Ebook Textbook of clinical neurology: Part 2

195

Disorders of the cranial nerves
Abstract
Diseases of the cranial nerves result in sensory function loss and/or the loss of facial
motor control. Hearing and sight may be lost, for example, double vision or vertigo may
occur, swallowing and speech may become difficult, and the face may become paralysed
or hypersensitive. Cranial nerve problems can usually be traced and resolved. That is the
case, for example, with viral infections, compression due to intracranial hypertension,
drug poisoning, autoimmune disorders and meningitis.

16.1General causes – 196
16.2Clinical presentation – 196
16.2.1Olfactory nerve (I) – 196
16.2.2Optic nerve (II) – 197
16.2.3Oculomotor nerve (III) – 198
16.2.4Trochlear nerve (IV) – 200
16.2.5Trigeminal nerve (V) – 200
16.2.6Abducens nerve (VI) – 200
16.2.7Facial nerve (VII) – 200
16.2.8Vestibulocochlear nerve (VIII) – 202
16.2.9Glossopharyngeal nerve (IX) and vagus nerve (X) – 204
16.2.10Accessory nerve (XI) and hypoglossal nerve (XII) – 205

16.3Failure of multiple cranial nerves – 205

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Chapter 16 · Disorders of the cranial nerves

Case 16.1
A sixty-year-old man has sometimes been seeing double
for the last three months. When it happens, the images are
displaced diagonally. The double vision is present when
the man wakes up in the morning, but after an hour or so
it disappears, and for the rest of the morning he is fine.
However, the double vision usually returns in the course
of the afternoon, especially if the man drives or has been
reading a lot. For the last week, the man’s right upper eyelid
has been drooping, particularly in the evening. However,
when the eyelid droops, the man no longer has double
vision. He has no other symptoms.
During a morning examination, no abnormalities are initially
observed. However, when the man directs his gaze upward
for about fifteen seconds, the double vision starts and his
left eye is observed to be lower than his right. When he
directs his gaze to the right, he experiences uncrossed
double vision and he cannot keep his right eye directed to
the side; it gradually drifts back towards his nose. The upper
eyelid also droops to partially cover the pupil.
Question 1: Which muscles are affected?
Question 2: What needs to be tested in order to establish

whether the patient has an eye muscle disorder or a cranial
nerve disorder?
Question 3: What is the most likely diagnosis?
Online: 7Answers to case study questions 16.1

Case 16.2

16

A fifty-five-year-old woman has experienced five acute
vertigo attacks in the last year, each lasting several hours.
During the attacks, she has the sense that everything
around her is spinning. Sometimes she is also nauseous;
indeed, during the last attack, she had to vomit. According
to her husband, her eye movements were jerky. Prior to the
attacks, she has often had the sensation that her right ear
was blocked. The attacks are also accompanied by tinnitus.
Onset appears unrelated to the woman’s posture or the
time of day, and there is no apparent trigger. Between
attacks, the woman’s balance is good, but her hearing on
the right remains affected.
When examined twenty-four hours after an attack, the
hearing in her right ear is found to be impaired. The
result of Rinne’s test is abnormal and Weber’s test reveals
lateralization to the left.
Question 1: What disorder does the patient probably have?
Question 2: How should the results of the tuning fork tests be
interpreted?
Question 3: What did the patient’s jerky eye movements
probably entail?

Online: 7Answers to case study questions 16.2

16.1

General causes

The twelve cranial nerves share the characteristic of running
over the base of the skull to and from the associated sense
organs or effectors. With the exception of the first two, they all
originate in the brainstem (7sect. 6.2).
Their location makes them vulnerable to traumatic injury
(especially nerves I, II, IV and VII) and to intracranial hypertension, whether or not accompanied by compression (nerves
III and VI). Furthermore, all of them can be affected by basal
meningitis (bacterial, granulomatous in sarcoidosis) and by
leptomeningeal metastases.
Local compression can be caused by cranial base processes
(chondroma, metastases) and nasopharyngeal tumours, meningiomas (mainly nerves II, V, VI, VII and X) and aneurysms
(nerve III, sometimes nerve II). The tumours that originate in
cranial nerves are olfactory meningioma (nerve I), optic glioma
(nerve II) and neurinoma (mainly in nerve VIII, but also nerve
V and occasionally nerves VII, X and XII).
Almost all cranial nerves (except XI and XII) can be
affected by herpes virus infection. Some cranial nerves are
involved in MS (nerves II, V, VI, sometimes nerve VII, occasionally nerves III, X and XII). Diabetes mellitus can affect
nerves II, III and VI and sometimes nerves VII and X. Collagen
disease and vasculitis can affect nerves II, III, V, VI and sometimes nerve VII. However, it is by no means always possible to
identify a cause for the impairment of cranial nerve function.
The clinical examination undertaken in cases of cranial
nerve disorder is described in 7sect. 6.3. This chapter discusses
the clinical presentations of cranial nerve disorders.

