Neurological disease

20

Epidemiology

1123 1123

History, symptoms and signs Neurological examination

Epidemiology
1126

1125

Functional neuroanatomy: an introduction Cranial nerves 1129 Brain stem lesions 1142 Motor control systems 1143 Corticospinal or pyramidal system 1143 Extrapyramidal system 1145 Cerebellum 1146 Tremor 1147 Lower motor neurone lesions 1148 Spinal reflex arc 1148 Sensory pathways and pain Lesions of the sensory system Pain 1152 1149 1150

Clinical neurology is a diverse and complex subject. The wide range of conditions seen in the UK is summarized in Table 20.1. Table 20.1
Incidence rates for commoner neurological conditions/ 100 000/year in the UK
Headaches (GP consultations) Cerebrovascular events Shingles (herpes zoster) Diabetic neuropathy Compressive neuropathies Epilepsy Parkinsons disease Post-herpetic neuralgia Primary CNS tumour Essential tremor Trigeminal neuralgia Meningitis Multiple sclerosis Severe brain injury Subarachnoid haemorrhage Subdural haematoma Metastatic CNS tumour Presenile dementia Cerebral palsy GuillainBarr syndrome Myasthenia gravis Transient global amnesia Motor neurone disease 2200 205 140 54 49 46 19 11 10 8 8 7 7 7 6 6 4 4 3 3 3 3 2

Control of the bladder and sexual function Neurological investigations Unconsciousness and coma 1154 1159

1153

Cerebrovascular disease and stroke 1163 Transient ischaemic attacks 1165 Typical stroke syndromes 1166 Cortical venous thrombosis and dural venous sinus thrombosis 1173 Epilepsy and causes of loss of consciousness Movement disorders 1182 Akineticrigid syndrome 1182 Dyskinesias 1186 Multiple sclerosis (MS) 1189 1191 1173

CNS infection and inflammation Brain tumours Hydrocephalus 1198 1201

Headache, migraine and facial pain Traumatic brain injury 1204

1201

History, symptoms and signs

The methods of recording the history and basic examination are outside the scope of this chapter. Notes should read chronologically and portray the story given by the patient, or relative. Pattern recognition interpretation of history, symptoms and examination is very reliable. Practical experience is vital. There are three critical questions:

Diseases of the spinal cord 1205 Spinal cord compression 1206 Paraplegia 1206 Degenerative neuronal diseases Congenital and inherited diseases Peripheral nerve disease 1211 Mononeuropathies 1212 Polyneuropathies 1214 Plexus and nerve root lesions 1217 Non-metastatic manifestations of malignancy Diseases of voluntary muscle 1219 1219 1208 1209

What is/are the site(s) of the lesion(s)? What is the likely pathology? Does a recognizable disease fit this pattern?

This is the essence of clinical diagnosis. The aim in this section is to mention several common problems and to relate these to core neuroanatomy.

1123

Neurological disease

Headaches
Headache is an almost universal experience, and one of the most common symptoms in general and neurological practice. It varies from an infrequent and trivial nuisance to a symptom of serious disease.

Headache of subacute onset

The onset and progression of a headache over days or weeks with or without the features of a pressure headache should always raise the suspicion of an intracranial mass lesion or serious intracranial disease. Encephalitis (see p. 1194), viral meningitis (p. 1192) and chronic meningitis (p. 1194) should also be considered.

Mechanism
Pain receptors are located at the base of the brain in arteries and veins and throughout the meninges, extracranial vessels, muscles of the scalp, neck and face, paranasal sinuses, eyes and teeth. Curiously, brain itself is almost devoid of pain receptors. Head pain is mediated by mechanical receptors (e.g. stretching of meninges) and chemical receptors (e.g. 5-hydroxytryptamine and histamine stimulation). Nerve impulses travel centrally via the fifth and ninth cranial nerves and via upper cervical sensory roots. The majority of headaches are benign, but the diagnostic issue and usual source of concern is that some are caused by serious disease. The following are some useful clinical pointers.

Headaches with scalp tenderness

Patches of exquisite tenderness overlying superficial scalp arteries are caused by giant cell arteritis (see p. 1203), which develops almost exclusively in patients aged over 50 years.

Headache following head injury

Subdural haematoma (see p. 1172) must be considered. However, the vast majority of post-trauma headaches lasting days, weeks or months are not associated with any serious intracranial pathology.

A single episode of severe headache

This common emergency is caused by one of the following:

Chronic (benign) and recurrent headaches

Almost all recurring headaches lasting hours or days band-like, generalized head pains, with a history going back for several years or months are vaguely ascribed to muscle tension, and/or migraine (see p. 1206). Depression often accompanies them. In localized pain of short duration, lasting some minutes or hours, sinusitis, glaucoma and migrainous neuralgia (p. 1203) should also be considered. Malignant hypertension, with arterial damage and brain swelling, occasionally causes headache (see p. 821). Headaches are not caused by high blood pressure alone. Eyestrain from underlying refractive error is not itself a cause of headache, though new prescription lenses sometimes provoke pain.

subarachnoid haemorrhage migraine, or other benign headaches meningitis (occasionally).

Particular attention should be paid to the suddenness of onset (suggestive of a subarachnoid haemorrhage), neck stiffness and vomiting (any meningeal irritation), or rash and fever (bacterial meningitis).

Difficulty walking and falls

A change in gait is a common presenting complaint in neurology; the main causes are given in Table 20.2. Arthritis and muscle pain also alter gait, making it stiff and slow (antalgia). Falls, especially in the elderly, are a common and important cause of morbidity. The pattern of abnormal gait is valuable diagnostically.

Pressure headaches
Intracranial mass lesions displace and stretch the meninges and basal vessels. Pain is provoked when these structures are shifted either by a mass itself or by changes in cerebrospinal fluid (CSF) pressure, e.g. coughing. Cerebral oedema around brain tumours causes further shift. These pressure headaches typically become worse on lying down. Any headache, however mild, that is present on waking and made worse by coughing, straining or sneezing may well be due to a mass lesion. Vomiting often accompanies pressure headaches. Such headaches are caused early, over weeks, by posterior fossa masses (and see hydrocephalus, p. 1201), but over a longer timescale months or even years by hemisphere tumours. A rare cause of prostrating headache with lower limb weakness is an intraventricular tumour causing intermittent hydrocephalus.

Spasticity
Spasticity (see p. 1144), particularly in extensor muscles, with or without pyramidal weakness, causes stiffness and jerkiness of walking. The toes of shoes catch level ground, and become scuffed. The pace shortens but a

Table 20.2
Common neurological patterns of difficulty walking
Spasticity/hemiparesis Parkinsons disease Cerebellar ataxia Sensory loss (joint position/loss) Distal weakness Proximal weakness Apraxia of gait

1124

History, symptoms and signs

narrow base is maintained. Clonus may be noticed as involuntary extensor rhythmic jerking of the legs. When the problem is predominantly unilateral and weakness is marked (in a hemiparesis), the weaker leg drags stiffly and is circumducted. acquired skill. Leg movement is normal when sitting or lying but initiation and organization of walking fail. This is apraxia of gait a failure of the skilled movement of walking. Shuffling small steps (marche petits pas), difficulty initiating walking (gait ignition failure) and undue hesitancy may predominate. Urinary incontinence and dementia are often present.

20

Parkinsons disease (see p. 1182)

There is muscular rigidity throughout leg extensors and flexors. While power is preserved, the gait slows. The pace shortens to a shuffle; the base remains narrow. Falls occur. A stoop becomes apparent and arm swinging is diminished. The gait becomes festinant (hurried) in small rapid steps. There is particular difficulty initiating movement and turning quickly. Retropulsion describes small backwards steps, taken involuntarily when a patient is stopped or is halted.

Falls
Falls, especially in the elderly, are a major cause of morbidity and a reason for hospital admission, for example following hip or upper limb fracture. Often no precise cause can be found. A multifactorial approach is essential and includes reviewing risk factors such as rugs, stairs, footwear and additional aids at home.

Dizziness, vertigo, blackouts and collapse

Cerebellar ataxia (see also p. 1147)
In disease of the lateral cerebellar lobes the stance becomes broad-based, unstable and tremulous. Ataxia describes this state of imperfect control. The gait tends to veer towards the side of the more affected cerebellar lobe. In disease confined to the cerebellar vermis, a midline structure, the trunk becomes unsteady without limb ataxia. There is a tendency to fall backwards or sideways truncal ataxia. Dizziness covers many complaints, from a vague feeling of unsteadiness to severe, acute vertigo. It is frequently used to describe light-headedness felt in anxiety and panic attacks, during palpitations, and in syncope or chronic ill-health. Therefore, the real nature of this symptom must be determined. Vertigo an illusion of movement is a more definite symptom. It is usually a sensation of rotation, or tipping in which the patient feels that the surroundings are spinning or moving. It is distinctly unpleasant and often accompanied by nausea or vomiting. For causes of vertigo, see page 1139. Blackout, like dizziness, is a descriptive term implying either altered consciousness, visual disturbance or falling. Epilepsy, syncope, hypoglycaemia, anaemia must be considered. However, commonly no sinister cause is found. A careful history, particularly from an eye-witness, is essential. Collapse is a vague term but often used. It is not a diagnosis and medically, its use should be avoided.

