NEUROLOGY

HANDBOOK

Prof CH van der Meyden

Prof C-M Schutte

Preface
This neurology text is aimed at the level of medical- and paramedical students and registrars
in internal medicine and psychiatry.

The goal is to attempt to bridge the gap between the patient’s clinical problem and the
disease oriented texts and literature.

The focus is on the definition of the neurology language, symptoms, signs, core concepts,
syndromes and approaches to common problems.

The most advanced strategy is the perfection of basic principles e.g., the history,
examination and their interpretation.

The respect of patients, teachers and students together with the love of the art and science
of medicine will remain universal values independent of time and place.

Education is the solvent and vaccine of illness, ignorance, prejudice, conflict and the so-
called class differences. Only the educated are free. The sick and the poor are in God’s
eyes assuredly the highest.

The paediatric neurological evaluation, chapter 20, was compiled by Prof I Smuts and
Dr TW de Witt, and the contributions by Dr M van Niekerk are acknowledged and
appreciated.

Prof CH van der Meyden Prof C-M Schutte

University of Limpopo, MEDUNSA campus. University of Pretoria.

2018
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INTRODUCTION

1. THE NEUROLOGICAL HISTORY

1.1 General and past medical history

1.2 Chronological evolution of the main complaint
1.3 Time course of neurological illness
1.3.1 Acute onset with gradual recovery
1.3.2 Gradual progression in degree of neurological deficit
1.3.3 Paroxysmal disturbances (begin and end abruptly)
1.3.4 Relapses and remissions (worsening and improvement of symptoms)
1.3.5 Relapse-remission relapse pattern

2. PROBLEM ORIENTED APPROACH

3. SPECIFIC SYMTOMS AND SIGNS

4. OVERVIEW OF IMPORTANT NEUROLOGICAL CONDITIONS

4.1 Treatable conditions

4.2 Life-threatening conditions
4.3 Core neurological syndromes and concepts
4.4 Neurological terms
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1. THE NEUROLOGICAL HISTORY

1.1 GENERAL AND PAST MEDICAL HISTORY

The history is the most important part of the neurological evaluation –

neurologists learn most about the likely pathology from the history rather
than the examination. The history is usually presented in a conventional
way so that doctors being informed know what they going to be told about
next.

The Neurological History

 Name, age, gender, handedness, occupation
 History of present complaint
 Neurological screening questions
 Past medical history
 Drug history
 Family history
 Social history

Basic background information

 The patient’s name should always be carefully remembered and
he/she should always be addressed by his or her name. We should
refer to the patient by name and not by their illness.
 The patient’s handedness is established. Handedness is important.
The left hemisphere contains language in almost all right-handed
individuals, and in 70% of patients who are left-handed or
ambidextrous.
 At the time of the initial history, attention should be given to the
patient’s family circumstances, occupation, living conditions and
general interests.
 The past history is noted – medical, surgical and familial.
 In particular, the patient’s use of medicines (prescribed drugs and
over-the-counter medicines) is of great importance. (Many women do
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not think of the oral contraceptive as a drug and need to be asked

about it specifically)
 An assessment of the patient’s smoking and alcohol habits is made.
Establish any exposure to toxins.
 A systematic inquiry to elicit disturbances of the various organ
systems is undertaken.

1.2 CHRONOLOGICAL EVOLUTION OF THE MAIN COMPLAINT

 The main complaint or principal problems are identified, and each one
is carefully examined. The history should be noted in chronological
fashion in a stepwise manner as the events had actually occurred
over time. At crucial stages of the history events are noted by the
second, minute or hour as they actually unfolded.
 The duration of the symptoms and the daily, weekly or monthly
frequency thereof is established.
 The pattern of the symptoms and whether they improve, remain
unchanged or worsen is assessed, particularly as this may be of
cardinal importance when decisions regarding treatment are made
(e.g., worsening of a neurological condition may prompt one to initiate
further investigations while a static neurological problem over time
may favor a conservative stance).
 Accompanying symptoms (“the company it keeps”) are often of great
importance, such as e.g., the presence of a headache associated with
an aura, nausea and photophobia, may favor the diagnosis of
migraine and e.g., severe retro-orbital pain with lachrimation, blocked
nasal passage, red eye and a Horner syndrome would point to the
underlying presence of a Cluster Headache.
 In conditions such as coma, epilepsy, syncope, language
disturbances, confusion and dementia the patients are frequently not
capable of giving a good account of themselves.
 In this setting, it is ESSENTIAL to obtain a collateral history from
relatives as soon as possible, usually by means of the telephone.
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 The history often reflects (like a video-film) disturbances of function

over long periods of time, whereas the physical examination is merely
an indication of a morphological picture at one moment in time (e.g.,
like a photograph).

1.3 TIME COURSE OF NEUROLOGICAL ILLNESS

This tells you about the tempo of the pathology - suggesting the nature of
the underlying cause.

 The onset: how did it come on?

 Progression: is it continuous or intermittend? Has it improved,
stabilised or progressed ?
 The pattern
 Precipitating or relieving factors
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1.3.2 ACUTE ONSET WITH GRADUAL RECOVERY

Grade of Severity of
Neurological Deficit

Onset Time scale

Examples of illnesses
Pathology Time Duration Possible Symptoms
Completed stroke Minutes to hours, e.g. hemiparesis
usually < 24h
Embolic stroce seconds e.g. hemiparesis
Guillain-Barre syndrome Hours to days, usually < e.g. lower motor neuron weakness
4 weeks
Head trauma Seconds to hours e.g. depression of level of
consciousness, loss of memory
Bell’s palsy Hours to days e.g. lower motor neuron palsy of N
VII
Cranial nerve III palsy due to Hours to days e.g. diplopia, dilated pupil
diabetes mellitus

1.3.1 GRADUAL PROGRESSION IN DEGREE OF NEUROLOGICAL DEFICIT

Grade of
Neurological
Deficit

Time scale
Examples

Pathology Time duration Possible symptoms

Brain tumours Weeks to months e.g. hemiparesis
Spinal cord tumours Weeks to months e.g. paraparesis
Muscle dystrophy Years to decades e.g. proximal muscle weakness,
selective muscle involvement,
hereditary history
Parkinson’s Disease Months to years e.g. bradykineasia, muscle rigidity,
tremor
Spinocerebellar ataxia Years e.g. in-coordination or ataxia
Primary Progressive Multiple Months to years Gradual appearance of upper motor
Sclerosis neuron signs in the legs
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1.3.3 PAROXYSMAL DISTURBANCES (BEGIN AND END ABRUPTLY)

Grade of Neurological
Deficit

Time scale

Examples of illness:
Pathology Time Duration Possible Symptoms
Transient Ischaemic less than 24 hours e.g., hemiparesis, hemi-sensory
Attacks (TIA’s) (frequently less than 2 hours change and mon-ocular blindness
Epilepsy seconds to a few minutes Loss of consciousness, tonic-clonic
movements
Migraine 4 – 72h Headache, nausea, vomiting, photo-
phonophobia
Cluster headache 15 min – 3 hours Headache, lacrimation, nasal
congestion, motor agitation
Chronic Paroxysmal 2 – 45 minutes
Hemicrania
Syncope Seconds to minutes Loss of consciousness
Meniere’s disease Hours to days Attacks of vertigo
Trigeminal neuralgia 20 – 40 seconds Sharp shooting stabbing pains, occurs
in bouts
Familial periodic Few hours e.g. muscle weakness in the arms and
paralysis legs

1.3.4 RELAPSES AND REMISSIONS (WORSENING AND IMPROVEMENT OF SYMPTOMS)

Grade of Neurological
Deficit

Time scale
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Examples of illnesses Multiple sclerosis: relapses and remission

e.g.,
1. Multiple sclerosis i) Optic neuritis
2. Myasthenia Gravis ii) Internuclear ophthalmoplegia
3. Polymyositis iii) Spastic paraparesis
4. Temporal arteritis iv) Cerebellar signs
5. Systemic lupus
erythematosus

1.3.5 RELAPSE-REMISSION-RELAPSE PATTERN

Grade of
Neurological
Deficit

A
Time scale

a = Time of trauma or initial bleed

b = Depression of level of consciousness
c = Patient starts to wake up
d = Secondary depression of level of consciousness

Examples of illnesses:
1. This pattern is characteristic of hemorrhage between the meninges,
e.g., a subdural or extradural haematoma.
2. A rebleed from a Berry Aneurysm.
3. Bleed or cystic change in a brain tumor and haemorrhage into the
metastases of e.g., melanomas and choriocarcinomas.
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2. THE PROBLEM ORIENTED APPROACH (Weed, 1968).

S - “Subjective” or history
O - “Objective” or examination
A - “Assessment” or evaluation of the symptoms, signs – many more
mistakes are made by not looking (or not listening, not examining the
patient repeatedly) than by not knowing
P - “Plan” is evaluated according to
1. Diagnostic strategy
2. Therapeutic strategy
3. Social harmonization of the patient’s illness is assessed, and its
impact on the social home- and work situation

3. SPECIFIC SYMPTOMS AND COMPLAINTS

 Fortunately, the history enables the diagnosis to be made in perhaps

some eighty to ninety percent of patients and
 in the event of an unhelpful history, the physical examination and
special investigations frequently tend to add little.
 Returning to the patient and repeating the history and examination,
frequently yields invaluable information.
 A good history and a good examination form the cornerstone of good
patient care which forms the royal road to the loving and learning of
neurology in particular, and medicine in general.

4. OVERVIEW OF SOME IMPORTANT NEUROLOGICAL CONDITIONS:

4.1. TREATABLE CONDITIONS (See lecture notes hand-outs)

1. Epilepsy
2. Headache
3. Myasthenia Gravis .
4. Wernicke’s encephalopathy
5. Delirium
6. Coma
7. Dementia
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8. Syncope
9. Stroke
10. Subdural- and epidural- and intracranial haematomas
11. Neurosyphilis
12. Parkinson’s disease
13. Neurocysticercosis
14. Brain abscesses and granulomas
15. Polymyositis; dermatomyositis; CIDP (chronic inflammatory demyelinating
poly-radiculo neuropathy); Guillain Barre syndrome
16. Multiple Sclerosis and ADEM (acute disseminated encephalomyelitis)
17. Raised intracranial pressure; hydrocephalus and brain tumours
(meningiomas; pituitary tumours, metastases and gliomas)
18. Sciatica, neurogenic claudication and lumbar spinal stenosis
19. Cervical spondylosis with cervical spinal stenosis (myelopathy) and
radiculopathies (root signs)
20. Spinal cord tumors
21. Entrapment neuropathies
22. Sleep disorders such as Obstructive Sleep Apnoea Syndrome and
Narcolepsy

4.2 LIFE-THREATENING CONDITIONS

1. Coma
2. Delirium
3. Meningitis
4. Guillain-Barré syndrome
5. Raised intracranial pressure; granulomas; brain abscess; brain tumour;
hydrocephalus
6. Temporal arteritis
7. Transtentorial herniation
8. epilepticus
9. Cerebral haemorrhage; strokes

4.3 CORE NEUROLOGICAL SYNDROMES AND CONCEPTS

1) Acute onset of severe headache (thunderclap headache)
2) Amyotrophic lateral sclerosis
3) Aphasia
4) Automatisms
5) Bulbar palsy
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6) Chronic paroxysmal hemicrania
7) Classic migraine
8) Completed stroke
9) Decerebrate posturing (all four limbs adduction and extension)
10) Decorticate posturing (arms flexion, legs extension and adduction)
11) Distal weakness of the arms and legs (peripheral neuropathy)
12) Dizziness
13) Dysarthria
14) Dystonia
15) Encephalitis
16) Exertional headache
17) Gerstmann’s syndrome
18) Glove and stocking-type sensory loss (peripheral neuropathy)
19) Grand mal attacks (tonic-clonic convulsions)
20) Guillain Barré syndrome
21) Hemiplegia
22) Horner’s syndrome
23) Internuclear palsy
24) Lacunar infarction
25) Lower motor neuron weakness
26) Metabolic et al coma
27) Migraine
28) Myoclonus
29) Narcolepsy
30) Obstructive sleep apnoea syndrome
31) Optic atrophy
32) Optic neuritis
33) Papilledema
34) Paraplegia
35) Parkinson’s disease
36) Parkinson’s syndrome
37) Persistent vegetative state
38) Petit mal attacks (typical absences)
39) Postero-lateral sclerosis of the spinal cord (with Vit B12 deficiency; HIV
vacuolar myelopathy)
40) Proximal weakness of the arms and legs (typical of a myopathy)
41) Pseudobulbar palsy
42) Quadriplegia
43) Reversible ischaemic neurological deficit (RIND)
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44) Right sided parietal lobe syndrome
45) Sensory level over the trunk
46) Stroke in evolution
47) Structural causes of coma
48) Third nerve palsy
49) Traction-inflammatory type headache (structural or secondary headaches)
50) Transient ischaemic attacks (TIA)
51) Transtentorial herniation
52) Upper motor neuron weakness
53) Vertigo
54) Viral meningitis
55) Wernicke’s encephalopathy

Neurological Terms

 Encephalopathy - abnormality of the brain

 Encephalitis – inflammation of the brain
 Meningitis – inflammation of the meninges
 Meylopathy – abnormality of the spinal cord
 Myelitis – inflammation of the spinal cord
 Radiculopathy – abnormality of a nerve root
 Poliradiculopathy – abnormality of many nerve roots
 Plexopathy – abnormality of nerve plexus (brachial or lumbar)
 Peripheral neuropathy – abnormality of peripheral nerves
 Polineuropathy – abnormality of many peripheral nerves
 Mononeuropahty – abnormality of a single nerve
 Myopathy – abnormality of muscle
 Myositis – inflammatory disorder of muscle
 Functional:
o Non-structural pathology – an abnormality of function
o Psychiatrically induced neurological abnormalities
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PART 1: IMPORTANT NEUROLOGICAL CONDITIONS

Chapter 1

1. STROKE

1.1 What is the clinical type of stroke?

1.2 What is the anatomical and vascular territory involved?
1.2.1 Cerebral artery branch occlusions
1.2.2 Lacunar infarctions
1.2.3 Internal carotid territory of involvement
1.2.4 Vertebro-basilar area of involvement
1.3 What is the pathology of the stroke?
1.3.1 Cerebral infarction may be caused by cerebral artery embolism or
thrombosis
1.3.2 Cerebral haemorrhages
1.4 Which are the risk factors for stroke?
1.4.1 Hypertension
1.4.2 Heart lesions
1.4.3 Diabetes Mellitus
1.4.4 Alcoholism
1.4.5 The oral contraceptive pill
1.4.6 Haematocrit
1.4.7 Smoking
1.4.8 Hyperviscosity syndromes
1.4.9 HIV and Strokes (Bradley’s text)
1.5 What are some of the complications of strokes?
1.5.1 Intracerebral complications
1.5.2 Extracerebral complications
1.6 Prognostic factors
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1. STROKE

Definition of stroke

An acute onset of focal neurological deficit of the central nervous system (above
the foramen magnum/excluding the spinal cord) from vascular origin.

• Acute onset indicates an immediate onset or one that occurs within minutes to
hours, or exceptionally, an onset, which stretches over days.

• Focal neurological deficit refers to e.g., hemiparesis; hemisensory change;

monocular blindness; aphasia; visual field defects etc.

• Involvement of the central nervous system refers, for example, to lesions of the
cerebral- or cerebellar hemispheres, the basal ganglia, the brainstem and the
optic nerves.
Stroke definition

APPROACH TO STROKE (History remains of utmost importance)

1.1 What is the clinical type of stroke?

Definition of Completed Stroke

A Completed stroke is defined as the presence of acute onset of focal neurological

deficit (from vascular origin) for a duration of more than 24 hours after the onset of
the event.

(In the presence of an early dense neurological deficit the diagnosis may often be
anticipated prior to the elapsing of the 24 hours.)
Stroke completed definition
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Definition of Transient Ischaemic Attack (TIA)

In TIA, the acute onset of a neurological deficit is usually followed by a speedy

recovery within 5 – 20 minutes and mostly within two hours, although by definition
recovery may take up to 24 hours.
TIA definition
Diagnostic criteria for Transient Ischaemic Attacks (TIA)

1. Focal non-convulsive neurological deficit which is understood to imply an ischaemic

origin (involuntary movements reflect ictal or epileptic rather than ischaemic
phenomena).
2. Usually has a sudden onset (embolic phenomenon) reaching a maximum intensity
almost immediately
3. Usually lasts 5 – 20 minutes (< 24 hours by definition). An attack of only a couple of
seconds’ duration rarely turns out to be a TIA.
4. Repeated attacks are usually clinically stereotyped.

A clear marching progression of symptoms from one part of the body to the other is very
rare and more reminiscent of a migraine phenomenon.
TIA diagnostic criteria
Definition of Stroke in Evolution

In this event the focal neurological deficit develops gradually or in a stepwise fashion,
usually over a few hours, but exceptionally over days.
After the peak of neurological disturbance is attained, the clinical picture becomes that of
a completed stroke.
Stroke in evolution definition
Definition of a Reversible Ischaemic Neurological Deficit (RIND)

The picture is that of a completed stroke, with good recovery and minimal residual signs
after three weeks.

Reversible ischaemic neurological deficit definition

1.2 What is the anatomical and vascular

territory involved?

1.2.1 Cerebral artery branch occlusions

• The middle cerebral artery is the largest

branch of the internal carotid.
The artery supplies a portion of the frontal lobe
and the lateral surface of the temporal and
parietal lobes, including the primary motor and
sensory areas of the face, throat, hand and
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arm and in the dominant hemisphere, the areas for speech.
The middle cerebral artery is the artery most often occluded in stroke.
• In the case of a middle cerebral artery
occlusion the arm is more affected than the leg,
and the following may be observed:
o Contralateral paresis of face and arm/leg
o Contralateral sensory loss in face and
arm/leg
o Aphasia (dominant hemisphere)
o Contralateral neglect syndrome (non-
dominant hemisphere)
• Cardiac and artery to artery embolism is the
most common cause of the cerebral a r t e r y
branch occlusion syndromes.

1.2.2 Lacunar Infarctions

A Lacunar infarction is caused by occlusion of a single deep
penetrating artery (small nonbranching end arteries) and is typically
smaller than 15 mm in width. Lacunar infarctions account for about 25%
of all ischeamic strokes.
These usually involve the deep penetrating end arteries to the internal
capsule and brainstem and are associated with micro-arteriosclerosis or
“lipohyalinosis” and the underlying presence of hypertension or diabetes
mellitus (Mohr 1982).

The following clinical pictures may be found with lacunar

infarctions:
1. Dense motor hemiplegia (pure motor dysfunction)
2. Hemihypoesthesia (pure sensory dysfunction)
3. Dysarthria clumsy-hand syndrome and
4. Homolateral ataxia with crossed paresis (“ataxic
hemiparesis”)
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1.2.3 Internal carotid territory of involvement

In this case a clinical
picture may be found with
the pattern of
involvement of the middle
cerebral- or anterior
cerebral arteries or with
internal capsule
involvement (lateral
striate artery ischaemia
with hemiplegia;
hemisensory change;
homonomous hemianopia). and

TIA’s are usually associated with athero- and arteriosclerosis at the origin
of the internal carotid artery (stenosis), with shedding of microemboli
(platelet-fibrin material), and are potentially treatable surgically.

Carotid TIA’s may present with the following:

1. Transient ipsilateral monocular blindness;
2. Contralateral body weakness or clumsiness;
3. Contralateral body sensory loss or paraesthesiae;
4. Aphasia with dominant hemisphere involvement;
5. Contralateral homonomous visual field defects etc, (Bradley’s text).

1.2.4 Vertebro-basilar area of involvement

The clinical picture fits that of a vertebral, basilar or posterior cerebral

artery dysfunction. Symptoms suggestive of vertebro-basilar ischaemia
include the following:
1. Bilateral weakness or clumsiness (may be unilateral or shifting);
2. Bilateral shifting or crossed sensory loss or pareaesthesiae;
3. Bilateral binocular visual defects;
4. The presence of two or more of the following symptoms:
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a. Vertigo;
b. Diplopia;
c. Dysphagia;
d. Dysarthria;
e. Ataxia (Bradley’s text)

With vertebro-basilar strokes the risk of cerebral arterial angiography is

significantly higher and surgical treatment possibilities are more limited
than with internal carotid artery territory strokes.

1.3 What is the pathology of the stroke?

1.3.1 Cerebral infarction may be caused by either cerebral artery embolism or

thrombosis.

• Athero-arteriosclerosis is the most common cause of

t h r o m b o s i s (approximately 30 % of strokes).
• Cerebral embolisation causes approximately 20 to 30 % of strokes.

1.3.2 Cerebral haemorrhages most frequently occur in the region of the internal
capsule.

• A subarachnoid bleed typically presents with a sudden onset of

severe headache in association with neck stiffness.
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• In contrast, the sudden onset of severe headache together with the

appearance of a focal neurological deficit (e.g., hemiparesis) and a
depression of the level of consciousness tends to point to a diagnosis of
an intracerebral haemorrhage.
• In hypertensive encephalopathy (and eclampsia) there is a
disturbance of the cerebral auto-regulation, which is exacerbated by
hypercarbia.
The Cushing reflex implies that increased intracranial pressure
causes a secondary increase in the mean arterial blood pressure,
possibly secondary to ischaemia of the brainstem. This represents
a compensatory mechanism to maintain the perfusion pressure of
the brain and is referred to as secondary reactive hypertension.
This compensatory mechanism should never be suppressed
with anti-hypertensive medication since this may cause
irreversible neurological deficit (with lowering of blood
pressure the ischaemic penumbra may be converted into an
area of infarction).

1.4 Which are the risk factors for stroke?

(Primary risk factors = before the stroke;
Secondary risk factors = after the occurrence of a stroke).

1.4.1 Hypertension
In stroke, hypertension is the single most important risk factor (Mitchell,
1983; Gautier, 1983). High blood pressure leads to the formation of
artherosclerosis and is associated with the development of lacunar
infarctions in particular and with cerebral bleeds. Hypertensive
encephalopathy is a clinical syndrome characterized by a high or rapidly
rising blood pressure in association with convulsions and/or focal
neurological deficit and confusion.
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1.4.2 Heart lesions

The heart is an important source of emboli to the brain and this is to be
considered particularly in the
event of neurological dysfunction
occurring in more than one
vascular territory.
• Atrial fibrillation is the most
important cause of cerebral
embolism.
• Atrial fibrillation and the
presence of valve lesions
(Coulshed 1970, Fleming 1971;
Barnett 1980; Turner 1965; Wood
1968; De Bnono 1983; Pruitt
1978) increase the risk of

cerebral embolism.
• Bacterial endocarditis may
have few obvious clinical signs and should always be considered if
there is the presence of an associated valvular lesion (Fleming,
1971).

1.4.3 Diabetes Mellitus

In terms of being a risk factor for vascular disease, diabetes has its
greatest impact on the development of peripheral vascular disease, with a
lesser effect on the cerebral vasculature and the least on the coronary
circulation (Kannel, 1983).

1.4.4 Alcoholism
Although there is probably a beneficial effect of moderate alcohol
consumption on the risk for myocardial infarction, there is a link between
the development of stroke in young adults and the abuse of alcohol. This
may be related to the development of cardiac arrhythmias, elevation of
the haematocrit or rebound thrombocytosis.
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1.4.5 The oral contraceptive pill

The pill, particularly if the estrogen content is high, is associated with a
higher incidence of cerebral infarctions (Bickerstaff, 1975). The infarctions
are often preceded by prolonged migraine auras or by transient ischaemic
attacks.

1.4.6 Haematocrit
An increase in the haematocrit leads to an increased risk of cerebral
infarction.

1.4.7 Smoking
Although smoking is a clear risk factor for the development of coronary
and peripheral vascular disease there is only a slight association with
stroke, particularly in males under the age of 65 years (Kannel 1983).

1.4.8 Hyperviscosity syndromes

Conditions like, for example, the antiphospholipid syndrome, low protein C
or S, high homocystein or fibrinogen level and anti thrombi III deficiency
may cause hyperviscosity.

1.4.9 HIV and Strokes (Bradley’s text)

Ischaemic and haemorrhagic stroke have been reported in up to 4% of
patients in clinical series. Associations with cerebral haemorrhage include
the following:
• Thrombocytopaenia;
• Coagulopathy associated with liver disease;
• Disseminated intravascular coagulation;
• Primary CNS lymphoma;
• Metastatic Kaposi’s sarcoma;
• and rarely with Toxoplasmosis.

Causes of HIV associated ischaemic stroke include:

• Bacterial endocarditis; Non bacterial thrombotic endocarditis;
21
• Vasculitis;
• Pro-coagulant states (anti-phospholipid antibodies; lupus
anticoagulant; deficiencies of protein C, protein S, heparin co-factor II,
anti-thrombin; and increased levels of Von Willebrand factor and D-
Dimers)
• Granulomatous angiitis of the nervous system
• Varizella zoster, TB meningitis and meningovascular Syphilis may
cause infectious vasculitis; as can the angio-invasive fungi Aspergillus
and Mucor.
• Aneurysmal disease of e.g., carotid arteries in adults and of the circle
of Willis in children are associated with HIV infection.
• Peripheral and cerebral venous disease (also of the cerebral venous
sinuses) occurs more frequently with HIV infections

Summary of cardiovascular risk factors (BMJ 2004;329:527)

• Nine in ten heart attacks can be predicted on the basis of nine risk factors
• The presence of the complete range of risk factors would increase the risk of heart
attack 335-fold. It is alleged that virtually all risk can be predicted
• Smoking (6-10 cigarettes/day doubles risk of heart attack; 20 cig/day increases risk
fourfold; 40 cig/day gives rise to a nine fold increase in risk of heart attack)
• Other risk factors: Hypertension; Diabetes Mellitus; Abdominal obesity (apple); Low
daily fruit and vegetable consumption; Lack of exercise; Stress; Abnormal ratio of
apolipoprotein A to apolipoprotein B (with smoking appeared to account for two thirds
of risk)

1.5 What are some of the complications of strokes?

1.5.1 Intracerebral complications

• Depression of the level of consciousness

and coma
• Permanent residual neurological deficits
e.g., hemiplegia or aphasia
• Transtentorial herniation with the
development of brain stem death (equals
brain death)
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1.5.2 Extracerebral complications:

• Dehydration
• Risk of fluid overloading intravenously with pulmonary oedema
• Pneumonia
• Urinary tract infections
• Bedsores

1.6 What are some of the prognostic factors in completed stroke?

Poor prognostic factors in stroke include:

• Depression of the level of consciousness

• Respiratory disturbances
• Pupillary changes
• Conjugate deviation of the eyes
• Dense motor weakness
• Loss of position sense in the limbs
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Chapter 2

1. SYNCOPE

1.1 Clinical types of syncope

1.1.1 Associated with decreased blood return to the heart
1.1.2 Associated with cardiac conditions
1.1.3 Associated with causes distal to the heart
1.2 The association of syncope with certain other clinical symptoms may
lead to the diagnosis
1.3 The distinction between syncope and epilepsy

2. EPILEPSY

2.1 Clinical types of epilepsy

2.1.1 Generalized epilepy
2.2 Classification of seizures
2.3 Classification of epilepsies (disease entities) and epilepsy syndromes
2.4 Non-epileptic seizures
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1. SYNCOPE

Definition of Syncope
p

Syncope is a transient and brief loss of consciousness, which is the result of a short-
lived decrease in cerebral blood flow.

The patient typically feels dizzy, experiences everything going grey to dark before his
eyes, becomes pale and falls, with loss of consciousness for seconds to
minutes an d then swiftly regains normal alertness.
Syncope Definition

1.1 Clinical Types of Syncope

The causes of syncope may be divided into three groups (Schrire, 1966):

1.1.1 Associated with decreased blood return to the heart

• Vasovagal attacks
• Orthostatic hypotension
• Oligaemia
• Apnea attacks after crying bouts in children

1.1.2 Associated with cardiac conditions

• Stokes-Adam attacks e.g., with third

degree heart block

• Aortic stenosis
• Tachyarhythmias, sick sinus syndrome,
lengthened Q-T interval syndrome and
other arrhythmias.
• Second degree heart block.

1.1.3 Associated with causes distal to the heart:

• Carotid sinus syncope
• Vertebro-basilar ischaemic incidents
• Anaemia
• Transient hypoxia
• Hypoglycaemia.
25

1.2 The Association of syncope with certain other clinical symptoms may lead to
the diagnosis:

- Syncope which develops with or after exercise is suggestive of aortic

stenosis, pulmonary hypertension and lengthened Q – T interval syndrome.

- Syncope which chiefly commences with standing or sitting may be a pointer

towards the underlying presence of orthostatic hypotension.

- Syncope which occurs independent of body posture, may favour the diagnosis
of a cardiac cause (brady- or tachyarrhythmia), hypoglycaemia or a panic
disorder.

- Immediate onset of syncope is suggestive of postural hypotension,

arrhythmias and carotid sinus syncope.

- If the attack occurs more gradually over minutes, the possibilities of panic
disorder and hypoglycaemia arise.

- The following symptoms associated with syncope may point to the underlying
presence of a panic disorder:
- development of a feeling of warmth;
- sweating;
- dizziness;
- shortness of breath and tachypnoea;
- palpitations;
- chest pain;
- a feeling of pins and needles over the hands and arms and around the
mouth;
- cramping of the fingers in extension (carpopedal spasm)
- and a feeling of fear or dread or unreality

- Syncope associated with palpitations may be associated with a panic

disorder, tachyarrhythmias and hypoglycaemia.
26

1.3 The distinction between syncope and epilepsy:

SYNCOPE EPILEPSY

Pathogenesis is related to a decrease of Associated with a diffuse electrical

cerebral blood supply discharge of the brain

The onset may not be as sudden as Onset is usually sudden

epilepsy

Frequently in upright position Patient may assume any position

Injuries are less frequent Patients may injure themselves

Recovery of consciousness usually takes Period of loss of consciousness tends to

place within seconds or minutes last longer

Usually not followed by prominent Frequently followed by confusion

confusion

Sphincter control is usually maintained Sphincter control may be lost

May be followed by myoclonic twitches or Often associated with involuntary

a grand mal seizure movements (tonic-clonic)

- syncopal convulsions may be found with, e.g.,

o cough-syncope;
o Stokes-Adams attacks (brady-arrhythmias);
o breathholding spells;
o and for that matter after any type of syncopy (the onset of the event assumes
a critical importance in the differentiation).
 27

2. EPILEPSY

Definition of Epilepsy (seizures)

Epilepsy is a paroxysmal electrical disturbance of cerebral neurons which may

give rise to dysfunction of the:

1. Motor system : Tonic attacks, tonic-clonic attacks, myoclonic or atonic attacks;

disturbance of the maintenance of posture with hypotonia and the occurrence of

automatisms.
2. Sensory system: Visual-, auditory-, olfactory-, taste- or somatosensory auras.
3. Behaviour: feeling of anxiety, fear or exceptionally, a feeling of elation.
4. Consciousness
• with loss of consciousness for a few seconds e.g., in petit mal or
• for some 10 – 45 minutes following upon a grand mal seizure
[Link] function blood pressure or pupillary changes with, eg,
pupillary changes. facial pallor, tachycardia, high blood pressure

2.1 Clinical types of epilepsy (Bancaud, 1981)

2.1.1 Generalized epilepsy

Definition of generalized epilepsy

Generalized epilepsy characteristically has no aura or warning prior to the attack.

e
The abnormal electrical impulses probably have their origin in the rostral brainstem
h
- with the subsequent spread to the diencephalon or both cerebral hemispheres and
- with involvement of the motor systems of the brainstem and spinal cord. m
- Often, an inherited decrease in the threshold for the development of seizures is present.
- Both petit mal (absences) and grand mal (tonic-clonic attacks) are
p
- examples of generalized epilepsy.
• Petit mal is frequently accompanied by a 3/second spike and wave activity on EEG
• Generalized epilepsy characteristically has no aura or warning prior to the attack.
-
- . Generalized epilepsy definition
2.1.2 a) Definition of petit mal or absence seizures
Petit mal attacks show the following characteristics (Penry 1975)

Sudden onset
- Vacant stare with disturbance of
consciousness
- Disruption of ong ing psychic activity

- Short duration – less than 10 seconds in

85 % of attacks; usually lasts less than
30 seconds.
- Eyes rotate upwards
- Normal alertness when consciousness is
regained immediately after the attack.
- Are frequently accompanied by a 3/second spike and wave activity on EEG.
With video recordings clonic components are sometimes observed during a prolonged
petit mal attack and automatisms and autonomic components may also appear.
Absence attack/Petit mal definition
28
(b) DDefinition of grand mal or tonic-clonic seizures
The grand mal seizure may be characterized by the following phases:

(i) Immediate loss of consciousness

(ii) The tonic phase, during which the patient falls to the ground, with stiffening of
the entire body, frequently in a posture of extension of the li bs; as a result of
rapid expulsion of air through the larynx, vocalization may ccur. This phase
lasts about 30 seconds and is sometimes associated with uri ary incontinence.
The EEG tends to show a series of spike discharges.
(iii) The clonic phase where rapid, rhythmic and m
o
symmetrical myoclonic jerks are observed. n
This phase lasts approximately 30 seconds.

The EEG shows a spike and slow wave

pattern: The spike corresponds to the muscle
jerk component and the slow wave (inhibitory
wave) to themrelaxation of muscle tone between
the myoclonic jerks.
(iv) Period of muscle relaxation during which
e
respiration gradually returns. This lasts about
30 seconds.
Confusion: After arousal the patient may be confused for a varying period
of time. The patient may also show automatisms.
(v) As consciousness is regained, the patient often complains of headache, muscle
h
pains and a feeling of exhaustion (postictal period). Occasionally, signs of
injury or biting of the tongue or cheek or urinary incontinence are found.
Grand mal seizure definition
(c) Definition of Myoclonic seizures
Myoclonus is a sudden, brief, shock-like involuntary jerking movement of muscles,
usually of sufficient force to induce a muscle movement around a joint.

- Symmetrical my oclonic jerks are present in some 10 to 50 % of patients with

idiopathic epilepsy, often as a prodrome prior to the onset of a Grand Mal seizure.
- Myoclonic jerks are characteristic of Juvenile Myoclonic Epilepsy (of Janz).
- Myoclonic jerks of the trunk with flexion and extension movements are seen in
Infantile Myoclonic Epilepsy (West-syndrome)
- Myoclonic jerks are also seen following hypoxia, in liver and renal failure.
- d
Myoclonus jerks also occur with slow virus infections, such as Subacute Sclerosing
Panencephalitis (SSPE) caused by measles, or Creutzfel t-Jacob disease

Myoclonus definition
(d) Definition of drop attacks or atonic attacks

In drop attacks, thew patient falls to the ground suddenly, either without loss of
consciousness, or with loss of consciousness of only a few seconds duration.
m
The causes of drop attacks include:
1. Transient ischae mic attacks in the vertebro-basilar territory

2. Myoclonic astatic epilepsy

3. Medio-orbito-frontal epilepsy
4. Sudden increase in intracranial pressure, e.g., with a colloid cyst of the third

ventricle or a parasitic cyst of the fourth ventricle. Typically, these attacks are
associated with sudden onset of headache (hydrocephalus) and weakness of the
legs.
5. Cataplexy in the sleep disorder narcolepsy
Drop attacks definition
29 (e) Definition of tonic seizures
In tonic seizures there is an increase in tone in the arms, legs and trunk, often resulting
in extension of the whole body.

Tonic seizures are c hharacteristically found in the Lennox-Gastaut syndrome. This is a

condition which presents between the age of two and sixteen years and has the
following features:
1. absence seizures
2. myoclonic jerks
3. drop attacks
4. tonic seizures ando
5. Intellectual deteri oration
. Tonic seizure definition

Definition of partial seizures

In partial seizures there is characteristically the presence of an aura or a warning at the

onset of a seizure and the initial clinical symptom, sign or electrical discharge all point
to the presence of a focal origin of the ictus from one of the cerebral hemispheres.

Partial epilepsy definition

The localized abnormal discharge A secondary subcortical discharge in

first activates the local neurons turn initiates the spread of the

and gives rise to an aura. The electrical discharge to both

subcortical structures are hemispheres with secondary

sometimes secondarily activated. generalization.

a Definition of simple partial seizures

Simple partial seizures are characterized by an aura without disturbance of

consciousness. The aura may be a motor-, somatosensory-, évisual-, auditory-,

olfactory-, gustatory-,c autonomic- or a psychic phenomenon (e.g., d ejà vu, jamais vu,

depersonalization) .Simple partial seizure definition

Definition of complex partial seizures

- This is a partial seizure with disturbance of consciousness

- Partial complex seizures may be preceded by an aura
- or may be characterized de novo by a disturbance of

a
consciousness.
- The patient may react, but is usually unable to answer
questions, appears to be confused, in a twilight phase

and “not all there”.

- The patient is subsequently unable to recall events or give
an account of what happened.
30

Definition of an Aura
- The aura is that part of the attack, which is noticed by the patient at the onset
of a seizure before the loss or disturbance of consciousness occurs.
- During the aura, the patient is still able to register and remember information.
At times the attack or electrical discharge is limited to the aura without any
further events (simple partial convulsion).
- The presence of an aura is a pointer to the presence of focal or partial
epilepsy.
- When an aura precedes a grand mal attack, it is a cardinal characteristic of
partial epilepsy.
- Somatosensory (sensory cortex) and olfactory (medial temporal lobe) auras
are frequently associated with underlying structural abnormalities.
- Formed visual hallucinations are usually of lateral temporal lobe origin. The
most common aura of temporal lobe (insular) origin is a feeling of epigastric
discomfort, which rises up to the neck.
Aura definition
Definition of Automatisms
- Automatisms comprise the following:
* a series of involuntary, fairly well organized movements,
* which are carried out during or immediately after an epileptic seizure and
* are associated with an alteration in the state of consciousness or a “twilight”
state
* and with amnesia for the events during which the behaviour occurred

- They are a characteristic feature of complex partial or temporal lobe epilepsy

and may occur after an epileptic seizure, for example, a grand mal seizure.
Automatisms probably are indicative of spread of the epileptic discharge to
both limbic lobes.

- Automatisms may assume a number of different forms:

1. Chewing and swallowing movements (likely of amygdaloid nucleus origin)
2. Walking around in no particular direction
3. Verbalization, simple and automatic
4. Gesturing, commonly picking at objects with the hands in an aimless way
5. Stereotyped movements, for example tying and untying shoes or rewriting the
same sentence several times.
Automatism definition
Grand mal status epilepticus (Delgado-Escueta 1982, 1983)

Grand mal or tonic clonic status epilepsy may be defined as:

1. Continuous tonic clonic seizure activity for a period > 30 minutes
2. The occurrence of two or more sequential tonic clonic seizures without full
recovery of consciousness between seizures
3. Frequent occurrence of tonic clonic seizures without clinical recovery for a
period exceeding 20 minutes

Morris and Bourgeois suggested treatment for status epilepsy with the occurrence of
two to three generalized tonic clonic seizures within a 24 hour period
(remember the oral route of AED [anti epileptic drug] loading)

Lowenstein and Alldredge advocate an operational definition:

1. Continuous seizures lasting at least 5 minutes
2. Two or more discrete seizures between which there is incomplete recovery of
consciousness.
Grand mal status epilepsy
31

Status epilepticus is a medical emergency since it results in local consumptive

(cerebral hemisphere) and systemic hypoxia. The longer the status epilepsy
continues the more refractory it becomes to treatment.

Other forms of status epilepticus

- Petit mal - and psychomotor status epilepsy are uncommon and are
suspected when a patient who is known to have epilepsy presents with a
depression of level of consciousness or with a mentally confused state.
- Continuous focal motor seizures (of e.g., the face, the tongue, a hand, an arm
or the leg) are also known as epilepsia partialis continuans.
- The presence of subtle status epilepsy is suspected when a patient with a
preceding history of seizures (usually; but occasionally de nova) remains
confused or depressed in level of consciousness and may show one of the
following:

1. Twitching movements of the eyelids

2. Localised jerking of the tongue muscles
3. Facial muscle twitching or myoclonic jerks
4. Nystagmoid eye movements to one side (observed with the eyelids held open;
with states of unconsciousness the phasic saccadic components of
nystagmus usually disappear)
5. Localised jerking of the fingers, toes or any other motor component.

- The EEG may show underlying generalized epileptiform discharges

accounting for the depression of level of consciousness or confusion with a
so-called “electro-mechanical (muscle) dissociation” between the cerebral
cortex discharges and the peripheral motor system.
- Subtle status epilepsy may account for some 20% of patients with status
epilepsy (some 8% of patients with delirium for which no other cause could
be found) and a high index of suspicion remains of paramount importance as
the lack of its recognition leads to a very serious patient morbidity and
mortality.
32

2.2 Classification of seizures: 1981 (Please refer to section 2.4 for new 2017
classification of seizures)
“Commission on classification and terminology of the International league against epilepsy” (Bancaud
1981).

1. Partial or focal
a) Simple partial seizures
• With motor signs
• With somatosensory and special sensory symptoms
• With autonomic symptoms and signs
• With psychic symptomatology
b) Complex partial seizures
 Simple partial onset followed by
impairment of consciousness
 With impairment of consciousness from the onset of
the seizures
c) Simple partial seizures with secondary generalization
towards a generalized tonic-clonic seizure

[Link] seizures (convulsive or non)

• Absence- typical/atypical
• Myoclonic
• Clonic
• Tonic
• Tonic-clonic
• Atonic

3. Other
Unclassified and recurrent convulsions in different circumstances.
Epilepsy seizures classification

2.3 Classification of epilepsies (disease entities)

and epilepsy syndromes:
(“Commission on classification and terminology of the International League against Epilepsy” 1989).

1. Localized, focal, partial epilepsy or syndromes:

a) Idiopathic with age-related onset
- Benign childhood convulsions with centro-temporal spikes
- Primary reading epilepsy.
b) Symptomatic
- Chronic progressive epilepsia partialis continuans
- Syndromes of temporal, frontal, parietal, occipital lobe onset

2. Generalized epilepsy or syndromes

a) Idiopathic with age related onset
Benign neonatal familial convulsions
- Benign myoclonic epilepsy of the newborn
- Childhood absence epilepsy
Juvenile absence epilepsy
- Juvenile myoclonic epilepsy
- Epilepsy with Grand mal seizures on awakening
- Generalized idiopathic epilepsy not defined above
- Epilepsy with seizures precipitated by specific modes of activation/precipitated (reflex provoked)
epilepsy.
 33 b) Cryptogenic or symptomatic

- West’s syndrome
- Lennox-Gastaut syndrome
- Epilepsy with myoclonic-astatic seizures
3. Other
- Situation related seizures
- Febrile convulsions
- Seizures associated with acute metabolic or toxic events
- Convulsions in neonates
- Acquired epileptic aphasia
- Cryptogenic group
Epilepsy and syndromes classification

2.3 Non-Epileptic Seizures (Pseudo seizures)

(Psychogenic seizures) (Boon 1993).

- Features which raise suspicions regarding the possible presence of non-

epileptic seizures include the following:

1. Prolonged length of actual seizure

2. Lack of stereotyped nature of the seizures;
3. Asynchronous movements of the extremities
4. Atypical vocalization
5. Alternating rocking type movements of the head and pelvis
6. The occurrence of frequent seizures of recent onset despite the use of
adequate doses and numbers of AED’s (anti epileptic drugs).

- Patients may experience a combination of both genuine and non-epileptic

seizures.

- Keep in mind that patients who have complex partial seizures (mostly
of frontal lobe origin) may have an atypical appearance to their
seizures.

- The final diagnosis of non-epileptic seizures requires the c a r e f u l

integration of all available information with a meticulous and repeated
scrutiny of the events.

- The EEG (may show slowing postictally), and prolactin levels (elevated
after a true seizure), may be of assistance.

- Video recordings of the clinical seizures and EEG simultaneously (in a

tertiary epilepsy unit) may sometimes be the only way to differentiate
between real and non-epileptic seizures.

- Home video or cell phone recordings (of the ictal events) may prove to
be valuable.
33 A 2.4 International League Against Epilepsy new classification

Epilepsia, 58(4):522–530, 2017

doi: 10.1111/epi.13670
34
Chapter 3

1. SPINAL CORD DYSFUNCTION

1.1 A spinal cord compression syndrome is a neurological
emergency
1.2 Anatomy of the spinal cord
1.3 Approach to a spinal cord lesion
1.4 What is the functional deficit that the patient may show on history and
examination
1.5 Features of an extra-medullary lesion
1.6 The presence of an extra-medullary but intra-dural lesion

1.7 Features favouring the presence of a central cord disease or an intra-

medullary lesion
1.8 Anatomical areas of involvement of the spinal cord itself
1.8.1 A transverse lesion of the spinal cord
1.8.2 Bown sequard or hemisection of the spinal cord
1.8.3 Causes of the central cord syndrome
1.8.4 Causes of the postero lateral spinal cord syndrome involvement
1.8.5 Anterior lateral spinal cord syndrome (typically of vascular origin)
1.9 Temporal profile of the onset of a spinal cord lesion
1.10 Aspects on several other conditions that may affect the spinal cord
1.10.1 The presence of an epidural abscess
1.10.2 Human lymphotropic virus type 1 (HTLV1) associated myelopathy or tropical
spastic paraparesis
1.10.3 Atlanto axial dislocation with rheumatoid arthritis
1.10.4 Conus medullaris lesions
1.10.5 Cauda equina lesions
35
 SPINAL CORD DYSFUNCTIONS (See also Section 3 in Chapter 8)

1.1 A spinal cord compression syndrome is a neurological

emergency:

The spinal cord has a diameter of approximately 1cm and the spinal canal
of 1.5cm. One should entertain a high index of suspicion for the possible
underlying presence of spinal cord compression syndrome which may
necessitate emergency decompression.

The following signs and symptoms could alert one to the possible presence of an
underlying spinal cord compression syndrome:

• The presence of weakness in the legs especially if it is upper motor

neuron type.
• The history of an associated sphincter disturbance with urinary or
faecal incontinence.
• The presence of severe backache (possibly associated with tenderness
over the back) and of fever, should alert one to the possible underlying
presence of an epidural abscess which constitutes an emergency.
• The presence of a sensory disturbance over the leg(s) up to the level of
the trunk or higher.

If one looses the critical time frame of management, one may have a patient that is
left with a permanent spinal cord problem; once a critical pressure on the spinal
cord is reached, a secondary vascular infarction of the spinal cord may occur.

A spinal cord syndrome represents an emergency situation!

36

1.2 Anatomy of the spinal cord:

The spinal cord consists of 31 segments;

• 8 cervical;
• 12 thoracic;
• 5 lumbar;
• 5 sacral and
• 1 coccygeal segment.

The spinal cord fills the upper 2/3 of the

spinal canal and it ends at the level of the
lower edge of the first lumbar vertebra (L1).
At the cervical area the spinal segments lie
approximately 1 vertebral level higher; in
the upper thoracic area, the spinal cord
segments lie approximately 2 vertebral
levels higher and in the mid-thoracic region
3 vertebral levels higher.
All the lumbar sacral segments lie against
3 vertebrae namely: T11, T12 and L1
vertebrae.
The L3 to S2 segments form the epiconus and the S3 to Coccygeal 1 the
conus medullaris.

 The blood supply of the spinal cord consists mainly of the anterior spinal
artery which is supplemented by 2 posterior spinal arteries.

 The anterior spinal artery supplies the anterior and lateral parts of the
spinal cord, and this consists of the spinothalamic tracts (anterior tracts) and
the cortical spinal tracts (lateral tracts).
37
The paired posterior spinal arteries supply the posterior part of the spinal
cord, the area which contains the posterior columns.
 The cervical spinal cord is supplied by an anterior spinal artery which derived
from both vertebral arteries above the foramen magnum and is
supplemented by a branch from the costo-cervical trunk at the C 7 level.
 The large spinal intramedullary artery which originates at the T 11 or T 12
level is also known as the artery of Adamkiewicz.
 The midthoracic area forms part of the watershed area of the spinal cord and
is more susceptible to pathological processes.

1.3 Approach to a spinal cord lesion:

The following questions may be answered:

i) What is the functional deficit on history and examination?
ii) What is the anatomical localization of the lesion in terms of the rostral-
caudal extent?
iii) What is the spinal cord segment/vertebral level of the lesion?
iv) Is the lesion extra- or intra-medullary (within or outside the spinal cord)?
v) What is the clinical picture with regards to involvement of the spinal cord in
its axial (transverse) plane?
38
a) Is it a transverse lesion of the whole spinal cord, involving all the tracts
of the spinal cord, as may be found with the so-called condition of
transverse myelitis?
b) Is it a central cord syndrome (involvement of the centre of the cord as
with syringomyelia or an intrinsic spinal cord tumour)?
c) Is it a hemi-spinal cord syndrome (a left or a right half of the cord
involved) as in the so-called Brown-Sequard syndrome?
d) Is it an antero-lateral spinal cord (as with involvement of the
spinothalamic- and corticospinal tracts) syndrome?
e) Is it a postero-lateral spinal cord (as with involvement of the dorsal
columns and the corticospinal tracts) syndrome?

vi) What is the pathology of the spinal cord lesion?

vii) What is the etiology (cause) of the lesion?