16.2

Clinical presentation

16.2.1 Olfactory nerve (I)

The connections between the receptors in the olfactory mucous
membrane and the olfactory bulb (7sect. 6.2) are vulnerable.
Consequently, the sense of smell may remain impaired after a
relatively minor traumatic brain injury, e.g. contrecoup phenomenon or fall-related impact injury to the occipital region
(7sects. 20.3.2 and 20.6.5). Loss of smell is a common resi­
dual symptom of ‘influenza’. Finally, loss of smell is sometimes
attributable to a frontal process, particularly olfactory meningioma. Olfactory sensations can be triggered by partial epilepsy
(uncinate fits; 7sect. 18.2.1), but may also originate from the
nasal sinuses.


197
16.2 · Clinical presentation

Loss of smell
Olfactory nerve dysfunction
5 traumatic brain injury
5 tumour in anterior cranial fossa (olfactory meningioma)
5sarcoidosis
5 status after meningitis
5 Parkinson’s disease
5 following ‘influenza’ or other viral infection
5 congenital defect (atrophy of the olfactory bulb)
Other causes

5 acute or chronic nasal infection, heavy smoking
5familial
5 unknown cause

16.2.2 Optic nerve (II)

Impaired visual acuity of one eye often requires ophthalmological investigation, but sometimes a neurologist is involved on
a secondary basis. A partial visual field disorder of both eyes
is nearly always a matter for a neurologist. The anatomy of the
visual system and the associated examination and testing procedures are described in 7 chap. 9.
In cases of neuropathy of the optic nerve, distinction is made
between disorders characterized by observable abnormalities of
the papilla and other disorders. Disorders that do not involve
papillary abnormalities are referred to as posterior or retrobulbar disorders, or as retrobulbar neuritis; where such abnormalities are present, the disorder is known as anterior optic neuritis.
About 3 % of the population has a ‘lazy eye’ (amblyopia)
with vision of between 1/10 and 1/60, without experiencing a
specific visual field disorder and without any other ophthalmological or neurological abnormalities being detectable.
Causes of vision impairment
Vascular
5 ischemia of the retina
5 ischemia of the optic nerve
Infection of the optic nerve
5 demyelinating optic neuritis (e.g. in MS)
5 viral infection (children)
Optic neuropathy
5toxic
5metabolic
5 hereditary (mitochondrial, SCA, 7sect. 25.3)
Mechanical injury to optic nerve
5 infiltration in basal meningitis

5 infiltration in leptomeningeal metastasis
5compression
5trauma

Primary eye disorders
5 retinopathy, macular degeneration
5 glaucoma and other primarily ophthalmological causes
Other
5amblyopia

Vascular causes of impaired vision
Distinction may be made between ischemia of the retina
(amaurosis fugax, retinal infarct) and ischemia of the optic
nerve papilla (anterior ischemic optic neuropathy, (AION) or
posterior ischemic optic neuropathy (PION)). Ischemic optic
neuropathy involves acute painless impairment to the vision
of one eye, often followed by further deterioration in the following hours or days. If abnormalities are visible in fundo, the
disorder is referred to as AION, and otherwise as PION. Consideration should always be given to temporal arteritis, but the
cause may also be generalized vascular pathology. Such optic
ischemia is attributable to local occlusion of minor blood vessels and not to a thromboembolic disorder; hence, neither
examination of the heart nor examination of the blood vessels
of the neck is indicated.
It is a different matter in cases of ischemia of the retina.
The retina is supplied with blood by the central retinal artery,
which branches off the ophthalmic artery, itself a branch of the
internal carotid artery (.fig. 11.3). In a case of ischemia of the
retina, whether temporary (amaurosis fugax) or permanent
(retinal infarct), examination and testing should be the same as
in a case of cerebral TIA or infarction (7 chap. 17).
Migraine can sometimes cause temporary visual field

impairment affecting one eye. However, if migraine is the
cause, the impairment is not sudden, but progresses gradually, often starting peripherally and migrating inward, and
the impairment soon disappears (retinal migraine). The latter
disorder mainly affects young adults. It is not clear whether
migraine-related visual field impairment is a vascular pheno­
menon (7sect. 21.1.3).