Sensory ataxia
Peripheral sensory lesions (e.g. polyneuropathy, p. 1214) cause ataxia when there is loss of perception of joint position proprioception. Broad-based, high-stepping, or stamping gait develops. This ataxia is made worse by removal of additional sensory input, such as in darkness. First described in the sensory ataxia of tabes dorsalis (p. 1196), this is the basis of the positive Rombergs test. Ask the patient to close the eyes while standing: observe whether or not they become unstable (and prevent falling).

FURTHER READING

Lower limb weakness

When weakness is distal, the leg has to be lifted over obstacles. When ankle dorsiflexors are weak, such as in a common peroneal nerve palsy (see p. 1213), the foot, having been lifted, returns to the ground with a visible and audible slap. Weakness of proximal lower limb muscles (e.g. in polymyositis or muscular dystrophy) leads to difficulty in rising from sitting or squatting. Once upright, the patient walks with a waddling gait, the pelvis being ill-supported by each lower limb as it carries the full weight of the body.

Lance JW, Goadsby PJ (1998) Mechanism and Management of Headache. Oxford: Butterworth-Heinemann. van Weel C, Vermuelen H, van den Bosch W (1995) Falls: a community perspective. Lancet 345: 15491551.

Neurological examination
A short five-part examination is shown in Practical box 20.1, and a full ten-part examination in Practical box 20.2.

Formulation Apraxia of gait

With frontal lobe disease (e.g. tumour, hydrocephalus, infarction), there is disorganization of walking as an The relevant findings are drawn together in a brief written diagnostic summary. This will form the basis for investigations, transfer of information, and management.

1125

Neurological disease

+
Gait

Practical box 20.1

Practical box 20.2

Five-part short neurological examination

1 Look at the patient
General demeanour Speech Arm swinging

Ten-part neurological examination

1 State of consciousness, arousal, appearance (e.g. coma) 2 Mental state, attitude, insight (see Box 21.5, p. 1230) 3 Cognitive function Orientation in time and place, recall of recent and distant events (memory, level of intellect, language and speech/ cerebral dominance, other disorders of skilled function, e.g. apraxia) 4 Gait and Rombergs test 5 Skull shape circumference, bruits 6 Neck stiffness, palpation and auscultation of carotid arteries 7 Cranial nerves (see Table 20.5) 8 Motor system Upper limbs: Wasting and fasciculation Posture of arms: drift, rebound, tremor Tone: spasticity or extrapyramidal rigidity Power: 05 scale (Table 20.3) Tendon reflexes: + or ++ normal; +++ increased: 0 absent with reinforcement Thorax and abdomen: Respiration Thoracic and abdominal muscles Abdominal reflexes Cremasteric reflexes Lower limbs: Wasting and fasciculation Tone, power and tendon reflexes Plantar responses 9 Coordination and fine movements 10 Sensory system

3 Examine the upper limbs

Posture of outstretched arms Wasting, fasciculation Power, tone Coordination Reflexes

2 Examine the head

Fundi Pupils Eye movements Facial movements Tongue

4 Examine the lower limbs

Power (hip flexion, ankle dorsiflexion) Tone Reflexes Plantar responses

5 Assess sensation
Ask the patient

Table 20.3
Six grades of muscle power
Grade 5 4 3 2 1 0 Definition Normal power Active movement against gravity and resistance Active movement against gravity Active movement with gravity eliminated Flicker of contraction No contraction

Functional neuroanatomy: an introduction

The neurone and synapse (Fig. 20.1)
The neurone is the functional unit of the nervous system. Its cell body and axon terminate in a synapse. The specificity, size and type of each group of neurones vary greatly. One -motor neurone within the anterior horn of the thoracic spinal cord has an axonal length over 1 metre and innervates between several hundred and 2000 muscle fibres to form a motor unit. By contrast, some spinal or intracerebral internuncial neurones have axons under 100 m long and terminate solely on one neuronal cell body.

First, ask whether feeling in the limbs, face and trunk is entirely normal Posterior columns: Vibration (using a 128 Hz tuning fork) Joint position Light touch 2-point discrimination (normal: 0.5 cm fingertips, 2 cm soles) Spinothalamic tracts: Pain: use a split orange-stick or a sterile pin Temperature: hot or cold tubes If sensation is abnormal, chart areas involved

Neurotransmitters
Synaptic transmission is mediated by neurotransmitters released by action potentials passing down an axon. Neurotransmitters then react with postsynaptic receptors and are removed by transporter proteins. The neurotransmitterreceptor reaction increases ionic permeability and propagates a further action potential. This

1126

combination of axonal electrical activity and synaptic chemical release is the basis of all neurological function. Neurotransmitters include acetylcholine, norepinephrine, (noradrenaline), epinephrine (adrenaline), 5hydroxytryptamine, gamma-aminobutyric acid (GABA), opioid peptides, prostaglandins, histamine, dopamine, glutamate, nitric oxide, neuromelanin and vasoactive intestinal peptide (VIP). Of these, glutamate is believed to be the principal excitatory neurotransmitter.

Functional neuroanatomy: an introduction

Neurone Dendrites Axon Nerve endings A Schwann cell Secretion of synaptic transmitter Action potential I Microtubules carrying neurotransmitter Action potential II

20

Site of lesion Frontal, either

Disorder Intellectual impairment Personality change Urinary incontinence Monoparesis or hemiparesis

Node of Ranvier

Frontal, left

Brocas aphasia

(ii) Ions B Ca++ Synaptic cleft G-proteins (i) Secondary messengers (protein kinases) Nucleus C Neurotransmitters

Temporo-parietal, Acalculia left Alexia Agraphia Wernickes aphasia Rightleft disorientation Homonymous field defect Temporal, right Confusional states Failure to recognize faces Homonymous field defect

DNA ATP

Parietal, either

Fig. 20.1

The functional unit: neurone and neurotransmitters. The action potential (i.e. nerve impulse) travels down the axon. Microtubules carry the neurotransmitters to the nerve endings (A). Action potential I depolarizes the synaptic membrane, opening the voltage-gated calcium channels (B). The influx of calcium causes the vesicles to fuse with the membrane (C), allowing the neurotransmitter to (i) bind to the receptor and activate the secondary messenger, which then modulates gene transcription (see Ch. 3), and also to (ii) open ligand-gated channels. This allows ions to enter, depolarizes the membrane and initiates action potential II.

Contralateral sensory loss or neglect Agraphaesthesia Homonymous field defect

Parietal, right Parietal, left

Dressing apraxia Failure to recognize faces Limb apraxia

The role of neurotransmitters and transporters in pathogenesis continues to be evaluated, but it is thought that a wide variety of acute and chronic neurological disease may be mediated, at least in part, by a final common pathway of neuronal injury involving excessive stimulation of glutamate receptors.

Occipital/ occipitoparietal

Visual field defects Visuospatial defects Disturbances of visual recognition

Clinical features of focal brain lesions: general mechanisms

Whilst it is unusual to have to recall detailed neuroanatomy, it is necessary to understand how the nervous system works and how each part is integrated. We need to be able to recognize, from symptoms and signs, the site in the nervous system which is malfunctioning; for example, the left frontal lobe, an internal capsule, or a seventh cranial (facial) nerve. Focal lesions of the cerebral cortex, and throughout the nervous system, cause symptoms and signs by two processes:

Fig. 20.2

Principal features of destructive cortical lesions (right-handed individual).

Synchronous discharge of neurones by irritative lesions (Fig. 20.3), e.g. brain lesions above the tentorium cause epilepsy, either partial or generalized.

Localization within the cerebral cortex

This subject causes considerable, and unnecessary difficulty. Work on neuronal networks, functional imaging and plasticity within the brain has tended to question traditional views relating to highly specific localization of function. However, in practical neurology at the bedside,

Suppression of function or destruction of neurones and surrounding structures (Fig. 20.2). This is the most common process part of the system fails to work.

1127

Neurological disease
Site of lesion Frontal Effects Partial seizures focal motor seizures of contralateral limbs Conjugate deviation of head and eyes away from the lesion Formed visual hallucinations Complex partial seizures Memory disturbances (e.g. dj vu) L R

Brocas aphasia (expressive aphasia, anterior aphasia)

A lesion in the left frontal lobe causes reduced fluency of speech with comprehension relatively preserved. The patient makes great efforts to initiate speech. Language is reduced to a few disjointed words and there is failure to construct sentences. Patients who recover from this form of aphasia say that they knew what they wanted to say, but could not get the words out.

Temporal

Wernickes aphasia (receptive aphasia, posterior aphasia)

Left temporo-parietal damage leaves language that is fluent but the words themselves are incorrect. This varies from insertion of a few incorrect or nonexistent words into fluent speech to a profuse outpouring of jargon (that is, rubbish with wholly nonexistent words). Severe jargon aphasia may be so bizarre as to be confused with psychotic behaviour. Patients who have recovered from Wernickes aphasia say that when aphasic they found speech, both their own and others like a wholly unintelligible foreign language. They could neither stop themselves nor understand themselves or those around them.

Parietal

Partial seizures focal sensory seizures of contralateral limbs Crude visual hallucinations (e.g. shapes in one part of the field) Visual disturbances (e.g. flashes)

Parieto-occipital

Occipital

Fig. 20.3

Effects of an irritative cortical lesion.

Nominal aphasia (anomic aphasia or amnestic aphasia)

This describes difficulty naming familiar objects. Naming difficulty is an early sign in all types of aphasia. A left posterior temporal/inferior parietal lesion causes a severe, isolated form.

it is necessary to understand the main functional roles of the cerebral cortex. The following paragraphs summarize areas of clinical importance.

