged by trauma,
haematoma, psoas abscess
Weak quadriceps, i.e. knee
extension
Sensory loss on front of thigh
Absent knee jerk
Posterior tibial nerve

Damaged by trauma and tibial
fractures
Weak foot and toe plantar flexion
Sensory loss on sole of foot
Absent ankle jerk
Fig. 9.8 Peripheral nerve palsies of the lower limb.
149
Radial nerve palsy (Fig. 9.9)

The nerve is most usually damaged where it runs down the posterior aspect of the humerus in the spiral groove. This may occur
as a result of acute compression, classically when a patient has
gone to sleep with his arm hanging over the side of an armchair
(Saturday night palsy!). It may occur in association with fractures of the midshaft of the humerus.
The predominant complaint is of difficulty in using the hand,
because of wrist drop. The finger flexors and small hand muscles are greatly mechanically disadvantaged by the presence of
wrist, thumb and finger extensor paralysis. There are usually no
sensory complaints.
The prognosis is good after acute compression, and more
variable after damage in association with a fractured humerus.
The function of the hand can be helped by the use of a special
lively splint which holds the wrist, thumb and fingers in partial extension.
Motor loss
Reflex loss
Brachioradialis
Wrist extensors
Finger extensors
Thumb extensors
and abductor
Absent brachioradialis
(supinator) jerk
Nerve sensitivity
Usually none
Fig. 9.9 Radial nerve palsy.
Sensory loss
150
CHAPTER 9
Ulnar nerve palsy (Fig. 9.10)

The commonest aetiology is chronic compression, either in the
region of the medial epicondyle, or a little more distally where
the nerve enters the forearm between the two heads of flexor
carpi ulnaris. Sometimes the nerve is compressed acutely in this
vicinity during anaesthesia or a period of enforced bedrest (during which the patient supports himself on the elbows whilst
moving about in bed). The nerve may be damaged at the time of
fracture involving the elbow, or subsequently if arthritic change
or valgus deformity are the consequence of the fracture involving the elbow joint.
The patient complains of both motor and sensory symptoms,
lack of grip in the hand, and painful paraesthesiae and numbness affecting the little finger and the ulnar border of the palm.
Persistent compression can be relieved by transposing the
compressed nerve from behind to the front of the elbow joint.
This produces good relief from the unpleasant sensory symptoms, and reasonable return of use of the hand even though
there may be residual weakness and wasting of small hand
muscles on examination.
Motor loss
Reflex loss
Flexor carpi ulnaris

Ulnar half of flexor
digitorum profundus
All the small muscles
of the hand except
abductor pollicis brevis
None
Sensory loss
Nerve sensitivity
Often quite marked
on the medial side
of the elbow
Fig. 9.10 Ulnar nerve palsy.
151
Median nerve palsy (Fig. 9.11)

Carpal tunnel syndrome is the commonest clinical expression
of median nerve palsy, and is probably the commonest nerve
entrapment syndrome. The median nerve becomes chronically
compressed within the carpal tunnel, which consists of the bony
carpus posteriorly and the flexor retinaculum anteriorly. The
carpal tunnel has a narrower cross-sectional area in women
than men, and patients with carpal tunnel syndrome have a significantly narrower cross-sectional area in their carpal tunnels
than a control population.
Carpal tunnel syndrome is five times more common in
women than men. It is more common in patients who have
arthritis involving the carpus, and is more frequent in pregnancy, diabetes, myxoedema and acromegaly.
The patient complains of sensory symptoms. Painful paraesthesiae, swollen burning feelings, are felt in the affected hand
and fingers, but often radiate above the wrist as high as the
elbow. They frequently occur at night and interrupt sleep. They
may occur after using the hands and arms. They are commonly
relieved by shaking the arms. There are not usually any motor
symptoms, except for possible impairment of manipulation of
small objects between a slightly numb thumb, index and middle
fingers.
Symptomatic control may be established by wearing a wristimmobilizing splint or by the use of hydrocortisone injection
into the carpal tunnel. Permanent relief often requires surgical
division of the flexor retinaculum, a highly effective and gratifying minor operation.
Motor loss
Reflex loss
Abductor pollicis
brevis
None
Nerve sensitivity
Sometimes at the
level of the carpus
Fig. 9.11 Median nerve palsy.
Sensory loss
152
CHAPTER 9
Common peroneal nerve palsy (Fig. 9.12)

The common peroneal (lateral popliteal) nerve runs a very superficial course around the neck of the fibula. It divides into the
peroneal nerve to supply the lateral calf muscles which evert the
foot, and into the anterior tibial nerve which innervates the anterior calf muscles which dorsiflex the foot and toes. The nerve
is liable to damage from trauma, with or without fracture of the
fibula. It is highly susceptible to acute compression during
anaesthesia or coma, and from overtight or ill-fitting plaster
casts applied for leg fractures.
The patients predominant complaint is of foot drop, and the
need to lift the leg up high when walking. He may complain of
loss of normal feeling on the dorsal surface of the affected ankle
and foot.
Treatment of common peroneal nerve palsy should be preventative wherever possible. A splint that keeps the ankle at a
right angle may assist walking. The condition is not usually
helped by any form of surgery.
Common peroneal nerve palsies due to acute compression
have a good prognosis.
Motor loss
Reflex loss
Foot evertors
Foot dorsiflexors
Toe dorsiflexors
None
Sensory loss
Nerve sensitivity
Sometimes at the
neck of the fibula
Fig. 9.12 Common peroneal

nerve palsy.
153
Meralgia paraesthetica (Fig. 9.13)

This irritating syndrome is the consequence of chronic entrapment of the lateral cutaneous nerve of the thigh as it penetrates
the inguinal ligament, or deep fascia, in the vicinity of the anterior superior iliac spine.
As the nerve is sensory, there are no motor symptoms. The
patient complains of annoying paraesthesiae and partial
numbness in a patch of skin on the anterolateral aspect of the
thigh. The contact of clothes is slightly unpleasant in the
affected area.
No treatment of meralgia paraesthetica may be necessary if
the symptoms are mild. Alternatively, nerve decompression or
section at the site of compression both give excellent relief.
Motor loss
Reflex loss
None
None
Nerve sensitivity
Usually none
Fig. 9.13 Lateral cutaneous nerve

of the thigh.
Sensory loss
154
CHAPTER 9
CASE HISTORIES
Which nerve or nerve root is the likely culprit in the
following brief histories?
a. A middle-aged man who has difficulty walking
because of weak dorsiflexion of the right foot.
b. A young man who cannot use his right hand
properly and is unable to dorsiflex the right wrist.
c. A middle-aged lady who is regularly waking at

night due to strong tingling and numbness of her
hands and fingers.
d. An elderly lady with persistent tingling in her left
little finger, and some weakness in the use of the
same hand.
10
CHAPTER 10
Motor neurone disease,

peripheral neuropathy,
myasthenia gravis and
muscle disease
Introduction
Fig. 10.1 The common

peripheral neuromuscular
disorders.
This chapter is about the common disorders that affect the

peripheral nervous system and muscle (Fig. 10.1). They tend to
produce a clinical picture of diffuse muscle weakness and wasting, and are sometimes difficult to distinguish from one another.
Generalized wasting, fasciculation

and weakness of muscles
Bulbar muscle involvement common
Associated upper motor neurone
symptoms and signs
No sensory symptoms and signs
Steadily progressive and fatal
Distal wasting and weakness of

muscles
Legs more involved than arms, and
bulbar involvement very rare
Associated distal sensory symptoms
and signs
Many causes, several of which have
specific treatment and are reversible
Myasthenia gravis
Muscle disease
X
Muscle weakness without wasting
Weakness which varies in severity and
which fatigues
Ocular and bulbar muscles commonly
involved
Responds well to treatment
X
Muscle weakness and wasting, the
distribution of which depends on
the type of disease, but with a strong
tendency to involve proximal muscles,
i.e. trunk and limb girdles
Some inherited and incurable, others
inflammatory or metabolic and treatable
155
156
CHAPTER 10

This disease consists of a selective loss of lower motor neurones
from the pons, medulla and spinal cord, together with loss of
upper motor neurones from the motor cortex of the brain. The
process is remarkably selective, leaving special senses, and
cerebellar, sensory and autonomic functions intact. Progressive
difficulty in doing things because of muscular weakness gradually overtakes the patient.
The cause of the neuronal loss in motor neurone disease (like
the selective loss of neurones from other parts of the CNS,
in diseases such as Alzheimers and Parkinsons) is not
understood.
There is variation in the clinical picture of motor neurone
disease from one patient to another, which depends on:
whether lower or upper motor neurones are predominantly
involved;
which muscles (bulbar, upper limb, trunk or lower limb) are
bearing the brunt of the illness;
the rate of cell loss. Most usually, this is steadily progressive
over a few years, but in a minority of cases it may be much
more gradual with long survival.
Motor neurone disease (MND) tends to start either as a problem in the bulbar muscles, or as a problem in the limbs, and initially the involvement tends to be either lower motor neurone
or upper motor neurone in nature. This has led to four clinical
syndromes at the outset of the illness, which are described
in Fig. 10.2.
As the illness progresses and the loss of motor neurones becomes more generalized, there is a tendency for both upper and
lower motor neurone signs to become evident in bulbar, trunk
and limb muscles. Sometimes, the illness may remain confined
to the lower motor neurones, or to the upper motor neurones,
but the coexistence of both, in the absence of sensory signs, is the
hallmark of motor neurone disease. A limb with weak, wasted,
fasciculating muscles, in which the deep tendon reflexes are
very brisk, and in which there is no sensory loss, strongly suggests motor neurone disease.
It is the involvement of bulbar and respiratory muscles that
is responsible for the inanition and chest infections which
account for most of the deaths in patients with motor neurone
disease.
The glutamate antagonist riluzole slows progression and
prolongs survival, but there is as yet no cure for motor neurone
disease. This makes regular medical input more important, not
less, and the neurologist should be part of an integrated team of
professionals providing advice and support.
Key features of motor neurone

disease
Muscle weakness
Muscle wasting
Muscle fasciculation
Exaggerated reflexes
No loss of sensation
Bulbar weakness
The medulla oblongata used
to be referred to as the bulb of
the brain. The cranial nerve
nuclei 9, 10 and 12 reside in
the medulla. This is why
weakness of the muscles they
supply (mouth, pharynx and
larynx) is known as bulbar
weakness or bulbar palsy.
Bulbar palsy and pseudobulbar
palsy imply that the cause is
respectively in the lower
and upper motor neurones
supplying these muscles, and
have specific physical signs
(see pp. 131 and 1345 and Fig.
10.2). MND characteristically
causes a mixture of the two
PERIPHERAL NEUROMUSCULAR DISORDERS
157
Lower motor neurone
Upper motor neurone
Muscles supplied by the lower cranial nerves

Bulbar palsy
W eakness, wasting and
fasciculation of the lower facial
muscles, and muscles moving
the palate, pharynx, larynx and
tonguemost conspicuous in
the tongue
Pseudobulbar palsy
Weakness, slowness and
spasticity of the lower
facial muscles, jaw, palate,
pharynx, larynx and tongue
muscles
Exaggerated jaw-jerk
Emotional lability
Dysarthria, dysphagia, weight loss and the risk of inhalation

pneumonia are the clinical problems facing patients described above
Muscles of the limbs and trunk
Progressive muscular atrophy
Weakness, wasting and
fasciculation of any of the
limb or trunk muscles
Often associated with
frequent muscle cramps
No sensory loss
Small muscles of the hand
frequently involved
Amyotrophic lateral sclerosis

Weakness, spasticity,
clonus and increased
deep tendon reflexes
Any limb, but more
commonly in the legs
Sphincter control not
affected
No sensory loss
Fig. 10.2 The four clinical syndromes with which motor neurone disease may present.
The quality of life for patients can be helped by:

humane explanation of the nature of the condition to the patient and his family, with the aid of self-help groups;
sympathy and encouragement;
drugs for cramp, drooling and depression;
speech therapy, dietetic advice, and often percutaneous gastrostomy feeding for dysphagia;
communication aids for dysarthria;
non-invasive portable ventilators for respiratory muscle
weakness;
provision of aids and alterations in the house (wheelchairs,
ramps, lifts, showers, hoists, etc.) as weakness progresses;
nursing help and respite care;
gentle and timely discussion and planning of terminal care,
often involving hospice staff.
158
CHAPTER 10
In this section, we are focusing on the axon of the anterior horn
cell and the distal axon of the dorsal root ganglion cell. These
myelinated nerve fibres constitute the peripheral nerves (Fig.
10.3). Each peripheral nerve, in reality, consists of very many
myelinated nerve fibres.
In patients with peripheral neuropathy, there is malfunction
in all the peripheral nerves of the body. Two types of pathology
may occur. In some cases there may be distal axonal degeneration, explaining the distal distribution of symptoms and signs in
the limbs. Alternatively segments of the nerve fibres become demyelinated (Fig. 10.4). The normal saltatory passage of the
nerve impulse along the nerve fibre becomes impaired. The impulse either fails to be conducted across the demyelinated section, or travels very slowly in a non-saltatory way along the
axon in the demyelinated section of the nerve. This means that a
large volley of impulses, which should travel synchronously
along the component nerve fibres of a peripheral nerve,
become:
diminished as individual component impulses fail to be
conducted;
delayed and dispersed as individual impulses become
slowed by the non-saltatory transmission (Fig. 10.4). Neurotransmission is most impaired in long nerves under such circumstances simply because the nerve impulse is confronted
by a greater number of demyelinated segments along the
course of the nerve (Fig. 10.5). This is the main reason why the
Skin, joints,
etc.
Spinal cord
Peripheral nerve
Node of
Ranvier
Muscle
Myelin
Axon

peripheral nerve components.
159
Record
A
Stimulate
Healthy nerve
Spinal cord
Muscle
Record
B
Stimulate
Nerve affected by
segmental demyelination
Voltage
A Normal recorded muscle action
potential
Stimulate
B The action potential is small

(failure of some nerve impulses to
arrive at the muscle), and both delayed
and dispersed (slow neurotransmission
across segments of demyelinated
nerve fibre)
Stimulate
Time
Fig. 10.4 Nerve conduction in healthy and segmentally demyelinated nerve fibres.
symptoms of neuropathy due to segmental demyelination

are most evident distally in the limbs, why the legs and feet
are more affected than the arms and hands, and why the distal deep tendon reflexes (which require synchronous neurotransmission from stretch receptors in the muscle to the
spinal cord and back again to the muscle the reflex arc) are
frequently lost in patients with peripheral neuropathy.
The peripheral nerve pathology may predominantly affect
sensory axons, motor axons, or all axons. Accordingly, the
patients symptoms and signs may be distal and sensory in
the limbs, distal and motor in the limbs, or a combination of
both. They are shown in greater detail in Fig. 10.6.
Fig. 10.5 Effect of nerve length

upon neurotransmission in
peripheral neuropathy.
160
CHAPTER 10
Skin, joints,
etc.
Spinal cord
Muscle
Sensory
Motor
Glove distribution of
tingling, pins and needles
and numbness
Weakness of grip
and fingers
Reflex
Symptoms
Upper limbs
Difficulty in manipulating
small objects in the fingers
because of loss of
sensation
Lower limbs
Stocking distribution of
tingling, pins and needles
and numbness
Foot drop
Unsteadiness of stance
and gait, especially in
the dark or when eyes
closed
Loss of spring at
the ankles for
running and climbing
stairs
Glove distribution of
sensory loss, affecting
any sensory modality
Distal lower motor

neurone signs
in hands
Loss of distal
reflexes, e.g.
supinator jerks
Distal lower motor

neurone signs in
legs and feet
Loss of distal
reflexes, especially
ankle jerks
Signs
Upper limbs
Sensory ataxia in
fingers and hands
Lower limbs
Stocking distribution
of sensory loss, affecting
any sensory modality
Sensory ataxia in legs
and gait
Rombergism (i.e.
dependence on eyes
for balance)
Fig. 10.6 Symptoms and signs of peripheral neuropathy.
161
Common causes of peripheral neuropathy

In developed countries the commonest identifiable causes of
peripheral neuropathy are alcohol and diabetes. In other parts
of the world, vitamin deficiency and leprosy cause more
disease, although this is gradually changing. In both settings,
many cases of peripheral neuropathy remain unexplained.
Figure 10.7 lists some of the most important causes of peripheral neuropathy.
Alcoholic neuropathy
Alcoholic neuropathy is common and usually more sensory
than motor. How much it is caused by the direct toxic effect of
alcohol on the peripheral nerves, and how much it is due to
coexistent vitamin B1 deficiency, is not completely known.
Vitamin B12 deficiency
Vitamin B12 deficiency is not a common cause of neuropathy, but
is an important one to recognize because of its reversibility.
Every effort should be made to reach the diagnosis before the
irreversible changes of subacute combined degeneration of
the spinal cord become established.
Fig. 10.7 Common causes of

peripheral neuropathy in the UK.
Deficiency
Vitamin B1 in alcoholics
Vitamin B6 in patients taking isoniazid
Vitamin B12 in patients with pernicious
anaemia and bowel disease
Toxic
Alcohol
Drugs, e.g. isoniazid, vincristine,
aminodarone
Metabolic
Diabetes mellitus
Chronic renal failure
Inflammatory
Chronic inflammatory
demyelinating polyneuropathy
Paraneoplastic
Bronchial carcinoma and other

malignancies
Connective tissue disease
Rheumatoid arthritis
Systemic lupus erythematosus
Polyarteritis nodosa
Hereditary
Hereditary motor and sensory

neuropathy (HMSN) (also known as
CharcotMarieTooth disease)
Haematological
Paraproteinaemia
Idiopathic
Perhaps accounting for 50% of cases
162
CHAPTER 10
Diabetes mellitus
Diabetes mellitus is probably the commonest cause of peripheral neuropathy in the Western world. It occurs in both
juvenile-onset insulin-requiring diabetes and maturity-onset
diabetes. It may be the first clinical suggestion of the presence of
diabetes. Excellent diabetic control has been shown to prevent
neuropathy, but does not reverse it once it has developed.
The commonest form of neuropathy in diabetes is a predominantly sensory one. The combination of neuropathy and atherosclerosis affecting the nerves and arteries in the lower limbs
very strongly predisposes the feet of diabetic patients to trophic
lesions, which are slow to heal.
There are a few unusual forms of neuropathy that may occur
in patients with diabetes:
painful weakness and wasting of one proximal lower limb,
so-called diabetic lumbosacral radiculo-plexopathy or diabetic amyotrophy;
involvement of the autonomic nervous system giving rise to
abnormal pupils, postural hypotension, impaired cardioacceleration on changing from the supine to the standing
position, impaired bladder, bowel and sexual function, and
loss of normal sweating;
a tendency for individual nerves to stop working quite
abruptly, with subsequent gradual recovery. Common
nerves to be involved are the 3rd and 6th cranial nerves and
the common peroneal nerve in the leg. Involvement of
several individual nerves in this way constitutes the clinical
syndrome of multifocal neuropathy.
Hereditary motor and sensory neuropathy
(HMSN, also known as CharcotMarieTooth disease)
There are several forms of this, with a complex genetic classification. One of the more common, HMSN type I, is due to a duplication in the gene for peripheral myelin protein 22; this and
some other forms can be diagnosed with a genetic test. The illness is usually evident in teenage life and very slowly worsens
over many years. Motor involvement predominates, with
lower motor neurone signs appearing in the feet and legs
(especially in the anterolateral muscle compartments of the
calves), and in the small muscles of the hands. Pes cavus and
clawing of the toes are very common consequences. Sometimes,
the pathology primarily involves the axons, but more often
there is demyelination and remyelination to be found in the
peripheral nerves.
Key features of HMSN

Pes cavus
Distal wasting (champagne
bottle legs)
Distal weakness
Absent reflexes
Mild distal sensory loss
Causes of death
GuillainBarr syndrome can
be fatal, but most of the causes
are avoidable:
aspiration pneumonia
DVT and pulmonary
embolism
cardiac arrhythmia
So monitor bulbar function,
vital capacity and the heart,
and anticoagulate
163
GuillainBarr syndrome is rather different from the other
forms of peripheral neuropathy. This is because of its rapid
evolution over several days, because it can produce a lifethreatening degree of weakness, and because the underlying
pathology clearly affects the nerve roots as well as the peripheral nerves.
The syndrome commonly occurs a week or two after an infection, such as Campylobacter enteritis, which is thought to trigger
an autoimmune response.
The patient notices limb weakness and sensory symptoms
which worsen day by day for 12 weeks (occasionally the
progression may continue for as long as 4 weeks). Often, the
illness stops advancing after a few days and does not produce
a disability that is too major. Not uncommonly, however, it
progresses to cause very serious paralysis in the limbs, trunk
and chest muscles, and in the muscles supplied by the
cranial nerves. Involvement of the autonomic nerves may cause
erratic rises and falls in heart rate and blood pressure and profound constipation.
Patients with GuillainBarr syndrome need to be hospitalized until it is certain that deterioration has come to an end,
because chest and bulbar muscle weakness may make ventilation and nasogastric tube nutrition essential. Daily, or twice
daily, estimations of the patients vital capacity during the early
phase of the disease can be a very valuable way of assessing the
likelihood of the need for ventilatory support. Prompt administration of intravenous immunoglobulin or plasma exchange
can prevent deterioration and the need for ventilation in many
cases. Steroids have not been shown to be of proven benefit.
Patients with GuillainBarr syndrome become very
alarmed by the progressive loss of function at the start of
their illness. They often need a good deal of psychological and
physical support when the disability is severe and prolonged.
The ultimate prognosis is usually very good, however. Incomplete recovery and recurrence are both well described, but by far
the most frequent outcome of this condition is complete
recovery over a few weeks or months, and no further similar
trouble thereafter.
The pathology is predominantly in the myelin rather than in
the axons of the peripheral nerves and nerve roots, i.e. a demyelinating polyneuropathy and polyradiculopathy. Recovery
is due to the capability of Schwann cells to reconstitute the
myelin sheaths after the initial demyelination. The involvement
of the nerve roots gives rise to one of the diagnostic features of
the condition, a raised CSF protein.
164
CHAPTER 10
Myasthenia gravis
Myasthenia gravis is the rare clinical disease that results from
impaired neuromuscular transmission at the synapse between
the termination of the axon of the lower motor neurone and the
muscle, at the motor end plate. Figure 10.8 is a diagram of a
motor end plate. Neuromuscular transmission depends on
normal synthesis and release of acetylcholine into the gap
substance of the synapse, and its uptake by healthy receptors on
the muscle membrane. The main pathological abnormality
in myasthenia gravis at the neuromuscular junction is the
presence of auto-antibody attached to receptor sites on the
post-synaptic membrane. This auto-antibody both degrades
and blocks acetylcholine receptor sites, thus impairing neurotransmission across the synapse.
Myasthenia gravis is an autoimmune disease in which the
auto-antibody appears clearly involved in the pathogenesis of
the muscle weakness.
Myasthenia gravis is rather more common in women than
men. In women, it tends to occur in young adult life, and in men
it more commonly presents over the age of 50 years. Various
subtypes of myasthenia gravis have been distinguished according to age and sex prevalence, HLAtype associations, incidence
of auto-antibodies, and other characteristics.
Muscle weakness, with abnormal fatiguability, and improvement after rest, characterize myasthenia gravis. Symptoms tend
to be worse at the end of the day, and after repetitive use of muscles for a particular task, e.g. chewing and swallowing may be
much more difficult towards the end of a meal than they were at
the start. The distribution of muscle involvement is not uniform, as shown in Fig. 10.9.
Termination of axon
of lower motor neurone
Acetylcholine molecules
contained in vesicles
Voltage-gated
channels for
release of
acetylcholine
Highly convoluted and
modified part of the
muscle membrane, on
the surface of which
are acetylcholine
receptor sites
Fig. 10.8 Diagram to show a motor end plate in skeletal muscle.
Common
Rare
165
Muscles
Symptoms
External ocular
Double vision and ptosis
Bulbar
Difficulty in chewing, swallowing

and talking
Neck
Difficulty in lifting head up from

the lying position
Proximal limb
Difficulty in lifting arms above

shoulder level, and in standing
from low chairs and out of the
bath
Trunk
Breathing problems and difficulty

in sitting from the lying position
Distal limb
Weak hand-grips, ankles and feet
Fig. 10.9 Frequency of muscle involvement and symptoms in

myasthenia gravis.
Confirmation of the diagnosis

Once suspected, the diagnosis of myasthenia gravis may be
confirmed by:
1. The Tensilon test. Edrophonium chloride (Tensilon) is a
short-acting anticholinesterase, which prolongs the action of
acetylcholine at the neuromuscular junction for a few minutes
after slow intravenous injection. This produces a transient and
striking alleviation of weakness. There may also be an equally
exciting bradycardia (reversed by atropine); the test should not
be performed lightly in patients who are frail or have heart
disease.
2. Detection of serum acetylcholine receptor antibodies. These
antibodies are not found in the normal population, but are detected in about 50% of patients with purely ocular myasthenia,
increasing up to about 90% of patients with more generalized
myasthenia.
3. EMG studies. Sometimes it is helpful to show that the amplitude of the compound muscle action potential, recorded by
surface electrodes over a muscle, decreases on repetitive stimulation of the nerve to the muscle.
4. Chest radiography and CT of the anterior mediastinum, to
demonstrate an enlargement of the thymus gland. The association of myasthenia gravis with thymic enlargement is not yet
fully understood. Of myasthenic patients, 1015% have a thymoma, and 5060% show thymic hyperplasia. Both sorts of
pathology may enlarge the thymus, which can be clearly shown
by suitable imaging procedures.
166
Management of myasthenia gravis

The management of myasthenia gravis includes:
1. The use of oral anticholinesterase drugs, pyridostigmine and
prostigmine. These are prescribed at intervals during the day,
and work quite effectively. Abdominal colic and diarrhoea, induced by the increased parasympathetic activity in the gut, can
be controlled by simultaneous use of propantheline.
2. Immunosuppression by prednisolone or azathioprine. In patients with disabling symptoms inadequately controlled by oral
anticholinesterase therapy, suppression of the autoantibody
can radically improve muscle strength.
3. Thymectomy. Remission or improvement can be expected in
6080% of patients after thymectomy, and must be considered
in all patients. It may make the use of immunosuppressive
drugs unnecessary, which is obviously desirable.
4. Plasma exchange to remove circulating auto-antibody to
produce short-term improvement in seriously weak patients.
5. Great care of the myasthenic patient with severe weakness
who is already on treatment. The muscle strength of such patients may change abruptly, and strength in the bulbar and respiratory muscles may become inadequate for breathing. The
correct place for such patients is in hospital, with anaesthetic
and neurological expertise closely to hand. There may be uncertainty as to whether such a patient is undertreated with
anticholinesterase (myasthenic crisis), or overtreated so that
the excessive acetylcholine at the neuromuscular junction is
spontaneously depolarizing the postsynaptic membrane, i.e.
depolarization block (cholinergic crisis). Fasciculation may be
present when such spontaneous depolarization is occurring.
Tensilon may be used to decide whether the patient is under- or
overdosed, but it is essential to perform the Tensilon test with an
anaesthetist present in these circumstances. If the weak state is due
to cholinergic crisis, the additional intravenous dose of anticholinesterase may produce further critical paralysis of bulbar
or respiratory muscles.
CHAPTER 10
Management of myasthenia
gravis
Anticholinesterase
Immunosuppression
Thymectomy
Plasma exchange
Crisis management
167
Muscle disease
These are a group of rare diseases in which the primary

pathology causing muscle weakness and wasting lies in the
muscles themselves. They are classified in Fig. 10.10 and short
notes about each condition are given in this section.
Inherited
1 Muscular dystrophies, whose genetic basis is increasingly
understood in terms of gene and gene product identification.
Duchenne
Myotonic dystrophy
Facio-scapulo-humeral
Limb girdle
X-linked recessive gene

Autosomal dominant gene
Autosomal dominant gene
Not a single entity (variable
inheritance)
2 Muscle diseases in which an inherited biochemical defect is

present.
Specific enzyme deficiencies occur which disrupt the pathways
of carbohydrate or fat oxidation, often with accumulation of
substrate within the muscle cell. The enzyme deficiency may be
within the muscle cell cytoplasm, interfering with the utilization
of glycogen or glucose, or it may be within the mitochondria of
muscle cells (and cells of other organs) blocking the metabolism
of pyruvate, fatty acids or individual elements of Krebs cycle.
In other diseases of this sort, there is uncoupling of the
electrical excitation of muscle fibres and their contraction.
This is the case in McArdle's syndrome, and in malignant
hyperpyrexia where sustained muscle contraction may occur
in the absence of nerve stimulation.
Acquired
1 Immunologically mediated inflammatory disease, e.g.
polymyositis
dermatomyositis
Fig. 10.10 Classification of

muscle diseases.
2 Non-inflammatory myopathy, e.g.