Neuritis of the optic nerve
Optic neuritis can occur in demyelinating disorders. Usually,
no abnormalities of the optic nerve are observable in the acute
stage and the condition is referred to as retrobulbar neuritis;
sometimes, however, oedema of the optic nerve papilla is immediately visible. Within hours or a few days, an impairment will
develop at the centre of the field of vision of one eye (central
scotoma), resulting in visual acuity declining to between 1/10
and 1/60. The peripheries of the fields of vision remain largely
unaffected. The eye will also be painful, particularly when
moved or subject to external pressure. The impaired vision is
often accompanied by impaired colour perception. Examination
should reveal an afferent pupil defect (7sect. 6.3.2).
The prognosis is generally good; a gradual improvement
starts after a week or so, and most patients regain normal
vision after three to six weeks. Relapse can occur, however,
and the same disorder may develop in the other eye. Generally

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Chapter 16 · Disorders of the cranial nerves

speaking, more than 50 % of patients with optical neuritis subsequently develop MS. The extent to which one should anticipate that possibility by additionally testing for MS and the
circumstances under which one should treat optic neuritis
resulting from demyelination are matters of debate.

Neuropathy of the optic nerve
Toxic neuropathy of the optic nerve can be caused by methanol,
excessive nicotine, drugs such as chloroquine, quinine, streptomycin, chloramphenicol, lead, arsenic and certain insecticides.
A deficiency of vitamin B12 or vitamin B1 can lead to vision
impairment.

Mitochondrial disorders
Hereditary mitochondrial optic neuropathy (Leber’s hereditary
optic neuropathy) usually develops gradually over the course of
weeks or months and is characterized by central vision impairment affecting both eyes. Sometimes, however, it starts with acute
retrobulbar neuritis. The disorder occurs mainly in men aged
between eighteen and thirty-five, but it can also become manifest later in life and is occasionally found in women. It is associated with various mutations in the mitochondrial DNA. However,
none of the mutations can fully explain the disorder’s manifestation, because the same DNA abnormalities can be found in family
members with unimpaired vision. The prognosis is relatively poor:
although improvement sometimes occurs, significant permanent
impairment ( < 0.1) accompanied by opticusatrophy is common.
Some families in which Leber’s disease is present are additionally
affected by other central nervous system (CNS) disorders.

Compression and infiltration

16

Compression or infiltration causes progressive vision impairment within weeks or months. If the primary problem is a
disorder of the chiasma, impairment begins with temporal

hemianopsia (7sect. 9.1.3). In such cases, the cause is usually a
tumour of the pituitary gland (7sect. 22.3.6), optic glioma (e.g.
in Von Recklinghausen’s neurofibromatosis, 7sect. 28.5.2), or a
tumour in the anterior cranial fossa (meningioma, neurinoma
of the olfactory nerve). Other possible causes are leptomeningeal dissemination, sarcoidosis, lymphoma and granulomatosis
accompanied by polyangiitis (Wegener’s disease).
16.2.3 Oculomotor nerve (III)

The oculomotor nerve has a three-fold function (7sect. 6.2).
Hence, a disorder of this nerve can have various symptoms and
differential diagnosis is a complex process.
If the nerve is completely damaged, the patient exhibits ptosis, a dilated pupil that does not respond to light, and convergence accompanied by impaired oculomotor control, such that
the eye tends to point outwards (due to activity of nerve VI)
and slightly downwards (due to activity of nerve IV). The structure of the nerve is such that the parasympathetic fibres responsible for pupil constriction are peripherally located. Hence, in
the event of external compression – as particularly associated
with an aneurysm of the posterior communicating artery or

of the carotid artery; 7sect. 11.3.2 – the pupillary responses
will be affected first. Conversely, internal infarction (as associated mainly with diabetes mellitus) will not affect pupillary
responses. As a patient recovers from a total loss of oculomotor nerve function, the ptosis disappears first, and vertical eye
movement is the last function to return to normal.
Causes of failure of the oculomotor nerve (nerve III)
5
5
5
5
5
5
5
5