The dominant hemisphere (usually left)

The concept of cerebral dominance arose with the simple observation that right-handed stroke patients with acquired language disorders had destructive lesions within the left hemisphere. Almost all right-handed and 70% of left-handed people have language function in the left hemisphere. Destructive lesions within the left fronto-temporoparietal region cause various disorders of human communication:

Global aphasia (central aphasia)

This is the combination of the expressive disturbance characteristic of Brocas aphasia and the loss of comprehension of Wernickes. The patient can neither speak nor understand language. It is due to widespread damage to the areas concerned with speech and is the most common form of aphasia after a severe left hemisphere infarct. Writing and reading are also affected.

Dysarthria
Dysarthria simply means disordered articulation slurred speech. Language is intact, cf. aphasia. Paralysis, slowing or incoordination of the muscles of articulation or local discomfort causes various different patterns of dysarthria. Examples are the gravelly speech of upper motor neurone lesions of the lower cranial nerves, the ataxic speech of cerebellar lesions, the monotone of Parkinsons disease and speech that fatigues and dies away in myasthenia. Many aphasic patients are also somewhat dysarthric.

spoken language aphasia, also called dysphasia writing agraphia reading acquired alexia.

Developmental dyslexia describes delayed and disorganized reading and writing ability in children with normal intelligence.

Aphasia
Aphasia is the loss of or defective language because of damage to the speech centres within the left hemisphere.

The non-dominant hemisphere Some varieties of aphasia

Numerous varieties of aphasia have been described. A brief review follows. Disorders in right-handed patients with right hemisphere lesions are often difficult to recognize. They comprise abnormalities of perception of internal and

1128

Cranial nerves
Table 20.4
Causes of the amnestic syndrome
Alcohol (WernickeKorsakoff syndrome) Head injury (severe) Anoxia Posterior cerebral artery occlusion (bilateral) Herpes simplex encephalitis Chronic sedative and solvent abuse Bilateral invasive tumours Arsenic poisoning Following hypoglycaemia

20

Cranial nerves

(see Table 20.5)

I: Olfactory nerve
This sensory nerve arises from olfactory (smell) receptors in the nasal mucosa. Branches pierce the cribriform plate and synapse in the olfactory bulb. The olfactory tract then passes to the olfactory cortex in the anteromedial surface of the temporal lobe. Anosmia (loss of sense of smell) is caused by head injury and tumours of the olfactory groove (e.g. meningioma, frontal glioma). It is often lost, occasionally permanently, after upper respiratory viral infections and diminished when the nose is blocked.

external space. Examples are losing the way in familiar surroundings, failing to put on clothing correctly (dressing apraxia), or failure to draw simple shapes constructional apraxia.

II: Optic nerve and visual system

The visual pathway is shown in Figure 20.4. The photic energy of light, its amount regulated by the pupillary aperture (see p. 1132), is converted to nerve action potentials by retinal rod, cone and ganglion cells. The lens, under control of the ciliary muscle (see p. 1132), causes the image on the retina to be inverted (1). An object in the lower part of the visual field is projected to the upper retina and one in the temporal field to the nasal retina. Each optic nerve (2), sheathed in piaarachnoid meninges, carries axons from retinal ganglion cells to the lateral geniculate bodies. At the optic chiasm (3), fibres travelling in the nasal portions of the optic nerves cross, join uncrossed temporal fibres of each optic nerve and form each optic tract. The fibres synapse at the lateral geniculate body (4). One optic tract thus carries fibres from the temporal ipsilateral retina and the nasal contralateral retina. Some Table 20.5
Cranial nerves
No. I II III Name Olfactory Optic Oculomotor Main clinical action Smell Vision, fields, afferent light reflex Eyelid elevation, eye elevation, ADduction, depression in ABduction, efferent (pupil) Eye intorsion, depression in ADduction Facial and corneal sensation, muscles of mastication Eye ABduction Facial movement, taste fibres Balance Hearing Sensation soft palate, taste fibres Cough, palatal and vocal cord movements Head turning, shoulder shrugging Tongue movement

Memory and its disorders (see also p. 1265)

Disorders of memory follow damage to the medial surfaces of both temporal lobes and their brainstem connections the hippocampi, fornices and mammillary bodies. Bilateral lesions are necessary to cause amnesia. It is characteristic of all organic disorders of memory that more recent events are recalled poorly, in contrast to the relative preservation of distant memories. Memory loss (the amnestic syndrome) is part of dementia (p. 1266) but also occurs as an isolated entity (Table 20.4).

FURTHER READING
Hoffman B, Lefkowitz R, Taylor P (1996) Neurotransmission: the autonomic and somatic nervous systems. In: Goodman and Gilmans The Pharmacological Basis of Therapeutics, 9th edn. New York: McGraw-Hill. Masson J, Sagn C, Hamon M, El Mestikawy S (1999) Neurotransmitter transporters in the central nervous system. Pharmacological Reviews 51: 439464.

Essential elements of neuroanatomy

For clinical purposes, and particularly in general medical practice, the extreme complexity of neuroanatomy must be reduced to its core elements. The following sections cover:

IV V VI VII VIII IX X XI XII

Trochlear Trigeminal Abducens Facial Vestibular Cochlear Glossopharyngeal Vagus Accessory Hypoglossal

cranial nerves three systems of motor control: (a) corticospinal or pyramidal system (b) extrapyramidal system (c) cerebellum the motor unit the reflex arc sensory pathways and pain control of the bladder and sexual function.

1129

Neurological disease
L 1 R 2 L R

Optic tract Eye Lateral geniculate body 3 4 1 2

Field defects are hemianopic when half the field is affected by a lesion of the optic tract, radiation or cortex (e.g. left homonymous hemianopia), and quadrantanopic when a quadrant is affected. Congruous denotes symmetry and incongruous lack of it. Bitemporal defects (damage to crossing nasal fibres) are caused by lesions of the optic chiasm (e.g. pituitary tumour).

Visual acuity
This is recorded with a Snellen chart and/or Near Vision Reading Types and corrected for refractive errors with lenses or a pinhole. The normal acuity is 6/6 to 6/9 in each eye; if less, an explanation is necessary. Visual loss is due to:

5 Optic radiation Occipital lobe 7 8 6

Ocular causes, e.g. glaucoma, macular degeneration, cataract, retinal detachment, diabetic vascular disease, trachoma, leprosy, vitamin A deficiency, trauma, onchocerciasis (river blindness). All are of major international health and economic importance. Lesions of the visual neural pathway (e.g. optic nerve lesions, chiasmal compression, tract, radiation or cortical lesions).

Fig. 20.4

Visual field defects

These are checked by confrontation with white- and redheaded pins and, if abnormal or in doubt, recorded in detail with a Goldmann (or similar) screen. Examples of field defects at different sites are shown in Figure 20.4.

The visual pathway. 1, Paracentral scotomaretinal lesion. 2, Mononuclear field lossoptic nerve lesion. 3, Bitemporal hemianopiachiasmal lesion. 4, Homonymous hemianopiaoptic tract lesion. 5, Homonymous quadrantanopiatemporal lesion. 6, Homonymous quadrantanopiaparietal lesion. 7, Homonymous hemianopiaoccipital cortex or optic radiation. 8, Homonymous hemianopiaoccipital pole lesion. Dark blue = lesion; pale blue = normal field.

Retinal and local eye lesions (site 1)

Lesions of the retina produce either scotomata (small areas of visual loss) or peripheral visual loss (tunnel vision). Common causes are diabetic retinal vascular disease, glaucoma and retinitis pigmentosa. Local lesions of the eye (e.g. cataract) can also cause visual loss.

1130

optic tract fibres reaching the lateral geniculate bodies pass to the brainstem (see p. 1132) to control refraction (lens) and pupillary aperture. From the lateral geniculate body, fibres pass in the optic radiation through the parietal and temporal lobes (5 and 6) to reach the visual, or calcarine, cortex of the occipital lobe (7 and 8). The upper retinae (lower visual fields) project in the optic radiation through the parietal lobes to the upper part of the visual cortex, and the lower retinae (upper fields) through the temporal lobes, beneath the parietal, to the lower visual cortex. Impulses reach the cortex in strictly maintained vertical topographical order (i.e. upper field to lower retina, tract, radiation and cortex, and vice versa, lower to upper). Within the visual cortex itself there are synaptic connections between groups of cells which detect lines, orientation, shapes, movement, colour, and depth. These are processed by the neighbouring visual association areas. Visual field defects caused by lesions of each optic tract, radiation and cortex are called homonymous to indicate the different (i.e. bilateral) origins of each unilateral pathway. (A homonym is the same word used to denote different things.)

Optic nerve lesions (site 2)

Unilateral visual loss, commencing as a central or paracentral (off-centre) scotoma, is the hallmark of an optic nerve lesion. A total optic nerve lesion causes unilateral blindness with loss of pupillary light reflex (direct and consensual) when the blind eye is illuminated (see afferent pupillary defect, p. 1132). For causes of optic nerve lesions, see Table 20.6. The principal pathological appearances of the visible part of the nerve (optic disc) seen on fundoscopy are:

disc swelling and hyperaemia (papilloedema) pallor (optic atrophy).

Papilloedema and optic neuritis

Papilloedema simply means swelling of the papilla the optic disc. There are many causes (Table 20.7). In all forms of disc oedema there is axonal swelling within the optic nerve, blockage of axonal transport with capillary and venous congestion. Optic neuritis is swelling of the disc and inflammation within the nerve.