corticosteroids
thyrotoxicosis
168
Duchenne dystrophy
Duchenne dystrophy is the most serious inherited muscular
dystrophy. The X-linked recessive inheritance gives rise to
healthy female carriers and affected male children. The affected
boys usually show evidence of muscular weakness before the
age of 5 years, and die of profound muscle weakness (predisposing them to chest infections), or of associated cardiomyopathy, in late teenage life. In the early stages, the weakness of
proximal muscles may show itself by a characteristic way in
which these boys will climb up their own bodies with their
hands (Gowers sign) when rising from the floor to the standing position. They also show muscle wasting, together with
pseudohypertrophy of the calf muscles (which is due to fat
deposition in atrophied muscle tissue).
The affected boys have elevated levels of creatine kinase
muscle enzyme in the blood, and the clinically unaffected
carrier state in female relatives is often associated with some
elevation of the muscle enzymes in the blood. The gene locus
on the X chromosome responsible for Duchenne dystrophy,
and its large gene product, dystrophin, have been identified.
Molecular genetic diagnosis of affected patients and female
carriers is possible, as is prenatal diagnosis.
This same region of the X chromosome is also implicated in
the inheritance of a more benign variant of Duchenne dystrophy (later in onset and less rapidly progressive), known as
Beckers musclar dystrophy.
The combination of family history, clinical examination,
biochemical and genetic studies allows the detection of the
carrier state, and the prenatal detection of the affected male
fetus in the first trimester of pregnancy. Genetic counselling of
such families has reached a high degree of accuracy. As a single
gene disorder, Duchenne muscular dystrophy is one of the
conditions in which gene therapy is being considered.
CHAPTER 10
Key features of Duchenne

muscular dystrophy
Young
Male
Generalized weakness
Muscle wasting
Calf pseudohypertrophy
Gowers sign
Key features of myotonic

dystrophy
Either sex
Glum-looking from facial
weakness and ptosis
Frontal balding
Glasses or previous cataract
surgery
Hand muscles show
wasting and myotonia
169
Myotonic dystrophy
Myotonic dystrophy is characterized by dystrophy of several
organs and tissues of the body, and the dystrophic changes in
muscle are associated with myotonic contraction.
The disease is due to an expanded trinucleotide repeat (see
box on p. 75). This is inherited as an autosomal dominant, so
men and women are equally affected, usually in early adult life.
The mutation tends to expand with each generation, especially
when transmitted from a woman to her child, causing a more
severe phenotype which is described below. Genetic testing
allows symptomatic, presymptomatic and prenatal diagnosis,
where appropriate.
Some impairment of intellectual function, cataracts, premature loss of hair, cardiac arrhythmia and failure, gonadal atrophy and failure, all feature in patients with myotonic dystrophy,
but the most affected tissue is muscle. The facial appearance
may be characteristic, with frontal balding, wasting of the temporalis muscles, bilateral ptosis and bilateral facial weakness.
Muscle weakness and wasting are generalized but the hands are
often particularly affected.
The myotonia shows itself in two ways:
1. The patient has difficulty in rapid relaxation of tightly contracted muscle, contraction myotonia, and this is best seen by
asking the patient to open the hand and fingers quickly after
making a fist.
2. Percussion myotonia is the tendency for muscle tissue
to contract when it is struck by a tendon hammer, and this is
best seen by light percussion of the thenar eminence whilst
the hand is held out flat. A sustained contraction of the thenar
muscles lifts the thumb into a position of partial abduction
and opposition.
From its appearance in early adult life, the illness runs a
variable but slowly progressive course over several decades.
The associated cardiomyopathy is responsible for some of
the early mortality in myotonic dystrophy.
Some children of females with myotonic dystrophy may
show the disease from the time of birth. Such babies may be very
hypotonic, subject to respiratory problems (chest muscle involvement) and feeding problems (facial muscle involvement).
Mental retardation is a feature of these children. Frequently, the
birth of such a child is the first evidence of myotonic dystrophy
in the family, since the mothers involvement is only mild.
170
CHAPTER 10
Facio-scapulo-humeral dystrophy
Facio-scapulo-humeral dystrophy is generally a benign form
of muscular dystrophy. It is due to an unusual dominantly
inherited gene contraction near the telomere of chromosome 4,
which can usually be detected for diagnostic purposes. It is
often mild and asymptomatic. Wasting and weakness of the facial, scapular and humeral muscles may give rise to difficulties
in whistling, and in using the arms above shoulder level and for
heavy lifting. The thinness of the biceps and triceps, or the abnormal position of the scapula (due to weakness of the muscles
which hold the scapula close to the thoracic cage), may be the
features that bring the patient to seek medical advice. Involvement of other trunk muscles, and the muscles of the pelvic
girdle, may appear with time.
Limb girdle syndrome

Weakness concentrated around the proximal limb muscles has a
wide range of causes, including ones which are treatable. Limb
girdle weakness should not be regarded as due to dystrophy
(and therefore incurable) until thorough investigation has
shown this to be the case. Even limb girdle dystrophy is heterogeneous, made up of a large number of pathologically and
genetically distinct entities, for example involving many of
the proteins which anchor the contractile apparatus of muscle
to the muscle membrane. Other important causes of limb
girdle syndrome are shown in the box.
Conditions caused by inherited biochemical defects

Muscle diseases in which an inherited biochemical defect is
present are rare. Of these conditions, malignant hyperpyrexia
is perhaps the most dramatic. Members of families in which
this condition is present do not have any ongoing muscle
weakness or wasting. Symptoms do not occur until an affected
family member has a general anaesthetic, particularly if
halothane or suxamethonium chloride is used. During or immediately after surgery, muscle spasm, shock and an alarming
rise in body temperature occur, progressing to death in about
50% of cases.
The pathology of this condition involves a defect in calcium
metabolism allowing such anaesthetic agents to incur a massive
rise in calcium ions within the muscle cells. This is associated
with sustained muscle contraction and muscle necrosis. The
rise in body temperature is secondary to the generalized muscle
contraction.
Limb girdle weakness:

polymyositis
myopathy associated with
endocrine disease
metabolic myopathies
drug-induced myopathies,
e.g. steroids
limb girdle dystrophy
171
Polymyositis and dermatomyositis

The muscle problems in polymyositis and dermatomyositis are
very similar. There is a mononuclear inflammatory cell infiltration and muscle fibre necrosis. In dermatomyositis, there is the
additional involvement of skin, particularly in the face and
hands. An erythematous rash over the nose and around the
eyes, and over the knuckles of the hands, is most typical.
Though all muscles may be involved, proximal limb, trunk and
neck muscles are most frequently made weak by polymyositis
with occasional involvement of swallowing.
The condition most usually develops subacutely or
chronically and is unassociated with muscle tenderness. Problems when trying to use the arms above shoulder level, and
difficulty when standing up out of low chairs and the bath, are
the most frequent complaints.
Both conditions are autoimmune diseases involving skeletal
and not cardiac muscle. Dermatomyositis, especially in older
men, can be triggered by underlying malignancy.
Both dermatomyositis and polymyositis respond to immunosuppressive therapy. High-dose steroids, with or without
other immunosuppressants, gradually reduced to reasonable
long-term maintenance levels, constitute the treatment of
choice. Effective control of the disease can be established in the
majority of cases.
Acquired non-inflammatory myopathy

Acquired non-inflammatory myopathy can occur in many circumstances (alcoholism, drug-induced states, disturbances of
vitamin D and calcium metabolism, Addisons disease, etc.),
but the two common conditions to be associated with myopathy are hyperthyroidism and high-dose steroid treatment.
Many patients with hyperthyroidism show weakness of
shoulder girdle muscles. This is usually asymptomatic. Occasionally, more serious weakness of proximal limb muscles and
trunk muscles may occur. The myopathy completely recovers
with treatment of the primary condition.
Patients on high-dose steroids, especially fluorinated triamcinolone, betamethasone and dexamethasone, may develop
significant trunk and proximal limb muscle weakness and wasting. The myopathy is reversible on withdrawal of the steroids,
on reduction in dose, or on change to a non-fluorinated steroid.
172
CHAPTER 10
Investigation of patients with generalized muscle

weakness and wasting
The last section of this chapter discusses the common investigations that are carried out in patients with generalized muscle
weakness and wasting. Of the four conditions discussed in this
chapter, myasthenia gravis does not produce muscle wasting,
and is usually distinguishable by virtue of the ocular and bulbar
muscle involvement, the abnormal degree of fatiguability, and
the response to anticholinesterase. The other three conditions
may be quite distinct on clinical grounds too, but investigation
is frequently very helpful in confirmation of diagnosis.
Figure 10.11 shows the investigations that are carried out on
patients of this sort.
Test
Motor
neurone
disease
Peripheral
neuropathy
Muscle
disease
Biochemistry
Creatine kinase
Normal
Normal
Elevated
Electrical studies
Electromyography
Denervation
Denervation
Muscle disease
Normal
Delayed
Normal
conduction
velocities and
reduced nerve
action
potentials
Denervation
Denervation
Motor and
sensory nerve
conduction studies
Histology
Histochemistry
Immunofluorescence
Electron microscopy
Muscle biopsy
Nerve biopsy
Molecular
genetics
Specific commentary
on the nature of the
muscle disease,
i.e. dystrophy,
polymyositis or
acquired myopathy
Sometimes
helpful in
establishing
the precise
cause of
peripheral
neuropathy
No help in
conventional
MND
Helpful in
hereditary
motor and
sensory
neuropathy
Helpful in the
inherited muscle
diseases
Fig. 10.11 The investigations

performed in patients with
generalized muscle weakness and
wasting.
173
(a) Normal
(b) Muscle disease
(c) Denervation
(d) Denervation
Fig. 10.12 The changes in muscle disease and denervation.

(a) Two normal motor units. Each lower motor neurone supplies several muscle fibres.
(b) In muscle disease (dystrophy, polymyositis or acquired myopathy), there is loss or damage directly
affecting muscle fibres. The number of functional muscle fibres decreases. Therefore, in muscle disease,
there is a normal number of abnormally small motor units.
(c) Damage to one lower motor neurone, either in the cell body (as in motor neurone disease), or in the
axon (as in peripheral neuropathy), results in denervated muscle fibres within the motor unit.
(d) The surviving lower motor neurone produces terminal axonal sprouts which innervate some of the
muscle fibres of the damaged motor unit. Therefore, in muscles affected by a denervating disease, there is a
reduced number of abnormally large motor units.
It is important that the consequences of denervation and

muscle disease on the motor unit are understood, and these are
shown in Fig. 10.12. Both electromyography (the recording of
muscle at rest and during contraction) and muscle biopsy are
able to detect the changes of chronic partial denervation and of
primary muscle disease.
174
CHAPTER 10
CASE HISTORIES
Case 1
Case 2
A 55-year-old bank manager reports a 12-month

history of numbness and burning in his feet. He takes
ranitidine for dyspepsia but has no other medical
history. He is married with grown-up children. He has
no family history of neurological disease. His GP has
checked his full blood count (MCV mildly raised at
101, otherwise normal), electrolytes and glucose
(normal).
On examination he is generally thin but has no
muscle wasting, fasciculation or weakness. His ankle
reflexes are absent. Both plantar responses are flexor.
He has impaired appreciation of pain and temperature
sensation below mid-shin on both sides. He can feel
light touch and joint position normally. Rombergs test
is negative (i.e. he can stand to attention with his eyes
shut, without falling over).
A previously healthy 26-year-old junior doctor asks

your advice. She has been choking on drinks for 2
weeks and last night a patient complained about her
slurred speech. She is extremely anxious because her
uncle died of motor neurone disease. She lives on her
own in a hospital flat; she has not registered with
a GP.
On examination she is anxious and breathless. Her
speech is nasal and becomes softer as she talks. Her
palatal movements are reduced. Her tongue looks
normal. Her jaw-jerk is normal. Examination of the
other cranial nerves is normal. She has no limb
wasting, fasciculation or weakness. Her reflexes are
brisk.There is no sensory loss.
a. What type of neurological problem does he have?

b. What is the most likely cause?

b. How would you manage her case?
11
CHAPTER 11
Unconsciousness
Introduction and definitions
Ear
Eye
Face, mouth,
head
Limbs and
trunk
Fig. 11.1 Diagram to show

important factors maintaining
consciousness.
Patients who become unconscious make their relatives and

their doctors anxious. A structured way of approaching the unconscious patient is useful to the doctor so that he behaves rationally and competently when those around him are becoming
alarmed.
Unconsciousness is difficult to define. Most people know
what is meant by the word. One way of defining unconsciousness is by asking the reader how he would recognize that a
person he had just found was unconscious. Answers to this
question would probably include statements like this, in a deep
sleep, eyes closed, not talking, not responding to his name or instructions, not moving his limbs even when slapped or shaken.
In terms of neurophysiology and neuro-anatomy, it is not
completely clear on what consciousness depends. Consciousness involves the normally functioning cerebrum responding to
the arrival of visual, auditory and somatic afferent stimulation
as shown in Fig. 11.1.
The ideal circumstances for normal loss of consciousness, in
sleep, are entirely compatible with this concept eyes closed in
a darkened room, where it is quiet, in a bed where the body is
comfortable, warm and still.
Abnormal states of unconsciousness occur firstly if there is
some generalized impairment of cerebral hemisphere function
preventing the brain from responding to normal afferent stimulation, or secondly, if the cerebral hemispheres are deprived of
normal afferent stimulation due to pathological lesions in the
brainstem, blocking incoming visual, auditory and somatic
sensory stimuli. The concept of unconsciousness being the consequence of either a diffuse cerebral problem, or a major brainstem lesion, or both, is useful from the clinical point of view.
A patient may present to the doctor with attacks of unconsciousness between which he feels well, i.e. blackouts, or he
may be in a state of ongoing unconsciousness which persists
and demands urgent management, i.e. persistent coma. We will
consider these two situations separately.
175
176
CHAPTER 11
Attacks of unconsciousness or blackouts

Here it is most likely that the patient, feeling perfectly well, will
consult the doctor about some blackouts which have been occurring. Very often a relative will be with him, since the attack
has caused as much anxiety in the witnessing relative as in the
patient. It is not common for doctors to witness transient blackouts in patients, for obvious reasons. The value of a competent witnesss account is enormous in forming a diagnosis. Arriving at a firm
diagnosis in a patient who has suffered unwitnessed attacks is often
much more difficult.
The common causes of blackouts are illustrated in Fig. 11.2.
Generalized cerebral malfunction
Severe localized brainstem lesion
No blood
Vasovagal syncope
Postural hypotension
Hyperventilation
Cardiac dysrhythmia
Transient ischaemic attacks in the

vertebro-basilar circulation
Both generalized cerebral and brainstem lesion
Blood no good
Primary generalized epilepsy
Hypoxia
Hypoglycaemia
Neither generalized cerebral nor brainstem lesion
Psychologically mediated (hysterical) attacks
Fig. 11.2 Diagram to show the common causes of blackouts.
UNCONSCIOUSNESS
177
Causes of blackouts
No blood
No blood
Vasovagal syncope
As a consequence of increased vagal and decreased sympathetic activity, the heart slows and blood pools peripherally.
Cardiac output decreases and there is inadequate perfusion of
the brain when the patient is in the upright position. He loses
consciousness, falls, becomes horizontal, venous return improves, cardiac output improves and consciousness is restored.
The attack is worse if the patient is held upright and it is relieved
or prevented by lowering the patients head below the level of
the heart.
Common features of vasovagal syncope are as follows:
they are more common in teenage and young adult life;
they may be triggered by standing for a long time, and by
emotionally upsetting circumstances (hearing bad news,
hearing or seeing explicit medical details, experiencing
minor medical procedures, e.g. venipuncture, sutures);
the patient has a warning of dizziness, visual blurring, feeling hot or cold, sweating, pallor;
the patient is unconscious for a short period (30120 seconds)
only, during which time there may be a brief flurry of myoclonic jerks but then the patient is flaccid and motionless;
the patient feels nauseated and sweaty on recovery, but is
back to normal within 15 minutes or so.
Postural hypotension
In circumstances of decreased sympathetic activity affecting
the heart and peripheral circulation, the normal cardioacceleration and peripheral vasoconstriction that occurs
when changing from the supine to the erect position does
not occur, and cardiac output and cerebral perfusion are inadequate in the standing position, resulting in loss of consciousness. The situation rectifies itself as described above in
vasovagal syncope. The cause of the decreased sympathetic
activity is usually pharmacological (overaction of antihypertensive agents or as a side-effect of very many drugs given for
other purposes), though it is occasionally due to a physical lesion of the sympathetic pathways in the central or peripheral
nervous system.
Postural hypotension should be suspected if the patient:
is middle-aged or elderly, and is on medication of some sort;
complains of dizziness or lightheadedness when standing;
only experiences attacks in the standing position and can
abort them by sitting or lying down;
has a systolic blood pressure which is lower by 30 mmHg or
more when in the standing position than when supine.
178
Hyperventilation
Patients who overbreathe, wash out carbon dioxide from their
blood. Arterial hypocapnia is a very strong cerebral vasoconstrictive stimulus. The patient starts to feel lightheaded, unreal
and increasingly dissociated from her surroundings.
The clues to hyperventilation being the cause of blackouts
are:
the patient is young and female;
the patient is in a state of anxiety;
the patient mentions that she has difficulty in getting her
breath as the attack develops;
distal limb paraesthesiae and/or tetany are mentioned (due
to the increased nerve excitability which occurs when the
concentration of ionized calcium in the plasma is reduced
during respiratory alkalosis);
the attacks can be culminated by reassurance and rebreathing
into a paper bag;
the symptoms may be reproducible by voluntary hyperventilation.
Cardiac arrhythmia
When left ventricular output is inadequate because of either
cardiac tachyarrhythmia or bradyarrhythmia, cardiac output
and cerebral perfusion may be inadequate to maintain consciousness. The cardiac arrhythmia is most usually (but not exclusively) caused by ischaemic heart disease. The most classical
form of this condition, known as the StokesAdams attack, occurs when there is impaired atrioventricular conduction leading to periods of very slow ventricular rate and/or asystole.
Cardiac arrhythmia should be suspected if:
the patient is middle-aged or elderly;
the attacks are unrelated to posture;
there is a history of ischaemic heart disease;
the patient has noticed palpitations;
episodes of dizziness and presyncope occur as well as
episodes in which consciousness is lost;
marked colour change and/or loss of pulse have been observed by witnesses during the attacks;
the patient has a rhythm abnormality at the time of
examination;
ischaemic, rhythm or conduction abnormalities are present
in the ECG.
CHAPTER 11
No blood
No blood
UNCONSCIOUSNESS
Blood no good
Blood no good
179
Hypoxia
Hypoxia is a very uncommon cause of attacks of unconsciousness. Even in patients with severe respiratory embarassment, e.g. major asthmatic attack, consciousness is usually
retained.
Hypoglycaemia
Except in diabetic patients who are taking oral hypoglycaemic
agents or insulin, hypoglycaemia is another very uncommon
cause of blackouts. This is because alternative causes of hypoglycaemia (e.g. insulinoma of the pancreas) are rare.
Amongst diabetics, hypoglycaemia should be high on the list
of possible causes of blackouts.
Hypoglycaemic attacks:
may be heralded by feelings of hunger and emptiness;
are associated with the release of adrenaline (one of the
bodys homeostatic mechanisms to release glucose from liver
glycogen stores in the face of hypoglycaemia). This explains
the palpitations, tremor and sweating that characterize
hypoglycaemic attacks;
may not proceed to full loss of consciousness; they may simply cause episodes of abnormal speech, confusion or unusual
behaviour;
may proceed quite rapidly through faintness and drowsiness
to coma, especially in children;
are most conclusively proved by recording a low blood
glucose level during an attack, but clearly this is not always
possible.
Vertebro-basilar transient ischaemic attacks
Vertebro-basilar transient ischaemic attacks rarely cause loss of
consciousness without additional symptoms of brainstem dysfunction. Thrombo-embolic material, derived from the heart or
proximal large arteries in the chest and neck, may lodge in the
small arteries which supply the brainstem. They may cause
ischaemia of the brainstem tissue until lysis or fragmentation
of the thrombo-embolic material occurs.
Vertebro-basilar ischaemia is suggested:
if the patient is middle-aged or elderly;
if the patient is known to be arteriopathic (i.e. history of
myocardial infarction, angina, intermittent claudication or
stroke), or has a definite source of emboli;
if the patient is having transient ischaemic attacks that do
not involve loss of consciousness, e.g. episodes of monocular blindness, speech disturbance, hemiplegia, hemianaesthesia, diplopia, ataxia, etc.
180
Epilepsy
Chapter 12 deals with epilepsy in detail. Epilepsy may be generalized or focal. In generalized epilepsy the abnormal electrical
activity starts in deep midline brain structures and spreads to all
parts of the cerebral cortex simultaneously. This gives rise to
tonicclonic and absence seizures in which consciousness is
invariably lost.
In focal epilepsy, the abnormal electrical activity is localized
to one area of the cerebral cortex. In focal seizures, there is
grossly deranged function in that part of the brain where
the epileptic activity is occurring, whilst the rest of the brain
remains relatively normal. So long as the seizure remains
localized, consciousness is maintained. It is only when focal
epileptic activity occurs in the temporal lobe, when regions
subserving memory are disrupted during the attack, that
patients seek help for blackouts which they cannot properly
remember. Memory, rather than consciousness, is lost in such
attacks.
Psychogenic non-epileptic attacks
Some patients attract attention to themselves, at a conscious or
subconscious level, by having blackouts. The attacks may consist of apparent loss of consciousness and falling, sometimes
with convulsive movement of the limbs and face. The patient
may report no memory or awareness during the attack, or he
may acknowledge awareness at a very distant level without any
ability to respond to his environment or control his body during
the attack.
Such psychologically mediated non-epileptic attacks:
are more common in teenage and young adult life;
are associated with self-reported previous physical or sexual
abuse;
may be suggested by the coordinated purposeful kinds of
movements which are witnessed in the attacks (shouting,
grasping, pelvic thrusting, turning the head from side to
side);
may occur in association with epilepsy. It is easy to understand why a young person with epilepsy might respond to
adversity by having non-epileptic attacks rather than developing some other psychosomatic disorder;
are disabling, very difficult to manage, and potentially
dangerous if treated inappropriately with anticonvulsant
drugs such as intravenous benzodiazepines.
CHAPTER 11
UNCONSCIOUSNESS
181
Making the diagnosis in a patient with blackouts
Which is it?
Making the diagnosis is best achieved by giving oneself enough

time to talk to the patient so that he can describe all that happens
before, during and after the attacks, and similarly by talking to a
witness so that he can describe all the observable phenomena of
the patients attack. Physical examination of the patient with
blackouts is very frequently normal, so it cannot be relied upon
to yield very much information of use. Occasionally, it may be
necessary to admit the patient to hospital so that the attacks may
be observed by medical and nursing staff.
The investigations that may prove valuable in patients with
blackouts are:
EEG and ECG, with prolonged monitoring of the brain and
heart by these techniques, if the standard procedures are not
diagnostic;
blood glucose and blood gas estimations (ideally at the time
of the attacks) may help to prove either hypoglycaemia or
hyperventilation as the basis of the attacks.
Often no tests necessary:

adolescent with a typical vasovagal
syncope
elderly person on medication with
demonstrable postural hypotension
'First you tell me what happened, and
then we will ask your husband for all
the details too'
Standard EEG
Ambulatory ECG
182
CHAPTER 11
Treatment of the common causes of blackouts

Apart from a general explanation of the nature of the attacks
to the patient and his family, there are two other aspects of
management.
Treatment
Specific treatment suggestions

Vasovagal syncope: lower head at onset.
Postural hypotension: remove offending drug, consider physical
and pharmacological methods of maintaining the standing
blood pressure (sleep with bed tilted slightly head up, fludrocortisone).
Hyperventilation: reassurance, and exercises to control
breathing.
Cardiac arrhythmia: pharmacological or implanted pacemaker
control of cardiac rhythm.
Hypoglycaemia: attention to drug regime in diabetics, removal of
insulinoma in the rare instances of their occurrence.
Vertebro-basilar TIAs: treat source for emboli, aspirin.
Epilepsy: anticonvulsant drugs.
Psychogenic non-epileptic attacks: try to establish the reason for
this behaviour, and careful explanation to the patient.
Care of personal safety
People who are subject to sudden episodes of loss of
consciousness:
should not drive motor vehicles;
should consider showering rather than taking a bath;
may not be safe in some working environments which involve
working at heights, using power tools, working amongst
heavy unguarded machinery, working with electricity wires;
may have to curtail some recreational activities involving
swimming or heights.
A firm but sympathetic manner is necessary in pointing out
these aspects of the patients management.
Sensible, but often unpopular

restrictions
UNCONSCIOUSNESS
183
Before leaving the causes of blackouts, there are two rare neurological conditions to mention briefly. One condition predisposes the patient to frequent short episodes of sleep, narcolepsy,
and the other gives rise to infrequent episodes of selective loss
of memory, transient global amnesia.
Narcolepsy
Sudden irresistible need to
sleep, for short periods
Legs give way, when highly
amused or angry
In this condition, which is familial and very strongly associated

with the possession of a particular HLA tissue type, the patient
has a tendency to sleep for short periods, e.g. 1015 minutes.
The sleep is just like ordinary sleep to the observer, but is unnatural in its duration and in the strength with which it overtakes
the patient. Such episodes of sleep may occur in circumstances
where ordinary people feel sleepy, but narcoleptic patients also
go to sleep at very inappropriate times, e.g. whilst talking, eating or driving.
The condition is associated with some other unusual
phenomena:
cataplexy: transient loss of tone and strength in the legs
at times of emotional excitement, particularly laughter and
annoyance, leading to falls without any impairment of
consciousness;
sleep paralysis: the frightening occurrence of awakening at
night unable to move any part of the body for a few moments;
hypnogogic hallucinations: visual hallucinations of faces occurring just before falling asleep in bed at night.
Narcolepsy, and its associated symptoms, are helped by
dexamphetamine, clomipramine and modafinil.
Transient global amnesia

A short period, lasting hours,
of very selective memory loss,
other cerebral functions
remaining intact
This syndrome, which tends to occur in patients over the age of

50, involves loss of memory for a few hours. During the period
of amnesia, the patient cannot remember recent events, and
does not retain any new information at all. All other neurological functions are normal. The patient can talk, write and carry
out complicated motor functions (e.g. driving) normally.
Throughout the episode, the patient repeatedly asks the same
questions of orientation. Afterwards, the person is able to recall
all events up to the start of the period of amnesia, remembers
nothing of the period itself, and has a somewhat patchy
memory of the first few hours following the episode.
Transient global amnesia may occur a few times in a patients
life. Its pathological mechanism remains poorly understood,
but may be related to that of migraine aura.
184
CHAPTER 11
Persistent coma
Assessment of conscious level
The observations that might be made by someone who finds
an unconscious body were mentioned at the beginning of
this chapter: in a deep sleep, eyes closed, not talking, not responding to instructions, not moving even when slapped or
shaken. These natural comments have been brought together
and elaborated in the Glasgow Coma Scale. This is used very
effectively to indicate and monitor a patients level of unconsciousness. The Glasgow Coma Scale records the level of
stimulus required to make the patient open his eyes and also
records the patients best verbal and motor responses, as
shown in Fig. 11.3. It may be helpful to amplify the scores with
a written description of the patients precise responses to
command or pain, in case subsequent staff are less familiar with
the numerical scale.
The responses of the Glasgow Coma Scale depend upon the
cerebrums response to afferent stimulation. This may be impaired either because of impaired cerebral hemisphere function
or because of a major brainstem lesion interfering with access
of such stimuli to the cerebral hemispheres. Direct evidence
that there is a major brainstem lesion may be evident in an
unconscious patient (Fig. 11.4). Dilatation of the pupils and lack
of pupillary constriction to light indicate problems in the midbrain, i.e. 3rd cranial nerve dysfunction. Impaired regulation of
Time
Coma scale
Eyes
open
(E)
Best
verbal
response
(V)
Best
motor
response
(M)
am
Spontaneously
To speech
To stimulus
None
Orientated
Confused
Inappropriate words
Incomprehensible sounds
None
Obey commands
Localise stimulus
Flexion-withdrawal
Flexion-abnormal
Extension
No response
10
Treatment
Glasgow Coma Scale score at 7am: E1, V2, M4 = 7
Fig. 11.3 Scheme to show the Glasgow Coma Scale. (After Teasdale G. & Jennett B. (1974) Lancet ii, 813.)
11
UNCONSCIOUSNESS
Regulation of pupils
Regulation of Temperature
BP
Pulse
Respiration
Fig. 11.4 Diagram to show

important functions of the
brainstem.
185
body temperature, blood pressure, pulse and respiration may

all indicate trouble in the pons/medulla, where the centres
controlling these vital functions exist.
When confronted with a patient in coma, a well-trained
doctor will assess:
vital functions of respiration, temperature control, pulse and
blood pressure;
pupil size and reactivity;
the patients eyes, speech and motor responses according to
the Glasgow Coma Scale.
This approach to the assessment of conscious level holds
good regardless of the particular cause of coma.
Causes of coma
There is a simple mnemonic to help one to remember the causes
of coma (Fig. 11.5). In considering the causes of coma it is helpful
to think again in terms of disease processes which impair cerebral hemisphere function generally on the one hand, and in
terms of lesions in the brainstem blocking afferent stimulation
of the cerebrum on the other. The common causes of coma are illustrated in this way in Fig. 11.6 overleaf.
Fig. 11.5 A simple mnemonic for

the recall of the causes of coma.
A = Apoplexy
Brainstem infarction
Intracranial haemorrhage
E = Epilepsy
Post-ictal or inter-ictal coma