5
5

vascular (diabetes, hypertension)
compression by an aneurysm
transtentorial compression (7sect. 19.3)
orbital process
infarction or haemorrhage in the midbrain
ophthalmic herpes zoster
ophthalmoplegic migraine
basal meningitis
leptomeningeal metastases
pathology of the cavernous sinus (7sect. 16.3)

Pupil abnormalities
A patient’s pupils may be abnormally small or abnormally large.
The pupils normally constrict in response to light or convergent
movement of the eyes; the absence of those reflexes may be
indicative of a cranial nerve disorder. Having pupils of different sizes is referred to as anisocoria. Dilation is induced by the
sympathetic system, and constriction by the parasympathetic
system (7sects. 6.2.2 and 7.5.1). A case of anisocoria should be
described in terms of which pupil is larger, not in terms of a left
or right-sided impairment.
Various medications influence pupil size. Pupil constriction can be induced by, amongst other things, barbiturates, opiates, pilocarpine (for the treatment of glaucoma); pupil dilators
include various anticholinergic agents (antidepressants, atropine) and cocaine.
A patient’s pupillary response to light may be poor, while
its response to convergence is normal, since the two responses
are controlled by different mechanisms. That is the case, for
example, in Adie’s syndrome and diabetic neuropathy, with
brainstem abnormalities (pretectal) and with Argyll-Robertson

pupils, as associated with syphilis.
Adie’s syndrome involves acquired neuropathy of the postganglionic fibres of the ciliary ganglion. The pupil is initially
dilated and does not respond to light; at a later stage, however, delayed constriction occurs following convergence, and
the pupil is subsequently slow to normalize (pupillotonia).
Ultimately, the pupil becomes permanently constricted and
unresponsive to light. Adie’s syndrome may be unilateral or
bilateral. The abnormal pupillary response is usually accompanied by impairment or loss of muscular extension reflexes in
the legs.
If a patient has a constricted, imperfectly rounded pupil
that does not respond to light but responds strongly to convergence, one should consider the possibility of Argyll-Robertson
pupil, attributable to a disorder of the dorsal midbrain associated with syphilis.


199
16.2 · Clinical presentation

Causes of abnormal pupil function
Dilation
5 dilation
5 oculomotor nerve palsy
5 bilateral optic nerve damage
5 pressure on the midbrain (pinealoma)
5neurosyphilis
5 intoxication (cocaine)
5 Adie’s syndrome (initial stage)
Constriction
5neurosyphilis
5 Adie’s syndrome (end stage)
5 intoxication (alcohol, opiates)
5 (partial) Horner’s syndrome (7sect. 7.5.3)

5 neuropathy of the sympathetic fibres in diabetes
(7sect. 13.7.2)

Ptosis
Drooping eyelids are common: many people have a droop on
one or both sides. It is usually interpreted as an inconsequential
personal characteristic, both by the person in question and by
people close to them. Drooping is sometimes due to a connective tissue abnormality; the prolonged use of hard contact lenses
can also play a role. Alternatively, ptosis may be attributable to a
muscular disorder. Where neurological disorders are concerned,
failure of the oculomotor nerve leads to marked ptosis, usually
sufficient to have sight implications, while in Horner’s syndrome
failure of the sympathetic mechanism causes only slight ptosis apparent to the patient only when looking in the mirror. It
should be borne in mind that other causal mechanisms can lead
to a patient without ptosis exhibiting abnormal narrowing of the
palpebral fissure, making his eyes seem smaller.
Causes of palpebral fissure narrowing (ptosis) and
related symptoms
5 oculomotor nerve palsy (ipsilateral pupil dilation)
5 Horner’s syndrome (ipsilateral pupil contraction;
7sect. 7.5.3)
5 myasthenia (7sect. 12.6.2)
5 myotonic dystrophy (7sect. 12.4.3)
5 mitochondrial myopathy (7sect. 12.4.6)
5 familial (myopathy?)
5fatigue
5 weakening of the connective tissue of the eyelid
(dermatochalasis, in the elderly)
5 posterior displacement of the eyeball within the
orbit (enophthalmos; possibly accompanied by

exophthalmos on the other side)
5 injury to the tendon of the superior tarsal muscle caused
by prolonged use of hard contact lenses
5 oedema of the eyelid (also present on the underside)