Cranial nerves
Table 20.6
Principal causes of an optic nerve lesion
Optic and retrobulbar neuritis Optic nerve compression (e.g. tumour or aneurysm) Toxic optic neuropathy (e.g. tobacco, ethambutol, methyl alcohol, quinine) Syphilis Ischaemic optic neuropathy (e.g. giant cell arteritis) Hereditary optic neuropathies Severe anaemia Vitamin B12 deficiency Trauma Infective (spread of paranasal sinus infection or orbital cellulitis) Papilloedema and its causes (see Table 20.7) Bone disease affecting optic canal (e.g. Pagets)

20

Table 20.7
Causes of optic disc swelling (papilloedema)
Raised intracranial pressure Brain tumour, abscess, haematoma, intracranial haemorrhage and SAH, idiopathic intracranial hypertension, hydrocephalus, encephalitis Optic nerve disease Optic neuritis (e.g. multiple sclerosis) Hereditary optic neuropathy Ischaemic optic neuropathy (e.g. giant cell arteritis) Toxic optic neuropathy (e.g. methanol ingestion) Hypervitaminosis A Venous occlusion Cavernous sinus thrombosis Central retinal vein thrombosis/occlusion Orbital mass lesions Retinal vascular disease Malignant hypertension Vasculitis (e.g. SLE) Metabolic causes Hypercapnia, chronic hypoxia, hypocalcaemia Disc infiltration Leukaemia, sarcoidosis, optic nerve glioma

The earliest ophthalmoscopic signs of disc swelling are pinkness of the disc followed by blurring and heaping up of its margins, the nasal first. There is loss within the disc of the normal, visible, spontaneous pulsation of the retinal veins. The physiological cup becomes obliterated, the disc engorged and its vessels dilated. Small haemorrhages often surround the disc. Various conditions simulate true disc oedema. Marked hypermetropic (long-sighted) refractive errors make the disc appear pink, distant and ill-defined. Opaque (myelinated) nerve fibres at the disc margin and hyaline bodies (drusen) can be mistaken for disc swelling. Disc infiltration also causes first a prominent, then a swollen disc with raised margins (e.g. in leukaemia). When there is doubt about disc oedema, fluorescein angiography is diagnostic. Fluorescein is injected intravenously: when there is oedema, retinal leakage is seen and photographed. Early papilloedema from causes other than optic neuritis (see below) often produces few visual symptoms the underlying disease is the source of the patients complaints. However, as disc oedema progresses, enlargement of the blind spot and blurring of vision develop. The disc becomes engorged, reducing its arterial blood flow and, then as papilloedema worsens, infarction of the nerve occurs. This causes sudden severe and permanent visual loss.

SAH, subarachnoid haemorrhage: SLE, systemic lupus erythematosus

nerve neuropathy develops over the course of days or weeks. There is sometimes disc swelling and telangiectasia around the disc in the acute phase, followed by optic atrophy. Severe bilateral visual loss is usual by the age of 40. Maternal inheritance and genetic analysis have pointed to pathogenic mitochondrial DNA mutations as the cause, many with a mutation at G11778A. Exceptional cases occur in women.

Optic atrophy
Optic atrophy means disc pallor, from loss of axons, glial proliferation and decreased vascularity that follows many pathological processes, e.g. infarction of the nerve from thromboembolism or following papilloedema, inflammation (demyelinating optic neuritis in MS, syphilis, LHON), optic nerve compression, previous trauma, toxic and metabolic causes (vitamin B12 deficiency, quinine and methyl alcohol ingestion). Optic atrophy is described as consecutive or secondary when it follows papilloedema of any cause. The degree of visual loss depends upon underlying pathology.

Optic chiasm (site 3)

Bitemporal hemianopic field are the typical defects when a mass compresses the chiasm. Common causes are:

Optic neuritis
The most common cause of inflammation of the optic nerve is demyelination (e.g. multiple sclerosis). Disc swelling due to optic neuritis is distinguished from other causes of disc oedema by early and severe visual loss. Retrobulbar neuritis implies that the inflammatory process is within the optic nerve but behind the bulb (i.e. the eye), so that no abnormality is seen at the disc itself in spite of visual impairment.

pituitary neoplasm (p. 1009) meningioma craniopharyngioma secondary neoplasm.

In any case of bilateral visual loss, chiasmal compression must be considered.

Optic tract and optic radiation (sites 4, 5 and 6)

Lebers hereditary optic neuropathy (LHON)

LHON is a common cause of isolated blindness in otherwise healthy young men. Unilateral or bilateral optic

Optic tract lesions (which are rare) cause field defects which are homonymous, hemianopic and often incomplete and incongruous. Optic radiation lesions cause

1131

Neurological disease
homonymous quadrantanopic defects. Temporal lobe lesions (e.g. tumour or infarction) cause upper quadrantic defects, and parietal lobe, lower. both lateral geniculate bodies, also relay to the convergence centre. This centre receives 1a spindle afferent fibres from the extraocular muscles principally medial recti which are innervated by the third nerve. The efferent route is from the convergence centre to the EdingerWestphal nucleus, ciliary ganglion and pupils. Voluntary or reflex fixation on a near object is thus accompanied by appropriate convergence and pupillary constriction. A darkened room makes all pupillary abnormalities easier to see.

Occipital cortex (sites 7 and 8)

Homonymous hemianopic defects are produced by unilateral posterior cerebral artery infarction. The macular region of the cortex (at the occipital pole) is spared because it has a separate blood supply from the middle cerebral artery: infarction of one occipital pole alone causes a small, congruous, scotomatous, homonymous hemianopia (8). Widespread bilateral occipital lobe damage by tumour, trauma or infarction causes cortical blindness (Antons syndrome). The patient cannot see but characteristically lacks insight into the degree of visual loss and may even deny it. The pupillary responses are normal (see also p. 1167).

Clinical abnormalities of the pupils

Pupillary abnormalities in coma are discussed on page 1161, and in brainstem lesions on page 955.

Physiological changes and old age

A slight difference between the size of each pupil is common (physiological anisocoria) at any age. The pupil tends to become small (33.5 mm) and irregular in old age (senile miosis); anisocoria is more pronounced. The convergence reflex becomes sluggish with ageing and a bright light becomes necessary to demonstrate constriction. Afferent pupillary defect. A blind left eye, for example from previous complete section of its optic nerve, has a pupil larger than the right. The features of a left afferent pupillary defect are:

Pupils
Sympathetic impulses dilate the pupils. Fibres in the nasociliary nerve pass to the dilator pupillae muscle. These arise from the superior cervical ganglion at C2. Sympathetic preganglionic fibres to the eye (and face) originate in the hypothalamus, pass uncrossed through the midbrain and lateral medulla, and emerge finally from the spinal cord at T1 (close to the lung apex) and form the superior cervical ganglion at C2. Postganglionic fibres leave the ganglion to form a plexus around the carotid bifurcation. Fibres pass to the pupil in the nasociliary nerve from the part of this plexus surrounding the internal carotid artery. Those fibres to the face (sweating and piloerection) arise from the part of the plexus surrounding the external carotid artery. This arrangement has some clinical relevance in Horners syndrome (p. 1133). Parasympathetic impulses cause pupillary constriction. Fibres in the short ciliary nerves arise from the ciliary ganglion and pass to the sphincter pupillae causing constriction. Parasympathetic pathways and the light reflex mechanism are shown in Figure 20.5.

the left pupil is unreactive to light (i.e. the direct reflex is absent) the consensual reflex (constriction of the right pupil when light is shone into the left) is also absent.

The light reflex

Afferent fibres (1) in each optic nerve (some crossing in the chiasm) pass to both lateral geniculate bodies (2) and relay to the EdingerWestphal nuclei (4) via the pretectal nucleus (3). Efferent (parasympathetic) fibres from each Edinger Westphal nucleus pass via the third nerve to the ciliary ganglion (5) and thence to the pupil (6). Light constricts the pupil being illuminated (direct reflex) and, by the consensual reflex, the contralateral pupil.

Conversely, the left pupil constricts when light is shone in the intact right eye, i.e. the consensual reflex of the right eye is intact. Relative afferent pupillary defect (RAPD). When there has been incomplete damage to one afferent pupillary pathway (i.e. of one optic nerve relative to the other), the difference between the pupillary reaction, and the relative impairment on one side is called a relative afferent pupillary defect (RAPD). The sign can provide evidence of an optic nerve lesion, when, for example, retrobulbar neuritis occurred many years previously and there has been apparent complete clinical recovery of vision (p. 1190). After previous left retrobulbar neuritis:

The convergence reflex

Fixation on a near object requires convergence of the ocular axes and is accompanied by pupillary constriction. Afferent fibres in each optic nerve, which pass through

Light shone in the left eye causes both left and right pupils to constrict. When light is shone into the intact right eye, both pupils again constrict (i.e. right direct and consensual reflexes are intact). When the light source is then swung back to the left eye, its pupil dilates, relative to its previous state.

1132

The finding of a left RAPD by the swinging light test, showing that the consensual reflex is stronger than the

Cranial nerves

20

Light source

(6)

(1)

Ciliary ganglion (5)

(5)

Lateral geniculate body (2) EdingerWestphal nucleus (4) Convergence centre

Pretectal nucleus (3)

Fig. 20.5 Pupillary light reflex. Afferent pathway. (1) A retinal image generates action potentials in the optic nerve. (2) These travel via axons, some of which decussate at the chiasm and pass through the lateral geniculate bodies. (3) Synapse at each pretectal nucleus.
Efferent pathway. (4) Action potentials then pass to each EdingerWestphal nucleus of III. (5) Then, via the ciliary ganglion. (6) Leading to constriction of pupil.

direct, indicates residual damage in the afferent pupillary fibres of the left optic nerve.