Status epilepticus
I = Injury
Concussionmajor head injury
I = Infection
Meningo-encephalitis
Cerebral abscess
O = Opiates
Standing for all CNS depressant drugs,

including alcohol
U = Uraemia
Standing for all metabolic causes for

coma. Quite a useful way of
remembering all possibilities here is
to think of coma resulting from
extreme deviation of normal blood
constituents
Oxygen
Carbon dioxide
Hydrogen ions
Glucose
Urea
Ammonia
Thyroxine
Anoxia
Carbon dioxide narcosis
Diabetic keto-acidosis
Hypoglycaemia
Renal failure
Liver failure
Hypothyroidism
186
CHAPTER 11
Swollen
tight
Mechanism of coma
Generalized impairment
of cerebral hemisphere
function, leading to a
sub-standard response
to normal afferent
stimulation
Major primary pathology

in the brainstem,
depriving the cerebral
hemispheres of their
normal afferent
stimulation
Unilateral cerebral
hemisphere mass lesion,
causing downward
herniation of the medial
part of the temporal
lobe through the tentorial hiatus, which results
in a sideways and downward shift of the brainstem. This situation of
secondary brainstem
malfunction is one form
of 'coning', and explains
such patients' coma.
It may progress to
medullary coning at
foramen magnum level,
if the mass lesion is left
untreated.
Generalized impairment
of cerebral hemisphere
function, associated
with bilateral cerebral
hemisphere swelling.
Bilateral medial temporal herniation occurs.
Downward shift of the
brainstem occurs at the
level of the midbrain
(tentorial hiatus) and
medulla (foramen
magnum). Coma is
due to both generalized
impairment of cerebral
hemisphere function and
coning at midbrain and
medullary levels.
Cause of coma
Overdose of CNS
sedative drugs
Severe alcoholic
intoxication
Diabetic comas
Renal failure
Hepatic failure
Brainstem infarction by
basilar artery
occlusion
Brainstem haemorrhage,
as occurs in severe
hypertension
Haematoma
Abscess
Tumour
Brain trauma
Cerebral anoxia or
ischaemia
Status epilepticus
Assessment of coma
Glasgow Coma Scale
works really well
in these patients, since
there is no focal
neurological damage,
and therefore no lateralizing or focal signs. In
severe instances, the
noxious process may
involve the brainstem as
well as the cerebral
hemispheres. Signs of
depressed brainstem
function appear . . .
impaired pupils and
impaired regulation of
vital functions
These patients have a

multitude of abnormal
neurological signs, since
the major brainstem
lesion is causing
malfunction in the:
descending motor
pathways
ascending sensory
pathways
pathways to and from
the cerebellum
cranial nerve nuclei
centres regulating vital
functions
These patients have

the signs of a unilateral
cerebral hemisphere
lesion and raised intracranial pressure
(papilloedema). In
addition the signs of
coning (pupillary dilatation and impaired
regulation of vital
functions) may appear
These patients have

the signs of bilateral
cerebral hemisphere
malfunction and raised
intracranial pressure
(papilloedema). They
too may show signs of
coning
In these patients, the assessment of eyes, speech and motor responses,

needed for the Glasgow Coma Scale, is somewhat interfered with, because
of the presence of the primary neurological deficit produced by the
primary CNS pathology. In such instances, the best eye, speech and limb
response which can be achieved (in either of the two eyes, or in any of the
four limbs) is the one which is used for the Coma Scale assessment. Despite
this interference the Coma Scale, charted at intervals as shown in Fig. 11.3,
provides a very valuable guide to an unconscious patient's progress
Fig. 11.6 Scheme to show the common causes of coma.
UNCONSCIOUSNESS
187
Investigation and management of a patient in coma
Airway
Level of coma
Cause of coma
Caution over lumbar puncture
Treat cause
Routine care of unconscious
patient

routine care of the unconscious
patient.
(a)
Chart of
observations
1. Check that the patients airway is clear, that breathing is

satisfactory, and that the patients colour is good.
2. Assess the level of coma, looking at vital functions, pupils
and items of the Glasgow Coma Scale as mentioned above.
3. Try to establish the cause of the coma by taking a history from
relatives or witnesses, physical examination and appropriate
tests. It is obviously important to have the common causes of
coma (A, E, I, O, U) clearly in ones mind whilst questioning,
examining and ordering investigations.
4. Remember the danger of lumbar puncture in patients in
whom coning is imminent or present (see p. 44). Reduction of
the CSF pressure below the foramen magnum may encourage
further downward herniation at the tentorial or foramen magnum level, so no lumbar puncture if papilloedema is present, or until
a head scan has excluded a mass lesion or any brain swelling.
5. Treat the specific cause of the coma as soon as this is
established, e.g. anticonvulsants for epilepsy, antibiotics for
meningitis, intravenous glucose for hypoglycaemia, specific
antagonists for specific CNS depressant drug poisonings.
6. Establish the routine care of an unconscious patient regardless of the cause (Fig. 11.7).
(a) Observations: assessment every 1530 minutes of
vital functions, pupils and Glasgow Coma Scale, to monitor
improvement or deterioration in the patients condition.
(b) Airway, ventilation, blood gases.
(c) Blood pressure, to maintain adequate perfusion of the
body, particularly of the brain and kidneys.
(d) Fluid and electrolyte balance.
(e) Nutrition and hydration.
(f) Avoidance of sedative or strong analgesic drugs.
(g) General nursing care of eyes, mouth, bladder, bowels,
skin and pressure areas, passive limb mobilization to
prevent venous stagnation and contractures, chest
physiotherapy.
(e) Nutrition by
nasogastric tube
(f)
(g)
PX
morphine
(c) Blood
pressure
(b) Airway
(d) Electrolytes
Nurse
Physio
188
Prognosis for coma

Patients whose coma has been caused by drug overdose
may remain deeply unconscious for prolonged periods and
yet have a satisfactory outcome. They may need respiratory
support during their coma if brainstem function is depressed,
but proceed to make a full recovery.
Prolonged coma from other causes has a much less satisfactory outcome. As an example of this, if one takes a group of
unconscious patients, whose coma is not due to drug overdose, who:
show no eye opening (spontaneous or to voice),
express no comprehensible words,
fail to localize painful stimuli,
remain like this for more than 6 hours,
more than 50% of them will die, and recovery to independent
existence will occur in a minority of the rest. This background
knowledge must remain in the doctors mind when counselling
relatives of patients in coma, and when planning the care of
patients in prolonged coma.
Some patients who fail to recover from coma enter a state of
unawareness of self and environment, in which they can
breathe spontaneously and show cycles of eye closure and
opening which may simulate sleep and waking. This is known
as the vegetative state, which can become permanent. It is difficult, and takes a long time, to determine the precise state of
awareness in such unfortunate patients.
Brainstem death
Deeply unconscious patients whose respiration has to be
maintained on a ventilator, whose coma is not caused by
drug overdose, clearly have a worsening prognosis as each day
goes by. Amongst this group of patients, there will be some who
have a zero prognosis, whose brainstems have been damaged to
such an extent that they will never breathe spontaneously
again.
Guidelines exist to help doctors identify patients who have
undergone brainstem death whilst in coma which has been supported by intensive care and mechanical ventilation (Fig. 11.8).
Testing for brainstem death must be done by two independent,
senior and appropriately trained specialists if treatment is to be
withdrawn.
CHAPTER 11
UNCONSCIOUSNESS
189
Preconditions
In coma on ventilator
The patient is deeply comatose, and maintained on a ventilator on account

of failure of spontaneous respiration
Diagnosis certain
The coma is due to irreversible structural brain damage. The diagnosis is

certain, and is a disorder which can lead to brainstem death
No drugs
No hypothermia
No metabolic abnormality
Any of which might be having a reversible effect on the brainstem
No paralytic drugs
The patient's unresponsiveness is not due to neuromuscular paralytic agents
Tests
Pupils 1
Doll's head and caloricinduced eye movement 2
Corneal reflex 3
1 Midbrain not working

Midbrain and pons
2 not working
3 Pons not working
Gag and tracheal reflex 4
4 Medulla not working
Motor responses in cranial

nerve territory on painful
stimulation of the limbs 5
Midbrain, pons and

5 medulla not working
No respiratory movements
when PaCO2 rises above
6.65 kPa (off ventilator) 6
6 Medulla not working
Fig. 11.8 Scheme to show the guidelines that exist to help identify those patients who have undergone
brainstem death whilst in coma.
190
CHAPTER 11
CASE HISTORIES
Case 1
Case 2
A 78-year-old man is referred after three blackouts. He

feels momentarily dizzy and then loses
consciousness. He comes round on the floor. On the
first occasion he sustained a nasty facial laceration,
called an ambulance and was taken to the emergency
department, where his examination, full blood count,
glucose and ECG were all normal.The second
happened as he was finishing his lunch and he
slumped out of his chair. He recovered rapidly and did
not call an ambulance.The third happened when he
was walking his dog but he was able to get home
unaided. He has not bitten his tongue on any
occasion.
He lives alone and none of the attacks has been
witnessed. He smokes 10 cigarettes per day. He takes
bendrofluazide for hypertension. He is otherwise well.
You find no abnormalities on examination.
A young man is brought to the emergency department

by ambulance. He has been found unconscious on
waste ground.The ambulance staff cannot provide
any other history, but he has a recent prescription for
the antibiotic oxytetracycline and the anticonvulsant
sodium valproate in his wallet.
He is breathing normally and maintaining normal
oxygen saturation. His temperature, pulse and blood
pressure are normal.There are no external signs of
injury. He does not have neck stiffness.There is no
spontaneous eye opening, speech or movement. He
makes no response to verbal command but briefly
opens his eyes, groans and tries to push your hand
away in response to painful stimulation.There is no
asymmetry in his motor responses.When you open his
eyelids, his eyes are roving slowly from side to side.
Limb tone and reflexes are normal with flexor plantar
responses. General examination is normal apart from
acne. His finger-prick glucose is normal.
a. What is the differential diagnosis?

b. What further questions would you ask?
c. What tests would you request?
a. What is your differential diagnosis?

b. How would you manage his case?
12
CHAPTER 12
Epilepsy
Introduction and definitions

The word epilepsy conjures up something rather frightening
and undesirable in most peoples minds, because of the apprehension created in all of us when somebody temporarily
loses control of his body, especially if unconsciousness,
violent movement and impaired communication are involved.
Epilepsy is the word used to describe a tendency to episodes,
in which a variety of clinical phenomena may occur, caused
by abnormal electrical discharge in the brain, between
which the patient is his normal self. What actually happens
to the patient in an epileptic attack depends upon the nature
of the electrical discharge, in particular upon its location and
duration.
The most major form of epilepsy is the generalized tonic
clonic seizure, which involves sudden unconsciousness and
violent movement, often followed by coma. This form of
seizure has traditionally been called grand mal. Unless otherwise stated, mention of an epileptic attack would generally
infer the occurrence of a tonicclonic seizure.
Amongst patients and doctors a single epileptic attack may
be called an epileptic fit, a fit, an epileptic seizure, an epileptic
convulsion or a convulsion. The words tend to be used synonymously. This is a somewhat inaccurate use of words since a
convulsion (violent irregular movement) is one component of
some forms of epilepsy. There is also a confusing tendency for
older patients and doctors to use the traditional term petit mal
for any form of epilepsy which is not too massive or prolonged.
Petit mal originally had a rather restricted and specific meaning
which has been lost over time; we describe the modern terminology in the following sections. Patients and their families
very often use softer words other than epilepsy, fits, seizures or
convulsions, which is quite natural. Within families, words like
blackout, episode, funny do, attack, blank spell, dizzy turn,
fainting spell, trance, daze and petit mal abound when describing epileptic attacks.
192
Epilepsy causes attacks

which are:
brief
stereotyped
unpredictable
EPILEPSY
193
We can see that the use of words is often imprecise here. A

minor fit may mean one thing to one person and another thing
to somebody else, and it may certainly be used by a patient or
relative to describe any form of epileptic or non-epileptic attack.
We must be tolerant of this amongst patients, but there is always
a need to clarify exactly what has happened in an individual
attack in an individual patient. Errors in management rapidly
appear when the doctor does not know the precise features of
each of the patients attacks.
The word epilepsy is generally used to indicate a tendency to
suffer from epileptic attacks, i.e. more than one attack and perhaps an ongoing tendency. Some medical and non-medical people are upset by the use of the word epilepsy when applied to
someone who has only ever had one epileptic attack.
Common forms of epilepsy

There are two main types of epilepsy: primary generalized and
focal. In primary generalized epilepsy, electrical discharges
arise within deep midline structures of the brain and spread
rapidly and simultaneously to all parts of the cerebral cortex
(Fig. 12.1). In focal epilepsy, the electrical discharges start in one
part of the cerebral cortex and spread to a greater or lesser extent
to other regions (see Figs 12.5 and 12.8).
The distinction between these two main forms of epilepsy is
important, because they have different causes and respond to
different drugs. Confusion arises because focal seizures can
start in one area and eventually spread to all of the cerebral cortex, resulting in a tonicclonic seizure. This kind of attack is
called a secondarily generalized tonicclonic seizure.
Fig. 12.1 EEG recording typical of

primary generalized epilepsy.
194
CHAPTER 12
Primary generalized epilepsy

There are three common manifestations of primary generalized
epilepsy:
primary generalized tonicclonic seizures;
absence seizures;
myoclonic seizures.
Individual patients often have a mixture of these seizure types.
Primary generalized tonicclonic seizures (Fig 12.2)

There is no warning or aura.
Tonic phase. The patient loses consciousness and suddenly
stiffens, as all the muscles in his body enter a state of sustained (tonic) contraction. The limbs and neck usually extend. The patient falls stiffly with no attempt to break his fall.
He makes a loud groan as air is forced out of the chest through
tightened vocal cords. He does not breathe and becomes
cyanosed. The tonic phase is brief, usually a matter of seconds, so that observers notice a cry, a fall and stiffening of the
body before the clonic phase commences.
Clonic phase. The sustained muscle contraction subsides into
a series of random, disorganized jerks and jitters involving
any and all muscles. These convulsive movements are in no
way purposeful, coordinated or predictable. The tongue may
protrude as the jaw closes, causing tongue biting. Urinary incontinence is frequent. Breathing recommences in the same
disorganized way. It is noisy and inefficient and cyanosis
usually persists. Saliva accumulates in the mouth which,
together with the disorganized respiration, results in froth
spilling from the mouth. The patient remains unconscious.
The clonic phase typically lasts a minute or two, but can be
briefer and can occasionally be very much longer.
Phase of coma. After the convulsive movements stop, the patient is in coma. Breathing becomes regular and coordinated.
The patients colour returns to normal so long as the upper
airway is clear. The period of time that the patient remains in
coma relates to the duration of the previous tonic and clonic
phases.
There follows a state of confusion, headache, restlessness
and drowsiness before final recovery. This may last for hours.
For a day or two, the patient may feel mentally slow and
notice aching pains in the limbs subsequent to the convulsive
movement, together with a sore tongue.
No warning
Tonic phase
Loss of consciousness
Tonic contraction
No breathing
Cyanosis
Less than 1 minute
Clonic phase
Still unconscious
Convulsive movements
Self injury
Incontinence
Irregular breathing
Cyanosis
Several minutes
Coma
Still unconscious
Flaccid
Regular breathing
Colour improves
Several minutes
Confusion, headache
Hours
Sore tongue
Aching limbs
Days
Fig. 12.2 Scheme to show typical

severe tonicclonic epilepsy.
EPILEPSY
Photosensitivity
Occasional patients with all
the types of primary
generalized epilepsy may
have seizures triggered by
flashing light, for example
from the television, computer
games or flickering sunshine
when driving past a row of
trees, and have to avoid such
stimuli. Almost all such
patients also get spontaneous
seizures unrelated to visual
stimulation
195
The description given above is of a severe attack. They may

not be as devastating or prolonged. The tonic and clonic phases
may be over in a minute and the patient may regain consciousness within a further minute or two. He may feel like his normal
self within an hour or so.
During a tonicclonic epileptic attack, cerebral metabolic rate
and oxygen consumption are increased, yet respiration is
absent or inefficient with reduced oxygenation of the blood.
The brain is unable to metabolize glucose anaerobically, so there
is a tendency for accumulation of lactic and pyruvic acid in the
brain during prolonged seizure. This hypoxic insult to the brain
with acidosis is the probable cause of post-epileptic (post-ictal)
coma and confusion.
Absence seizures (Fig. 12.3)

Whole attack lasts less than
10 seconds
Young person
Sudden onset, sudden end . . .
switch-like
Unaware, still, staring
May occur several times a day

absence epilepsy.
Absence seizures are much less dramatic and may indeed pass
unnoticed. The attack is sudden in onset, does not usually last
longer than 10 seconds, and is sudden in its ending. The patient
is usually able to tell that an episode has occurred only because
he realizes that a few moments have gone by of which he has
been quite unaware. Conversation and events around him have
moved on, and he has no recall of the few seconds of missing
time.
Observers will note that the patient suddenly stops what he is
doing, and that his eyes remain open, distant and staring, possibly with a little rhythmic movement of the eyelids. Otherwise
the face and limbs are usually still. The patient remains standing
or sitting, but will stand still if the attack occurs while walking.
There is no response to calling the patient by name or any other
verbal or physical stimulus. The attack ends as suddenly as it
commences, sometimes with a word of apology by the patient if
the circumstances have been such as to make him realize an attack has occurred.
Absence seizures usually commence in childhood, so at the
time of diagnosis the patient is usually a child or young teenager. It is quite common for absence seizures to occur several
times a day, sometimes very frequently so that a few attacks
may be witnessed during the initial consultation.
196
CHAPTER 12
Myoclonic seizures (Fig. 12.4)

Myoclonic seizures take the form of abrupt muscle jerks, typically causing the upper limbs to flex and the patient to drop anything that they are holding. The jerks may occur singly or in a
brief run. Consciousness is not usually affected. They often happen in the first hour of the day, especially if the patient has
consumed alcohol the night before. Myoclonic seizures almost
always occur in association with the other generalized seizure
types, and their significance in this setting is that they help
to confirm a diagnosis of primary generalized epilepsy (most
often a specific syndrome called juvenile myoclonic epilepsy).
Isolated myoclonic jerks can occur in a wide range of conditions
with no relation to epilepsy, including waking and falling
asleep in normal people (see Chapter 5, p. 77).
One or more brief jerks

Duration up to a few seconds
Consciousness preserved
Coexists with tonic-clonic and
absence seizures

myoclonic epilepsy.
Focal epilepsy
If the primary site of abnormal electrical discharge is situated in
one area of the cortex in one cerebral hemisphere, the patient
will be prone to attacks of focal epilepsy (Fig. 12.5).
*
*
Fig. 12.5 EEG recording typical of
focal epilepsy. In this figure, the
focal EEG abnormality might
generate focal motor twitching
of the right side of the face. The
recordings marked by an asterisk
are from adjacent locations of the
brain, and show spike discharges.
The phenomena that occur in focal epileptic attacks entirely

depend on the location of the epileptogenic lesion. The most obvious form of focal epileptic attack is when the localized epileptic discharge is in part of the motor cortex (precentral gyrus)
of one cerebral hemisphere. During the attack, disorganized
EPILEPSY
197
Fig. 12.6 Scheme to show the

common forms of focal epilepsy.
Subjective
Dj vu
Memories rushing through the brain
Loss of memory during the attack
Hallucination of smell/taste
Sensation rising up the body
Objective
Diminished contact with the
environment
Slow, confused
Repetitive utterances
Repetitive movements
(automatisms)
Lip-smacking and sniffing
movements
Fig. 12.7 Scheme to show the

subjective experiences and
objective observations in a patient
with temporal lobe epilepsy.
A Focal motor seizures

Strong convulsive movements of one part of the contralateral face,
body or limbs
B Focal sensory seizures
Strong, unpleasant, slightly painful, warm, tingling, or electrical sensations
in one part of the contralateral face, body or limbs
C Frontal lobe epilepsy
Strong, convulsive turning of the eyes, head and neck towards the
contralateral side ('adversive seizure', epileptic activity in the frontal eye
fields) or complex posturing with one arm flexed and one arm extended
like a fencer (epileptic activity in the supplementary motor area)
D Temporal lobe epilepsy
Strong, disorganized aberration of temporal lobe function
(see text and Fig. 12.7)
strong convulsive movement will occur in the corresponding

part of the other side of the body. These are focal motor seizures
(Fig. 12.6).
Figure 12.6 illustrates the common forms of focal epilepsy.
Focal motor seizures, focal sensory seizures and frontal lobe seizures
are quite straightforward. Temporal lobe epilepsy deserves a special mention, not least because it is probably the commonest
form of focal epilepsy. It is necessary to remember what functions reside in the temporal lobe in order to understand what
might happen when epileptic derangement occurs in this part
of the brain. Apart from speech comprehension in the dominant
temporal lobe, the medial parts of both temporal lobes are significantly involved in smell and taste function, and in memory
(Fig. 12.7).
NB Any indication that a patients epilepsy is focal should
lead to a search for an explanation of localized cortical pathology, i.e. Why is this area of cerebral cortex behaving in this
way? What is wrong with it? In other words, these patients need
a brain scan, preferably using MRI.
Focal epileptic discharges may remain focal, but sometimes
the abnormal electrical discharge spreads over the surface of
the cerebral hemisphere and then triggers generalized epileptic
discharges involving all parts of both cerebral hemispheres
(Fig. 12.8).
198
CHAPTER 12
(a)
(b)
If the focal discharge begins in one part of the motor cortex,

the first symptom might be convulsive twitching of the right
side of the face, spreading rapidly into the right arm and then
the right leg before affecting the whole body in a secondarily
generalized tonicclonic seizure. This particular kind of attack
is known as Jacksonian epilepsy (after the nineteenth-century
neurologist Hughlings Jackson).
Any warning or aura in the moments before a tonicclonic
seizure indicates that it is a manifestation of focal epilepsy
rather than primary generalized epilepsy, with the implication
that there is a problem with a localized area of the cerebral cortex which must be investigated. The nature of the aura depends
on the location of this area, and it can take any of the forms listed
in Figs 12.6 and 12.7. Sometimes the patient himself may not be
aware of the warning, if for example it simply consists of turning the eyes and head to the side or extending one limb. This
highlights the importance of talking to someone who has seen
the patient right through an attack.
The main indication that a tonicclonic seizure has a focal
cause and that the patient does not suffer from primary generalized epilepsy sometimes occurs after the fit rather than before it.
A tonicclonic seizure which is followed by a focal neurological
deficit (e.g. weakness of the right side of the body), lasting for an
hour or two, strongly indicates a localized cortical cause for the
fit (in or near the left motor cortex in this example). The tell-tale
post-epileptic unilateral weakness is known as Todds paresis. It
may be apparent to the patient, but is commonly recognized
only by a doctor examining the patient shortly after the
tonicclonic seizure has ceased.
Fig. 12.8 (a) EEG showing seizure

discharges that have spread
across all of the left hemisphere
(lower eight tracings). (b) A few
seconds later the seizure
discharges have become
generalized and more intense.
EPILEPSY
199
Febrile convulsions
The young, immature brain of children is less electrically stable
than that of adults. It appears that a childs EEG is made more
unstable by fever.
Tonicclonic attacks occurring at times of febrile illnesses are
not uncommon in children under the age of 5 years. Such attacks
are usually transient, lasting a few minutes only. In the majority
of cases, the child has only one febrile convulsion, further
convulsions in subsequent febrile illnesses being unusual
rather than the rule.
A febrile convulsion generates anxiety in the parents. The
attack itself is frightening, especially if prolonged, and its
occurrence makes the parents wonder if their child is going to
have a lifelong problem with epilepsy.
The very small percentage of children with febrile convulsions who are destined to suffer from epileptic attacks unassociated with fever later in life are identifiable to a certain extent by
the presence of the following risk factors:
a family history of non-febrile convulsions in a parent or
sibling;
abnormal neurological signs or delayed development identified before the febrile convulsion;
a prolonged febrile convulsion, lasting longer than 15
minutes;
focal features in the febrile convulsion before, during or after
the attack.
Those who do go on to have epilepsy in later life usually have
a pathology called mesial temporal sclerosis affecting one
hippocampus, giving rise to focal epilepsy with temporal lobe
seizures. They may be cured by having the abnormal hippocampus surgically resected.
200
Epilepsy syndromes
There are a large number of recognizable epilepsy syndromes,
which are mainly rare, but two require special mention.
Juvenile myoclonic epilepsy

This is the commonest cause of tonicclonic seizures starting in
teenagers. It is a form of primary generalized epilepsy, so the
tonicclonic seizures come on without warning. They often follow a late night with too much alcohol. The patient may not
volunteer the characteristic history of myoclonic seizures
unless asked, having thought that their messy breakfasts were
due to clumsiness rather than epilepsy. Similarly their absence
seizures may have been attributed to day-dreaming. Crucially,
juvenile myoclonic epilepsy is not usually fully controlled by
carbamazepine or phenytoin, but it is by sodium valproate or
lamotrigine. It is upsetting for all concerned when patients receive the wrong treatment for many years, with all the disability
that attends poorly controlled epilepsy.
LennoxGastaut syndrome
Lennox (no relation) and Gastaut described one of the most severe forms of so-called epileptic encephalopathy, where a range
of metabolic and genetic disorders of brain development give
rise to severe learning disability and epilepsy. In addition to all
the seizures types described above, patients with Lennox
Gastaut syndrome (LGS) may have seizures characterized by
isolated tonic or clonic phases. They also experience a very
distressing type of seizure called atonic seizures, in which
there is abrupt loss of consciousness, muscle power and tone.
The patient falls abruptly, often injuring his head or face.
The prognosis in LGS is very poor, although some of the newer
anticonvulsants have a partial effect in reducing seizure
frequency.
CHAPTER 12
EPILEPSY
201
Non-epileptic attacks
The differential diagnosis of epilepsy, including non-epileptic
attacks of psychogenic origin, has been discussed in Chapter 11
(see p. 180). It is worth repeating here that non-epileptic seizures
generally resemble tonicclonic seizures but tend to be more
prolonged, to involve more coordinated and purposeful movements (arching the back repetitively, turning the head from side
to side, grasping attendants) and to preserve reflexes which are
lost in tonicclonic seizures (pupil, lash and tendon stretch reflexes and flexor plantar responses). Cyanosis does not occur
unless the diagnosis has been missed and the patient has been
given sufficient intravenous lorazepam or diazepam to suppress respiration. Because of the risk of iatrogenic respiratory
arrest, it is important, but sometimes difficult, to distinguish
lengthy non-epileptic attacks from status epilepticus.
Status epilepticus
Unless otherwise stated the term status epilepticus indicates the
occurrence of one tonicclonic seizure after another without
recovery of consciousness between attacks. During tonicclonic
seizures, there exists a state of increased cerebral metabolism
and oxygen requirement and decreased respiratory efficiency
and cyanosis. If further tonicclonic seizures occur at short
intervals, it is easy to understand that increasing metabolic
acidosis and oedema will occur in the brain, and progressively
increasing coma will overtake the patient. There is evidence
that, even if hypoxia and metabolic acidosis are prevented,
seizures themselves can cause permanent brain damage if they
last more than 1 hour (as a toxic consequence of excessive release of excitatory neurotransmitters). Hence, urgent control of
seizures and attention to respiration are required in patients
with tonicclonic status, usually needing admission to an intensive care unit. The treatment is discussed in more detail on
p. 208. Even with careful management, status epilepticus has
a significant mortality rate and it is common for survivors to
have new cognitive or physical difficulties or more refractory
epilepsy.
202
Diagnosis
Clinical history and a good witnesss account
Epilepsy is a difficult condition to diagnose, and mistakes
(both underdiagnosis and overdiagnosis) are not infrequent.
Certainty of diagnosis in patients with epilepsy depends
mainly on the establishment of a clear picture of the features of
the attack both from the patient and from a witness. This
applies to the diagnosis of any sort of blackout, as emphasized
in Chapter 11. One has to go through the precise details of how
the patient felt before, during (if conscious) and after the
attack, and also obtain a clear description of how the patient behaved during each stage from a witness. One cannot overstate
the importance of the clinical history in evaluating attacks.
Investigations, such as EEG, should be used to support a
diagnostic hypothesis based on the clinical information. Diagnostic certainty is always much more difficult in unwitnessed
episodes of loss of consciousness.
The following comments can be made about the differential
diagnosis of the different forms of epilepsy.
Tonicclonic seizures have to be differentiated from the other
causes of attacks of loss of consciousness mentioned in Chapter 11 (see Fig. 11.2, p. 176). Cardiovascular disorders are the
main consideration (simple faints and vasovagal syncope in
young patients, arrhythmias and postural hypotension in
older patients). Confusion often arises because of the brief
flurry of myoclonic jerks that sometimes accompany syncope
of any cause. Non-epileptic attacks of a psychogenic kind are
a common source of diagnostic difficulty in specialist clinics.
Diabetics on treatment of any sort should prompt one to
think of hypoglycaemia. In general, tongue biting, irregular
noisy breathing, strong convulsive movement, incontinence,
post-ictal confusion and limb pains all suggest tonicclonic
epilepsy.
Absence seizures may be confused with absent-minded daydreaming, or with wilful inattention of a young person to his
environment. The sudden nature of the start and finish of the
trance, switch-like, typifies absence epilepsy.
Focal motor seizures do not really have a differential diagnosis.
Focal sensory seizures may be difficult to differentiate from
episodes of transient cerebral ischaemia, but are usually
shorter and more frequent, and cause tingling rather than
numbness.
Frontal lobe seizures can be confused with dystonia (see
Chapter 5, p. 75) and can appear so bizarre that they are
thought to have a behavioural basis.
CHAPTER 12
Listen to the patient

Listen to a witness
in detail
in person
on the phone
via letter
EPILEPSY
203
Temporal lobe seizures mainly have to be distinguished from

anxiety and panic attacks. Attacks which are provoked in any
way, or which last more than a few minutes, are unlikely to
be due to temporal lobe epilepsy. Attacks which involve
unusual behaviour requiring alert, clear thought and/or
well-coordinated physical behaviour (fighting, shoplifting)
are most unlikely to be due to epilepsy.
Establishing the cause of epilepsy

Think about the cause
Family history
Past history
System review
Alcohol history
Drug history
Focal neurological
symptoms or signs
Apart from accurate information regarding the attacks, there is

further information to be elucidated at the interview and examination. If the attacks are thought to be epileptic, an enquiry
should be mounted as to the cause of the epilepsy. Primary generalized epilepsy (primary generalized tonicclonic, absence or
myoclonic seizures, with or without photosensitivity) is familial, so questioning may reveal other members of the family who
suffer from epileptic attacks. Any form of focal epilepsy (and
some cases with no apparent focal features) reflects the presence
of intracranial pathology. Most commonly, this pathology is an
area of scarring subsequent to some previous active pathology,
though sometimes epileptic attacks may occur when the pathological process is in the active phase:
following birth trauma to the brain;
following trauma to the skull and brain later in life;
during or following meningitis, encephalitis or cerebral
abscess;
at the time of, or as a sequel to, cerebral infarction, cerebral
haemorrhage or subarachnoid haemorrhage;
as a result of the inevitable trauma of neurosurgery.
Sometimes, the epileptic attacks are caused by biochemical
insults to the brain, rather than by localized physical disease,
such as:
during alcohol or drug withdrawal;
during hepatic, uraemic, hypoglycaemic comas;
whilst on major tranquillizing or antidepressant drugs.
At the front of ones mind must be the possibility that the patients epilepsy is an early symptom of a brain tumour. Brain tumours are not a common cause of epilepsy, but they are not to be
missed. Epilepsy of adult onset, especially if focal and/or associated with evolving abnormal neurological signs, should initiate strong thoughts about the possibility of a tumour.
204
CHAPTER 12
Physical examination
Physical examination is clearly important since it may reveal
abnormal neurological signs which indicate evidence of:
previous intracranial pathology;
current intracranial pathology;
evolving intracranial pathology as indicated above.
EEG and other investigations

The EEG is not a test for epilepsy. In the appropriate clinical
setting (i.e. a patient with a history suggestive of epilepsy) it
may provide additional support for the diagnosis and help
in distinguishing between the generalized and focal types of
epilepsy. But remember:
10% of the normal population show mild, non-specific EEG
abnormalities, such as slow waves in one or both temporal
regions;
30% of patients with epilepsy will have a normal EEG
between their attacks (falling to 20% if the EEG includes a
period of sleep).
In other words, the EEG can mislead through both false positives and false negatives, and must be requested and interpreted with care. Prolonged ambulatory EEG recording (an
immensely laborious activity) can be useful in very difficult
cases with frequent attacks, allowing the EEG to be sampled
during the attack itself. But even here there is a false-negative
problem, with 10% of focal seizures arising deep in a fold of the
cerebral cortex and failing to give rise to abnormal discharges
on the EEG.
Imaging of the brain, preferably with MR rather than CT
scanning, is an important part of the assessment of focal epilepsy, and in cases where the type of epilepsy is uncertain. It is probably unnecessary in patients with primary generalized epilepsy
confirmed by EEG.
The other routine tests (glucose, calcium, ECG) are very
rarely informative.
Summary of the stages in diagnosis in a patient with epilepsy

1.
2.
3.
4.
Listen to the patient.