1.4 What is the functional deficit that the patient may show on history and
examination?

The neurological history and examination determine the functional deficits

that are found.
i) One looks specifically for the presence of local backache and back
tenderness. The back is examined looking for the possible presence of a
kyphosis (a sharp angulation of the vertebral column in the antero- posterior
plane; which suggests the underlying presence of a vertebral body collapse,
with its associated vertebral body disease), a step (where one spinous
process lies at a deeper level than the other one (also suggesting the
presence of vertebral body disease); The spinous processes are palpated
serially for the presence of tenderness.

ii) The presence of backache, back tenderness, a kyphosis, “a step” and a

history of neuralgic pains (sharp shooting, stabbing and electric pains in e.g.,
an intercostal nerve distribution) would suggest that there is the presence of
vertebral body disease such as may be found with, e.g Tuberculosis (TB of
the vertebral body is known as Pott’s disease), Myeloma (primary tumour of
bone) or Metastases.
 39

iii) The presence of sphincter incontinence (urinary or faecal) is noted.

Urinary incontinence, e.g., in combination with weakness of the legs, signifies
the presence of an emergency situation; the diagnosis and management
require urgency in an attempt to try and salvage sphincter function and to try
and improve neurological deficit. If the sphincter disturbance lasts longer than
24 –48 hours, it tends to become irreversible.

iv) With the motor examination the motor level of spinal cord involvement is
assessed:

a. Should the weakness stretch up to the level of hip flexion, one would think of
a motor level of L1, L2 (the iliopsoas muscle is supplied by the L1 and L2
myotomes).
b. Should the motor level move up to the level of e.g., the umbilicus (on sitting up
the umbilicus may move upwards, indicating weakness of the rectus abdominus
muscles below the level of the umbilicus, the so-called Beevor sign) the level
would then be at T10.
c. Should the patient be unable to sit up, one may expect the weakness
level to at least be up to the T8 level.
d. Should the abductor muscles of the fingers be affected, one would localise
the level at T1 level.
e. Weakness of triceps muscle would localise the lesion at C7, while
f. Weakness of the deltoid may localise it at the C5 level.
g. Weakness of the diaphragm would localise the lesion at C2,3,4
(cervical plexus level).
 40

v) One looks for the presence of a possible “ reflex level”:

a. If the deep tendon reflexes of e.g., the knee are lively (3+ to 4+[clonus]),
the level will be above L3;

b. If the lower abdominal reflexes are absent, the level may be at T11, T12; if
the upper abdominal reflexes are absent, the level may be at T8, T9;

c. If the triceps reflex is 3-4+ the level of pathological involvement would lie above
C7. If the triceps reflex is absent (in the cortex of a spinal cord lesion), it may
suggest the presence of a lower motor neuron lesion at C7, and point to the
level of spinal cord involvement at C7.

d. The presence of a so-called “inverted biceps reflex” (i.e., an absent biceps reflex
[lower motor neuron involvement at C5] with concomitant contraction of the
finger flexors [upper motor neuron involvement at C8]) would suggest that the
level would be at C5.

e. On examination of the anal reflex, the anal reflex may be absent and may show
hypotonia and weakness of the anal sphincter.
41

vi) Looking for the presence of a sensory level:

The presence of a sensory level

(i.e., loss of light touch or pinprick
sensation below a certain
dermatomal level) forms a
characteristic hallmark of a spinal
cord lesion.

 The sensory level may be up to the

level of e.g., the groin T12/L1;

 up to the level of the umbilicus at

T10;

 up to the level of the xiphisternum at T8:

 or involving the hand with the level at

C8.

 One may also find a position sense

level starting with the toes and moving
up towards the ankles, the knees and
the hips.

 It is useful to remember that the C4

dermatomes are contiguous over the
upper chest with T2; the thumb,
the middle finger and the fifth finger are innervated by C6, C7 and C8
respectively. Over the posterior axial line of the leg (medial thigh) the
lumbar and sacral dermatomes are contiguous.
42

1.5 Features of an extra-medullary/ extradural lesion:

The following may be pointers to the possible presence of vertebral body or

extradural disease:

 presence of backache,

 neuralgic pains (sharp shooting pains in the

distribution of e.g., an intercostal nerve),

 vertebral tenderness,

 and the presence of a vertebral spinous process step or

of a kyphosis

The presence of a Brown Sequard type of syndrome (hemi-spinal cord

syndrome) may favour the presence of an extra-medullary lesion.

Examples of an extra-medullary lesion include:

• Tuberculosis of the vertebral body;

• myeloma of the vertebral body;

• metastases of the vertebral body.

1.6 The presence of an extra-medullary but intra-dural lesion:

Two benign conditions presenting with a spinal cord compression syndrome

include e.g., a meningioma and a neurofibroma. Both occur typically in the
extra-medullary (outside the spinal cord substance) and intradurally. One
would always like to rule out these causes as they are eminently treatable and
curable. It would be reasonable to consider a diagnosis of a possible
meningioma or neurofibroma in the patient presenting with a spinal cord
problem, until proven otherwise.
43

1.7 Features favouring the presence of a central cord disease or an intra-

medullary lesion:

 The presence of a suspended sensory level over several

dermatomes for example, a decreased pin prick and light touch
sensation from C5 to T2 on the one side or on both sides. One
may also, for example find a suspended sensory level at the
thoracic level, e.g., between T8 and T12.
 The presence of a dissociated sensory deficit in which one may
have a decreased pain and temperature sensation and relative
sparing of light touch over these suspended areas of sensory
dysfunction.
 The presence of sacral sensory sparing.
 The sphincters tend to be affected early with the presence of
central cord disease.
 There is a tendency to present with symmetrical deficits.
 If the lesion is at the cervical level, for example with the presence
of a syringomyelia , one may find suppressed or absent deep
tendon reflexes in the arms, and one may find an associated
lower motor neuron involvement due to anterior horn cell
involvement in deficits at e.g., the C8 to T1 myotomes.

1.8 Anatomical areas of involvement of the spinal cord itself:

1.8.1 A transverse lesion of the spinal cord:

A transverse lesion of the spinal cord or so-called transverse myelitis is often

an acute condition that develops over a day or several days. The transverse
lesion of the spinal cord may be demyelinating in nature. It may also result
from other pathology of the spinal cord which causes compression of the cord
with secondary infarction.

Causes of transverse spinal cord involvement:

• Post infectious, after exanthemous infection or other infections.
• Multiple sclerosis; ADEM (acute disseminated encephalo-myelitis);
Devic’s disease (neuromyelitis optica);
44

• Late signs of the spinal cord compression syndrome.

• Late phase of radiation therapy myelopathy.
• Idiopathic
• Possibly vascular involvement of the spinal cord

1.8.2 Brown Sequard or hemisection of the spinal cord:

Clinically the patient will show the following:
 ipsilateral upper motor neuron signs;
 ipsilateral dorsal column deficit and
 contra-lateral spinothalamic sensory loss up to the
level of the lesion.

Trauma of half of the spinal cord; tumours that compress

the spinal cord e.g., meningioma or neurofibroma are
some of the more common causes.

1.8.3 Causes of the central cord syndrome:

 Syringomyelia associated with an Arnold Chiari malformation. With an
Arnold Chiari malformation,
one has varying degrees of
herniation of the medulla
oblongata and the
cerebellar tonsils through
the foramen magnum. With
an Arnold Chiari
malformation one may find one of the following:
o an associated syringomyelia;

o an associated hydrocephalus;

o upper motor neuron signs in arms, legs

o posterior column signs in the arms;

o lower cranial nerve deficits

o cerebellar signs and down beating nystagmus

especially on lateral gaze. (Daroff sign)
 45

 Spinal cord tumours such as astrocytomas may present with a central cord
syndrome.
 Post traumatically one may find a post traumatic syringomyelia dissecting
upwards with a rostral extension of neurological signs above the original level
of the spinal cord trauma.
 Post traumatic after, for example, a hematomyelia.
 Occasionally a central cord syndrome may be found with a severe cervical
spinal stenosis picture or with an area of extensive demyelination of the
cord itself.

1.8.4 Causes of the postero lateral spinal cord syndrome involvement:

i) Vitamin B12 deficiency with pernicious anaemia. It is also called a
“postero- lateral sclerosis” of the spinal cord. There may be a degree of
a sensory peripheral neuropathy on top of the spinal cord picture. It is
possible to have a Vit. B12 spinal cord involvement without the
haematological complications.

ii) Friedreich’s Ataxia, which represents a hereditary spino- cerebellar

degenerative condition, may cause
o Dorsal column sensory disturbance in the legs
o Extensor plantar responses
o Ankle reflexes typically absent
o Pes cavus and scoliosis
o Cerebellar signs in the arms
o Cerebellar dysarthria and
o Gaze evoked nystagmus.

The diagnosis of Friedreich’s Ataxia may be made by molecular biology

techniques and testing the expansion of a repeated trinucleotide repeat
(GAA) sequence.
46

iii) HIV associated Vacuolar Myelopathy: Vacuolar myelopathy is the most

comman cause of spinal cord dysfunction in patients with AIDS, affecting
up to 25-55% pathologically in an AIDS autopsy series. Vacuolar
myelopathy complicates late HIV infection and frequently co-exists with
HIV associated dementia and distal sensory peripheral neuropathy. The
cortico spinal tracts and dorsal columns are affected and this may also
be accompanied by sphincter involvement. On MRI there may be
atrophy of the spinal cord.

iv) Posterior spinal cord tumours

v) Tabes dorsalis, a parenchymatous form of neurosyphilis, which may

involve the dorsal columns and be associated with severe shooting

or lightning type of pains and cortico spinal tract involvement may also
be found. A combination of Tabes Dorsalis and General Paresis of the
Insane (parynchymatous brain involvement causing corticospinal tract
signs) or the so-called Tabo- Paresis may be found.

Causes of absent ankle reflexes associated with bilateral extensor

plantar responses:
Vitamin B12 deficiency with postero-lateral sclerosis of the spinal cord
Friedreichs Ataxia
Tabo-paresis
Multiple level cervical and lumbar disc disease (mixed upper and lower motor neuron signs)

1.8.5 Anterior lateral spinal cord syndrome (typically of vascular origin):

The clinical picture is that of upper motor neuron

signs associated with a spinothalamic sensory level
below the level of the lesion. The classical example is
that of the anterior spinal artery thrombosis syndrome
which is usually associated with an acute onset
picture, sphincter involvement and it may be
associated with sacral sensory sparing.
47

Causes of an anterior spinal artery thrombosis include:

(1) Secondary to meningitis for example: pneumococcal meningitis;

cryptococcal meningitis; tuberculous meningitis or meningovascular
syphilis.
(2) After abdominal artery aneurysm surgery during which the
abdominal aorta may be clamped off for a while, one may find a
spinal cord vascular syndrome (with the underlying presence of
widespread atherosclerosis of especially the abdominal aorta).

Causes of “mainly cortico-spinal tract involvement” in the legs and

arms:

One may frequently find a clinical picture which is dominated by the presence of
spasticity or cortico-spinal tract signs in the legs and arms. The causes for this
clinical picture include the following:

 Cervical spinal stenosis: this may be associated with

underlying osteoarthritis of the cervical vertebrae; there
may be the presence of associated root signs but the
dominant picture may be that of long tract signs with
spasticity. The bladder function tends to remain intact until
late.
 The presence of underlying Multiple Sclerosis. The
primary progressive type of multiple sclerosis and the
secondary progressive type tend to affect the legs with
increasing spasticity. One may find a relapse of the
relapsing remitting type of Multiple Sclerosis affecting the
spinal cord.
 One would have to exclude the underlying
presence of a meningioma or neurofibroma.
 Hereditary forms of spastic paresis.
 Presence of vitamin deficiencies such as
Vit B12 and nicotinamide[niacin]
(Pellagra).
 Presence of Arnold Chiari malformation.
48
 Mantakassa seen in Mozambique; from 1981 to 1984 there
was an epidemic in Mozambique of a spastic paraparesis
syndrome which was associated with the intake of the green
cassava (type of wild spinach) root which appeared to have
a higher concentration of cyanide.
 HTLV 1 infection associated myelopathy (tropical
spastic paraparesis).

1.9 Temporal profile of the onset of a spinal cord lesion

• Gradual onset of neurological dysfunction will suggest the presence of an

underlying space occupying lesion or HTLV1 or HIV spinal cord involvement.
• An acute onset spinal cord syndrome will make one think of the possible
underlying presence of ADEM (acute disseminated encephalo myelitis) or an
anterior spinal artery occlusion syndrome.

1.10 Aspects on several other conditions that may affect the spinal cord

1.10.1 The presence of an epidural abscess:

The presence of backache together with fever would alert one to the
possibility of the possible presence of an underlying epidural abscess which
constitutes an emergency. The patient may have associated neck stiffness or
meningism. One requires a high index of suspicion for the possible presence
of this condition.

1.10.2 Human lymphotropic virus type 1 (HTLV1) associated myelopathy or

tropical spastic paraparesis:
HTLV 1 myelopathy is also known as tropical spastic paraparesis or HAM
(HTLV 1 associated mylopathy). It occurs endemically in the Caribbean, south
western Japan, equatorial Africa and South Africa and parts of central and
South America.
HTLV 1 associated mylopathy is gradually progressive and
49

• usually begins after the age of 30 years and presents with a

• progressive spastic paraparesis.
• The patient may complain of lower extremity paraesthesiae,
• a painful sensory neuropathy
• and bladder dysfunction.
• There may rarely be the development of optic atrophy or a spino-
cerebellar ataxia.

The definitive diagnosis is made with positive serology in blood and CSF. If
the clinical picture is typical and other causes have been excluded, the
presence of positive blood serology for HTLV 1 infection would probably
suffice for such a diagnosis. No anti-viral agent has been effective up to date
but there is some evidence that plasmaphoresis may have a beneficial effect.

Prevalence and transmission of HTLV 1 infection in Natal/KwaZulu:

The sero-prevalence of HTLV 1 was 2,6% in Ngwelezane district.
Up to December 1991, 90 cases of HTLV 1 associated mylopathy or tropical spastic
paraparesis were seen at the Neurology Unit at the Wentworth Hospital.

Spectrum of causes of myelopathy in HIV sero-positive South African patients:

The cause of myelopathy was assessed in 33 HIV sero-positive individuals in KwaZulu
Natal.
The main associations were:

i. HTLV 1 infection in 12 subjects (36%)

ii. Tuberculosis in 6 subjects (18%)

iii. Zoster Myelitis in 3 subjects (9%)

iv. Herpes simplex infections 2 subjects (6%)

v. Syphilis 2 subjects (6%)

vi. Bilharzia 2 subjects (6%)

50

1.10.3 Atlanto axial dislocation with rheumatoid arthritis:

Atlanto axial dislocation may give rise to neck pain and bilateral corticospinal
tract signs and one should have a high index of suspicion for the development
of this serious complication.

1.10.4 Conus medullaris lesions:

The following would point to a conus medullaris lesion:

 The development of a symmetrical loss of saddle segment (S1-S5)

sensation;
 absent ankle reflexes;
 weakness of plantar flexion at the
ankles (sometimes with
dorsiflexion weakness at the
ankles);
 urinary and faecal incontinence.
Causes could include: Bilharzia
infection; ependimoma;

demyelinating lesion as with

ADEM or Multiple Sclerosis.

1.10.5 Cauda equina lesions:

The most serious complication of lumbar disc disease (fortunately
uncommon,<3% of cases) is the development of a large central disc herniation
with neurological symptoms of sciatica often in both legs and the compression
of the cauda equina with bilateral e.g., S1-S5 root compression
and sphincter loss. This clearly constitutes an emergency. Cauda equina
lesions tend to be asymmetrical and painful.
51

Chapter 4

1. MULTIPLE SCLEROSIS
1.1 Common clinical features of Multiple Sclerosis
1.2 Clinical features NOT suggestive of Multiple Sclerosis
1.3 Considerations when evaluating a patient
1.4 Diagnostic criteria for the diagnosis of Multiple Sclerosis
1.5 MRI demonstration of space dissemination from McDonald et al. (2001)
diagnostic criteria of Multiple Sclerosis
1.6 Clinical course of Multiple Sclerosis

2. ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM)

3. CENTRAL PONTINE MYELINOLYSIS

52

1. MULTIPLE SCLEROSIS
• A demyelinating disorder of the white matter of the central nervous
system
• uncertain origin
• symptoms and signs (usually two or more), occur sub-acutely (over
hours or days)
• disseminated in terms of anatomical distribution of
involvement and also in terms of temporal or time profile.
• Usually relapsing
• Often eventually progressive

Multiple Sclerosis typically begins in early adulthood and has a variable

prognosis. Fifty percent of patients will need help with walking within 15
years after the onset of the disease. The short-term clinical and MRI
manifestations of disease activity have been reduced by new therapies
although the degree of presumed long-term benefit from these treatments
will require further studies.

The material contained in this chapter has relied on the following source: “MJ Olek, DM
Dawson. Multiple Sclerosis and other inflammatory demyelinating diseases of the central
nervous system in: “Neurology in Clinical Practice”. Edited by: WG Bradley, RB Daroff,
GM Fenichel, and J Jankovic 2004, Butterworth, Heinemann”.

1.1 Common clinical features of Multiple Sclerosis:

• Onset between ages 15 and 50
• Relapses and remissions
• Optic neuritis (monocular blindness; delayed P-100 component on
the VEP (visual evoked potential laboratory test)
• Lhermitte sign (with an electrical shock-like feeling down the back,
legs and/or arms on flexing the neck).
• Inter-nuclear ophthalmoplegia (presenting with diplopia)
• Fatigue
• Worsening with increased body temperature
• Sensory disturbances
53

• Limb weakness and clumsiness (bilateral or unilateral upper motor

neuron signs in the legs and at times the arms).
• Gait ataxia (cerebellar signs)
• Urinary and bowel symptoms

1.2 Clinical features not suggestive of Multiple Sclerosis:

• Onset before the age of 10 or after 60.
• Steady progression
• Early dementia
• Rigidity and sustained dystonia
• Cortical deficits such as aphasia, apraxia, alexia and neglect
• Deficit developing within minutes

1.3 Considerations when evaluating a patient:

• Obtaining objective evidence of dissemination in time and anatomical space of
lesions typical of Multiple Sclerosis is essential in making a secure diagnosis.
One would like to have at least two lesions in different anatomical regions occurring
on two separate occasions in time.
• It is often difficult to decide what is meant by the term objective and the question
may be asked: Are sensory and visual changes objective?

• It was suggested by a panel of experts on Multiple Sclerosis that: "Historical

accounts of symptoms may lead to a suspicion of the disease but cannot be
sufficient on their own for a diagnosis of Multiple Sclerosis".
• An historical account can often be used as evidence of one lesion and that a firm
diagnosis of Multiple Sclerosis can usually be made on the basis of an historical
account combined with the current evidence of a second lesion.
• Detailed description of attacks typical of Multiple Sclerosis clearly has greater
diagnostic value than vague accounts of less typical attacks.
• Dissemination in time may be partly established on history alone or on a subjective
basis.
• The question may be asked as to what constitutes an attack. The panel on
Multiple Sclerosis suggested the following: "For general diagnostic purposes an
attack… should last for at least 24 hours". Physicians should exercise caution with
attacks lasting less than 48 hours.
• The panel also suggested that: "single paroxysmal episodes e.g., a tonic spasm,
do not constitute a relapse, but multiple episodes occurring over not less than 24
hours do".
54

• As to what constitutes separate attacks the panel suggested the following: "It was
agreed that 30 days should separate the onset of the first event from the onset of
the second event".

1.4 Diagnostic criteria for the diagnosis of Multiple Sclerosis:

The diagnostic criteria for Multiple Sclerosis of Poser et al., 1983 have been
replaced by the criteria of McDonald et al., 2001.

Clinical presentation Additional data needed for MS diagnosis

• Two or more attacks; objective clinical None
evidence of 2 or more lesions
• Two or more attacks; objective clinical Dissemination in space, demonstrated by MRI
evidence of 1 lesion OR two or more MRI-detected lesions
consistent with MS Plus positive CSF OR
await further clinical attack implicating a
different site.
• One attack; objective clinical evidence of 2 Dissemination in time, demonstrated by MRI
or more lesions OR second clinical attack
• One attack; objective clinical evidence of 1 Dissemination in time: demonstrated by MRI
lesion (monosymptomatic presentation; OR second clinical attack OR two or more
so-called “clinically isolated syndrome”) MRI-detected lesions consistent with MS Plus
positive CSF AND dissemination in time
• Insidious Neurological progression Positive CSF AND MRI dissemination in
suggestive of MS space, demonstrated by (1) nine or more T2
lesions in brain or (2) 2 or more lesions in
spinal cord or (3) 4-8 brain lesions plus one
spinal cord lesion OR abnormal VEP
associated with 4-8 brain lesions, or with fewer
than 4 brain lesions plus 1 spinal cord lesion
demonstrated by MRI AND dissemination in
time, demonstrated by MRI OR continued
progression for 1 year.

1.5 MRI demonstration of space dissemination from McDonald et al (2001)

diagnostic criteria for Multiple Sclerosis:

Three or four of the following:

1. One Gadolinium-enhancing lesion OR nine T2-hyperintense lesions
if there is no Gadolinium enhancing lesion
2. At least one infratentorial lesion
3. At least one juxtacortical lesion
4. At least three periventricular lesions.
55

(Adapted from Barkhof et al., (1997) and Tintore et al., (2000)

MRI demonstration of time dissemination by McDonald et al. (2001)

diagnostic criteria for Multiple Sclerosis:

• If a first scan occurs 3 months or more after the onset of the clinical event, the
presence of a Gadolinium-enhancing lesion is sufficient to demonstrate
dissemination in time, provided that it is not at the site implicated in the original
clinical event. If there is no enhancing lesion at this time, a follow-up scan is
required. The timing of this scan is not crucial, but 3 months is recommended. A
new T2 or Gadolinium-enhancing lesion at this time then fulfils the criteria for
dissemination in time.

• If the first scan is performed less than 3 months after the onset of the clinical
event, a second scan done 3 months or more after the clinical event showing a
new Gadolinium-enhancing lesion provides sufficient evidence for dissemination in
time. However, if no enhancing lesion is seen at this second scan, a further scan
not less than 3 months after the first scan that shows a new T2 lesion or an
enhancing lesion will suffice.

The new criteria have advantages. The former categories of possible, probable, and definite
MS have become obsolete. The MRI criteria are based on extensive data of Barkhof et al.,
(1997) and Tintore et al., (2000) and are designed to retain sensitivity while enhancing
specificity. They will have little usefulness in patients with clear-cut demyelinating
syndromes such as optic neuritis or a brainstem syndrome; in such cases many clinicians
will be satisfied with less stringent MRI criteria. In patients the criteria will help avoid
premature or erroneous diagnosis and treatment. In addition, criteria for primary
progressive MS are proposed.
Revisions to the McDonald criteria have been proposed. For example, the role of spinal
cord lesions needs to be evaluated and expanded. It may take some time before the final
criteria are decided on and accepted as the standard for diagnosis.

1.6 Clinical course of Multiple Sclerosis:

1. Relapsing Remitting Multiple Sclerosis:

Clearly defined relapses with full recovery or with sequelae and
residual deficit on recovery. The periods between disease relapses
are characterized by a lack of disease progression.
56

2. Primary Progressive Multiple Sclerosis :

Disease progression from onset with occasional plateaus and
temporary minor improvements allowed.

3. Secondary Progressive Multiple Sclerosis:

Initial relapsing-remitting disease course followed by progression
with or without occasional relapses, minor remissions and plateaus.

4. Progressive-relapsing Multiple Sclerosis:

Progressive disease from onset, with clear acute relapses, with or
without full recovery. The periods between relapses are
characterized by continuing progression.

2. ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM)

- ADEM is a monophasic demyelinating syndrome that occurs in

association with immunization or vaccination (post vaccination
encephalomyelitis) or systemic viral infection (para-infectious
encephalomyelitis) or idiopathically.
- It is characterized by peri-vascular inflammation, edema and
demyelination within the CNS and clinically by rapid development of
focal- or multi-focal neurological dysfunction.
57

• Symptoms, signs and features of ADEM

o Bilateral optic neuritis
o Depression of level of consciousness
o A spinal cord picture or syndrome
o Recovery of ADEM is more rapid compared with Multiple
Sclerosis: Days vs. weeks and usually more complete.
o The CSF usually shows less than 100 cells per mm3, and a
modest increase in protein.
o The MRI usually shows multiple demyelinating lesions.
o Current favoured therapy for ADEM is corticosteroids, based
to some extent on their effect on the experimental allergic
encephalitis model, although no clinical controlled studies
have been conducted.

3. CENTRAL PONTINE MYELINOLYSIS

- Central Pontine
Myelinolysis (CPM) is a
symmetrical focus of
myelin destruction at the
centre of the basis of the
pons.

- It is typically associated
with the underlying
presence of
hyponatremia or hypo-
osmolality;

- It was described in patients with alcoholism and also in patients with

hypophosphatemia.
58

- Clinically the patient develops a picture of upper motorneuron signs

involving the legs, the arms and the lower bulbar region

- A rapid correction of hyponatremia may predispose the patient to the

development of CPM.
59

Chapter 5
HEADACHE AND FACIAL PAIN

1. HEADACHE
1.1 Intracranial pain sensitive structures
1.2 Clinical types of headache (classification)
1.2.1 Vascular headache
1.2.2 Tension headache
1.2.3 Traction inflammatory (structural brain or meningeal involvement headache)

1.2.4 International Headache Classification

1.3 The time course of headache
1.3.1 Chronic longstanding headache

1.3.2 Acute episodes of headache intermittently over

months

1.3.3 Chronic headaches with acute exacerbations

1.3.4 Headaches occurring in bouts, eg. cluster

1.3.5 Acute episodes of headache occurring daily, eg. Chronic paroxysmal

hemicrania

1.3.6 Short sharp stabbing headache (icepick headache)

1.3.7 Chronic progressive headache
1.3.8 Acute immediate-onset headache (including thunderclap headache)
1.3.9 Exertional headache
1.4 Migraine

2. FACIAL PAIN
2.1 Trigeminal neuralgia
2.2 Trigeminal neuropathy
2.3 Glossopharyngeal neuralgia
2.4 Lower half headache
2.5 Atypical facial pain
2.6 Temporal arteritis
2.7 Tempero-mandibular dysfunction (Costen Syndrome)
60

1. HEADACHE

DEFINITION OF HEADACHE
Headache is a pain that is experienced in the distribution of the ophthalmic division
of the trigeminal nerve and of the second and third cervical roots.
Headache definition

1.1 Intracranial pain sensitive structures

1. Dilatation and traction of the intracranial arteries, particularly their

proximal regions, and of the middle meningeal artery.
2. Venous sinuses and the large veins which drain into the sinuses.
3. Basal dura mater.
4. Pressure and traction of the nerves, which carry pain sensation,
such as the trigeminal, glossopharyngeal and facial nerves and the
second cervical root.

Pain derived from the supratentorial compartment is referred to the

ophthalmic division of the trigeminal nerve (the forehead).

Pain of infratentorial origin is referred to the distribution of the second

and third cervical roots (over the occipital and neck areas
respectively).

1.2 a Clinical types of headache (Older Classification) (Dalessio, 1974)

Dalessio’s classification of headache allows for a practical simplified approach at the bedside.
[Link] headaches

[Link] headaches and

[Link] inflammatory (structural brain or meningeal involvement) headaches. The combination

of headache symptoms point to one of the above three syndromes of categories from where the
nature of the headache may then be further analysed.

1.2.1 Vascular headache General characterristics

• Periodic occurrence over time with paroxysmal nature (starts and stops suddenly)
• Throbbing in character
Unilateral
Severe degree of intensity of the headache (frequently >7/10)
Movement or shaking of the head aggravates the headache
Accompanying features of migraine may include nausea and vomiting; phonophobia; photophobia; sensory-, motor-,
visual- and aphasic auras, which sometimes take place in a sequential fashion over a period of 5-30 minutes; typically patients lie
down and keep still and find that sleep relieves their headache.
• Accompanying features of cluster headache may include conjunctival injection, tearing from the eye, a blocked
nose, Horner’s syndrome, and motor hyperactivity during the pain (walk or run around in agony, the opposite of migraine).
 Causes of vascular headache:
61
• Migraine
Cluster Headache
Systemic infections
Hypertension
Post-ictal (convulsive)

Tension headaches General characteristics

• The frequency is often daily, and the headache may last from hours to the whole day
• The character tends to be a dull, constant or pressure‐like headache

• It occurs bilaterally in approximately 90 percent of patients

• Long history which may stretch over months, years and even decades
• Accompanying symptoms of anxiety and an affective disorder may be found which may have to be addressed in their own
right; sometimes associated with a somatiform disorder and a polysymptomatology.

Causes of tension headache include:

• Primary tension or “muscle tension headache” where the cause may be unknown or where underlying conflicts may contribute to
the headache and at times to muscle spasm of the neck‐, temple‐, forehead‐ and jaw muscles. The brain may become sensitized to pain
where minor triggers aggravate the headache
• Co‐existing eye or neck problems or tempero‐mandibular dysfunction, which, may induce or exacerbate the headache or
muscle spasm.
• Migraine headache which may transform into a tension headache or daily headache syndrome which may be worsened by die
overuse of analgesics or ergot/triptan preparations.

1.2.1 Traction‐inflammatory” headache( Headaches with structural or meningeal involvement): General characteristics
• Sudden onset of severe headache with neck stiffness (typical of a subarachnoid bleed)
• Neck stiffness (seen with meningitis and subarachnoid haemorrhage)
• Headache worsening in severity and frequency

• Exertional element (headache worsens with coughing, sneezing or exercise)

• Headache associated with nausea and vomiting

• Early morning headaches

Causes of “traction‐inflammatory” headache

• Space occupying lesions (results from pulling or traction of pain‐ sensitive structures) and raised intracranial pressure per se (e.g.,
Idiopathic Intracranial Hypertension, tumors)
• Diseases of the meninges (e.g., meningitis)

• Low CSF pressure (or volume) syndrome (either post LP or spontaneous)

• Venous sinus thrombosis

• Subarachnoid bleed

• Sinusitis associated with cerebral thrombophlebitis or the so‐called neighborhood syndrome (CSF tends to show a pleocytosis)
• Temporal arteritis (red swollen arteries, high S‐CRP and sedimentation rate)

1.2.b New Headache classification (also refer to 1.2.4)

A primary headache is a headache that is due to the headache condition itself and not due to
another cause. A secondary headache is a headache that is present because of another
condition such as a sinus headache from sinusitis.

The three types of primary headache are:

1. Migraine

2. Tension

3. Trigeminal autonomic cephalalgias (eg, cluster)

 Examples of secondary headaches are:
61 a
1. Sinus headache: By definition, this type of headache should resolve when the course of
antibiotics for sinusitis is completed.

2. Medication overuse headache: By definition, this type of headache should resolve when
the medication being overused is discontinued.

3. Acute illness headache: An example here is meningitis.

4. Post-traumatic headache: From trauma such as an accident with a head injury.

5. Spinal headache: This type of headache is common after an epidural (eg, during labor).

6. Headache due to a space-occupying lesion: This headache is experienced by patients

with eg, a brain tumor\abscess

7. Cervicogenic headache: This type of headache is related to the underlying neck

condition such as degenerative disc disease of the cervical vertebrae.

8. Thunder-clap headache: This type of headache is often seen in patients with an acute
subarachnoid hemorrage.

Danger signs (i.e., red flags) should prompt further evaluation; a useful way to differentiate
between primary and secondary headaches is to look at the following list of questions
which is sometimes referred to as the “SNOOP” list:

1. Systemic Signs or Symptoms: Look for the presence of fever, weight loss, history of
cancer, abnormal blood tests; this could point to meningitis, cancer, or illness to be the
cause of the headache.

2. Neurologic Exam: if the neurologic exam is abnormal, then a secondary headache

should be ruled out. Examples of an abnormal neurological exam include abnormal
speech, abnormal gait, confusion, and dizziness.

3. Onset: if onset of headache is sudden then a secondary headache such as an

aneurysm or a bleed should be suspected. Onset less than 2 weeks could indicate an
illness such as meningitis. If the onset was greater than 6 months ago, then this may
point to a benign headache condition.

4. Progressive: this would refer to a headache pattern that is progressively worsening

over time. Even migraine patients can develop a secondary headache such as a tumor
or aneurysm so a progressive pattern of worsening headaches in a known migraine
individual may require a work-up such as an MRI or spinal tab or blood work.

Reassuring history that indicates a primary headache includes:

1. Stable pattern of headache for over 6 months

2. Predictable triggers for headache

3. The individual feels fine in between headache attacks

Practical Evaluation of headache in adults

Clinicians can easily become familiar with the most common primary headache disorders and
how to distinguish them.
62

Misconceptions — A number of misconceptions may hinder headache evaluation and

diagnosis.

●Although sinus headache is commonly diagnosed by physicians and self-diagnosed by

patients, acute or chronic sinusitis appears to be an uncommon cause of recurrent
headaches, and many patients presenting with sinus headache turn out to have migraine
●Patients frequently attribute headaches to eye strain. However, an observational study
suggested that headaches are only rarely due to refractive error alone. Nevertheless,
correcting vision may improve headache symptoms in some of these patients.
●There is a common belief, particularly among patients, that hypertension can cause
headaches. While this is true in the case of hypertensive emergencies, it is probably not
true for typical migraine or tension headaches.

EVALUATION — The appropriate evaluation of headache complaints includes the following:

● Rule out serious underlying pathology and look for other secondary causes of headache
● Determine the type of primary headache using the patient history as the primary
diagnostic tool. There may be overlap in symptoms, particularly between migraine and
tension-type headache and between migraine and some secondary causes of headache
such as sinus disease.
63

Clinical picture of tension headache

Tension headache may be divided into episodic (headache for <15 days a
month, <180 days a year) and chronic (on average more than 15 days a
month or more than 180 days a year), lasting longer than 6 months, and
associated with or without tenderness (or increased EMG activity) of the
pericranial muscles. Criteria for tension headache include:
1. At least 10 previous headache episodes which meet criteria B to D
2. Duration of headache from 30 minutes to 7 days
3. The pain demonstrates at least 2 of the following characteristics

1. Pressing (not throbbing) quality

2. Slight to moderate intensity
3. Bilateral localization
4. No element of worsening with head movement
4. Both:
1. No nausea or vomiting (loss of appetite may be present)
2. Photophobia and phonophobia are absent or one, but not the
other is present.

1.2.4 New International Headache Classification (The International

Headache Classification, 3nd ed., (Cephalgia Vol 33, Issue 9, 2013)

Part one: the primary headaches

1. Migraine

2. Tension-type headache

3. Trigeminal autonomic cephalalgias

4. Other primary headache disorders

 Part two: the secondary headaches

5. Headache attributed to trauma or injury to the head and/or neck

6. Headache attributed to cranial or cervical vascular disorder

7. Headache attributed to non-vascular intracranial disorder

8. Headache attributed to a substance or its withdrawal

9. Headache attributed to infection

10. Headache attributed to disorder of homoeostasis

11. Headache or facial pain attributed to disorder of the cranium, neck, eyes, ears,
nose, sinuses, teeth, mouth or other facial or cervical structure

12. Headache attributed to psychiatric disorder

Part three: painful cranial neuropathies, other facial pains and other
headaches

13. Painful cranial neuropathies and other facial pains

14. Other headache disorders

1.3 The time course of headache

The time course of the various types of headache is often a pointer or guide to the
nature or type of the underlying headache.

Headache intensity

Time scale – (months to years)

65

1.3.1 Chronic longstanding headache

This typically shows an all-day every day headache pattern.

EXAMPLE:
1. Tension type headache
2. Analgesic overuse headache
3. Hemicrania continua which may show the following features
i. Continuous unilateral headache
ii. Moderate intensity
iii. Involve the entire hemicranium or simply be confined to a focal
area
iv. Occurrence of painful unilateral exacerbations lasting 20 minutes
to several days
v. May alternate sides
vi. Hemicrania continua represents one of the indomethacin
responsive headaches together with Primary Stabbing
Headache, Paroxysmal Hemicranias and shortlasting unilateral
neuralgiform pain with conjunctival injection and tearing,
SUNCT, (moderately severe unilateral cephalgia characterized
by neuralgiform pain around the eye of very short duration;
attack frequency of 1-2/day to 30/hour; prominent ipsilateral
conjunctival injection and lacrimation).

1.3.2 Acute episodes of headache intermittently over months

This typically shows recurrent active episodes over a long time.

EXAMPLES:
1. Migraine
2. Orthostatic headache gives rise to a severe paroxysmal headache related
to posture: to the assuming of the upright posture. The headache typically
comes on within approximately 5 to 20 minutes of assuming the upright
position and is rapidly relieved within about 5 to 30 minutes of lying down.
The headache is often very severe in degree, of a throbbing nature,
associated with neck stiffness and nausea. Low CSF pressure syndrome
may occur secondary to a LP, an epidural procedure, a ventriculo-
66

peritoneal shunt or spontaneous CSF leaks (e.g., ruptured nerve root

sleeve).
3. This is a rare type of headache occurring at least 15 time per month,
waking the patient from sleep and lasting 10-180 minutes. No other
features (e.g., nausea, autonomic features) should be present and SOL
need to be excluded.

1.3.3 Chronic headaches with acute exacerbations

Headache intensity

Time scale 1 month

Typically, the patient experiences background pain with episodic worsening of pain.

EXAMPLES:
Mixed tension type headache and migraine.

1.3.4 Headache occurring in bouts

Headaches occurring e.g., twice a day for approximately 3-6 weeks followed by a
remission of about 3-12 months.

Headache
intensity

6-weeks 6-weeks
Time scale- one year
67

Headache
intensity

11:00 01:00
Time scale- One day

EXAMPLES:
Cluster headache

There is a tendency for the headache to occur at the same time of the day. The
pain is boring in character, usually occurs behind the eye and is very severe in
degree and usually lasts less than two to three hours.

Clinical picture of cluster headache

Cluster Headache is a vascular-type of headache with the following

characteristics
(1) A periodic onset to the attacks of pain is characteristic: two to five attacks
of headache are encountered daily for a period of from four to eight weeks,
followed by a headache-free period for months to years
(2) The duration of an attack is usually longer than 15 minutes and shorter
than three hours
(3) The attack has a tendency to present at the same time of the day,
frequently between one and two AM and again between ten and eleven in
the morning
(4) The pain is of a boring, intense quality, most severe over the eye (likely
due to vasodilatation of the internal carotid which refers pain to the eye)
(5) Sympathetic palsy (Horner’s syndrome) is present in about half of patients
( possibly because of the dilatation of the internal carotid artery in the
carotid canal with compression of the sympathetic fibres)
(6) Ipsilateral parasympathetic disturbances such as tearing from the eye and
a blocked nose (possibly caused by the discharge of the superior salivary
nucleus via nervus intermedius to the greater superficial petrosal nerve
and the spheno-palatine ganglion, and then via branches to the nose and
lacrimal glands)
(7) Hyperactive motor activity during the attack, the patient walking hither and
thither, in contrast to migraine
(8) Reddening and oedema of the face (due to extracranial vessel dilatation)
(9) Red eye (due to dilatation of the ophthalmic artery)
(10) Characteristic high prevalence amongst male gender (male/female ratio
9/1)
Cluster headache characteristics
68

A chronic form of cluster headache can occur either de novo or may develop from
established cluster headache (Olesen, 1988).

1.3.5 Acute episodes of headache occurring daily

Multiple unilateral attacks of headache may occur on a daily basis, varying in

number from 3 to 11 attacks per day. This headache responds particularly
well to indomethacin. Characteristically seen in women.

Headache
intensity

15-25min
Time scale-One day

EXAMPLE:
Chronic paroxysmal hemicrania.

1.3.6 Short stabbing episodic pain

This headache has a characteristic brief duration of seconds to minutes.

The pain is sharp or stabbing in character and sometimes feels like an
electric shock. It may be uni- or multifocal. The pain may be an isolated
event or may occur in clusters.
69

Headache intensity

Seconds-minutes
Time scale one hour

EXAMPLE:
Primary stabbing headache (Icepick Headache).

These primary stabbing headaches may occur on their own, as a benign

transient entity, but are frequently associated with the underlying presence of
migraine. They may be associated with other underlying headaches such as
Cluster Headache, Chronic Paroxysmal Hemicrania or Tension Type of
headache. Primary Stabbing Headache responds characteristically to
indomethacin treatment.

1.3.7 Chronic progressive headache

Headache
intensity

Time scale - weeks to months

EXAMPLE: A space-occupying lesion such as a brain tumor, granuloma or

abscess.
70

1.3.8 Acute immediate onset headache

Headache
intensity

Time scale-One Day

EXAMPLE

• Subarachnoid haemorrhage, in which the acute onset headache is

associated with neck stiffness, and lateralizing signs are usually absent or
develop later.
• Intracerebral haemorrhages in which lateralizing signs and depression of
the level of consciousness are present early on.
• Thunderclap headache. Diagnostic criteria include the following:
1. Very severe pain intensity
2. Hyperacute onset of pain (<30 seconds)
3. Headache lasts 1 hour to 10 days (may last up to 4 weeks)
4. Headaches may recur over a 7-day period but do not recur regularly over
subsequent weeks or month (may recur over subsequent months to years).
Causes of Thunderclap Headache include:
i. Subarachnoid haemorrhage
ii. Pituitary apoplexy
iii. Cerebral venous sinus thrombosis
iv. Low CSF pressure syndrome
v. Reversible segmental cerebral vasoconstriction
vi. Idiopathic

1.3.9 Headache associated with exercise/exertional headache

This is a headache, which is induced by exercise or exertion such as

coughing, laughing, bending forward and lifting objects. Typically, the pain
will last for seconds to minutes after the exertion.
71

Exertion triggers the headache

Headache
intensity

Time scale One Day

EXAMPLE:
1. Space occupying lesion.
2. Exertional headache (benign).

This headache is sometimes relieved with indomethacid. The headache

may only be regarded as benign once CT scanning has excluded a
mass in the brain.

1.4 Migraine (Olesen, 1989)

Definition of Migraine without Aura (common migraine)

(A) At least five attacks which meet the criteria laid down in B to D
(B) Headache of 4 to 72 hours duration
(C) The headache has at least two of the following characteristics:
(1) Unilateral localization
(2) Throbbing quality
(3) Moderate to severe intensity
(4) Movements of the head exacerbate the pain
(D) During the headache at least one of the following occur:
(1) Nausea and/or vomiting
(2) Photophobia or phonophobia.
Migraine without aura (common migraine)
72

Migraine with aura (classic migraine)

Two attacks fulfilling the criteria of B above. At least three of the following four
characteristics occur:
(1) One or more completely reversible aura symptoms
(2) At least one aura develops gradually over more than 4 minutes or two or more
symptoms develop in succession
(3) No aura lasts longer than 60 minutes
(4) Headache follows the aura within 60 minutes (it may develop before or with the
aura).
Migraine with aura (classic migraine)

Complicated migraine refers to migraine with a prolonged aura lasting longer than
60 minutes. Migraine infarction represents an infarction associated with migraine in
which clinically the neurological deficit lasts longer than 7 days or an infarction is
diagnosed on neuro-imaging. An ophthalmoplegic migraine (third nerve palsy) and
retinal migraine (monocular blindness) may only be diagnosed as migraine by
exclusion of other organic pathology.

2. FACIAL PAIN

Definition of facial pain

Facial pain is pain that is experienced in the maxillary and mandibular divisions
of the trigeminal nerve.
Facial pain definition

Causes of facial pain

• Trigeminal neuralgia
• Trigeminal neuropathy
• Glossopharyngeal neuralgia
• Lower half facial pain
• Atypical facial pain
• Temporal arteritis
• Tempero-mandibular dysfunction
73

2.1 Trigeminal neuralgia (Van der Meyden, 1985)

Pain in trigeminal neuralgia

Headache intensity

Time scale One Day

This is a paroxysmal, sharp, shooting neuralgic type of pain lasting from

seconds to minutes and is frequently compared to an “electrical shock”.
Typically particular movements will trigger the pain, such as speaking or
chewing. This condition usually presents after the age of 50 and
accompanies enlarged atherosclerotic vascular loops, which may compress
the proximal part of the trigeminal nerve. The paroxysmal neuralgic
character of the pain lasting for a few seconds up to a few minutes is
characteristic. The pain is sharp, stabbing with its predominant distribution
being in the maxillary area. Usually there is no objective sensory deficit.
Occasionally multiple sclerosis or tumors of the trigeminal nerve root area
may give rise to a similar picture.

2.2 Trigeminal neuropathy

Sensory loss with or without pain is indicative of a trigeminal neuropathy.

Herpes Zoster infections, tumours of the posterior nasal space and of the
middle cranial fossa, lues and multiple sclerosis are examples of its causes.

2.3 Glossopharyngeal neuralgia

Paroxysmal neuralgic sharp stabbing pains arising in the pharynx and
external ear canal characterize Glossopharyngeal neuralgia.
74

2.4 Lower half headache (FACIAL AREA PAIN)

Migraine and cluster headache may affect this area.

2.5 Atypical facial pain

This pain usually occurs in the maxillary area and has a chronic course. The
examination is normal and an underlying depression is frequently found.
The pain will often improve with anti-depressant treatment e.g., with
amitriptyline and characteristically becomes worse following surgical
intervention.

2.6 Temporal arteritis

This is an important condition since it can lead to loss of vision if not

diagnosed and treated. The characteristic features are headache,
tenderness over the extracranial vessels, red swollen pulseless extracranial
vessels, and claudication of the muscles of mastication. Optic neuritis, a
high sedimentation rate and an association with polymyalgia rheumatica may
also occur. This condition characteristically presents in patients over the age
of 50 years group and is treated with steroids.

2.7 Tempero-mandibular dysfunction (Costen Syndrome) (Howe, 1971)

- The cardinal symptoms and signs are:

- Pain and tenderness in the area of the tempero-mandibular joint.
- Restriction of jaw movement.
- A clicking noise is heard over the tempero-mandibular area on opening of
the jaw and there is associated spasm of the muscles of mastication.

The main cause is more likely to be due to predominantly local masticatory muscle
spasm than to a problem of mal-occlusion.
75
Chapter 6

1. VERTIGO
1.1 Clinical types of vertigo
1.1.1 Paroxysmal vertigo
1.1.2 Positional vertigo
1.1.3 Longstanding dizziness
1.2 Differences between central and peripheral vertigo
76

1. VERTIGO

Definition of vertigo
Vertigo is a hallucination of a spinning movement either of the person himself or
of his surroundings. It is often accompanied by autonomic symptoms such as
sweating, pallor, tachycardia, nausea and vomiting (The symptoms may be so
intense as to mimic an acute myocardial infarction).
Vertigo definition

Vertigo should be carefully differentiated from dizziness.

Definition of dizziness
Dizziness is a hallucination of movement other than a spinning motion. The
hallucination of movement occurs forwards, backward, to the sides or up- or
downward.
Dizziness definition

1.1 Clinical types of vertigo (Symonds, 1970)

1.1.1 Paroxysmal vertigo

Episodes of vertigo start acutely and resolve relatively rapidly.

Intensity of
vertigo

Time scale

Examples:
- Meniérè disease with deafness as an additional symptom (lasting hours
to days).
- Transient ischaemic attack (duration of minutes to hours).
- Multiple sclerosis (duration of minutes to hours to days).
- Vestibular neuronitis (without deafness) (lasting hours to days).
77

1.1.2 Positional vertigo

The onset of the dizziness or vertigo is induced by a positional change of the

head. The vertigo characteristically lasts only for a few seconds to minutes
after the movement. The test for positional vertigo and nystagmus (Hallpike
maneuver) may confirm the diagnosis and may help to differentiate between
central and peripheral types of positional vertigo and nystagmus.

1.1.3 Longstanding dizziness

Intensity of dizziness

Time scale weeks to months

The longer the duration of the dizziness and the more vague and
polysymptomatic the complaints, the bigger are the chances that the
dizziness is of psychogenic [Link]: Psychogenic dizziness.

1.2 The difference between central and peripheral vertigo

One of the main goals in a patient with vertigo is to determine whether

the lesion is located centrally (CNS) or peripherally (in the ear area).
78

Central vertigo Peripheral vertigo

• Vertigo is less dramatic • Vertigo is more severe or
except in diseases intense
involving the vestibular
nuclei
• Concomitant ENT disease
• Concomitant neurological or signs are found
symptoms and signs are • Deafness and tinnitus
found characteristically occur in
• Deafness rarely occurs in peripheral lesions
central origin • Jerk nystagmus is
maximally seen when
• Vertical jerk-nystagmus is looking away from the
present side of the lesion
• Previous ENT disorder is
• Previous history or signs of noted
central nervous system
disease are found
Pathology: Pathology:
• Strokes of the brainstem • Diseases of the middle ear
• Multiple sclerosis • Ménière’s disease
• Tumors of the cerebello • Drugs (e.g.,
pontine angle aminoglycosides)
(e.g., acoustic neurofibroma)
79

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80
Chapter 7

1. SLEEP DISORDERS
1.1 Evaluation of sleep disorders
1.2 Classification of sleep disorders
1.3 Examples of sleep disorders
81

1. SLEEP DISORDERS

1.1 Evaluation of sleep

During a detailed history of the sleeping pattern the following are noted

• Whether the sleep was refreshing

• Whether sleep onset is disturbed
• Whether awakening from sleep occurs
• Whether early morning waking occurs
• Whether the patient is wide awake on waking in the morning
• Whether daytime sleepiness occurs

1.2 Classification of sleep disorders (Weitzman, 1984)

1. Disorders of initiating and maintaining sleep: (in ± one-third of the

subjects)
• Psychiatric disturbances
• Drug and ethanol dependence
• Restless-legs syndrome
• Sleep related myoclonus
2. Disorders of excessive daytime somnolence: (in ± one-half of the
subjects)
• Sleep apnoea syndrome
• Narcolepsy
• Idiopathic hypersomnia
• Sleep drunkenness
3. Disorders of the sleep-wake cycle
• Transient type in shift workers and jet travelers
• Delayed sleep phase syndrome
• Early wake sleep phase syndrome
• Irregular sleep-wake pattern
4. Disorders associated with sleep (parasomnias)(approximately one-sixth of
subjects)
• Sleep walkers
• Night terrors
• Sleep related enuresis
• Sleep related bruxism (teeth grinding)
• Sleep related head banging
Sleep disorders classification

Alcohol, hypnotics, stimulants such as coffee, appetite suppressants and

methylphenidate often disturb the sleep pattern. Underlying medical
82

conditions such as arthritis, renal failure and hyperthyroidism may contribute

to insomnia.