Table 20.8
Causes of Horners syndrome
Hemisphere and brainstem Massive cerebral infarction Pontine glioma Lateral medullary syndrome Coning of the temporal lobe Cervical cord Syringomyelia Cord tumours T1 root Bronchial neoplasm (apical) Apical tuberculosis Cervical rib Brachial plexus trauma Sympathetic chain in neck Following thryoid/laryngeal surgery Carotid artery occlusion and dissection Neoplastic infiltration Cervical sympathectomy Miscellaneous Congenital Migrainous neuralgia (usually transient) Isolated and of unknown cause

Horners syndrome
This collection of signs of unilateral pupillary constriction with slight relative ptosis and enophthalmos indicates a lesion of the sympathetic pathway on the same side. The conjunctival vessels are slightly injected. Causes of Horners syndrome are given in Table 20.8. There is loss of sweating of the same side of the face or body; the extent depending upon the level of the lesion:

Central lesions affect sweating over the entire half of the head, arm and upper trunk. Neck lesions proximal to the superior cervical ganglion cause diminished facial sweating. Lesions distal to the superior cervical ganglion do not affect sweating at all.

1133

Neurological disease
Pharmacological tests help to indicate the level of the lesion. For example, a lesion distal to the superior cervical ganglion causes denervation hypersensitivity of the pupil, which dilates when 1:1000 adrenaline (epinephrine) is instilled. This dose has little effect on the normal pupil or a Horners pupil from a proximal lesion. In clinical practice the test is of limited value.

III, IV, VI: Oculomotor, trochlear and abducens nerves

Mechanisms controlling eye movement are:

Argyll Robertson pupil

This small, irregular (3 mm or less) pupil is fixed to light but constricts on convergence. The lesion is in the brainstem in neural tissue surrounding the aqueduct of Sylvius. The Argyll Robertson pupil is (almost) diagnostic of neurosyphilis. Similar changes are occasionally seen in diabetes mellitus.

central upper motor neurone mechanisms that drive the normal yoked parallel eye movements (conjugate gaze) individual movements generated by each oculomotor, abducens and trochlear nerve.

Conjugate gaze
Fast voluntary and reflex eye movements originate in each frontal lobe. Fibres pass in the anterior limb of the internal capsule and cross in the pons to end in the centre for lateral gaze (paramedian pontine reticular formation PPRF, Fig. 20.6a), close to each sixth nerve nucleus. The PPRF also receives fibres from:

Myotonic pupil (HolmesAdie pupil)

This is a dilated pupil seen most commonly in young women. It is usually unilateral, and the pupil is often irregular. There is no reaction (or a very slow reaction) to bright light and also incomplete constriction to convergence. The condition is due to denervation in the ciliary ganglion, of unknown cause. The myotonic pupil is of no more pathological significance than this, but is often associated with diminished or absent tendon reflexes.

the ipsilateral occipital cortex a pathway concerned with tracking objects both vestibular nuclei pathways linking eye movements with position of the head and neck (dolls head reflexes, p. 955).

Conjugate lateral eye movements are coordinated by the PPRF through the medial longitudinal fasciculus (MLF,

(a) R Frontal cortex L Frontal cortex

(b) Right eye Left eye

Nystagmus To right medial rectus To left lateral rectus III Occipital cortex Centre for lateral gaze (PPRF) Occipital cortex Lesion of right medial longitudinal fasciculus VI VI III

Brainstem

VN

VN Centre for lateral gaze (PPRF) Centre for lateral gaze (PPRF)

1134

Fig. 20.6 (a) Principal input to PPRF. Impulses from the right frontal cortex, the left occipital cortex and both vestibular nuclei (VN) drive the left centre for lateral gaze, or paramedian pontine reticular formation (PPRF). (b) Principal output from PPRF. Impulses from the PPRF pass via the ipsilateral VIth nerve nucleus to the lateral rectus muscle (ABduction) and via the medial longitudinal fasciculus to the IIIrd nerve nucleus and thus to the opposite medial rectus muscle (ADduction). X shows lesion with failure of ADduction of right eye and nystagmus of ABducting left eye.

Cranial nerves
Fig. 20.6b). Fibres from the PPRF pass both to the ipsilateral sixth nerve nucleus and, having crossed the midline, the opposite third nerve nucleus via the MLF. Each sixth nerve nucleus (supplying lateral rectus) and the opposite third nerve nucleus (supplying medial rectus and others) are thus linked by the MLF, driving the eyes laterally with parallel axes and with the same velocity.

20

Weakness of extraocular muscles (diplopia)

Diplopia (double vision) indicates weakness of one or more extraocular muscles. The causes are:

Abnormalities of conjugate lateral gaze

A destructive lesion of one side of the brain allows lateral gaze to be driven by the intact opposite pathway. For example, a destructive left frontal lobe lesion (e.g. an infarct) leads to failure of conjugate lateral gaze to the right. In an acute lesion the eyes are often deviated past the midline to the side of the lesion, here to the left, and therefore look towards the normal limbs, as there is usually a contralateral (i.e. right) hemiparesis. An irritative left frontal lobe lesion (e.g. an epileptic focus), stimulates the opposite, right, PPRF and drives lateral gaze away from the side of the lesion (i.e. to the right) during an attack. In the brainstem itself a unilateral destructive lesion involving the PPRF leads to failure of conjugate lateral gaze towards that side. There is usually a contralateral hemiparesis and lateral gaze is deviated towards the side of the paralysed limbs.

lesions of the third, fourth and/or sixth cranial nerves or nuclei disorders of the neuromuscular junction (e.g. myasthenia gravis) disease of, or injury to the ocular muscles orbital lesions.

Squint (strabismus)
This describes the appearance of the eyes when the visual axes fail to meet at the fixation point, and is either convergent or divergent. Paralytic squint. Paralytic or incomitant squint occurs when there is an acquired defect of movement of an eye the usual situation in neurological disease. There is a squint (and hence diplopia) maximal in the direction of action of the weak muscle/s. Non-paralytic squint. Non-paralytic or concomitant squint describes a squint beginning in childhood in which the angle between the visual axes does not vary when the eyes are moved the squint remains the same in all directions of gaze. Diplopia is almost never a symptom. The deviating eye (the one that does not fixate) usually has defective vision; this is called amblyopia ex anopsia. Non-paralytic squint may be latent (i.e. only visible at certain times), such as when the patient is tired. The cover test. The cover test is used principally to assess non-paralytic squint and to recognize latent squint. The patient is asked to fix on a light. A pinpoint reflection is seen in the exact centre of each pupil if there is no squint. The eye that is fixing the light centrally is covered quickly. If a squint has been present the other (uncovered) eye moves to take up central fixation. The test is repeated with the opposite eye. The dominant, fixing eye will not move when the other, squinting, amblyopic eye is covered or uncovered.

Internuclear ophthalmoplegia
This is due to damage to one MLF. Internuclear ophthalmoplegia (INO) is one of the more common complex brainstem oculomotor signs and is seen frequently in multiple sclerosis. When present bilaterally, INO is almost pathognomonic of this disease. Unilateral lesions are also caused by small brainstem infarcts. In a right INO there is a lesion of the right MLF (Fig. 20.6b). On attempted left lateral gaze the right eye fails to ADduct. The left eye develops coarse nystagmus in ABduction. The side of the lesion is on the side of impaired adduction, not on the side of the (obvious, unilateral) nystagmus.

Oculomotor (third) nerve

The nucleus of the third nerve lies ventral to the aqueduct in the midbrain. Efferent fibres to four external ocular muscles (superior, inferior and medial recti, and inferior oblique), levator palpebrae superioris and sphincter pupillae (parasympathetic) enter the orbit through the superior orbital fissure. The common causes of an oculomotor nerve lesion are given in Table 20.9. Signs of a complete third nerve palsy are:

Dolls head reflexes and skew deviation

These are of some diagnostic value in coma (see p. 1162).

Abnormalities of vertical gaze

A failure of up-gaze is caused by upper brainstem lesions, such as a supratentorial mass pressing from above, or a tumour of the brainstem (e.g. a pinealoma). When the pupillary convergence reflex fails as well, this combination is called Parinauds syndrome. Defective up-gaze also occurs in certain degenerative disorders (e.g. progressive supranuclear palsy). Some impairment of up-gaze develops as part of normal ageing.

unilateral complete ptosis the eye facing down and out a fixed and dilated pupil.

Sparing of the pupil means that parasympathetic fibres which run in a discrete bundle on the superior surface of the nerve remain undamaged, and so the pupil is of

1135

Neurological disease
Table 20.9
Common causes of an oculomotor nerve lesion
Aneursym of the posterior communicating artery Coning of the temporal lobe Infarction of IIIrd nerve In diabetes mellitus atheroma Midbrain infarction Midbrain tumour
Pontine nucleus of V VI nucleus Pons VII nucleus Medulla Genu of facial nerve V1

SOF

V2 GG FR

FO SF

V3 Facial muscles frontalis orbicularis oculi buccal muscles nasal muscles orbicularis oris platysma

normal size and reacts normally. In diabetes, infarction of the third nerve usually spares the pupil. In a third nerve palsy the eye can still ABduct (sixth nerve) and rotate inwards or intort (fourth nerve). Preservation of intortion (inward rotation) means that the fourth (trochlear) nerve is intact. In a patient with a right third nerve palsy, when the attempt is made to converge and look downwards, the conjunctival vessels of the right eye are seen to twist clockwise, indicating that the eye is intorting and the fourth nerve is intact (see below).