Listen to witnesses.
Wonder why the patient has epilepsy.
Try to establish a cause:
from the history;
from the examination.
5. Investigate by EEG, often by MR brain scan,

sometimes by other tests.
6. Assess patient reaction and the consequences of
the diagnosis of epilepsy in his or her life.
EPILEPSY
205
Management
On the spot
Airway
Prevent injury
Await recovery
If somebody suddenly has a tonicclonic fit, people close at

hand should:
1. look after the patients airway (by putting the patient into the
recovery position when you can and pulling the jaw forward);
2. prevent self-injury during strong convulsive movements (by
keeping the patient away from hard, sharp or hot objects).
Relatives of newly diagnosed epileptic patients certainly appreciate advice of this sort. In an adult, one would generally
wait for the convulsions to cease and for the patient to regain
consciousness. If one can establish that the patient is subject to
epileptic fits, no further medical action is required. If the patient
has never had a fit before, and especially if there are focal features to the fits or post-ictal stage, further medical advice will be
required either immediately or in the next few days. In children
with febrile convulsions, diazepam may be administered per
rectum if it is available, and further medical attention should be
sought if the convulsion has not stopped (with or without
diazepam) within 5 minutes.
Explanation
Reassurance
Listen to patients anxieties
Listen to familys anxieties
Correct any wrong
preconceptions
Generally supportive comments and explanation about

epilepsy, appropriate to the level of intelligence of the patient
and his relatives, constitute the first steps in the management of
newly diagnosed epilepsy. The actual word epilepsy greatly
upsets some families. It is a good idea to ask the patient and relatives what epilepsy means to them as a starting point for explanation and reassurance. Not uncommonly, it is the relatives,
rather than the patient, who are more anxious about attacks
involving unconsciousness. They need to be reassured that
although the epileptic attack may have looked profoundly
life-threatening, death during epileptic convulsions is actually
extremely rare. Associations of epilepsy with mental subnormality, madness, brain tumours, epileptic homes, incurability,
transmission to the next generation, etc. are all likely to be introduced during this phase of explanation, and it is important that
the doctor has a chance of correcting preconceived ideas in the
patient or his family at the outset.
The facts that young people tend to grow out of their tendency to epilepsy, and that epilepsy can be controlled by drugs
in the majority of cases, need to be clearly stated.
206
Drug therapy
Positive motivation towards taking anticonvulsant drugs,
which have side-effects, and which may need to be taken regularly for several years, is not automatic in patients with epilepsy.
It will be optimized by careful explanation about the drugs, and
allowing time for the patient to express his feelings about the
prospect of taking the pills regularly.
You need to help the patient to decide which drug has the best
chance of controlling the seizures with the least risk of sideeffects that would be unacceptable from their point of view.
Patients will prioritize possible side-effects differently depending on their circumstances: sedation may be a major concern to
an academic; teratogenic hazard may be the greatest worry to a
woman who is planning a family. Once you have chosen a drug,
this should be introduced gradually with the aim of finding the
smallest dose that completely suppresses the seizures. If the
patient builds the dose up to a level that is causing side-effects
and is still having seizures, then you need to move on to another
drug. Serum anticonvulsant levels are not an important part of
this titration process, but can be a way of making sure the
patient is actually taking the pills.
Primary generalized epilepsy usually responds to drug treatment. The first-line drugs are sodium valproate and lamotrigine. Myoclonus often responds to the addition of clonazepam,
and absences may respond specifically to ethosuximide. There
are several second-line options, including phenobarbitone,
topiramate and levetiracetam.
The choice of drugs for focal epilepsy is even wider, but only
80% of patients achieve complete control. The first-line drugs
are carbamazepine, sodium valproate and lamotrigine. If none
of these drugs work on their own, then the possibility of surgical
treatment should be considered (see p. 210) before moving on to
a combination of a first-line and a second-line drug (phenytoin,
topiramate, levetiracetam, gabapentin, phenobarbitone, etc.). If
the patient responds to the combination, think about withdrawing the first-line drug to see if control is maintained on the
second-line drug alone. In general, most patients prefer to take a
single drug because this reduces the risk of side-effects; from the
doctors point of view, it also reduces the risk of interactions between the two drugs and, in the case of those anticonvulsants
metabolized by the liver, interactions with other drugs such as
the oral contraceptive and warfarin.
Most anticonvulsants have both idiosyncratic (occasional
and unpredictable) side-effects and dose-dependent ones. The
most important side-effects of the first-line drugs are shown in
the table on the next page. Teratogenicity (for example neural
tube defects) is a serious concern when treating young women.
CHAPTER 12
EPILEPSY
207
Idiosyncratic side-effects
Dose-dependent
side-effects
Approximate
teratogenic risk
Valproate
Weight gain, transient hair loss,

liver dysfuction
Tremor, sedation
>10%
Carbamazepine
Rash, hyponatraemia
Ataxia, sedation
5%
Lamotrigine
Rash, influenza-like symptoms
Sedation
23%
They should be counselled about this before starting treatment

and again before any planned pregnancy. Folate supplements
may reduce some of this risk.
It is generally possible to treat most patients with a twicedaily regimen (phenobarbitone, phenytoin and slow-release
preparations of sodium valproate can be given once daily). This
helps patients to remember their pills. Sticking to a thrice-daily
schedule is very difficult.
Once anticonvulsant treatment is started, it is usual to maintain the treatment for a minimum of 2 or 3 years. If the seizures
have been fully controlled, treatment can be withdrawn gradually but there is a high risk (about 40% overall) of seizures returning. The risk is much higher in some forms of epilepsy such
as juvenile myoclonic epilepsy, in patients where it has taken
several different drugs to achieve control, and where the epilepsy was caused by a structural brain lesion that is still present.
Even patients with lower risk may prefer to stay on treatment
rather than take a chance of losing their driving licence by having another attack.
Febrile convulsions
Cool the child
Rectal diazepam
Consider meningitis
The main things to remember here are the early use of rectal
diazepam, steps to cool the child (including paracetamol),
correct management of any underlying infection (with particular consideration to the possibility of meningitis) and reassurance of parents about the benign nature of febrile convulsions.
208
Status epilepticus
Any form of status epilepticus is an indication for hospitalization to establish control of the seizures, but in the case of
tonicclonic status the problem is a medical emergency requiring admission to an intensive care unit. There are three main
directions of treatment of grand mal status:
1. Routine care of the unconscious patient (see Chapter 11,
p. 187).
2. Control of seizure activity in the brain. This means the maintenance of the patients normal anticonvulsant regime, supplemented by the use of intravenous anticonvulsants; diazepam or
lorazepam early; phenytoin or phenobarbitone if seizures
persist; thiopentone to produce general anaesthesia if seizures
have failed to come under control within 60 minutes.
3. Maintenance of optimal oxygenation of the blood. This may
mean the use of oxygen and an airway, but may be the main
indication for anaesthesia, paralysis and ventilation. It is
important to remember the control of seizure activity in the
brain of the paralyzed. Vigorous anticonvulsant therapy must
be continued.
CHAPTER 12
Emergency admission
Care of the unconscious
patient
Continue and intensify
anticonvulsant therapy
i.v. benzodiazepine
i.v. phenytoin
i.v. barbiturates
Maintain oxygenation
Sensible restrictions
Until satisfactory control of epileptic attacks has been established, and for a little while longer, it is important for patients
with most forms of epilepsy to be aware of the dangers of
bathing, driving, riding a bicycle, heights, open heavy machinery, swimming and water sports. Advice to each patient has to
be individualized, bearing in mind the type and frequency of
his epileptic attacks. In the UK, the driving licensing authorities
have definite guidelines for patients and doctors to follow, and
it is the doctors role to make sure that the patient understands
where he stands in relation to these. A patient who has had one
or more attacks of epilepsy has to refrain from driving until 1
year has elapsed, regardless of whether the patient is on medication or not. This is the general rule in the UK, which has
exceptions for nocturnal attacks and provoked attacks. Very
gentle but firm explanation is often required when pointing out
the need for these restrictions.
Most patients with photosensitive epilepsy can attend discos
where lights flash at rates too slow to stimulate epilepsy. Such
patients should avoid video games and sit well back from the
TV screen.
Driving
Other potentially
dangerous pursuits
Understand frustration
EPILEPSY
209
Occupation
The financial benefits and personal prestige of working are
important in the life of a patient with epilepsy, just as for
everybody else. Nevertheless, it is more difficult for people with
epilepsy to become employed. Some occupations are completely closed to patients with epilepsy, e.g. jobs requiring an
HGV or PSV driving licence, and jobs in the armed forces, police
or fire services. Some occupations may be very difficult for
patients with incompletely controlled epilepsy, e.g. teaching,
working with young children, nursing, and working near fire or
water, at heights, or around unguarded machinery. Individual
employers may need education and encouragement to accept
that their employee with epilepsy can continue to work normally. In many countries disability discrimination legislation is
helping to strengthen their resolve.
Special considerations in women with epilepsy

1. As already mentioned, the metabolism and transport systems of anticonvulsant drugs interact with those of oral contraceptive drugs, so special advice and monitoring are necessary if
the two sorts of medication are to be taken concurrently.
2. All anticonvulsant drugs seem to be teratogenic to some
extent. Stopping all medication and experiencing uncontrolled
epilepsy during pregnancy may pose a greater risk to mother
and fetus, although scientific data are extremely scanty. The aim
should probably be to control all but the mildest forms of epilepsy using a single drug of low teratogenicity, in the lowest effective dose. The metabolism of most anticonvulsant drugs is
increased during pregnancy, so drug level monitoring may
show the need for slightly increased anticonvulsant dosage
during pregnancy.
3. Most anticonvulsants appear in the mothers milk during
lactation, but not in quantities that affect the neonate. Mothers
on anticonvulsants should breast feed if this is their wish.
4. Focal epilepsy, due to an epileptogenic cortical scar, is not an
hereditary condition. Idiopathic, primary generalized, epilepsy
is a familial condition so a little genetic counselling may be
necessary in such cases. The risk of epilepsy in a child born to a
couple, one of whom suffers from idiopathic epilepsy, is small.
If both parents suffer from epilepsy, the risk becomes much
greater (up to 1 in 4).
5. Care of infants and toddlers may be difficult for a parent
who is unlucky enough to suffer from epileptic attacks which
remain frequent despite drug therapy.
Interaction of
anticonvulsants and oral
contraceptives
Teratogenicity of
anticonvulsants
Genetics
Breast feeding
Childcare
210
Psychological factors
1. Unpredictable attacks of one sort or another, the word
epilepsy, the need to take tablets, restrictions on driving and
some recreational activities, exclusion from some occupations
and difficulty in obtaining employment may all make patients
with epilepsy feel second class, depressed, victimized or aggressive. The size of this psychological reaction is a measure of
the patients personality on the one hand, and of the support he
receives from his family and doctor on the other.
2. Patients and relatives have particular problems accommodating seizures which are themselves prolonged, or from which
it takes the patient a long time to recover. Self-injury in the
seizures, and severe automatisms (shouting, undressing, running, hitting) are also very upsetting.
3. Stress is probably not a major factor in causing individual
attacks in a person prone to epileptic fits. It is perfectly natural
for patients to look round for causes explaining the occurrence
of attacks, but the main difficulty of most patients epilepsy is
its complete unpredictability.
4. Emotional thought and behaviour are aspects of normal temporal lobe function. In patients with abnormal temporal lobes,
for whatever reason, there may be some abnormalities of such
functions in addition to temporal lobe epilepsy. This is not an inevitable association but it is one to bear in mind when managing
patients with this form of epilepsy.
5. If an epileptic patient has difficulty coping with life and develops psychosomatic symptoms, it is possible that he will start
to have blackouts that are not epileptic but emotional in origin.
This need for careful differentiation applies to individual attacks, and also to status epilepticus versus prolonged nonepileptic attacks. It emphasizes the need to establish the precise
features of a patients attacks for correct management.
Surgical treatment
Occasionally, in a patient with a highly localized epileptogenic
focus, which is producing intractable epileptic attacks, the focal
area of the brain may be removed with benefit. Identification of
the small percentage of patients with epilepsy who are likely to
benefit from such surgery has improved very significantly in
the last decade. It is more common in patients with focal pathology which has been clearly demonstrated on the medial side of
the temporal lobe by sophisticated EEG and imaging techniques (MR scan). Outcomes are best in children and young
adults; older patients may have difficulty in picking up the
threads of a life disrupted by epilepsy even if the seizures themselves are abolished.
CHAPTER 12
The patients reaction, and

that of his family, are
important to understand, and
to modify if inappropriate
Surgical treatment should be

considered if:
drugs are failing
a focus can be confidently
identified, with
concordance between:
clinical features
EEG
MR scan
the focus is resectable
the patient wishes to
proceed
EPILEPSY
211
CASE HISTORIES
Case 1
Case 2
A psychiatric colleague asks you to see a patient who

has been referred to him because of possible
psychosis.The 34-year-old business woman gives a
6-month history of experiences which she believes
have religious meaning. She finds it hard to put the
experiences into words but describes a warm
sensation in her stomach which rises rapidly to her
head and is accompanied by a pleasant feeling of
tranquillity and a feeling that she understands all the
mysteries of the world.This lasts less than a minute.
The experiences happen unpredictably several times
each week. Between these times she feels entirely
normal and has no psychiatric symptoms apart from
anxiety about the cause of her symptoms.
Neurological examination is normal.
A previously healthy 25-year-old security guard is

admitted to the emergency department in status
epilepticus. His generalized tonicclonic seizures
started 25 minutes ago. He is cyanosed despite
supplementary oxygen, with a tachycardia and fever
of 39.0C.

b. How would you manage her case?
a. Outline your emergency management.

Ten minutes later the seizures have stopped but he
remains deeply unconscious and he is transferred to
ITU, intubated and ventilated. Over the next hour his
fever increases to 39.8C.
b. What would you advise now?
13
CHAPTER 13
Headache and facial pain
Introduction
Any pain in the head or face must reflect activity in some painsensitive structure, and attempts have been made to classify
headache and face pains on this basis. The idea seems intellectually attractive but has little else to commend it. In practice it is
more useful to think of four broad categories of head and face
pain (see the table below), and to become familiar with the common disorders in each category. By far the most common are the
longstanding headaches which affect the patient most of the
time, namely tension headaches and analgesic-dependent
headaches, and the longstanding but intermittent headaches of
migraine. Contrary to public belief, it is extremely rare for brain
tumours to present with chronic headache without additional
symptoms.
Head pain
Face pain
Chronic, continual
Tension headache
Analgesic-dependent headache
Atypical facial pain
Chronic, episodic
Migraine
Cluster headache
Icepick headache
Subacute, evolving

Giant cell arteritis
Acute, severe
Subarachnoid haemorrhage
Benign sex headache
213
214
CHAPTER 13
Tension headache
Tension headache is very common. It is frequently described as
a tight band around the head, often radiating into the neck. Most
of us have experienced headaches of this kind from time to time,
when tired or stressed. Patients who seek medical advice about
tension headaches tend to get them most of the time and with
considerable severity. The source of the pain is believed to be
chronic contraction of the neck and facial muscles.
There is usually a background of stress and worry, sometimes
with clinically significant anxiety or depression. The patient is
often concerned about the possibility of a brain tumour, creating
a vicious circle where headaches cause anxiety and anxiety
causes more headaches.
Treatment therefore starts with trying to help the patient to
understand the nature of the headache, with reassurance (based
upon a careful neurological examination) that there is no serious physical cause. Some patients cannot be reassured without
a brain scan but this is not a good use of resources from a medical
point of view. Modification of lifestyle may be desirable but cannot always be achieved. Relaxing therapies and small nighttime doses of amitriptyline may be beneficial. Underlying
depression should be treated if present. Regular analgesic use is
generally counterproductive (see p. 216). It is often difficult to
satisfy patients complaining of tension headaches.
'You've got to do something Doctor.'
Migraine
Migraine affects about 15% of women and 8% of men. It is
familial. Most patients experience their first attack before the
age of 40 years. It characteristically causes episodes of
headache, lasting between a few hours and a few days; the
patient feels normal between these attacks.
Attacks may be precipitated by a wide range of triggers,
and commonly by a combination of these. Most patients have a
prodrome, for example yawning or passing urine frequently.
Some then have an aura, which characteristically evolves over a
period of about 20 minutes. The commonest is visual, with an
area of blurred vision or an arc of scintillating zigzags slowly
spreading across the visual field. Less commonly there may be
tingling, for example in one hand spreading slowly to the ipsilateral tongue, or dysphasia, or unilateral weakness, or a succession of all three. It used to be thought that these aura
symptoms reflected cerebral ischaemia, but recent research has
shown that the phenomena have a neuronal rather than a
vascular basis.
The next phase of the attack is the headache itself, although
older patients may find they gradually stop experiencing this.
'I'm OK in between, but when it

comes I'm awful, I'm sick and I can't
see properly.'
HEADACHE AND FACIAL PAIN
Some triggers for migraine

Stress and fatigue
Relaxation after stress
(Saturday morning
migraine)
Skipping meals
Binge eating
Specific foods (cheese,
citrus fruits, etc.)
Specific drinks:
caffeine (too much or
sudden withdrawal)
red wine (too much)
Menstruation or ovulation
Oral contraceptive
Early post-partum period
Menopause
Bright sunshine, flicker or

patterns
Strong smells
Hypertension
Head injury
215
The headache is typically throbbing, severe, felt at the front of

the head and worse on one side, and lasts for several hours. It is
often accompanied by pallor, nausea or vomiting, and an intense desire to lie still in a quiet darkened room. A period of
sleep will often terminate an attack. A few unfortunate patients
have attacks that last for a couple of days.
Patients with migraine have no abnormal physical signs on
examination. So long as this is the case, brain scanning is not
required.
It is not always possible to persuade the patient to pursue the
life of sustained tranquillity and regularity that seems to suit
migraine best. They should, nonetheless, be encouraged to
avoid their triggers where possible. Individual attacks may respond to simple analgesia taken promptly, if necessary together
with a dopamine antagonist, e.g. aspirin and metoclopramide.
Failing that, serotonin agonists such as ergotamine and the triptan family of drugs can be of great benefit. If vomiting is pronounced, such agents can be given sublingually, by nasal spray,
by suppository or by injection. Some patients respond well,
only to get a rebound attack the following day. This is a particular problem with ergotamine but can also occur with the triptans and can lead to overuse of medication and a chronic
headache (see overleaf).
Preventive treatment should be considered if migraine attacks are frequent or when there are rebound attacks. Regular
administration of beta-adrenergic blockers such as atenolol, the
tricyclic amitriptyline and the anticonvulsant sodium valproate
all have significant benefits. Current research suggests that
some of the newer anticonvulsants may be even more effective,
supporting the view that migraine may be a disorder of ion
channels.
216
CHAPTER 13
Cluster headache
Cluster headache gets its name from its tendency to occur
repetitively, once or twice each day, for several weeks, with long
intervals of a year or more until it recurs in the same way. During
the cluster, the attacks themselves are brief, lasting between 30
and 120 minutes. They often occur at the same time in each
24-hour cycle, with a predilection for the early hours of the
morning. Functional brain imaging studies have shown activation of the brains clock and central pain-sensitive areas at the
onset of the attack.
Cluster headache is much less common than migraine, and
mainly affects men. The pain is excruciatingly severe, located
around one eye, and accompanied by ipsilateral signs of autonomic dysfunction including redness and swelling, nasal
congestion, or Horners syndrome. The pain can sometimes
be controlled by inhaling high-flow oxygen or injecting
sumatriptan.
Prophylactic treatment can be enormously helpful, starting
with steroids and verapamil and moving on to methysergide or
anticonvulsants (such as topiramate) if necessary.
'Mainly at night, I could set the

clock by it. Really severe.'
Icepick headaches
Icepick headaches are momentary jabbing pains in the
head. They often occur in people who also have migraine, or
tension headaches, or both. They are alarming but entirely
benign. Reassurance is usually all that is required but regular
administration of non-steroidal anti-inflammatory drugs can
suppress them.
'I'm scared of them.'
Analgesic-dependent headache
Patients with either tension headaches or migraine can develop
headaches due to overuse of analgesia, especially the milder
opiates such as co-proxamol and codeine, ergotamine and the
triptans. They develop a daily headache, often with a throbbing
quality, that is transiently relieved by the offending drug. Treatment consists of explanation, gradual introduction of headache
prophylaxis (such as amitriptyline) and planned, abrupt discontinuation of the analgesic. The daily headaches usually settle after a short but unpleasant period of withdrawal headache.
'I daren't stop the pills.'
217
'It's not too bad really. Mainly in the

mornings when I wake.'
Patients with raised intracranial pressure often have little or no

headache, and when they do have headache, it often lacks specific features. The diagnosis of raised intracranial pressure
therefore largely relies upon the detection of the focal symptoms and signs of the causative intracranial space-occupying
lesion (tumour, haematoma, abscess, etc.) or the other features
of raised intracranial pressure (impairment of conscious level,
vomiting, papilloedema). Such features tend to arise in the context of a subacute and clearly deteriorating illness, and require
prompt investigation with brain scanning. Lumbar puncture
should not be performed unless a focal cause has been confidently excluded.
The headaches of raised intracranial pressure tend to be
worse when the patient is lying flat, and may therefore wake the
patient from sleep or be present on waking. Migraine is, however, a much more common cause of headaches with these
features. As a general rule, raised-pressure headaches are
more likely to be occipital and mild.
Low intracranial pressure
'I'm OK lying down, but the headache's

awful when I'm upright.'
Low-pressure headaches have the opposite characteristic: they

are relieved by lying flat but rapidly return when the patient resumes an upright posture. They may follow a lumbar puncture
(diagnostic or accidental during epidural anaesthesia), due to a
persistent leak of CSF through the hole in the theca. They can
also occur spontaneously, because of a leak arising in a thoracic
nerve root sheath following coughing or air travel. They often
settle with bedrest and caffeine supplements.
Benign sex headache
'Right at the moment when

I'm most excited.'
Benign sex headache causes a sudden, severe headache which

occurs at the moment of orgasm in men or women. The first
attack may be difficult to distinguish from subarachnoid haemorrhage (which can, of course, occur at times of exertion). It is
very reassuring if one discovers a history of previous attacks
under similar circumstances. Happily, and unlike subarachnoid haemorrhage, benign sex headaches are not associated
with loss of consciousness or vomiting. The attacks tend to disappear spontaneously but beta-adrenergic blockers can be a
very effective preventive treatment.
218
CHAPTER 13
Giant cell arteritis

In elderly people, the extracranial and the intra-orbital arteries
may become affected with an arteritis which is painful and
dangerous. The danger lies in the fact that the lumen of these
arteries may become obliterated because of the thickening of
their walls and associated thrombosis.
Patients with giant cell arteritis generally feel unwell, short of
energy and apathetic. The condition overlaps with polymyalgia
rheumatica, in which similar symptoms are associated with
marked stiffness of muscles.
The arteritis causes headache and tenderness of the scalp
(when resting the head on the pillow, and when brushing the
hair), because of the inflamed arteries. The superficial temporal
arteries may be tender, red, swollen and non-pulsatile. The condition is sometimes known as temporal arteritis because of the
very frequent involvement of the superficial temporal arteries,
but the facial arteries are often involved, as are other arteries in
the scalp.
The arterial occlusive aspects of the disease chiefly concern
the small branches of the ophthalmic artery in the orbit. Sudden
and irreversible blindness due to infarction of the distal part of
the optic nerve is the main danger.
Giant cell arteritis is an emergency requiring urgent estimation of the ESR (usually elevated above 60 mm/hour, with an
accompanying elevation in C-reactive protein) and immediate
high-dose steroid treatment. In all but the most clear-cut cases,
the diagnosis should be rapidly confirmed with a temporal
artery biopsy. Most patients will continue to need steroids in
much diminished doses for a couple of years, and sometimes for
much longer.
'I don't feel at all well, Doctor. I can't

put my head on the pillow.'
Subdural haematoma
Elderly people, alcoholics and people on anticoagulants who
bang their heads may subsequently develop a collection of
blood in the subdural space. This condition is described in more
detail elsewhere (see pp. 64 and 232). The usual presentation is
with cognitive decline. Occasional cases present with a subacute headache and raised intracranial pressure.
'He's sleepy, slow and unsteady.

He wet the bed last night.'
219
Subarachnoid haemorrhage and meningitis
'Never had a headache like this before.