The patient is asked to keep a three-week diary of his sleep pattern. In

selected patients a polysomnogram is performed. This technique is
especially useful in the diagnosis of the sleep apnoea syndrome.

1.3 Examples of sleep disorders

Definition of Narcolepsy
Narcolepsy characteristically consists of a tetrad of daytime somnolence,
cataplexy, sleep paralysis, and hypnogogic hallucinations. Just a single
phenomenon or different combinations may occur. The combination of excessive
daytime somnolence, together with cataplexy is almost diagnostic of narcolepsy.
The association of HLA-DR2 with narcolepsy is reported to be present in more
than 90 % of Caucasians. A high association has also been found between
narcolepsy, cataplexy and DQw1. Patients suffering from narcolepsy can be
woken up instantly and benefit greatly from planned short sleep sessions during
the day. A sleep onset of <5minutes with two early episodes of REM on the MSLT
support the diagnosis. Undetectable CSF hypocretin-1(hypothalamic peptide;
stimulatory influence on ARAS) levels are highly specific for narcolepsy.
Narcolepsy definition
Definition of the sleep apnoea syndrome
Apnoea alternating with snoring during sleep are characteristic of sleep apnoea.
The diagnosis of sleep apnoea syndrome is suspected if the following conditions
are present:
(1) Excessive daytime somnolence
(2) Snoring
(3) Night restlessness
(4) Abnormal awakenings
(5) Night time insomnia
(6) Obesity
(7) Pulmonary hypertension
(8) Right-sided heart failure and
(9) Polycythemia.
Sleep apnoea syndrome definition

Diagnosis of sleep disorders

MSLT or refers to the “multiple sleep latency test”. The patient is given
the opportunity to sleep during the day – five opportunities every two hours
are typically given where the time to sleep onset (sleep latency) and early
REM-onset are determined.

An afternoon or all-night polysomnogram helps to confirm the diagnosis of

sleep apnoea. One episode of apnoea is defined as a cessation of airflow
83

for more than 10 seconds. The diagnosis of sleep apnoea is supported if

more than 10 episodes of apnoea are seen in one hour, or if more than 10
episodes of oxygen desaturations or awakenings per hour are found in
association with hypoventilation.

Insomnia
If the patient does not experience exhaustion or sleepiness during the day,
the insomnia is probably not of clinical importance. The presence of pain,
arthritis, uremia, and heart failure may contribute to insomnia and needs to
be treated separately.

Similarly, depression is treated as a separate entity. The long-term use of

hypnotics often induces sleeping disorders and the offending drug is weaned
off gradually. In senile dementia reversal of the sleep – wake rhythm is often
found.

Sleep drunkenness
Patients with sleep drunkenness characteristically are unable to be woken
up at a specific time. They also experience an increased total sleep time
and excessive daytime somnolence. When woken up, they show
disorientation, confusion, and slow motor coordination and are inclined to fall
asleep repeatedly.
84

PART 2: THE NEUROLOGICAL EXAMINATION

Chapter 8

1. CEREBRAL HEMISPHERE FUNCTIONS

1.1 Orientation
1.2 General knowledge
1.3 Hallucinations, illusions and delusions
1.4 Memory
1.4.1 Immediate (working or soundboard) memory
1.4.2 Anterograde memory
1.4.3 Long-term memory
1.5 Ability to calculate and concentrate
1.6 Emotional state and drive
1.7 Insight and intellect
1.8 One minute animal category fluency test
1.9 Folstein Mini-Mental State Examination (MMSE)
85

1. CEREBRAL HEMISPHERE FUNCTIONS:

Consciousness is traditionally subdivided into the cognitive (intellect), the

conative (the drive or will) and the affective (emotion) components. While
conversing, the patient is evaluated for any obvious dysfunction of these
faculties. The ability to think quickly is observed, as well as the drive or
apathy and the emotional state (whether the patient acts rationally,
empathetically or seems depressed). The personality of the patient is
observed. If something amiss is noted, some further tests are performed.

1.1 Orientation:
The patient is asked what his name is, where he is, what day it is, which
month and which year. Orientation ability is frequently disturbed in patients
with a confused state, a delirium or a severe dementia and is tested when
something is suspected to be amiss.

1.2 General knowledge:

The patient is asked to name the province of a country, the President,
different models of cars, different types of vegetables, trees or flowers.

1.3 Hallucinations, illusions and delusions:

These phenomena are the main characteristics of the psychoses. Visual
hallucinations often indicate an underlying delirium. In schizophrenia auditory
hallucinations are prominent.

1.4 Memory
Memory is the corner stone of the intellect. Three aspects of memory may be
assessed.

1.4.1 Immediate (working or soundboard) memory (Hodges, 1994):

Soundboard memory is the ability to repeat just - received verbal, melodious
or spatial information immediately. Asking the patient to repeat a number
with seven digits plus or minus two may test working memory. The patient
86

may also be asked to repeat a long and complicated sentence. This form of
memory depends upon the patient’s ability to concentrate as well as on an
intact frontal lobe managing function, the language abilities of the left
hemisphere and the spatial ability of the right hemisphere. Sound-board
memory will, for example, be disturbed in acute confusional states.

1.4.2 Anterograde memory (Hodges, 1994):

Anterograde memory indicates the ability to learn new information. The
patient is asked, for example, to learn and remember a number of items or
names and about five minutes later the recall ability of this information is
tested. The phenomenon of confabulation may be seen in patients with
antegrade memory loss. They are unable to remember data but do not
realize this and fill in the gaps with confabulated data.

Disorientation regarding place and time is usually associated with

dysfunctional anterograde memory. Anterograde memory is closely related
to episodic memory. Episodic memory is a form of explicit memory
(accessible to consciousness) which is specific regarding personal
experience of time, place and context; receiving an emotional impact
capping and is probably served by the anatomic limbic lobe substratum. The
tracts involved in the registration of episodic memory probably include the
following: integration of language symbols at the Wernicke area, the
transmission of information to the inferior medial temporal surfaces and the
hippocampi, the transfer of information to the medial dorsal nuclei of the
thalami and hence the projection to the orbitofrontal cortices and back to the
temporal cortical areas. This is a useful circuit framework to remember
since lesions of these areas (i.e., the inferior medial temporal lobes, the
medial dorsal nuclei of the thalami and orbito frontal areas) may give rise to
episodic and anterograde memory loss. In Wernicke encephalopathy the
medial dorsal nuclei of the thalami are usually the dominant site of
involvement pathologically.

In cortical dementia anterograde memory loss is often seen as an early sign.

Alzheimer’s disease, cerebral hypoxia and herpes simplex encephalitis may
87

show early and selective involvement of the hippocampal areas.

Electroconvulsive therapy causes transient recent memory disturbance
(immediate and possibly anterograde memory circuitry can be considered to
work on an “electrical network” basis).

1.4.3 Long-Term memory:

Retrograde memory loss is a useful clinical term and indicates an inability
to recall previously learned information. Dysfunctions of episodic, semantic
or explicit memory (not time and context specific, and learned factual and
vocabulary information, with the temporal neocortex as anatomical
substratum) and implicit (or procedural) memory (learning of, e.g., motor
capabilities or skills, largely not accessible to consciousness, with the
anatomical substratum situated in the basal ganglia) may be found. In
episodic memory disturbance the patient is unable to recall memory pictures
of the remote past. Details of the patient’s previous history are important
and are usually obtained by a family member. As an example it may be
asked in which city the patient lived as a child, the names of the school
principal and priest may be requested, etc. General knowledge, vocabulary,
fluency in categories (list of e.g., animals, words starting with particular
letters, fruits, car models, etc.) may test semantic memory.

1.5 Calculation and concentration abilities:

These two abilities are closely related and may be tested together. The
calculation of the 100 – 7 test and its serial subtraction of sevens thereafter
are useful tests in practice. The time it takes to complete the test as well as
the number of mistakes the patient makes are noted. This test may be
repeated at a later stage to observe deterioration or improvement. In left-
sided parietal lobe lesions a severe inability to manipulate numbers may be
found.

1.6 Emotional state and drive:

The patient may appear euphoric or depressed. Patients with depression
often do not talk a lot, show a low drive, sleep badly with early morning
awakening, have a low energy level, appear tired and have polysymptomatic
88

complaints. Patients with hypomania and mania may appear overly

courageous, show psychic and motor hyperactivity, have a pressure of
thoughts (show rambling speech), a tendency not to sleep much, feel
as though they have all the energy in the world and tend to have
superficial thought associations. Patients with apathy, slowness of
emotions, drive and intellect may have the presence of an underlying
lesion of the thalamus, the basal ganglia, the supplementary motor
cortices or of the orbito-frontal lobe areas. This may be referred to or
known as abulia minor (if severe abulia major) and may also be found
in the so-called subcortical dementias (e.g., Parkinson’s disease,
Huntington’s chorea, and multi-system degeneration).

1.7 Insight and intellect:

General conversation will show whether the patient has insight into his
own condition. The patient may ignore an obvious deficit while he might
refer to some unimportant side issue when asked what the problem is. The
patient’s scholastic and post-school training and work as well as hobbies
will reflect on his functional productivity status. It is determined whether
the patient acts psychically relevantly when past training and previous
progress is taken into consideration. The suspicion of intellectual
deterioration may be confirmed by doing specific psychometric tests (by a
clinical psychologist).

1.8 One minute animal category fluency test

(Canning et. Al., 2004)
The subject is asked to name as many animals as he can within a 60
second period. Normal for a literate person is >15

1.9 Folstein Mini-Mental State Examination

89
90
Chapter 9

1. DYSFUNCTIONS OF THE CEREBRAL HEMISPHERES

1.1 Confusional state (delirium)
1.1.1 Diagnostic criteria of delirium
1.1.2 Short (6-item) orientation-memory-concentration test
1.1.3 Causes of the confusional state or delirium
1.1.4 Predisposing factors to the development of the confusional state
1.1.5 Subcategory of “over-activity delirium”
1.2 Definition of dementia
1.2.1 Causes of dementia
1.2.2 Subcortical dementia
1.2.3 Treatable causes of dementia
1.3 Frontal lobe disorders
1.4 Parietal lobe disorders
1.5 Temporal lobe disorders
1.6 Occipital lobe disorders
1.7 Memory disorders
1.7.1 Transient amnesia (Transient Global Amnesia syndrome)
1.7.2 Lasting disorders of memory
91

1. DYSFUNCTIONS OF THE CEREBRAL HEMISPHERES

1.1 The confusional (delirium) state (Van der Meyden, 1990):

The term confusional state and delirium may be considered to be
overlapping concepts. Delirium is derived from the Latin term meaning,
“groove” and indicates a mental disturbance.

Definition of confusional state: (delirium) (Taylor & Lewis,1993)

Delirium is an acute, reversible, diffuse, organic cerebral hemisphere failure or
dysfunction (emergency), with disturbance of the contents of consciousness.
Decreased awareness of the external surroundings and decreased alertness and
wakefulness (decrease in the level of consciousness and disturbance of sleep-
wake cycle) are seen. The faculty of attention is impaired with an inability to
focus, maintain or shift attention. Disturbance of the immediate or working
memory is the primary problem, which gives rise to secondary involvement of the
anterograde (short-term) memory and disorientation for time, place and person.
Delirium definition Confusional state definition

1.1.1 Diagnostic criteria of delirium (DSM III)

A. Clouding of consciousness (reduced clarity of awareness of
environment), with reduced capacity to shift focus and sustain attention
to environmental stimuli (*attention and concentration span may be
assessed by tests such as serial subtraction of sevens or three’s.
Normally the 100-7 test may be performed without error within 35
seconds)

B. At least two of the following:

1. Perceptual disturbance: misinterpretations, illusions, or
hallucinations
2. Speech that is at times incoherent
3. Disturbance of sleep-wakefulness cycle with insomnia or daytime
drowsiness
4. Increased or decreased psychomotor activity

C. Disorientation and memory impairment (if testable: *disorientation for

name, age, city, time, day, month and year; inability to retain new
memories with an anterograde or short term memory problem. This
92

function can be tested by asking the subject to recall e.g., four objects
after 5 minutes)

D. Clinical features that develop over a short period of time (usually hours
or days) and tend to fluctuate over the course of a day

E. Evidence from the history, physical examination’ or laboratory tests, of a

specific organic factor judged to be etiologically related to the
disturbance
* Not part of the DSM III.

1.1.2 Short (6 item) orientation-memory-concentration test (Herndon R/MIN. 1999; Katzman et al. 1983):

Items Maximum Score X Weight = Sum

error

1. What year is it now? 1 X 4 =

2. What month is it now? 1 X 3 =

3. Repeat this phrase:

John Modise; Block L, Soshanguve

4. About what time is it? 1 X 3 =

(within 1 hour)

5. Count backwards 20-1 2 X 2 =

6. Say the months in 2 X 2 =

reverse order

7. Repeat the phrase just 5 X 2 =

given

Total (maximum weighted error score=24) =

Score of 1 for each incorrect response

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1.1.3 Causes of the confusional state or delirium:

I. Secondary to systemic disease
i) Organ failure, such as renal, liver or respiratory failure
ii) Intoxication such as with medication, for example, the anti-
cholinergics, dopaminergic agents and digitalis. Other medication
known to cause confusion includes benzodiazpines, digoxin, and
anti-depressants, NSAIDs, opiates, H2-blockers, diuretica (low Na)
(McKhanan et al 1984).
iii) Withdrawal states, e.g., benzodiazepines, alcohol.
iv) Systemic infections such as malaria, typhoid, tick-bite fever and
septicaemia.
v) Metabolic derangements such as hypoxia, hypoglycaemia,
hyponatraemia, hypophosphataemia and hypomagnesemia.
vi) Other causes such as disturbances of calcium metabolism,
thaismine deficiency, pellagra, porphyria, arthritis.

II. Primary central nervous system causes

i) Diseases affecting primarily the subarachnoid space such as
meningitis and subarachnoid haemorrhage (also known as “red-
meningitis”)
ii) Infarctions of specific areas of the brain (e.g., in the posterior
cerebral and right middle cerebral artery distribution)
iii) Epilepsy and postictal states
iv) Head injuries and subdural haematoma

1.1.4 Predisposing factors to the development of the confusional state:

• Age over 60 years, which is associated with a decreased ability to
metabolize and excrete drugs and a decreased reserve of organ function
• Alcohol and drug addiction
• Previous brain injury
• Other, more relative factors such as:
o Psychological stress
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o Sleep deprivation
o Sensory deprivation
o Sensory overload
o Immobilization

1.1.5 Subcategory of “over-activity delirium” :

This may be recognized characteristically by over-activity of cerebral
functions. Its value lies in it pointing to a circumscribed group of underlying
causes.
• Psychic over-activity with prominent visual hallucinations, illusions and
delusions
• Motor hyperactivity with tremor and myoclonic jerks in particular
• Autonomic hyperactivity with tachycardia, excessive perspiration and
hypertension
• Fever is frequently present as a result of underlying infection.
• The affect of the patient is typically that of an apparent fear or anxiety.

Causes of “over-activity delirium”

• Infections, such as malaria, typhoid, septicaemia,tick bite fever and
meningitis
• Withdrawal from drugs particularly alcohol withdrawal with delirium
tremens; also barbiturates, dagga and other drug withdrawals.

1.2 Definition of dementia (McKhann et al., 1984)

Dementia represents a deterioration of memory and cognitive functions when
compared to the patient’s previous level of functioning as determined by the
history of deterioration in capabilities (on a social and working level) and by
dysfunctions that have been determined by clinical examination and
neuropsychologic tests. A diagnosis of dementia cannot be made if delirium,
drowsiness, stupor or coma or other clinical dysfunctions prevent adequate
evaluation of the psyche. Dementia is a diagnosis that is based on behaviour and
cannot be determined by computer tomography, electroencephalography or other
laboratory instruments.
Dementia definition
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1.2.1 Causes of dementia (Adams, 1977):

II. Primary dementia such as Alzheimer’s disease and Pick’s disease.

Alzheimer’s disease has a prominent disturbance of temporal and
parietal lob functions with memory disturbance, spatial disorientation,
and language disturbances (aphasia) in particular. The frontal lobe is
relatively spared and characteristically the patient’s personality may
be preserved until the late stages of the disease. Pick’s disease, or
fronto-temporal dementia, in contradistinction, shows early personality
changes and a lack of social abilities and a later onset of spatial
disorientation.
III. Neurological diseases associated with dementia, such as Parkinson’s
disease, Huntington’s chorea, myotonic dystrophy, multiple sclerosis,
hydrocephalus, Creutzfeld-Jakob disease, frontal lobe tumors,
posterior and middle cerebral artery infarctions, hypoxic
encephalopathy, neurosyphilis etc.
IV. Systemic diseases such as hypothyroidism, hypophosphataemia,
uraemia, pellagra, Wernicke’s encephalopathy, Wilson’s disease, etc.

1.2.2 Subcortical dementia

Dementia may be classified as cortical and subcortical dementia, with
Alzheimer’s disease an example of a typical cortical dementia.

In subcortical dementia, the basal ganglia, deep white matter and thalamic
nuclei may be involved. Patients often show slowness and rigidity of thinking
(bradyphrenia) prominently, and problems with planning and sequencing of
events are found (executive functioning is impaired). The patient often is
withdrawn and appears depressed and visuospatial and perceptial difficulties
are seen.

Examples of subcortical dementias:

• Parkinson’s disease
• Huntington’s disease
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• Vascular dementia
• Progressive supranuclear palsy
• Wilson’s disease
• AIDS dementia complex

1.2.3 Treatable causes of dementia:

About 5-10 percent of patients with dementia may prove to have an
underlying treatable condition such as hydrocephalus, frontal lobe tumours,
thiamine deficiency, vitamin B12 deficiency; hyponatraemia, hypocalcemia,
hypomagnesemia, hypothyroidism, Wilson’s disease, uraemia, hepatic
encephalopathy and depression or pseudodementia. A dementia picture
may be seen as a result of medications, particularly as a result of the use of
phenothiazines, digitalis, anticholinergics and sedatives, amongst others.

1.3 Frontal lobe disorders:

With the help of the history a reasonably good idea may be obtained as to
whether there is the presence of an underlying frontal lobe lesion. On
suspecting the presence of a subtle executive cognitive dysfunction frontal
lobe tests evaluating executive functioning may be employed (Royal DR,
1992).

Focal motor epilepsy may point to a lesion involving the precentral gyrus.

Broca’s aphasia may point to a lesion involving the posterior part of the
inferior frontal gyrus or L Sylvian fissure region.

Paresis (monoplegia) of the arm, the leg or the face – these focal
weaknesses suggest a likely involvement of the motor cortex.

Cerebral diplegia or the clinical picture of cortical paraplegia. This clinical

picture is found with pathological conditions, which involve the medial
aspects of the motor corticices bilaterally. The causes of the so-called
cerebral paraplegias include: hydrocephalus, bilateral anterior cerebral
97

artery infarction (e.g., secondary to rupture of an anterior communicating

artery aneurysm), parasagittal meningioma, superior sagittal sinus
thrombosis and cerebral diplegia (Little’s disease, which represents a form of
cerebral palsy following upon hypoxia in a premature neonate).

Conjugate deviation of the eyes towards the side of the acute frontal lobe
lesion may be encountered in association with depression of the level of
consciousness. In seizures, where there is stimulation of the cortical frontal
eye field area, the eyes may deviate away from the side of stimulation.

Dementia associated with the appearance of primitive reflexes (e.g., grasp

reflex, snout reflex and suction reflex) and slow reaction times may be a
prominent feature encountered in extensive bilateral frontal lobe damage as
seen e.g., with a “butterfly glioma”, where there is a spread of tumor across
the corpus callosum, involving both frontal lobes resembling a butterfly on
CT scanning, and also seen in the late stages of Alzheimer’s disease.

1.4 Parietal lobe disorders:

The following features in the history may point to the diagnosis of parietal
lobe disease:
1. Spatial disorientation (right sided lesion)
2. Dressing apraxia (right sided lesion)
3. Constructional apraxia – inability to draw figures (right sided lesion)
4. Denial of the existence (anosognosia) of hemiplegia (right sided lesion).
5. Right – left disorientation (left sided lesion)
6. Inability to do arithmetic calculations, i.e., acalculia (left sided lesion)
7. Wernicke or sensory aphasia (left sided lesion)
8. Finger agnosia (left sided lesion)

Gerstmann syndrome consists of one or more of the following features

e.g.,
(1) Finger agnosia
(2) Right-left disorientation
(3) Acalculia, and
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(4) Agraphia and usually points to the presence of a dominant parietal lobe
lesion (originally thought to be due to a lesion of the angular gyrus).

1.5 Temporal lobe disorders:

The following features in the history may point to the diagnosis of temporal
lobe disease:

Transient global amnesia

Temporal lobe epilepsy (See Chapter 2, Section 3.1.2)
Wernicke’s aphasia (See Chapter 4, Section 4.3)

Transient global amnesia (Frederiks, 1993; Hoffman et al 1996; Jensen,

1981; Simon and Hodges,2000):
In transient global amnesia the patient may present with an abrupt onset of
global amnesia, repetitive questioning and disorientation in time. Typically,
the patient forgets what day it is, where he is going to and what the plan for
the rest of the day was going to be. There is a retrograde memory loss for a
period lasting from hours to a few days, and an anterograde memory loss, in
that the patient is unable to lay down new memories in sequence and asks
the same questions repeatedly. Consciousness and self awareness are
preserved and the patient remains communicative. Epileptic features are
absent and no focal neurological deficits are detected. Information from a
reliable witness on observations during the attack are usually required for a
firm diagnosis. The attack resolves within 24 hours. The attack is followed
by a persistent amnesic gap for the event. Soundboard or immediate memory
is characteristically spared. SPECT (single photon emission tomography),
PET (positron emission tomography) and DWMRI (diffusion- weighted MRI)
in the acute phase of transient global amnesia have observed changes in the
medial temporal lobes, thalami and other areas. These disturbances may be
of uncertain etiology, possibly be related to hypoperfusion, ischaemia, ictal
disturbance or complicated migraine. In its pure form it has a good prognosis
and is unlikely to recur.
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1.6 Occipital lobe disorders:

Homonymous hemianopia
There may be a tendency to walk or drive into objects on the side of the
visual field deficit. At times only one half of an object or a face can be made
out.

Cortical blindness (Caplan, 1980)

Transient cortical blindness with an inability to see may result from migraine
or from artery-to-artery embolism in the vertebro-basilar territory involving
both posterior cerebral artery territories. Malignant hypertension
(papilledema with hypertension; some authors would include active stage III
hypertensive retinopathy), eclampsia, or hypertensive encephalopathy, may
sometimes present with cortical blindness (associated radiologically with
posterior leucoencephalopathy, also known as posterior reversible
encephalopathy syndrome or PRES).

Visual-motor apraxia (Caplan, 1980)

In the case of bilateral parietal-occipital lobe lesions, problems are
encountered with correctly assembling visual input into a meaningful whole,
particularly in terms of orientation of visual material in space. There is a
tendency to walk into objects and difficulties are encountered in attempting
to reach out and touch objects. It is indeed sometimes easier to reach for
objects with the eyes closed (eliminating faulty visual input).

Visual hallucinations
Primitive visual hallucinations with the classical fortification spectra and
marching light spectra starting gradually over 5 to 30 minutes, are a typical
feature of migraine. In addition, a homonymous hemianopic visual field
deficit may also be found. Abnormalities in Brodmann areas 18 and 19 are
responsible for the production of more formed visual hallucinations, typically
a result of temporal lobe epilepsy (posterior parts of the middle and inferior
temporal gyri) and are often seen in delirium.
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1.7 Memory disorders

1.7.1 Transient amnesia (Croft, 1973; Wood, 1984):

(i) Alcohol abuse with transient amnesia is typically found in subjects used
to a heavy consumption of alcohol.
(ii) Both temporal lobe and grand mal epilepsy may present with the
clinical features of transient amnesia. It may be the only symptom and
may respond well to therapy.
(iii) Migraine may sometimes present with a picture of transient global
amnesia during an attack.
(iv) Vertebro-basilar stroke with ischaemia of both the medial and inferior
regions of the temporal lobes may sometimes present with a picture of
global amnesia with or without other symptomatology of a stroke in
those regions. The condition of transient global amnesia is referred to
above (2.5)
(v) Psychogenic fugue may be characterized by:
(1) Circumstances where the patient only becomes aware of his
whereabouts days later
(2) Inability to remember past events especially those pertaining
to his life personally, and often the presence of
(3) Underlying life stressors are found.
(vi) Medications such as sedatives, benzodiazepines, phenothiazines and
certain anti-convulsants may be associated with periods of amnesia.

1.7.2 Lasting disorders of memory

(i) Herpes simplex encephalitis has a predilection for the medial

temporal lobes, the orbito-frontal- and limbic areas and may
characteristically be associated with severe short-term memory
impairment.
(ii) Alcohol abuse may result in thiamin deficiency and this may give rise to
the development of Wernicke’s-encephalopathy which may consist of
the triad of:
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1. Korsakoff’s amnestic syndrome with severe short-term

memory loss, which is out of keeping with the degree of
impairment of the other cortical functions.
2. Bilateral cerebellar signs;
3. Eye signs, such as nystagmus, gaze palsy, internuclear-
and ocular nerve palsies.
(iii) “Global amnesia syndrome” may be a lasting consequence of a stroke
in the vertebro-basilar territory.
(iv) The duration of post-traumatic amnesia (PTA) is a good yardstick of the
degree of severity of the head injury as well as of its prognosis.
(v) Post hypoxic encephalopathy
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Chapter 10

1. LANGUAGE ABILITIES AND DISTURBANCES

The evaluation of language
1.1 Screening test for aphasia
1.2 Determination of fluency of language
1.3 Testing ability to name objects
1.4 Testing ability to repeat
1.5 Testing of comprehension
1.6 Testing ability to read
1.7 Testing ability to write
1.8 Testing ability to tell a story

2. NORMAL PERCEPTION, INTEGRATION AND REPRODUCTION OF

LANGUAGE THROUGH VISION AND HEARING
2.1 The perception of language symbols of vision (reading) and their
integration and transit into speech and writing
2.2 The perception of the language symbols of hearing, their integration
and their transit into speech and writing
2.3 Lesions involved in aphasia

3. CLASSIFICATION OF APHASIA
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1. LANGUAGE ABILITIES AND DISTURBANCES

Definition of language
Language is the ability to receive language symbols by means of vision and
hearing at a cerebral hemisphere level, to interpret the symbols at Wernicke’s
area; to send the language symbols through via the arcuate fasciculus to Broca’s
area and to execute the language symbols by means of speech, writing or mime.
Language definition
Definition of aphasia
Aphasia is an acquired disorder of language.
Aphasia definition

The evaluation of language (Benson, 1979):

1.1 Screening test for aphasia

The ability to repeat a sentence represents a very useful screening test
for the possible presence of aphasia. If this ability is intact, the patient
probably does not have a Broca-, a Wernicke- or a conduction aphasia. The
presence of neologisms (new words) and paraphasias (where words and
sounds are incorrectly used) are very characteristic and suggestive of the
presence of an underlying aphasia. If the patient is right handed, and
presents with a right hemiparesis with a communication problem, he
probably has a diagnosis of an underlying aphasia.

1.2 Determination of fluency of language

The language is considered to be fluent if the patient is able to speak
fluently without effort at a rate of about 50 – 200 words per minute with
phrase lengths of five words or more with a retention of the melody of
speech. The lesion with a fluent aphasia is usually situated in Wernicke’s
area (see diagram) or posterior to the Rolandic fissure. Paraphasias may be
seen together with a fluent language and with the substitution of sounds
(literal paraphasias) and the substitution of words (verbal paraphasias)
and the presence of new words (neologisms)[the tell tale pointers to the
presence of an underlying aphasia].
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Language may be considered non-fluent when:

- The phrase-length consists of four or less words
- The patient speaks less than 20 to 30 words per minute
- The melody of speech is lost and
- Speech production is associated with considerable effort. The
lesion is usually located in the Broca language area (see diagram)
or anterior to the Rolandic fissure.

Motor and sensory language areas

Rolandic fissure Wernicke area

Broca area
4

1: Posterior one-third of Sylvian fissure

2: Supramarginal gyrus (embrances elongation of Sylvian fissure)
3: Gyrus angulus (embraces elongation of superior temporal sulcus)
4: Inferior frontal gyrus

1.3 Testing ability to name objects

The ability of naming a number of objects is tested. If the patient is unable to
name the object, it is determined whether he is able to recognize the object.
In word production anomia, there is a problem with the production of the
word while the object is recognized. In semantic (meaning) or dysphasic
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anomia, inability to recognize and name the object is found. In word

selection anomia the word is forgotten (amnestic anomia).

Anomia deficits

1. Word production anomia

2. Semantic anomia
3. Word selection anomia

1.4 Testing ability to repeat

The patient is requested to repeat words, phrases or sentences. The patient
shows an inability to repeat with lesions involving Wernicke-area, the arcuate
fasciculus and Broca-area.

Repetition ability

1: Wernicke area
2: Arcuate fasciculus which connects Wernicke to Broca area
3: Broca area

Some patients are unable to communicate but can still repeat relatively well.
Under these circumstances one considers the presence of a transcortical
aphasia in which one may find echo – and palilalia (repetition of a word or of
the last phrase of a word respectively).
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1.5 Testing of comprehension

The patient is requested to obey simple commands such as the protrusion of
the tongue, lifting up of an arm, placing of the thumb on the nose etc. The
patient is asked to choose the correct item from a row of objects held before
him/her. The examiner may aid the patient by pointing from object to object
until the patient gives a signal that the correct item is pointed at. The patient
is asked to answer yes or no to a number of questions e.g., “Is it night time?”
or “Is it white?” or “Is it a book?” More complex commands (e.g., a three-
step command) may also be evaluated.

1.6 Testing ability to read

Observe for the presence of paraphasias and neologisms, the fluency of the
language, the pronunciation and the ease with which the patient reads and
whether there is understanding for what is being read.

1.7 Testing ability to write

Writing is evaluated for arrangement of letters and words to each other.
Note the prevalence of paraphasias and neologisms, and whether the patient
can read his own writing.

1.8 Testing ability to tell a story

The patient relates the story of e.g., Little Red Riding Hood or Snow White.
With a fluent dysphasia characters and roles are frequently interchanged
with each other.
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2. THE NORMAL PERCEPTION, INTEGRATION AND REPRODUCTION OF

LANGUAGE THROUGH VISION AND HEARING:

2.1 The perception of language symbols of vision (reading) and their

integration and transit to speech and writing

A= Wernicke area
B= Arcuate fasciculus
C= Broca area
D= Subcortical fasciculus connecting Broca area with motor cortex of
speech
E = Motor cortex of the muscles of speech
F = Subcortical fasciculus connecting Broca area with premotor writing
centre
G = Premotor writing centre
H = Subcortical fasciculus connecting premotor writing centre with motor
cortex of the hand’s muscles of writing
I = Motor cortex-representing muscles of writing
J = Transcortical information required to programme Wernicke area
K + L = Transcortical information required to programme Broca area

The light stimulus is perceived at retinal level where light energy is

transformed by the rods and cones into electrical energy which is

conducted via the bipolar and ganglion cells through the optic nerve,
the chiasm and optic tract to the lateral geniculate body. From the

lateral geniculate body, by means of a synapse the impulse conducts

through the optic radiation to the primary visual cortex (area 17),
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towards the secondary visual cortex (area 18) and the tertiary visual
cortex (area 19) and through to Wernicke area (A). Wernicke area is
programmed by the transcortical area (J). Wernicke area sends through
information via the arcuate fasciculus (B) to Broca area (C). The
transcortical area (L and K) programs Broca area. Broca area sends
information via the subcortical fasciculus (D) to the motor cortex of the
speech area and via subcortical fasciculus (F) to the premotor writing
centre (G). This writing centre sends information via subcortical
fasciculus (H) to the writing centre of the motor cortex (I). From the
motor cortex the muscles of speech and writing are activated via the
upper – and lower motor neuron pathways.

2.2 The perception of the language symbols of hearing, their integration

and their transit to speech and writing:

Sound

Cochlear nuclei (pons)

Medial geniculate body

A = Wernicke Area
B = Arcuate fasciculus
C = Broca area
D = Subcortical fasciculus connecting Broca area with motor cortex of speech
E = Motor cortex area of muscles of speech
F = Subcortical fasciculus connecting C with G
G = Premotor writing centre
H = Subcortical fasciculus connecting G with I
J = Transcortical information for programming Wernicke area
K + L = Transcortical information for programming Broca area
109

Sound is conducted via the middle ear to the inner ear where it is received
by the organ of Corti and transformed into an electrical impulse and is
conducted via cochlear ganglion cells towards the cochlear nuclei of the
pons and via the lateral lemniscus towards the medial geniculate body and
onwards to the primary auditory cortex namely Brodmann area 41 and 42.
The information is sent to the secondary auditory cortex, e.g., Brodmann
area 21 and 22, and on to Wernicke area. Wernicke area is programmed by
transcortical information from (J), which helps with the integration of previous
information. From Wernicke area (A) information is forwarded via the arcuate
fasciculus (B) onto Broca language area (C). Broca area is programmed to
function by transcortical areas (K and L). From Broca area information is
forwarded to the motor cortex via (D) and (E) and for writing via (F) towards
the writing centre (G) and via (H) to the motor cortex for writing (I). From
the motor cortex the speech and writing muscles are activated by means
of the upper and lower motor neuron pathways.

2.3 Lesions involved in aphasia

• Lesion of Wernicke-area (A) – Wernicke aphasia

• Lesion of Arcuate fasciculus (B) – Conduction aphasia
• Lesion of Broca-area (C) – Broca aphasia
• Lesion of Transcortical motor area (K and L) – Transcortical motor
aphasia
• Isolation of primary hearing cortex (41, 42) – Cortical word deafness
• Left occipital lobe lesion with right homonymous hemianopia - visual
agnosia with inability to write (J)
• Lesion of subcortical fibre (D) – Aphemia – only sigh or grunt possible,
but can write
• Lesion of premotor writing centrum (G) – Agraphia, but can speak.
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3. SIMPLIFIED CLASSIFICATION OF APHASIA

Motor aphasia

Sensory aphasia

Conduction aphasia

Global aphasia

Broca’s dictum:
The presence of aphasia points as a rule to the presence a focal lesion
of the left hemisphere. An acute onset lesion is usually caused by an
underlying stroke, while a gradual onset lesion is commonly caused by an
underlying brain tumor (e.g., meningioma or glioma) or metastases, or an
underlying granuloma (cysticercosis, toxoplasmosis, tuberculosis or
cryptococcoma).

Stroke

Grade

Space occupying lesion

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Characteristics of the aphasias

Broca Wernicke Global Conduction Transcortical Transcortical
Aphasia Aphasia Aphasia Aphasia Sensory Motor
Aphasia Aphasia
Fluent aphasia No Yes No Yes Yes No
Non-fluent aphasia Yes No Yes No No Yes
Paraphasia/Neurologism - + - + + -
Anomia but recognizes Yes No No Yes No Yes
Objects
Anomia and does not No Yes Yes No Yes No
recognize objects
Ability to repeat Lost Lost Lost Lost Saved Saved
Comprehension Saved Lost Lost Partly Lost Partly saved
saved
Ability to read Lost Lost Lost Lost Lost Lost
Ability to write Lost Lost Lost Lost Lost Lost
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Chapter 11

1. DEPRESION OF LEVEL OF CONSCOUSNESS/COMA

1.1 Pathogenesis
1.2 Assessment of level of consciousness (Glasgow Coma Scale)
1.3 Determining the differences clinically between a space-occupying
lesion (structural) and a metabolic and company coma
1.3.1 Metabolic and company coma syndrome
1.3.2 Space-occupying (structural) coma syndrome
1.4 Brain herniation
1.4.1 Subfalx herniation of the medial frontal lobe
1.4.2 Transtentorial herniation
1.4.3 Medial temporal lobe – transtentorial herniation
1.4.4 Foramen magnum herniation
1.5 Vegetative state
1.6 Approach to coma
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1. DEPRESSION OF THE LEVEL OF CONSCIOUSNESS AND COMA

1.1 Pathogenesis (Plum, 1980):

The level of consciousness records the degree of alertness of the patient.
Depression of the level of consciousness leads to drowsiness, sleepiness
and coma. The ascending reticular activating system which is located in the
tegmentum of the rostral third of the pons; the midbrain; and the medial
thalamic nuclei projects impulses to the cerebral hemispheres via the
thalamus and is associated with the maintenance of wakefulness and
alertness. Structural or functional involvement of the ascending reticular
activating system or the cerebral hemispheres diffusely causes depression
of the level of consciousness and coma.

The following processes may affect the ascending reticular activating

system (ARAS):
* Unilateral cerebral hemisphere space occupying lesion (e.g., cerebral
tumor, cerebral haemorrhage, cerebral infarction with mass effect), which
causes transtentorial herniation of the medial and inferior part of the
temporal lobe, i.e., of the para-hippocampal gyrus with secondary
compression of the midbrain in the tentorial hiatus.

* Posterior fossa space occupying lesions (e.g., haemorrhage, infarction or

tumor of the cerebellum) may cause direct pressure upon the upper
brainstem (and ARAS).

* Rostral intrinsic brainstem lesions, such as a haemorrhage or infarction of

the brainstem may affect the ARAS directly.

* Metabolic and drug causes may affect the ARAS and cerebral hemispheres
simultaneously.
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Conditions that affect the brain extensively, bilaterally and diffusely e.g.,
hypoxia, liver failure, renal failure, hypoglycemia and encephalitis may
disturb cerebral hemispheral and/or brainstem function and consequently
affect the level of consciousness.

DEFINITION OF COMA
(The term coma is derived from Greek and means sleep).
Coma points to a depression of the level of consciousness with increasing
degrees of drowsiness and sleepiness. Difficulty is experienced to arouse the
patient.
Coma definition

• A detailed clinical history and examination is required to assess the

level of consciousness and its chronological course and ascertain
the clinical type and cause of the coma.

1.2 Assessment of the level of consciousness or the degree of the coma

(Teasdale, 1977)

The assessment of the course of the patient’s level of consciousness is of

primary clinical importance. It is established whether the patient’s level of
consciousness is improving, static or deteriorating. With patients
demonstrating primary structural brainstem pathology, brainstem signs are
found early in the course of the coma. With transtentorial herniation the
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presence of cerebral hemisphere clinical signs (e.g., hemiparesis) are

followed by the progressive development of rostral brainstem signs (e.g.,
pupillary dilatation and decerebrate rigidity). With metabolic-and-company
coma clinical lateralizing signs are characteristically absent.

The Glasgow-Coma Scale and the pupillary reactions are used in the
determination of the patient’s level of consciousness.

Quantification of the level of consciousness “Glasgow-coma-scale” (Teasdale, 1977)

EYES Open Spontaneously E4
To verbal command 3
To pain 2
No response 1
BEST MOTOR RESPONSE To Verbal Command Obeys M6

Localizes pain 5
Flexion-withdrawal 4
Flexion-abnormal (decorticate 3
rigidity)
Extension (decerebrate rigidity) 2
No response 1
BEST VERBAL RESPONS To Pain Stimulus Oriented and converses V5
Disoriented and converses 4
Inappropriate words 3
Incomprehensible sounds 2
No response 1
TOTAL 15
Glasgow coma scale

The quantification of the coma scale from three to fifteen gives the so-called
responsiveness count or the EMV sum.
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1.3 Determining the differences between a space occupying lesion and a

metabolic and company coma :

Approximately two thirds of coma patients demonstrate a metabolic

and company clinical syndrome and one third that of a structural (space-
occupying) coma syndrome (Plum, 1980). With the help of a clinical
history and examination it is determined which of the two clinical pictures
the patient shows.

Clinical pictures or syndromes of coma (Plum, 1980)

METABOLIC AND COMPANY SPACE-OCCUPYING OR

COMA STRUCTURAL COMA

Pupillary reaction Normal until late Unilateral dilated pupil suggests

a space-occupying lesion and
transtentorial herniation
Eye position Symmetrical with the eyes in Eyes are asymmetrical,
the forward position (abnormal convergent, divergent or deviate
late) to the sides, upward or
downward

Oculocephalic movements Intact until late Unmasks the presence of a N.

III or N. VI palsy, gaze paralysis
or internuclear ophthalmoplegia
Lateralizing signs e.g., Characteristically absent and Characteristically present
unilateral weakness or may appear late
hyperreflexia or Babinski
response

Fundi Are normal May show papilledema

Etiology Appropriate Appropriate

Tests Biochemical CT brain scan or MRI

Coma-syndromes
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1.3.1 Metabolic and company coma syndrome

The term metabolic and company is a group name for a number of etiological
conditions that have a tendency to show a similar clinical picture. The
metabolic and company clinical coma syndrome may be caused amongst
others by, e.g., drug intoxication, poisonings, electrolyte disturbances,
hypoxia, epilepsy, subarachnoid haemorrhage and meningitis. Lateralizing
signs are characteristically absent in clinical metabolic and company coma
syndrome.

1.3.2 Space-occupying (structural) coma syndrome

Space-occupying coma syndromes may be caused by space-occupying or
structural lesions of the cerebral hemispheres, the posterior fossa structures,
e.g., the cerebellum or by lesions directly intrinsic to the brainstem. These
latter conditions share a number of clinical features with each other in order
to form a recognizable clinical syndrome. The presence of lateralizing
signs is characteristic of the space-occupying coma syndrome.
Lateralizing signs refer to the presence of physical signs such as, e.g.,
hemiparesis; asymmetrical deep tendon and superficial reflexes, pupillary
inequality; gaze deviation; cranial nerve palsies and abnormalities of the
oculocephalic reflexes.

A most valuable test in a patient with coma is to open the eyelids and
observe the position and movement of the eyes for some minutes.
Deviation of the eyes from the symmetrical centrally forward position would
favour the presence of an underlying structural lesion. One may observe the
following:
(1) Skew deviation [vertical divergence of the eyes favoring a brainstem
lesion]
(2) Ocular bobbing [rapid spontaneous conjugate downward movement of
the eyes returning more slowly to the forward position indicating a
lesion of the pons]
(3) Asymmetrical ocular roving [suggesting a VIth or IIIrd nerve palsy]
118

(4) Forced conjugate downward deviation of the eyes [favoring the

presence of a thalamic haemorrhage]
(5) Conjugate deviation of the eyes to one side [favoring an ipsilateral
frontal lobe- or contralateral pontine lesion] or
(6) Phasic nystagmoid eye movements to one side in a comatose patient
may be reminiscent of possible underlying subtle status epilepticus.

Headache, nausea/vomiting and papilledema from the triad of raised

intracranial pressure. The depression of the level of consciousness with
drowsiness and increasing degrees of coma is probably the single most
important sign of an underlying space-occupying lesion and frequently
reflects the presence of the process of transtentorial herniation. A history
of trauma and alcoholism is suggestive of an underlying subdural haematoma.
The prior history of a severe headache of an immediate onset is strongly
suggestive of a subarachnoid or intracerebral haemorrhage. The history of
headache of gradual progression both in degree of intensity and in
frequency requires the exclusion of a space- occupying lesion. The
following associations with headache may suggest a space-occupying
lesion, e.g., presence of nausea and vomiting (excluding migraine); presence
of neurological symptoms or signs; aggravation of headache by exertion,
e.g., sneezing or coughing and early morning headaches.

In patients with a space-occupying lesion a lumbar puncture is contra-

indicated as it increases the risk of brain herniation (hours or days after a
lumbar puncture) as the cerebrospinal fluid may continue to leak to the
extradural space (under pressure). The dura mater is like a rubber sac
around the brain and spinal cord and may continue to leak on being
punctured.
119

1.4 Brain herniation

The following respiratory changes may be observed during the rostral

to caudal course of transtentorial herniation:
• Cheyne-Stokes-respiration (seen with diffuse hemisphere lesions or
diencephalon involvement – usually non-structural)
• Central neurogenic hyperventilation (midbrain involvement).
• Apneustic breathing with prolonged end-inspiratory phase (lesion of the
pons).
• Ataxic respiration in both rhythm and volume (medullary or terminal
phase)

Herniation of the brain

1 = Subfalx herniation
2 = Transtentorial herniation
2 = Foramen magnum herniation

A = Falx cerebri
B = Tentorium cerebelli
C = Foramen magnum
D = Left cerebral hemisphere

1.4.1 Subfalx herniation of the medial frontal lobe

Mass effect (D) displaces the frontal gyri beneath the falx cerebri with the
risk of an ipsilateral anterior cerebral artery compression and infarction.

1.4.2 Transtentorial herniation

Mass effect (D) displaces the parahippocampal gyrus (inferior medial part of
temporal lobe) through the tentorial opening with compression of the
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midbrain and subsequent depression of the level of consciousness. With

anterior located lesions the anterior part of the parahippocampal gyrus
(uncus) herniates more through the tentorial opening with the production of
an associated dilated pupil or third nerve lesion. Posterior mass lesions
produce herniation of the posterior part of the parahippocampal gyrus with
compression of the dorsal midbrain and results in an inability to look upwards
(with or without pupillary light reflex changes) also referred to as Parinaud
syndrome.

1.4.3 Medial temporal lobe – transtentorial herniation

Midbrain
Tentorial opening

Parahippocampal gyrus

E = Anterior transtentorial herniation (uncus compressing Nervus III)

F = Posterior transtentorial herniation (e.g., hydrocephalus and setting
sun sign – inability to look upwards)
G = Parahippocampal gyrus situated medially and inferior on the medial
edge of the tentorium cerebelli compressing the midbrain at this level
between the parahippocampal gyrus and the opposite edge of the
tentorium cerebelli
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1.4.4 Foramen magnum herniation

The clinical picture of foramen magnum herniation is observed at a late

phase of transtentorial herniation and with posterior fossa space-occupying
lesions.

The clinical signs of foramen magnum herniation include

(i) Neck stiffness
(ii) Apnoea
(iii) Hypotension
(iv) Hypothermia
(v) Arrhythmias

1.5 The vegetative state

Definition of the Vegetative State

The diagnosis of the vegetative state may be made by careful clinical observation at a
given moment in time. The patient appears awake with the eyes open and retains the
sleep-wake cycle. Interaction with other people is absent and the patient shows no
focused or voluntary reaction to visual, auditory, touch or painful stimuli.
Vegetative state definition

Persistent vegetative state (PVS). The diagnosis of PVS requires a period

of prolonged observation in which the condition persists for longer than four
weeks. The prognosis of PVS relates closely to the aetiology, the duration of
the state and the age of the patient (Celesia, 1993). Post hypoxic PVS of
duration of longer than three months shows a uniformly poor prognosis.
Post head injury PVS in children requires a 12 month period of observation
(Spudis, 1991).
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1.6 Approach to coma

EMERGENCY TREATMENT

CLINICAL EVALUATION

Lateralizing signs absent Lateralizing signs present

Metabolic and company type of Space-occupying type of coma

coma

CT Brain
Diagnosis Diagnosis
established unknown Scan MRI

* Blood tests
Confirm diagnosis
and treatment * Pharmacological
and toxicological
screening

Diagnosis established Diagnosis unknown

* CT brain scan
Treat * Consider:
Lumbar puncture
and EEG
123
Chapter 12

1. EXAMINATION OF THE HEAD, NECK AND BACK

1.1 Inspection of the head
1.2 Neck movements
1.2.1 Clinical testing for the presence of meningism (neck stiffness)
1.2.2 Causes of meningism (neck stiffness)
1.3 Vessels of the neck
1.4 Muscles/bones of the neck
1.5 Examination of the back
124

1. EXAMINATION OF THE HEAD, NECK AND BACK

1.1 Inspection of the head

The head is examined for signs of trauma and sepsis. It is often easier
to palpate a haematoma than to see it. The form of the skull is observed.
It is essential to measure the head circumference in young children and to
chart it on a percentile chart.

1.2 Neck movements

Neck movements of flexion and extension, of abduction to both sides and

of lateral rotation to both sides are assessed.
Neck stiffness or neck pain of
especially neck flexion is probably
indicative of meningeal irritation and
may point to the presence of an
underlying
subarachnoidal haemorrhage or meningitis.