Cord Spinal nucleus of V

Fig. 20.7

Trochlear (fourth) nerve

This supplies the superior oblique muscle. An isolated fourth nerve lesion is a rarity. The head is tilted away from the side of the lesion. The patient complains of diplopia when attempting to look down and away from the side affected.

Sensory input of trigeminal nerve (red) and motor output of facial nerve (blue). SOF, superior orbital fissure; FO, foramen ovale; FR, foramen rotundum; GG, Gasserian ganglion; SF, stylomastoid foramen.

Abducens (sixth) nerve

The abducens nerve supplies the lateral rectus muscle which causes the eye to ABduct. In a sixth nerve lesion there is a convergent squint with diplopia maximal on looking to the side of the lesion. The eye cannot be ABducted beyond the midline. There are many causes of a sixth nerve lesion, as the nerve has a long intracranial course. The nerve can be involved within the brainstem (e.g. MS or pontine glioma). In raised intracranial pressure it is compressed against the tip of the petrous temporal bone. The nerve sheath may be infiltrated by tumours, particularly nasopharyngeal carcinoma. An isolated sixth nerve palsy due to infarction occurs in diabetes mellitus. A sixth nerve lesion is a common sequel of head trauma.

and mandibular (V3) pass to the trigeminal (Gasserian) ganglion at the apex of the petrous temporal bone, within the cavernous sinus. From here central fibres enter the brainstem. Ascending fibres transmitting the sensation of light touch enter the V nucleus in the pons. Descending fibres carry pain and temperature sensation form the spinal tract of the fifth nerve and end in the spinal V nucleus in the medulla; this extends into the upper cervical cord. Motor fibres arise in the upper pons and join the mandibular branch to supply the muscles of mastication.

Signs of a V nerve lesion

A complete fifth nerve lesion causes unilateral sensory loss on the face, tongue and buccal mucosa. When motor fibres are damaged the jaw deviates to the side of the lesion as the mouth is opened. Diminution of the corneal reflex is an early, and sometimes isolated sign of a fifth nerve lesion. Central (brainstem) lesions of the lower trigeminal nuclei (e.g. in syringobulbia, p. 1206) produce a characteristic circumoral sensory loss. When the spinal tract (or spinal nucleus) alone is involved, the sensory loss is restricted to pain and temperature sensation, i.e. dissociated (p. 1151).

Complete external ophthalmoplegia

Complete external ophthalmoplegia describes the immobile eye when III, IV and VI nerves are paralysed by lesions at the orbital apex (e.g. a metastasis) or within the cavernous sinus (e.g. sinus thrombosis).

V: Trigeminal nerve
This nerve is large, mainly sensory but with some motor fibres. Sensory fibres (Fig. 20.7; and see Figs 20.11 and 20.12) from the three divisions ophthalmic (V1), maxillary (V2)

Causes
Within the brainstem, lesions involve the fifth nuclei and central connections, e.g.:

1136

brainstem glioma multiple sclerosis

Cranial nerves

20

infarction syringobulbia.

Treatment
The anticonvulsant carbamazepine 6001200 mg daily reduces the severity of attacks in the majority of patients. Phenytoin, gabapentin and clonazepam are also used, but are less effective. If drug therapy fails, surgical procedures (radiofrequency extirpation of the ganglion, neurovascular decompression or sectioning of the sensory root) are useful in difficult cases. Alcohol injection into the trigeminal ganglion or peripheral fifth nerve branches can also be carried out.

At the cerebellopontine angle, the nerve is compressed by: acoustic neuroma meningioma secondary neoplasm.

As these lesions enlarge, the neighbouring seventh and eighth nerves become involved, producing facial weakness and deafness. At the apex of the petrous temporal bone, spreading infection from the middle ear, or a secondary tumour, damages the nerve. The combination of a V nerve lesion with pain and a sixth nerve lesion is called Gradenigos syndrome. Within the cavernous sinus, the trigeminal (Gasserian) ganglion is compressed by:

Secondary trigeminal neuralgia

Trigeminal neuralgia occurs in MS (p. 1190), with tumours of the fifth nerve (e.g. neuroma) and with lesions of the cerebellopontine angle (see below). There are usually physical signs initially a depressed corneal reflex, progressing to trigeminal sensory loss.

aneurysm of the internal carotid artery lateral extension of a pituitary neoplasm thrombosis of the cavernous sinus secondary neoplasm.

Idiopathic trigeminal neuropathy

A chronic and isolated fifth nerve lesion sometimes develops without apparent cause. When sensory loss is severe, trophic changes (facial scarring and corneal ulceration) develop.

The trigeminal ganglion becomes infected in ophthalmic herpes zoster (p. 1195), the most common lesion of the ganglion itself. Trigeminal postherpetic neuralgia commonly occurs, often affecting the first (ophthalmic) division (p. 1195). Peripheral branches of the trigeminal nerve are affected by neoplastic infiltration of the skull base.

VII: Facial nerve

The facial nerve is largely motor in function, supplying muscles of facial expression. The nerve carries sensory taste fibres from the anterior two-thirds of the tongue via the chorda tympani. It also supplies motor fibres to the stapedius muscle. The facial nerve (Fig. 20.7) arises from the seventh nerve nucleus in the pons and leaves the skull through the stylomastoid foramen. Part of each facial nucleus supplying the upper face (principally frontalis muscle) receives supranuclear fibres from each hemisphere. Therefore in a unilateral upper motor neurone lesion of the facial nerve the upper part of the face is usually not affected.

Trigeminal neuralgia
Trigeminal neuralgia (tic douloureux) is a condition of unknown cause, seen most commonly in old age. It is almost always unilateral.

Symptoms
Severe paroxysms of knife-like or electric shock-like pain, lasting seconds, occur in the distribution of the fifth nerve. The pain tends to commence in the mandibular division (V3) and spreads upwards to the maxillary (V2) and to the ophthalmic division (V1). Spasms occur many times a day. Each paroxysm is stereotyped, brought on by stimulation of a specific and often tiny trigger zone in the face. Washing, shaving, a cold wind or eating are examples of the trivial stimuli that provoke the intense pain. The face may be screwed up in agony (hence the term tic an involuntary movement). The pain characteristically does not occur at night. Spontaneous remissions last for months or years before recurrence, which is almost inevitable.

Unilateral facial weakness

Lower motor neurone (LMN) lesions. A unilateral LMN lesion causes weakness of all the muscles of facial expression on the same side. The face, especially the angle of the mouth, falls, and dribbling occurs from the corner of the mouth. There is weakness of frowning (frontalis) and of eye closure since the upper facial muscles are weak. Corneal exposure and ulceration occurs if the eye does not close during sleep. The platysma muscle is also weak. Upper motor neurone (UMN) lesions. UMN lesions cause weakness of the lower part only of the face on the side opposite the lesion. Frontalis is spared. Thus the normal furrowing of the brow is preserved, and eye

Signs
There are no signs of trigeminal nerve dysfunction. The corneal reflex is preserved. Features in the history alone make the diagnosis.

1137

Neurological disease
closure and blinking are not affected. The earliest sign is simply slowing of one side of the face, for example on baring the teeth. In UMN lesions, too, there is sometimes relative preservation of spontaneous emotional movement (e.g. smiling) compared with voluntary movement. the parotid to supply muscles of facial expression are damaged here by parotid gland tumours, mumps (p. 59), sarcoidosis (p. 897) and trauma. Each nerve is also affected in polyneuritis (e.g. GuillainBarr syndrome, p. 1214), usually simultaneously. Weakness of the facial muscles themselves is also seen in primary muscle disease and neuromuscular junction disorders. Weakness is usually symmetrical. Causes include:

Causes of facial weakness

A common cause of facial weakness is a supranuclear (UMN) lesion (e.g. cerebral infarction), leading to UMN facial weakness and hemiparesis. Lesions at four lower levels are recognized by the association of LMN facial weakness with other signs. Pons. Here the sixth (abducens) nerve nucleus is encircled by seventh nerve fibres (Fig. 20.7). The sixth nucleus is thus often involved in pontine lesions of the seventh, causing a convergent squint (lateral rectus palsy) with unilateral facial weakness. When the neighbouring PPRF and corticospinal tract are involved, there is the triple combination of:

dystrophia myotonica (p. 1224) facioscapulohumeral dystrophy (p. 1223) myasthenia gravis (p. 1222).

Bells palsy
This common, acute, isolated facial nerve palsy is probably due to a viral (often herpes simplex) infection that causes swelling of the nerve within the petrous temporal bone and as it traverses the stylomastoid foramen in the skull base. The patient notices marked unilateral facial weakness, sometimes with loss of taste on the anterior two-thirds of the tongue. Pain behind the ear is common at onset. Diagnosis is made on clinical grounds. No other cranial nerves are involved.

LMN facial weakness failure of conjugate lateral gaze (towards the lesion) contralateral hemiparesis.