My neck feels stiff.'
When the meninges are irritated and inflamed, pain is felt

throughout the head and neck, especially in the occipital region.
Forward flexion of the neck moves the inflamed meninges, and
is involuntarily resisted by the patient. This gives rise to the
classical sign of meningeal irritation (meningism) known as
neck stiffness.
The headache and neck stiffness are severe and sudden in
onset when the meningeal irritation is due to blood in the
subarachnoid space (subarachnoid haemorrhage) (see pp.
327). The symptoms and signs evolve a little more gradually in
the case of meningitis caused by pyogenic bacteria and acute
viral infection (see pp. 2424). Evidence of raised intracranial
pressure (depression of conscious level, papilloedema) and abnormal neurological signs are not uncommon in patients with
subarachnoid haemorrhage or meningitis. Urgent admission to
hospital (for CT brain scanning, lumbar puncture, antibiotics,
etc.) is the ideal early management of patients with headaches
associated with neck stiffness.
'Sudden cruel severe stabs of pain

which make me jump.'
Trigeminal neuralgia causes sudden, momentary pains like

electric shocks (lancinating pain) in the distribution of one
trigeminal nerve. The pain is unilateral, usually in the maxillary
or mandibular territory, close to the mouth or nose. Each stab of
pain is severe and sudden enough to make the patient jump
(hence the French term tic douloureux). There may be a background ache between the stabs. The pain may recur many times
a day and tends to be triggered by contact with the skin of
the affected area. Cold wind on the face, washing, shaving,
teeth-cleaning, talking, eating and drinking may all trigger the
pain, and the patient may reduce or stop these activities.
Trigeminal neuralgia tends to occur in patients over the age of
55 years, often with a history of hypertension, and may be due to
irritation of the proximal part of the trigeminal nerve by an adjacent blood vessel. Younger patients and those with abnormal
physical signs should be investigated to exclude other rare
causes such as multiple sclerosis and compression of the
trigeminal nerve by a neuroma or meningioma.
Trigeminal neuralgia tends to wax and wane spontaneously
but is usually controlled by anticonvulsants like carbamazepine, lamotrigine or gabapentin. Failing this, the pain can
be controlled by selectively damaging the nerve with, for example, a radiofrequency lesion (with a risk of troublesome facial
numbness and subsequent recurrence of pain) or by surgical
decompression of the trigeminal nerve (with all the risks of
neurosurgery, including brainstem stroke).
220
CHAPTER 13
Atypical facial pain

Atypical facial pain causes a dull, persistent ache around one
cheek with a great deal of accompanying misery. The pain is
constant, not jabbing. Most patients are women aged between
30 and 50 years. Physical examination and investigations are
normal. Like tension headaches, atypical facial pain is hard to
relieve. Tricyclic antidepressants can, however, be curative.
'I'm beginning to think I'm neurotic.'
Post-herpetic neuralgia
In the face and head, herpes zoster infection most commonly
affects the ophthalmic division of the trigeminal nerve. The
condition is painful during the acute vesicular phase and, in a
small percentage of patients, the pain persists after the rash has
healed. The occurrence of persistent pain, post-herpetic neuralgia, does not relate to the age of the patient or the severity of the
acute episode, nor is it definitely reduced by the early use of
antiviral agents.
Post-herpetic neuralgia can produce a tragic clinical picture.
The patient is often elderly (since shingles is more common in
the elderly) with a constant burning pain in the thinned and depigmented area that was affected by the vesicles. Not infrequently, the patient cannot sleep properly, loses weight and
becomes very depressed.
The pain rarely settles of its own accord but can be ameliorated by tricyclic antidepressants, gabapentin and topical
capsaicin ointment. Capsaicin is a derivative of chilli pepper
that suppresses activity in substance P-containing sensory
neurones; care must be taken to keep it out of the eye.
'It's been there every day since I

had the shingles.'
Post-concussion syndrome
Patients of any age who have an injury to their head resulting in
concussion may suffer a group of symptoms during the months
or years afterwards, even though physical examination and investigation are normal.
The head injury is usually definite but mild, with little or no
post-traumatic amnesia. Subsequently, the patient may complain of headaches (which are frequent or constant and made
worse by exertion), poor concentration and memory, difficulty in taking decisions, irritability, depression, dizziness,
intolerance of alcohol and exercise.
The extent to which these symptoms are physical (and the
mechanism of this) or psychological, and the extent to which
they are maintained by the worry of medico-legal activity in relation to compensation, is not clear and probably varies a good
deal from case to case.
'I've not been myself ever since

that accident.'
221
Other causes of headache and facial pain

It is important to remember that pains in the head and face may
be non-neurological in nature, and more the province of the
general practitioner or other medical or surgical specialists.
Some of these conditions are very common. They are shown
below.
Sinuses
infection
malignancy
Eyes
eyestrain
glaucoma
uveitis
Temporo-mandibular joints
arthritis
Ears
otitis media
Teeth
malocclusion
caries
Neck
trauma
disc degenerative disease
carotid dissection
Heart
angina may be felt in the
neck and jaws
Fig. 13.1 Non-neurological causes of headache and facial pain.
222
CHAPTER 13
CASE HISTORIES
The diagnosis of headache and facial pains
concentrates very much on the history. Abnormal
examination findings are important but rare, except in
the acute and evolving headaches. Patients often use
key phrases which start to point you towards the
correct diagnosis. In reality you would want to hear a
great deal more before committing yourself to a
diagnosis, but see if you can identify the likely causes
in these cases.
a. It was like being hit on the back of the head with a
baseball bat.
b. Its a tight band around my head, holding it in a
vice, like somethings expanding inside.
c. Every night at 2 oclock, like someone is squeezing

my eyeball very hard.
d. Mainly in the mornings and when I bend over.
e. All over my head, it feels so tender, Ive never felt
so bad in all my years.
f. Maybe once or twice a month, at the front on this
side.
g. Cruel, sudden, I cant brush my teeth.
h. Awful when I sit up, fine lying flat.
i. Endless, nagging ache in this cheek: Im desperate.
j. Its very embarrassing, doctor, it always happens
just as Im, you know. . . .
14
CHAPTER 14
Dementia
Introduction
Dementia is a progressive loss of intellectual function. It is common in the developed world and is becoming more common as
the age of the population gradually increases, putting more
people at risk of neurodegenerative disease. In the developing
world there are other serious causes, including the effects of
HIVAIDS and untreated hypertension. Patients with dementia place a major burden on their families and on medical and social services. Reversible causes of dementia are rare. Treatment
is generally supportive or directed at relieving symptoms, and
is usually far from perfect. But dementia is now an area of intense scientific study, bringing the prospect of more effective
therapies in the future.
Recognizing dementia is easy when it is severe. It is much
harder to distinguish early dementia from the forgetfulness
due to anxiety, and from the mild cognitive impairment that
often accompanies ageing (usually affecting memory for names
and recent events), which does not necessarily progress to more
severe disability. It is also important to distinguish dementia
from four related but clinically distinct entities: delirium (or
acute confusion), learning disability, pseudodementia and
dysphasia.
Delirium
Delirium is a state of confusion in which patients are not fully
in touch with their environment. They are drowsy, perplexed
and uncooperative. They often appear to hallucinate, for example misinterpreting the pattern on the curtains as insects. There
are many possible causes, including almost any systemic or
CNS infection, hypoxia, drug toxicity, alcohol withdrawal,
stroke, encephalitis and epilepsy. Patients with a pre-existing
brain disease, including dementia, are particularly susceptible
to delirium.
224
Dementia
Progressive
Involvement of more than
one area of intellectual
function (such as memory,
language, judgement or
visuospatial ability)
Sufficiently severe to
disrupt daily life
DEMENTIA
225
Learning disability
Special
school
Learning disability is the currently accepted term for a condition that has in the past been referred to as mental retardation,
mental handicap or educational subnormality. The difference
between dementia and learning disability is that patients with
dementia have had normal intelligence in their adult life and
then start to lose it, whereas patients with learning disability
have suffered some insult to their brains early in life (Fig. 14.1)
which has prevented the development of normal intelligence.
While dementia is progressive, learning disability is static unless a further insult to the brain occurs. The person with learning
disability learns and develops, slowly and to a limited extent.
Early disruption of brain function results in any of:
1. Impaired thinking, reasoning, memory, language, etc.
2. Behaviour problems because of difficulty in learning social
customs, controlling emotions or appreciating the emotional
needs of others.
3. Abnormal movement of the body, because of damage to the
parts of the brain involved in movement (motor cortex, basal
ganglia, cerebellum, thalamus, sensory cortex) giving rise to:
delayed milestones for sitting, crawling, walking;
spastic forms of cerebral palsy including congenital hemiplegia and spastic diplegia (or tetraplegia);
dystonic (athetoid) form of cerebral palsy (where the
intellect is often normal);
clumsy, poorly coordinated movement;
repetitive or ritualistic stereotyped movements.
4. Epilepsy, which may be severe and resistant to treatment.
It is now clear that in developed countries anoxic birth injury,
once thought to account for most cases of learning disability
and cerebral palsy, is actually an unusual cause; genetic disorders are the major culprit. Parents may appreciate early diagnosis, genetic counselling and in a few cases prenatal diagnosis.
Genetic abnormality
e.g. Down's syndrome
Fragile X syndrome
Low IQ
Young
Movement
disorder
Intra-uterine event
e.g. Infection
Stroke
Drug exposure
Epilepsy
Brain
Birth anoxia

common insults that can occur to
the developing brain, and their
consequences.
Insult early in life

e.g. Meningitis
Head injury
Non-accidental injury
Behaviour
problems
226
CHAPTER 14
Pseudodementia
A few patients may deliberately affect loss of memory and impaired intellectual function, usually in response to a major life
crisis. Anxiety commonly interferes with the ability to take in
new information. In the main, however, pseudodementia refers
to the impaired thinking that occurs in some patients with depression. Severely depressed patients may be mentally and
physically retarded to a major degree. There may be long intervals between question and answer when interviewing such
patients. The patients feelings of unworthiness and lack of confidence may be such that he is quite uncertain whether his
thoughts and answers are accurate or of any value. Patients like
this often state quite categorically that they cannot think or remember properly, and defer to their spouse when asked questions. Their overall functional performance, at work or in the
house, may become grossly impaired because of mental slowness, indecisiveness, lack of enthusiasm and impaired energy.
'. . . I can't remember . . I think

you'd better ask
my wife . .'
Dysphasia
The next clear discrimination is between dementia and dysphasia. It is very likely that talking to the patient, in an attempt to
obtain details of the history, will have defined whether the
problem is one of impaired intellectual function, or a problem
of language comprehension and production, or both. The
discrimination is important, not least because of the difficulty in
assessing intellectual function in a patient with significant dysphasia. Moreover, dysphasia can be mistaken for delirium,
leading to the neglect of a treatable focal brain problem such as
encephalitis.
Patients with dysphasia have a language problem. This is not
dissimilar to being in a foreign country and finding oneself
unable to understand (receptive dysphasia), or make oneself
understood (expressive dysphasia).
Figure 14.2 sets out the two main types of dysphasia (as seen
in the majority of people, in whom speech is represented in the
left cerebral hemisphere). Not infrequently, a patient has a
lesion in the left cerebral hemisphere which is large enough to
produce a global or mixed dysphasia. Brocas and Wernickes
areas are both involved; verbal expression and comprehension
are both impaired.
Involvement of nearby areas of the brain by the lesion causing
the dysphasia may result in other clinical features. These
are shown in Fig. 14.3. Stroke, ischaemic or haemorrhagic, and
cerebral tumour are the common sorts of focal pathology to
behave in this way.
Language problem
DEMENTIA
227
Broca's area
Wernicke's area
Motor dysphasia
Expressive dysphasia
Non-fluent dysphasia
Anterior dysphasia
Sensory dysphasia
Receptive dysphasia
Fluent dysphasia
Posterior dysphasia
The patient can understand spoken words

normally. Other people's language is
making appropriate sensible ideas in his
brain. He is not able to find the words to
express himself. Speech is non-fluent,
hesitant, reduced, with grammatical errors
and omissions. Speech may resemble the
abbreviated language used in text messages.
The difficulty and delay in word-finding
lead to frustration in the patient. Not
infrequently there is an associated
dysarthria and motor disturbance affecting
face and tongue
The patient is not able to understand spoken

words normally. Other people's speech is heard
and transmitted to the brain normally, but
conversion to ideas in the patient's brain is
impaired. His ability to monitor his own speech,
to make sure that the correct words are used
to express his own ideas, is impaired. Speech is
excessive, void of meaning, words are substituted
(paraphasias) and new words used (neologisms).
The patient does not understand what is said to
him, and has difficulty in obeying instructions.
The patient may appear so out of contact to be
regarded as psychotic. Awareness of his speech
problem and frustration are not very evident
Fig. 14.2 The two broad groups of dysphasia.
1 Weakness of the right face,

hand and arm
2 Sensory impairment in the
right face, hand and arm
3 Difficulties with:
written words . . . dyslexia
and dysgraphia
numbers . . . dyscalculia
visual field . . . right
homonymous hemianopia
4 Impairment of memory,
alteration of behaviour
2
3
Fig. 14.3 The common associated neurological abnormalities in dysphasic patients.
228
CHAPTER 14
Features of dementia
The commonly encountered defects in intellectual function that
occur in patients with dementia, together with the effects that
such defects have, are shown below. Because the dementing
process usually develops slowly, the features of dementia
evolve insidiously, and are often absorbed by the patients
family. This is why dementia is often advanced at the time of
presentation.
. . . a forgetful person, in no
real distress, who can no
longer do their job, can no
longer be independent, and
who cannot really sustain any
ordinary sensible
conversation
Defect in:
Effects
Memory
Disorientation, especially in time

Impaired knowledge of recent events
Forgets messages, repeats himself, loses things about the house
Increasing dependence on familiar surroundings and daily routine
Thinking, understanding,
reasoning and initiating
Poor organization
Ordinary jobs muddled and poorly executed
Slow, inaccurate, circumstantial conversation
Poor comprehension of argument, conversation and TV programmes
Difficulty in making decisions and judgements
Fewer new ideas, less initiative
Increasing dependence on relatives
Dominant hemisphere
function
Reduced vocabulary, overuse of simple phrases

Difficulty in naming things and word-finding
Occasional misuse of words
Reading, writing and spelling problems
Difficulties in calculation, inability to handle money
Non-dominant hemisphere
function
Easily lost, wandering and difficulty dressing (spatial disorientation)
Insight and emotion
Usually lacking in insight, facile

Occasionally insight is intact, causing anxiety and depression
Emotional lability may be present
Socially or sexually inappropriate behaviour
DEMENTIA
229
Testing intellectual function (with some reference to

anatomical localization)
Talking to the patient, in an attempt to obtain details of the history, will have indicated the presence of impairment of intellectual function in most instances. The next tasks are to find out
how severely affected intellectual function is, and whether all
aspects of the intellect are involved, i.e. global dementia, or
whether the problem is localized.
Dysphasia
Bilateral frontal and temporal regions
Establish first whether there is significant expressive

or receptive dysphasia, because these make it hard
to use most of the other bedside tests of intellectual
function. The features of dysphasia are described in
Fig. 14.2
Listen for expressive dysphasia: hesitant speech,
struggling to find the correct word and using
laborious ways round missing words (circumlocution)
Test for receptive dysphasia by asking the patient
to follow increasingly complex commands, e.g. 'close
your eyes,' 'put one hand on your chest and the other
on your head,' 'touch your nose, put on your
spectacles, and stand up'
In both situations the patient may use the wrong
words
Look for and ask about behavioural changes of

frontotemporal disorder:
self-neglect, apathy and social withdrawal
socially or sexually inappropriate behaviour
excessive and uncharacteristic consumption
of alcohol or sweet foods
lack of insight into these changes
Test orientation in time and place, and recall of
current affairs (if the patient does not follow the
news, try sport or their favourite soap opera)
Test attention and recall, asking the patient to
repeat a name and address immediately and after
5 minutes
Test frontal lobe function. Ask the patient to give
the meaning of proverbs (looking for literal or
'concrete' interpretations rather than the abstract
meaning), or to generate a list of words beginning
with a particular letter (usually very slow and
repetitive)
C
A
Dominant hemisphere
Non-dominant hemisphere
Spoken language (A)

Test vocabulary, naming of objects, misuse of words
and ability to follow multicomponent commands
Test for contralateral limb sensory inattention or

visual field inattention
Ask about loss of spatial awareness, where the
patient becomes lost in familiar surroundings, or
struggles with the correct arrangement of clothes
on his body (dressing dyspraxia)
Test ability to draw a clock and copy a diagram of
intersecting pentagons
Written language (B)

Test reading, writing and calculation
Parietal dysfunction (C)
Test for contralateral limb sensory inattention or
visual field inattention
230
CHAPTER 14
Causes of dementia
The commoner causes of dementia are listed below, followed by
brief notes about each of the individual causes.
1. Alzheimers disease.
2. Dementia with Lewy bodies.
3. Vascular dementia.
4. Other progressive intracranial pathology:
brain tumour;
chronic subdural haematoma;
chronic hydrocephalus;
multiple sclerosis;
Huntingtons disease;
Picks disease;
motor neurone disease;
CreutzfeldtJakob disease.
5. Alcohol and drugs.
6. Rare infections, deficiencies, etc.:
HIVAIDS;
syphilis;
B vitamin deficiency;
hypothyroidism.
Alzheimers disease
This is very common, especially with increasing age, and accounts for about 65% of dementia in the UK. Onset and progression are insidious. Memory is usually affected first, followed by
language and spatial abilities. Insight and judgement are preserved to begin with. After a few years all aspects of intellectual
function are affected and the patient may become frail and unsteady. Epilepsy is uncommon.
The main pathology is in the cerebral cortex, initially in the
temporal lobe, with loss of synapses and cells, neurofibrillary
tangles and senile plaques. These changes also affect subcortical
nuclei, including the ones that provide acetylcholine to the cerebral cortex. This may contribute to the cognitive decline;
cholinesterase inhibitors such as rivastigmine and donepezil
sometimes provide symptomatic improvement.
People with the apolipoprotein E e4 genotype are at increased
risk of developing Alzheimers disease, and mutations in the
amyloid precursor protein gene and the presenilin genes can
cause familial Alzheimers disease. Environmental factors are
probably also important.
Atrophy of both temporal lobes

due to Alzheimers disease.
DEMENTIA
Key features of Lewy

body dementia
Dementia
Parkinsonism
Visual hallucinations
Flucuating cognitive
impairment
231
Dementia with Lewy bodies

Dementia with Lewy bodies accounts for about 15% of
dementia in the UK. The intellectual symptoms are similar to
those of Alzheimers disease but patients are much more likely
to develop parkinsonism, visual hallucinations and episodes of
confusion.
The distribution of pathology is also similar, but affected neurones form Lewy bodies rather than tangles. The cholinergic
deficit is more profound, and the response to cholinesterase
therapies usually more marked. Neuroleptic drugs can exacerbate the parkinsonism to a severe, or even fatal, degree.
Vascular dementia (see also p. 31)

This accounts for about 10% of UK dementia. Most cases are
caused by widespread small vessel disease within the brain
itself, due to hypertension or diabetes, giving rise to extensive
and diffuse damage to the subcortical white matter. These patients present with failing judgement and reasoning, followed
by impaired memory and language, together with a complex
gait disturbance, consisting of small shuffling steps (marche
petits pas). Emotional lability and pseudobulbar palsy are often
present, with a brisk jaw-jerk and spastic dysarthria.
A few cases are due to carotid atheroma giving rise to multiple discrete cerebral infarcts, i.e. multi-infarct dementia. These
patients have a stepwise evolution with events that can be recognized as distinct strokes and which give rise to focal neurological deficits (such as hemiparesis, dysphasia or visual field
defect). Intellectual impairment accrues as these deficits start to
summate.
Treatment of vascular risk factors, especially hypertension
and hyperlipidaemia, can have a useful preventive effect in
both forms of vascular dementia.
Extensive signal change in the

white matter due to small vessel
disease.
232
CHAPTER 14
CT scan demonstrating a frontal

meningioma with surrounding
cerebral oedema. The tumour
enhances after contrast
administration (right-side image).
Other progressive intracranial pathology

Frontal and temporal tumours occasionally become large
enough to cause significant intellectual impairment before
producing tell-tale features like epilepsy, focal deficit or raised
intracranial pressure.
Patients with chronic subdural haematoma are usually
elderly, alcoholic or anticoagulated. They may not recall the
causative head injury, but become drowsy, unsteady and intellectually impaired over a few weeks. Because the collection of
blood is outside the brain, focal neurological signs appear late.
Any process which causes hydrocephalus slowly may declare itself by failing intellectual ability, slowness, inappropriate behaviour and drowsiness, often with gait disturbance,
incontinence of urine and headache.
Neurosurgery may help all these patients.
Severe multiple sclerosis can cause dementia, often with emotional lability.
Dementia that particularly affects frontal lobe functions, with
disinhibited and illogical behaviour, is characteristic of Huntingtons disease, the dementia that sometimes accompanies
motor neurone disease, and Picks disease. This pattern of dementia, where language and spatial abilities are affected late, if
at all, is particularly challenging to manage. The patient has no
insight into the nature of his problems, often neglects himself
and refuses help.
CreutzfeldtJakob disease (CJD) classically causes a very
rapidly progressive and devastating dementia with ataxia
and myoclonic jerks. The patient gets worse day by day and is
helpless and bed-bound within weeks or months, dying within
a year. It is mercifully very rare.
Severe atrophy of the frontal and

temporal lobes due to Picks
disease.
DEMENTIA
233
Transmission and CJD

There are two aspects to mention:
1. Whereas most cases of classic CJD are caused by
spontaneous deposition of an abnormal form of
prion protein within the patients brain, a few
cases have been caused by iatrogenic, person to
person, transmission of this abnormal protein
through pituitary extracts, corneal grafts and
neurosurgical procedures.
2. The variant form of CJD, which occurs mainly in

the UK, takes a slightly slower course and affects
younger people. It is thought to be due to
transmission of the closely related prion disease
in cattle, bovine spongiform encephalopathy, to
people by human consumption of contaminated
beef.
Alcohol and drugs

In addition to the rather flamboyant syndromes seen in alcoholics who become deficient in vitamin B1, namely Wernickes
encephalopathy and Korsakoffs psychosis, it is being increasingly recognized that chronic alcoholism is associated with
cerebral atrophy and generalized dementia.
Patients, especially those who are elderly, may become
confused and forgetful whilst on medication, especially antidepressants, tranquillizers, hypnotics, analgesics and anticonvulsants.
It is most important to bear in mind alcohol and drugs before
embarking upon a detailed investigation of dementia.
Rare infections, deficiencies and metabolic disorders

HIVAIDS
HIVAIDS can cause dementia, either through a primary HIV
encephalitis or through the CNS complications of immunodeficiency such as opportunistic infection (toxoplasmosis, cryptococcal meningitis) and lymphoma. The risk of all of these is
greatly reduced by current highly active retroviral therapy
regiments, but tragically these are least available in the countries with the greatest prevalence and need.
Frontal toxoplasmosis abscess

due to HIVAIDS.
Tertiary syphilis
Tertiary syphilis was the AIDS of the nineteenth century but is
now rare because of improvements in public health and the
widespread use of penicillin, which is lethal to the spirochaete
Treponema pallidum. It gives rise to general paralysis of the insane (a dementia with prominent frontal lobe features), tabes
dorsalis (with loss of proprioception and reflexes in the lower
limbs), taboparesis (a mixture of the two) and meningovascular
syphilis (with small vessel strokes, especially of the brainstem)
(see p. 248 for further details). Blood tests for syphilis should be
a routine part of the investigation of dementia.
234
B vitamin deficiency
Vitamin B1 deficiency in Western society occurs in alcoholics
whose diet is inadequate, and in people who voluntarily modify their diet to an extreme degree, e.g. patients with anorexia
nervosa or extreme vegetarians.
Impairment of short-term memory, confusion, abnormalities
of eye movement and pupils, together with ataxia, constitute
the features of Wernickes encephalopathy. Though associated
with demonstrable pathology in the midbrain, this syndrome
is often rapidly reversible by urgent intravenous thiamine
replacement.
Less reversible is the chronic state of short-term memory impairment and confabulation which characterize Korsakoffs
psychosis, mainly seen in advanced alcoholism.
Whether vitamin B12 deficiency causes dementia remains
uncertain. Certainly, peripheral neuropathy and subacute
combined degeneration of the spinal cord are more definite
neurological consequences of deficiency in this vitamin.
Hypothyroidism
Hypothyroidism is usually rather evident clinically by the
time significant impairment of intellectual function is present.
It is a cause worth remembering in view of its obvious
reversibility.
Investigation of dementia
Usually an accurate history, with collateral information from
family and friends, together with a full physical examination,
will highlight the most likely cause for an individual patients
dementia.
It is worth thinking whether the problem has begun in the
temporal and parietal lobes (with poor memory, then language
and spatial problems, usually indicating Alzheimers disease or
dementia with Lewy bodies) or in the frontal lobes (with
changes in behaviour, raising the possibility of surgically treatable pathology like tumour or hydrocephalus). It is important to
ask about the patients past medical history (vascular risk factors) and family history (vascular risk factors again, and genetic
dementias such as Huntingtons disease, familial Alzheimers
disease and Picks disease). It is particularly important to ask
about the patients social circumstances, because of the need to
provide support and care during an illness that is usually arduous and prolonged.
The examination must include tests of memory, language,
spatial ability and reasoning as already outlined. It must include a search for neurological clues to the cause of the dementia (see table on opposite page), and a careful examination of the
CHAPTER 14
DEMENTIA
235
Clinical clues to the cause of dementia

Myoclonus
Neurodegenerative disease: usually Alzheimers or dementia with Lewy bodies but

think of CJD if the myoclonus is severe
Parkinsonism
Dementia with Lewy bodies
Focal deficit
Tumour or vascular dementia
Drowsiness
Delirium not dementia; hydrocephalus; subdural haematoma
Chaotic gait
Vascular dementia; hydrocephalus; Huntingtons disease; syphilis; CJD
mental state, looking for symptoms and signs of anxiety and

depression that may be contributing to the clinical problem.
Bedside testing of intellectual function can be usefully supplemented by standardized neuropsychological testing; this is
especially helpful in distinguishing between early dementia
and the worried well.
Blood tests rarely detect treatable pathology, but should
routinely include:
full blood count and ESR;
electrolytes, glucose, calcium and liver function tests;
thyroid function tests;
syphilis serology;
vitamin B12;
antinuclear antibody.
In selected cases these should be supplemented with more
specific tests, after appropriate counselling, such as HIV serology, genetic tests for Huntingtons disease and so on.
Brain imaging, with a CT scan to rule out tumour, hydrocephalus and vascular disease, is now routine in developed
countries. MR brain scanning may be useful in delineating the
rarer focal brain atrophies such as Picks disease and has a research role in monitoring disease progression. Other tests, such
as CSF examination or pressure monitoring, are sometimes
required.
236
Management of dementia
Careful explanation is important. Patients vary greatly in the
amount of information that they want and can absorb, and establishing this in an individual person requires patience and
sensitivity. Family members tend to want more information, especially if there may be genetic implications. The family must be
helped to realize that the demented patient will continue to be
dependent on them, will thrive better on a familiar routine each
day, and may need care to prevent injury from hazards around
the home. Additional help and support may be available from
disease-specific self-help groups like the Alzheimer Society;
voluntary care organizations that provide someone to sit with
the patient while the carer pursues activities out of the house;
social services facilities like day centres; or old age psychiatry
services including community nurses and day hospitals. Ultimately many patients require full-time nursing care.
It may be necessary to encourage the healthy spouse to seek
power of attorney, and to obtain advice regarding pensions, investments and wills from a solicitor.
Clearly any treatable underlying condition will be treated. In
Alzheimers disease, and especially in dementia with Lewy
bodies, a careful trial of a cholinesterase inhibitor (such as rivastigmine, donepezil or galantamine) may produce transient
symptomatic benefit. Unfortunately many patients do not improve or experience adverse effects, and should not continue
the treatment unnecessarily. Coexisting conditions such as anxiety and depression can often be usefully treated.
Most of the drugs that are prescribed for people with dementia are, however, sedatives or neuroleptics directed at modifying undesirable or dangerous behaviours, such as irritability or
wandering. There is surprisingly little evidence that such treatments are effective, and increasing evidence that they may
be hazardous. In an ideal world they would be used very sparingly, with greater emphasis on non-pharmacological ways
of modifying behaviour and supporting carers.
CHAPTER 14
Explanation
Practical support
Careful trials of
symptomatic therapy
Power of attorney
DEMENTIA
237
CASE HISTORIES
Case 1
Case 2
A man of 76, who has suffered from atrial fibrillation

for 15 years and takes regular warfarin therapy, starts
to cause concern in his wife. He is usually very alert for
his age, keeping well abreast of current affairs, and a
source of inspiration to his children and grandchildren.
She is unable to date the precise onset of his
symptoms, but it is all very recent. She has been
surprised by her husband forgetting items when
shopping, his mistakes over the grandchildrens
names, his lack of concern, and his tendency to sleep
more in the daytime.The GP is consulted but cannot
really confirm any definite impairment of neurological
function, and examination is normal except for his
controlled atrial fibrillation.Thyroid function tests are
checked and are normal.
Over the next 3 weeks the situation does not
improve.All the family can see the change in the
patient, who is now mentioning generalized
headaches.
A 69-year-old woman is brought to the emergency

department by her distressed husband. Over the last 2
days she has become increasingly agitated. She has
repeatedly seen people around their home, and thinks
that he is involved in a conspiracy to house illegal
immigrants. She has become aggressive when he tries
to persuade her that there is in fact no-one else in the
house and no conspiracy. He feels that she has been
more forgetful over recent months, and has developed
difficulty in navigating around the familiar streets of
their town centre. He also describes a recent episode
of confusion which settled after 2 days of treatment
for a presumed urinary tract infection.
She is highly aroused, shouting angrily for help. She
cannot cooperate with a detailed neurological
examination but uses all four limbs effectively and has
normal eye and facial movements. Her chest is clear
and she is afebrile.
a. What are your thoughts about diagnosis?