1.2.1 Clinical testing for the presence of meningism

Meningism is a general term used to refer to

meningeal irritation and is characterized by the
development of neck stiffness and the inability to
perform the straight-leg-raising test (Laseque
test) due to pain experienced over the posterior
aspect of the leg and back.
The Neck-Brudzinski sign is elicited by noticing that with active flexion of
the neck an involuntary flexion of the legs is observed. The lateral-leg-
Brudzinski sign refers to the effect observed with the straight-leg-
raising-test of an involuntary active flexion of the contralateral leg.
125
Kernig-sign may be elicited by flexing
the hip and extending the flexed knee
and observing for the development of
pain over the back and posterior aspect
of the thigh and for flexion of the
contralateral leg.

1.2.2 Causes of meningism

The causes of meningism include the following:

(i) Subarachnoid haemorrhage
(ii) Meningitis
(iii) Encephalitis
(iv) Transtentorial or foramen magnum herniation in patients with
space- occupying lesions or raised intracranial pressure
(v) Tetanus
(vi) Retropharyngeal abscess (side to side neck movements are
frequently limited)
(vii) Urinary tract infections and pneumonia (occasionally).

Definition of viral meningitis

The clinical syndrome of viral meningitis is characterized by:
(1) A febrile illness
(2) Headache and neck stiffness
(3) Clear sensorium without any clinical neurological deficits
3
(4) Cerebrospinal fluid (CSF) total white cell count of generally less than 500/mm ,
lymphocyte predominance, especially later in the illness
(5) CSF protein value of less than 1000mg/L
(6) CSF sugar value higher than half the blood sugar value
3
(7) Peripheral white cell count value of less than 11 000/mm .
Viral meningitis definition
Definition of Encephalitis
Encephalitis shows a similar clinical picture to that of viral meningitis except that the
patient demonstrates the presence of an associated confusional state (or delirium),
focal neurological deficits or epilepsy.
Encephalitis definition
126
Definition of bacterial meningitis
The clinical picture of bacterial meningitis is characterized by the following features
(1) Febrile illness
(2) Headache and neck stiffness
(3) The presence or absence of focal neurological deficits and delirium
3
(4) CSF polymorph count of > 100 – and frequently more than 500/mm [with HIV and
partial antibiotic treatment, low and very low cell counts may be found]
(5) A severely depressed CSF sugar value
(6) CSF protein value frequently higher than 1000 mg/l
3
(7) Peripheral white cell count > 11000/mm with a polymorph pleocytosis
(8) Positive gram stain on CSF or blood culture
(9) Positive capsular antigen test for pneumococcus, meningococcus or
Haemophilus influenza and
(10) Raised CSF pressure of > 180 mm CSF.
Bacterial meningitis definition

Definition of chronic meningitis

Chronic meningitis demonstrates the following features:
(1) Subacute course of onset over days, a week or longer
(2) Febrile illness
(3) Headache and neck stiffness
(4) Presence or absence of delirium, or focal neurological deficits
3
(5) CSF polymorph count of usually < 500/mm
(6) CSF protein of frequently > 1000 mg/l
(7) Lowered CSF sugar < 2.4 mmol/l (or CSF – to blood sugar ratio of < 50 %)
3
(8) Peripheral white cell count of frequently < 10 000/mm
(9) Suspicion of an associated cause e.g., cryptococcus infection (in HIV patients),
tuberculosis or malignant meningitis
(10) Raised CSF pressure.
Chronic meningitis definition

1.3. Vessels of the neck

In patients with stroke the examination of the neck vessels is essential.

The temporal, carotid, radial and other peripheral pulses are palpated. The
neck vessels are auscultated with the patient supine, with placement of the
neck in slight extension, while the patient holds his/her breath. Bruits that
become louder higher up in the neck are of practical importance and
probably point to a stenosis of the internal carotid artery at its origin.
Takayasu’s aortic arteritis (radial pulse asymmetry sought in patients with
stroke) bruits are typically audible over the subclavian arteries.
127

1.4 Muscles/Bones of the neck

Muscle spasm or tension headache may cause local tenderness of the

extensor muscles of the neck, especially at the site of their attachment to
the skull. Local cervical vertebral body disease tends to limit neck
movements in all directions, frequently including the rotation movements.

1.5 Examination of the back

The back of patients with upper motor neuron weakness in the legs
is carefully inspected for the presence of scoliosis; kyphosis and a
spinous- process-step phenomenon together with local tenderness over
the spinae may frequently point to the presence of an underlying vertebral
body disease such as, e.g., tuberculosis, myelomatoses or metastases.
128

Chapter 13

1. THE CRANIAL NERVES (I TO VI)

1.1 Nervus olfactorius
1.2 Nervus opticus
1.2.1 Visual acuity
1.2.2 Visual fields
1.2.3 The normal optic fundus
1.2.4 Abnormalities on funduscopy
1.2.5 The pupillary reactions
1.3 Nervus oculomotorius, trochlearis and abducens
1.3.1 Eye muscle movements
1.3.2 Dysfunctions of eye movements
1.3.3 Diplopia
1.3.4 Evaluation of eye movements in comatose patients
1.3.5 Pupillary reflexes
1.3.6 The eyelids
1.3.7 Nystagmus
1.3.8 Nervus III paralysis
1.4 Nervus trigeminus
1.4.1 Anatomy of Nervus V
1.4.2 Testing of Nervus V
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1. THE CRANIAL NERVES

1.1 Nervus olfactorius (nerve of smell)

Each nasal passage is tested separately (while occluding the other) by sniffing
the test substance (a familiar substance, i.e., coffee, cigarette or soap) actively.
With eyes closed the patient is asked if he can smell anything and whether
he can identify the test substance.

Inability to smell is often due to a local problem. Neurological causes for anosmia
include early Parkinson’s disease and frontal lobe tumors.

1.2 Nervus opticus (optic nerve)

The optic nerve evolves embryologically as an outgrowth of the diencephalon

and consists of brain tissue with axons, which are surrounded by myelin from
oligodendrocytes. Multiple sclerosis has an affinity to affect the oligodendrocytes
and this often involves the optic nerves clinically. The ophthalmic and retinal
arteries supply the optic nerve. Vascular involvement of the optic nerve
is considered as a stroke syndrome as the nerve is regarded to be part of
the central nervous system. Transient monocular blindness (amaurosis fugax)
may be regarded as a transient ischaemic attack while ischaemic optic neuritis
may be looked upon as a type of completed stroke.

1.2.1 Visual acuity

It is mandatory to test visual acuity in any patient with any visual complaints.
The reading of a Snellen- or Jaeger (reading distance) chart forms an integral
part of a general practioner’s assessment of a patient. It is an easy, simple
and essential examination. The visual assessment card is kept ideally in a
diary in the shirt’s pocket, easy at hand. Optic atrophy, optic neuritis, glaucoma
and macular degeneration are some causes of a decreased visual acuity.
130

1.2.2 The visual fields

The visual fields may be tested by confrontation in which the examiner uses his
hands. It is often easier to test the visual fields diagonally with, for example,
the one hand in the outer upper quadrant and the other in the lower outer
quadrant. The eyes may be assessed separately in quadrants. The use of a
redheaded pin will allow the examiner to elicit earlier (subtler) visual field
defects. In patients who are unable to cooperate optimally, one may, for
example, use two well known objects e.g., a banana and orange, each held in a
separate half of the visual field. Ignoring of an object may reflect the presence
of an underlying homonymous hemianopia.

1 = Visual field by which deficits are

named
2 = Temporal retina
3 = Nasal retina
4 = Optic nerve
5 = Optic foramen
6 = Anterior clinoid process
7 = Optic chiasm (superior to sellae turcica)
8 = Dorsum sellae
9 = Optic tract
10= Lateral geniculate body
11= Inferior (temporal) optic radiation
12= Superior (parietal) optic radiation
13= Occipital cortex
14= Internal capsule fibres of optic
radiation
15= Pupillary fibres
16= Edinger-Westphal nucleus
131

Visual field deficits

A. Monocular blindness

B. Bitemporal hemianopia

C. L Homonymous hemianopia

D. L Homonymous hemianopia

E. L Inferior homonymous quadrantanopia

F. L Superior homonymous quadrantanopia

G. L Homonymous hemianopia

Side view of visual fields

Superior visual field (H)

projects to inferior retina (K)
and inferior visual field (I)
projects to superior retina (J).
The superior retinal fibers (J)
track superioriorly through the
optic nerve, chiasm and optic
tract and optic radiation towards
the occipital lobe. From the
inferior retina (K) the visual
fibres run inferiorly throughout
the visual pathways towards the
occipital lobe.
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1.2.3 The normal optic fundus

The optic fundus is examined with an ophthalmoscope. This skill is to be
mastered and used routinely by every doctor and the examination is greatly
facilitated by the regular use of e.g., Mydriacyl drops (e.g., one drop 15-20
minutes before examination). The examiner uses his right eye for the patient’s
right eye and vice versa. The appearance of the optic disc, the retina, the
blood vessels and the macula are assessed.

The optic disc is usually well demarcated from the rest of the retina. The
nasal margin may normally appear less clear. The optic cup is paler than the
optic disc, which has a light pink colour. An enlarged optic cup may mistakenly
give the appearance of a pale optic disc. The colour of the optic disc is judged
by the colour of that part of the disc that lies between the optic cup and the
optic disc margin.

Optic disc margin

Optic cup

Assess colour e.g., for pallor

The optic disc margin is crossed by more

than 12 capillary vessels. The arterial
venous ratio of the retinal vessel
diameters is approximately 2 – 3.
133
The macula is situated 2 – 3 disc diameters to the temporal side of the optic
disc. The macula may be observed by asking the patient to look at the light.
The presence of abnormal pigment in the macular area together with a
decreased visual acuity may point to the diagnosis of macular degeneration.

1.2.4 Abnormalities on fundoscopy

Definition of optic atrophy
Optic atrophy is characterized by the following features:
(1) Visual acuity is characteristically diminished
(2) The optic disc appears pale in colour
(3) A Gunn-pupil sign is found (see below)
(4) A diminished number of capillary vessels (< 7) cross the disc margin.
Optic atrophy definition
Causes of optic atrophy include glaucoma, pituitary tumors,
craniopharyngioma, suprasellar meningioma, and anterior communicating artery
aneurysm and the late phase of ischaemic optic neuritis.
Definition of Papilledema
The characteristics of papilledema include the following
(1) The disappearance of retinal venous pulsations
(2) Engorgement of retinal veins (diameter of veins/arteries > 3/2)
(3) The optic cup becomes swollen and unclear
(4) The margin of the optic disc becomes elevated and unclear
(5) Retinal edema develops
(6) Flame shaped retinal hemorrhages develop near to and at right angles to the
disc margin
(7) During the chronic phase of papilledema the risk of visual acuity loss develops.
Papilledema definition
Causes of Papilledema
The causes of papilledema include the following
(1) Raised intracranial pressure
(2) Retinal venous thrombosis (or engorgement secondary to a high pa CO2 of
chronic obstructive airway disease)
(3) Retinal arterial vascular disease e.g., malignant hypertension and temporal
arteritis
(4) Markedly elevated cerebrospinal fluid protein level as seen exceptionally with
e.g., Guillain Barré syndrome or a spinal cord tumor.
Papilledema causes

Mechanism of papilledema with raised intracranial

pressure
Generalized raised intracranial pressure is transmitted via the cerebrospinal
fluid and subarachnoid space to the optic nerve sheath’s subarachnoid space
and
134

causes compression of the ophthalmic vein with resultant swelling of the

optic disc.

1 Optic nerve
2 Internal carotid artery with ophthalmic and retinal arteries
3 Ophthalmic vein (courses inside optic nerve sheath)
4 Dura mater and arachnoid mater of the optic nerve which reaches up to
the globe of the eye
5 Cerebrospinal fluid space which is continuous with the subarachnoid
space around the brain and spinal cord

Definition of optic neuritis

The characteristic features of optic neuritis include the following
(1) Acute onset of monocular blindness or partial monocular visual field defects
(e.g., central scotoma, latitudinal or quadrantic visual field defects)
(2) Pain over the eye
(3) Gunn-pupil sign (see below)
(4) The optic disc may appear swollen and look like papilledema (e.g., with
ischaemic optic neuritis and ADEM) or it may appear normal in the acute phase
(e.g., as with the retrobulbar optic neuritis of multiple sclerosis)
(5) A picture of optic atrophy appears in the chronic phase.
Optic neuritis definition
135
Retinal hemorrhages
Hypertension causes haemorrhages in the deep fibre layer of the retina,
which runs at right angles to the surface of the retina and appear small and
round.

Diabetes mellitus causes the appearance of small point haemorrhages known

as micro aneurysms and flame-shaped haemorrhages (in the superficial fiber
layer, which runs parallel to the retina).

Subarachnoid haemorrhage with its sudden increase in intracranial pressure

gives rise to large superficial subhyaloid or preretinal haemorrhages. A clear
margin may be visible to the edge of the haemorrhage and this may vary with
the position of the head or gravity.
Subacute bacterial endocarditis may give rise to septic emboli to the retina
and manifest as Roth spots (haemorrhage with a pale centre) or with flame-
shaped haemorrhages.

Retinal
exudates
Soft exudates appear white and cloud-like, with indefinite margins, and are
found, e.g., with malignant hypertension and renal failure
Hard exudates appear clearly demarcated and may possess pigment. These
are seen with hypertensive retinopathy (occasionally in a star shaped form
around the macula) and in diabetes mellitus.

Retinitis
pigmentosa
Night blindness is the prominent symptom. The peripheral parts of the retina,
which contain the rods, are affected more severely and are concerned with
night vision. The pigment changes have the appearance of spider webs or bone
spicules in the peripheral parts of the retina. Electro-retinography may be
supportive of the diagnosis.
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1.2.5 The pupillary reactions

The pupillary light reflexes (These are described in more detail later). The
afferent limb (optic nerve) and the efferent limb (parasympathetic fibres
running with the oculomotor nerve) of the reflex are tested. Optic atrophy is
a cause (afferent pathway) for an absent pupillary light reflex.

The assessment of the Gunn pupil phenomenon (de-afferented pupillary

phenomenon).
The pupillary reaction is observed while the light source is moved from eye to
eye. In conditions that affect the optic nerve, the side of involvement will show a
smaller degree of contraction (of both pupils) than the healthy side.

Patient with left sided optic atrophy:

Light source Light source

Both pupils constrict clearly Both pupils constrict slightly. When
(R eye) the light source is moved briskly
from eye to eye it appears as
though both pupils enlarge with
movement of the light source to the
left (L eye)
The weaker pupillary reaction on the left is probably the result of a smaller number
of afferent optic nerve fibers responding to the light source with a resultant smaller
number of viscero efferent fibers being activated bilaterally to the pupils.
137

1.3 Nervus oculomotorius ([Link]), nervus trochlearis ([Link]) and nervus

abducens ([Link])

These three oculomotor nerves supply the intrinsic and extrinsic muscles of
the eye and are examined together in practice.

1.2.3 Eye muscle movements

The eye muscles are tested in pairs in six primary positions
Horizontal eye movements
TO THE LEFT TO THE RIGHT

Medial rectus Lateral rectus Lateral rectus Medial rectus

(N. III) (N. VI) (N. VI) (N. III)
Vertical eye movements
TO THE LEFT AND UPWARD TO THE LEFT AND DOWNWARDS

Inferior oblique Superior rectus Superior oblique Inferior rectus

(N. III) (N. III) (N. IV) (N. III)
TO THE RIGHT AND UWARD TO THE RIGHT AND DOWNWARD

Superior rectus Inferior Oblique Inferior rectus Superior oblique

(N. III) (N. IV) (N. III) (N. IV)
138

NB: Remember that the oblique muscles in isolation turn the eyeball
outwards, but when the eye movements are tested as here, the obliques
move the eyes inwards.

1.3.2 Disturbances of eye movements

Paralysis of the ocular muscles or of the eye movements is not
categorized into the traditional lower and upper motor neuron syndromes, but
they are divided into:
(1) Supranuclear paralyses (ophthalmoplegias).
(2) Internuclear paralysis (ophthalmoplegia) and
(3) Infranuclear lesions/pareses or paralyses or ophthalmoplegias

(1) Supranuclear ophthalmoplegia

Supranuclear lesions cause paresis of eye movements
(usually paired eye movements).

a) Normal functioning of supranuclear eye movements

The frontal voluntary eye

Brodmann area 8
movement centres (frontal
eye fields) (A and C) are
situated in Brodmann
area 8 (posterior one-third
of the middle frontal gyrus)
and activate the
subcortical gaze centres
(parapontine reticular
formation next to
abducens nucleus) (B and
D).
139

The frontal voluntary eye movement centre (A) on the right activates
the subcortical eye movement gaze centre (B) on the left and initiates a
conjugate movement of the eyes horizontally to the left. The frontal voluntary
eye movement centre (C) activates the subcortical gaze centre (D) and
moves the eyes to the right.

These two systems (AB and CD) are in tonic balance with each other.
A lesion of either of the gaze centres (A or B) leads to an over action of
the gaze centres (C and D) with deviation of the eyes to the right.

b) Destructive lesion of the right frontal lobe (e.g.,

haemorrhage).
The frontal eye movement
Brodmann area 8
centre (A) is destroyed with
interruption of the tract A-B
and over-activity of the

Conjugate gaze to the R unopposed tract CD with

resultant conjugate deviation of
the eyes to the right (the side
of the hemisphere lesion) and
a left sided hemiparesis. The
combination of a conjugate
deviation of the eyes in the
direction contralateral to that of
the hemiplegia is characteristic
of a cerebral hemisphere
lesion (the eyes look towards
the side of the cerebral Intact left frontal eye
movement centre
hemisphere pathology)
(Brodmann area 8).

L hemiparesis Moves eyes to right.

140

Stimulating (epileptogenic) lesion right frontally (e.g., a meningioma)

The supranuclear tracts AB

are overactive with conjugate
gaze of the eyes to the left
(sometimes the head, neck
and at times the patient turns
about following his/her eyes).
This attack is also known as a
fronto-contraversive seizure.

d) Destructive lesion of the right subcortical gaze centre (D) at

pons level e.g., a haemorrhage or tuberculoma.
The supranuclear tract CD is
interrupted and the tract AB
is overactive or unopposed
with resultant deviation of
the eyes to the left (away
from the side of the pons
lesion) and an associated
left hemiparesis is found.
Conjugate deviation of the
eyes is
found to the side of the
IVth Ventricle
hemiplegia (left). The
D
corticospinal tract (CS) is
affected ventrally in the
CS
pons and the subcortical R L
gaze centre (D) or the
parapontine reticular
formation more dorsally.
141

e) Progressive supranuclear ophthalmoplegia (Steele-

Richardson syndrome) (Steele, 1975).

(1) The characteristic features of this syndrome include:

(i) Supranuclear paresis with an inability of the eyes to look upwards,
downwards (colloquially known as “cannot look up or down disease”) or
to the sides. The oculocephalic and vestibular ocular reflexes are
characteristically retained.
(ii) Spastic dysarthria
(iii) Gait ataxia
(iv) Bradykinetic rigidity
(v) Dystonia of the neck and at times of the feet.

(2) Internuclear paralysis or ophthalmoplegia

Internuclear ophthalmoplegia results from a lesion of the medial
longitudinal fasciculus which connects an ipsilateral VIth nerve nucleus with
its contralateral medial rectus nucleus. The side of the medial rectus
muscle paresis points to the side of the internuclear ophthalmoplegia
(the side of medial longitudinal fasciculus involvement). The opposite
abducting eye shows uni-ocular phasic nystagmus (laterally). The eyes are
dysconjugate in the vertical plane (called skew deviation) and diplopia
may be present in many directions of gaze. Multiple sclerosis is an
important cause of internuclear ophthalmoplegia, especially if it is present
bilaterally. A small lacunar brainstem stroke is another frequent cause.

Skew deviation: In the vertical

plane, one eye is higher than the
other. It constitutes a hallmark of
brainstem involvement.
142

Right-sided internuclear
ophthalmoplegia
(R) Internuclear ophthalmoplegia
(medial longitudinal fasciculus lesion)
The left subcortical gaze centre (B)
activates the ipsilateral abducens
nucleus (and lateral rectus muscle) and
the contralateral oculomotor nucleus (and
medial rectus muscle) via the right-sided L esio n R med ial
L on g itu din al
medial longitudinal fasciculus. With a fascicu lu s

lesion of the right medial longitudinal

fasciculus the right medial rectus muscle
is activated only partially with resultant Patient is asked to look to the left
decreased movement of the right-sided
medial rectus muscle.

Medial rectus paresis Monocular nystagmus of

abducting eye

(3) Infranuclear paralysis or ophthalmoplegia

This category may be analogous to the lower motor neuron paralysis and
its anatomical stations of involvement include e.g., the nucleus of the
affected nerve in the brainstem, the oculomotor nerve itself, its
neuromuscular junction and the muscle itself. The oculocephalic eye
reflexes are abnormal and the affected eye cannot move.

1.3.3 Diplopia
Double vision usually points to the presence of an underlying eye muscle
paralysis (paralytic strabismus). The doctor may be alerted to the underlying
presence of an ocular motor paralysis by noting the presence of blepharospasm
(closure) of
143

one eye, which may occur involuntarily. The patient closes one eye to eliminate
the double vision. The presence of strabismus without diplopia (i.e., concomitant
strabismus) points to an oculomotor disturbance since early childhood frequently
associated with decreased vision of one of the eyes (also referred to as
amblyopia of an eye).

It is ascertained whether the diplopia occurs in a horizontal direction (images

next to each other) or vertically (images above each other) and in which of
the six primary eye positions the double vision is present maximally. The
images are separated maximally from each other in the direction in which the
weak eye muscle shows its purest action. By this method of testing the muscle
pair responsible for the diplopia may be identified and one of the two muscles
is paretic. At times it remains difficult to identify the weak muscle and one may
use a spectacle with one red and one green lens to identify the affected eye or
muscle (the false image which is often displaced the furthest, is derived from
the affected eye muscle). If the colour of the false image is red, for example, the
false image is derived from the eye that is covered with the red lens. Vertical
diplopia is frequently associated with abnormalities involving the superior oblique
muscle (e.g., trochlear nerve paralysis; superior oblique muscle myokymia;
superior oblique muscle pulley problem [“Brown syndrome”]).

1.3.4 Evaluation of eye movements in comatose patients

(1) Doll’s eye movements (Oculocephalic
reflexes).
The testing of oculocephalic reflexes is a technique of assessing brainstem
function in the unconscious patient (in whom the voluntary frontal eye
movement centres are in reality “switched off”).

The term doll’s eye movement has its origin in the dolls whose eyes
move with the movement of the head in the vertical direction. The principle
that the eyes move in the opposite direction than the head is also applicable
to patients in coma.
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Doll’s eye or oculocephalic movements

Doll’s eye The doll’s head moves The patient’s head The patient’s head
resting backwards (A) and the moves backwards (A) moves forward (A) and
position eyes forwards (B) and the eyes forward the eyes upward (B)
(B)

The same principle that applies for doll’s eye movements in the vertical plane
is also applicable to horizontal movements, namely that the eyes move in the
opposite direction to that of the head (e.g., with movement of the head to the
left or the right).
Horizontal doll’s eye movements or oculocephalic reflexes (in patients in
coma).

The head is turned towards the

Head
right and the eyes move
conjugately to the left (brainstem
is intact)
The eyes move conjugately to the left
145
The head is turned to the left and Head

the eyes move conjugately to the

right (brainstem is intact).

The eyes move conjugately to the right

If the patient is awake, the doll’s eye movement maneuver becomes invalid
as the patient can look voluntarily just where he wants to look (the reflex
movements are overruled).

The patient with e.g., a right- Head

sided oculomotor [Link] paralysis
(in coma). The head is turned to

the right and the expectation is

that the eyes move conjugately to The right-sided medial rectus paresis is
the left. observed.

Head
The patient with a right-sided
abducens paralysis (in coma):

The head is turned to the left with

the expectation that the eyes will The right-sided lateral rectus paralysis
turn conjugately to the right. can be seen.

The patient with a right-sided

Head
lesion of the pons (in coma)
(with a gaze paralysis to the
right). The head is turned to the
left with the expectation that the The eyes do not turn conjugately to the

eyes will turn conjugately to the right.

right.
146
With movement of the head to the Head
R, the eyes move conjugately to
the left.

The patient demonstrating clinical brain death (on ventilator)

This patients with brain death, the oculocephalic reflexes are

absent The head is turned to the left and
towards the right without any movement
of the eyes (the eyes move passively
with the head. The in-built doll’s eye
movements (oculocephalic reflexes) are
absent. This absence of oculocephalic
reflexes may be imitated by:
(i) Use of muscle relaxants
(ii) Use of anti epileptic drugs e.g., phenytoin, phenobarb, carbamazepine
and sedatives e.g., benzodiazepines
(iii) Hypothermia
(iv) Bilateral eighth nerve (N. VIII) lesions e.g., secondary to meningitis
or aminoglycoside poisoning

Bilateral internuclear paralysis will show a bilateral medial rectus paresis

(in coma).

A right-sided medial rectus Head

paralysis is demonstrated by moving
the head towards the right.
147
The left-sided medial rectus
Head
paralysis is demonstrated by moving
the head towards the left.

Bilateral internuclear ophthalmoplegia during coma is seen at times during

the course of a transtentorial herniation and with drugs such as barbiturates and
phenytoin.

(2) Caloric testing of the vestibular

system
This tests eye movements and brainstem function (vestibular system) in
unconscious patients.

The technique of caloric testing

• The eardrums are checked for

intactness.
• The external auditory canal is R
irrigated with ice water for several
seconds. The cold stimulus is
translated via the eardrums towards Cold water

the semicircular canals.

• The eyes are inspected for the
development of conjugate eye
movements.
The eyes deviate towards the
• Nystagmus of the jerk type is
direction of the cold water
observed towards the opposite
stimulus.
direction (of the cold water stimulus)
in awake patients, but disappears
with coma (the nystagmus is
dependent on the saccadic voluntary
eye movement centre).
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1.3.5 Pupillary reflexes

(1) Abnormalities of the pupillary light reflex include a [Link] palsy,

Horner syndrome and syphilis.

Findings with a Third Nerve Palsy

The characteristic features of an oculomotor nerve palsy include
(1) Enlarged pupil which is non-reactive to light stimulation
(2) Dense or severe degree of ptosis
(3) Displacement of the eye to the outward and downward position (with the eyes
looking forwards) and
(4) Paralysis of the superior-, inferior- and medial rectus muscles and of the
inferior oblique.
Third nerve palsy finding Oculomotor palsy
Features of a Horner Syndrome (sympathetic denervation)
The features of a Horner syndrome include the following:
(1) A smaller pupil than the normal side
(2) A retained reaction of the pupil to light
(3) A ptosis of a mild degree in contradistinction to the dense ptosis of a third
nerve palsy and
(4) Anhydrosis of the forehead (with involvement of the sympathetic pathway
proximal to the common carotid artery’s bifurcation).
Horner syndrome features
Causes of a Horner syndrome
(1) Involvement of the first sympathetic neuron, which courses from the posterior
hypothalamus, and run through the lateral brainstem and lateral cervical
spinal cord, up to the intermediary lateral column of the spinal cord at the T1
level. Causes of a Horner syndrome at this level include:
(i) Strokes of the lateral medulla, pons and midbrain and
(ii) Cervical spinal cord injuries.
(2) Involvement of the pre-ganglionic sympathetic neuron by:
(i) T1 radiculopathy
(ii) Enlarged supraclavicular neck glands.
(3) Involvement of the post-ganglionic neuron by:
(i) Enlarged supraclavicular lymph glands
(ii) Internal carotid artery thrombosis (sympathetic fibers are affected in the
wall of the artery)
(iii) Cluster headache (sympathetic fibres are compressed in the carotid
canal with carotid dilatation)
(iv) Cavernous sinus thrombosis (where sympathetic fibers accompany the
ophthalmic division of V to the orbit). Forehead sweating remains intact
with cavernous sinus involvement.
Horner syndrome causes

With tertiary syphilis of the brain Argyll-Robertson pupils may be found (“Ar”
stands for “accommodation retained” and “ll” for “light-lost”). The pupils are
149

usually small and irregular but may occasionally be enlarged. The anatomical
area involved is unclear. It is probably located in the efferent pathway possibly
at midbrain tectum level or more peripherally at distal innervation level of the
iris muscle.

The patient is asked to change his focus from a distant object to a nearby
point e.g., his/her nose and one observes how the pupils constrict and the eyes
converge.

(2) The accommodation reflex

(3) The ciliospinal reflex (sympathetic reflex)

The application of a painful stimulus to the neck e.g., pinching of the skin
of the neck causes a dilatation of the ipsilateral pupil.

1.3.6 The eyelids

An approach to ptosis (drooping of the upper eyelid)

Determine whether the patient shows
(1) An unilateral or a bilateral ptosis
(2) Involvement of the pupil (a third nerve lesion dilates the pupil while a Horner
syndrome constricts it)
(3) Associated over-action of the frontalis muscle (with myasthenia gravis the
frontal muscle may also be affected).
(4) Blepharospasm of the orbicularis oculi muscles, which may falsely mimic a
ptosis. The lower eyelid on the affected side lies at a higher level. In these
instances the causes of blepharospasm are assessed.
Ptosis an approach

The causes of ptosis include the following:

(1) A third nerve palsy due to a berry aneurysm of the posterior

communicans artery (pupillary involvement) and diabetes mellitus (with
sparing of the pupil).
(2) Horner syndrome
(3) Myasthenia Gravis
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(4) Muscle diseases e.g., progressive external ophthalmoplegia, which

is usually caused by a mitochondrial myopathy, myotonic dystrophy and
centronuclear congenital myopathy.

1.3.7 Nystagmus

Nystagmus definition

Nystagmus is a rapid, involuntary, usually conjugate movement of the eyes, which

may show a phasic, rotatory or pendular quality.
Nystagmus definition

Phasic or jerk
nystagmus
The eyes oscillate to and fro between fast and slow phases. The direction of
the fast phase signifies the direction of the nystagmus. A vertical upward
nystagmus with upward gaze (the fast phase is upwards) points to an intrinsic
brainstem lesion usually at the ponto-medullary junction (phenytoin intoxication
may rarely be a cause). Vertical downward nystagmus with downward gaze
(accentuated with lateral gaze – Daroff’s sign) is characteristic of a cranio-
cervical junction lesion such as basilar impression or Arnold Chiari
malformation. Horizontal jerk nystagmus with gaze to both sides is found with
diseases of the cerebellum, with brainstem lesions and with drug intoxication
(phenytoin). Peripheral vestibular conditions tend to give rise to a jerk
nystagmus in the direction opposite to that of the lesion.
Rotatory
nystagmus
The eyes show an oscillatory jerk movement in which the clockwise and
anti- clockwise movements alternate with each other. Rotatory nystagmus
usually points to involvement of the vestibular system.
Pendular
nystagmus
The oscillations are pendular to and fro and frequently maximal with fixation of
the eyes on an object in the straight forward position. Pendular nystagmus
develops frequently at an early age and is associated with pathology of the
retina, the optic nerves or with congenital nystagmus.
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1.3.8 Nervus III – Paralysis

A = Sellae tursica
B = Dorsum sellae
C = Cavernous sinus
D = Superior orbital fissure
E = Interpeduncular cistern
F = Tentorium cerebelli
G = Os petrosus

Clinical picture of an oculomotor nerve paralysis

The characteristics of an oculomotor palsy include:
(1) Dense ptosis
(2) Dilated non-reactive pupil (mydriasis)
(3) Position of the eye downward and outward (paralysis of the medial-,
inferior- and superior recti muscles and of the inferior oblique)

Causes of oculomotor nerve paralysis (N. III)

Anatomical area involved Causes
• Nucleus of N. III (1) • Wernicke encephalopathy
• Midbrain tegmentum (2) • Stroke, e.g., an ipsilateral N. III paralysis
with contralateral hemiplegia (Weber-
syndrome)
• Inter-peduncular cistern (3) • Menigitis (e.g., TB)
• Tentorial edge • Parahippocampal gyrus herniation
(ipsilateral) dilatation of pupil occurs
early
• Just proximal to cavernous sinus • Posterior communicans artery aneurysm.
Most important and common cause of
isolated N. III paralysis
• Cavernous sinus (4) • Cavernous sinus thrombosis with
oedema of the conjunctiva, proptosis and
deficit of N. III, N. IV and VI. If patient
suffers from diabetes mellitus the fungal
infection mucormycosis will be the most
likely cause
• Superior orbital fissure (5) • Disease of bone such as Pagets and
sclerostosis (marble bone disease)
• Neuromuscular junction (6) • Myasthenia Gravis
• Muscle disease (7) • Myotonic dystrophy, endocrine,
ophthalmoplegia
Oculomotor nerve palsy Third nerve palsy
With diabetes mellitus, a third nerve palsy with pupillary sparing may be found
(infarction of nerve).
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1.4 Nervus Trigeminus (Nervus V)

1.4.1 Anatomy of nervus trigeminus

A = Midbrain mesencephalic nucleus and tract of N. V
(proprioception) B = Pons. Chief sensory nucleus of N. V (light
touch)
C = Medulla. Medullary nucleus and tract of N. V (pain and
temperature)
The trigeminal nerve consists of a motor and a
sensory component. The motor nucleus is situated
in the tegmentum of the middle one-third of the pons.
The motor root of N.V exits ventrolaterally from the
pons and courses inferior to the trigeminal ganglion
(Gasser) and exits the skull via foramen ovale and
supplies the muscles of mastication. The sensory
nucleus and tract of nervus (V) are situated
laterally in the brainstem from the midbrain up
to the upper cervical segments.

The trigeminal ganglion lies on the medial part, and anterior

(Meckle’s cave) to the os petrosus and connects
proximally via the trigeminal root with the nucleus and tract
of N.V and distally with the three sensory divisions. The
three subdivisions of the trigeminal nerve are the
ophthalmic (V1), maxillary (V2) and mandibular (V3)
branches. The ophthalmic division of the trigeminal nerve
transmits sensation from the forehead and courses via
the orbit, the superior orbital fissure, and the cavernous
sinus to the trigeminal ganglion. The maxillary devision
transmits sensation from the upper part of the face via
foramen rotundum to the trigeminal ganglion. The
mandibular division conducts sensation from the lower
face (over the jaw region) via foramen ovale to the
trigeminal ganglion.
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1.4.2 Testing the trigeminal nerve

a) Sensory testing
The ophthalmic (V1), maxillary (V2) and mandibular (V3) divisions of
the trigeminal nerve are tested for the sensory modalities of light touch, pain
and temperature. The two sides are compared with each other. If all
three sensory areas are affected it points to a lesion at or proximal to the
trigeminal ganglion. With individual branch involvement the lesion is usually
at or distal to the trigeminal ganglion. Lesions that are located in the
supratentorial compartment of the cranial cavity refer pain via their
connections with the ophthalmic division towards the forehead or eye
region.

b) Testing the motor branch of the trigeminal nerve

On inspection atrophy may be observed of the temporal and masseter
muscles as a concavity over the temporal regions and as flattening over
the angle of the jaw. The temporalis and masseter muscles are inspected
and palpated while clenching the teeth. While opening the mouth against
resistance (separating the lips at the same time to visualize the centres
of the top and bottom rows of teeth) one observes for displacement of the
jaw towards one or other side. The jaw displaces to the side of the
paralyzed muscles (the healthy lateral pterygoid muscle displaces the jaw
towards the paralyzed side). With myasthenia gravis the mouth may at
times hang open and the patient may hide this sign by supporting his chin
with his hand.

c) Testing of trigeminal nerve reflexes

The corneal
reflex
Discuss the test that is to be undertaken with the patient. Stimulate the
cornea with a wisp of cotton wool (supporting the examiners medial
three fingers against the patient’s cheek) and moving the wisp of cotton
whool gently and controlled from the white sclera to the edge of the
cornea)
154

observe the blinking response of both the eyelids. The afferent limb of
the reflex arc is subserved by the ophthalmic division of the trigeminal nerve
and the efferent limb by the facial nerve innervating the orbicularis occuli
muscles.

The jaw
reflex
The mouth is held relaxed in the slightly open
position. The examiner's index finger is placed
between the lower lip and the tip of the jaw. The
reflex hammer is tapped downwards against the index
finger and contraction of the muscles of mastication
is noted, pulling the jaw upwards. The jaw reflex may
normally be absent or slightly noticeable. With
pseudobulbar palsy the jaw reflex may be increased
or show clonic movements.
155

Chapter 14
CRANIAL NERVES VII - XII

1. NERVUS FACIALIS (N. VII)

1.1 Anatomy of the facial nerve
1.2 Motor function of the facial muscles
1.3 Bell’s palsy
1.4 Upper motor neuron facial paralysis

2. THE VESTIBULO-COCHLEAR NERVE (N. VIII)

2.1 The anatomy of the vestibulo-cochlear nerve (N. VIII)
2.2 Testing of vestibular function
2.2.1 Caloric testing
2.2.2 Positional vertigo and nystagmus
2.2.3 Other tests
2.3 Testing of hearing
2.3.1 Whisper test
2.3.2 Rinne test
2.3.3 Weber test
2.3.4 Other tests

3. THE GLOSSOPHARYNGEAL (N. IX) AND VAGAL (N. X) NERVES

3.1 The anatomy of the glossopharyngeal nerve
3.2 The anatomy of the vagal nerve
3.3 Testing of the glossopharyngeal and vagal nerves

4. NERVES ACCESSORIUS (N. XI)

4.1 The anatomy of nervus accessorius
4.2 Testing the sternocleidomastoid muscles
4.3 Testing the upper third of the trapezius muscle

5. THE NERVUS HYPOGLOSSUS (N. XII)

5.1 The anatomy of nervus hypoglossus
5.2 Testing the tongue
5.3 Lower motor neuron paralysis of the tongue
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5.4 Upper motor neuron paralysis of the tongue

6. BULBAR PALSY

7. PSEUDO-BULBAR PALSY

8. GENERAL ANATOMY OF THE CRANIAL NERVES: The anatomical

arrangement of the cranial nerve nuclei in the brainstem
8.1 Basic pattern of arrangement of neurons (nuclei) in the spinal cord

8.2 Schematic representation of cranial nerve nuclei in medulla oblongata

8.3 Pons represented schematically as unfolded spinal cord

8.4 Schematic representation of midbrain

8.5 Schematic representation of motor nuclei in longitudinal sections

8.6 Schematic representation of sensory nuclei of brainstem in
longitudinal section

9. THE CORTICO-SPINAL TRACT AND THE BRAINSTEM

10. THE SPINO-THALAMIC AND SYMPATHETIC TRACTS; NUCLEUS AND

TRACT OF TRIGEMINUS AND THE BRAINSTEM
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1.1 Anatomy of the facial nerve

The facial motor nucleus is situated in the dorsum of the tegmentum of the
lower one third of the pons. The facial nerve courses as a dorsal loop
around the abducens nucleus and procedes ventrally to exit at the ponto-
medullary junction between the abducens nerve medially and the vestibulo-
cochlear nerve laterally. The facial nerve courses through the cerebello-
pontine angle cistern to enter the internal acoustic meatus. From the internal
acoustic meatus the facial nerve courses in the petrous temperal bone, first
laterally, then posteriorly and finally inferiorly to exit from the skull via the
stylomastoid foramen. The facial nerve courses through the parotid gland
and innervates the muscles of the face. The facial nerve supplies a small
area of sensation as can be seen with herpes zoster infection of the facial
nerve, when herpetic vesicles may be found in the external ear canal and
earlobe, betraying the somatosensory supply of the facial nerve to the
region.

A viscero-efferent supply courses with the facial nerve from the superior
salivary nucleus, via nervus intermedius and chordae tympani to the sub-
mandibular and sublingual salivary glands and via the greater superficial
petrosal nerve to the lacrimal gland.

Viscero-afferent fibres from the taste receptors of the anterior two thirds of
the tongue course centrally towards the tractus solitarius of the brainstem.
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1.2 Motor function of the facial muscles

The face is inspected for asymmetry of the frontalis

muscles, the nasolabial folds, the lips and the corners
of the mouth and for drooling.
The following tests are undertaken:
- Forehead frowning
- Closing eyelids tightly (feel resistance of eyelids
with thumbs)
- Forced smiling, moving the corners of the mouth
laterally
- Whistling with natural smile
- Piercing together of the lips (feel resistance with
thumbs)
- Blowing up of cheeks (feel resistance against
cheeks)
- Contracting chin downwards (platysma muscles)

1.3 Bell’s palsy

The acute onset of a lower motor neuron paralysis of the facial nerve is
called a Bell’s palsy. It affects the muscles of both the lower and upper half
of the face. A Bell’s sign refers to the inability to close the eyelids and the
appearance of the white sclera between the eyelids due to the reflex upward
movement of the eye during eye closure. A Bell’s sign is pathognomonic of
a lower motor neuron facial palsy.
Associated features of a Bell’s palsy may include:
• Decreased or absent lacrimation (lesion proximal to the geniculate
ganglion)
• Hyperacuses (involvement of the branch to the stapedius muscle, distal to
the geniculate ganglion but proximal to the chordae tympani branch)
• Loss of taste over the anterior two thirds of the tongue (with chordae
tympani branch involvement)
• Vesicles in the external ear canal and ear lobe due to herpes zoster
infection.
159
• Testing for the presence of so-called synkinesis: e.g., During forceful
closure of the eyelids there is the simultaneous contraction of the muscles
of the corner of the mouth or vice versa (favoring the presence of a lower
motor neuron lesion associated with a faulty re-innervation or ephaptic
transmission phenomenon of the facial nerve). This is usually seen in a
patient who has had a previous Bell’s palsy.
• Hypoaesthesia may be found over the face due to a not infrequently
associated Vth nerve involvement as part of a so-called viral “polyneuritis
cranialis”.

1.4 Upper motor neuron facial paralysis

With an upper motor neuron facial palsy the lower half of the face (LHF) is
paralyzed (following the same pattern as the limbs) and the upper half of the
face (UHF) (frontalis muscle) is spared (following the same pattern as the
other motor cranial nerve muscles with their bilateral corticobulbar
connections).

Upper and lower motor neuron facial nerve paralysis

• A lower motor neuron paralysis of
the facial nerve affects the whole
of the face i.e., LHF+UHF
• An upper motor neuron facial
paralysis (of e.g., the corticobulbar
CBT
tract, CBT, on the right) involves
the muscles of the lower half of the
face (LHF) on the contralateral
side (left). The upper half of the
MUHF
MUHF facial muscles (UHF or frontalis) is
spared due to the innervation it
MLHF
MLHF
receives from the ipsilateral
corticobulbar tract (left).
MUHF= Muscles of the upper half of the
face
MLHF = Muscles of the lower half of the
face.
160
The facial muscles receive a double corticobulbar innervation, namely a
tract that subserves voluntary movement of the face and another which
subserves the emotional or mime (e.g., smiling), part of the face. In practice
one may observe dissociation between these two components as the two
corticobulbar tracts partake in different anatomical pathways. The patient
may, for example, show a paralysis of voluntary facial movement while the
emotional component may be intact or vice versa. The face with its voluntary
and emotional (body) language has been referred to as: “the masterpiece of
God”.

2. THE VESTIBULO-COCHLEAR NERVE ([Link])

2.1 The anatomy of the vestibulo-cochlear nerve ([Link])

The vestibulo-cochlear nerve consists of two functional components namely

the hearing (auditory)- and the vestibular (balance) parts. The cochlea is
connected to the cochlear nuclei in the lateral pons, which connects with the
medial geniculate body via the lateral lemniscus and onto the medial
geniculate body towards the transverse gyri of Heschl of (auditory cortex)
which is situated on the superior surface of the superior temporal gyrus
(within the Sylvian fissure).

The vestibular impulses from the utriculus and semi-circular channels are
transmitted to the vestibular nuclei of the brainstem. The vestibular nuclei
have connections with the flocculo-nodular lobe of the archicerebellum
(concerned with balance of the trunk), with the spinal cord via the vestibulo-
spinal tract (concerned with the development of decerebrate rigidity) and
with the oculomotor nuclei (concerned with the vestibular effect on eye
movements) and may present in the awake person with nystagmus and in
the unconscious person with deviation of the eyes.
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2.2 Testing of vestibular function

The clinical symptoms of vestibular dysfunction may include the following:

• Vertigo with accompanying sympathetic over-activity (tachycardia,
paleness and sweating) and nausea and vomiting
• Ataxia associated with the vertigo
• Nystagmus of the rotation or jerk type
• Positional vertigo and nystagmus (evoked by a change in position)

2.2.1 Bedside vestibular tests:

• Examine the patient for nystagmus under the following circumstances:
(1) Sitting position
(2) Supine position on the back and in the L- and R lateral positions (for at
least 30 seconds in each position)
(3) The Dix-Hallpike maneuver

30°

30°

Dix-Hallpike maneuver
The patient sits on the examination couch; legs are pointed towards the foot
end of the bed, the head is turned 30°to the left or the right, and while the
examiner stabilizes the patient’s head between his hands, the patient is
moved backwards past the head end of the bed to a level 30°below the
horizontal. The patient is observed for the development of nystagmus and
162
vertigo. The latency for the development of nystagmus, its duration and
fatiguability are noted.

Differentiating between peripheral and central vertigo nystagmus with

Hallpike maneuver
1. Benign positional vertigo and nystagmus (peripheral)
A short latent period of several seconds is noted before the vertigo and
nystagmus develop and the nystagmus only lasts several seconds. On
repetition of the test fatiguability of the response is noted. The ear that
is situated inferiorly points to the side of the pathology. Cupulolithiasis is
the commonest cause of benign positional vertigo and nystagmus
although it may also be caused by head injuries, infective and vascular
lesions of the vestibular system.
2. Central positional vertigo and nystagmus
With the Hallpike maneuver the vertigo and nystagmus develop
immediately, are of longer duration and do not accommodate with
repetition of the test.

Therapeutic particle repositioning maneuver (Parnes Price Jones-, Epley- or Semont

maneuvers) (Parnes, 1993; Fife et al, 2000)
Should the Hallpike maneuver test turn out to be positive, one can perform the “therapeutic
particle repositioning maneuver” for benign paroxysmal positional vertigo and nystagmus.
Should the vertigo and nystagmus develop with right ear in the lower position, one would
assume that it is the right posterior semicircular canal that is the offending or pathologic
side. One would then keep the right ear in the lower position with the head turned 450 to the
right, and the head hanging 300 below the horizontal for about a 2 minutes period; the head
would then be turned 900 to the left and again held in that position for a period of about 2
minutes (the head was then turned from the right lateral position to the left lateral position
through a 900 angle); then the whole body and the head is again turned a further 900 to the
L and held in that position for another 2 minutes period. The patient is instructed to assume
the upright position and not to bend over forwards for a period of about 48 hours. He is
asked to sleep in the sitting position. He is also asked to avoid lying on the side that was
affected. This maneuver often leads to the dramatic clearing of the positional vertigo.
Should the symptoms still be present, or recur, the maneuver could be repeated as
necessary.
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3 The head thrust sign: The head thrust sign entails horizontal (jaw-plane) high
acceleration head thrusts, alternately to the R or L, while the patient
maintains gaze on e.g., the examiner’s nose. During this maneuver, one
looks for a “catch up” saccade when the head is rapidly turned toward the
lesioned side.
4 Head-shaking nystagmus: This maneuver may demonstrate vestibular
asymmetry. The head is vigorously turned back and forth horizontally with
the eyes closed for about 30 seconds, to “charge” the brainstem’s velocity
storage mechanism. Upon stopping and opening the eyes (preferably using
Frenzel lenses), nystagmus usually beats away from the lesioned side (such
nystagmus is absent in normal subjects).
5 Vestibular ocular reflex (VOR) assessment: The vestibular-ocular reflex
serves a very specific function, to stabilize gaze in space during head
movements. In general the clinical detection of vestibular damage will be
easier the more severe and the more acute the lesion is.
1. Assessment of slow doll’s head maneuver: The eyes are observed while
the patient is sitting in front of the examiner and is fixating his eyes on
e.g., the examiner’s nose. The examiner then oscillates the patient’s head
from side to side, at a frequency of about 0.5-1Hz (per second). In the
absence of a VOR the patient’s eye movements will not be smooth but
will be interrupted by “catch up” saccades towards the fixation target
(these head velocities of 94-188 degrees per second are too high for the
pursuit and cervico-ocular reflex to compensate). Bilateral VOR deficits
(gentamycin ototoxicity & meningitis) give a positive maneuver (patients
show an unsteadiness in the dark and oscillopsia while moving fast).
2. Assessment of dynamic visual acuity: This test is used for subjects
suspected of having bilateral vestibular loss and the abnormality
correlates with the finding of oscillopsia. If on a similar horizontal doll’s
eye maneuver the visual acuity drops two to three lines from baseline it
suggests an abnormality of the vestibular ocular reflex.
3. Ophthalmoscopy: The powerful magnifying power of the ophthalmoscope
allows one to detect nystagmus of a small amplitude readily. Remember
that its direction is inverted. By keeping the other eye closed with the
patients hollow hand and examining the eye with a light source, the
focusing mechanism of the eyes is neutralized (similar effect as with
Frenzel glasses [10+ diopter lens]) augmenting the amplitude of the
vestibular induced nystagmus)
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2.2.2 Quantitative Caloric- and rotational chair testing (gold standards)-laboratory tests
Please refer to chapter 13, Section 1.3.4(2).
Electronystagmography (ENG)/infrared video-nystagmography (IRV)) includes the
following tests:
(1) Caloric irrigation

(2) Testing for spontaneous and positional nystagmus

(3) Smooth pursuit tracking

(4) Saccadic eye movements and

(5) Optikokinetic nystagmus

Vestibular testing consensus recommendations on indications (Fife et al, 2000; report

from Therapeutics and Technology Subcommittee American Academy of Neurology)

Symptom/disease Recommended tests Findings

Vestibular neuronitis ENG, audiogram Unilateral vestibular
loss
Labyrinthitis ENG, audiogram Unilateral vestibular
and hearing symptoms
Benign paroxysmal Clinical examination Paroxysmal positional
positional vertigo using Dix-Hallpike nystagmus
maneuver
Ménière disease ENG, audiogram Unilateral vestibular
loss, hearing reduction
Ototoxic vestibulopathy Rotational chair and Bilateral vestibular
ENG. loss
Acoustic neuroma Audiogram, MRI Unilateral sensori-
neural hearing
reduction, enhancing
8th cranial nerve tumor
on MRI

2.3 Testing of hearing

2.3.1 Whisper test

The perception of a whisper (while the contralateral ear passage is closed
off by finger pressure) is evaluated and compared between the two sides.
165

2.3.2 Rinne test (use tuning fork vibrating at 256 cycles/second)

Test whether the patient can hear the vibrations. The tuning fork is activated
and its base is placed against the mastoid process. The patient comments
when he/she can no longer hear the vibration and the blades of the tuning fork
are placed next to the external ear canal to assess air conduction (the note is
normally still audible). With patients demonstrating nerve deafness both air
and bone conduction are diminished. Air conduction remains the one that is
better audible. With conduction deafness the bone conduction is better than
air conduction e.g., with otosclerosis.