Causes include pontine tumours (e.g. glioma), demyelination and vascular lesions. The facial nucleus itself is affected unilaterally or bilaterally in poliomyelitis (see p. 52) and in motor neurone disease (p. 1208) the latter usually bilaterally. Cerebellopontine angle (CPA). The fifth, sixth and eighth nerves are affected with the seventh where they are clustered together in the CPA. Causes are acoustic neuroma, meningioma and secondary neoplasm. Petrous temporal bone. The geniculate ganglion (sensory for taste) lies at the genu (knee) of the facial nerve (Fig. 20.7). Fibres join the facial nerve in the chorda tympani and carry taste from the anterior two-thirds of the tongue. The (motor) nerve to stapedius muscle leaves the facial nerve distal to the genu. Facial nerve lesions within the petrous temporal bone cause the combination of:

Management and course

Spontaneous improvement of Bells palsy usually begins during the second week. Thereafter, recovery continues but this may take 12 months to become complete. Occasionally (fewer than 10%) patients are left with a severe, unsightly, residual weakness. Electrophysiological tests (EMG, p. 1156) are of some help in predicting outcome but rarely used in practice. After the third week, absence of an evoked potential from facial muscles (the nerve is stimulated over the parotid) indicates that recovery is unlikely. Steroids (e.g. prednisolone 60 mg daily, reducing to nil over 10 days) reduce the proportion of patients left with a severe deficit, provided they are given early. Aciclovir is of unproven value but sometimes given. Suturing of the upper to lower lid (tarsorrhaphy) is essential to prevent prolonged corneal exposure if the eye cannot be closed. Adhesive tape to hold the eye closed is an invaluable temporary protective measure. If there is severe late residual paralysis, cosmetic surgery is sometimes helpful. Bells palsy occasionally recurs and is very rarely bilateral.

loss of taste on the anterior two-thirds of the tongue hyperacusis (unpleasantly loud noise distortion) caused by paralysis of stapedius.

Causes include:

Bells palsy trauma middle ear infection herpes zoster (Ramsay Hunt syndrome, p. 1138) tumours (e.g. glomus tumour).

Ramsay Hunt syndrome

This is herpes zoster (shingles) of the geniculate ganglion. There is a facial palsy (identical in appearance to Bells palsy) with herpetic vesicles in the external auditory meatus (because this receives a sensory twig from the facial nerve) and sometimes on the soft palate. Deafness, or a fifth nerve lesion may occur. Complete

1138

Skull base, parotid gland and in the face itself. Swelling of the facial nerve within the stylomastoid foramen develops in Bells palsy (see below). The nerve can be damaged by skull base tumours and in Pagets disease of bone. Branches of the facial nerve that pierce

Cranial nerves
recovery is less likely than in Bells palsy. Treatment for shingles should be given (aciclovir, p. 1277).

20

Hemifacial spasm
This is an irregular, painless clonic spasm of the facial muscles, usually occurring in middle or old age, and more commonly in women. It varies in severity from a mild inconvenience to a severe and disfiguring condition when it affects all the facial musculature of one side. The causes are:

The vibrating tuning fork is placed adjacent to the external auditory meatus. In sensorineural deafness, perception improves when the base of the vibrating tuning fork is placed on the mastoid process: sound is conducted directly to the ossicles through bone.

Investigations of cochlear lesions

idiopathic pressure from vessels in the cerebellopontine angle following Bells palsy acoustic neuroma Pagets disease in the skull base.

Pure tone audiometry. Measurement of auditory evoked potentials. These record from scalp electrodes the response from a repetitive click auditory stimulus. The level of the lesion may be detected by abnormalities in the response.

Causes of sensorineural deafness are shown in Table 20.10.

There are clonic spasms of the facial muscles on one side. A mild LMN facial weakness is common.

Vestibular nerve
Nerve impulses generated by movement of the sensory epithelia of the three semicircular canals, saccule and utricle pass to vestibular nuclei in the pons. The vestibular nuclei are also connected to the cerebellum, nuclei of the ocular muscles, PPRF, extrapyramidal system, reticular formation, temporal lobes and spinal cord. The maintenance of balance and posture also depends upon the interaction of proprioceptive (joint position sense) impulses passing between the neck, spinal muscles and limbs and the vestibular system. The main symptom of a vestibular lesion is vertigo and loss of balance. Vomiting frequently accompanies acute vertigo of any cause. Nystagmus is the principal physical sign.

Management
Mild cases require no treatment. In severe cases, various decompressive procedures on the facial nerve in the cerebellopontine angle are sometimes helpful. Local injection of botulinum toxin into facial muscles reduces the spasm for some months, and can be repeated. Drugs are of no value.

Myokymia
Facial myokymia describes a rare, continuous, fine, sinuous or wave-like movement of the lower face that is seen in brainstem lesions (e.g. multiple sclerosis, brainstem glioma). The term myokymia is also used to describe the innocent twitching around the eye that commonly occurs in fatigue.

Vertigo
Vertigo, the definite illusion of movement of the subject or surroundings, indicates a disturbance of vestibular, eighth nerve, brainstem or, very rarely, cortical function. The principal causes are given in Table 20.11. Deafness and tinnitus accompanying vertigo indicate that its origin is from the ear or eighth cranial nerve.

VIII: Vestibulocochlear nerve

This nerve has two parts cochlear and vestibular.

Cochlear nerve
Auditory fibres from the spiral organ of Corti within the cochlea pass to the cochlear nuclei in the pons. Fibres from these nuclei cross the midline and pass upwards through the medial lemnisci to the medial geniculate bodies and thence to the temporal gyri. The symptoms of a cochlear nerve lesion are deafness and tinnitus. The deafness is called sensorineural (or perceptive) deafness. Clinical detection is by tuning fork (256 Hz, not 128 Hz) tests, principally Rinnes test, which distinguishes conductive from sensorineural deafness:

Table 20.10
Causes of sensorineural deafness
End organ Advancing age Occupational acoustic trauma Mnires disease Drugs (e.g. gentamicin, neomycin) Acoustic neuroma Cranial trauma Inflammatory lesions: Tuberculous meningitis Sarcoidosis Neurosyphilis Carcinomatous meningitis Multiple sclerosis Infarction

Eighth nerve lesions

The contralateral ear is masked (with the examiners forefinger).

Brainstem lesions (rare)

1139

Neurological disease
Table 20.11
Principal causes of vertigo
Mnires disease Drugs (e.g. gentamicin, anticonvulsant intoxication) Toxins (e.g. ethyl alcohol) Vestibular neuronitis Multiple sclerosis Migraine Acute cerebellar lesions Cerebellopontine angle lesions (e.g. acoustic neuroma) Partial seizures (temporal lobe focus) Brainstem ischaemia or infarction Benign paroxysmal positional vertigo

is almost always binocular, horizontal and present in all directions of gaze. Its causes are almost invariably ocular, when there is poor visual fixation (e.g. longstanding, severe visual impairment) or as a congenital lesion, when it is sometimes associated with headnodding. Exceptionally, it occurs in brainstem disease: a fine pendular, jelly-like nystagmus is a sign of a multiple sclerosis (MS) brainstem plaque, or brainstem glioma.

Investigation of vestibular lesions

Magnetic resonance provides the best structural images of this region. Caloric tests are used to assess function of the labyrinth. These record the duration of evoked nystagmus when first ice-cold, then warm, water is run into the external meatus. In the normal caloric test:

Nystagmus
Nystagmus is a rhythmic oscillation of the eyes. It is a sign of disease of either the ocular or vestibular system and its connections. Nystagmus is classified on its appearance as either jerk or pendular. For true nystagmus to be present it must be demonstrable within binocular gaze and be sustained.

ice-cold water in the left ear causes nystagmus with the fast movement to the right warm water in the left ear causes nystagmus with the fast movement to the left.

Jerk nystagmus
Jerk nystagmus (the usual nystagmus of neurological disease) has a fast and a slow component. It is seen in vestibular end-organ, eighth nerve, brainstem, cerebellar and (very rarely) cortical lesions. The direction of nystagmus is decided by the fast component, which can be thought of as a reflex attempt to correct the slower, primary movement. Considerable difficulties exist when attempts are made to use the direction alone of jerk nystagmus as a localizing sign, although nystagmus is both a common and valuable indication of abnormality within the vestibular system as a whole. The following are useful diagnostic starting points:

The right ear gives opposite responses. Decreased or absent nystagmus indicates ipsilateral labyrinth, eighth nerve or brainstem involvement. The technique is also used in the diagnosis of brainstem death (p. 955). There are, however, difficulties relating minor abnormalities in caloric tests to clinical symptoms of dizziness.

Vestibular and auditory lesions central, VIIIth nerve and end organ
Drugs (e.g. anticonvulsant toxicity), alcohol and brainstem vascular disease are common central causes of vertigo. It may also be possible to recognize lesions from clinical features at six distinct levels. Cerebral cortex. Vertigo is occasionally part of the aura of a partial (temporal lobe) seizure. Vertigo is also a psychological and perceptual sensation when experiencing unaccustomed heights, or in panic attacks. Deafness is very rare in acquired cortical disease; bilateral lesions are necessary. Pons and brainstem. Vertigo is also common when lesions involve the vestibular nuclei and their connections (demyelination, vascular, tumour, syrinx). A sixth or seventh nerve lesion, internuclear ophthalmoplegia, lower cranial nerve lesions or contralateral hemiparesis will help localization. Nystagmus is frequently present, while deafness is rare. Transient vertigo occurs in basilar migraine (p. 1202), in syncope, and occasionally in hypoglycaemic attacks; its site of origin is often difficult to ascertain. Cerebellum. Nystagmus, towards the side of a cerebellar mass (e.g. tumour, haemorrhage or infarct) develops. Limb ataxia is usually present. Bilateral cerebellar, or cerebellar connexion disease (e.g. olivo-pontocerebellar degeneration) causes bilateral nystagmus. Deafness does not occur.