b. And your management?
a. What are the diagnostic possibilities here, and how

would you manage her?
CHAPTER 15
Infections of
the nervous system
Introduction
In this chapter, we will try to simplify our understanding of such
a large area of neurological disease, identifying the features that
many of these infections have in common. We will pay most attention to those that are already common in the UK, or which are
becoming more common in the early twenty-first century.
Having said this, the human nervous system can be infected
by a wide range of organisms:
parasites (hydatid cysts and cysticercosis);
protozoa (Toxoplasma);
Rickettsia (Rocky Mountain spotted fever);
mycoplasma (M. pneumoniae);
spirochaetes (syphilis, leptospirosis, Lyme disease);
fungi (Cryptococcus, Candida);
chronic mycobacteria (tuberculosis and leprosy);
pyogenic bacteria (Haemophilus influenzae, meningococcus);
chronic viruses (AIDS, SSPE (subacute sclerosing panencephalitis), etc.);
acute viruses (herpes simplex and herpes zoster, poliovirus).
238
15
INFECTIONS OF THE NERVOUS SYSTEM
239
Common localized infections

Viral infections
Some viruses have a predilection for particular elements of the
nervous system.
Poliomyelitis
Herpes zoster,
herpes simplex, type 2
Herpes simplex, type 1
Acute poliomyelitis
Acute poliomyelitis is very uncommon in the UK nowadays,
because of the effective immunization programme. Rare cases
are usually the consequence of inadequate primary or booster
immunization. It remains a serious problem in some other parts
of the world.
After a gastro-enterological infection, the virus takes up
residence in the lower motor neurones (in the spinal cord and
brainstem). Paralysis ensues, which is often patchy and asymmetrical in the body. Where the infection has been very severe in
the neuraxis, lower motor neurones do not recover and the
paralysis is permanent. Some of the damage to motor neurones
is less complete, and frequently there is recovery of some muscle function after a few weeks. Permanently affected muscles
remain wasted and areflexic.
Herpes zoster infections
Herpes zoster infections, or shingles, have been described in
Chapters 8 (pp. 123 and 125), 9 (p. 143) and 13 (p. 220). The virus
probably enters the dorsal root ganglion cells in childhood at
the time of chickenpox infection, and remains there in some sort
of dormant condition. Later in life, as a result of some impairment in immunological surveillance (e.g. age, immunosuppressive treatment, lymphoma, leukaemia), the virus becomes
activated and causes shingles.
Herpes simplex infections
Cold sores around the mouth and nose are the commonest clinical expressions of herpes simplex virus type 1 infection. A similar dormant state to that in herpes zoster infections occurs here,
with the herpes simplex virus resident in the trigeminal nerve
ganglion. The altered immunological status that accompanies
another viral infection (usually a cold) allows the herpes simplex virus to become activated in the ganglion, and cause the
skin rash in trigeminal territory.
In the case of herpes simplex virus type 2 infections, the site
of latent infection is the dorsal root ganglion in the sacral
region, and reactivation leads to genital herpes, in which the
vesicular ulcerative lesions occur in the urogenital tract and
perineal region.
240
CHAPTER 15
Pyogenic bacterial infections

Cerebral abscess and spinal extradural abscess are the consequences of localized pyogenic bacterial infection. Cerebral
abscess is the more common of the two. Figure 15.1 shows the
common clinical features of these two conditions.
Cerebral abscess
In the case of cerebral abscess, the continuing mortality from
this condition is due to delay in diagnosis. The presence of a
cerebral abscess must be anticipated whenever some of the features depicted in the left part of Fig. 15.1 occur. In particular, the
presence of one of the local infective conditions which can give
rise to cerebral abscess must put one on guard.
The non-specific features of an infection, i.e. fever, elevated
white cell count and ESR, may not be very marked in patients
with cerebral abscess. Epilepsy, often with focal features, is
common in patients with a brain abscess. The diagnosis should
be established before the focal neurological deficit (the nature of
which will depend upon the site of the abscess) and evidence of
raised intracranial pressure are too severe.
Urgent CT brain scan and referral to the local neurosurgical
centre are the correct lines of management of patients with suspected cerebral abscess. Lumbar puncture is contra-indicated
and potentially dangerous. Neurosurgical drainage, bacteriological diagnosis and intensive antibiotic treatment are required for a successful outcome.
CT brain scan showing a large

cerebral abscess, with
surrounding cerebral oedema and
shift of midline structures to the
opposite side.
Fever
(elevated WCC and ESR)
Cerebral
abscess
Reason for the abscess
1 Local infection
Compound skull fracture
Sinusitis
Orbital cellulitis
Otitis media
Apical tooth infection
Epilepsy
241
Fever
(elevated WCC and ESR)
Focal
neurological
deficit
Raised
intracranial
pressure
Spinal
extradural
abscess
Reason for the abscess
1 Source of infection in the skin,
e.g. boil
Pain in spine
Nerve root
pain and
compression
Spinal cord
compression
2 Impaired immunity/diabetes
2 Blood-borne infections
Bronchiectasis
Lung abscess
Empyema
Cyanotic congenital heart disease
Drug addict
3 Impaired immunity/diabetes
Fig. 15.1 Common localized pyogenic bacterial infections.
Spinal extradural abscess

Patients with a spinal extradural abscess present like any patient with a localized spinal cord lesion, except that pain and
tenderness in the spine are often very conspicuous. The clinical
picture is one that worsens very quickly. There may be clinical
evidence of infection, and possibly some predisposition to
infection.
Urgent MR scanning of the relevant part of the spine leading
to decompressive surgery, organism identification (usually
Staphylococcus aureus), and antibiotic therapy constitute the
correct management.
Other localized infections

Localized tuberculous infection may occur in the brain, known as
a tuberculoma, or in the spine.
Localized Toxoplasma or fungal brain abscesses may occur in
immunodeficient or immunosuppressed patients, especially
those with AIDS (see p. 233).
242
CHAPTER 15
Common acute generalized CNS infections

Acute meningo-encephalitis is probably the best term to describe
acute generalized viral or bacterial infections of the nervous
system. Clinically and pathologically, there is almost always
some degree of encephalitis in acute meningitis, and some degree
of meningitis in acute encephalitis. Frequently, both aspects are
apparent clinically. The close apposition of the meninges to the
highly convoluted surface of the brain makes it very unlikely
that meninges and brain tissue could escape sharing the same
acute inflammatory illness.
Figure 15.2 shows the features of acute meningo-encephalitis.
The emphasis on meningitic and encephalitic features varies
from one patient to another, and according to the particular infecting agent. The drowsiness and coma may be due to raised
intracranial pressure, or to direct involvement of the brainstem
in the encephalitic process. The raised intracranial pressure
may be due to brain swelling (encephalitis), failure of CSF
absorption over the surface of the brain (meningitis), or thrombosis of the sagittal sinus (either encephalitis or meningitis).
Causes
Features
Fever
Rigors
Flushed
Tachycardia
Elevated WCC
and ESR
Meningitis
Headache
Photophobia
Neck stiffness
Encephalitis
Any focal neurological
deficit
Epileptic fits
Confusion
Disorientation
Hallucinations
Drowsiness/coma
Raised intracranial
pressure
Headache
Vomiting
Drowsiness/coma
Papilloedema
Fig. 15.2 Common acute generalized CNS infections.
Viruses
Measles
Mumps
EpsteinBarr
ECHO
Coxsackie
Herpes simplex
Bacteria
E. coli
Group B streptococci
Haemophilus
Meningococcus
Pneumococcus
Listeria monocytogenes
Others (not common)
Weil's disease
Lyme disease
Mycoplasma pneumoniae
243
Viral infections
MR brain scan showing changes

in both temporal lobes due to
oedema and haemorrhage in
herpes simplex encephalitis.
In the case of acute viral infections, the clinical picture may be

of acute meningitis, acute meningo-encephalitis or acute encephalitis. A mild degree of acute meningo-encephalitis probably occurs in many acute viral infections, certainly in the
common exanthematous infections of childhood, especially
mumps. In adults with the clinical picture of acute viral
meningo-encephalitis, the particular virus may not be identified, though ECHO and coxsackie viruses are those most frequently responsible. Meningo-encephalitis occurs at the time of
seroconversion in HIV infection.
Herpes simplex encephalitis, caused by herpesvirus type 1, is
the most potentially lethal acute viral infection of the CNS. It can
occur at any age, and produces a largely encephalitic clinical
picture with or without features of raised intracranial pressure.
Brain swelling, especially in the temporal regions, is common,
and reflects a highly damaging and necrotic process occurring
in the brain tissue. Death, or survival with a significant neurological deficit (intellectual, physical and/or epileptic), are common sequels to this infection. Fortunately, the outcome is
significantly improved if the antiviral agent, aciclovir, is given
early in the course of the disease. Identification of herpes simplex antigen in the cerebrospinal fluid using the polymerase
chain reaction is helpful in early diagnosis, but there is no certain way of knowing early enough in the course of the illness
which cases of acute encephalitis are due to herpes simplex
virus and which are due to other viruses. For this reason, any patient with acute encephalitis should receive aciclovir immediately, especially where the infection is fulminant and producing
brain swelling on CT scan.
Japanese encephalitis is a similar illness caused by a mosquito-borne flavivirus, endemic in India and Asia. There is no
specific treatment but the infection can be prevented by vaccination. A related virus causes West Nile viral encephalitis in
North America.
Bacterial infections
Acute bacterial infections of the CNS generally give rise to the
clinical picture of acute meningitis (also known as bacterial
meningitis, purulent meningitis and septic meningitis). It is
helpful to remember, however, that these infections are
meningo-encephalitic, since confusion or some alteration of the
mental state, epilepsy and drowsiness are common features of
bacterial meningitis. Furthermore, it is the brain involvement
that is so worrying in fulminating infections, e.g. meningococcal meningitis, which may progress to coma within hours. Persistent purulent meningitis is very likely to give rise to CSF
244
absorption problems, so that the clinical features of raised

intracranial pressure appear, along with CT scan confirmation
of hydrocephalus.
Bacterial meningitis is pre-eminently a disease of neonates
and infants. It is a medical emergency. Delay in diagnosis
and treatment increases both morbidity and mortality. In
neonates, the common organisms tend to be Escherichia coli
and Group B streptococci, but thereafter the common bacteria are Haemophilus influenzae, meningococcus and pneumococcus. The speed with which meningococcal meningitis can
evolve requires stressing. Fulminating septicaemia and meningitis, with fever, shock, petechial or purpuric rash, rapid deterioration in conscious level, neck stiffness and a positive Kernigs
sign, may evolve with startling rapidity. Doctors should give
penicillin to patients with suspected bacterial meningitis before
transferring them to hospital. Once in hospital, intravenous
treatment with a broad-spectrum antibiotic such as ceftriaxone
should be started immediately, without being delayed for
tests such as scans or lumbar puncture, and should be continued until there is good evidence that the causative organism
is likely to be sensitive to a more specific antibiotic, or the diagnosis of bacterial meningitis has been refuted or the treatment is
complete.
In all case of bacterial meningitis, perhaps more so in
adults, and especially in any case of recurrent infection, a reason
for the infection must be sought. The predisposition to infection
may be:
1. local, for example:
head trauma involving the floor of the anterior cranial
fossa, possibly with CSF rhinorrhoea,
head trauma involving the temporal bone, with access of
bacteria to the CSF from the ear,
shunt devices in situ for relief of hydrocephalus.
2. general, for example:
diabetes mellitus,
immunodeficiency or immunosuppression.
Other infective agents

Apart from viruses and bacteria, other infective agents causing
acute meningo-encephalitis are not common in the UK. Two
treponemal infections, leptospirosis and Lyme disease, are
occasional causes of meningitis. Meningitis may occur at the
height of Weils disease (infection with Leptospira icterohaemorrhagica), and the meningitis of Lyme disease (infection with Borrelia burgdorferi) is often associated with facial nerve palsy. An
infection with M. pneumoniae in the lungs may be complicated
by a meningo-encephalitis, which is usually not too severe.
CHAPTER 15
245
Subacute and chronic generalized CNS infections

Viruses
AIDS
Rabies
Subacute sclerosing
panencephalitis
Progressive rubella
panencephalitis
Progressive multifocal
leucoencephalopathy
Bacteria
Tuberculous meningitis
Tetanus
Leprosy
Spirochaete Syphilis
NonMalignant meningitis
infective
Fig. 15.3 Subacute and chronic

generalized CNS infections.
None of these infections is common in the UK currently. This is

because of comprehensive immunization of the population
(tetanus, tuberculosis), widespread frequent use of antibiotics
(syphilis), or because the condition, though common elsewhere
in the world, has not yet reached the UK in significant numbers
(rabies). None of the infections will be described in great detail
therefore, though some awareness of each of them is certainly
justified (Fig. 15.3).
AIDS
AIDS patients are predisposed to three groups of problems
from the neurological point of view, as shown in Fig. 15.4. The
direct effects of HIV and the secondary effects of immunosuppression are both considerably reduced by highly active retroviral therapy regimens, where these are available. In the UK
therefore they tend to occur mainly in people who do not realize
that they are infected with HIV. Such patients typically present
with headache, focal deficit and epilepsy, with a low lymphocyte count and muted evidence of an inflammatory response to
infection. Because the immune system is suppressed, microbiological diagnosis relies more on detecting antigens and DNA
from the offending organisms than on identifying antibody responses from the patient. Initially it is often necessary to treat
the infection that is most likely on clinical and radiological
grounds, considering alternative diagnoses (such as lymphoma) if the response to treatment is poor.
Opportunist infection
(see Fig. 15.5)
Opportunist malignancy
Direct effect of HIV
Viruses
Herpes simplex
Herpes zoster
Cytomegalovirus
Papovavirus
Cerebral lymphoma
Early
Bacteria
Not common
Spirochaete
Syphilis
Fungus
Cryptococcus
Protozoan
Toxoplasma
Fig. 15.4 Neurological problems in AIDS patients.
Intermediate
Meningitis
Myelopathy
Radiculopathy
Dementia
Late
Meningitis
Myelopathy
Dementia
246
Progressive multifocal leucoencephalopathy

This is a rare condition, occurring in immunocompromised patients, characterized by the subacute accumulation of neurological deficits due to multiple areas of demyelination in the brain,
mainly in the cerebral hemispheres. It is caused by opportunistic activation of a human papovavirus (the JC virus) infecting
oligodendrocytes.
CHAPTER 15
Subacute accumulation of
neurological deficits
Rabies
This viral illness is usually contracted because the patient is bitten by an infected dog, which has the virus in its saliva. After a
variable incubation period (usually 28 weeks, but sometimes
much longer), a progressive encephalomyelitis occurs (hallucinations, apprehension, hydrophobia, flaccid paralysis with
sensory and sphincter involvement), leading to bulbar and
respiratory paralysis. Treatment is difficult, not very specific,
prolonged and often unsuccessful. Post-exposure prophylaxis
with immunoglobulin may be helpful.
Dog bite abroad

Incubation period
Progressive
encephalomyelitis
Subacute sclerosing panencephalitis and progressive

rubella panencephalitis
After a latency of some years following measles in early
childhood or congenital rubella, a slowly progressive, fatal
syndrome characterized by personality change, dementia,
myoclonic seizures, and ataxia occasionally occurs.
Specific anti-measles or anti-rubella antibodies become
increasingly evident in the CSF during the illness, indicating
the presence of viral antigen within the CNS.
Dementia
Myoclonus
Ataxia
Tuberculous meningitis
Tuberculosis is increasing in incidence and becoming more resistant to treatment. Tuberculous meningitis causes the same
symptoms as other forms of meningo-encephalitis (shown in
Fig. 15.2) but evolves more slowly, over days or weeks. The
meningitis is often concentrated around the base of the brain,
causing cranial nerve palsies, and interfering with the circulation of CSF. Elevated intracranial pressure and hydrocephalus
are common in tuberculous meningitis. It can also cause inflammation of the blood vessels as they leave the meninges to enter
the brain, causing strokes.
Subacute meningoencephalitis
Hydrocephalus
Evidence of infection
Deep dirty wound

Progressively severe muscle
spasms
247
Tetanus
Tetanus occurs worldwide, almost exclusively in people
who have never been immunized or who have not had a
booster immunization in the previous decade. Clostridium tetani
spores enter the body through a dirty wound and replicate
in anaerobic conditions. The bacteria produce a range of toxins
that circulate and bind to targets in the brain, spinal cord,
peripheral motor nerves and sympathetic nerves, interfering
with inhibitory neurotransmission. This results in increased
muscle tone, spasms and seizures. Spontaneous muscle spasms
typically begin in the face, referred to as trismus or lockjaw and
risus sardonicus. They spread to the trunk and limb muscles,
where spasms termed opisthotonus arch the body backwards
and may be strong enough to cause crush fractures of the vertebrae or death from respiratory failure and exhaustion. Involvement of the autonomic nerves may cause hypertension and
tachycardia.
Debridement of the necrotic wound, antibiotics, tetanus antitoxin, sedation, neuromuscular blocking agents and ventilation
may all feature in the management of patients with tetanus,
which still has a mortality of more than 10%. Perhaps surprisingly, survivors still require immunization to prevent
recurrence.
Leprosy
Severe sensory loss
Painless sores
Muscle weakness and
wasting
Leprosy is caused by Mycobacterium leprae infection of skin,

nerves and mucous membranes. It affects about 600 000 new patients each year, mainly in the Indian subcontinent and parts of
Africa and South America. The incidence is falling in response
to an international eradication programme, but there are still
between 1 and 2 million people worldwide who are disabled by
the chronic sequelae of the disease.
The milder, paucibacillary form causes hypopigmented
skin macules. The macules have reduced pain and light touch
sensation. The more severe, multibacillary form causes larger
symmetrical skin lesions, nodules and plaques. Patients
with this form of the disease are more likely to get a slowly
progressive multifocal neuropathy (Chapter 9, see p. 146). Individual peripheral nerves become thickened and there is wasting, weakness and anaesthesia in the distribution of these
nerves.
Treatment requires combination antibacterial therapy, with
rifampicin and dapsone, combined with clofazimine in multibacillary cases.
248
Syphilis
The CNS is involved in the tertiary phase of infection by
the spirochaete, Treponema pallidum. Neurosyphilis is mainly
encountered in patients with AIDS in the UK nowadays. The
manifestations of neurosyphilis are:
optic atrophy;
ArgyllRobertson pupils, which are small, unequal, irregular, react to accommodation but do not react to light;
general paralysis of the insane, in which meningoencephalitic involvement of the cerebral cortex (especially
the frontal lobes, including the motor cortex) occurs. It gives
rise to a combination of dementia and upper motor neurone
paralysis of the limbs;
tabes dorsalis, in which the proximal axons of the dorsal root
ganglion cells, destined to travel in the posterior columns of
the spinal cord, become atrophied. The clinical picture is one
of proprioceptive sensory loss, especially in the legs. An unsteady, wide-based, stamping gait, and a tendency to fall in
the dark, typify patients with tabes dorsalis;
meningovascular syphilis is perhaps the most frequent
clinical expression of neurosyphilis these days. The small
arteries perforating the surface of the brain become inflamed
and obliterated in the subacute syphilitic meningitis.
Acute hemiplegia and sudden individual cranial nerve
palsies are the most common clinical events in this form of
the disease.
CHAPTER 15
Optic disc pallor

Abnormal pupils
Cranial nerve palsy
Dementia
Upper motor neurone signs
Posterior column sensory loss
Abnormal gait
Stroke
Malignant meningitis
This is a relentlessly progressive meningitis, often with cranial
nerve and spinal nerve root lesions, and often associated with
headache, pain in the spine or root pain. It is due to infiltration
of the meninges by neoplastic cells rather than infection. The
neoplastic cells may be leukaemic or lymphomatous, or may
be derived from a solid tumour elsewhere. Such patients are
often immunosuppressed, so the differentiation of malignant
meningitis from an opportunistic infection of the meninges
may be difficult. Cytological examination of the CSF may be
very helpful, the malignant cells showing themselves in centrifuged CSF samples.
Remorseless progression
Painful
Spinal nerve root lesions
249
CNS infections in immunocompromised patients

The prolonged survival of patients with impaired immunity
is becoming more and more commonplace. The number of
patients on cytotoxic drugs and steroids for the treatment of
malignant disease, and to suppress immunity in connective
tissue disorders and after organ transplantation, is increasing.
The incidence and prevalence of AIDS are also increasing.
These immunosuppressed or immunodeficient patients are
susceptible to infections (Fig. 15.5):
by organisms which are capable of causing infection in normal individuals, but which cause abnormally frequent and
severe infections in the immunocompromised;
by organisms which are not pathogenic in normal circumstances, so-called opportunistic infections.
The clinical features of these infections are often ill-defined
and not distinct from the patients underlying disease. The different organisms do not create diagnostic clinical syndromes.
Intensive investigation, in close collaboration with the microbiology laboratory, is usually required to establish the diagnosis
and the correct treatment.
Infections due to normal pathogens, but of increased
incidence and severity
Opportunistic infections
Viruses
Herpes simplex
Encephalitis
Herpes zoster
Shingles
Myelitis
Encephalomyelitis
Viruses
Cytomegalovirus
Encephalitis
Retinitis
Papovavirus
Progressive multifocal
leucoencephalopathy
Bacteria
Common pathogens and less common
ones, e.g. Pseudomonas, tuberculosis
Meningitis
Cerebral abscess
Bacteria
Listeria monocytogenes
Spirochaetes
Treponema pallidum
Neurosyphilis
Fungi
Cryptococcus
Meningitis
Candida
Meningitis
Cerebral abscesses
Aspergillus
Cerebral abscesses
Protozoa
Toxoplasma
Cerebral abscess(es)
Fig. 15.5 CNS infections in immunocompromised patients.
250
Management of infections of the nervous system

Prophylaxis
It is not trite to emphasize the importance of the preventive
measures which are current in the UK to control CNS infections.
We must not become lulled into complacency or lack vigilance
over such matters.
Comprehensive immunization of the population in the case
of polio, tetanus and tuberculosis.
Encouragement of immunization against measles, mumps
and rubella.
Measures to prevent the spread of rabies and AIDS.
Proper care of patients with compound skull fractures, CSF
rhinorrhoea and otorrhoea, otitis media, frontal sinusitis and
orbital cellulitis.
Early active treatment of any infection in diabetic or
immunocompromised patients.
Diagnosis
Some infections will be identified from their clinical features
alone, e.g. herpes zoster.
In the case of acute meningo-encephalitis, the ideal way to establish the diagnosis is urgent CT scan (to exclude a cerebral
abscess mass lesion), followed by immediate lumbar puncture.
Blood culture and other investigations are important, but it is
the CSF which is usually most helpful in diagnosis, as shown in
Fig. 15.6.
In patients with a suspected cerebral abscess, urgent CT brain
scan is the investigation of choice, followed by bacteriological
diagnosis of pus removed at neurosurgery.
Treatment
Appropriate oral and intravenous antibiotic administration,
always in consultation with the microbiology laboratory,
constitutes the main line of treatment for pyogenic bacterial,
tuberculous, fungal and protozoal infections.
Topical and systemic administration of the antiviral agent
aciclovir is used in herpes simplex and zoster infections.
Ganciclovir is active against CMV infections. There is now a
wide range of drugs active against HIV, and it has been shown
that combinations of these drugs are very useful in reducing
viral load, maintaining the effectiveness of the immune system
and preventing the complications of AIDS. This approach is
referred to as HAART (highly active anti-retroviral therapy).
General supportive measures for patients whose conscious
CHAPTER 15
Prevent
Diagnose
Treat
Ask why
Polymorph
count
Lymphocyte
count
251
Protein
conc.
Glucose
conc.
Microscopy
and
culture
Viral
antibodies
in blood and
CSF
Pyogenic
bacterial
meningitis
Viral
meningitis
or
meningoencephalitis
N or
Tuberculous
meningitis
N or
Fungal
meningitis
N or
Cerebral
abscess
Lumbar puncture should not be performed, it is potentially dangerous
N or
Fig. 15.6 CSF abnormalities in various CNS infections.
level is depressed are often required of medical, nursing and

physiotherapy staff (see Fig. 11.7, p. 187).
Reason for the infection

In every patient with a CNS infection, the question must be
asked Why has this infection occurred in this patient?. Such a
question will detect imperfections of immunization, the presence of diabetes, a state of impaired immunity, a previously undetected site of access or source for infection, a personal contact
accounting for the infection, or a visit to a part of the world
where the infection is endemic. Such an enquiry is an essential
part of the patients management.
252
CHAPTER 15
Post-infective neurological syndromes

Figure 15.7 shows this group of rare conditions involving the
central or peripheral nervous system soon after an infection;
GuillainBarr syndrome is probably the most common.
Nature of prior
infection
Target structure in
the nervous system
Syndrome
Many and varied
Myelin around blood

vessels in the central
nervous system
Acute disseminated
encephalomyelitis
Many and varied
Myelin in nerve roots,

cranial nerves and
peripheral nerves
GuillainBarr
syndrome
Influenza, varicella
and other viruses
Mitochondria in
brain and liver
Reye's syndrome
Group A
streptococci
Basal ganglia
Sydenham's chorea
Tourette syndrome
Post-encephalitic
parkinsonism
Acute disseminated encephalomyelitis