2.3.3 Weber test

The vibrating tuning fork is placed over the middle of the forehead. It is
assessed whether the vibration stimulus lateralizes. With nerve deafness the
vibrating sound is audible over the normal ear. With conduction deafness
e.g., middle ear disease, the vibration is transmitted to the affected ear. In
normal people, the sound is heard centrally/equally in both ears.

2.3.4 Other tests

Audiometry, loudness recruitment, auditory evoked potentials and other
tests may be performed in specialized laboratories.

3. THE GLOSSOPHARANGEAL ([Link]) AND VAGAL (N.X) NERVES

These two nerves are tested together.

3.1. The anatomy of the glossopharyngeal nerve

The glossopharyngeal motor nerve originates from the nucleus ambiguus and
supplies the stylopharyngeus muscle. With trans-section of the
glossopharyngeal nerve there is no clinical significant observable loss of
motor function of the soft palate. The motor component appears to be of little
clinical importance. The glossopharyngeal nerve supplies sensation to the
posterior one-third of the tongue, the nasopharynx, the pharynx, the
Eustachian tube, the middle ear and a part of the external ear canal. The
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pharynx is at times supplied by sensory branches of the maxillary division of

the trigeminal nerve. Viscero-afferent sensory fibers transmit taste sensation
from the posterior one-third of the tongue to the nucleus of the tractus
solitarius. Secreto-motor fibers course from the inferior salivary nucleus with
the glossopharyngeal nerve to the parotid gland.

3.2 The anatomy of the vagus nerve

Somato-motor fibers originate from nucleus ambiguus and innervate the

voluntary muscles of the soft palate, the pharynx and the larynx. Viscero-
motor and viscero-afferent fibers innervate the thoracic and abdominal
viscera.

Both the glossopharyngeal and vagal nerves exit the medulla lateral to the
olivary nucleus and course laterally through the jugular foramen towards their
areas of innervation.

3.3 Testing of the glossopharyngeal and vagal nerve

1. Patients with a vagal nerve lesion may show the following

symptoms and signs
- Dysphagia, more so for fluids than solids, nasal regurgitation,
laryngeal regurgitation and a tendency to choke.
- Nasal speech with an inability to imitate throat sounds e.g., “ke, ke,
ke” or “ing, ing, ing”.
- Hoarseness with an unilateral vagal paralysis or aphonia and a
“bovine cough” with a bilateral paralysis.

2. Gag reflex (afferent-glossopharyngeal nerve, and efferent-vagus

nerve)
While keeping the mouth open, the patient is asked to say “ah” while the
soft palate is inspected. With a spatula the throat may be touched while
observing the reflex movement of the soft palate. The soft palate pillars
to the base of the throat are compared with each other during contraction.
 Right-sided soft palate paralysis
167

Normal contraction of the soft With e.g., a right vagus nerve

palate, both pillars contract paralysis the soft palate muscles
equally on the left contract normally
leaving a “lengthened-pillar”
appearance on the paralyzed
right side

4. NERVUS ACCESSORIUS ([Link])

4.1 The anatomy of nervus accessorius

The cranial fibers of nervus XI originate from the nucleus ambiguus and join
the vagal nerve to supply the larynx. The cervical part of nervus XI is
derived from the intermediate roots of the upper four cervical segments
which enter the skull via the foramen magnum and exit via the jugular
foramen. The sternocleidomastoid and trapezius are both supplied by the
nervus accessorius and by branches of the cervical plexus.

4.2 Testing the sternocleidomastoid muscles

When inspecting these two anterior “pillar muscles” of the neck, atrophy may
be observed. The power of flexion and rotation of the neck is tested. When
testing the left muscle the chin is rotated to the right side against resistance.
Atrophy and weakness of the sternocleidomastoid muscles are found in the
following conditions:
(i) Myotonic dystrophy
(ii) Myasthenia gravis
(iii) Polymyositis
 168
4.3 Testing the upper third of the trapezius muscle

On inspecting the posterior aspect of the neck, the natural sloping curvature
of the neck to the shoulder is observed. With a palsy of the trapezius muscle
the natural sloping curve disappears and a rectangular appearance is seen.
The patient is unable to elevate the arm in abduction above the horizontal;
with the arm in the abducted vertical position there is an inability to exert power
backwards (into extension) against resistance. From posteriorly, lateral
winging of the scapula is noted with abduction of the arm to the horizontal level
(the superior medial point of the scapula wings laterally, away from the
midline). In the presence of a nervus accessorius palsy, the levator scapula
muscle may be activated as a compensatory mechanism giving the
appearance of “pseudo-webbing” of the neck and may be mistaken for the
presence of a trapezius muscle (the levator scapula muscle inserts onto the
transverse processes of the cervical vertebrae whereas the trapezius muscle
inserts onto the spinous processes) contraction. The combination of the
trapezius muscle weakness and the activation of the levator scapula muscle
may give rise to the characteristic high riding of the scapula sign as viewed
from anteriorly. The bulk and form of the superior edges of the two trapezius
muscles are compared with one another.

5. NERVUS HYPOGLOSSUS ([Link])

5.1 The anatomy of nervus hypoglossus

The nucleus of nervus XII is situated ventrally in the medulla near the
midline. The nerve leaves the medulla lateral to the pyramid and medial to
the olivary nucleus. The twelfth cranial nerve courses ventero-laterally to
leave the cranium via the hypoglossal foramen on its way to the tongue.
169
5.2 Testing the tongue

When inspecting the tongue for fasciculations, the tongue has to be in a resting
position in the mouth. Atrophy may best be seen during protrusion of the
tongue where the appearance of grooves on the sides of the tongue confirm
the atrophy. When one side of the tongue is paralyzed the protruded tongue
will deviate to the weak side (the stronger healthy side pushes the tongue over
to the paralyzed side). The power of the tongue may be tested by pushing it
against the inside of the cheek and comparing the outward resistance of the
two sides with each other. Tremulousness or shivering of the tongue may at
times be misinterpreted as fasciculations.

5.3 Lower motor neuron paralysis of the tongue

A progressive bilateral lower motor neuron paralysis of the tongue is most

commonly caused by progressive bulbar palsy, a form of motor neuron
disease. A progressive unilateral lower motor neuron tongue paralysis may
be caused by e.g., a meningioma or neurofibroma at the cranio-cervical
junction or by syringomyelia. A medial paramedian medullary infarction may
present with an ipsilateral lower motor neuron paralysis of the tongue and a
contralateral upper motor neuron weakness of the arm and leg.

5.4 Upper motor neuron paralysis of the tongue

An unilateral upper motor neuron paralysis of the tongue (e.g., as part of a

hemiplegia) may be asymptomatic (due to the bilateral corticobulbar supply)
or may on occasion exceptionally show a clinical paralysis (e.g., on the side
of the hemiplegia). With a bilateral upper motor neuron paralysis of nervus
XII, known as a pseudo-bulbar palsy, a paralytic spastic tongue may be
seen. Asking a patient to perform rapid movements of the tongue (e.g., “la-
la-la”) is a useful way of testing for tongue dysfunction. With a spastic tongue
the movements will be slow and jerky.
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Development of an acute “cortical pseudo-bulbar” stroke: If the patient has a
previous stroke (overt or covert, on history) over the motor cortex
(representing e.g., the face, the tongue and the soft palate on one side), a new
stroke of the homologous opposite motor cortex, may result in a sudden onset
of bilateral weakness of the muscles of the tongue, face and soft palate. This
is known as the so-called “operculum syndrome” or cortical pseudo-
bulbar palsy. The associated involvement of the corticospinal tracts may at
times give rise to associated UMN involvement in the arms and legs.

6. BULBAR PALSY

Definition of a bulbar palsy

The term “bulbar palsy” refers to a lower motor neuron paralysis of a single or a
number of motor cranial nerves that originate in the pons (N.V, [Link]) and the medulla
(N.X, [Link]). The lower motor neuron may be affected at the nucleus-, the cranial
nerve-, the neuro-muscular junction- or muscle levels.
Bulbar palsy definition

7. PSEUDO-BULBAR PALSY

Definition of a pseudo-bulbar palsy

The term “pseudo-bulbar palsy” indicates a bilateral upper motor neuron paralysis of
the motor cranial nerves that originate from the pons (N.V, NVII) and the medulla
(N.X, NXII). Clinically, the patient may appear to have features that mimick a lower
motor neuron paralysis of the cranial nerves – thus the term pseudo-bulbar.

The patients may be found to have spastic dysarthria and dysphagia with positive
snout and jaw reflexes. The glabella tap may be positive. The soft palate may lack
voluntary movement but the gag reflex may be hyperactive when the throat is
stimulated. Emotional lability with involuntary laughing and crying may develop due
to the interruption of the upper motor neuron tracts to the brain stem areas involved
with the control of laughing and crying.

Since the cortico-bulbar and cortico-spinal tracts lie anatomically close together,
additional upper motor neuron signs of the arms and legs (cortico-spinal tract
involvement) may not infrequently be seen with a pseudo-bulbar palsy (cortico-
bulbar tract involvement). The upper motor neuron involvement, anatomically
speaking, has to be bilateral, and may involve the following anatomical areas: the
motor cortex, the corona radiata, the internal capsule, the midbrain or the pons.
Some of the common causes of pseudo-bulbar palsy include bilateral strokes at e.g.,
the level of the corona radiata, internal capsule or the brainstem.
Pseudo-bulbar palsy definition
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8. GENERAL ANATOMY OF THE CRANIAL NERVES:

THE ANATOMICAL ARRANGEMENT OF THE CRANIAL NERVE NUCLEI

IN THE BRAINSTEM

8.1 The basic pattern of arrangement of the neurons (nuclei) in the spinal
cord.

Alar lamina (sensory)

f- sulcus limitans

Basal lamina (motor)

The anterior horn cell (1) represents the somato-efferent outflow and is
situated anterior-medially. The viscero-efferent neurons (2) are situated in
the intermediate horn more anteriorly. (3) The viscero-afferent neurons are
situated in the intermediate horn more posteriorly. (4) The somato-afferent
neurons are situated in the posterior horn.

This spinal cord basic pattern appears to be retained at brainstem level e.g.,
at the level of the medulla oblongata which may be visualized as an
unfolded spinal cord with its alar lamina having migrated more laterally and
ventrally.

8.2. Schematic representation of the cranial nerve nuclei in the medulla

oblongata
1. Nucleus of nervus XII (somato-efferent).
2. Inferior salivary nucleus rostrally and
dorsal motor nucleus of vagus caudally
(viscero-efferent).
3. Nucleus of nervus IX and X (branchial-
efferent).
9 4. Viscero-afferent nucleus (tractus
solitarius)
5. The spinal nucleus and tract of nervus V
(somato-afferent nucleus).
6. The vestibular and cochlear nuclei
(special somato-afferent nuclei).
7. Pyramids
8. Inferior cerebellar peduncles.
9. Olivary nucleus.
172

8.3. The pons may be represented schematically as an unfolded spinal

cord

1. The somato-efferent nucleus (nervus VI) is situated medio-dorsally in

the pons and is the equivalent of the anterior horn cell.
2. The viscero-efferent nucleus (superior salivatory nucleus) is equivalent
to the viscero-efferent neurons of the spinal cord.
3. The branchial-efferent nucleus of nervus VII is an additional motor
nucleus, which is situated lateral to the other motor nuclei in the basal
lamina.
4. The viscero-afferent nucleus (of the tractus solitarius) is the equivalent
of the viscero-afferent neurons.
5. The somato-afferent nucleus and tract of nervus V is equivalent to the
somato-afferent neurons of the spinal cord.
6. The special somato-afferent nuclei (vestibular and cochlear nuclei) are
the additional nuclei in the alar or sensory laminae and are situated
lateral to the other sensory nuclei.

8.4 Schematic representation of the midbrain

1. Nervus III nuclei (somato-

efferent) on the level of the
superior colliculi
2. Edinger-Westphal nucleus,
which is viscero-efferent to the
pupil and ciliary muscle.
3. Mesencephalic tract and
nucleus of nervus V (somato-
afferent).
173

8.5 Schematic representation of the motor nuclei in longitudinal sections

The somato-efferent nuclei with only motor
fibres to the voluntary muscles are situated as
a group near the midline. They leave the
brainstem near the midline and their nuclei are
situated dorsally in the tegmentum (Nervus III,
IV, VI and XII).
The viscero-efferent nuclei are situated slightly
more laterally ; the fibres of the Edinger-
Westphal nucleus to the pupil course together
with nervus III; the fibres of the superior
salivary nucleus to the salivary glands, (sub-
mandibular and sublingual) and to the lacrimal
gland course together with nervus VII. The
fibres of the dorsal motor nucleus accompany
the vagal nerve.
The branchial-efferent fibres course even
more laterally and the nerves as a group exit
more laterally (nervus V, VII, X, XI).

8.6 Schematic representation of the sensory nuclei of the brainstem in

longitudinal section
1. Somato-sensory nucleus and tract of
nervus V with:
(i) a main sensory nucleus (la)
(ii) a spinal nucleus and tract (1b)
and
(iii) a mesencephalic nucleus and tract
(1c)
2. Viscero-afferent nucleus of tractus
solitarius
3. Vestibular group of nuclei
4. Cochlear nucleus
5. Middle cerebellar peduncle
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9. THE CORTICO-SPINAL TRACT AND THE BRAINSTEM

The cortico-spinal tract is regarded as the ventral and medial “highway” of the
brainstem. This tract is situated in the crus cerebri of the midbrain, the ventral
basal pons area and in the pyramids of the medulla. The motor nuclei of the
brainstem are also situated medially, but more dorsally. The blood supply of
the brainstem consists of a basic pattern comprising the paramedian arteries
(which supply the medial part of the brainstem) and the circumferential arteries
(which supply the lateral part of the brainstem area). Every sub-division of the
brainstem (i.e., midbrain, pons and medulla) has its own paramedian and
circumferential arteries of blood supply. With a paramedian artery infarction
of the midbrain for example, an ipsilateral nervus III paralysis and a
contralateral hemiparesis may be found. With a paramedian infarct of the
pons one may find e.g., an ipsilateral nervus VI, nervus VII or internuclear
ophthalmoplegia with a contralateral hemiplegia. With a medial medulla
oblongata infarction an ipsilateral nervus XII may be found with a contralateral
hemiplegia.

10. THE SPINO-THALAMIC AND SYMPATHETIC TRACTS, NUCLEUS AND

TRACT OF NERVUS TRIGEMINUS AND THE BRAINSTEM

The spino-thalamic and sympathetic tracts traverse the brainstem laterally

and may be regarded as the lateral highways of the brainstem. The nervus
V nucleus and tract also traverse the brainstem in the lateral position. The
circumferential arteries of the brainstem supply the lateral parts e.g., the
posterior inferior cerebellar artery supplies the lateral medulla (an infarct here
is known as Wallenberg syndrome); the anterior inferior cerebellar artery
supplies the lateral part of the lower pons; the superior cerebellar artery
supplies the lateral rostral pons and the posterior cerebral artery the lateral
midbrain.

With a posterior inferior cerebellar (or vertebral) artery thrombosis

(Wallenberg syndrome) the following may be found:
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(1) An ipsilateral loss of pain and temperature over the face (involvement of
the spinal nucleus and tract of nervus V)
(2) Vertigo, nausea, vomiting and hiccoughing (involvement of the vestibular
nucleus and vomiting centre),
(3) Ipsilateral Horner syndrome (sympathetic tract involvement),
(4) Ipsilateral cerebellar signs (involvement of the inferior cerebellar
peduncle),
(5) Ipsilateral paralysis of the soft palate (involvement of nervus X) and
(6) contra-lateral loss of pain and temperature over the back of the head, the
limbs or the trunk (involvement of spino-thalamic tract).

Midbrain

KS = Cortico-spinal tract
ST = Spino-thalamic tract
S = Sympathetic tract
DK = Dorsal column

Pons Medulla Oblongata

176

Chapter 15

1. EXAMINATION OF THE MOTOR SYSTEM

1.1 General inspection
1.2 Inspection of involuntary movements
1.2.1 Definition of fasciculation
1.2.2 Definition of fibrillation potentials
1.2.3 Myokymia
1.2.4 Neuromyotonia
1.2.5 Isaac’s syndrome
1.2.6 Definition of tremor
1.2.7 Tics
1.2.8 Definition of chorea
1.2.9 Definition of athetosis
1.2.10 Definition of dystonia
1.2.11 Definition of dyskinesias
1.2.12 Epilepsy

2. MUSCLE TONE (PALPATION)

2.1 Definition of muscle spasticity
2.2 Definition of muscle clonus
2.2.1 Ankle clonus
2.2.2 Patellar clonus
2.3 Muscle rigidity
2.4 Myotonia

3. MUSCLE POWER
3.1 Functional impairment of muscle movement
3.2 Grading of muscle power
3.3 Muscle actions and segmental/peripheral nerve supply
3.4 Techniques of testing of power
177

1. EXAMINATION OF THE MOTOR SYSTEM

The motor system of the arms is best examined in the sitting position
and that of the legs in the supine position.

1.1 General inspection

The following is noted e.g., presence of atrophy, contractures of muscles

or around joints, differences in size or length of the limbs (if a hemiplegia
has been present since childhood the limbs are often smaller and shorter
than the healthy side).

1.2 Inspection for the presence of involuntary movements

The following conditions represent examples of enhanced motor

unit excitability:
(1) Fasciculations
(2) Isaac’s syndrome (described in Johannesburg,
1961)
(3) Neuromyotonia
(4) Myokymia

1.2.1 Definition of fasciculation

A fasciculation consists of an extremely rapid involuntary contraction of a small
group of muscle fibres, usually not sufficiently strong to cause a movement around a
joint. A fasciculation represents a spontaneous discharge of a hyper-excitable motor
unit with its muscle fibres.
Fasciculation definition

Causes of muscle
fasciculations
The most common cause for the development of fasciculations is the
presence of a chronic degenerative disease of the anterior horn cells
or motor nuclei. Causes of fasciculations include:
178

(1) Motorneuron disease as the most common condition that causes

the combination of generalized fasciculations of the muscles together
with associated muscle weakness and atrophy
(2) Spinal muscular atrophy (Werdnig-Hoffmann with onset before the
age of 2 years and Kugelberg-Welander disease with onset
between the ages of 2 to 17 years)
(3) Cervical spondylosis (osteoarthritis with cervical spinal
stenosis)
(4) Multifocal motor neuropathy with conduction
block
(5) Benign (physiologic) fasciculations, frequently occurring normally
especially after exercise. Fasciculations of the calves and the eyelids
are normal phenomena.

1.2.2 Definition of fibrillation potentials

A fibrillation potential refers to a contraction of a single muscle fibre that can only
be seen electromyographically and is not visible to the naked eye. It is a very
valuable laboratory sign of denervation.
Fibrillation potential
definition
Causes of fibrillation potentials
A fibrillation potential is a very useful EMG (electromyographic) finding
indicating denervation of muscle fibres, usually of recent onset or of a
persistent nature. Causes include
(1) Denervation secondary to neuropathies and anterior horn cell
diseases (most common causes).
(2) Polymyositis and Duchenne muscle dystrophy (muscle diseases
characterized by muscle fibre necrosis and fibrillation potentials
on EMG; necrosis at muscle fibre level denervates a part of the
muscle fibre distal to the necrosis). A muscle disease that
demonstrates fibrillation potentials is referred to as an “active
myopathy”.
(3) The presence of fibrillation potentials in the topographical distribution
of the myotomes supplied by a nerve root, caused e.g., by
a radiculopathy of a prolapsed intervertebral disc, denotes active
pathology in the distribution of the nerve root concerned.
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1.2.3 Definition of Myokymia

Myokymia is an involuntary, fine, repetitive, wave-like muscle movement, lasting about one
to two seconds. Multiple fasciculations or an underlying conduction block may be the
cause of such a finding. Facial myokymia may be associated with Multiple Sclerosis or
may point to the presence of an underlying brainstem glioma.
Myokymia definition

1.2.4 Definition of Neuromyotonia

Delay in muscle relaxation after voluntary muscle contraction originating from a peripheral
nerve rather than muscle is known as pseudomyotonia or “neuromyotonia” to distinguish it
from myotonia caused by a disorder of muscle membrane. It may represent a contraction
induced “muscle cramp”. Neuromyotonia is reported to occur in motor neuropathies
(heredofamilial and sporadic), and in association with fasciculations and myokymia.
Neuromyotonia responds to the treatment of carbamazepine and phenytoin.
Neuromyotonia definition

1.2.5 Definition of Isaac’s syndrome (Johannesburg, 1961)

Progressive stiffness of muscle made worse with exercise, associated with abnormal postures
and showing continuous muscle fiber activity on EMG. Muscular weakness, fasciculations
and areflexia suggest the presence of an underlying motor neuropathy. Isaac’s syndrome is
probably a more severe and persistent form of motor axonal hyperexciteability responsible
for the neuromyotonia. The hyperexcitability persists during general anaesthesia and is
abolished by neuro-muscular blockade. It responds to phenytoin treatment.

1.2.6 Definition of tremor

A tremor is a rhythmic, repetitive movement of the muscles around a joint. Tremors
are subdivided into three common groups depending on the position in which the
tremor intensity occurs maximally (Fahn, 1972).
Tremor definition

(1) Resting tremor

A resting tremor shows maximum intensity in the resting position. The
tremor may be accentuated by letting the patient concentrate on a
task (e.g., asking the patient to count backwards from 20 to 1) or by
letting the patient walk. Parkinson’s disease characteristically causes
a 4 to 8 cycles per second alternating resting tremor that improves
when a voluntary movement is made.
180

(2) Postural tremor

The patient is asked to hold the arms in the extended forward position.
Hyperthyroidism, essential familial tremor (autosomal dominant),
valproate toxicity and ethanol withdrawal are causes of a postural
tremor.

(3) Intention tremor

The patient moves the index finger from one
point to another e.g., from the
examiner’s fingertip to the tip of the
patient’s nose. The tremor attains maximal
intensity as the finger approaches its
destination. Cerebellar dysfunction is the
commonest cause of an intention tremor.

Other Tremors:
• Kinetic tremor: Occurs during voluntary movement
• Isometric tremor: Occurs during a muscle contraction against a rigid
stationary object
• Action tremor: Occurs during any voluntary contraction of skeletal
muscle
• Orthostatic tremor: Postural tremor in the torso and lower limbs while
standing; may also occur in the upper limbs. Suppressed by
walking.
• Neuropathic tremor: Postural and kinetic tremor in involved extremities
• Toxic or drug induced tremor: Usually a mixture of postural- and
kinetic tremors
• Cortical tremor: Irregular high frequency (>7Hz) postural- and kinetic
tremor associated with action myoclonus
• Rubral or midbrain tremor: A mixture of rest-, postural- and intention
tremor.
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1.2.7 Definition of Tics
Tics are involuntary repetitive brief movements (motor tics) or sounds (vocal tics) that
occur in a stereotype manner.
Tourette syndrome is present when both motor and vocal tics are present for >1year (may
be associated with symptoms of obsessive compulsive- and/or attention deficit hyperactivity
disorders)
Family of tic disorders may include:
(1) Chronic motor tics
(2) Chronic vocal tics
(3) Transient tic disorder (<1yr)
Vocal tics: e.g., Sniffing, snorting, grunting; more complex with barking and squeaking.
Definition of Tics
Characteristics of motor tics (Fahn, 1982)
1. Basic phenomenon of an individual tic: (A) A patterned sequence of coordinated
movements (B) Range: complex pattern to simple (myoclonic) jerk
2. Intermittent, not continuous
3. Variable in time (remissions)
4. Variable in location (migration)
5. Variable in frequency of individual tics
6. Variable in amplitude
7. Duration of individual tics (A) Clonic-brief and unsustained (B) Tonic-sustained
(dystonic)
8. Distribution: Face; head; neck >> arms>>legs
9. Usually not stimulus induced
10. May be preceded by irresistible urge; followed by relief
11. Temporarily suppressable (when mild)
Etiological classification of Tics
I. Physiologic tics
(A) Mannerisms
(1) Normals

(2) Mental retardation

II. Pathologic tics
(A) Primary
(1) Hereditary
(2) Idiopathic
(B) Secondary
(1) Postencephalitic
(2) Head trauma
(3) Drug induced
(a) Stimulants; amphetamine; Methylphenidate; Pemoline; Levodopa
(b) Antipsychotics; Tardive tics
(4) Carbon monoxide intoxication
(5) Degenerative disorders

1.2.8 Definition of Chorea (means “dancing movements”)

Chorea is an involuntary-, non-repetitive-, quick-, semi-purposeful-, jerky movement
that generally appears more distally in the limbs. The tone of the muscles is diminished.
The extended upper limbs (in front of the patient) sometimes show a characteristic
posture of slight flexion at the wrist joint and hyperextension of the fingers (the
choreiform hand). The patient is asked to grip the examiner’s fingers and the
examiner notes the varying intensity of the grip (Milker’s hand).
Chorea definition
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Some of the more common causes of chorea include:

(1) Sydenham’s or rheumatic chorea (Nausieda, 1980)
(2) Huntington’s chorea (Martin, 1984)
(3) The oral contraceptive pill
(4) Drugs (L-dopa, phenytoin, phenothiazines)
(5) Wilson’s disease and
(6) Cerebral palsy
(7) Stroke with e.g., hemichorea. Hemiballismus represents a
hemichorea of a severe degree, usually acute in onset and of
vascular origin (anatomical site e.g., infarct of the subthalamic
nucleus).

1.2.9 Definition of athetosis (snake-like movements)

Athetoid movements are slower and more proximal than those of chorea. When it is
difficult to distinguish athetosis from chorea the term chorea-athetosis may be used.
The modern trend is to reserve the term athetosis for cerebral palsy, but to prefer the
term dystonia in general as including the athetosis group.
Athetosis definition
Causes of athetosis include e.g., kernicterus, infantile hemiplegia, Wilson’s
disease and Lesch-Nyhan syndrome.

1.2.10 Definition of Dystonia (mobile torsion spasms)

Dystonia is a syndrome of sustained muscle contraction (mobile spasm), frequently
causing twisting and repetitive movements (jerks or myoclonic movements), or
abnormal postures. Axial musculature is frequently involved. Simultaneous
contractions of the agonist and antagonist muscles are observed. Pain is frequently
an associated accompaniment.
Dystonia definition
Causes of dystonia (Fahn, 1986)
Two types of dystonia are distinguished: A generalized (e.g., dystonia musculorum
deformans) and a focal form. The focal form can present with torticollis,
oromandibular dystonia with blepharospasm (Meige syndrome) or without,
dysphonia and focal dystonia of a limb (ranging from writer’s cramp to arm dystonia)
(Marsden 1974; 1966). The condition may be acquired or hereditary. Some acquired
causes include kernicterus, Parkinson’s disease, post-hemiplegic-dystonia, drug
induced (phenothiazines, L-dopa, haloperidol), encephalitis, head trauma, brain
tumor, hypoparathyroidism, manganese toxicity and Wilson’s disease. Segai
Segmental dystonia may refer to: Cranial (affects two or more parts of the cranial
and neck musculature); Axial (neck and trunk); Brachial (affects one arm and axial or
both arms ± trunk); Crural (affects one leg and trunk or both legs ± trunk).
Generalised dystonia: A combination of segmental crural dystonia and any other
segment.
Multifocal dystonia: Affects two or more non-contiguous body parts.
Hemidystonia: Affects ipsilateral arm and leg.
Dystonia causes
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1.2.11 Definition of Dyskinesias

In some patients combinations of abnormal movements (chorea, athetosis, dystonia,
myoclonus, tics or tremor) are seen which are difficult to define. The term
“dyskinesias” may be used as a general term and is particularly useful in drug induced
abnormal movements.
Dyskinesias definition

1.2.12 Epilepsy
Focal motor epilepsy generalized motor attacks (tonic-clonic) and
automatisms present with involuntary movements. In general, the
movements are paroxysmal in nature and of short duration (usually of
a duration of less than 3 to 5 minutes) although epilepsy partialis
continuans (as a focal motor status epilepsy) may carry on for hours, days
and at times even for weeks.

Myoclonus: Is a sudden, brief, shock-like involuntary jerking movement

of muscles, usually of sufficient force to induce a movement around a
joint. They may be focal, multifocal or symmetrical (see under epilepsy
section).

2. MUSCLE TONE (PALPATION)

The muscles are palpated and stretched to determine their texture and
resistance to movements. In polymyositis, the muscles may be tender,
stiff and fibrotic and show some limitation of movement with the
development of contractures. In muscular dystrophy the muscles may have
a firm rubbery consistency. Contractures can be felt rather than seen. In
a patient with a hemiplegia, contractures develop characteristically in the
flexor and pronator muscles of the upper limbs and the gastrocnemius
and soleus muscles of the lower limbs. In paraplegic patients flexion
contractures develop around the hips, knees and ankles. Muscle tone
may be determined by observing the resistance in the muscles while they
are being stretched around a joint.
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2.1 Definition of muscle spasticity

Spasticity is the type of increased muscle tone that is found in an upper
motorneuron lesion (i.e., with a hemiplegia or paraplegia). The increased muscle
tone is found characteristically in the flexor muscle groups of the upper limbs and
the extensor muscle groups of the lower limbs. On stretching the spastic muscle a
sudden increase in muscular tone may be found, followed by a reduction in tone
(clasp knife or relaxation – contraction – relaxation phenomenon). This phenomenon
becomes more obvious if the muscles are stretched rapidly, i.e., it is a velocity –
dependant phenomenon.
Muscle spasticity definition Spasticity definition

Testing for muscle spasticity

Stretching of a muscle

Muscle tone from the shortened to

(Resistance the lengthened muscle
felt) position.
a) Relaxation of
the muscle
b) Contraction
reaction (resistance
felt)
c) Relaxation or
decrease in tone

It is important to anticipate the increased tone in the correct muscle at

the correct stage of the muscle stretching i.e., often during the first 30
degrees of the movement. Compare the tone of the two concordant
muscles of the two sides with each other.
185
Testing for spasticity may include the following procedures

i) Move the elbow from flexion to

extension and feel the resistance in the
biceps muscle.

ii) Turn the hand and forearm from a

position of pronation to supination and
feel the resistance in the pronator teres
muscle.

iii) The wrist joint is moved from a position

of full flexion to full extension and the
resistance of the flexor muscles in the
forearm is observed.

iv) The knee joint is moved from a position of

full extension to flexion and the resistance
in the quadriceps femoris muscle is
determined.

v) The ankle joint is moved from plantar

flexion to dorsiflexion (gastronemius and
soleus)

vi) The hip is moved from adduction

to abduction (adductors of the
hip).

vii) Roll the leg (patient is lying down) to and

fro, determine the resistance of this
movement and look at how much the foot
moves together with the lower leg (this
maneuver is known as tree trunk or log
rolling test).
186

viii) Pick the thigh up rapidly (patient is lying

down) and look at the lower leg – whether
it drops to the bed rapidly or whether it
does linger for a while (contraction-
relaxation reaction of the quadriceps
muscle) before it falls to the bed (visual
representation of the clasp knife
phenomenon). Normally the heel
remains down, touching the bed-sheet.

In a hemiplegic gait the arm is held in a position of flexion and

adduction and the leg in a position of extension with circumduction
of the leg, and a dragging of the forefoot. In a patient with a
paraplegic, spastic gait, both legs are stiff, both forefeet are dragged
and the steps are short.

2.2 Definition of muscle clonus

Clonus is seen characteristically in upper motorneuron lesions and can be described
as a four plus (4+) deep tendon reflex. Clonus is a repetitive contraction – relaxation
reaction of a muscle, which may be elicited by a movement that stretches the muscle
rapidly and keeps it in this stretched position. Muscle clonus may sometimes occur
spontaneously. Five or more serial contractions in succession are regarded as clonus.
Clonus is a hard and useful sign of an upper motorneuron lesion.
Muscle clonus definition

2.2.1 Ankle Clonus

The patent’s knee is flexed slightly (patient is supine)

and the patient’s foot is grasped tightly (the examiner’s
thumb is over the dorsum of the patient’s foot and the
fingers over the plantar aspect). A quick dorsiflexion
movement of the foot is performed and the foot is
maintained in a position of dorsiflexion. A repetitive
contraction-relaxation reaction of the gastrocnemius is
seen. The hypertonic muscles are stretched, contract
and are stimulated to contract againduring the
relaxation phase (by the continuous stretching of the
muscle).
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2.2.2 Patellar Clonus

The patella is grasped between the thumb and Push patella

downwards
index finger and pushed rapidly distally and
maintained in that position. Repetitive
contraction – relaxation movements are noted
in the quadriceps muscle.

2.3 Definition of Muscle Rigidity

Muscle rigidity is a form of increased muscle tone that is seen characteristically in
Parkinson’s disease and Parkinson syndrome. The muscle tone is increased
throughout the whole range of the muscle stretching movement (lead pipe rigidity) or it
shows an additional jerky component (cogwheel rigidity, possibly due to an underlying
tremor). The flexor muscles of the body (neck, arms, trunk, and legs) are affected more
severely than the extensors by the rigidity.

Lead pipe rigidity

The tone of the muscle is increased throughout the entire range of
movement. Rigidity is tested in both flexor and extensor muscles as follows:

- Around the neck by moving the head from flexion to extension

and back and by observing the resistance in the muscles (“gently”).
- Around the wrist by performing flexion to extension movements
or rotation movements.
- Around the elbow by moving the arm from full flexion to full
extension and back.

Rigidity is also tested around the knee and ankle joints. Rigidity may be
elicited or enhanced by asking the patient to clench the contralateral fist
or by clenching the teeth or by moving the contralateral hand up and down.
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Cogwheel rigidity
With cogwheel rigidity the rigidity is seen together with a
cogwheel phenomenon (jerky element as a result of an additional
underlying tremor).

PARKINSON’S DISEASE (Bradykinetic rigidity syndrome)

2.3.1 Definition of Parkinson’s Disease

Parkinson’s disease is a specific progressive degenerative disease of predominantly
the nigrostriatal tract (these neurons contain eosinophylic cytoplasmic inclusions
known as Lewy bodies), with a dopamine deficiency, which shows the following clinical
characteristics:
(1) Resting tremor (alternating, at 4 to 8 cycles per second)
(2) Bradykinesia with poverty of spontaneous, automatic and associated
movements. The reaction time for initiation, cessation and correction of
movements is prolonged
(3) Rigidity of the lead pipe and/or cogwheel type
(4) Postural or gait disturbances [flexed posture; short shuffling steps; freezing;
festinance; imbalance]
(5) Other symptoms e.g., loss of weight, bladder dysfunction, depression and
constipation.
Parkinson’s disease definition
Exclusion criteria for the diagnosis of Parkinson’s disease (Koller, 1992)
(1) Sudden appearance of symptoms
(2) Remission in the course of the disease
(3) Step wise progression
(4) Treatment with neuroleptica in the previous year
(5) Exposure to drug or toxin known to cause Parkinsonism
(6) History of encephalitis
(7) Oculocephalic crisis
(8) Supra-nuclear downward or lateral gaze palsy
(9) Cerebellar signs
(10) Unexplained UMN signs, or
(11) LMN signs
(12) Autonomic failure with repeated syncopal attacks
(13) Dementia at onset of the disease
(14) Postural instability early in the course of the disease
(15) Signs of cerebrovascular disease
(16) Unilateral dystonia associated with apraxia or sensory deficits.
Parkinson’s disease exclusion criteria
189

2.3.2 Definition of Parkinson Syndrome/ Parkinsonism

Parkinson syndrome is a condition of:
(1) Bradykinetic rigidity with or without tremor caused by conditions other than
idiopathic Parkinson’s disease
(2) Gait disturbances and psychic impairment are usually found earlier in the course
of the disease
(3) UMN signs and cerebellar signs may appear
(4) Causes of Parkinson Syndrome include the following:
(i) Drug-induced Parkinsonism (typical neuroleptic antipsychotic drugs;
antidopaminergic antiemetics; drugs depleting presynaptic nerve terminals
of dopamine, reserpine and tetrabenazine; newer or atypical antipsychotics,
the relative propensity to cause drug induced parkinsonism is as follows:
risperidone>olanzapine>quetiapine>clozapine
(ii) Huntington’s chorea (rigid form; juvenile or Westphal-variant)
(iii) Alzheimer’s disease
(iv) Multiple system atrophy (MSA) with components or combinations of the
Shy-Drager syndrome [orthostatic hypotension is dominant], olivo ponto
cerebellar degeneration [cerebellar dysfunction dominant], and [or
Striatonigral degeneration [bradykinetic rigidity is dominant]; early
cognitive involvement; upper motor neuron signs
(v) Progressive supranuclear ophthalmoplegia [Steele Richardson syndrome].
The patient is unable to look up voluntarily (sometimes also downwards or
to the sides). Furthermore a spastic dysarthria, gait ataxia (difficult to define),
a bradykinetic rigidity and dystonia of the neck and sometimes the muscles
of the feet may be found. Oculocephalic reflexes are preserved
(vi) Dementia with Lewy Bodies: dementia; parkinsonism; fluctuating cognitive
impairment; visual hallucinations prominent
(vii) Corticobasal degeneration: asymmetrical rigidity; apraxia; cortical sensory
dysfunction; dystonia; tremor; aphasia
(viii) Toxin induced parkinsonism: manganese toxicity; acute carbon monoxide
poisoning; MPTP toxicity (contaminated IV synthetic heroin)
Parkinson’s syndrome definition

MOTOR COMPLICATIONS OF PARKINSONS

DISEASE
While treating a patient with levodopa for Parkinson’s disease a spectrum
of “on-off” phenomena may be seen. In the “off-phase”, (with low
dopamine blood levels) the patient may present with bradykinesia, end-
of-dose akinesia and early morning akinesia. Freezing episodes do not
correlate specifically with levodopa blood levels. The “on-phase” (high
dopamine blood levels), is associated with improved function and
dyskinesias, (abnormal movement often choreiform), with the peak of
dose dyskinesias as the next common form. Early morning dystonia may
be associated with a high levodopa dose. In late and severe Parkinson’s
disease the patients may show alternations between bradykinesia and
dyskinesia without a specific relation between the levodopa dosage and
the clinical effect, which is known as the “yo-yo” phenomena.
190

2.4 Myotonia
Myotonia refers to the inability of the muscle to relax immediately after
contraction. It occurs characteristically in the muscle disorders of myotonia
dystrophica and myotonia congenita.

Testing for myotonia:

i) The patient clenches the fist and tries to open the hand quickly. In
patients with myotonia the hand can only be opened slowly from a
position of flexion of the wrist and finger joints.

ii) The thenar eminence is tapped with a patella hammer and the
myotonia is observed as a slow relaxation of the contracted thenar
eminence muscles (percussion myotonia).

iii) Myotonia of the tongue may be elicited by tapping the sharp edge of a
spatula, held against the tongue, with a patella hammer. The
contracted, slowly relaxing muscle can be seen in the form of grooves
on the sides of the tongue.

iv) Forearm myotonia is elicited by briskly tapping the forearm muscles

with a patella hammer and noting how the wrist and hand extend
rapidly and move back only very slowly to a position of flexion (the
forearm is supported by the examiner).
191

3. MUSCLE POWER

The screening of the power of the muscles is best assessed by asking

the patient about impairment of activities of daily living. The muscles may
be tested systematically on inspection around the different joints and the
power of the individual muscles may be graded and charted. If the
patient has rigidity or spasticity, the grading of the muscle power will be
less accurate, as the increased tone may also impede movement.

3.1 Functional impairment of muscle power

If the patient has no functional impairment, it is most improbable

that he will have significant underlying muscle weakness.
It is determined whether the patient participates in outdoor activities
and
whether he can do the following: walking; running; climbing stairs; walking
on heels; walking on toes; walking heel-to-toe; getting up from a chair;
rising from haunches with folded arms; rising from the supine position with
folded arms; getting out of the bath, etc. Normally the arms (each in turn)
can be held up against gravity, above the horizontal level, for
approximately three minutes and the legs for 1½ to 2 minutes.

Dynamic muscle movement and power assessment at a so-called

“macro-level” by inspection:
Before the power of individual muscles is assessed (so-called
“microscopic
level evaluation”), the so-called “macro level” may be assessed first
dynamically. The patient is asked to pick up each limb in turn and execute
movements around the joints in turn (a normal performance probably
points to a power of at least grade 4/5 on the MRC scale). E.g., The
outstretched arm is held above the horizontal (patient sitting) and the
patient is asked to perform abduction- and adduction- and flexion- and
extension movements around the shoulder joint; flexion- and extension
movements around the elbow- and wrist joints; and extension-, flexion-,
abduction- and adduction
192
movements around the finger joints. In the supine position the leg is
elevated (flexed) at about a 60° angle at the hip and the patient is asked
to perform flexion-, extension-, abduction- and adduction movements
around the hip joint; flexion and extension movements around the knee
joint; flexion- extension-, inversion- and eversion movements around the
ankle joint and flexion- and extension movements around the toes.

The Barré test for the presence of

latent upper motor neuron muscle
weakness looking for the so-called
“pronator drift”
The patient is asked to hold the arms in the extended forwards position, 30°
above the horizontal, with the palms facing upwards and the fingers
adducted; then asked to close the eyes. The patient is asked to count
audibly backwards from 20-1 while the arms are observed. With an upper
motorneuron lesion the following may be seen:
(1) The arm drifts down- and outwards
(2) Wrist and elbow joints may become more flexed
(3) The little finger abducts
(4) The forearm tends to turn into pronation (“pronator drift” secondary to
the spasticity affecting the pronator teres muscle more).

3.2 Grading of muscle power

The power is determined systematically around every joint in selected

patients when the history and examination show that it is necessary.
Grading of muscle power (“Medical Research Council Scale”, 1943)
Grade (0) No movement
Grade (1) Flicker of movement
Grade (2) Normal movement is possible when gravity is eliminated (e.g., turning
the wrist vertically, with flexion and extension movements of the wrists).

Grade (3) The muscle can be moved against gravity but not against resistance.
Grade (4) The power is between 3 and 5. The power of the muscle can be
overcome by resistance. Sometimes a (4+) and (4-) scale is
employed.
Grade (5) Normal power.
193
The Mayo-clinic muscle power scoring system is reflected below:
NEUROLOGIC DISABILITY SCORE FOR PERIPHERAL NEUROPATHY
Name …………………………………………………………..................................……..
Evaluation ……………………..........…………….Date ………………………........................
(invaluable in assessing patient progress and monitoring effect of treatment e.g., immunoglobulin treatment
for patient with CIDP (chronic inflammatory demyelinating polyradiculoneuropathy)
Scoring: Weakness is scored 0 = normal (100% normal power); 1 = 25% (75-100% of normal power; 2
= 50% (50-75% of normal power); 3 = 75% (25-50% of normal power) and 4 = 100% (0-25% of
normal power).
Papilledema is scored: 0 – absent; 1 = present
Reflexes and sensation are scored: 0 = normal; 1 = decreased; 2 = absent.

Evaluation
Cranial nerves Right Left
Papilledema ……. …….
EOM weakness, Cr III ……. …….
EOM weakness, Cr VI ……. …….
Face weakness ……. …….
Palate weakness ……. …….
Tongue weakness ……. …….
Muscle weakness
Respiratory …... …….
Shoulder abduction ……. …….
Biceps brachii ……. …….
Brachioradialis ……. …….
Extension of elbow ……. …….
Extension of wrist ……. …….
Flexion at wrist ……. …….
Extension of fingers ……. …….
Flexion of fingers ……. …….
Intrinsic hand muscles ..…… …….
Iliopsoas ..…… …….
Glutei ..…… ….....
Quadriceps ...…... …….
Hamstrings ..…… …….
Dorsiflexors ……. …….
Plantar flexors ……. ….….
Reflexes
Biceps brachii ..…… …......
Triceps brachii ..…… .…….
Brachioradialis …..… …......
Quadriceps femoris ..…… …..….
Ankle reflex …..… ….…..
Sensation
Index finger (below base of nail; JP at MC-P joint) .……. ….…..
Touch-pressure .…..… ..…….
Pricking pain ..…… ..…….
Vibration …….. …..….
Joint position (JP) …….. …..….
Great toe (below base of nail; TP at MT-P joint) ..……. ..…….
Touch-pressure ...…… ..…….
Pricking pain .....… …….
Vibration …..… …….
Joint position (JP) …….. …….
Sum Total …… …….
194

Figure 35-5: The Neurologic Disability Score (NDS) is derived from a neurologic examination obtained in a standard way
by a specially trained neurologist. Decisions are based on the neurologist’s judgement of what is normal considering site,
age, sex, weight, height and physical fitness. Specificity is preferred over sensitivity. A neurologic deficit from a known
unrelated illness, e.g. a missing limb, is scored the same as for the contralateral limb. If both lower limbs are absent,
normal scores are used. Subset scores: W-NDS, S-NDS, A-NDS; others can also be used. (Slightly modified from Dyck, P.J.
Sherman, W.R. Hallcher, L.M. et al: Human diabetic endoneurial sorbitol, fructose, and myo-inositol related to sural nerve morphometry.
Ann. Neurol, 8:590. 1908. Reprinted by permission). ; Dyck PJ, Quantitating severity of neuropathy (p. 686-697) Ed
rd
Dyck PJ; Thomas PK; Griffin JW; Low PA; Poduslo JF; Peripheral neuropathy, 3 edition;

1993; WB Saunders Company, Mexico

NEUROPATHY SYMPTOM SCORE

Score 1 point for presence of a symptom.

1. Symptoms of muscle weakness
A. Bulbar
1. Extraocular …….
2. Facial ….….
3. Tongue ….….
4. Throat …….
B. Limbs
5. Shoulder girdle and upper arm ….….
6. Hand ….….
7. Glutei and thigh ….….
8. Legs ….….
II. Sensory disturbances
A. Negative symptoms
9. Difficulty identifying objects in mouth ….….
10. Difficulty identifying objects in hands ….….
11. Unsteadiness in walking ….….
C. Positive symptoms
12. “Numbness,” “asleep feeling” like ….….
Novocain”, “prickling”. – at any site ….….
13. Pain – burning, deep aching, tenderness – at any location ….….
III. Autonomic symptoms:
14. Postural fainting ….….
15. Impotence in male ..……
16. Loss of urinary control ….….
17. Night diarrhea ….….