Horizontal jerk or rotary jerk nystagmus may be either of peripheral origin (middle ear) or central origin (eighth nerve, brainstem, cerebellum and their connections). In peripheral lesions, nystagmus is usually acute and transient (minutes or hours) and associated with severe prostrating vertigo; in central lesions it is long-lasting (weeks, months or more). Vertigo caused by central lesions tends to wane after days or weeks, the nystagmus outlasting it. Vertical jerk nystagmus. This is caused only by central lesions. Down-beat jerk nystagmus. This rarity is caused by lesions around the foramen magnum (e.g. meningioma, cerebellar ectopia.

Pendular nystagmus

1140

Pendular describes movements to and fro that are similar in both velocity and amplitude. Pendular nystagmus

Cranial nerves
Cerebellopontine angle. Sensorineural deafness and vertigo occur. Sixth, seventh and fifth nerve lesions develop, followed by cerebellar signs (ipsilateral) and later pyramidal signs (contralateral). Nystagmus is often present. Causes include acoustic neuroma (p. 1199), meningioma and secondary neoplasm, carcinomatous meningitis and inflammatory lesions (Table 20.11). Petrous temporal bone. Facial weakness (seventh nerve) often accompanies the eighth nerve lesion. Causes include trauma, middle ear infection, secondary neoplasm and Pagets disease of bone (see also Gradenigos syndrome, p. 1137). End organs (cochlear and semicircular canals). Causes include: The disturbance lasts for several days or weeks but is self-limiting and rarely recurs. Treatment is with vestibular sedatives. The condition is sometimes followed by benign positional vertigo. Very similar symptoms can be caused by demyelination or vascular lesions within the brainstem, but usually other abnormalities are almost invariably apparent (see above).

20

Benign paroxysmal positional vertigo (BPPV)

Positional vertigo is vertigo precipitated by head movements, usually into a particular position. It may occur when turning in bed or on sitting up. The onset is typically sudden and distressing. Vertigo is transient, lasting seconds or minutes. The phenomenon fatigues (becomes less severe on repeated movement). Vertigo can be produced by moving the patients head suddenly (Hallpikes test). There is a latent interval of a few seconds, followed by nystagmus, which fatigues on repeating the test several times, though repetition is unpleasant for the patient. Whilst no serious underlying cause is found for BPPV, it is a distressing syndrome that sometimes follows vestibular neuronitis (see above), head injury or ear infection. It usually lasts for some months. There are no sequelae, although it sometimes recurs. Positional nystagmus (and vertigo) that is immediately apparent on movement (i.e. there is no latent interval) and that persists (it does not fatigue) is occasionally seen with cerebellar mass lesions. Vertigo is treated symptomatically with vestibular sedative drugs. The evidence base for these compounds is limited. Vestibular exercises (Cawthorne Cooksey exercises) are also recommended. In these, the head and neck are rotated to provoke, and hence fatigue, the unpleasant illusion of movement.

Mnires disease drugs (e.g. gentamicin) noise (acoustic trauma, p. 997) middle ear infection mumps vestibular neuronitis benign paroxysmal positional vertigo advancing age intrauterine rubella congenital syphilis.

Mnires disease
This condition is characterized by recurrent attacks of the three symptoms vertigo, tinnitus and deafness. It is associated with a dilatation of the endolymph system of unknown cause.

Symptoms
Sudden, unprovoked attacks of vertigo with vomiting and loss of balance last from minutes to hours. Tinnitus and deafness accompany an attack but may be overshadowed by the degree of vertigo. Attacks are recurrent over months or years. Ultimately deafness develops and vertigo ceases.

Signs
Nystagmus often accompanies an attack. Sensorineural deafness is present.

Lower cranial nerves IX, X, XI, XII

The glossopharyngeal (IX), vagus (X) and accessory (XI) nerves arise in the medulla and leave the skull base together through the jugular foramen. The hypoglossal (XII) also arises in the medulla but leaves the skull through the anterior condylar foramen. All four lower cranial nerves lie close together, just outside the skull, and are related to the carotid artery and ascending sympathetic innervation to the eye.

Management
Medical treatment consists of rest and vestibular sedatives (e.g. cinnarizine, betahistine, prochlorperazine), but is unsatisfactory. Each attack is, however, self-limiting. Recurrent severe attacks may require surgery, such as surgical endolymph drainage, ultrasound destruction of the labyrinth or vestibular nerve section.

Glossopharyngeal (IX)
This mixed nerve is largely sensory. Sensory fibres supply all sensation to the tonsillar fossa and pharynx (the afferent pathway of the gag reflex), and taste to the posterior third of the tongue. Motor fibres supply the stylopharyngeus muscle, autonomic fibres supply the parotid gland, and a sensory branch supplies the carotid sinus.

Vestibular neuronitis
This common but poorly understood syndrome describes an acute attack of isolated severe vertigo with nystagmus, often with vomiting, but without loss of hearing. It is believed to follow or accompany viral infections that affect the labyrinth or vestibular nerve.

1141

Neurological disease
Vagus (X)
This mixed nerve, largely motor, supplies the striated muscle of the pharynx (efferent pathway of the gag reflex), larynx (including the vocal cords via the recurrent laryngeal nerves) and upper oesophagus. There are sensory fibres from the larynx. Parasympathetic fibres supply the heart and abdominal viscera. Recurrent laryngeal nerve lesions. Paralysis of this branch of each vagus causes hoarseness (dysphonia) and failure of the forceful, explosive part of voluntary and reflex coughing. There is no visible weakness of the palate but vocal cord paralysis is seen endoscopically. Bilateral acute lesions (e.g. postoperatively) are a serious emergency and cause respiratory obstruction. The left recurrent laryngeal nerve (which loops beneath the aorta) is more commonly damaged than the right. Causes of recurrent laryngeal nerve lesions include:

Accessory (XI)
This motor nerve supplies the trapezius stomamastoid muscles.

Hypoglossal (XII)
This motor nerve supplies the tongue.

Ninth and tenth nerve lesions

Isolated nerve lesions are most unusual, since disease at the jugular foramen affects both nerves and sometimes the accessory nerve. A unilateral ninth nerve lesion causes diminished sensation on the same side of the pharynx. A tenth nerve palsy produces ipsilateral failure of voluntary and reflex elevation of the soft palate, which is drawn over to the opposite side. Bilateral combined lesions of the ninth and tenth nerves cause visible weakness of elevation of the palate, depression of palatal sensation and loss of the gag reflex. The vagal recurrent laryngeal branches are involved. The cough is depressed and the vocal cords paralysed. The patient complains of difficulty in swallowing, hoarseness, nasal regurgitation and choking (particularly with fluids) a dangerous situation. Bulbar palsy is a general term describing palatal, pharyngeal and tongue weakness of LMN type (p. 1142). Ninth and tenth nerve lesions often accompany eleventh and twelfth nerve lesions. Causes are given in Table 20.12.

mediastinal primary tumours (e.g. thymoma) secondary spread from carcinoma of the bronchus aneurysm of the aorta trauma or surgery to the neck glossopharyngeal neuralgia (rare).

Glossopharyngeal neuralgia. This describes intensely painful, paroxysmal neuralgic spasms of the pharynx triggered repeatedly by swallowing. There are no physical signs. Treatment of this rare condition is with carbamazepine (see trigeminal neuralgia, p. 1137) or section of the nerve in the pharynx. Table 20.12 shows the principal causes of lesions.

Eleventh nerve lesions

A lesion of the eleventh nerve causes weakness of sternomastoid (rotation of the head and neck to the opposite side) and trapezius (shoulder shrugging). Section of the nerve is followed by persistent neuralgic neck pain and is often accompanied by other lower cranial nerve lesions (see Table 20.12).

Twelfth nerve lesions

LMN lesions of the twelfth nerve lead to unilateral tongue weakness, wasting and fasciculation. When protruded the tongue deviates towards the weaker side. For the principal causes, see Table 20.12. Bilateral supranuclear (UMN) twelfth nerve lesions (see below) produce slow, limited tongue movements; the tongue is stiff and cannot be protruded far. Fasciculation is absent.

Table 20.12
Principal causes of ninth, tenth, eleventh and twelfth nerve lesions
Within the brainstem Infarction Syringobulbia Motor neurone disease (motor fibres) Poliomyelitis (motor fibres) At the skull base (jugular and anterior condylar foramina) Carcinoma of nasopharynx Glomus tumour Neurofibroma Jugular venous thrombosis (XIIth is spared) Trauma Within the neck and nasopharynx Carcinoma of nasopharynx Metastases Polyneuropathy Trauma

Brain stem lesions

Bulbar palsy
Bulbar palsy describes weakness of LMN type of muscles whose cranial nerve nuclei lie in the medulla (the bulb). Paralysis of bulbar muscles is caused by disease of lower cranial nerve nuclei (e.g. motor neurone disease), lesions of ninth to twelfth cranial nerves (Table 20.12), malfunction of their neuromuscular junctions (e.g. myasthenia gravis, botulism), or disease of muscles themselves (e.g. muscular dystrophies).

1142