Days or weeks after an infection or immunization, a multifocal
perivascular allergic reaction in the CNS, associated with
perivascular demyelination, may occur.
The clinical expression of such an occurrence varies from
mild features of an acute encephalomyelitis, to more major focal
or multifocal neurological deficits, to a life-threatening or fatal
syndrome with epileptic seizures, major bilateral neurological
signs, ataxia, brainstem signs and coma.
In this condition (fully described in Chapter 10, see p. 163), the
post-infectious immunological lesion affects the spinal nerve
roots, and the cranial and peripheral nerves. There is damage to
the myelin. After a phase of damage, which may show itself by
progressive weakness and numbness over 14 weeks, the clinical state and pathological process stabilize, with subsequent
gradual recovery.
Recovery from GuillainBarr syndrome is usually complete,
whereas persistent deficits are not uncommon after severe acute
disseminated encephalomyelitis. Schwann cells can reconstitute peripheral nerve and nerve root myelin with much greater
efficiency than oligodendrocytes can repair myelin within the
CNS.
Fig. 15.7 Post-infective

neurological syndromes.
253
Reyes syndrome
In this condition, which occurs in young children, there is damage to the brain and liver in the wake of a viral infection (especially influenza and varicella). Treatment of the childs infection
with aspirin seems to increase the chances of developing Reyes
syndrome (so avoidance of the use of aspirin in young children
has been recommended).
Vomiting is a common early persistent symptom, rapidly
progressing to coma, seizures, bilateral neurological signs and
evidence of raised intracranial pressure due to cerebral oedema.
The primary insult seems to involve mitochondrial function
in both the brain and liver. Abnormal liver function is evident on
investigation, with hypoglycaemia, which may clearly aggravate the brain lesion.
Post-streptococcal syndromes
The classic neurological sequel of Group A steptococcal sore
throat is subacute chorea, sometimes accompanied by behavioural disturbance, termed Sydenhams chorea or St Vitus
dance. It is usually self-limiting, subsiding after a few weeks. It
is now rare in the UK but remains common in some parts of the
world such as South Africa (see p. 75).
A different post-streptococcal syndrome is now seen more
often in the UK, where the movement disorder is dominated by
tics or sometimes parkinsonism, and does not always remit.
Such cases can be indistinguishable from Gilles de la Tourette
syndrome and post-encephalitic parkinsonism (encephalitis
lethargica) respectively, and it is possible that an autoimmune
response to streptococcus lies behind these conditions (see p.
76).
In all these conditions, positive streptococcal serology (raised
antistreptolysin O or anti-DNAse B titres) points towards the
diagnosis.
254
CHAPTER 15
CASE HISTORIES
Case 1
A 63-year-old warehouse worker with a past history of
bronchiectasis is admitted as an emergency after a
series of epileptic seizures which begin with jerking of
the left leg. He is unrousable but his wife says that over
the last 4 days he has complained of severe headache
and has become increasingly drowsy and apathetic.
On examination he has a temperature of 37.8C
but no neck stiffness. He does not open his eyes to
pain but groans incoherently and attempts to localize
the stimulus with his right hand.The left-sided limbs
remain motionless and are flaccid and areflexic. His
left plantar response is extensor. He does not have
papilloedema. General examination is unremarkable
apart from crackles in his right lower chest.
a. How would you manage his case?
Case 2
A 18-year-old student spends a month in Thailand on
her way back from a gap year in Australia. On
returning to the family home she feels lethargic and

irritable. She sleeps badly, has no appetite and is
losing weight.After 2 weeks she begins to experience
continuous dull headaches; she is referred to hospital
after a further 2 weeks when she begins to vomit.
She is drowsy but orientated with a normal
Glasgow coma score. She looks unwell and has lowgrade fever and neck stiffness with no focal
neurological deficit. She has a mild neurophilia and a
markedly raised CRP. Other routine blood tests, and
films looking for malarial parasites, are normal. Her CT
brain scan shows no definite abnormality, although
there is a suggestion of abnormal enhancement of the
meninges around the base of the brain.At lumbar
puncture her CSF contains 20 polymorphs and 80
lymphoctytes (normally less than 4), a protein of 1.8 g/
dl (normally less than 0.5) and a glucose of 2.2 mmol/l
at a time when her blood glucose is 6.6 mmol/l (when
normal CSF glucose is at least 50% of the blood level).
a. What is the diagnosis?
Answers to
case histories
Chapter 2
Case 1
a. CT brain scan, which in this instance showed no
definite abnormality, just a suggestion of subarachnoid
blood.
Lumbar puncture produced unequivocal, uniformly
bloodstained CSF.
b. Neurosurgical referral.
Cerebral angiography, which demonstrated a single posterior communicating artery aneurysm on the left.
The patient was started on nimodipine and proceeded to surgery without delay. A clip was placed around the neck of the
aneurysm. The post-operative period was complicated by a
transient right hemiparesis, which recovered completely after
10 days. The patients BP settled to 120/80. She has had no further problems.
Case 2
a. Right-sided intracerebral haemorrhage.
b. Right-sided intracerebral haemorrhage.
c. CT brain scan, which shows a large clot of blood centred on
the right internal capsule. ECG and chest X-ray both confirm
left ventricular hypertrophy.
d. He needs to know that his wife is in a grave situation, with
regard to both survival (comatose, obese lady in bed) and
useful neurological recovery.
All appropriate care was given to this woman, but she developed a deep vein thrombosis in her left calf on day 2, and died
suddenly from a pulmonary embolus on day 4.
255
256
ANSWERS TO CASE HISTORIES
Chapter 3
Case 1
a. She has signs of a lesion in the left cerebellopontine angle,
with ataxia, loss of the corneal reflex (cranial nerve 5) and
deafness (cranial nerve 8). It has come on slowly, starting
with deafness, so it is more likely to be an acoustic neuroma
than a metastasis from her previous cancer.
An MR scan confirmed this. The neuroma was too large
to treat with radiotherapy but was successfully removed,
giving her a transient left facial weakness (cranial nerve 7)
and permanent complete left deafness.
Case 2
a. It is hard to localize his symptoms to a single part of the
brain. There are problems with memory (temporal lobes),
behaviour and expressive language (frontal lobes) and
spatial ability (parietal lobes). This could indicate a multifocal process such as metastases, a widespread diffuse
process such as a glioma, or just possibly a focal tumour
with hydrocephalus.
The drowsiness and papilloedema indicate raised intracranial pressure, making a degenerative disease or metabolic encephalopathy unlikely. There are pointers towards
an underlying systemic disease in the weight loss and lymphadenopathy.
His CT brain scan showed widespread multifocal areas of
high-density tissue in the white matter around the lateral
ventricles. The appearances were typical of a primary CNS
lymphoma. His HIV test was positive and his CD4 lymphocyte count was very low, indicating that the underlying
disorder was AIDS. He continued to deteriorate despite
steroids and highly active anti-retroviral therapy, and died 4
weeks later. The diagnosis of lymphoma was confirmed by
autopsy.
Areas with a very high HIV prevalence, such as subSaharan Africa, are seeing a huge and increasing burden of
neurological disease due to AIDS.
Chapter 4
Case 1
a. Your initial management plan would comprise:
admission to hospital;
half-hourly neurological observations recorded on a
257
Glasgow Coma Scale chart by competent staff, regardless

of the boys location, e.g. A & E, ward, CT scanner etc.;
urgent CT brain scan.
b. When he starts to deteriorate, you should obtain immediate
neurosurgical advice and follow it.
The clinical situation strongly suggested a left-sided extradural
haematoma which was confirmed by the CT scan and immediately drained in an emergency operation. Thankfully the
boy made a full recovery from a potentially life-threatening
situation.
Case 2
a. The point here is that you cannot simply assume that his
coma is due to alcohol intoxication. People who abuse
alcohol are very prone to head injury either through accidents
or assaults. If they have a long-standing problem their clotting may be defective because of liver disease, increasing the
risk of subdural, extradural and intracranial haemorrhage.
They have an increased risk of epileptic seizures when intoxicated, when withdrawing from alcohol and as a consequence of previous head injuries, and he could be in a
post-epileptic coma. A seizure can cause a head injury. Alcohol
abuse increases blood pressure and the risk of stroke. People
with alcohol problems often neglect their health: he could be
in a diabetic coma. He may be depressed and have taken an
overdose. He might even have contracted meningitis in the
back bar. Do not jump to conclusions when assessing an
intoxicated patient.
Chapter 5
Case 1
a. She has clearly got parkinsonism, with gait disturbance,
bradykinesia and rigidity. Parkinsons disease is quite common at this age, but is not usually symmetrical like this.
There are no additional neurological features to suggest a
more complex neurodegenerative disease.
The vital part of the history is to establish what pills she is
taking. The treatment for her gastro-oesophageal reflux
turned out to be the dopamine antagonist metoclopramide.
This was stopped and her drug-induced parkinsonism
slowly resolved, although it was a year before she felt back to
normal.
258
Case 2
a. This is a very difficult case. The main problem is cerebellar
ataxia, with ataxia of gait accompanied by milder limb
ataxia and cerebellar dysarthria. But the disease process is
affecting several other systems: his optic nerves, causing
optic atrophy; his spinal cord, giving extensor plantars; and
his peripheral nerves, causing areflexia and impaired distal
vibration sense.
b. This is a condition called Friedreichs ataxia. It is a recessively inherited, early-onset form of multisystem degeneration that is classified under the term spinocerebellar ataxia.
It is due to a trinucleotide expansion in the frataxin gene,
which is believed to have a role in the function of mitochondria. It goes on to affect other systems of the body, causing
cardiomyopathy or diabetes mellitus. It is very rare, except
in clinical exams.
You may have considered the possibility of multiple sclerosis, which is a much commoner cause of cerebellar ataxia,
optic atrophy and extensor plantars, but rare at this age, not
usually gradually progressive and not affecting the peripheral nerves like this. If he was much older you would consider alcohol toxicity. This is a common cause of ataxia
and peripheral neuropathy, but would not readily explain
the optic atrophy or extensor plantars.
c. Clearly there are going to be a great many difficult matters to
discuss with the patient and his family as they face the
prospect of a progressive, disabling degenerative disease in
early adult life. But there may be particular issues for the parents in relation to the genetics. Firstly, they may feel irrationally guilty that they have unknowingly each carried a
genetic mutation that has contributed to their sons illness.
Secondly, they will have worries about his younger siblings,
who each have a 25% risk of inheriting the illness too. The
help of specialists in medical genetics is likely to be invaluable in helping them to approach these issues.
Chapter 6
Case 1
a. You should probably start by examining the patient yourself, but it is perfectly legitimate to say that if you are in doubt
you should get advice from a more experienced colleague
too.
The patient has a pattern of weakness that is typical of
upper motor neurone weakness in the lower limbs, where
the extensor muscles remain relatively strong and the hip
259
flexors, knee flexors and ankle dorsiflexors become weak.

The extensor plantar responses also indicate an upper motor
neurone problem. It often takes a few days before the reflexes become brisk when upper motor neurone weakness
comes on acutely, so you should not worry that the reflexes
are still normal here.
In other words, the problem is somewhere in the spinal
cord. It is unlikely to be in the neck because the arms are
not affected. You need an MR of the thoracic region, not the
lumbosacral region, because the cord ends adjacent to the L1
vertebral body (Fig. 6.1).
The sensory signs may be very helpful in this situation.
The patient felt pin-prick as blunt in his lower abdomen
below his umbilicus and throughout both lower limbs. This
is a T9 sensory level, indicating that the cord problem is at or
above this dermatome. You call the radiologist back and
ask for the appropriate scan. This shows a metastasis compressing the cord in the mid-thoracic region. His pain and
neurological deficit improve with high-dose steroids and
radiotherapy. Further investigation reveals several other
bone metastases from prostate cancer. This is treated medically, with a useful period of palliation.
Case 2
a. The sensory symptoms on neck flexion (so-called LHermittes symptom) help to localize the problem to the neck.
Urgency of micturition is also a helpful indicator of a problem in the spinal cord.
The signs are more specific, indicating pathology at the
level of the 5th and 6th cervical vertebrae. He has the segmental sign of absent C56 biceps and supinator jerks, and
tract signs below this level: brisk C78 triceps jerks and
upper motor neurone signs in the lower limbs.
b. The most common pathology at this location in someone of
this age is cervical spondylosis, with a C56 disc bulge and
osteophyte formation compressing the cord. This turned
out to be the cause here. There are several other rarer possibilities, including a neurofibroma. Because of his delicate
manual occupation, he was keen to have decompressive
surgery. This stopped his electric shocks and improved the
sensation in his hands. The signs in his legs remained
unchanged. His bladder symptoms persisted but responded
to anticholinergic drugs.
260
Chapter 7
Case 1
a. This is not typical of multiple sclerosis. There is dissemination in time (two separate episodes 4 years apart) but not in
place (both episodes seem to arise from the right pons). You
need to exclude a structural cause for this.
His MR brain scan showed a vascular anomaly called a
cavernoma centred on the right side of his pons, surrounded by concentric rings of altered blood products, suggesting that it had bled repeatedly over the years. There
were several other cavernomas elsewhere in the brain. His
sisters doctors were, with his permission, told of this unusual, dominantly inherited diagnosis and, after further imaging, her diagnosis was revised to multiple cavernomas too.
b. Both patients were managed conservatively.
MS is common, but you should always consider the possibility
of an alternative diagnosis if all the symptoms can be attributed
to a lesion in a single place.
Case 2
a. The weakness selectively affects the intrinsic hand muscles
supplied by the ulnar nerves. Wasting is an LMN sign not
typical of MS, which is a disease of the central nervous
system. The likely cause is compression of the ulnar nerves at
the elbow by the arm-rests of her wheelchair.
b. Her wheelchair was modified and she was advised to try to
avoid resting her elbows on firm surfaces. She declined ulnar
nerve transposition.
Chapter 8
a. The problem is in the left optic nerve. The most likely cause is
optic neuritis, which comes on rapidly and often causes pain
on eye movement. It usually resolves spontaneously but
improves rapidly with high-dose steroids.
b. This is most likely to be a right 6th nerve palsy, although a left
3rd nerve palsy or left internuclear opthalmoplegia can all
cause the same symptom (see pp. 11719). Less commonly
this symptom reflects weakness of the right lateral rectus or
left medial rectus muscles (for example due to myasthenia or
thyroid eye disease), and very rarely it is due to a mass in one
orbit, displacing the eye. Examination will clarify matters.
You can suppress the diplopia with an eye patch and then
need to establish the underlying cause.
261
c. This history of sudden jolts of pain, triggered by touch, in an

area supplied by one of the branches of the trigeminal nerve,
is typical of trigeminal neuralgia (see pp. 122 and 219). The
patient is often unable to wash his face or brush his teeth
for fear of triggering the pain. Carbamazepine is usually a
very effective treatment.
d. If you were taking this history face-to-face then the left facial
weakness would be obvious. The likely cause is a Bells palsy
(see p. 124). This affects the lower motor neurones of the
facial nerve, so the whole of the face is affected, including the
forehead (unlike upper motor neurone facial weakness, say
from a stroke, where the forehead is spared). A short course
of oral steroids will improve the chances of a good recovery.
e. This is likely to be a left homonymous hemianopia (see p.
114). Patients are often unaware of the visual field loss so
long as the central field is intact. The most likely cause would
be a stroke or tumour in the right occipital lobe. He needs to
stop driving while you organize a brain scan.
f. The patient is describing positional vertigo and the loss of
confidence that accompanies all vestibular disorders. The
most likely cause of recurrent brief bouts of positional vertigo is benign paroxysmal positional vertigo (BPPV) (see
p. 128) due to particles of debris in one of the semicircular
canals of one ear. Mnires disease causes more prolonged
periods of vertigo which are not necessarily provoked by
changes in the position of the head and which are accompanied by muffled hearing and tinnitus. Acute vestibular
failure or labyrinthitis again causes persistent vertigo,
usually with ataxia and vomiting. BPPV can sometimes be
cured with a manoeuvre to clear the particles, or the symptoms can be suppressed with vestibular sedative drugs.
Chapter 9
a.
b.
c.
d.
Common peroneal nerve palsy or an L5 nerve root lesion.

Radial nerve palsy.
Carpal tunnel syndrome, i.e. bilateral median nerves.
An ulnar nerve lesion at the elbow.
Chapter 10
Case 1
a. He has symptoms and signs of a peripheral neuropathy
which is selectively affecting the sensory nerves. The two
common causes are excessive alcohol and diabetes mellitus.
He is not taking neurotoxic drugs, the length of the history
would be against an underlying malignancy, and the lack of
262
motor involvement or family history is against a hereditary

neuropathy.
b. The macrocytosis may have made you think of vitamin B12
deficiency. This can certainly give rise to neurological problems without causing anaemia, but it typically causes spinal
cord pathology in addition to peripheral nerve damage (subacute combined degeneration of the spinal cord, Chapter 6),
with extensor plantar responses and a positive Romberg
test.
Diabetes mellitus is an important possibility. Even mild
diabetes can cause a sensory neuropathy, without the classical symptoms of weight change, polyuria and polydipsia. To
exclude diabetes you need a fasting sample, or better still a
glucose tolerance test.
But the diagnostic test here was a polite but persistent
enquiry into the patients alcohol history. He had been drinking at least a bottle of wine each day for many years. Alcohol
is a common cause of macrocytosis. His liver function tests
were also abnormal. Nerve conduction studies were not performed (but might have shown damage to the axons in the
form of reduced sensory action potentials).
He was advised to stop drinking, to take vitamin B1 supplements, and to take care of his numb feet. He eventually managed to do this with considerable psychological help and after 6
months his symptoms of neuropathy started to subside.
Case 2
a. She has the symptoms and signs of a bulbar palsy (see pp.
131 and 156). Her breathlessness could be due to anxiety but
could indicate worrying respiratory muscle weakness. You
need to clarify this by checking her vital capacity (not her
peak expiratory flow rate, which is a test for asthma) as part
of your examination.
The most likely cause of this clinical picture in a young
woman would be myasthenia gravis. The observation that
her speech worsens (fatigues) as she talks is characteristic
of myasthenia. You should not be put off by the absence of
myasthenia in her eyes or limbs.
Motor neurone disease is only rarely familial, and in her
case the lack of tongue wasting and fasciculation is very
reassuring.
b. You should arrange urgent hospital admission to make sure
that she does not develop life-threatening bulbar or respiratory weakness. If you are not a neurologist, you should telephone one for advice. If her vital capacity is reduced you
should also consider talking to your friendly ITU consultant.
263
Intravenous edrophonium (Tensilon) gave her a normal

voice for a few minutes. She was started on prednisolone and
pyridostigmine but continued to deteriorate until she underwent plasma exchange, which helped a great deal. Her
acetylcholine receptor antibodies came back 2 weeks later
and were strongly positive. CT scans of her thorax revealed a
thymus mass which, when resected, turned out to be due to
benign hyperplasia. Twelve months later her myasthenia is
in remission and she is off all medication.
Chapter 11
Case 1
a. It is always much harder to diagnose blackouts without a
witness account, and you would want to make sure that, for
example, no fellow dog walker saw the blackout in the park.
A description of a minute or two of random limb jerks,
cyanosis and noisy breathing would point you towards
epilepsy; slumping pale and motionless for several seconds
would steer you towards cardiovascular syncope.
b. Without this vital information, we need more information
about the parts of the blackouts he can recall. What does he
mean by dizziness? Is he describing the lightheadedness of
an imminent faint, the dj vu of a temporal lobe aura, or the
ataxia of a vertebro-basilar TIA? We also need to clarify his
state after the attacks: did he have any confusion, headache
or limb pains to suggest a tonicclonic epileptic seizure?
c. In this case, and in the absence of any other clues, his rapid
recovery from the attacks was suggestive of a cardiovascular
cause. His vascular risk factors (smoking and hypertension)
provide weak support for this. His ECG showed first-degree
heart block (not present in the emergency tracing) and a 24hour ECG showed evidence of sick sinus syndrome with
several prolonged ventricular pauses. A cardiologist confirmed the diagnosis of StokesAdams attacks, which she
cured with a permanent pacemaker.
Case 2
a. This man is unconscious with a moderately reduced
Glasgow coma score of 9 (E2 V2 M5). There no signs of
meningitis (fever, neck stiffness) and no focal signs to suggest a localized problem within the brain (encephalitis,
abscess, tumour, haemorrhage). The roving horizontal eye
movements are helpful, demonstrating that the parts of
his brainstem responsible for generating such movements
(Fig 8.6, p. 117) are intact. Taken together, these are hopeful
264
signs, suggesting that he has a potentially reversible diffuse

problem with his cerebrum, rather than focal disease in his
cortex or brainstem.
The only clue to the cause of his coma is the prescription
for valproate. He could be in a post-ictal coma after a
tonicclonic seizure or he could have taken an overdose of
valproate. Ongoing epileptic states without visible convulsions are very rare. Valproate can cause liver dysfunction but
this is accompanied by jaundice.
b. In terms of management, you have already made sure that
his airway, breathing and circulation are satisfactory. You
check his full blood count, electrolytes, liver function tests
and glucose (normal), and start regular observations, monitoring his Glasgow Coma Scale. You are looking for a gradual improvement to confirm your suspicion of a transient
post-ictal state but his score actually drops to 7 over the next
hour. You are in the process of arranging an EEG, CT brain
scan and possible lumbar puncture when his parents arrive
and tell you that he has become increasingly depressed since
his recent diagnosis of epilepsy, with the loss of his driving licence and his lucrative job as a scaffolder. The diagnosis is a
valproate overdose. He recovers with supportive management but needs continuing psychiatric help.
Chapter 12
Case 1
a. She is having brief, stereotyped, repetitive and unprovoked
episodes, which should make you think of epilepsy even
when the content of the episodes is bizarre. The common
manifestations of temporal lobe seizures are a warm, queasy
feeling rising from the stomach to the head and disturbances
of memory, like dj vu. Less commonly patients experience
olfactory hallucinations, with a brief experience of a strong,
unpleasant but indefinable smell. Emotional changes are
also less common, and typically take the form of a feeling of
impending doom rather than the pleasant calm and omniscience of this case.
b. Her MR brain scan showed a small vascular anomaly called
a cavernoma in the right temporal lobe. Neurosurgical advice was that the risk of harming her by removing the
cavernoma would be greater than the risk of stroke due to
bleeding from it. After learning about the driving regulations, she opted for anticonvulsant therapy. She had completed her family and was not concerned about teratogenic
issues. She started treatment with carbamazepine which
abolished the attacks.
265
Case 2
a. The emergency management of status epilepticus starts
with the establishment of an airway but it is generally impossible to secure this until you have controlled the seizures.
You need to obtain intravenous access and administer intravenous lorazepam (or diazepam) followed by intravenous
phenytoin. As the seizures subside you can insert an oral
airway.
b. The second phase in the management of status epilepticus
is to find out the cause. The commonest is lack of concordance with medication in patients who are known to have
epilepsy. When the first manifestation of epilepsy is status
epilepticus, the underlying cause is often serious (tumour,
stroke, etc.).
The worry here is the persistent fever. It is common to have
a fever during status epilepticus because of the heat generated by muscle activity. The white cell count also usually rises.
But you would expect the temperature to fall subsequently,
rather than to rise. He could have aspirated while fitting and
have pneumonia. Ecstasy toxicity can also present in this
way. But the priority is to exclude intracranial infection.
His CT brain scan showed no abnormality. At lumbar
puncture his CSF pressure was mildly elevated at 26 cm. The
CSF contained 110 lymphocytes (normal less than 4), no
polymorphs and no organisms on Gram staining. The CSF
protein was mildly elevated at 0.6 g/dl and the CSF glucose
level was a normal proportion of his blood glucose.
As you will discover in Chapter 15, this CSF picture suggests intracranial infection, most likely viral encephalitis. He
was treated with intravenous aciclovir. He subsequently had
an MR brain scan which showed signal changes consistent
with swelling and haemorrhage in both temporal and
frontal lobes, typical of herpes simplex encephalitis. HSV
was detected in his CSF using PCR. He rapidly improved but
was left with persistent forgetfulness and occasional focal
seizures.
Chapter 13
a. This is the typical sudden, severe history that should make
you think of subarachnoid haemorrhage.
b. Tension headache gives a constant feeling of pressure in the
head, often described in powerful language and accompanied by an overt or unspoken fear of an underlying brain
tumour.
c. The predictable diurnal timing and intense orbital pain are
typical of cluster headache.
266
d. This is a textbook, so here is the textbook description of the

headache of raised intracranial pressure. Bear in mind that
morning headaches are more often due to migraine, hangovers, anxiety about the day ahead or obstructive sleep apnoea, while headaches on stooping are very common in
sinusitis, but look hard for signs of raised intracranial pressure when you hear this story.
e. In an elderly patient, a new headache with scalp tenderness
and malaise means giant cell arteritis until proved otherwise: ESR and steroids in the meantime.
f. Occasional, intermittent headaches are usually due to
migraine.
g. Brief, lancinating pains triggered by stimulation of the face
or mouth suggest trigeminal neuralgia.
h. Low-pressure headache is postural and relieved by lying
flat.
i. This chronic, unremitting maxillary ache and the accompanying misery in a young or middle-aged woman are typical
of the paradoxically named atypical facial pain.
j. Repeated sudden headaches at the moment of orgasms are
due to benign sex headache.
Chapter 14
Case 1
a. Possible raised intracranial pressure, due to a mass lesion
affecting intellectual function, but not producing any other
focal neurological deficit.
b. Urgent neurological assessment and brain scan.
The patient was seen 2 days later by the local urgent neurological service. No new signs had appeared. The urgent CT brain
scan revealed a large subdural haematoma on the left. This
was evacuated through burrholes by the neurosurgeons
(after correcting the prolonged prothrombin time induced by
warfarin), and the patient underwent a slow, but entirely satisfactory, recovery.
Case 2
a. The key question is whether this is an acute confusional state
or a dementia. In acute confusional states (for example due
to infection) the patient is usually drowsy and not fully in
touch with their surroundings. In this case the patient is
alert; moreover there is a history suggestive of a more gradual dementing process, with poor memory and some
visuospatial impairment. This, together with the previous
267
episode of confusion, raises the possibility of dementia

with Lewy bodies.
You should do your best to settle her by talking to her
calmly, reminding her repeatedly that she is in safe hands,
and providing a quiet, well-lit environment. If she remains
dangerously agitated then you may have to sedate her carefully, for example with a small amount of intravenous lorazepam. You should avoid using conventional neuroleptics
because they can cause catastrophic parkinsonism in dementia with Lewy bodies.
Once she is settled, you can rule out causes of acute confusion (full blood count, ESR, electrolytes, urine dipstick, chest
X-ray) and can perform a more detailed neurological examination, firstly to make sure that she does not have a focal
deficit that would require further investigation and secondly to establish if there is evidence of a dementing process.
In this case the routine tests were all normal. The following
day she was found to have problems with memory, language
and especially visuospatial function (she could not draw a
clock face). She was found to have a stooped, shuffling gait
and mild rigidity in the upper limbs. The diagnosis of dementia with Lewy bodies was confirmed and both her hallucinations and her memory problems improved markedly on
treatment with rivastigmine, a cholinesterase inhibitor. She
went home with support from the community old age psychiatry nurse.
Chapter 15
Case 1
a. The focal onset to the seizures suggests a problem in the right
frontal lobe. This is confirmed by finding a left hemiparesis.
Remember that it takes several days for the classic upper
motor neurone signs of spasticity and hyper-reflexia to
develop.
The crucial investigation is therefore an urgent CT brain
scan. This showed a ragged ring of enhancing tissue with a
low-density centre, surrounded by considerable oedema: a
cerebral abscess. There was no evidence of a source of infection in the paranasal sinuses.
Fever is not always a feature of cerebral abscess, especially
if there is no active infection elsewhere in the body. Many
patients have a low-grade fever after prolonged seizures,
and in this case you might also have wondered if the fever
was arising from a chest infection. But in a patient with neurological symptoms or signs, a fever should always make
you think about infection within the nervous system, and in
268
the presence of focal deficit you should think specifically

about cerebral abscess and encephalitis.
He was treated with broad-spectrum intravenous antibiotics, intravenous phenytoin and neurosurgical drainage
of the abscess. The organism was not cultured from his blood
or the abscess itself, but was assumed to have originated in
his chest; chronic suppurative lung disease and congenital
heart disease both predispose to cerebral abscess although
direct spread from infections in the mastoid or paranasal
sinuses is more common.
After a stormy time in the intensive care unit, the patient
made a gradual recovery but required long-term carbamazepine therapy for continuing epileptic seizures.
Case 2
a. She has a subacute meningitis, with a predominantly lymphocytic CSF pleocytosis and a low CSF glucose. The most
likely cause is tuberculosis, acquired on her travels. Fungal
meningitis can cause a similar illness, but is very rare in
immunocompetent young people. Malignant meningitis is
also rare in young people. The low CSF glucose excludes
viral meningitis.
She had not been immunized with BCG. Her Mantoux test
was strongly positive and DNAfrom Mycobacterium tuberculosis was detected in her CSF by a PCR technique. She was
started on three anti-TB drugs and slowly recovered. CSF
culture eventually confirmed the diagnosis 6 weeks later.
Index
abscess
cerebellum 79
cerebral 240, 254, 2678
fungal 241
spinal extradural 93, 241
ACE inhibitors 31
aciclovir
in herpes simplex infection
250
in herpes zoster 143, 243, 250
acoustic neuroma
case history 54, 256
and deafness 1278
and facial palsy 125
management 51
prognosis 53
symptoms 49
acquired non-inflammatory
myopathy 171
acromegaly 151
action myoclonus 77
acute confusion 224
acute disseminated
encephalomyelitis 252
afferent pupillary defect 103
AIDS see HIVAIDS
air embolism 28
alcohol
and dementia 233, 234
effects on balance 128
intoxication 16, 66, 79, 257
peripheral neuropathy 161,
174, 2612
and tremor 69
Alzheimers disease 77, 230
amantadine 71, 72
amblyopia, tobaccoalcohol
113
amitriptyline 214, 215, 216
amnesia
post-traumatic 63
transient global 182, 183
amyloid precursor protein 230
amyotrophic lateral sclerosis
157
amyotrophy, diabetic 162
analgesics, headache dependent
on 216
anarthria 132
aneurysm
berry 32
in subarachnoid haemorrhage
34, 36
angina 28, 221
angiotensin-converting enzyme
inhibitors 31
anosmia, idiopathic 112
anterior cerebral artery 26
ischaemia 29
anterior dysphasia 227
antibiotics
in bacterial meningitis 244
in infection 250
anticholinesterase, response to
13
anticipation 75
anticoagulant therapy 31, 206
anticonvulsant therapy
brain tumour 52
and cerebellar malfunction 79
cluster headache 216
drug interactions 206, 209
epilepsy 2068
migraine 215
side-effects 207, 209
trigeminal neuralgia 219
antiemetics, parkinsonism
induced by 73
antiphospholipid antibodies 28
antipsychotics
movement disorder induced by
75
parkinsonism induced by 73
anxiety 203, 214
aortic valve disease 28
apathy 229
apolipoprotein E e4 genotype 230
apomorphine 72
areflexia 96
ArgyllRobertson pupils 121,
248
ArnoldChiari malformation 79,
96, 97
arteriovenous malformations 32,
93
arteritis 28
aspiration pneumonia 131, 132,
134
aspirin
in cerebral ischaemia/
infarction 30, 31
and Reyes syndrome 253
astrocytoma 48, 49
ataxia 17, 29, 79
cerebellar 75, 789
fingernose 16
Friedreichs 75, 79, 82, 258
gait 16
heelkneeshin 16
in multiple sclerosis 104
sensory 801
in vestibular disease 81
atenolol 215
atheroma 28
athetosis 74
269
270
atlanto-axial subluxation 93
atrial fibrillation 28
atrial myxoma 28
atypical facial pain 220, 222, 266
aura
in epilepsy 197, 198
in migraine 214
auto-antibodies 164
axillary nerve palsy 147
azathioprine
in myasthenia gravis 166
baclofen 94
bacterial endocarditis 28
bacterial infections 2401, 2434,
2467, 249, 254, 2678
balance disorders 126, 1278
barbiturates, effects on balance
128
basal ganglia 3
anatomy and function 5
infarcts 73
lesions 15, 135
and speech 135
basilar artery 26
ischaemia 29
Beckers muscular dystrophy 168
behaviour, inappropriate 229
Bells palsy 1245, 136, 261
bends 93
benign intracranial hypertension
49
benign paroxysmal positional
vertigo 127, 128, 136, 261
berry aneurysm 32
beta-blockers
in benign sex headache 217
in migraine 215
beta-interferon in multiple
sclerosis 108
BIH 49
biopsy, brain tumour 51
bitemporal hemianopia 48, 112,
113
blackouts 17683
bladder function
abnormalities 10
in paraplegia 92, 94
blepharospasm 75
body weight in paraplegia 945
botulinum toxin therapy 75
INDEX
bovine spongiform
encephalopathy 233
bowel function in paraplegia 92,
94
brachial neuritis 145
brachial plexus lesions 1445
bradykinesia in Parkinsons
disease 15, 70, 135
brain
anatomy 41
arteries 26, 27
arteriovenous malformations
32
intracranial compartments 40,
41
localization of function within
28
lymphoma 53
metastases to 49, 51, 53
primary injury 56, 57, 59
secondary injury 56, 57, 58, 59
tumour 4054, 203, 213, 232
brainstem 40
ischaemia/infarction 127, 128
lesions 10
primary injury 56
tumour 47
brainstem death 1889
breast feeding and anticonvulsant
therapy 209
breathing problems 13
Brocas area 226, 227
bromocriptine 72
bronchial carcinoma 21
BrownSquard syndrome 9, 86,
87
BSE 233
bulbar palsy 130, 131, 1345
in motor neurone disease 132,
156, 157
bulbar weakness 156
cabergoline 72
caeruloplasmin 76
Campylobacter enteritis 163
capsaicin in post-herpetic
neuralgia 220
carbamazepine 206, 207, 219
cardiac arrhythmia 176, 178, 182,
202
carotid arteries 26
dissection 267, 28, 221
carotid endarterectomy 31
carpal tunnel syndrome 151, 154,
261
cataplexy 183
cauda equina 83, 140, 141
compression 85, 143
intermittent claudication 143
cavernoma 260, 264
central sulcus 7
cerebellar ataxia 75, 789
cerebellum 3, 40
abscess 79
anatomy and function 5, 78
lesions 16, 135
localization of lesions 78
and speech 135
tumour 47
cerebral abscess 240, 254, 2678
cerebral cortex, haemorrhage in
37
cerebral hemispheres 40, 41
lesions 17, 81
in tentorial herniation 42
cerebral ischaemia/infarction 25,
267
management 30
prevention of recurrence 301
symptoms and signs 289
cerebral palsy 225
cerebrospinal fluid
flow 40, 41
in infection 250, 251
cervical myelopathy 91, 143
cervical rib 145
cervical spondylosis 98, 143, 259
Charcot joints 96, 97
CharcotMarieTooth disease
162
chemotherapy, brain tumour 52
chickenpox 239
chlorpromazine, parkinsonism
induced by 73
cholinergic crisis 166
chorea 74, 135
circle of Willis 26
circumflex nerve palsy 147
circumlocution 229
CJD 77, 2323
claudication 28
clinical skills 123
clonazepam 206
INDEX
clonus in upper motor neurone