Figure 35-1. The Neuropathy Symptom Score (NSS) is derived from a neurologic
history that is obtained in a standard way by a specially trained neurologist using
approaches outlined in the text. Neurologic symptoms attributable to a co-incident
neurologic disorder (for example, a previous mechanical nerve injury unrelated to
the neuropathy studies or to age (impotence in a man 65 years old) are not scored
as present. A total (T- NSS) is obtained from scoring all items. Subset scores, e.g.
weakness (W- NSS), sensory (S-NSS) or autonomic (A-NSS) or upper or lower limb
or proximal or distal limb W-NSS can be calculated. (From Dyck. P.J. Sherman,
W.R. Hallcher, L.M. et al; Human diabetic endoneurial sorbitol, fructose, and myo-inositol
related to sural nerve morphometry. Ann Neurol. 8:590, 1980. Reprinted by permission;
Dyck PJ, Quantitating severity of neuropathy (p. 686-697) Ed Dyck PJ; Thomas PK; Griffin
rd
JW; Low PA; Poduslo JF; Peripheral neuropathy, 3 edition; 1993; WB Saunders
Company, Mexico.)
195
3.3.1 Muscle action and segmental/Peripheral nerve supply

Movement around joint Muscle Segmental supply Peripheral Nerve

Shoulder

Abduction (0-30°) Supraspinatus C5, C6 N. Suprascapularis

Abduction (30-90°) Deltoideus C5, C6 [Link]

Adduction Pectoralis major C5, C6, C7, C8 N. Pectoralis

Latissimus dorsi C6, C7, C8 N. Thoraco dorsalis
Flexion Pectoralis major C5, C6, C7, C8 N. Pectoralis

Extension Latissimus dorsi C6, C7, C8 N. Thoraco dorsalis

Internal rotation of the arm Teres major C5, C6, C7 N. Subscapularis/

Thraco dorsalis
External rotation of the arm Infraspinatus C5, C6 N. Suprascapularis

Elbow

Extension Triceps C6, C7, C8 N. Radialis

Flexion Biceps C5, C6 N. Musculocutaneus

Thumb to nose Brachioradialis C5, C6 N. Radialis

196
Movement around joint Muscle Segmental supply Peripheral Nerve

Wrist

Extension Extensor carpi radialis longus C6, C7 N. Radialis

Extensor carpi radialis ulnaris C7, C8 N. Radialis
Flexion Flexor carpi radialis C6, C7 N. Medianus
Flexor carpi ulnaris C8, T1 N. Ulnaris
Fingers

Metacarpal-phalangeal joint:

Flexion Lumbricalis C8, T1 Medianus (I + II)

Ulnaris (III + IV)
Extension Extensor digitorum C7, C8 N. Radialis

Proximal interphalangeal joint: Flexor digitorum superficialis C8, T1 Medianus

Flexion
Distal interphalangeal joint: Flexor digitorum profundus C8, T1 Medianus (I + II)
Flexion Ulnaris (III + IV)
Finger – Abduction Interossei C8, T1 Ulnaris

Abduction (index finger) First dorsal interosseus C8, T1 Ulnaris

Little finger – Abduction Abductor digiti minimi C8, T1 Ulnaris

Thumb

Interphalangeal joint: Flexor pollicis longus C8, T1 Medianus

Flexion
Metacarpophalangeal joint: Flexor pollicis brevis C8, T1 Medianus (anterior)
Flexion interosseus branch)
Extension Extensor pollicis longus C7, C8 Radialis

Abduction (90°to the palm of the hand) Abductor pollicis brevis C8, T1 Medianus

Adduction Adductor pollicis C8, T1 Ulnaris

Opponation (thumb to little finger) Opponens pollicis C8, T1 Medianus

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Movement around joint Muscle Segmental supply Peripheral Nerve

Hip

Flexion Iliopsoas L1, L2, L3

Adduction Adductor longus L2, L3, L4 Obturator Ischiaticus

Adductor magnus L4, L5 obturator
Abduction Gluteus medius L4, L5, S1 N. Gluteus superior
Gluteus minimus
Tensor fasciae latae
Extension Gluteus maximus L5, S1 S2 N. Gluteus inferior

Knee

Flexion Biceps femoris Semi- L5, S1, S2 Ischiadicus

membranosus
Extension Quadriceps femoris L2, L3, L4 Femoralis

Ankle

Dorsiflexion Tibialis anterior L4, L5 Peronealis

Profundus
Plantar flexion Gastrocnemius S1, S2 Tibialis

Soleus

Eversion (foot) Peroneus longus and brevis L5, S1 Peronealis

superficialis
Inversion (foot in plantar flexion) Tibialis posterior L4, L5 Tibialis

Toes

Dorsiflexion (toes) Extensor digitorum longus L4, L5 Peroneus profundus

Dorsiflexion (big toe) Extensor hallucis longus L5, S1 Peroneus profundus

Plantar flexion Flexor digitorum longus S1, S2 Tibialis

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When testing the power of the fingers, it may be helpful to test the power of the
so-called “thumb – index finger pinch” and the “thumb-little finger pinch”
comparing the two sides. The fingers are forced apart from the inside of the circle
that is thus formed, by the examiners thumb.

3. 4. Techniques of testing power

C C5 C6
C5 C6 C7

Deltoid muscle testing Biceps muscle testing Pronator teres muscle testing

C7
C8 T1 C8 T1

Extensor muscles of wrist

Abductor pollicis brevis Flexor pollicis longus
C8 T1 L4 L5 S1

L2
L3
L4

Abductors of the fingers Abductors of the hip A d d u c to r s o f h ip

L1 L2 L3 L2 L3 L4
L5 S1 S2

Flexors of hip Extensors of hip Extensors of knee

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Special note on the perhaps more accurate (purer muscle

action) evaluation of muscle power around the hip joint:
• Abduction or gluteus medius muscle power: The patient turns on
the side and the leg is elevated against gravity in abduction and
pressure is applied downward at the level of the knee.
• Adduction or adductor longus and magnus power: The patient turns
on the side and the upper leg is abducted at the hip; the lower leg is
then adducted against gravity, and pressure is applied at the knee,
on the lower leg , downwards.
• Extension or gluteus maximus muscle power: The patient lies
prone and the leg is extended against gravity at the hip and
pressure is applied downwards at the level of the knee.
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Chapter 16

1. THE CLINICAL APPROACH AND ASSESSMENT OF MUSCLE

WEAKNESS
1.1 Schematic representation of the lower motorneuron (LMN) tract
1.1.1 Definition of the “lower motor neuron weakness syndrome”
1.2 Determination of the “lower motorneuron station” or anatomical site of
involvement
1.2.1 Anterior horn cell disease
1.2.2 Peripheral neuropathies
1.2.3 Mononeuropathies
1.2.4 Radiculopathies
1.2.5 Diseases of the neuromuscular junction
1.2.6 Myopathies/Muscular dystrophies
1.3 Distribution of LMN weakness
1.4 Upper motorneuron weakness
1.4.1 Schematic representation of the upper motorneuron tract
1.4.2 Definition of an upper motorneuron weakness or syndrome
1.4.3 Pertinent vascular supply areas
1.4.4 Clinical importance of the distribution of the weakness in UMN lesions
1.4.5 Common combinations of involvement of the UMN and LMN tracts
1.5 Muscle weakness due to non-organic factors (conversion)
1.5.1 Dissociation between the functional impairment of the patient on history and
the muscle power tested on examination
1.5.2 Sudden collapse of muscle power and jerky muscle weakness
1.5.3 The Hoover sign
1.5.4 The sternocleidomastoid muscle
1.5.5 The acrobatic or hysterical gait
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1. THE CLINICAL APPROACH AND ASSESSMENT OF MUSCLE

WEAKNESS:

Answer the following questions:

(A) Is the muscle weakness really present or not?

If the muscle weakness is obvious, the important question is whether the patient has
a clinical picture of either:

1.1 A lower motorneuron weakness syndrome?

1.2 An upper motorneuron weakness syndrome?
OR
1.3 A combination of both a LMN and UMN picture as in e.g., cervical spondylosis or
amyotrophic lateral sclerosis

Important supportive sub-questions may include:

(1) What is the anatomical distribution of the weakness?

(2) What is the temporal (time course) profile of the weakness?
(3) Which anatomical area (anatomical station) of the lower or upper motorneuron is the
one most likely to be affected?
(4) What is the most probable etiology?

(B) Which conditions may imitate muscle weakness?

The following conditions may imitate muscle weakness:

1. Parkinson’s disease or syndrome

2. Conversion, or no organic pathology demonstrated (this may be the preferred
term).
3. Polymyalgia rheumatica with prominent pain and stiffness of muscles.
4. Ankylosing spondylitis, rheumatoid arthritis and other diverse pain syndromes.
202

1.1 Schematic representations of the lower motorneuron pathway:

I Spinal cord
II Brain stem
1= Anterior horn cell
2= Peripheral or cranial nerve
3= Neuromuscular junction
4= Muscle

Conditions that affect the lower motorneuron pathway at different anatomical

substation (e.g., anterior horn cell; peripheral nerve; neuromuscular junction
and muscle) levels give rise to a similar clinical picture referred to as the so-
called “lower motor neuron weakness syndrome”.
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1.1.1 Definition of the “lower motor neuron weakness syndrome”

The conditions that affect the lower motor neuron pathway (i.e., anterior horn cell,
peripheral nerve, neuro-muscular junction or muscle) result in a similar clinical
picture with the following characteristics:
(1) Muscle weakness
(2) Muscular atrophy
(3) Decreased muscle tone
(4) Diminished or absent deep tendon reflexes
(5) Fasciculations
(6) Distribution of weakness specific to certain conditions, e.g., distal weakness
in peripheral neuropathies; proximal weakness in myopathies; eyelid, ocular,
bulbar and proximal weakness in myasthenia gravis, and localized weakness
in radiculopathies and mononeuropathies
(7) The plantar reflexes are flexor and the abdominal reflexes are usually present
(8) Electromyography is a useful tool to confirm a lower motorneuron syndrome
and to differentiate between e.g., denervating conditions and myopathies
(9) The creatine phosphokinase enzyme level may be raised particularly with
muscle diseases which show accompanying muscle fibre necrosis
(10) Muscle- and nerve biopsy may be useful in selected patients.

Some examples of conditions that cause a LMN weakness:

• Anterior horn cell disease e.g., amyotrophic lateral sclerosis

• Peripheral neuropathies e.g., diabetes mellitus
• Motor neuropathies e.g., Guillain Barre syndrome
• Radiculopathies e.g., sciatica with an L5 radiculopathy
• Neuromuscular junction dysfunctions e.g., Myasthenia Gravis
• Myopathies e.g., polymyositis

1.2 Determination of the anatomical site (station) of involvement of the

lower motor neuron pathway

1.2.1 Anterior horn cell disease

Anterior horn cell diseases often share the following characteristics:

i) Fasciculations
ii) Decreased deep tendon reflexes (however, in amyotrophic lateral
sclerosis the accompanying upper motorneuron involvement is
frequently associated with brisk deep tendon reflexes in spite of the
accompanying atrophy).
iii) Absence of sensory disturbances
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iv) Denervation on electromyography with retained relatively normal
conduction velocities.
v) Denervation on muscle biopsy with spinal muscular atrophy showing
characteristic group atrophy of muscle fibres.
vi) Creatine kinase enzyme levels that are normal or slightly raised.

Some causes of anterior horn cell (nucleus) disorders

Poliomyelitis, motorneuron disease (MND), syringomyelia, cervical
spondylosis (osteoarthritis) e.g., cervical spinal stenosis, spinal muscular
atrophy group of illnesses.

Spinal Muscular Atrophy:

The spinal muscular atrophies represent a group of hereditary conditions
affecting the anterior horn cells and demonstrate certain clinical features in
common with each other that are found with this group of conditions (see
Handbook of Neurology under the heading of “Anterior horn cell diseases often
share the following characteristics”e,g.,: proximal muscle weakness; muscle
fasciculations; absence of sensory findings; denervation changes on
electromyography; defective SMN genetic study).

The causes of spinal muscular atrophies(SMA) are divided into the following
subgroups:
1. Infantile spinal muscular atrophy (or the Werdnig-Hoffmann type) which
has an age of onset of between birth and six months; the prognosis is
usually poor with death by the age of two years; it has an autosomal
recessive inheritance and a defective SMN (survival motor neuron) gene.
2. An intermediate spinal muscular atrophy form is also found, which has
an onset before eighteen months of age; where the subject is usually
unable to walk but may survive into childhood; it has an autosomal
recessive inheritance and the defective gene is the “SMN” gene.
3. The juvenile SMA form (also called the Kugelberg Welander subtype)
usually has an age onset after eighteen months and they usually
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survive into adulthood; it has an autosomal recessive inheritance and the

defective gene is the “SMN” gene.
4. Adult onset spinal muscular atrophy has an onset after twenty years of
age, it has a slow progression, an autosomal recessive inheritance and
its gene defect is unknown.
All three types of childhood SMA have been genetically mapped to the same
telomeric region on chromosome 5; namely at chromosome 5Q11. 2 to 5q13.3.
Two unrelated genes in this region have been identified to be responsible for
SMA namely:
The survival motor neuron gene (SMN) and the neuronal apoptosis inhibitory
protein (NAIB) gene. More than 98% of patients with SMA have a deletion
involving exons 7 & 8. The diagnosis of childhood SMA can characteristically
be made, quite readily with DNA testing, locally in SA.

Diagnostic criteria for the diagnosis of amyotrophic lateral sclerosis (ALS as a form
of MND)
• Presence of
(i) LMN signs
(ii) UMN signs
(iii) Progression (more likely if it appears in a larger number of areas e.g., brain
stem, cervical, thoracic and lumbosacral. Definitive MND if these criteria
appear in 3 areas).
• Absence of
(i) Sensory signs
(ii) Sphincter dysfunction
(iii) Parkinson’s disease
(iv) Alzheimer disease.
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Schematic representation of the motorneurons of the motor cortex, the
brainstem and the spinal cord.

a) The clinical picture of motorneuron

disease
If the neurons of the motor cortex are
involved (1+1), bilateral upper
motorneuron signs (primary lateral
sclerosis) of the speech, masticatory
and deglutition muscles as well as of
the arms and legs may be found.
If the brainstem motor nuclei (2+2) are
involved, bilateral bulbar palsy may be
the dominant problem.
If the anterior horn cells (3+3) are
involved, a bilateral lower motorneuron
picture of progressive muscle atrophy of
the arms and legs may appear.
Widespread fasciculations and muscle
atrophy appear and may at first be seen
asymmetrically and monomelically (in
one limb).
Motorneuron disease characteristically
results in a combination (simultaneous
appearance) of lower and upper motor
neuron signs with a rapidly progressive
clinical course of weakness over an
average period of about two and a half
years.
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(i) Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis is the most common form of motorneuron
disease. The “amyotrophy” part of the term refers to the muscle atrophy
(often maximal in the hands) and reflects on the presence of a lower
motorneuron component (involvement of 3+3) in the limbs and trunk.
The “lateral sclerosis” part of the term refers to the lateral
(corticospinal tracts) involvement (involvement of cortical
motorneurons, i.e., 1+1) with upper motorneuron signs often maximal in
the legs. The presence of the combination of lower- and upper
motorneuron signs, widespread fasciculations and the absence of
sensory dysfunction is characteristic.

(ii) Progressive muscle atrophy form

In progressive muscular atrophy, mainly a lower motorneuron form of
motorneuron disease is found (involvement of motorneurons, i.e. 3+3),
with signs of lower motorneuron involvement. Muscle atrophy and
fasciculations are the dominant findings. If no upper motorneuron signs
are found one hesitates to diagnose motorneuron disease. A treatable
condition known as multifocal motor neuropathy with conduction
block (which looks like a purely motor distal mononeuritis multiplex
with prominent fasciculations) may be differentiated from motorneuron
disease by the absence of upper motorneuron findings and the
presence of conduction blocks on electroneuronographic examination.

(iii) Progressive bulbar atrophy (plus pseudobulbar component)

In progressive bulbar atrophy a combination of upper- and lower motor
neuron signs of the lip-, tongue-, soft palate- and masticatory muscles
may be found. A bulbar (2+2, motorneuron involvement) and a
pseudobulbar component (1+1, motorneuron involvement) are present.
Progressive bulbar atrophy is the most common cause of a slowly
progressive dysarthria and dysphagia.

(iv) Primary Lateral Sclerosis form of motor neuron disease:

Clinically, bilateral upper motor neuron signs (involvement of 1+1) with
upper motorneuron signs maximally in the legs (reflecting the term
primary lateral sclerosis; which refers to the pathology at dorsal
spinal cord level) is found. This condition also represents a clinical
syndrome that may be imitated by a diverse number of conditions. The
diagnosis of motorneuron disease is controversial if the expected
associated lower motorneuron signs are absent.
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1.2.2 Peripheral neuropathy

Peripheral neuropathies tend to share the following characteristics
with each other:
i) Decreased or absent deep tendon reflexes
ii) Distal muscle weakness
iii) The characteristic associated glove-and-stocking distribution of sensory
loss.
iv) Electromyographic findings of denervation such as the presence of
e.g., fibrillation potentials and large polyphasic motor units.
v) Normal to slightly raised creatine phosphokinase enzyme levels
vi) Nerve biopsy may be diagnostic, e.g., in vasculitis and amyloidosis.

The clinical importance of the demyelinating neuropathies e.g., Guillain Barré syndrome and
its variants and CIDP (chronic inflammatory demyelinating polyradiculoneuropathy) and
other acquired demyelinating neuropathies (e.g., MMN; MADSAM neuropathy; MASAM
neuropathy and DADS neuropathy [see below]) lie in the fact that they are eminently
treatable and should be recognized and diagnosed as such.

[Link] AIDP
(AIDP-Acute inflammatory demyelinating polyradiculoneuropathy)
Guillain-Barré syndrome is an acute onset (maximal deficit occurs frequently within
days, but by definition within four weeks), symmetrical, peripheral neuropathy with
both distal and proximal muscle weakness of all four limbs, with areflexia and a
raised CSF protein level with <10 cells/mm3. Mild sensory symptoms or signs may be
present as well as a cranial neuropathy e.g., bifacial paralysis. Electrodiagnostic
features of nerve conduction slowing or block may be found. The
polyradiculoneuropathy often develops after an infection (e.g., viral infections,
Campylobacter jejuni etc.) and after vaccinations. Complications such as respiratory
failure, problems with swallowing and aspiration, deep venous thrombosis and
pulmonary embolism, as well as autonomic complications such as hypo- or
hypertension and tachy- or brady arrhythmias are monitored and treated carefully
and pro-actively.

Classification of Guillain Barré subtypes:

• Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
• Acute motor axonal neuropathy (AMAN)
• Acute motor sensory axonal neuropathy (AMSAN)
• Miller-Fisher syndrome (ophthalmoplegia, ataxia and areflexia)
• Acute pandysautonomia
• Sensory Guillain Barré Syndrome
209

Diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy

(CIDP)
[Bradley’s neurology text, 2004, p. 2346]

I. Mandatory clinical criteria

• Progressive or relapsing muscle weakness for 2 months or longer
• Symmetrical proximal and distal weakness in upper or lower extremities
• Hyporeflexia or areflexia
II. Mandatory laboratory criteria
• Nerve conduction studies with features of demyelination (motor nerve
conduction<70% of lower limit of normal)
• Cerebrospinal fluid protein level >45 mg/dL, cell count <10/mm3
• Sural nerve biopsy with features of demyelination and remyelination including
myelinated fiber loss and perivascular inflammation
III. Mandatory exclusion criteria
• Evidence of relevant systemic disease or toxic exposure
• Family history of neuropathy
• Nerve biopsy findings incompatible with diagnosis
IV. Diagnostic categories

A. Definite: Mandatory inclusion and exclusion criteria and all laboratory criteria
B. Probable: Mandatory inclusion and exclusion criteria and 2 of 3 laboratory criteria
C. Possible: Mandatory inclusion and exclusion criteria and 1 of 3 laboratory criteria

CIDP with concurrent diseases and other chronic acquired

demyelinating neuropathies
(Sander and Latov, 2003)
Treatment
CIDP with concurrent diseases

• CIDP-MGUS Corticosteroids; plasma exchange;

IVIg Corticosteroids; plasma
exchange; IVIg
• CIDP-DM

Other demyelinating
neuropathies IVIg; Corticosteroids; plasma
exchange
• Sensory CIDP
IVIg
Corticosteroids; IVIg
• MMN IVIg
• MADSAM neuropathy Plasma exchange; IVIg;
• MASAM neuropathy chemotherapy, rituximab

• DADS neuropathy
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CIDP = chronic inflammatory MADSAM = multifocal acquired

demyelinating demyelinating sensory and motor
polyradiculoneuropathy MGUS = MASAM = multifocal acquired sensory
monoclonal and motor
gammopathies of undetermined DADS = distal acquired
significance demyelinating symmetric
IVIg = intravenous
immunoglobulin
DM = diabetes mellitus MMN
= multifocal motor
neuropathy

1.2.3 Mononeuropathies
Mononeuropathies occur frequently in clinical practice.
Examples of mononeuropathies include:
i) Nervus facialis: an acute onset of a Nervus VII palsy of unknown
etiology is known as a Bell’s palsy.
ii) Nervus medianus: Carpal tunnel syndrome often presents with
paraesthesias over the palmar aspect of the lateral three and a half
fingers (often at night) with weakness of the abductor pollicis brevis-
and sparing of the flexor pollicis longus muscle. The Phalen test
(flexion or extension of the wrist for some 30 seconds elicits
paraesthesias in the affected fingers) and Tinel sign (percussion or
pressure over the median nerve at the wrist elicits a neuralgic pain in
the distribution of the lateral three and a half fingers) are often positive.
iii) Radial nerve: Saturday night palsy is a palsy of acute onset that
involves the brachioradialis and the extensors of the wrist, fingers and
thumb, but spares the triceps muscle. Sensory deficit may be found
over the lateral aspect of the forearm and dorsum of the hand. Varying
and partial associated weakness may found in the distribution of the
median and ulnar nerves.
211

iv) Ulnar nerve: With the cubital-tunnel-syndrome the ulnar nerve is

compressed at the elbow in the cubital tunnel. Paraesthesias are
experienced in the distribution of the medial one and a half fingers.
Weakness may be found in the abductors of the fingers, in abductor
digiti minimus, adductor pollicis and flexors of the metacarpo-
phalangeal joints and extensors of the proximal and distal inter-
phalangeal joints (lumbrical muscle weakness).
v) Peroneal nerve: Compression of the deep peroneal nerve behind the
head of the fibula presents with loss of sensation in a triangular area
between the big- and the second toe as well as weakness of the tibialis
anterior-, the extensor hallucis- and the extensor digitorum brevis
muscles. Involvement of the superficial peroneal nerve results in
sensory dysfunction over the lateral aspect of the lower leg and the
dorsum of the foot, and causes weakness of eversion of the foot.
vi) Lateral cutaneous nerve of the thigh: Compression of this nerve at
the inguinal ligament causes sensory hypo- or paraesthesias over the
lateral aspect of the thigh and is also known by the name of Meralgia
Paraesthetica. Meralgia paraesthetica is probably the most common
compression neuropathy of the lower limb.

Causes of a peripheral neuropathy

The causes of peripheral neuropathy may be clinically subdivided and
categorized by the nerve conduction velocities (i.e., as axonal [relatively
normal] or demyelinating [slowed]), by the dominant presence of a motor or
sensory deficit and by the clinical phenotype of the peripheral neuropathy,
i.e.
(1) Symmetrical peripheral neuropathy
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(2) Mononeuropathy
(3) Mononeuritis multiplex or
(4) Autonomic neuropathy.

Normal conduction velocity (axonal neuropathy): Alcohol associated

neuropathy, thiamin deficiency, uremia, drugs (vincristine, nitrofurantoin, gold,
and isoniazide etc).
Slowed conduction velocity (demyelinating neuropathy): Guillain-Barré
syndrome, chronic inflammatory demyelinating polyradiculoneuropathy
(CIDP), hereditary motor and sensory neuropathy type I (Charcot-Marie-
Tooth) (Harding, 1984), diabetes mellitus and Refsum disease. Predominantly
motor neuropathy: Guillain-Barré syndrome, porphyria, lead poisoning and
hereditary motor and sensory neuropathy type II and I (Harding, 1984).
Predominantly sensory neuropathy: Diabetes mellitus, alcohol-associated
neuropathy, drugs (isoniazide ,allopurinol, statins etc. important category,
check the drugs the patient is on), arsenic, familial sensory neuropathy,
idiopathic, MGUS (monoclonal gammopathy of uncertain significance) and
ischaemic neuritis.
Symmetric peripheral neuropathy: Diabetes mellitus, alcohol-associated
neuropathy, uremia and Guillain-Barré syndrome.
Mononeuropathy or mononeuritis multiplex: Compression neuropathies
(most common cause of a mononeuropathy) e.g., Carpal tunnel syndrome,
cubital tunnel syndrome, meralgia paraesthetica and nervus peroneus palsy
(see above); diabetes mellitus, polyarteritis nodosa, Churg-Strauss syndrome
and leprosy.

Multifocal motor neuropathy with conduction block is a condition

characterized by a pure motor distal mononeuropathy (or
mononeuropathies), mostly in the arms with prominent fasciculations and
antibodies against ganglioside GMI.
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1.2.4 Radiculopathies
Radiculopathies occur frequently in clinical practice.
Of all the thoracic, lumbar and sacral radiculopathies, the L5 and S1
roots tend to be involved most frequently - some 90% of instances.
The sensory involvement of dermatomes may tend to be more accurate
and useful in the determination of the level of lumbar root involvement
than the distribution of the weakness.

i) L5 radiculopathy. Neuralgic sharp shooting

pains spread from the back to the posterior aspect
of the thigh, the lateral aspect of the lower leg and
the dorsum of the foot. Sensory disturbances
(hypoesthesias, paraesthesias or pain) are found
over the lateral aspect of the lower leg, the
dorsum of the foot and the big toe. The straight
leg raising test (Laséque test), elicits pain in the
back on the side of the disc lesion, also with the
contralateral side Laséque test, with or without
spreading of pain over the posterior part of the
thigh or a neuralgic pain in the L5 distribution.
The patient may be unable to walk on his heel
with the toes and forefoot elevated, and the foot
and toes may drop on the side of the
radiculopathy. Weakness may be found in the
extensor hallucis-, tibialis anterior-, tibialis
posterior-, and gluteus medius and gluteus
maximus muscles.
214

ii) S1 radiculopathy. Neuralgic pain spreads from

the back to the posterior aspect of the thigh and
lower leg and to the lateral aspect of the foot
and little toe. The straight leg-raising test elicits
back pain ipsi - or contra laterally (towards the
side of disc involvement), with or without
spreading of pain over the posterior thigh and in
the distribution of the S1 root. When walking on
the toes the heel sometimes sags on the side of
the radiculopathy (intact heel-walking with
weakness of toe-walking may be strong pointer
to lumbar S1 root involvement). The ankle reflex
is often decreased or absent. Weakness of
plantar flexion of the big toe, other toes, plantar
flexion of the foot, and of the hamstring and
gluteus maximus muscles may be found.

Cervical radiculopathies usually involve the C6, C7 or C5 roots. Motor

weakness may generally be a more reliable sign of specific root
involvement than sensory dermatome deficits.
i) C6 radiculopathy. Pain,
paraesthesias and
hypoesthesias are found over
the lateral aspect of the forearm
and lateral two fingers.
Neuralgic pain may appear over
this same area. Decreased
biceps and brachioradialis
reflexes may be found as well
as weakness of biceps and
pronator teres muscles. Neck
movement may be limited and at
times elicits root pain.
215

ii) C7 radiculopathy. Pain,

paraesthesias and
hypoesthesias are found over
the posterior aspect of the
upper arm and forearm as well
as in the area of the middle
finger. The triceps muscle is
weak and the triceps reflex is
decreased. Pronator teres
muscle weakness may be
found.

iii) C5 radiculopathy. Pain, paraesthesias, and hypoesthesias are found

over the lateral aspect of the upper arm. Weakness of the deltoid-,
supra- and infraspinatus muscles are demonstrated together with
decreased biceps- and brachioradialis reflexes. An inverted biceps
reflex may be found where the absence of the biceps reflex reflects a
lower motorneuron component at C5 level and the associated finger
flexion points to an upper motorneuron affection of C8 innervated
muscles.

1.2.5 Diseases of the neuromuscular junction

Myasthenia Gravis may show the following characteristics:
(1) Frequent involvement of the ocular and bulbar muscles.
(2) Weakness developing or worsening on muscle exertion and fatigue.
Positive eyelid fatigue test (observing for the development of ptosis
while the patient sits and looks upwards for some 3 minutes without
blinking). Fatigue test in the arms: The patient sits. Deltoid muscle
power is assessed and the arms are held in the outstretched forward
position, above the horizontal for three minutes [normally arms can be
held up for >3 minutes], and the deltoid muscle power is re-assessed
immediately thereafter. Fatigue test in leg: Hip flexion power is
assessed in the supine position and again 90 seconds later [legs tested
individually].
216

(3) Absence of sensory deficit.

(4) Mestinon test may be valuable: A weak muscle(s) is targeted and
tested before and after Mestinon administration (60mg and possibly
extra 30mg 30-45 minutes later if no response while the patient is
waiting in the consulting room) .
(5) Decremental response of motor action potentials with repetitive
stimulation at 2 Hz.
(6) Normal creatine phosphokinase levels.
(7) Raised acetylcholine receptor antibody titer in the blood.
(8) Increased “jitter” on single fibre electromyography.

Myasthenic syndrome (Lambert-Eaton syndrome) has the following

characteristics:
(i) Weakness of the proximal muscles of the lower limbs (quadriceps
muscles are frequently involved).
(ii) Decreased to absent deep tendon reflexes.
(iii) Normal creatine phosphokinase levels.
(iv) Decreased compound motor action potential amplitude values. EMG
shows incremental responses of compound motor action potential
amplitudes (>140 %) after sustained contraction of the muscle.
(v) Other causes of disorders of the neuromuscular junction may include
“neuromyotonia”, organophosphate poisoning and botulism.

1.2.6 Myopathies and muscular dystrophies

Myopathies may share the following characteristics with each other:

(i) A predominantly proximal muscle weakness of the limbs.
(ii) Raised creatine phosphokinase enzyme levels, especially in those
myopathies characterized by muscle fibre necrosis, e.g., Duchenne
muscular dystrophy and poly- or dermatomyositis.
(iii) Myotonia (inability of the muscle to relax immediately after contraction)
in myotonic disorders.
(iv) Positive family history in e.g., muscular dystrophy, myotonic disorders
and some congenital myopathies.
217
(v) Electromyographically low amplitude, short duration, polyphasic motor
units, or so-called myopathic motor units.
(vi) Fibrillation potentials in muscle disorders with active muscle fibre
necrosis, e.g., in Duchenne muscular dystrophy and polymyositis.
(vii) Muscle biopsy may show a characteristic histopathology in amongst
others, Duchenne muscular dystrophy, polymyositis, congenital and
other myopathies.

Some causes of muscle disorders include the following: Polymyositis,

dermatomyositis, endocrine myopathies (hyperthyroidism, hypercortisolism),
hypokalaemia, drug-induced myopathies (e.g., Clofibrate especially when
used with a statin, Zidovudine, alcohol, amphotericin B) hyponatremia,
muscle dystrophies (Duchenne – X-linked, young boy; Facioscapulohumeral-
autosomal dominant; myotonic dystrophy – autosomal dominant; limb girdle-
autosomal recessive with proximal weakness of mainly the hips; and
oculopharyngeal muscle dystrophy- autosomal dominant) and congenital
myopathies.

Muscle dystrophies tend to share the following three characteristics with each other
(1) They are hereditary
(2) Muscle weakness tends to be slowly progressive
(3) Muscles tend to be involved selectively.

Duchenne muscle dystrophy may show the following:

(1) Hypertrophy of the calves
(2) Gower’s sign (patient gets up and presses with hands on lower legs,
knees and upper legs to get up from a ventral position)
(3) Onset often by 3 – 5 years of age
(4) Wheelchair bound often by 12 years of age
(5) Cardiomyopathy
(6) Mental retardation in one-third of patients
(7) Very high CK levels (several thousands)
(8) Absence of dystrophin protein on muscle biopsy and
(9) Xp21 chromosome dysfunction with its X-linked inheritance.
218

Facioscapulohumeral muscle dystrophy has the following characteristics:

(1) Facial weakness,
(2) High riding of the scapulae
(3) Weakness of the trapezius-, serratus anterior- and pectoralis anterior
muscles with at times selective atrophy of the brachioradialis-, pectoralis
major- and some other muscles
(4) Autosomal dominant inheritance with defect localizing to chromosome
4q35.

Myotonic dystrophy may show the following signs:

(1) Involvement of the distal muscles of the limbs, as well as of the eyelid-,
masticatory-, facial-, neck flexor- and cardiac muscles
(2) Cataracts
(3) Frontal alopecia
(4) Mental retardation
(5) Myotonia which forms a main characteristic of myotonia dystrophica and
may be found in some 90 % of patients
(6) Autosomal dominant inheritance with two types of mutations on
chromosome 19 [CTG expansion in DMPK gene] and 3 [CCTG repeat
expansion in 2NF9 gene].

Limb Girdle Muscular Dystrophy:

The clinical heterogeneity, which has long been recognised in the limb girdle
muscular dystrophy, has been shown to be associated with the detection of
a large number of different causal genes in this condition. At least eight
forms of autosomal recessive limb girdle muscular dystrophy and three of
autosomal dominant diseases are now recognised. A group of proteins
associated with dystrophin in the muscle fibre membrane has been identified.
This is the dystrophin-glyco-protein complex which comprises dystroglycan,
the sarcoglycans and syntrophins. At least fifteen genetic sub types of limb
girdle syndrome have been identified. Amongst others there are the calpain
deficient and the dysferlin deficient limb girdle muscular dystrophies (Bushby,
1999).
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Polymyositis may show the following

characteristics:
(1) Sub-acute onset of proximal muscle weakness over
three to six months
(2) Muscle pains and muscle tenderness
(3) Arthralgia,
(4) Dysphagia,
(5) Raised creatine phosphokinase levels
(6) Electromyography may show fibrillation potentials and
small, short-duration, polyphasic myopathic motor
units
(7) Muscle biopsy may show muscle fibre necrosis with
lymphocyte and plasma cell infiltrates (EMG and
muscle biopsy are essential investigations in the
evaluation of a patient with suspected polymyositis
where treatment with steroids may often be
administered for several years).

Inclusion Body Myositis:

Including body myositis is frequently mistakenly
diagnosed as polymyositis. It has a characteristic clinical
distribution of muscle involvement with selective
affectation of both quadriceps muscles, muscles of
swallowing; and the wrist- and the finger flexor
muscles are typically affected. Characteristically, this
may start with weakness of the flexor digitorum profundus
muscles. On muscle histology there may be rimmed
vacuoles and inclusion bodies on electron microscopy.
The condition is characteristically resistant to immune
modulating therapy.
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1.3 Distribution of LMN weakness
The distribution of the muscle weakness often helps in the differential
diagnosis and the pinpointing of the lower motorneuron disorder e.g.:
• Proximal muscle weakness probably points to an underlying muscle
disorder.
• Distal weakness probably suggests the presence of a peripheral
neuropathy.
• Bulbar weakness with ptosis and eye muscle palsies, together with
proximal weakness is very suggestive of myasthenia gravis.
• A monoparesis of acute onset accompanied by fever is in favor of a
diagnosis of polio (practically eradicated).
• An acute onset symmetrical muscle weakness (polyneuropathy) that
causes both proximal and distal muscle weakness with absent
reflexes is strongly in favor of a diagnosis of Guillain Barré syndrome
(with high CSF protein level and< 10 cells/mm3; frequently follows upon
a viral infection).
• Proximal weakness of the flexors of the hips and extensors of the
knees (quadriceps muscle), absent deep tendon reflexes of the
knees, a dry mouth and impotence may suggest the diagnosis of an
underlying Facilitating Myasthenic Syndrome (Eaton Lambert-
syndrome refer to section 1.2.5).
• Weakness of the bulbar muscles that develops insidiously and
progressively with increasing dysarthria and dysphagia probably points
to a diagnosis of progressive bulbar atrophy (a form of motorneuron
disease).
• Lower motorneuron weakness of the hands together with upper
motorneuron signs in the legs may be caused among others by
motorneuron disease, cervical spondylosis and syringomyelia.
• Weakness of single muscles may be seen in e.g., a
mononeuropathy or a radiculopathy.
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1.4 Upper motorneuron weakness
4.4.1 Schematic representation of the upper motorneuron tract or pathway

1 = Motor cortex
2 = Corona radiata
3 = Capsula interna
4 4 = Midbrain
5 = Pons
6 = Medulla
7 = Spinal cord
8 = Bulbar tract
9 = Lower motorneuron tract
I = Cortico spinal tract to spinal
cord
II = Cortico bulbar tract to
brainstem

The term Upper Motorneuron tract or pathway refers to the cortico-spinal

tract. Conditions that affect this tract at different anatomical levels (e.g.,
motor cortex, corona radiata, internal capsule, midbrain, pons, medulla
oblongata and spinalcord) tend to result in a common clinical picture known
as the so-called “Upper Motorneuron Weakness Syndrome” (UMN).

1.4.2 Definition of the so-called “Upper Motor Neuron Weakness Syndrome”

The conditions that affect the upper motorneuron pathway or tract frequently
result in a similar clinical picture or syndrome known as the so-called upper
motor neuron weakness syndrome and may show the following characteristics:
(1) Muscle weakness
(2) Hemi-, para- or quadriplegic distribution of weakness
(3) Presence of increased muscle tone in the form of clasp-knife spasticity,
which tends to be more prominent in the flexor muscles of the arms and the
extensor muscles of the legs
(4) Increased deep tendon reflexes
(5) Ankle or patellar clonus
(6) Extensor plantar responses [Babinski-responses]
(7) Decreased or absent abdominal reflexes
(8) Little or no atrophy
(9) Flexor-withdrawal reflex, e.g., an involuntary withdrawal response or partial
contraction of the flexor muscles (also the quadriceps- and tensor fascia
latae muscles) of the paralysed limb after a painful stimulus is applied to
e.g., to the sole of the foot
(10) Electromyography and conduction studies are usually normal.
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1.4.3 Pertinent vascular supply areas

Vascular supply areas

1 = Anterior cerebral artery

involvement
2 = Middle cerebral artery involvement
3 = Posterior cerebral artery
involvement

Motor homunculus (motor cortex)

1 = Foot
2 = Leg
3 = Hip
4 = Trunk
5 = Arm
6 = Hand
7 = Face
8 = Lips
9 = Tongue
10 = Larynx
A: Anterior cerebral artery area
B: Middle cerebral artery area

The development of a dense hemiplegia of acute onset involving the arm-,

leg- and facial muscles is most probably due to lesion of the posterior limb
of the internal capsule (e.g., secondary to occlusion of the deep
penetrating lateral lenticulo-striate arteries) or to the involvement of the
basal pons area (e.g., occlusion of paramedian penetrating arteries).
Lacunar infarcts (size<15mm in cross section; secondary to lipohyalinosis of
penetrating artery) and haemorrhages constitute the most prevalent
pathologies in the internal capsule region. A proximal middle cerebral- or
internal carotid artery occlusion may cause in addition to the hemiplegia
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(due to internal capsule involvement with lenticulo-striate artery [branches

from proximal stem of middle cerebral artery] involvement) dysphasia and
conjugate deviation of the eyes due to the concomitant effects of cerebral
cortical branch occlusions (e.g., involvement of the language area and frontal
eye-fields respectively).

1.4.4 The distribution of the clinical weakness in upper motor neuron lesions is of
great localising value

(1) The term hemiplegia implies a unilateral combined paralysis of the face,
the arm and the leg all on the same side and is practically always due to
an upper motorneuron pathway lesion and upper motor neuron
weakness syndrome (ipsilateral paralysis of the arm and the leg without
facial involvement may perhaps be referred to, semantically more
correct, as a unilateral diplegia). A facial paralysis, which appears
ipsilaterally to an arm and leg paralysis as part of a hemiplegia, is
for practical purposes of upper motor neuron origin even if the
frontalis muscle should show some variable degree of weakness (in
circa 10 % of instances of an UMN facial weakness, the frontalis muscle
may also show a significant degree of weakness tending to mimic a lower
motor neuron facial weakness ).

(2) Hemiparesis of acute onset with the arm weaker than the leg is in
favour of a middle cerebral artery lesion or occlusion (e.g., often distal
to the origin of the penetrating lenticulo-striate artery branches, which
course deeply to supply the internal capsule, and arise from the stem of
the middle cerebral artery, proximal to its bifurcation into its cortical
branches).

(3) Hemiparesis of acute onset with the leg weaker than the arm is
probably due to an anterior cerebral artery lesion.
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(4) A dense hemiplegia with a hemisensory deficit and a
homonymous hemianopia (all on the same side) probably indicate
the presence of a lesion of the internal capsule.

Internal capsule (1C)

1: Anterior limb (1C)
2: Knee (1C)
3: Posterior limb (1C)
4: Caudate nucleus
5: Globus pallidus
6: Putamen
7: Thalamus
M: Motor fibres
S: Sensory fibres
OR: Optic radiation
MC: Motor cortex
SC: Sensory cortex
VC: Visual cortex

(5) The presence of a localized paralysis e.g., a monoparesis with e.g., an

upper motor neuron paralysis of the face (alone), or the arm (alone) or
the leg (alone) probably favors the presence of a localized lesion of the
motor cortex and may be characteristic of a so-called “cortical branch
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occlusion syndrome” frequently secondary to cerebral artery

embolism affecting a distal cerebral artery branch.

(6) The presence of a spastic (or at times flaccid) paraparesis

(paraplegia) usually develops secondary to a lesion of the spinal cord
at dorsal- (thoracic) or cervical cord level. More rarely there may be
the presence of a so-called cortical paraplegia resulting from bilateral
medial motor cortex involvement (representing the leg parts of the
motor cortex). The associated presence of confusion or other cerebral
symptoms may be a pointer to this condition. The causes of cortical
paraplegia may include the following conditions:
(1) Cerebral palsy associated with prematurity and hypoxia (cerebral
diplegia or Little’s disease)
(2) Parafalx meningioma
(3) Butterfly astrocytoma of the corpus callosum which may involve
both medial motor cortex areas
(4) Sagittal sinus thrombosis and
(5) Anterior communicans artery aneurysm with spasm of both anterior
cerebral arteries
(6) Proximal anterior cerebral artery stem occlusion (in 20% of
instances one proximal anterior cerebral artery may be the only
supply of both anterior cerebral arteries)

(7) A spastic quadriplegia is caused characteristically by lesions of the

cervical spinal cord.

(8) A patient with a crossed hemiplegia may demonstrate ipsi-lateral

upper motor neuron (UMN) signs of the arm and the contra-lateral UMN
leg involvement pointing to a lesion in the area of the crossing of the
corticospinal tracts at the level of the lower medulla oblongata. Lesions
at the lower level of the medulla oblongata (cranio-cervical junction)
tend to show a characteristic pattern of progression of their UMN
weakness i.e., ipsi-lateral arm weakness followed by ipsi-lateral leg
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weakness, then contra-lateral arm weakness and later contra-lateral leg

weakness.

(9) In a hemi-section of the spinal cord (Brown Sequard syndrome)

upper motorneuron signs and dorsal column signs are found ipsilaterally
while spinothalamic signs are seen contralaterally below the level of
the lesion.

(10) At the level of the midbrain the contralateral hemiplegia of acute

onset may be associated with an ipsilateral nervus III lesion (i.e., with
an infarct of the paramedial midbrain territory, through paramedian
artery involvement, and is known as Weber Syndrome).

(11) A lesion at the level of the lower third of the pons of acute onset may
be associated with a contra-lateral hemiplegia and an ipsi-lateral
nervus VI, nervus VII (Millard-Gübler Syndrome), gaze paralysis
(Foville syndrome) or internuclear ophthalmoplegia (e.g., an infarct of a
paramedian artery, involving paramedian pons territory).

(12) With bilateral infarctions of the paramedian arteries of the pons a so-
called locked-in syndrome may be found i.e., the patient may lie
motionless and paralyzed, but be awake and vertical eye movements
may be intact. There may be the presence of a pseudobulbar palsy
(i.e., bilateral upper motorneuron paralysis of the facial-, soft palate-
and tongue muscles), with an inability to look to the sides. Only the
ability to look up and down may be preserved. The arms and legs may
show a dense upper motorneuron paralysis. Some variants of the
locked-in syndrome may show a marked degree of depression of the
level of consciousness or intactness of the oculocephalic reflexes.
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(13) With a lesion of the medulla

oblongata a contralateral
hemiplegia (crossing of the
corticospinal tract (7) occurs
caudally in the medulla) and an
ipsilateral XII paralysis (1) may
be found Hughlings-Jackson
syndrome.

1.4.5 Simultaneous affectation of the ventrically coursing upper motor neuron

pathway or tract and the horizontally coursing lower motor neuron pathway
or tract at their respective “crossings” or “proximity” points:

A characteristic clinical picture may be found when there is a

simultaneous affectation of the vertically coursing upper motorneuron

pathway and the horizontally coursing lower motor neuron pathway of
the cranial nerves or cervical roots.

UMN SIGNS PATHOLOGY LOCALIZATION

• Ipsilateral N III paralysis Acute stroke known as Cerebral peduncle
• Contralateral hemiplegia Weber Syndrome midbrain
• Ipsilateral N V motor Acute stroke Mid-pons
• Contralateral hemiplegia
• Ipsilateral N VI, N VII Acute stroke known Basal medial pons
gaze paralysis e.g., as Millard-Gübler Lower one-third
• INO and Foville syndrome
• Contralateral arm and
leg paralysis
• Ipsilateral N XII Acute stroke known as Pyramid of medulla
• Contralateral arm and Hughlings-Jackson oblongata
leg syndrome
• Ipsilateral root deficit, • Neurofibroma or Level of the root lesion.
e.g., C5, C6, C7 meningioma Often extra-medullary
• Ipsilateral upper motor • Cervical spondylosis lesion.
neuron signs arm and • Cervical stenosis
leg
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1.5 Muscle weakness associated with the condition of so-called “No

organic pathology found” or possibly due to conversion

The diagnosis of conversion muscle weakness may only be made by an

experienced clinician and even then doubt often remains whether an
underlying hidden associated organic condition may not be present. It is
often wiser to comment that “no organic pathology was observed” than to
commit oneself (almost indefinitely; ‘closed minded’) to a dogmatic “one-
way-street” diagnosis of a psychogenic or conversion weakness picture as a
positive finding (the underlying conflict situation has to be addressed in its
own right anyway). Please inform colleagues about the situation and follow
the patient up closely. The following signs may be helpful.

1.5.1 Dissociation between the degree of functional impairment of the patient in

the history and the muscle power tested on examination:
The patient with a conversion picture of muscle weakness complains of the
presence of weakness but may not be able to give a detailed or accurate
description of the way in which the weakness affects him/her in everyday life
e.g., that he/she cannot get out of bed or climb stairs etc. Functional testing
in terms of activities of daily living in these patients is often still normal.

1.5.2 The sudden collapse of muscle power and the jerky type of muscle
weakness:
In pathological conditions a gradual collapse of muscle power may usually
be found. With weakness due to conversion the muscle may suddenly
collapse out of proportion to the force applied or collapses in a jerky way
(alternating contraction of the agonistic- and antagonist muscles). This jerky
type of muscle weakness may, however, also occur in patients with
Myasthenia Gravis.
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1.5.3 The Hoover sign:

The examiner stands at the foot end of the bed and places the palms of the
hands beneath the heels of the supine patient. The patient lifts the legs
alternately and the resistance of the opposite heel against the palm of the
examiner is determined. If, for example, the left leg is (organically) weak, the
resistance will be higher against the examiner’s left palm when the left leg is
lifted, and vice versa. With conversion no clear difference may be found.

1.5.4 The sternocleidomastoid muscle:

In a patient with a left-sided hemiparesis the patient may possibly have
expected that movement of the neck to the right side should be weak due to
left sternocleidomastoid weakness. The right sternocleidomastoid is in reality
the weak muscle in a left hemiplegia due to the fact that the UMN fibers
to sternocleidomastoid muscle cross twice (Willoughby, 1984).

1.5.5 The acrobatic or hysterical gait:

(See later under coordination and gait).
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Chapter 17

1. THE DEEP TENDON REFLEXES

1.1 Biceps reflex
1.2 Brachioradial reflex
1.3 Triceps reflex
1.4 “Crossed” reflexes
1.5 Knee reflexes (Quadriceps muscles)
1.6 Ankle reflex (Gastrocnemius and soleus)

2. SUPERFICIAL REFLEXES
2.1 Abdominal reflexes
2.2 Cremasteric
2.3 Plantar reflex
2.4 Hoffmann reflex
2.5 Anal reflex

3. BRAINSTEM REFLEXES INVOLVED IN THE DETERMINATION OF

BRAIN DEATH
3.1 The sleep-wake cycle
3.2 Pupillary reflexes
3.3 Doll’s eye movements (oculo cephalic reflex)
3.4 Caloric oculovestibular reflex
3.5 Corneal reflex
3.6 Ciliospinal reflex
3.7 Gag reflex
3.8 Tracheal reflex
3.9 Medullary centra
3.10 Pain stimuli

4. PRIMITIVE REFLEXES
4.1 Grab reflex
4.2 Suck reflex
4.3 Rooting reflex
4.4 Bite reflex
4.5 Reflexes of dubious significance
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EXAMINATION OF REFLEXES

1. THE DEEP TENDON REFLEXES

GENERAL:
It is important to test the limbs in symmetrical positions. The deep tendon
reflexes in the arms are usually tested in the sitting position and those of
the legs in the supine position. When eliciting the deep tendon reflexes
the examiner observes the following:
• Note the muscle that is expected to contract
• Note the movement around the joint involved
• Note the contraction of adjacent muscles of the limbs for spreading
of the reflex

Deep tendon reflexes are graded as follows:

- = No response.
+ = Contraction of muscle without movement around the joint.
++ = Contraction of muscle with slight movement round the joint.
+++ = Obvious and brisk contraction of muscles with an obvious
movement around the joint with or without spread
to other muscle groups.
++++ = Clonus (5 or more contractions)

The deep tendon reflexes are characteristically depressed in lower motor

neuron lesions, increased in upper motor lesions and not really affected in
Parkinson’s disease. If a deep tendon reflex is absent, the examiner tries
to elicit the reflex by utilizing an enhancement technique i.e., by asking
the patient to e.g., clench his fists or pulling the fingers of both hands
against each other outwards, and repeating the reflex during this
maneuver.

1.1 Biceps reflex

The examiner places the tip of the thumb over the biceps tendon in the
antecubital fossa and taps (lightly at first and then more firmly) on this
232

point. The segmental innervation is via C5, C6, and the peripheral nerve
is musculocutaneus.

1.2 Brachioradialis reflex

The radial aspect of the forearm (5 cm proximal to the wrist joint) is
tapped directly (or indirectly with two interposing fingers of the examiner)
with the reflex hammer. The segmental innervation is via C5 and C6
segments, and the radial nerve innervates the muscle.

1.3 Triceps reflex

The triceps tendon is tapped directly about 4 cm above the elbow.
Segmental innervation is via C6, C7 and C8 and the radial nerve
innervates the muscle.

1.4 “Crossed” (inverted) reflexes

A crossed (inverted) biceps reflex indicates an absent biceps reflex
with simultaneous contraction of the brachioradialis or finger flexors.

A crossed (inverted) brachioradial reflex indicates an absent

brachioradial reflex with simultaneous contractions of the finger flexors.

These two reflexes indicate a lower motor neuron involvement at the level
of the reflex arc, for example C5, with upper motor neuron involvement of
lower segments. This is characteristic of cervical spondylosis (tonic
vibration reflex activates muscle spindles of, for example, C8 segment,
the finger flexors, which show hyperreflexia as a result of upper motor
neuron signs).

1.5 Knee reflexes (quadriceps muscle)

With the knee held at slight flexion the patellar tendon is tapped below the
patella, at first lightly and then harder. The segmental innervation is via
L2, L3, L4 and the femoral nerve supplies the muscle.
233

1.6 Ankle reflex (Gastrocnemius and Soleus)

The knee is flexed slightly and the ankle is held in a position of
dorsiflexion. The Achilles tendon is tapped with a reflex hammer. The
examiner takes care that the tendon of the tibialis anterior muscle is not
tensed up over the dorsum of the foot since this may immobilize or splint
the ankle and may mask the reflex. The examiner places the index finger
over the tibialis anterior tendon to determine its degree of tension.
Segmental innervation of the gastrocnemius/soleus muscles is via
[Link] (S1, S2).

2. SUPERFICIAL REFLEXES

2.1 Abdominal reflexes

The abdominal reflexes are examined in
the supine position. The tip of a key (or
an orange stick) is moved over the
abdominal wall from lateral to medial to
stimulate the four quadrants of the
abdomen sequentially. The muscle
contractions of the quadrants are
compared to one another.

2.2 Cremasteric reflex

The patient is examined in the supine position with the hips in flexion and
slight abduction. The medial part of the upper leg is scraped with a pin
(head of the pin) from distal to proximal and the degree of movement of
the testis (through contraction of the cremasteric muscle) is observed.
The reflex movements of the two sides are compared. The segmental
innervation is via L1 and L2. This reflex diminishes or disappears with an
upper motor neuron lesion.
234

2.3 Plantar reflex

The maneuver is explained to the patient and
he is asked to relax. The leg is positioned in
such a way that the knee is in slight flexion and
the hip in slight external rotation. The lateral
aspect of the sole is scraped with a blunt object
(for example the tip of a key or an orange stick)
from the heel towards the base of the fifth toe
and then medially up to the middle
metatarsophalangeal joint. The stimulus is
firm but not painful (note that the patient is not
hurt). During the examination of
the reflex the movement of the toes, the foot and leg are observed. In a
normal plantar reflex the big toe and other toes move downwards into a
position of plantar flexion. In an extensor plantar response (Babinski
response) the big toe moves into extension (dorsiflexion) and the toes
abduct. It is especially the tonic (continuous) extension reflex of the
big toe that is characteristic of an upper motor neuron lesion. In
babies the extensor plantar response may be a normal phenomenon. In
patients with brain death or complete transection of the spinal cord the
extensor plantar response disappears and the flexor plantar response
returns (the brainstem tract that influences the local spinal cord reflex arc to
induce an extensor plantar response is severed in these patients).

In patients who are too ticklish, or where the sole consists of thick
callosities, the Chaddock reflex may be utilized. A blunt object
stimulates the skin from the posterior inferior aspect of the lateral
malleolus over the lateral part of the foot up to the base of the fourth toe.
The flexor or extensor response is similar as with the usual plantar reflex.

In upper motor neuron lesions the area where an extensor plantar

response may normally be elicited (the small area at the plantar base of
the big toe and second toe) enlarges to an area equivalent to the whole
foot or lower leg.
235

2.4 Hoffmann reflex

The wrist joint of the patient is held in extension
with the fingers in mid position (palm of hand is
held to the front and downwards). The
examiner’s thumb induces a rapid flexion
movement of the patient’s middle finger (around
the distal interphalangeal joint). The examiner
notes an opponens flexion movement of the thumb and a flexion
movement of the fingers. The presence of this reflex may indicate an
upper motorneuron lesion (especially if it occurs unilaterally).

When the finger reflex is elicited the

hand is held in a similar position. The
stimulus for the reflex consists of a rapid
extension movement of the fingers. The
response is the same as with the
Hoffmann’s reflex.

2.5 Anal reflex

When stimulating the perianal skin (tip of a pin) reflexive contraction of the
anal sphincter is seen. In bilateral upper motor neuron lesions and in
cauda equina lesions involving the sacral segments (S2, S3, and S4), the
anal reflex disappears.

3. BRAINSTEM REFLEXES INVOLVED IN THE DETERMINATION OF

BRAIN DEATH
Testing of the brainstem reflexes is amongst others important in the
determination of brain death. In practice brainstem death equals brain
death. The patients that are evaluated for the possibility of brain death
are always connected to ventilators.
236

3.1 The sleep wake cycle

The ascending reticular activating system in the rostral brainstem is
damaged in brain death and deep coma results.

3.2 Pupillary light reflexes

The pupillary light reflex is absent and the pupil is dilated or in mid-
position.

3.3 Doll’s eye movements (oculocephalic reflex)

The doll’s eye movement phenomenon disappears in brain death.

3.4 Caloric oculovestibular reflex

This reflex is absent in brain death.

3.5 Corneal reflex

Corneal reflex is absent in brain death.

3.6 Ciliospinal reflex

Disappears in brain death

3.7 GAG reflex

Disappears in brain death

3.8 Tracheal reflex

Note the absence or presence of the cough reflex when intratracheally
suctioning the patient or when moving the intratracheal tube. Nervus X
forms the afferent tract and the lower cranial nerves and connections to
the respiratory muscles the efferent tract. This reflex is absent in brain
death.

3.9 Medullary centra

(1) Temperature regulation
When the medulla is destroyed and in brain death the patients
become hypothermic.
237
(2) Blood pressure control
The brain dead patient is characteristically hypotensive and the blood
pressure is often maintained artificially by intravenous drugs.
(3) Respiratory control
Brain dead patients characteristically do not breathe (this is the
reason that they are on ventilators).

3.10 Pain stimuli

When a painful stimulus is applied no response is elicited.

WHEN DETERMINING BRAIN DEATH, THE FOLLOWING

CONDITIONS SHOULD BE MET:

(1) Resuscitate the patient

In some patients cerebral perfusion improves after resuscitation and
the brain stem reflexes return.

(2) Determine the cause of brain death

The effects of drugs are ruled out. Determine whether the patient
suffers from an organic condition such as a brain hemorrhage or a
brain tumor. If the cause of brain death is unknown, a treatable
condition may still be missed. With a history of hypoxia or
hypoglycemia and “brain death” a more conservative approach is
adopted (with regard to the disconnection of the ventilator).

(3) Drug overdose is excluded

Barbiturates and other drugs may imitate brain death clinically.

(4) Primary hypothermia is excluded

Hypothermia may imitate the clinical picture of brain death.
238

4. PRIMITIVE REFLEXES

Primitive reflexes appear in conditions involving both frontal lobes or both

cerebral hemispheres diffusely (e.g., hypoxia, hypoglycemia, and
Alzheimer’s disease).

4.1 Grasp reflex

The examiner moves his fingers (index and middle) over the palm of the
patient’s hand (from laterally to medially). The reflex is present if the
patient’s fingers close involuntarily in flexion around the examiner’s
fingers. The patient may, for example, be asked to count aloud from one
to ten and may be lifted from the bed simultaneously with the grasp reflex.

4.2 Suck reflex

The patient re-enters a phase where objects are continuously or
repetitively placed into or examined by the mouth. When the tip of a
spatula is held against the patient’s lips a sucking reaction is elicited.

4.3 Rooting reflex

On stimulating the corner of the mouth with a piece of cotton wool, the
patient’s mouth moves into the direction of the stimulus.

4.4 Bite reflex

When the spatula is inserted into the mouth the patient bites down onto it
and the spatula can only be removed after several efforts. The patient
may badly injure his tongue in this way.

4.5 Reflexes of dubious significance

The reflexes thus mentioned are seen as a rule more often in association
with upper motor neuron signs as accompanying signs. These reflexes
supposedly are found more often in patients with dementia, but may also
be seen in the third or up to a half of normal individuals.
239

(1) Glabellar tap test

The patient looks at a point above him and tries actively not to blink.
The examiner percusses (from above) with the middle finger over
the glabella. Normally, the eyes blink a few times and then stop
blinking. The test is positive if the eyes continue blinking with every
tap. In Parkinson’s disease and pseudobulbar palsy the glabella tap
test is characteristically positive.

(2) Palmomental reflexes

While the tip of a key is scraped across the palm of the hand and
thenar eminence the examiner notes whether the ipsilateral mentalis
muscle (facial muscle) contracts.

(3) Corneal-mandibular reflex

With firm stimulation of the cornea (with a cotton wool tip) the jaw
(chin) moves to the contralateral side and the mentalis contracts
ipsilaterally.

(4) Snout reflex

The lips lie in a resting position on one another and are tapped with
a percussion hammer in line with the lips over the middle.
Contraction of the orbicularis oris indicates a positive reflex.
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Chapter 18

1. THE SENSORY SYSTEM

1.1 Dermatome sensation

1.2 Cutaneous sensory nerves
1.3 Examination of spinothalamic sensation
1.3.1 Schematic anatomy of the spinothalamic tract
1.3.2 Clinical signs with involvement of the spinothalamic tract
1.4 Examination of dorsal column sensation
1.4.1 Schematic anatomy of the dorsal column tract
1.4.2 Clinical signs with involvement of the dorsal column tract
1.5 Testing cortical sensation or parietal lobe function
1.5.1 Schematic representation of the parietal lobe
1.5.2 Testing cortical sensory abnormalities which may be found with both right and
left-sided parietal lobe lesions
1.5.3 Testing cortical sensation in a patient with a right-sided parietal lobe lesion
1.5.4 Testing cortical sensation in left-sided parietal lobe lesions

2. DISTRIBUTIONS OF SENSORY DEFICIT PATTERNS

2.1 Sensory level of a spinal cord lesion

2.2 Brown-Sequard (or hemi-spinal cord lesion) sensory deficit syndrome
2.3 Central spinal cord syndrome
2.3.1 Clinical picture
2.3.2 Causes of a central cord syndrome
2.3.3 Onion peel sensory deficit over the face
2.4 Hemi-hypoesthesia and hemi-hypoalgesia
2.5 Crossed spinothalamic sensory deficit: Lateral Medullary Infarct
(Wallenburg syndrome)
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EXAMINING THE SENSORY SYSTEM

1.1 Dermatome sensation (Bickerstaff, 1963):

C2 supplies the occiput
C3 circumference of the
neck
C4 shoulders over the
superior, anterior and
posterior aspects
C5 lateral part of the
upper arm
C6 lateral part of the
forearm and the 2
lateral fingers (thumb,
index finger)
C7 middle finger
C8 medial 2 fingers (ring
and little finger),
medial part of the
hand the forearm
distally
T1 medial part of the
forearm and elbow
T2 medial part of the
upper arm
T3 axilla
T8 skin at the level of the
lower edge of the ribs
and xiphisternum
T10 skin at the level of the
navel
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T12 skin at the level of the

pubis
L2,3 anterior aspect of the
upper leg and knee
L4 medial part of the
lower leg
L5 lateral part of the
lower leg, the dorsum
of the foot and big toe
S1 little toe, lateral
aspect of the foot,
heel and the posterior
part of the lower leg
S2 posterior part of the
upper leg
S3,4,5 posterior part around
the anus in concentric
rings from the outside
to the inside
respectively.

The sensation of the skin is tested with cotton wool and a pin specifically in a
dermatome distribution while the two sides are compared with one another.
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1.2 Cutaneous sensory nerves

1: Radial nerve
2: Median nerve. Sensory loss may be found in the carpal tunnel syndrome
with weakness of the abductor pollicis brevis muscle.
3: Ulnar nerve. Sensory loss may be found in the cubital tunnel syndrome with
associated weakness of the interossei-, adductor pollicis-, lumbricalis IV and V-
and flexor digitorum profundus IV and V muscles.
4: Medial cutaneous nerve of the forearm.
5: Posterior cutaneous nerve of the radial nerve (high or proximal lesion of
[Link]).
6: Femoral nerve with sensory loss over the anterior aspect of the thigh; involvement
of the saphenus branch of [Link] causes sensory loss over the medial part
of the lower leg.
7: Lateral cutaneous nerve of the thigh (meralgia paresthetica).
8: Posterior cutaneous nerve of the thigh.
9: Occipital nerve supplies the occiput.
10: Intercosto-brachial nerve.
11: C3-supply
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Cutaneous sensory nerves (continued):

1: Obturator nerve
2: Deep peroneal nerve (1st web space on foot)
3: Obturator nerve
4: Ischiatic nerve deficit
5: Lateral cutaneous nerve of the lower leg (lateral popliteal nerve)
6: Lateral plantar nerve
7: Medial plantar nerve
8: Sural nerve
9: Ilio-inguinal nerve
10: Genito-femoral nerve
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1.3 Examination of spinothalamic sensation:

1.3.1 Schematic anatomy of the spinothalamic tract:

1: Dorsal ganglion (first sensory neuron)

2: Second sensory neuron that crosses

over ventrally to the central canal and
synapses with the third sensory
neuron in the thalamus (5 = medulla,
6 – pons, 7 = midbrain).

3: Third sensory neuron (thalamus)

which passes through the internal
capsule (8) and the corona radiata (9)
to the sensory cortex (4) (post-
Rolandic gyrus).

1.3.2 The following clinical signs may be found with involvement of the spinothalamic
tract:
• Decrease or loss of pain and temperature sensation contralaterally

• Decrease or loss of coarse and non-discriminatory touch contralaterally

• Paraesthesias over the opposite side of the body

• Lhermitte’s sign. With flexion of the neck the patient may experience a
feeling of a type of electrical shock feeling down into the back, the legs or
the arms.
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1.4 Examination of dorsal column sensation:

1.4.1 Schematic anatomy of the dorsal column tract:

1: Dorsal ganglion (first sensory neuron)

cell.

2: Second sensory neuron situated in

the nuclei of gracilis and cuneatus
which are located in the lower
medulla where the tract crosses over
and joins the third neuron in the
thalamus.

3: Third sensory neuron is situated in the

posterior ventrolateral nucleus of the
thalamus and courses through the
internal capsule (4) and the corona
radiata (5) to join the sensory cortex
(6).

1.4.2 The following clinical signs may be found with involvement of the dorsal
column tract:
(Ipsilaterally if the lesions is at spinal cord level, and contralaterally if the lesion is at
brainstem level)

• Loss of position sense

• Loss of vibration sense (test with tuning fork of 128 cycles per second)
• Loss of fine discriminatory touch
• Decreased or absent deep tendon reflexes
• Feeling of a tight band around a limb or the trunk
• Feeling of enlargement of a part of a limb, for example, the foot and a
feeling that the patient is walking on something like rubber or cork.
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1.5 Testing cortical sensation or parietal lobe function:

1.5.1 Schematic representation of the parietal lobe:

1: Rolandic fissure
2: Post central sulcus
3: Parieto-occipital fissure over the
medial surface, which is
joined via an imaginary line
4: Pre-occipital indenture
(notch) to form the posterior
border of the parietal lobe
5: Supramarginal gyrus
6: Angular gyrus
5+6: Infraparietal lobule
7: Supraparietal lobule
8: Sylvian fissure: The
horizontal part of the
posterior leg is extended
backwards to form the
inferior border of the parietal
lobe

Validity of the cortical sensory tests:

The tests for cortical sensation are only valid if the patient does not show an
underlying dysfunction of the basic sensations for touch or pain in the specific
limb that is tested. The power in the limbs must be of sufficient strength allow
the tests to be performed.

1.5.2 Testing cortical sensory abnormalities which may be found with both right and
left-sided parietal lobe lesions

(1) Stereognosia
Stereognosia is the ability to observe the form and characteristics of objects
by touch or by palpation without the use of auditory or visual cues. If a patient
is unable to do this, it is known as astereognosia. This diagnosis may only be
248
made if the basic underlying sensations of the hand in question are intact and
if enough muscle strength is available to examine the object.

(2) Two-point discrimination:

Two-point discrimination indicates the ability of the skin to distinguish
between the application of a single or two simultaneous stimuli of touch. A
paper clip may be used for the purposes of this test. The importance of the
test lies in the picking up of differences by the comparison of corresponding
areas on both sides of the body by means of a non-painful stimulus.

(3) Cutaneous sensory extinction refers to the inability to experience sensation

on one side of the body when both sides are stimulated simultaneously by
touch. Each side, may however be capable of experiencing sensation
individually. The extinction occurs in the limb contralateral to the hemispheric
lesion.

(4) Visual sensory extinction may be elicited by presenting two objects

simultaneously to the two visual fields. Only the healthy normal visual field is
able to perceive the object. With individual testing of the visual fields both
sides are able to recognize the object. The deficit is seen in the visual field
contralateral to the hemispheric lesion.

(5) Dysfunction of position sense:

Position sense is tested around a joint. The patient closes his eyes and has
to mention when a movement takes place and in which direction. Normally,
one is able to perceive a displacement of some millimeters. Sometimes a
patient may be completely unaware
of the position of his limb in space
and e.g., be lying on his arm or the
leg, or leg may hang over the side of
the bed. The patient is unable to
localize the position of touch over the
contralateral limb, with his healthy
side, when his eyes are closed.
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(6) Touch localization:

Touch localization is the ability to localize the specific point where the patient
has been touched. The patient tries to localize the spot where he has been
touched while his eyes are closed.

(7) Inferior homonymous quadrantanopia:

An inferior homonymous quadrantanopia may
be found contralaterally to the side of the
parietal lobe lesion.

(8) Digit writing:

The test refers to the ability to recognize digits that are traced on the limbs or
palm of the hand. The two parts of the body are compared with one another.
Tests for recognition of digit writing are not well standardized and at present it
is unclear whether loss of this ability on both sides is of clinical significance.

1.5.3 Testing of cortical sensation in a patient with a right-

sided parietal lobe lesion:

(1) Spacial disorientation:

Patients are inclined to ignore the left-sided body
space and tend to move in the opposite direction,
e.g., towards the right side, without realizing it (e.g.,
doors and passages on the L may be ignored and those on the Rt may
inadvertently be favoured). The patient may get lost in his home or
surroundings.

(2) Dressing apraxia

This is the inability to put on, for example, a shirt or a gown and is the result
of the ignoring the left body space. Dressing apraxia may present as a main
complaint.
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(3) Constructional apraxia

The patient may, for example, not be
able to copy a triangle or a star or
imitate them with matches. The left body
space may be ignored which may be
observed when the patient is asked to
draw a clock – the digits may, for
example, all be written in the right half
of a circle and the left half may be
ignored.

(4) Non-recognition of hemiplegia:

With a left-sided hemiplegia or hemiparesis the patient may at times be
unaware of the left half of the body and may not recognize that the left side
may be paralyzed. Exceptionally the paralyzed side is experienced as a
foreign object, for example a wooden log, or as somebody else’s limbs.

1.5.4 Testing cortical sensation in left-sided parietal lobe lesions

(1) Finger agnosia:

Finger agnosia is the inability of the patient to name his own fingers or those
of the examiner (was described by Dr Joseph Gerstmann in 1924). Objects in
the common surroundings, may however, be named correctly. There may be
an additional inability to calculate the number of fingers between two fingers
that have been touched.

(2) Right-left disorientation:

The patient is unable to differentiate between his/hers left and right sides.
The patient may have difficulty with simple tests/commands such as e.g.,
place your R hand on your left ear.
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(3) Inability to calculate:

Simple addition or subtraction sums may become difficult or impossible.
Gerstmann’s syndrome:
The combination of the following is known as Gerstmann’s syndrome:
• finger agnosia,
• right–left disorientation,
• inability to calculate and
• agraphia .
Gerstmann localized this lesion to the dominant angular gyrus. It may
however be more accurate to localize the symptoms to involvement of
the dominant parietal lobe.

(4) Ideational apraxia:

Ideational apraxia is the inability to execute a planned motor act
although the individual components of the plan are recognized. When,
for example, a cigarette is lit, the matches are put into the mouth or the
cigarette is drawn against the matchbox as a match.
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(5) Ideomotor apraxia

Definition of apraxia

Apraxia is a dysfunction of the execution of learned movements that

cannot be explained on the basis of
(1) weakness
(2) in-coordination
(3) sensory deficit or
(4) lack of understanding of commands or an attention deficit.

When the general term of apraxia is used, it is understood to mean

ideomotor apraxia. Criteria for the diagnosis of ideomotor apraxia include
(1) Inability to execute a motor command
(2) Patient can be shown to understand the command [by describing the
action verbally or by making a choice from a number of gestures]
(3) Patient is able to execute the same motor action in a different context
[by asking the patient to imitate the examiner’s action].

(6) Sensory aphasia:

Wernicke’s aphasia may, for example, be
found with a dominant parietal lobe lesion
which involves the infraparietal lobule
(supramarginal- and angular gyrusses).

2. SEVERAL DISTRIBUTIONS OF SENSORY DEFICIT PATTERNs

2.1 The sensory level of a spinal cord lesion

(See also Chapter 3)

The finding of a sensory loss below a certain level over the trunk or neck
(and up to a dermatome level in the arms) is a pointer towards the
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presence of an underlying lesion of the spinal cord.

The presence of a sensory level is practically pathognomonic of a

lesion of the spinal cord.

2.2 The Brown Sequard (or hemi-spinal cord lesion) sensory deficit
syndrome

With a right-sided spinal cord lesion at, for example, th e thoracic level of T10
the following signs may be found:

• Ipsilateral corticospinal tract signs below the level

of the lesion (+++)

• Ipsilateral dorsal column signs below the level of

the lesion (loss of position and vibration): (ooo)

• Contralateral spinothalamic deficit below the level

of the lesion (loss of pain and temperature) (:::)
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2.3 The central spinal cord syndrome:

2.3.1 Clinical picture:

• Spinothalamic “suspended sensory deficit” with intact sensation

above and below the lesion. The central cord lesion tends to affect
the second spinothalamic neuron tracts, where they cross antero-
centrally in the spinal cord.

• The level and the number of segments involved are dependent upon the
location of the central spinal cord lesion and the length of cord that is
involved. The pattern of spinothalamic deficit sometimes resembles the
form of a coat (cape), sometimes a quadrant (half a coat), and
sometimes just one or two adjacent dermatomes (for example C8 and
T1 deficit).

• With selective dissociated spinothalamic deficit light touch is preserved

over the area of the spinothalamic sensory deficit (the dorsal column
function namely position and vibration sense is preserved).

• Signs of anterior horn cell deficits may be found at the level (height) of
the lesion (lower motorneuron weakness = atrophy, fasciculations,
absent reflex)
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Absent deep tendon reflexes are found, for example, in the arms with a
cervical central cord syndrome.

• Corticospinal tract signs may be found below the level of the lesion.

• With extensive spinal cord involvement a sensory level may be found in

addition (due to involvement of the spinothalamic tract). An
intramedullary lesion may spare the sacral segments as sacral
sensation is represented in the periphery of the ventrolateral
spinothalamic tract.

Sacral sparing in an intramedullary spinal cord lesion

Dorsal

Lesion

2.3.2 Causes of a central cord syndrome:

• Syringomyelia
• Glioma of the spinal cord
• Hematomyelia (post traumatic)
• Post traumatic paraplegia with a secondary upward progression/worsening
of neurological deficit may be the result of the development of a
posttraumatic syringomyelia.
• Cervical spondylosis: Associated with a hyperextension injury of the neck
and infarction of the central spinal cord. Cervical spinal stenosis may mimic
such a picture.
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2.3.3 Onion peel sensory deficit over the face:

In a high cervical syringomyelia involvement of the lower spinal nucleus and

tract of nervus V sometimes causes loss of sensation over the face (of the
outer circle 3). In a more rostral lesion of the brainstem sensory deficit is
sometimes found in the inner circle (1).

2.4 Hemi-Hypoesthesia and Hemi-Hypoalgesia

The anatomic localizations of a lesion causing a

contralateral hemisensory deficit include the
following:

1) Rostral half of the brainstem

2) The posterior ventrolateral nucleus of

thalamus

3) The internal capsule and

4) The inferior corona radiata.

The most common cause of an isolated hemi-

hypoesthesia is probably a lacunar infarct in the
area of the internal capsule.
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2.5 Thalamic syndrome:

Also known as "Dejerine-Roussy syndrome",

Thalamic syndrome is a condition in which the body becomes oversensitive to

pain usually unilaterally as a result of damage to the sensory relay station in the
brain known as the thalamus.

Dysesthesia, which refers to feeling pain or uncomfortable sensations after being

touched by an ordinary stimulus or even in the absence of stimulation, can occur
during thalamic syndrome.
The thalamic syndrome describes a condition that consists of
(1) Hyperpathia together with
(2) Hemi-hypoesthesia
(3) Homonymous hemianopia and
(4) Hemiparesis.

Hyperpathia indicates the presence of a severe pain that is difficult to describe,

which is elicited by light touch or by more intense stimulation or one that may
occur spontaneously in the area of the hemi-hypoesthesia.
Thalamic syndrome can lead to continuing crude pain in the arms and/or legs.
The pain in thalamic syndrome can be made worse with hot and cold
temperature, emotional distress, and even music.
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2.6 Crossed spinothalamic sensory deficit: Left lateral medullary infarct

(Wallenberg syndrome)

Wallenberg’s syndrome is a neurological condition caused by a stroke in the

vertebral or posterior inferior cerebellar artery of the brain stem.

3: Lateral medullary infarct on the left

4: Middle cerebellar peduncle

• The spinothalamic tract (1) lesion

gives rise the contralateral loss of
pain and temperature over the body
up to C2.

• Involvement of the spinal nucleus and

tract of nervus V (2) gives rise to the
ipsilateral loss of pain and
temperature over the face and V1.
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Chapter 19

THE AUTONOMIC NERVOUS SYSTEM

1. Clinical signs in autonomic neuropathy

1.1 Orthostatic hypotension
1.2 Bladder dysfunction
1.3 Impotence
1.4 Diarrhea
1.5 Gastric symptoms
1.6 Cardio-respiratory arrest

2. Investigations in autonomic neuropathy

2.1 Testing the sympathetic nervous system
2.2 Testing the parasympathetic nervous system
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THE AUTONOMIC NERVOUS SYSTEM

1. CLINICAL SIGNS IN AUTONOMIC NEUROPATHY (Ewing, 1982; Histed, 1983)

1.1 ORTHOSTATIC HYPOTENSION

Postural orthostatic hypotension is one of the most disturbing symptoms of an

autonomic neuropathy. It is defined as a drop of more than 30 mmHg (sometimes a
figure of 20 or 15 mmHg is taken) in the systolic blood pressure when rising.
Orthostatic hypotension may result from: (1) A loss of peripheral vascular resistance
upon rising, which may again be the result of a degeneration of sympathetic
vasoconstrictory nerve fibres (2) Hyponatremia and hypovolumia.

1.2 BLADDER DYSFUNCTION

Bladder dysfunctions are divided into conditions of mainly urinary retention and
those of urinary incontinence.

(1) Problem of urinary retention (inability to empty the bladder)

Neurological causes of urinary retention include:

 Phase of spinal shock in paraplegia

 Lesions of the conus medullaris (involve viscero-efferents S2, S3, S4 to the
bladder)
 Lesions of the cauda equina (involve viscero-efferents S2, S3,S4 to the bladder)
 Autonomic neuropathy (involve viscero-efferents S2, S3, S4 to the bladder)
 Spasticity of the external sphincter (chronic phase of a spinal cord lesion).

(2) Problem of urinary incontinence (inability to store the urine)

Neurological causes of urinary incontinence include:
 Mediofrontal lobe lesion with a disinhibited bladder
 Spinal cord lesion with a spastic reflex bladder.

1.3 IMPOTENCE
The sacral viscero-efferents via S2, S3, S4 roots regulate the ability to sustain an
erection. The sympathetic outflow via the viscero-efferent neurons from T10 to L2
segments regulates ejaculation. The development of impotence in patients with an
autonomic neuropathy is frequently irreversible.
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1.4 DIARRHEA
The mechanism for the development of diarrhea in, for example, diabetic autonomic
neuropathy is unclear.

1.5 GASTRIC SYMPTOMS

Symptomatic gastric atonia and gastric retention may occur.

1.6. CARDIO RESPIRATORY ARREST

Sudden and unexpected deaths may sometimes occur in patients with diabetes
mellitus and autonomic neuropathy. Unexpected deaths may be the result of cardio
respiratory arrest associated with hypoxia.

2. INVESTIGATIONS IN AUTONOMIC NEUROPATHY (Ewing 1982; Histed 1983).

2.1 TESTING THE SYMPATHETIC NERVOUS SYSTEM

(1) The response of blood pressure on rising

On rising, blood pools in the legs. This is rapidly corrected by peripheral
vasoconstriction.
In patients with autonomic neuropathy the blood pressure drops to a level lower
than in the supine position.

The systolic blood pressure is measured both in the upright and the supine position
and the difference is determined. Normally the systolic blood pressure drops by
less than 10 mmHg; in borderline cases, the drop is between 10 and 29 mmHg and
in clearly abnormal patients, the drop is more than 30 mmHg. This test is often
abnormal with severe peripheral nerve damage. It is important to remember that
orthostatic hypotension also occurs in conditions other than autonomic neuropathy
such as in anemia, and hypovolaemia where the vascular volume is diminished
(e.g., after use of diuretics).

(2) The response of the blood pressure to static isometric exercise

A dynamic meter utilizing handgrip may be used. The maximum voluntary
contraction is determined. The handgrip is maintained at 30 % of the maximum for
as long as possible. The blood pressure is determined three times before the test
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and at one-minute intervals during the test. The result is given as the difference
between the highest diastolic blood pressure during the test and the average of the
three-diastolic blood pressure recordings before the test. Normally, the difference is
more than 16 mmHg; borderline abnormal cases show readings of between 11 and
15 mmHg and a rise of less than 10 mmHg is clearly abnormal. The rise in blood
pressure occurs as a result of a heart tempo-dependant rise of the cardiac output
without change on the peripheral resistance.

(3) Perspiration test

Diminished secretion of sweat in the absence of sweat gland dysfunction is
indicative of a sympathetic neuropathy. Reflex sweat secretion may be tested by
heating the body so that the temperature rises one degree Celsius and local
perspiration is observed by sprinkling or applying iodine starch over the skin and
observing a change in colour.

2.2 TESTING THE PARASYMPATHETIC NERVOUS SYSTEM

(1) Heartbeat-to-heartbeat variation during deep breathing (sinus

arrhythmia)
The cardinal parasympathetic nerve (nervus vagus) regulates sinus arrhythmia
since atropine administration and severance of the vagal nerve (in animals) may
terminate it. Sinus arrhythmia may be quantified by measuring the difference
between the maximum (shortest R-R interval) and the minimum (longest R-R
interval) heart tempo during normal and deep respiration. Deep breathing at six
breaths per minute (five seconds in – five seconds out) for one minute in the quietly
sitting position is the simplest way. An ECG is performed during the one-minute
period and onset of in – and expiration is marked. The maximum and minimum R-
R-intervals are measured of the six breathing cycles and calculated as each
heartbeat per minute. The result is given as the average difference between the
maximum and minimum heart tempo as measured over the six cycles in heartbeats
per minute. Normally, the difference is more than 15 beats per minute; in borderline
cases between 11 and 14 beats per minute, and in obviously abnormal cases less
than 10 beats per minute.
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(2) Immediate response of heart tempo on rising: the 30:15 ratio

While rising from the supine to the upright position a characteristic immediate rapid
acceleration of heart tempo occurs which is maximal at approximately the 15th
heartbeat after rising. A relative overshoot bradycardia follows maximally at
approximately the 30th heartbeat. This response is mediated via the vagal nerve.
During the test one lets the patient relax while lying down and measures the heart
tempo with an ECG continuously while the test is performed. The patient gets up
without help and the instant that the patient is standing upright is recorded on the
ECG. The shortest R-R interval at approximately the 15th beat and the longest R-R
interval at approximately the 30th beat after rising are measured with a ruler. This
characteristic heart tempo response is expressed as the 30:15 ratio. A ratio of more
than 1,04 is normal, between 1,01 and 1,03 is borderline and less than 1 is
abnormal.

(3) Heart tempo response to Valsalva maneuver

During the exertion phase of the Valsalva maneuver the blood pressure drops and
the heart tempo rises. After relaxing the blood pressure rises (more than the
baseline value) and the heart tempo drops. The response of the heart tempo
seems to be vagus associated and may be blocked by atropine. In patients with
involvement of the autonomic nervous system the blood pressure drops during
exertion and returns to baseline slowly after relaxation without an overshoot
phenomenon of the blood pressure or a change in the heart tempo. The test is
performed with the help of a blood pressure apparatus, by blowing into the
connection, and by keeping the pressure constant for 15 seconds at 40 mmHg. An
ECG is taken continuously. This maneuver is repeated three times with one-minute
intervals. The test is expressed as the ratio between the longest R-R interval after
the maneuver and the shortest R-R interval during the maneuver and is known as
the Valsalva ratio. The average of three measurements is taken. Normally the
Valsalva ratio is more than 1,21; in borderline cases it lies between 1,11 and 1,20
and in patients with obvious autonomic neuropathy it is less than 1,10.
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Chapter 20
THE NEUROLOGICAL EXAMINATION : PART V

1. THE CEREBELLUM

1.1 Schematic representation of the cerebellum

1.1.1 The vermis of the cerebellum
1.1.2 The anterior lobe of the cerebellum (paleocerebellum)
1.1.3 The posterior lobe of the cerebellum (neocerebellum )
1.1.4 Flocculo-nodular lobe of the cerebellum (archi-cerebellum)
1.2 Examining motor coordination
The co-called “neurological examination in 5 minutes”
1.2.1 Speech
1.2.2 Hands
1.2.3 Gait
1.3 Testing coordination (traditionally used in the evaluation of
cerebellar dysfunction)
1.3.1 Muscles of speech
1.3.2 Upper limbs
1.3.3 Lower Limbs
1.3.4 Trunk and gait
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1. THE CEREBELLUM

1.1 Schematic representation of the cerebellum

1: Vermis

2: Anterior lobe (paleocerebellum)

2
3: Posterior lobe (neo cerebellum)

4: Flocculo-nodular lobe
(archicerebellum)

5: Dentate nucleus

6: Posterior lobe (part of the neo-

cerebellum also known as middle
lobe)

1.1.1 The posterior lobe of the cerebellum

(Neocerebellum or cerebellar hemisphere)

• The posterior lobe of the cerebellum is controlled by the cerebral

motor cortex
• and is involved in the fine coordination movements of the hand-,
speech- and other muscles.
• The cerebral motor cortex supplies the neocerebellum via the
cortico-pontine cerebellar tract (which courses through the middle
cerebellar peduncle).
• Acute dysfunctions of a cerebellar hemisphere give rise to the
development of ataxia and hypotonia of the ipsilateral limbs of the
body.
• In chronic cerebellar hemisphere dysfunction, compensation or
adaptation may occur with few or no clinical signs.
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Causes of one-sided cerebellar hemisphere dysfunction include

amongst others:

1. Cerebellar astrocytoma (often cystic with tumor nodule)

2. Cerebellar hemangioblastoma (often cystic with tumor nodule)
3. Cerebellar infarct or hemorrhage (both may present with mass-
effect)
4. Cerebellar abscess (is suspected especially in patients with otitis
media).

1.1.2 Flocculo-nodular lobe of the cerebellum

(ARCHI-CEREBELLUM or VESTIBULO-CEREBELLUM)

• Also known as the vestibulo-cerebellum,

• This lobe is mainly involved in the control of vestibular functions
(spatial orientation of the head, neck and eyes).
• The vestibular nuclei and the archi-cerebellum are interconnected.

With dysfunction of the flocculo-nodular lobe of the cerebellum the

patient may have the following signs:
• Ataxia of the trunk.
• Central positional vertigo and nystagmus.

Causes of bilateral cerebellar dysfunction include amongst others:

1. Drugs [phenytoin overdose]
2. Alcohol intoxication
a. Acute intoxication,
b. Subacute affectation with Wernicke encephalopathy [triad of
Korsakov amnestic syndrome, cerebellar ataxia and
nystagmus/ophthalmoplegia]
c. Chronic alcoholic cerebellar degeneration
3. Multiple sclerosis
4. Cerebellar tumors [astrocytoma, hemangioblastoma,
medulloblastoma and metastases]
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5. Disorders of the cranio-cervical junction:

a. Arnold-Chiari-malformation with
herniation of the cerebellar tonsils
through the foramen magnum and
compression of the cerebellum, the
lower brainstem and the lower
cranial nerves (e.g., of the pons and
medulla).
b. Basilar impression with upwards herniation of the rostral
cervical vertebrae through the foramen magnum and
compression of the cerebellum, caudal brainstem, and lower
cranial nerves.

6. Cerebellar degenerations e.g.,:

a) Friedreich’s ataxia (an inherited disorder causing
progressive degeneration of the
nervous system, resulting in
symptoms ranging from gait
disturbance, speech problems to heart
disease)
o depressed or even absent deep
tendon reflexes,
o extensor plantar reflexes,
o sensory ataxia (signs of dorsal column dysfunction),
o pes cavus,
o scoliosis and
o an autosomal recessive pattern of inheritance,
o usually beginning in the second decade.

b) In olivo-ponto cerebellar degeneration (more recently referred

to as autosomal dominant cerebellar ataxia [ADCA] and
spinocerebellar ataxia’s [SCA with its particular number], see
below)
• increased deep tendon reflexes, and
• autosomal dominant pattern of inheritance and
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• onset of the disease between the third to fifth decade

• Additional clinical characteristics include amongst others:
Bradykinesia, rigidity, optic atrophy, retinitis pigmentosa,
dementia, ophthalmoplegia, deafness and myoclonus.

c) In cerebellar degeneration per se of the Holmes type, the onset

is usually in adult life and an autosomal dominant pattern of
inheritance is seen.

d) Multiple system atrophy (see above) may also cause cerebellar

dysfunction.

7. Other causes, e.g., subacute cerebellar degeneration with

underlying malignancy, infarcts of the brainstem (cerebellar infarct or
hemorrhage), arteritis (systemic lupus erythematosus, syphilis) and
cerebellar abscess.

1.1.3 Autosomal dominant cerebellar ataxias (ADCA) [Miyoshi, 2001].

The autosomal dominant cerebellar ataxias are a genetically
heterogenous group of neurodegenerative disorders affecting the
cerebellum and other components of the nervous system and usually have
their onset in the third to fifth decade of life.
Harding classified ADCA’s into three goups based on associated signs:
• ADCA I: Included optic atrophy, ophthalmoplegia, pyramidal signs,
extrapyramidal signs, peripheral neuropathy or dementia (SCA-1, -2,
-3, -8, -12, -13, and-14)
• ADCA II: Retinopathy (SCA -7)
• ADCA III: Absence of associated signs (SCA-5, -6, -10 and -11)

Progressive cerebellar autosomal dominant ataxia’s are labeled as spino-

cerebellar ataxias (SCA’s) and are followed by a number assigned for
each new gene locus (> 23).
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Phenotypical feature (Bradley’s, 2004) Disorders

Young adult SCA-1, SCA-2, MJD
Older adult SCA-6
Childhood onset frequent SCA-7, DRPLA
Benign course SCA-6
Upper motor neuron signs SCA-, -7, -8, MJD, rare in SCA-2
Akinetic rigid Parkinson signs MJD, SCA-2, SCA-17
Chorea Prominent in DRPLA, late in SCA-2, -1,
MJD
Action tremor SCA-12, SCA-16
Very slow saccades early SCA-2, -7
Very slow saccades late SCA-1, MJD
Very slow saccades never in SCA-6
Downbeat nystagmus SCA-6, EA-2
Generalised areflexia SCA-2, SCA-4, older adult onset MJD
Visual loss SCA-7
Seizures SCA-10, early onset DRPLA, SCA-7
DRPLA = Dentatorubral-pallidoluysian
atrophy; EA = Episodic ataxia;
MJD = Machado-Joseph disease;
SCA = Spino cerebellar ataxia

DNA testing is available for Friedreich ataxia, SCA-1, SCA-2, MJD, SCA-
6, SCA-7, SCA-8, SCA-10, SCA-12, SCA-17 and DRPLA.

1.2 Examining motor coordination

Introduction
The assessment of the motor coordination tests in practice evaluate
the functioning of a whole number of motor systems simultaneously
e.g.:
(1) Upper motor neuron tract or pathway
(2) Lower motor neuron tract or pathway
(3) Cerebellar functions
(4) Basal ganglia functions (e.g., nigro-striatal tract in Parkinson’s
disease e.g., bradykinesia).
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When the patient does not show any muscle weakness, (e.g., there is
no lower or upper motorneuron lesion) or signs of Parkinsonism
(e.g., bradykinesia) the presence of in-coordination may probably be
attributed to cerebellar tract dysfunction almost by default.

Traditionally, the examination of coordination and its tests is applicable to

patients with cerebellar dysfunction.
However, it should be noted that patients with an upper motor neuron
lesion, clumsiness of movement is frequently a characteristic early sign.
In the same way in Parkinson’s disease the ability to execute rapid
alternating movements is lost early in the course of the disease as a sign
reflecting the presence of bradykinesia. These abnormalities should not
be mistaken for cerebellar dysfunction.
Local disorders of, for example, the hand, foot and mouth, may also
clearly interfere with movements of coordination. Examples of such
disorders include rheumatoid arthritis, polymyalgia rheumatica and local
infections.
The term ataxia is usually reserved for incoordination not due to
weakness or Parkinsonism e.g., cerebellar or dorsal column sensory
ataxia.

The so-called “neurological examination in 5 minutes” relies heavily

on the coordination tests and the fact that coordination is a way of
collectively testing the UMN-, LMN-, nigro-striatal- and cerebellar systems
at the same time:

The postulate is that a patient, who can pass or perform the following
tests faultlessly, is unlikely to harbor a serious neurological disorder:
• Jumping on each leg separately
• Performing rapid alternating movements of the hands and fingers
• Performing rapid alternating movements of the lips, tongue and
palate e.g., “pitteke-pitteke-pitteke”
• Has normal visual acuity, visual fields (by confrontation) and
fundoscopy
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• Normal eye movements to the sides and up and down

Testing the function of coordination in the evaluation of the motor

systems
If the patient is able to do all the tests mentioned below, it is unlikely that
dysfunctions of the motor systems exist (i.e., LMN, UMN, extrapyramidal
or cerebellar).

1.2.1 Speech
Normal speech with normal rapid repetition of the following sounds:
• “la-la-la-la-la” (tongue)
• “mie-mie-mie-mie-mie” (lips)
• “ng-ng-ng” (soft palate)
• Test the combination of sounds with part of a sentence, e.g.,
“pitteke-pitteke”, “eleven benevolent elephants”

The examiner notes whether the patient can imitate the intonation of the
sounds.

1.2.2 Hands

The fingers of the hand (as a group) are

tapped rapidly alternately dorsally and
palmarly against the palm of the
contralateral hand.
The extended thumb and fingers are
rapidly tapped against each other.
“Marsden’s test” :
The index finger is tapped up- and
downwards as rapidly as possible against
the thumb’s interphalangeal joint.
With the fingers extended and the thumbs
abducted the hands and forearms are
alternately pronated and supinated as
rapidly as possible.
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1.2.3 Gait
In the evaluation of gait it is very valuable to routinely test a set sequence
of motor actions often at the onset of the neurological examination. The
following sequence and order of gait tests are suggested:

• Romberg test (balancing with heels held together and closing the
eyes [see later])
• Normal walking and turning round observing the normal heel-toe gait
and associated swinging arm movments
• Walking on the heels
• Walking on the toes
• Tandem walking (at least five steps)
• Balancing on one leg (eyes open and eyes closed)
• Jumping on each leg separately on one spot

1.3 Examinations for testing coordination (traditionally used in the

evaluation of cerebellar dysfunction).

Cerebellar lesions generally result in alteration in fluidity of motion;

therefore, cerebellar functions are tested to assess coordination of
movements:

1. Speech

2. Upper limbs

3. Lower limbs

4. Trunk and gait

1.3.1 Muscles of speech

Normal speech: production of sound:

While listening to the patient’s speech the following are noted:
• Prosody of speech (the melody of a patient’s voice)
• The volume of the voice
• The intonation of speech
• Scanning (words are prolonged by breaking them up into syllables).
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• Slurring of the tongue

Rapid alternating movements of the muscles of speech are tested as
mentioned above (see section 1.2.1).

Abnormal speech: Types of dysarthria (incoordination of speech)

(1) Cerebellar dysarthria may show an abnormality of prosody, atactic

intonation, scanning speech and an element of slurring. The patient
is unable to imitate rapid alternating movements of the lips, tongue
and soft palate. Concomitant signs of cerebellar dysfunction are
usually found in the arms, trunk or legs.

(2) Dysarthria due to Parkinson’s

disease may show a monotone (loss
of melody), low volume (sometimes
whispering) speech. Patients with
Parkinson’s disease are unable to
perform rapid alternating movements
of the lips, tongue and soft palate
(sounds), in addition to slurring.
Concomitant signs of Parkinson’s
disease in the trunk and limbs
(tremor, bradykinesia, rigidity and
postural dysfunction) confirm the
diagnosis.

(3) Pseudobulbar speech (bilateral UMN involvement) shows a slow,

spastic, low pitched slurring speech. Rapid alternating movements
of the lips, tongue and soft palate reveal the slow, jerky, low volume
sounds reminiscent of the spastic stiff muscles of speech.
Concomitant upper motor neuron signs e.g., positive jaw jerk, snout
reflex, positive glabella tap as well as spasticity and hyper-reflexia of
the limbs and extensor plantar responses may be found.
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(4) Bulbar dysarthria (lower motor neuron affectation) may show a

diminution of the volume of speech with an element of slurring, a
nasal element or unclear lip sounds. The quantitative contribution of
the weakness of the lips, tongue or soft palate to the dysarthria is
assessed determining which components are involved maximally.
The test of rapid alternating movements is, until relatively late,
reasonably normal. Concomitant signs of the specific causative
disease may be present.

In progressive bulbar atrophy (a form of motor neuron disease with

degeneration of motor neurons or nuclei in the pons and the medulla, with
a pseudobulbar component additionally present as a rule) the tongue may
show atrophy and widespread fasciculations. The facial muscles around
the mouth may be weak and the soft palate may be paralysed.
Progressive bulbar atrophy presents characteristically with a slow onset
progressive dysarthria and dysphagia. Additional signs of motor neuron
disease are often seen in the arms, legs and trunk.
Myasthenia gravis, Guillain-Barré syndrome and polymyositis are other
causes of a bulbar dysarthria.

1.3.2 Examinations of coordination in the upper limb

(1) Rapid alternating movements of the hands

(dysdiadochokinesia)
The fingers of the hand (as a group) are tapped rapidly alternately
palmarly and dorsally against the palm of the contralateral hand.
Note the smoothness, speed and neatness of the movement and
how much movement occurs at the more proximal joints of the
limbs. Compare the two sides with each other. The inability to
perform rapid alternating movements is called
dysdiadochokinesis.

(2) Rapid alternating movements of the fingers

The fingers are, for example, opposed alternately against the
thumb while the speed and smoothness of the movements are
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evaluated. Marsden test: The index finger is tapped as rapidly as
possible up and down against the distal interphalangeal joint of
the thumb (see 1.2.2).

(3) Index finger-circle test

With the index finger a circle is drawn on the dorsum of the
contralateral hand. The speed and smoothness of the movement
and roundness of the circle are determined. In conditions causing
incoordination, the circle has multiple angles.

(4) The index finger-nose-finger (examiner’s) test

• The patient moves his
index finger between two
points (his own nose and
the examiner’s finger,
which is held in front of
him).
• The shoulder is held in abduction (to accentuate
abnormalities of this test) and the patient needs to “reach out
at a distance” to the endpoint.
• Intention tremor is determined by noting the development of a
tremor that increases in frequency and amplitude of
oscillation, as the index finger is nearing its target. This
tremor is sometimes so severe that the patient is unable to
keep the finger on the target (because of the induction of the
tremor).
• Note the ability to reach the target directly or whether the
finger passes the target or stops too early (dysmetria) and to
which side the finger diverges or overshoots.

(5) The rebound phenomena

While the biceps muscle is activated against resistance (the
examiner’s other arm prevents the patient’s arm from rebounding
excessively and injuring the patient) the arm is suddenly released
and the velocity, force and degree of the backward movement are
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determined. In patients with cerebellar dysfunction the biceps

muscle is unable to relax in time and the forearm often knocks
against the examiner’s protecting forearm.