lesions 8
Clostridium tetani 247
clozapine 72, 73
clumsiness 17, 18
cochleo-vestibular nerve 1268
codeine 216
cold sores 239
coma
causes 1856
in epilepsy 194
Glasgow Coma Scale 58, 59,
1845, 186
and hypoglycaemia 179
investigation and management
187
mechanisms 186
prognosis 188
common peroneal nerve palsy
12, 23, 148, 152
communication 20
compensation following head
injury 65
computed tomography see CT scan
COMT inhibitors 71, 72
confusion 224
coning 42, 43, 45
due to lumbar puncture 44
in primary brain injury 56, 57
consciousness 175
in raised intracranial pressure
45
see also unconsciousness
contraction myotonia 169
copaxone 108
coprolalia 76
co-proxamol 216
corneal reflex 122
corpus callosum 40, 41, 42
tumour 47
corpus striatum 69, 71, 73
cortical thrombophlebitis 28
corticosteroids
in cluster headache 216
in dermatomyositis/
polymyositis 171
in giant cell arteritis 218
myopathy associated with
high-dose therapy 171
271
coxsackie viruses 243

cranial nerve disorders 11136
creatine kinase 168, 172
CreutzfeldtJakob disease 77,
2323
CT scan
brain tumour 50
cerebral abscess 240
cerebral ischaemia/infarction
25, 30
cerebral haemorrhage 37
head injury 60, 61
subarachnoid haemorrhage 36
cytomegalovirus infection 249
dantrolene 94
deafness 126
and acoustic neuroma 49
common causes 1278
death, brainstem 1889
deep palmar branch of ulnar
nerve palsy 147
dj vu 197, 264
delirium 224
dementia 224
assessment 229
causes 2304
features 228
investigation 2345
with Lewy bodies 6970, 77,
231, 237, 2667
management 236
multi-infarct 231
in Parkinsons disease 6970
vascular 231
demyelination
in multiple sclerosis 1001,
103, 128
and paraplegia 93
and peripheral neuropathy
158, 159
depression
in Parkinsons disease 69, 70
and tension headache 214
dermatomes 88, 89
dermatomyositis 171
developmental dyspraxia 225
dexamethasone 52
diabetes mellitus
and atheroma 28
facial palsy in 125
and hypoglycaemia 179
and median nerve palsy 151

162
and small vessel disease 27
diabetic amyotrophy 162
diabetic lumbosacral radiculoplexopathy 162
diagnosis 20
diazepam
in febrile convulsions 205
in status epilepticus 208
differential diagnosis 20
diplopia 13
dissociated sensory loss 9
donepezil 723, 230, 236
dopamine 69
dopamine agonists 71, 72
dorsal root ganglion 6, 84
dressing dyspraxia 229
driving
and blackouts 182
and epilepsy 208
and paraplegia 95
Duchenne dystrophy 168
dura 84, 85
dysarthria 16, 29, 79, 1335
in bulbar palsy 131
scanning/staccato 135
spastic 134
dyscalculia 29
dysdiadochokinesia 16, 79
dysgraphia 29
dyskinesia
levodopa-induced 72
tardive 75
dyslexia 29
dysphagia 13, 29
in bulbar palsy 131
dysphasia 29, 2267, 229
dysphonia 131
dystonia 74, 202
axial 76
focal 75
generalized 76
segmental 76
dystrophin 168
ECG 181
ECHO viruses 243
272
echolalia 76
EEG see electroencephalography
electrocardiography (ECG) 181
electroencephalography (EEG)
blackouts 181
epilepsy 193, 196, 198, 204
electromyography (EMG) 172,
173
myasthenia gravis 165
encephalitis
acute 211, 2424, 265
herpes simplex 243
Japanese 243
West Nile virus 243
encephalitis lethargica 253
encephalomyelitis 246
acute disseminated 252
encephalopathy
bovine spongiform 233
epileptic 200
post-traumatic 63
Wernickes 233, 234
endocarditis, non-bacterial 28
endoscopic surgery, brain tumour
52
entacapone 72
entrapment neuropathy 146
epilepsy
absence 192
in brain tumour 45
in cerebral abscess 240
diagnosis 2024
differential diagnosis 49, 201
establishment of cause 203
focal 180, 193, 1968, 203
forms 1939
investigation 204
Jacksonian 198
juvenile myoclonic 77, 196,
200, 207
and learning disability 225
management 20510
and occupation 209
post-traumatic 58, 59, 64
primary generalized 180, 193,
1946, 203
psychological factors 210
temporal lobe 197, 203, 211, 264
terminology 1923
tonicclonic 192
unconsciousness in 176, 180,
182
INDEX
women patients 209

see also seizures
ergotamine 215, 216
Escherichia coli 244
ethosuximide 206
expressive dysphasia 226, 227, 229
eye movements 115, 118
disorders 11521
face
numbness/weakness/palsy
29, 104, 1245, 244
pain 213, 21920, 221, 222, 266
facial expression in Parkinsons
disease 70
facial nerve 1245
facio-scapulo-humeral dystrophy
170
fainting 202
falls 16, 18
false localizing signs 44
45
falx cerebri 40, 41
fasciculation 11
fat embolism 28
febrile convulsions 199, 205, 207
femoral nerve palsy 148
festination 70
fingernose ataxia 16
fish oil supplements in multiple
sclerosis 108
fluent dysphasia 227
folate supplements 207
foot drop 152
foramen of Luschka 40, 41
foramen of Magendie 40, 41
foramen of Munro 41
foramen magnum 40
fourth nerve palsy 119
fourth ventricle 40, 41
fragile X syndrome 75
Friedreichs ataxia 75, 79, 82, 258
fungal infections 241, 249
gabapentin
in epilepsy 206
post-herpetic neuralgia 220
in trigeminal neuralgia 219
gait, in Parkinsons disease 70
gait ataxia 16
galantamine 236
ganciclovir 250
gaze palsy 11617
general paralysis of the insane
233, 248
genital herpes 239
gentamicin 128
giant cell arteritis 113, 218, 222,
266
Gilles de la Tourette syndrome 76
Glasgow Coma Scale 58, 59,
1845, 186
glaucoma 221
glioblastoma multiforme 48
glioma 48, 49, 51, 53
glossopharyngeal nerve 1305
glycogen metabolism disorders
170
Gowers sign 168
GuillainBarr syndrome 132,
163, 252
HAART 250
haematoma
intracranial 56, 57, 58, 59
subdural 64, 218, 232, 237, 266
Haemophilus influenzae 244
hallucinations 197, 264
hypnogogic 183
haloperidol
in chorea 74
parkinsonism induced by 73
halothane 170
head injury
after-care 62
case histories 66, 2567
causes 55, 62
consequences 635
effects 569
management 602
medico-legal aspects 65
severity 63
headache 21322
analgesic-dependent 216
benign sex 217, 222, 266
cluster 216, 222, 265
icepick 216
in intracranial haemorrhage
33, 34, 35, 219
in low intracranial pressure
217, 222, 266
INDEX
in meningitis 219
non-neurological causes 221
45, 217, 222, 266
in subdural haematoma 218
tension 214, 222, 265
heart attack 28
heart disease 28
heelkneeshin ataxia 16
hemianaesthesia 8, 114
in intracerebral haemorrhage
33, 35
hemiballismus 74
hemichorea 74
hemiparesis/hemiplegia 114
contralateral 8
33, 35
ipsilateral 9
hereditary motor and sensory
neuropathy 162
herpes simplex 239
encephalitis 243
herpes zoster 123, 125, 143, 220,
239
highly active anti-retroviral
therapy 250
history-taking 23
HIVAIDS
and dementia 233
and localized infection 241
meningo-encephalitis in 243
neurological problems 245
HMSN 162
HolmesAdie syndrome 121
homonymous hemianopia 8, 29,
112, 114
33, 35
Horners syndrome 120, 216
Huntingtons disease 74, 75, 232
hydrocephalus 35, 232, 244
hyperacusis 124
hyperlipidaemia 28, 231
hypertension 27, 28
benign intracranial 49
and intracerebral haemorrhage
37
and vascular dementia 231
hyperthyroidism 171
273
hyperventilation 176, 178, 182

hypnogogic hallucinations 183
hypoglossal nerve 1305
hypoglycaemia 176, 179, 182
hypotension, in head injury 56,
57, 58, 59
hypothalamic tumour 47
hypothyroidism 79, 234
hypotonia 79
hypoxia 176, 179
in head injury 56, 57, 58, 59
illness, elements of 19
immunization 250
immunocompromised patients,
CNS infection in 249
infection 23854
diagnosis 250
following head injury 56, 57,
58, 59
opportunistic 249
prophylaxis 250
inferior oblique muscle 118
inferior rectus muscle 118
intellectual function, testing 229
intermittent claudication, cauda
equina 143
internal capsule 7
internal carotid arteries 26
occlusion 29, 31
internuclear ophthalmoplegia
104, 117
intervertebral disc
degenerative disease 221
prolapse 12, 23, 13843
protrusion 85
intervertebral facet joint 85
intervertebral foramen 85
intracerebral haemorrhage 25,
323, 35, 37
intracranial haematoma 56, 57,
58, 59
intracranial haemorrhage 25, 28,
328
Japanese encephalitis 243
jaw-jerk 122
JC virus 246
KayserFleischer ring 76
Kernigs sign 244
Korsakoffs psychosis 233, 234

kyphoscoliosis 96
labyrinthitis, acute 128
lamotrigine 206, 207, 219
lancinating pain 219
lateral corticospinal tract 83, 84
lateral cutaneous nerve of thigh,
entrapment 148, 153
lateral medulla, infarction 132
lateral rectus muscle 118
lateral spinothalamic tract 84
lateral ventricles 40, 41
learning disability 225
Lebers hereditary optic
neuropathy 113
LennoxGastaut syndrome 200
leprosy 146, 247
leptospirosis 244
levator palpebrae superioris 118
levetiracetam 206
levodopa in Parkinsons disease
712
Lewy bodies 6970, 77, 2301,
237, 2667
LHermittes symptom 259
limb girdle syndrome 170
Listeria monocytogenes 249
liver flap 77
long thoracic nerve palsy 147
lorazepam in status epilepticus
208
low intracranial pressure 217,
222, 266
lower limb
champagne bottle legs 162
peripheral nerve palsies 12, 23,
148, 152
peripheral neuropathy 160
segmental nerve supply 142
lower motor neurones 3
anatomy and function 4
lesions 1112
156, 157
in speech 133, 1345
lumbar puncture
contraindications 50, 187, 240
headache following 217
risks 44
in subarachnoid haemorrhage
36
274
lumbar spondylosis 143

lumbosacral plexus lesions 144
lumbosacral radiculo-plexopathy,
diabetic 162
Lyme disease 125, 244
lymphoma, brain 53
magnetic resonance imaging see
MRI
malignant hyperpyrexia 1701
MAO-B inhibitors 71, 72
marche petits pas 31, 231
medial rectus muscle 118
median nerve palsy 147, 151
medulla 40
coning 40, 42, 43, 44, 45, 56, 57
demyelination 104
lesions 10, 78
Mnires disease 127, 128
meningioma 48, 51, 53, 93, 112
meningism 219
meningitis 33, 207, 219
acute 2424
bacterial 2434
cryptococcal 233
malignant 248
tuberculous 246, 254, 268
viral 243
meningococcal infection 244
meningo-encephalitis, acute
2424
meralgia paraesthetica 153
mesial temporal sclerosis 199
metastatic tumour
brain 49, 51, 53
spine 98, 144, 2589
methanol poisoning 113
methysergide 216
metoclopramide
movement disorder induced by
75
parkinsonism induced by 73,
2578
micro-aneurysms 32
midbrain 40
demyelination 104
lesions 10, 78
middle cerebral artery 26
ischaemia 29
middle ear infection 125
migraine 33, 113, 21415, 217
INDEX
mitoxantrone in multiple sclerosis

108
mitral valve disease 28
MND see motor neurone disease
monocular blindness 112, 113
monoparesis
contralateral 8
ipsilateral 9
motor cortex 7
motor dysphasia 227
motor end plate 164
motor neurone disease (MND)
155, 1567
bulbar palsy in 132, 156, 157
and dementia 232
lower motor neurones 11, 156,
157
paraplegia in 93
and trinucleotide repeats 75
upper motor neurones 156, 157
movement
abnormalities 3
involuntary 15, 747
normal 67
MRI
brain tumour 50
epilepsy 204
focal epilepsy 197
intervertebral disc disease 143
multiple sclerosis 106
paraplegia 93
spinal extradural abscess 241
multifocal neuropathy 146, 162
multi-infarct dementia 31
multiple sclerosis 22, 79, 99
aetiology 107
clinical expression 1035, 113,
128
and dementia 232
diagnosis 106
lesions 1001
dissemination 102
management 1078
paraplegia in 90, 91, 94
tremor in 68
multiple system atrophy 73
muscle 3
biopsy 172, 173
denervation 173
incoordination 16
primary disease 14, 155, 16771

tone
decreased 11
increased 8
in primary muscle disease 14
wasting 11, 14, 1723
weakness see weakness
muscular dystrophies 167,
16870
musculocutaneous nerve palsy
147
myasthenia gravis 155, 1645
bulbar muscle involvement
132
characteristics 13
diagnosis 165
management 166
ocular involvement 120
myasthenic crisis 166
Mycobacterium leprae 247
myoclonus 77
myopathy 120, 155, 16771
myotomes 88, 89
myotonic dystrophy 75, 169
myxoedema 151
narcolepsy 183
neck stiffness/rigidity 33, 34,
219, 244
neologisms 227
nerve root lesions 13844
nervous system, components and
anatomy 318
neurofibroma 93, 144
neuromuscular blockade 13
neuromuscular junction 3
lesions 13
neuropathy see peripheral
neuropathy
neurosyphilis 233, 248
nigrostriatal pathway 69, 71, 73
nimodipine 36
non-bacterial endocarditis 28
non-fluent dysphasia 227
non-steroidal anti-inflammatory
drugs in icepick headache
216
nystagmus 16, 79
rotatory 81
INDEX
obsessivecompulsive disorder in
Gilles de la Tourette
syndrome 76
obturator nerve palsy 148
occupation
and epilepsy 209
and paraplegia 95
occupational therapy, after stroke
30
oculogyric crisis 75
olfactory nerve 112
ophthalmic artery
ischaemia 29
thrombo-embolism 113
optic chiasm 112
compression 113
tumour 47
optic nerve
disorders 11214
in multiple sclerosis 100, 103,
113
optic neuritis 103, 113, 136, 260
oral contraceptives, interaction
with anticonvulsant
therapy 206, 209
orbital mass lesions 121
osteoarthritis of the spine 139,
142, 143
otitis media 221
oxygen therapy 216
pain sensation 6
loss of 9
Pancoast tumour 145
panhypopituitarism 48
panic attacks 203
papillitis 103
papilloedema 187
33, 34, 35
45, 113
paradoxical embolism 28
paralysis 3
paraparesis 10
paraphasias 227
paraplegia 8398
in multiple sclerosis 105, 108
Parinauds syndrome 116
parkinsonism 69, 73
post-encephalitic 253
275
Parkinsons disease 22, 67, 6970

dysarthria in 135
features 15, 70
management 713
tremor 15, 68, 70
patient, response to symptoms
19
percussion myotonia 169
pergolide 72
perimesencephalic haemorrhage
36
peripheral nervous system 137,
158
lesions 14653
15860
causes 1613
hereditary motor and sensory
162
loss of sensation 18
lower motor neurone weakness
11
and sensory ataxia 81
pes cavus 97
phenobarbitone 206, 207, 208
phenytoin 206, 207, 208
photosensitivity 195, 208
physiotherapy
after stroke 30
in paraplegia 94
Picks disease 232
pituitary gland tumour 47, 48, 51,
53, 113
plantar responses
absent 11
extensor 8
flexor 11
plasma exchange in myasthenia
gravis 166
pneumococcal infection 244
poliomyelitis 11, 239
polycythaemia 28
polymyalgia rheumatica 218
polymyositis 170, 171
polyneuropathy see peripheral
neuropathy
pons
demyelination 104
lesions 10, 37, 78
position sense 6
loss of 9, 17, 18, 80
post-concussion syndrome 63,

220
posterior cerebral arteries 26
ischaemia 29
occlusion 113, 114
posterior column 84
posterior dysphasia 227
posterior fossa 40
tumour 79
posterior interosseous nerve
palsy 147
posterior tibial nerve palsy 148
post-herpetic neuralgia 143, 220
post-infective syndromes 2523
post-streptococcal syndromes
253
post-traumatic amnesia 63
post-traumatic encephalopathy
63
postural hypotension 176, 177,
182, 202
posture
abnormalities 15
prednisolone 166
pregnancy
and Bells palsy 125
carpal tunnel syndrome in 151
premipexole 72
presenilin 230
primary motor pathway 3
progressive multifocal
leucoencephalopathy
246
progressive muscular atrophy
157
progressive rubella
panencephalitis 246
progressive supranuclear palsy
73
propantheline 166
proptosis 121
prostigmine 166
pseudoathetosis 80
pseudobulbar palsy 31, 134, 156,
157
pseudodementia 226
psychogenic non-epileptic
attacks/blackouts 180,
182, 202
ptosis 13, 119, 120
punch-drunk state 63
276
pupils
afferent pupillary defect 103
ArgyllRobertson 121, 248
constriction 118
dilatation 118
pyramidal tract 84
pyridostigmine 166
quadriparesis 10
quetiapine 72, 73
rabies 246
radial nerve palsy 147, 149
radiation myelopathy 93
radiculopathy 13844
radiotherapy, brain tumour 52
raised intracranial pressure 113
in brain tumour 45, 46
differential diagnosis 49
headache in 45, 217, 222, 266
RamsayHunt syndrome 125
receptive dysphasia 226, 227, 229
recurrent laryngeal nerve 130
palsy 132
reflex arc 159
reflex arc segmental values 88, 89
reflexes
in lower motor neurone lesions
11
in upper motor neurone lesions
8
rehabilitation
following intracerebral
haemorrhage 37
following subarachnoid
haemorrhage 36
reserpine, parkinsonism induced
by 73
respite care, in paraplegia 95
retrobulbar neuritis 103
Reyes syndrome 253
rigidity
cogwheel 70
lead pipe 70
riluzole 156
rivastigmine 723, 230, 236
road traffic accidents 55, 90
Rombergs sign 18, 80
ropinirole 72
INDEX
S1 nerve root syndrome 12

sagittal sinus thrombosis 28
sarcoidosis 125
Saturday night palsy 149
sciatic nerve palsy 148
second sensory neurone 6
seizure
absence 195, 202
adversive 197
atonic 200
focal motor 197, 202
focal sensory 197, 202
frontal lobe 197, 202
myoclonic 196
temporal lobe 197, 203, 211,
264
tonicclonic 192, 198, 202
primary generalized 1945
secondarily generalized 193
selegiline 72
self-neglect 229
sensation 3, 6
loss of 1718
sensory ataxia 801
sensory cortex 7
sensory dysphasia 227
sexual dysfunction 10
in paraplegia 92, 94
shingles 123, 125, 143, 220, 239
shunting, ventricular 52
ShyDrager syndrome 73
sinus disorders 221
sixth nerve palsy 44, 119, 136, 260
sleep disturbance in Parkinsons
disease 70
sleep paralysis 183
small vessel disease 27, 29, 31
smoking 28
social withdrawal 229
sodium valproate
in epilepsy 206, 207
in migraine 215
overdose 190, 264
spastic paraparesis 96
speech 133
speech therapy, after stroke 30
sphincter function, loss of 10
spinal accessory nerve 129
spinal cord
anatomy 835
demyelination 1045
disease 18, 81
lesions
cervical 10
indications of level 10, 86
segmental signs 889
tract signs 867, 89
upper motor neurone signs
10
subacute combined
degeneration 93
spinal extradural abscess 93, 241
spinal nerve 138, 139
spine
anatomy 835
malignant disease 90, 91, 98,
144, 2589
osteoarthritis 139, 142, 143
spondylotic myelopathy 91, 93
squint, concomitant 120
Staphylococcus aureus 241
startle myoclonus 77
status epilepticus 201, 208
StokesAdams attack 178, 190,
263
streptococci
Group A 253
Group B 244
streptomycin 128
stroke 21, 2538
lacunar 27, 29
progressive focal neurological
deficit 49
subacute sclerosing
panencephalitis 246
subarachnoid haemorrhage 25,
28, 324, 36, 219
case histories 38, 222, 255, 265
subdural haematoma 64, 218,
232, 237, 266
substantia nigra 71
in parkinsonism 73
sumatriptan 216
superior oblique muscle 118
superior rectus muscle 118
supranuclear gaze palsy 116
surgery
brain tumour 512
epilepsy 210
Parkinsons disease 72
suxamethonium chloride 170
Sydenhams chorea 75, 253
INDEX
symptoms
mode of onset 23
patients response to 19
syphilis, tertiary 233, 248
syringobulbia 97
syringomyelia 93, 967
tabes dorsalis 233, 248
taboparesis 233
tardive dyskinesia 75
taste perception 130
alteration in 124
teeth, disorders 221
temperature sensation 6
loss of 9
temporal arteritis (giant cell
arteritis) 113, 218, 222,
266
temporo-mandibular joint,
arthritis 221
Tensilon test 165, 166
tentorial herniation 42, 43
after head injury 56, 57
in lumbar puncture 44
45
tentorial hiatus 40, 42
tentorium cerebelli 40, 41
tetanus 247
tetrabenazine, parkinsonism
induced by 73
tetraparesis 10
tetraplegia 88
thiazide diuretics 301
thiopentone 208
third nerve palsy 119
third sensory neurone 6, 7
third ventricle 40, 41, 42
thrombo-embolic disease 267,
28, 113, 179
thrombolytic therapy 30
thymectomy 166
thymoma 165
thymus, in myasthenia gravis
165
TIA 27, 28, 176, 179, 182
tics 76
tinnitus 126
tissue plasminogen activator 30
tizanidine 94
tobaccoalcohol amblyopia 113
Todds paresis 198
277
topiramate 206, 216

torticollis 75
touch sensation, loss of 17, 801
toxoplasmosis 233, 241, 249
transient global amnesia 183
transient ischaemic attack 27, 28,
176, 179, 182
trapezius muscle 129
trauma
brachial plexus 145
paraplegia due to 90, 91, 93
peripheral nerves 146
tremor 689
and alcohol 69
classification 68
dystonic 69
familial essential 68, 69
intention 68, 79
kinetic 16, 68
in Parkinsons disease 15, 68,
70
physiological 68, 69
Trendelenburg weakness 14
Treponema pallidum 248
tricyclic antidepressants
in atypical facial pain 220
post-herpetic neuralgia 220
trigeminal nerve 1223
trigeminal neuralgia 122, 136,
219, 222, 261, 266
trinucleotide repeat 75, 169
triptans 215, 216
tuberculoma 241
tuberculous meningitis 246, 254,
268
ulnar nerve palsy 147, 150, 154,
261
unconsciousness 17590
upper limb
peripheral nerve palsies 147,
14951
peripheral neuropathy 160
segmental nerve supply 141
upper motor neurones 3
anatomy and function 4, 7
lesions 810
157
in speech 133, 134
uveitis 221
vagus nerve 1305

vasculitis 146
vasovagal syncope 176, 177, 182,
202
vegetative state 188
venous infarction 28
verapamil 216
vertebrae 83, 85
vertebral arteries 26
dissection 267, 28
ischaemia 29
vertebro-basilar ischaemia 179
vertigo 29, 81, 126
benign paroxysmal positional
127, 128, 136, 261
in Mnires disease 128
vestibular disease, ataxia in 81
vestibular failure 127, 128
vestibular neuronitis 128
viral infections 211, 239, 243,
2456, 249, 265
visual disorders 11214
in cerebral
ischaemia/infarction 29
in giant cell arteritis 218
in multiple sclerosis 103, 104,
113
45
visual inattention/neglect 114
visual pathways 112
vitamin B1 deficiency 234
vitamin B12 deficiency 161, 234
vocal cord palsy/paralysis 132
vomiting
33, 34, 35
in migraine 215
45
in Reyes syndrome 253
warfarin
interaction with anticonvulsant
therapy 206
in stroke prophylaxis 31
weakness 3
investigation 1723
in lower motor neurone lesions
11, 12
278
proximal 14
in upper motor neurone lesions
8, 9
Weils disease 244
INDEX
Wernickes area 226, 227

Wernickes encephalopathy 233,
234
West Nile virus encephalitis 243
wheelchair acceptance and skills
945
Wilsons disease 76
women and epilepsy 209
wrist drop 149
writers cramp 75

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Essential

2005 by Blackwell Publishing Ltd

Four Dragons edition 1993

Library of Congress Cataloging-in-Publication Data

Preface to the fourth edition, vii

gravis and muscle disease, 155

Answers to case histories, 255

The three previous editions of this book were written by only

Excellent textbooks of neurology already exist, which deal with

We both want to acknowledge the very great help of Drs N.

Clinical skills, physical

Theres something wrong with my left leg, and Im not walking

Fig. 1.1 The patients history

Components of the nervous system required for

Upper motor neurone

Lower motor neurone

Lesions along the primary motor pathway, UMNLMN

An upper motor neurone involved in left leg

A lower motor neurone involved in left leg

Basal ganglion control of the left leg

Cerebellar control of the left leg

Pain and temperature sensation in the left leg

Position sense in the left leg

The final piece of anatomical knowledge which is helpful

Having reviewed the components of the nervous system

Upper motor neurone

Upper motor neurone

Lower motor neurone

Characteristic of upper motor neurone lesions:

A spinal cord lesion more usually causes upper motor

Paraparesis, if the lesion is at or

Lesions anywhere between the midbrain and lower spinal cord

Lower motor neurone

Upper motor neurone

Lower motor neurone

Characteristics of lower motor neurone lesions:

Generalized LMN weakness may

LMN weakness may be confined

Upper motor neurone

Lower motor neurone

The classic disease of the neuromuscular junction is myasthenia

Upper motor neurone

Lower motor neurone

Characteristics of primary muscle disease:

Upper motor neurone

Lower motor neurone

Two main syndromes, each with different characteristics:

Upper motor neurone

Lower motor neurone

Characteristics of cerebellar lesions are:

Upper motor neurone

Lower motor neurone

Characteristics of movement in the presence of sensory loss:

Cerebral hemisphere lesions:

Loss of proprioceptive sense in

The patients response to his symptoms

A physical illness with an

Diagnosis, explanation and planning

Patience with patients