(6) Oscillation phenomenon of extended arms

The arms are extended in front of the patient (with eyes closed),
while the examiner lightly taps one arm after the other and notes
the amplitude and length of the oscillations. The side of the
cerebellar dysfunction has oscillations of larger amplitude and
longer duration.

(7) Line drawing test

The patient tries to draw a
line between two lines
without touching either of
them. In cerebellar dysfunction the sides are touched repeatedly
(also seen in chorea and other motor dysfunctions).

1.3.3 Examinations of coordination in the lower limbs

(1) The heel-knee-to-ankle test

• The patient (who is lying supine) moves
the heel to the contralateral knee and
then down the shin to the ankle as
accurately as possible up through the
air and back to the knee, and repeats
the movement.
• It is a good habit to demonstrate the maneuver requested to
and on the patient
• In cerebellar dysfunction an intention tremor is sometimes
seen (as the heel nears the knee or even over the shin) and
wide to and fro oscillations are noted while the heel moves
along the lower leg.
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(2) The big toe-to-finger (examiner’s) test

While the patient moves his leg and big
toe towards the examiner’s index finger
(above the foot end of the bed) an
intention tremor and dysmetria may be
observed.

(3) The test of rapid alternating tapping movements of the foot

Dysdiadochokinesis of the foot against the examiner’s palm of the
hand is observed. The side of the cerebellar dysfunction will
show the slower and ataxic movements.

(4) Heel-to-shin-circle test

The patient moves his heel up and down onto a specific point (a
circle drawn with ink on the contralateral shin) and the presence
of dysmetria or tremor is noted.

1.3.4 Examination of coordination of the trunk and gait

Refer to section 1.2.3

(1) Truncal ataxia

Ataxia of the trunk may be seen in isolation or together with other
cerebellar signs. Truncal ataxia as the only positive symptom or
sign with, for example, a normal neurological examination in the
supine position, may be found. Ataxia may be present on sitting,
standing or walking during which reeling may occur to the sides or
backwards. There may in addition be the presence of an inability
to walk tandem or to stand or jump on one leg separately. Lesions
of the vermis and archi-cerebellum (midline areas)
characteristically cause truncal ataxia.

(2) Cerebellar ataxia (incoordination) of gait

• The normal gait is narrow based (footsteps not wide apart,
near one another).
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• In cerebellar dysfunction the patient tends to walk on a broad

base with a reeling component. It is amazing how patients
nevertheless maintain their balance.
• While standing upright with feet placed next to one another,
continuous contractions of the small muscles of the feet
(dancing muscles of the feet) may be observed.
• In patients with a slight cerebellar dysfunction the finer test of
heel-to-toe walking (at least five steps
without falling) and of standing alternately
on one leg (eyes closed) and jumping up
and down on one leg at a time are utilized.
The test of standing on one leg (eyes open
or closed) may be quantitized by counting
the number of seconds that the patient is
able to maintain the relevent position.

(3) Gait dysfunction in Parkinson’s disease (postural

dysfunction)
Dysfunction of gait is very commonly seen in Parkinson’s disease.
• The gait tends to be shuffling (toe-to-heel-gait), consists of
short steps
• and may show propulsion [festination] (increasingly rapid
short steps forwards with an inclination towards running and
falling) or retropulsion.
• The patient shows decreased or absent normal associated
movements (swinging of the arms) while walking,
• and a stooped flexed attitude may be characteristic of the
later phases of the disease (almost stature like).
• The patients tend to turn around slowly (with multiple small
steps).
• Freezing, or catching of the foot with a very short step or
inability to move are characteristic of Parkinson’s disease.
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• Orthostatic hypotension may interact as

an additional complication.
• A gait ignition failure failure may be
found as an isolated phenomenon,
where the patient is unable to initiate
walking, but walks well once started and
this disorder may respond well to
levodopa treatment.

(4) Spastic gait dysfunction

The legs appear stiff and straight, the front of the
feet drag (touch the floor first) and the legs may
be moved in slight circumduction, while the steps
are relatively short with a reasonably narrow base
gait. The knees are usually held in extension.

(5) Drop-foot gait in peripheral neuropathy

The feet slap (sometimes with a sound) against
the ground, with the front of the feet touching the
ground first, the knees are lifted up relatively high
to prevent the toes from dragging. A broad-based
component may sometimesbe found.

(6) Sensory ataxia (dorsal column gait)

The balance is characteristically disturbed in the dark. The
patient walks with a high stepping slapping gait (patient does not
quite know where foot is situated in space or when it is going to
touch the ground). The Romberg test is abnormal. The Romberg
test consists of the determination of the patient’s balance with
eyes closed (patient keeps feet against one another and balances
with eyes open at the beginning of the test). The patient should
not sway more than a few centimeters to and fro and should keep
his balance. In dorsal column dysfunction the patient usually
sways to and fro and may show a tendency to fall.
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(7) Myopathic “waddling” gait

The patient’s pelvis drops on the side of the
elevated leg (the contralateral gluteus medius
muscle is too weak to lift the pelvis). When the
gait is inspected from behind, the up and down
rocking movement of the pelvis on both sides
is seen. Patients with muscular dystrophy often Normal Abnormal

show this type of gait abnormality.

(8) Vestibular gait abnormality

The main accompanying symptom is vertigo which may be
increased by movement of the head. In addition to the vertigo,
the patient is often nauseous, vomits and may show a tachycardia
(signs of sympathetic over-activity associated with the vertigo).
Patients with vestibular dysfunction may also be subject to
sudden falling attacks.

(9) Acrobatic, conversion or “no organic pathology found” gait

The diagnosis of conversion may only be made by an
experienced clinician and even then, the diagnosis is still often
wrong or an underlying associated masked organic condition may
be present. The “acrobatic gait” seems dramatically severe and
usually consists of a series of rapid repetitive movements, with
short steps, on a narrow base, while the legs tend to be held in
flexion (in reality excellent coordination). The gait appears totally
bizarre and fits into no other category of gait disturbance.
Suggestion by the examiner that he understands the cause of the
gait disturbance, and that balance may characteristically be
retained or even better on one leg than two may help in the
unmasking of the gait disturbance. The patient frequently
maintains his balance on one leg (often with bizarre posturing). It
may be wiser to refer to the condition as “no organic pathology
found” than to conversion.
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Chapter 21
THE PAEDIATRIC NEUROLOGICAL EVALUATON

Prof. I Smuts, TW de Witt and Department of Paediatrics

1.1 Objectives in the Neurological Evaluation of a child

1.2 Objectives in taking the history
1.3 Objectives in the neurological examination
1.4 Objectives in the evaluation of the Musculoskeletal system
1.5 Skeletal examination
1.6 Clinical examination
1.6.1 Development of Communication “ELM Scale (Early learning milestones)
1.6.2 Modified Glasgow Coma Scale for children
1.6.3 DSM IV criteria for attention deficit
1.7 Definitions: Head Shapes
1.8 Skull Growth
1.9 Cranial nerves
1.10 Definition of visual acuity and vision of normal child
1.11 N. VII palsy
1.12 Neck and Back
1.13 Raised intracranial pressure
1.14 Motor system
1.15 Gross motor milestone
1.16 Distribution of power
1.17 Reflexes
1.18 Primitive reflexes
1.19 Sensory system
1.20 Basal ganglia
1.21 Cerebellar function
1.22 Developmental assessment
1.22.1 Gross motor development
1.22.2 Fine motor development/manipulation
1.22.3 Language and communication development
1.22.4 Psychosocial development
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1.22.5 Normal milestones

2. NEUROLOGICAL EVALUATION OF A NEONATE

2.1 Signs of meningeal irritation

3. REFERENCES
283

The neurological evaluation of a child does not only differ from the adult
neurological examination, but is also differing with regard to the different age
groups. The evaluation is not always systematic and should be adapted to the
circumstances. Informal observation of the younger child is essential. Both the
emotional status and underlying disease should be taken into account.

The young brain undergoes a process of maturation and sufficient time should be
allowed for primitive reflexes to be integrated before certain milestones can be
evaluated.

The stage of development should always be taken into consideration when the
child is examined; therefore, the developmental assessment forms an integral part
of the neurological examination. It is important to determine whether the
development has reached a plateau or if any regression has occurred.

The paediatric disease profile includes cerebral palsy, meningitis, degenerative

diseases and learning disabilities.

It is important to take an accurate, chronological history. Special attention should

be paid to the frequency, duration and characteristic of the specific symptoms.
The integrity of all other systems should be investigated, because abnormalities of
the central nervous system can manifest as diverse symptomatology, e.g.,
vomiting, pain or constipation. The interaction between the child and the parent,
the presence of tics, abnormal movements and level of activity should be noted.

The chronological age should be corrected for prematurity when a developmental

assessment is done, i.e., three months should be subtracted from a premature
baby’s age when he was born three months too early.

1.1 Objectives in the Neurological Evaluation of a child

The objective is to determine the integrity of the central nervous system
by taking a detailed history and performing a physical examination. The
site, extent of the lesion and aetiology of the abnormal function should be
determined.
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The following information should be included in the diagnosis:

• Clinical pathological diagnosis which describes the site and extent of

the lesion
• Aetiology
• The degree of illness
• Complications present

1.2. Objectives in taking the history

Main complaint Reason for referral
Chronological exposition
Related complaints and symptoms

Family history Mother

Father
Grandparents
Siblings

Pregnancy history Age of mother

Antenatal risk factors
Parity
Gravity
Abortions
Illnesses during pregnancy
Smoking
Drugs taken

Birth history Gestation

Type of delivery
Apgar scores
Birth weight, height and skull circumference

Neonatal period Condition after delivery

Admission to a neonatal unit
Course
Duration of hospitalisation

Developmental history Milestones

Medical history Nature

Age of onset
Duration
Complications
Previous and current treatment
Previous surgery
Previous trauma

Family structure Including occupation of parents

Behaviour Including sleeping patterns

Progress in school
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1.3 Objectives in the Neurological Examination

a) General impression
b) Higher functions
c) Head and face
d) Cranial nerves
e) Neck and back
f) Signs of raised intracranial pressure
g) Motor system
h) Sensory system
i) Basal ganglia
j) Cerebellar function
k) Autonomic system
l) Neuro-cutaneous markers
m) Developmental assessment

1.4 Objectives in the evaluation of the Musculoskeletal System

The neurological evaluation is adapted with emphasis on the motor
system and the motor milestones.

In addition the joints, skeleton and muscles are also examined.

1.5 Skeletal examination

Joints Inspection Gait

Contour
Swelling and/or effusions
Skin
Position joint kept in
Palpation Tenderness
Heat
Effusions
Crepitations
Contractures
Movement Range of movement
Stability

Skeleton Inspection Gait

Posture
Symmetry
Body ratios
Spinal column
Swellings
Palpation Tenderness
Rickety rosy
Craniotabes
Broad wrists
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It is important to examine all the other systems in order to not to miss any
additional markers of disease.
Equipment:

Tape measure

Stethoscope

Torch

Ruler

Tongue spatulas

Container with coffee

Container with salt and sugar

Otoscope

Ophthalmoscope

Turning-Fork 256 Hz

10 mℓ Syringe

Cotton wool

Two glass tubes for hot and cold water

Pins

Reflex hammer

Objects to test stereognosis, e.g., coin, key, paper-clip

Blood pressure apparatus

Toys: blocks, beads and string, bell, books

Ball

Denver developmental chart

Snellen chart

Rattles for hearing screening

Crayons

Paper
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1.6 Clinical examination

General Describe the physical habitus of the patient

Weight
Height
Skull circumference

Dysmorphic features
Posture
Movement

Higher functions General behaviour Normal

Hyperactive
Quiet
Neatness

Mood Appropriate responses

Quiet
Laughter
Tantrums

Thought content Fears

Hallucinations

Intellectual ability Mathematical ability

Language ability
Development of
communication1

Sensorium Consciousness2
Attentionspan3
Orientation
Memory
Insight and judgement

Speech Fluency
Dysphonia
Dysarthria
Dysphasia

The age of the patient should be taken into account when the higher
functions are evaluated.

In a baby, attention should be given to the responsiveness, interest in the

surroundings, attentiveness and concentration.

In older children the perception, mathematical abilities, attention, memory,

reading, writing and spelling abilities should be tested.
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The emotional welfare of the child should be taken into consideration:

e.g., motivation, depression, dependence, self-image, child-parent
interaction and signs of deprivation.

Early danger signs:

• Inconsolable crying
• Bruxism
• Hand admiration after 20 weeks
• Everything brought to the mouth after 9 months
• Purposeless throwing of toys after 9 months
• Self-stimulation behaviours
• Self-injurious behaviour

1.6.1 Development of Communication

“ELM Scale (Early learning milestones)”
(SAMJ Vol 82:24,July 1992)

1.6.2 Modified Glasgow Coma Scale for Children

Opening of the eyes 4 Spontaneous
3 To verbal command
2 To pain stimuli
1 No response

Motor response 6 Spontaneous

5 Localises pain
4 Withdrawal on pain stimuli
3 Decorticate posture – Flexion on pain
2 Decerebrate posture – Extension on pain
1 No response

Best. verbal response 5 Orientated

Social smile
Localises sound
Follows objects
Appropriate interaction with surroundings

4 Disorientated
Consolable crying
Aware of surroundings
Uncooperative interaction

3 Inappropriate/continuous crying
Changing awareness of his surroundings

2 Incomprehensible sounds
Agitated
Restless
Inconsolable
Unaware of surroundings

1 No response
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1.6.3 DSM IV criteria for attention deficit (Abridged from ADHD criteria)
• An inability to pay focussed attention to detail is present and the patient makes
careless mistakes in his schoolwork, or other activities
• It is difficult to keep attention to an activity or play
• It appears as if he is not listening
• It is difficult to organise tasks
• Does not follow instructions until the end and does not complete tasks
• Dislikes difficult tasks
• Constantly looses objects
• Forgetful
• Distracted by external stimuli

Head and face Inspection Configuration4

Skull circumference5
Facial expression
Eyes, nose, hair, mouth
Facial symmetry

Palpation Sutures
Fontanels (close at 6-18 months)
Craniotabes

Auscultation For possible bruits

Percussion “McEwen” cracked pot sign

1.7 Definitions: Head Shapes

Plagiocephaly:
The head appears as a parallelogram from above.
It is most often a “postural” defect.
Scaphocephaly:
The head is long in the antero-posterior diameter.
Associated with: prematurity or sagital synostosis.
Turricephaly:
The head is tall due to compensatory upward growth.
Associated with: Bilateral coronal synostosis.
Bragicephaly:
The back of the head is flattened.
Associated with: Down syndrome
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1.8 Skull growth

Microcephaly:
The skull circumference is less than two standard deviations of mean for age, race and
sex.
Aetiology: Perinatal hypoxia
Intrauterine infections
Chromosomal abnormalities
Familial
Severe metabolic disorders
Macrocephaly:
The skull circumference is more than two standard deviations of mean for age, race and
sex.
Aetiology: Hydrocephalus
Storage disorders
Space occupying lesions
Familial macrocephaly
Sotos syndrome

Head circumference graphs: Pediatrics 41:106, 1968.

Delayed closure of fontanels:

Hydrocephalus
Down syndrome
Hypothyroidism
Rickets
Skeletal dysplasias

1.9 Cranial nerves

I Smell Impossible to test in babies
Coffee, soap or an orange can be used in older
children

II Visual accuracy Pupil light reflex

(Can he see?) Gunn phenomena
Visual field
Visual acuity6
Colour vision
Night vision
Fundoscopy

III, IV, VI Eye movement Ptosis

Strabismus
Diplopia
Eye movements
Doll’s eye movement

V Chew Motor division: Muscle bulk, bite on a spatula,

and chew.

Sensory division: Corneal reflex, rooting reflex,

jaw reflex.
291

VII7 Facial expression when crying and laughing.

Frowning
Closure of eyes
Smiling
Whistle
Press lips together

VIII (refer) Vestibular part: Vertigo

Balance
Nystagmus
Hearing: Whispering
Rinne (Mastoid)
Weber (Forehead)
Rattles

IX, X (refer) Swallow

Dysphagia
Nasal speech
Hoarseness or aphonia
Gag reflex

XI (refer) SCM
Trapezius
Turn head against resistance

XII (refer) Protrude tongue

Fasciculation

The most common cranial nerve problems in children include: Strabismus

and facial nerve palsy (congenital or acquired). Weak or absent sucking
in a baby is a serious neurological sign. There is reason for concern
when a baby does not have a social smile at the age of 6 weeks.

1.10 Definition of visual acuity and vision of normal children

Visual acuity –
Distance from a patient to the chart (6 m or 20 ft)
Distance where smallest letter a patient can read has an angle of incidence of 1

Neonate 20/400
1 year 20/200
2 years 20/40
4 years 20/30
5-6 years 20/20

A normal neonate can follow, but this action is well established at the age of 6-8 weeks.
292

1.11 N VII palsy

Distinguish from:

1. Cayler syndrome (Congenital absence of the M Depressor Anguli Oris).

2. Moebius syndrome with absent nuclei of N VI and N VII.

1.12 Neck and Back

Neck and Back Signs of meningeal Neck stiffness
irritation Kernig
Brudzinski

Spina Bifida

Kyphosis

Lordosis

Scoliosis

Tenderness

1.13 Raised intracranial pressure

Signs of raised Headache Early morning throbbing
intracranial pressure Vomiting N VI

Diplopia

Bulging fontanel

Diastasis of the sutures

Sun setting sign Sclerae are visible above the iris

and is present with
hydrocephalus. It can be
present in normal babies.

McEwen “cracked pot” Tympanic sound obtained

when a skull is percussed
with raised intracranial
pressure.

Cushing response Blood pressure raised and

pulse slows down.

Papilloedema Hyperaemia, venous

congestion, no pulsation,
blurring of the edge of the
optic disk.
293

1.14 Motor system

Motor system Gait Broad or narrow based toe or
flat foot walking
Eversion of feet
Limping
Hemiplegia/circumduction
Ataxic
Waddling
“Slapping” = distal limb
weakness

Posture and Symmetry

Motor milestones8
Muscle bulk Hypertrophy
Pseudohypertrophy
Atrophy

Muscle tone Hypertonia

Hypotonia

Power Distribution of fall out9

Grading
Range of movement of joints

Cerebellar system
Fasciculations
Myotonia
Palpate Tenderness (Pain elicited when
muscle is pressed in myositis)
Texture (Muscle feels like
dough with polymyositis)

Tone is age dependent. Head lag, head control in ventral suspension and
passive movement of the joints are the best indicators of muscle tone in a
neonate. Continuing clonus should disappear after the age of two
months.
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1.15 Gross motor milestones

At Birth Flexion dominates

2 weeks Prone position: Lifts the chin

2.5 months Prone position: Holds with 45°to back

4.5 months Role over

5 months Proper head control when sitting

6 months Sits and leans forward on hands, lifts head when lying,
bears weight on both legs. No “head lag”.

9 months Sits, rotates, stand up when pulled

1 year Crawls, walk with help

14 months Stands alone

15 months Walks, crawls stairs upward

18 months Walks UP and down, runs, and walk up stairs

2 years Runs, kick a ball, walks up and down stairs

3 years Stands on one leg, jump from stairs

4 years Jumps on one leg

5 years Hop

1.16 Distribution of power

Upper Limb Proximal Shoulder falls through
Cannot walk on the hands like a
wheelbarrow

Distal Cannot pull himself up when held by his

hands

Lower Limb Proximal Gower

Cannot climb stairs

Distal Cannot stand on his toes

1.17 Reflexes
Reflexes (Part of the Deep tendon Nerve roots
motor system) reflexes Grading

Superficial Nerve roots

reflexes

Primitive Types
10
reflexes
295

Reflexes can be suppressed when a baby is crying or has voluntary

movements. Upper motor neuron lesions cause brisk reflexes, crossing
and mass reflexes.

1.18 Primitive reflexes

Reflex Appear Disappear

Moro 28 weeks 2-5 months

Grasp reflex At birth Palmar 2-6 months

Plantar 9 months

ATNR 2 Weeks 3-5 months

(Asymmetric tonic neck reflex) Maximum: 2 months
Rooting and sucking 34 weeks 9 months

Parachute 8-9 months Never

Primitive reflexes have a normal pattern of appearing and disappearing

they play an important role in the ability to develop voluntary actions.

Moro: The moro reflex is elicited by the sudden dropping of the head at
about 30° in your hands. The hands will open and the arms will extend
and abduct with subsequent flexion of the arms.

An exaggerated or persisting Moro reflex may be an indicator of cerebral

palsy. An asymmetric Moro may indicate a paresis or if it is absent the
baby’s level of consciousness may be suppressed.

Palmar grasp reflex: A finger is placed in the baby’s hand from the ulnar
side and the baby then grasps it. An absent reflex may indicate an Erb’s
paralysis.

Plantar grasp reflex: If it is still present after the age of nine months, it
may be an indicator of cerebral palsy.
296

ATNR: The head is turned passively to the side; the limbs will be in
extension on the same side and in flexion on the contra-lateral side. A
persisting ATNR may be an indicator of cerebral palsy.

Parachute: The baby is suddenly “dropped” with the head downward; the
arms will extend in a protective manner. An asymmetric reflex may be an
indicator of paralysis.

1.19 Sensory system

Sensory system Superficial sensation Pain
(refer) Temperature
Light touch

Turning fork 128 Hz

Proprioception (4-5 years)

Stereognosis (1mm on the

tongue, 2 mm on finger tip,
8-12 mm on palm of the
hand)

Deep sensation Position

The procedure should be adapted in children. Posterior column sensation

can only be tested in bigger children. Be careful when testing for pain.

1.20 Basal ganglia

Basal ganglia These movements often flow from the one into the other except for
ballismus. They are usually absent in sleep.
Chorea Quick uncoordinated movements
Often hypotonic “Spooning”
“Milkmaid’s Hand”
“Stick out the tongue” Harlequin tongue.
Athetosis Writing and turning movements usually in the
distal muscle groups
Trunk and limbs
Flexion of the thumbs
Grimacing
Dysarthria
Drooling
Dystonia Irregular movements of the trunk and/or limbs

Ballismus Proximal movements

Arms > Legs
297

1.21 Cerebellar function

Cerebellar function Gait Ataxic
(Part of the motor Broad base
system) Legs Hypotonic
Pendullar reflexes
Cannot walk on a straight line
Dysmetria: Heel on shin
Dysdiadochokinesis

Trunk Truncal ataxia

Neck Titibation

Eyes Nystagmus
Opsoclonus

Arms Intention tremor

Finger-nose test
Dysmetria
Dysdiadochokinesis

Speech Slow, slurred or explosive

Autonomic System: Sphincter control

(refer) Blood pressure
Ability to sweat
Horner

Markers Dysmorphic features

Neuro-cutaneous markers, e.g., hypo or hyperpigmented areas.
Abnormal smells may be present in neurometabolic diseases.

Developmental Gross motor

11
assessment Fine motor
Speech and language
Psychosocial development

1.22 Developmental assessment

Development follows an orderly pattern. A developmental history is
important in older children, but the maturity of the actions should also be
evaluated.
298

1.22.1 Gross motor development

Definition
“The progression of abilities which ultimate enable the child to assume an upright
position and perform skilled activities while maintaining posture and equilibrium.”

Neonate: - Relative hypotonic

- Flexor position
- Primitive reflexes: Moro
Grasp
Placing and stepping
Rooting and sucking

First few months: - General increase in tone

- Extensor facility develops

Six months: - Extension of all joints possible

- Possible to break with total flexion or extension patterns

Extension of the back and flexion of the hips are required to sit. Primitive reflexes
should integrate: The palmar reflex should be integrated before weight can be borne on
the forearms. Righting reactions and parachute equilibrium responses should be
present before a child can walk, run and climb.

1.22.2 Fine motor development/manipulation

Definition
“A series of skills which develop through a visually guided ability to grasp, thumb
apposition and the transition from unilateral to bimanual manipulation”.
Neonate Grasp reflex dominates
1 month Follow with eyes to midline
2 months Hands open
2.5 months Follow with eyes past the midline
Next three months Visually guided reaching
5 months Reach and grasp for an object
Mouthing
6 months Transfers objects
Palmar grasp
Holds bottle
8 months Holds objects in both hands
9 months Explores with index finger
12 months Good pincer
Releases an object
Mouthing less frequent
Throws toys out of cot
15 to 18 months Increasing bimanual
Next three years Perceptual skills
Sensory integration
299

1.22.3 Language and communication development

Definition
“Communication refers to the transfer of meaningful symbols. Language is the primary
medium of communication and involves the formalisation of thought”.
Neonate Cries
Responds to bell
8 weeks Vocalises
3 months Laughs
7 months Babbling
8.5 months Turns to voice
1 year Two to three meaningful words
13.5 months Says “mama” and “papa” with meaning
18 months Two words utterances
2 years Short phrases
3 years Extensive vocabulary
Immature articulation
5 years Mature articulation
Full sentences

1.22.4 Psychosocial development

Definition
“Personal development is assessed on culturally monitored skills of daily living and
social development is behaviour which is in accordance with social expectations. This
acquired through socialisation.
Neonate Bonding
1 month Focuses on face
6 weeks Visual smile
3 to 4 months Smiles and vocalises with strangers
Tries to hold bottle
6 months Responds to mirror image
Can chew
8 to 9 months Stranger anxiety
Can drink from a cup when held
Hold and eat a biscuit
15 to 18 months Domestic mimicry
Attempt to use a spoon
Pulls up trousers
Indicate that nappy is wet
2 years Plays mainly alone
Starts to be dry during day
3 years Group activities
Potty trained
4 to 5 years Dress without supervision
300

1.22.5 Normal milestones

6 weeks Fixates, follows and smiles
Little “Head lag” when pulled to sit
Prone: Lift chin momentarily
3 months Hands open
Starts to look at hands
In ventral suspension: Lifts head to level above back
Prone: Lifts chin and upper part of thorax from bench
Babbling
6 months Reaches for toys with palmar grasp
Transfers from hand to mouth and from hand to hand
Sits with support or tripod sitting
Rolls from prone to supine
Prone: Lifts head and trunk with elbows in extension
9 months Sits without support
Can pick up toys without falling
Rolls over easily
Starts to crawl
Understands “no”
1 year Walks around furniture
Can walk alone
Pincer grasp
Throws away
Enjoys pictures
Understand several words
“Papa” and “mama”
2 years Walks, runs, kneels and climbs stairs with two feet on a
step
Feeds self with spoon
Can be dry by day
Builds tower with six blocks
Can use three word utterances
3 years Climbs stairs with one foot per step
Rides tricycle
Can imitates a circle
Uses sentences
Knows several of colours
Knows rhymes
Can count up to ten
4 years Climbs stairs normal
Can hop
Can wash, dresses and undress, but cannot tie shoelaces
Listen to stories
Can draw a man with head, chest and legs
Can copy a cross
301

2. NEUROLOGICAL EVALUATION OF A NEONATE

2.1 State of alertness

State of alertness Sleep Regular breathing, eyes closed, no eye
movements or motor activity (non-REM sleep)

Light sleep Eyes closed, rapid eye movements, irregular

breathing (REM-sleep)

Drowsy Eyes open or closed, varying motor activity

Awake Eyes open, sparse movement

Eyes open Active

Crying

Note: The state of awareness has an influence on the response when the
neurological examination is performed. The environmental
temperature,and light also influence the state of alertness as well as,
handling and whether the baby has been fed recently.

Posture Normal Moderate flexions of the limbs, hands are

closed.

Abnormal Persistent asymmetry, mainly extension of the

limbs, persistent rotation of the head to one
side, opisthotonus and adduction of the thumb
in the palm of the hand (“fisting”).

Social behaviour Consolable

Eye contact

Abnormal A high frequency tremor with low amplitude may be normal,

movements especially when the baby cries. Slow, coarse, clonic and mainly
asymmetric movements are abnormal.
302

Cranial nerves II Blinks eyes in response to light. Transient

irregular pupils may be normal. Pupil
constriction to light is usually present, but
it is difficult to test in babies and the
response may be slow. A baby starts to
fixate at 2 to 4 weeks.

III, IV, VI Ptosis, strabismus and nystagmus

Elicit “doll’s eye movement”
Note:
Technique: Hold the baby under the
armpits and support the head vertical in
the midline with the thumbs against the
cheeks. Rotate slowly in one direction.
The eyes will show conjugate deviation in
the direction of rotation and the fast
component of nystagmus will be in the
opposite direction. The direction changes
as soon as the rotation is stopped. Tilt
the baby forward: The eyes open wider
and the eye movement is often upwards.

V The Masseter and Temporalis muscle are

used when the baby is drinking.

V + XI Rooting reflex

V + VII Corneal reflex

V Jaw reflex
VII Observe for symmetry of movement when
the baby is crying or drinking.
The baby may open his eyes when he is
held in ventral suspension.

VIII Vestibular part: Test for rotation

nystagmus (labyrinth).
Hearing

IX, X, XII Gag-reflex

Observe the way the baby is sucking and
swallowing.
Gently press on the baby’s nostrils: The
mouth will open and the tip of the tongue
will move upwards.
303

Motor system Posture Moderate flexion dominates in normal

neonates. Persistent extension of the
limbs, asymmetry, the frog position and
opisthotonus are abnormal.

Movements Normal movement of a neonate:

Alternating flexion and extension of all
four limbs. >6 Hz low amplitude tremor
especially when the baby cries can be
normal. Slow <6 Hz coarse, clonic and
asymmetric are abnormal.

Muscle bulk Compare left and right.

Tone Observe the spontaneous movements
Power and posture.

Sensory system It has limited value in babies. Gentle pricking with a pin can be
done. Normal babies have a higher pain threshold than older
children. Note the facial expression and the movement of the
limbs. Absence of the withdrawal response can be due to a
lesion to either the motor or sensory system. No movement
rather indicates to a paralysis than anaesthesia. Reflex
withdrawal can be present with a spinal cord lesion without any
changes in the facial expression.

Reflexes Tendon A finger can be used instead of a patella

hammer.
Knee reflex is present at birth.
Ankle reflex is absent at birth and appears
at 4 months.
Plantar Babinski response can be normal until 2
years of age.

Palmar grasp Subcortical and C6 to C8. Present after

reflex 32 weeks of gestation.

Moro Subcortical. Can be present beyond 4

months in premature babies.

Stepping To a great deal dependant on parietal

function.

Crawl

ATNR Subcortical. Well established on 1 month.

Galant Subcortical, absent with transverse spinal

lesion.

Plantar grasp Subcortical. Present at birth.

304

Crossed adductor Present shortly after birth. Keep the leg in

extension, stroke the sole of the foot; the
opposite leg will have rapid flexion
followed by slow extension and adduction.

Rooting Subcortical. Present 2-3 days after birth.

Serious CNS pathology is suspected
when it is absent.

Sucking and Subcortical. Sucking is already present at

swallowing 24 weeks gestational age and it
disappears at one year. Absence at any
age is serious.

Ventral The normal response is flexion at the hips

suspension and knees. Scissoring may indicate
towards cerebral palsy or spastic diplegia.

Ankle clonus > 10 contractions are abnormal.

Anal reflex S5

Pupil reflex Present at 29 weeks of gestation.

Auditory reflex Present at birth.

The baby blinks his eyes or the breathing
pattern changes when there is a sudden
loud noise.

Abdominal and Present at birth.

Cremaster reflex

2.2 Signs of meningeal irritation

Skull Circumference
Shape
Sutures
Fontanels

Back Scoliosis
Fistulas
Meningocoele
305

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316
INDEX

Acalculia ----------------------------------------------------------------------------- 97
Abdominal reflexes---------------------------------------------------------------- 40,203,221,233
Accessory nerve ------------------------------------------------------------------ 167,168
Accommodation Reflex ---------------------------------------------------------- 148,149
ADEM (Acute Disseminated Encephalomyelitis) ------------------------- 9,43,48,50,56-57,134
Amyotrophic lateral sclerosis--------------------------------------------------- 9,201,203,205,207
Anal reflex --------------------------------------------------------------------------- 40,235
Ankle reflex ------------------------------------------------------------------------- 45,46,50,214,233,303
Anosognosia ------------------------------------------------------------------------ 97
Anterior horn cell disease ------------------------------------------------------- 171,177/8,202-
206,254
Aphasia ------------------------------------------------------------------------------ 103-111
Archicerebellum ------------------------------------------------------------------- 160,165
Argyll Robertson pupils ---------------------------------------------------------- 148
Athetosis ---------------------------------------------------------------------------- 182,296
Atlanto Axial dislocation --------------------------------------------------------- 50
Atypical facial pain ---------------------------------------------------------------- 74
Aura epilepsy ----------------------------------------------------------------------- 27,29,30
Aura migraine---------------------------------------------------------------------- 3,20,61,71,72
Automatism epilepsy ------------------------------------------------------------- 27
Autonomic neuropathy ---------------------------------------------------------- 260,261
Autosomal dominant cerebellar ataxias (ADCA) -------------------------- 267,268
Babinski response ------------------------------------------ ----------------------- 116,221,234,303
317

Barré test ---------------------------------------------------------------------------- 192

Bell’s palsy -------------------------------------------------------------------------- 158,210
Biceps reflex ------------------------------------------------------------------------ 40,215,231,232
Bite reflex ---------------------------------------------------------------------------- 238
Brachioradialis reflex ------------------------------------------------------------- 232
Brain death------------------------------------------------------------------------- 21,146,234,235-
237
Brainstem reflexes ----------------------------------------------------------------- 238
Brown Sequard syndrome (hemi-spinal cord syndrome) --------------- 253,
Bulbar palsy- ------------------------------------------------------------------------
169,170,206
Calculation Ability ----------------------------------------------------------------- 87
Carpal tunnel syndrome --------------------------------------------------------- 210,212,293
Central pontine myelinolysis---------------------------------------------------- 57
Central spinal cord syndrome -------------------------------------------------- 254,255
Cerebellum -------------------------------------------------------------------------- 113,117,150,160,
265-67,277
85
Cerebral hemisphere functions ------------------------------------------------
Chorea- ------------------------------------------------------------------------------
88,95,181-
183,269,276
CIDP (Chronic Inflammatory Demyelinating Radiculo-Neuropathy) - 193,208,209

Ciliospinal reflex -------------------------------------------------------------------

149
318

Clonus ------------------------------------------------------------------------------- 40,186-7,221,231,293

Cluster Headache ----------------------------------------------------------------- 3, 6,61,67-9,74,148
Coma -------------------------------------------------------------------------------- 21,94,113-18
Complex partial seizures -------------------------------------------------------- 29,32,33
Concentration ability -------------------------------------------------------------- 87,91,287
Constructional apraxia ----------------------------------------------------------- 97,250
Conus medullaris lesions-------------------------------------------------------- 36,50,260
Co-ordination---------------------------------------------------------------------- 252,256,269-278
Corneal reflex ---------------------------------------------------------------------- 153,236,290,302
Corneal-mandibular reflex ------------------------------------------------------ 239
Cortical blindness ----------------------------------------------------------------- 99
Cortical paraplegia/diplegia ----------------------------------------------------- 96,225
Cortical sensation testing ------------------------------------------------------- 247,249-51
Cortical spinal tract -------------------------------------------------------------- 36
Cranial nerve nuclei arrangement------------------------------------------ 171
Cremasteric reflex------------------------------------------------------------------ 233
Crossed/inverted reflexes-------------------------------------------------------- 232
Crossed sensory deficit ---------------------------------------------------------- 16
De-afferented pupillary phenomenon ------------------------------------- 136
Deep tendon reflexes------------------------------------------------------------- 40,43,117,186,203,208,21
6,221,231-33,255,267

Delirium ----------------------------------------------------------------------------------- 31,85,91-4,99,126

319

Dermatome sensation------------------------------------------------------------ 241-2

Digit writing-------------------------------------------------------------------------- 249
Diplopia ------------------------------------------------------------------------------ 5,17,52,141,142-
3,290

Dizziness ---------------------------------------------------------------------------- 76-7

Doll’s eye movement ------------------------------------------------------------- 143-6,163,236,302

Dorsal column- clinical findings------------------------------------------------ 226,246,253-

4,267,270,279

Dressing apraxia------------------------------------------------------------------- 97,249

Duchenne muscular dystrophy ------------------------------------------------ 178,216-7
Dyskinesia - definition ------------------------------------------------------------ 183,189
Dystonia -------------- ----------------------------------------------------------------- 53,141,182-
3,187,189,296

Emotional state and drive ------------------------------------------------------- 85,87

Encephalitis - ------------- ---------------------------------------------------------- 28,57,86,100,114,125
182,188

Epidural abscess------------------------------------------------------------------ 34,35,48,65

Epiepsia partialis continuans --------------------------------------------------- 31,32
Epilepsy aura ---------------------------------------------------------------------- 30
Epilepsy automatism ------------- ------------------------------------------------ 27,28,30,183
Epilepsy focal motor -------------------------------------------------------------- 31,96,183
Epilepsy partial complex seizures--------------------------------------------- 29
320

Epilepsy simple partial seizures ----------------------------------------------- 29,30,32

Epilepsy status grand mal---------------------------------------------------------- 30,31
Epilepsy subtle status ------------------------------------------------------------ 31
Epilepsy, classification ----------------------------------------------------------- 27,32,33
Eye muscle movements --------------------------------------------------------- 137,138
Facial nerve ------------------------------------------------------------------------- 60,154,157-9,291
Facial pain--------------------------------------------------------------------------- 64,72,74
Facioscapulohumeral muscular dystrophy ---------------------------------- 217-8
Fasciculation – --------------------------------------------------------------------- 169,177-179,
203,204,206,207,212,
Fibrillation potential - ------------------------------------------------------------- 178,208,217,219
Finger agnosia --------------------------------------------------------------------- 250,251
Frontal lobe disorders ------------------------------------------------------------ 96
Fundoscopy ------------------------------------------------------------------------- 133,270
Gait - --------------------------------------------------------------------------------- 272-280
Gerstmann syndrome ------------------------------------------------------------ 97,251
Glabellar tap test ------------------------------------------------------------------ 239
Glasgow Coma Scale ------------------------------------------------------------ 115,288
Glossopharyngeal nerve-------------------------------------------------------- 60,72-3,165-66
Grasp reflex -------------------------------------------------------------------------
97,238,295,298
321

Guillain Barré syndrome –---------------------------------------------------------- 203,208,220

Gunn-pupil phenomenon -------------------------------------------------------- 133,134,136,290
Hallucinations, illusions and delusions -------------------------------------- 30,82,85,91,94,99,189,287
Headache -------------------------------------------------------------------------- 64-74
Hearing tests ----------------------------------------------------------------------- 103,109,160,164,286
Hemiplegia -------------------------------------------------------------------------- 222-223, 224-227

Hemisensory deficit --------------------------------------------------------------- 224,256

Herniation of the brain------------------------------------------------------------ 21,44,113,114,119-21
HIV associated myelopathy----------------------------------------------------- 46
322

Hoffmann reflex -------------------------------------------------------------------- 235

Homonomous hemianopia ------------------------------------------------------ 16
Horner’s syndrome - ---------- --------------------------------------------------- 3,61,67,148,149,175,297
HTLV1 Myelopathy---------------------------------------------------------------- 48-9
Hughlings-Jackson syndrome-------------------------------------------------- 227
Ideational apraxia ----------------------------------------------------------------- 251
Ideomotor apraxia ------------------ -------------------------------------------------- 252
Immediate memory --------------------------------------------------------------- 98
Inclusion body myositis ---------------------------------------------------------- 219
Inferior homonomous quadrantanopia--------------------------------------- 249
Infranuclear ophthalmoplegia -------------------------------------------------- 138,142
Insight----------------------- --------------------------------------------------------------- 88,287
Insomnia ----------------------------------------------------------------------------- 82
Internuclear ophthalmoplegia -------------------------------------------------- 7,101,116,138,141-
2,146,147,174,226
Inverted reflexes ------------------------------------------------------------------- 40,215,232
Isaac’s syndrome------------------------------------------------------------------ 177,179
Jaw jerk reflex ---------------------------------------------------------------------- 154,170,273
Knee reflex -------------------------------------------------------------------------- 303
Language definition --------------------------------------------------------------- 86,103-109,223,287
Lateral cutaneous nerve of the thigh--------- -------------------------- Limb 211,243
Girdle muscular dystrophy----------------------------------------------- 217,218
Locked-in syndrome -------------------------------------------------------------- 226
Lower half headache ------------------------------------------------------------- 74
Lower motor neuron pathway -------------------------------------------------- 203
Medulla oblongata ---------------------------------------------------------------- 44,171,174,221,225
Memory ------------------------------------------------------------------------------ 85-87,91,95,98
Memory disorders--------------------------------------------------------------------- 100-101,287
323

Meningism -------------------------------------------------------------------------- 48,124-5

Meningitis –------------------------------------------------------------------------- 47,63,93,117,124,125-
6,146,283

Midbrain ----------------------------------------------------------------------------- 113,119,148,152,172-5

Migraine----------------------------------------------------------------------------------- 6,61,64,71-2,99,100
Millard Gubler syndrome -------------------------------------------------------- 226,227
Mononeuropathies--------------------------------------------------------------- 203,210
Monoparesis ------------------------------------------------------------------------ 220,224
Motor level (Spinal Cord) -------------------------------------------------------- 39
Motor neuron disease ------------------------------------------------------------ 169,207,274
Motor nuclei in the brain stem-------------------------------------------------- 173,206
Motor system examination ------------------------------------------------------ 177,285
MSLT (Multiple sleep latency test)-------------------------------------------- 82
Multiple sclerosis ------------------------------------------------------------------ 7,43,47,52-
56,78,129,141
324

Muscle dystrophy –--------------------------------------------------------------- 5,178,217-18

Muscle power----------------------------------------------------------------------- 191-99
Neurologic disability score ------------------------------------------------------ 194
Muscle rigidity - definition ------------------------------------------------------- 221
Muscle tone ------------------------------------------------------------------------- 28,183,184,187,203,221,
293

Muscle weakness –--------------------------------------------------------------- 6,168,178,191,192,201,

204,208,216,219,220,221,
228
Myasthenia Gravis ---------------------------------------------------------------- 7,149,150,167,203,215-
16,220,228,274

Myoclonus --------------------------------------------------------------------------- 28-9,81,180,183,268

Myokymia - definition ------------------------------------------------------------- 143,177,179

Myopathies and muscular dystrophies -------------------------------------- 203,216-17
Myotonia ----------------------------------------------------------------------------- 190,216,218,293
Myotonia dystrophica------------------------------------------------------------- 190,218
325

Narcolepsy -------------------------------------------------------------------------- 28,81,82

Neck vessel examination -------------------------------------------------------- 126
Neuromyotonia --------------------------------------------------------------------------- 177,179,216
Non epileptic seizures------------------------------------------------------------ 33
Nystagmus ------------------------------------------------------------------------------- 31,44,45,77,78,101,141,
142,147,160,161-
166,297,302
Oculocephalic reflexes ----------------------------------------------------------- 116,117,141,142,143-
46,188,236
136,137,142,143,145,148,
Oculomotor nerve -----------------------------------------------------------------
151
129
Olfactory nerve ---------------------------------------------------------------------

Optic atrophy --------------------------------------------------------------------------- 49,129,133,134,136,268

Optic nerve -------------------------------------------------------------------------- 13,107,129,130,131,134,
136,150

Optic neuritis --------------------------------------------------------------------------- 7,52,55,57,74,129,133,

134
Palmomental reflex --------------------------------------------------------------- 239
Papilloedema - --------------------------------------------------------------------- 99,116,118,133,134,193
Paraplegia---------------------------------------------------------------------------------- 96,184,225,255
Parietal lobe disorders ------------------------------------------------------------ 44,97-8
Parkinson’s disease – ----------------------------------------------------------- 5,88,95,129,179,182,187,
188-89,201,270,273,278

189
Parkinson’s syndrome------------------------------------------------------------
76
Paroxysmal vertigo --------------------------------------------------------------
29
Partial complex seizures --------------------------------------------------------
45,46,193,208,211-
Peripheral neuropathy – ---------------------------------------------------------
12,220,268,279
326

Peroneal nerve palsy ------------------------------------------------------------- 211,244

Persistent vegetative state------------------------------------------------------ 121
Petit mal ---------------------------------------------------------------------------- 27,31
Plantar reflex ----------------------------------------------------------------------- 203,234
Polymyositis ------------------- ---------------------------------------------------- 7,167,178,183,217,219,
274
Primary lateral sclerosis --------------------------------------------------------- 206,207
Primitive reflexes ------------------------------------------------------------------ 97,238,283,294-5,298

Progressive bulbar atrophy ----------------------------------------------------- 169,207,220,274

Progressive muscular atrophy ------------------------------------------------- 207
Progressive supranuclear ophthalmoplegia -------------------------------- 96,141,189
Pseudo seizures ------------------------------------------------------------------- 33
Pseudo-bulbar palsy- ------------------------------------------------------------- 154,170,226,239
Psychomotor status epilepsy--------------------------------------------------- 31
Ptosis – ------------------------------------------------------------------------------ 148,149,151,215,220,2
90,302
Pupillary light reflexes ------------------------------------------------------------ 120,136,148
Radial nerve palsy ---------------------------------------------------------------- 210,232,243
Radiculopathies-------------------------------------------------------------------- 203,213-15
63,118,125,133,292
Raised intracranial pressure----------------------------------------------------
133-35
Retinal abnormalities--------------------------------------------------------------
135,268
Retinitis pigmentosa --------------------------------------------------------------

97
Right- left disorientation-----------------------------------------------------------
165,291
Rinne test----------------------------------------------------------------------------
238,290,295,298
Rooting reflex-----------------------------------------------------------------------
210
Saturday night palsy --------------------------------------------------------------
327

Sciatica------------------------------------------------------------------------------- 50,203
Sensory deficit patterns---------------------------------------------------------- 252-258
Sensory level (Spinal Cord) ---------------------------------------------------- 41,43,46,253,255

Sensory nuclei of the brainstem ------------------------------------ 173

Simple partial seizures ----------------------------------------------------------- 29,30,32
Skew deviation --------------------------------------------------------------------- 117,141
Sleep apnoea syndrome –------------------------------------------------------- 81,82
Sleep disorders-------------------------------------------------------------------- 81
Sleep drunkenness --------------------------------------------------------------- 83
Snout reflex ------------------------------------------------------------------------- 97,170,239,273
Spasticity ---------------------------------------------------------------------------- 47,184-85,221,260,273
Spatial disorientation ------------------------------------------------------------- 95,97
Spinal cord anatomy-------------------------------------------------------------- 36
Spinal cord lesions----------------------------------------------------------------- 37-50
328
Spinal muscular atrophy --------------------------------------------------------- 178,204-5
Spino-cerebellar ataxia (SCA)------------------------------------------------ 267,269
Spinothalamic tract – examination-------------------------------------------- 245
Status epilepticus------------------------------------------------------------------ 30-31,118
Stereognosis--------------------------------------------------------------------------- 247,286,296

Sternocleidomastoid muscle testing------------------------------------------ 167,229

Strabismus ---------------- --------------------------------------------------------- 142,143,290,291
Suck reflex -------------------------------------------------------------------------- 238,295,304
Supranuclear ophthalmoplegia ------------------------------------------------ 138,141
Suspended sensory level ------------------------------------------------------- 43,254
Sympathetic tract ---------------------------------------------------------------- 174
Temporal arteritis------------------------------------------------------------------ 7,72,74
Temporal lobe disorders--------------------------------------------------------- 98
Temporo-mandibular dysfunction --------------------------------------------- 62,72,74
Tension headache ---------------------------------------------------------------- 60,62-3,127
Thalamic syndrome --------------------------------------------------------------- 257
Third nerve palsy ------------------------------------------------------------------ 72,148,151

Tics – ---------------------------------------------------------------------------------- 181

Tongue testing --------------------------------------------------------------------- 169-170
Touch localisation ----------------------------------------------------------------- 249
Transient global amnesia ------------------------------------------------------- 98,100
Transtentorial herniation--------------------------------------------------------- 21,113,116,118,119-21

Trapezius muscle testing -------------------------------------------------------- 168

329

Tremor ---------------------------------------------------------------------------- 179-

180,183,188,189,273
152-153
Trigeminal nerve ----------- ------------------------------------------------------

Trigeminal Neuralgia ------------------------------------------------------------- 6,72,73

Trigeminal Neuropathy----------------------------------------------------------- 73
Truncal ataxia ---------------------------------------------------------------------- 277
Two-point discrimination --------------------------------------------------------- 248
Ulnar nerve-------------------------------------------------------------------------- 211,243
Upper motor neuron facial palsy ---------------------------------------------- 159
Upper motor neuron weakness – -------------------------------------- 221
Vagal nerve ------------------------------------------------------------------------- 166
Vegetative state definition------------------------------------------------------- 121
Vertigo definition------------------------------------------------------------------- 76
Vertigo differentiation between central and peripheral ------------------ 77-78
Vestibular function – bedside testing----------------------------------------- 161
Vestibular system caloric testing ---------------------------------------------- 147
Vestibulo-cochlear nerve -------------------------------------------------------- 160
Visual acuity ------------------------------------------------------------------------ 129,133,290
330

Visual fields ------------------------------------------------------------------------- 130,131,248

Visual hallucinations -------------------------------------------------------------- 30,85,94,99,189
Visual-motor apraxia-------------------------------------------------------------- 99
Wallenberg syndrome ------------------------------------------------------------ 174,258
Weakness of muscles – clinical approach ---------------------------------- 201
Weber syndrome ------------------------------------------------------------------ 151,226-7
Weber test--------------------------------------------------------------------------- 165