. Table 16.1  Causes of double vision
eye abnormality

– monocular double vision

eye muscle abnormality

– non-neurological esophoria
(‘squinting’) or exophoria (‘wall
eyes’)
– intermittent in myasthenia
gravis (7sect. 12.6.2)
– very occasionally myositis

orbital process

– often a degree of exophthalmos
(‘bulging’ eye), e.g. bilateral in
myopathy
– thyroid disorder

orbital fracture
nerve III palsy

– crossed double vision, often

also ptosis and anisocoria

nerve IV palsy

– diagonal double vision during
downward convergence

nerve VI palsy

– horizontal uncrossed double
vision in the distance

brainstem injury

– the eyes often differ in height
(skew deviation) and/or internuclear ophthalmoplegia is present

5 blepharospasm (active tight closure of the orbicularis
oculi muscle; 7sect. 26.3.3)
5 pseudoptosis (due to tonus loss in the frontalis muscle
or the orbicularis oculi muscle associated with facial
nerve paralysis; 7sect. 16.2.7)

Binocular double vision
The first step in evaluation of double vision is to establish
whether it disappears when one eye is covered. If not, the
patient has monocular double vision, which usually has an
ophthalmological cause (e.g. early-stage cataract), although
no cause is identified in many cases. Oculomotor nerve palsy
causes crossed double vision (7sect. 6.2.2). The main causes of

binocular double vision are listed in .tab. 16.1.

Vascular disorder
The cause of acute double vision is often a vascular disorder,
especially in elderly people with diabetes or hypertension. In
such cases, pupillary response is unaffected, because the fibres
responsible are located peripherally within the nerve and therefore, due to external vascularization, remain functional in the
event of central artery failure. The prognosis is favourable.
Examination using imaging techniques is not strictly necessary in the absence of a pupil disorder, but is nevertheless carried out in many cases. If the patient exhibits pupil dilation, it
is important to investigate the possibility of compression by an
aneurysm, originating in the posterior communicating artery
or the carotid artery, or by a space-occupying process in the
middle cranial fossa.

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Chapter 16 · Disorders of the cranial nerves

16.2.4 Trochlear nerve (IV)

The core symptom of trochlear nerve palsy (7sect. 6.2.3) is
double vision that is greatest when the gaze is downwards and
towards the healthy eye (towards the tip of the nose); when
looking in that direction, the images are not parallel. Typically,
therefore, the patient experiences double vision when going
down stairs. The image from the affected eye is lower than that
from the good eye. The double vision disappears when the head

is inclined towards the shoulder on the side of the healthy eye;
hence, the patient may present with torticollis.
The main cause of isolated trochlear nerve palsy is a traumatic brain injury (e.g. birth trauma). In most cases, however,
no cause is found.
Causes of the trochlear nerve palsy (IV)
5 traumatic brain injury
5 vascular disorder (diabetes)
5 orbital pathology
5ethmoiditis
5 pathology of the cavernous sinus (7sect. 16.3)
5 cranial base abnormalities
5 brainstem syndrome

neurological abnormalities, the first possibility to consider is
mixed connective tissue disease or tumour metastasis (bone or
leptomeningeal); the patient will typically experience gradually
increasing numbness of the chin and adjacent half of the lower
lip (numb chin).
A trigeminal nerve disorder will rarely cause masseter
weakness, but that is possible in the event of compression and
trauma, mainly as associated with traumatic injury due to thermocoagulation in trigeminal neuralgia, 7sect. 21.3.1.
Causes of abducens nerve palsy (nerve VI)
5vascular
5 intracranial hypertension
5 CSF hypotension (7sect. 10.8.4)
5toxic
5 traumatic brain injury
5 basal meningitis, syphilis
5 polyradiculoneuritis (7sect. 13.3.2)
5 leptomeningeal metastases (7sect. 22.5)

5 acute vitamin B1 deficiency (7sect. 8.7.3)
5 injury to the pons (vascular, MS, tumour; .fig. 16.1)
5 orbital or periorbital process (7sect. 16.3)

16.2.6 Abducens nerve (VI)
Causes of sensation disorders of the face and oral
cavity

16

5 postherpetic neuralgia (7sect. 23.4.5), (mainly of the
upper ramus of nerve V: 1st ramus)
5 dental extraction (nerve V: 2nd and 3rd rami)
5 facial trauma (nerve V: 2nd and 3rd rami)
5 sinusitis (nerve V: 2nd and 3rd rami)
5 Intoxication (trichloroethylene)
5 syringobulbia (7sect. 15.5.3)
5 brainstem process
5 trigeminal neurinoma
5 acoustic neurinoma (7sect. 22.3.5)
5MS
5 meningioma (usually located at the sphenoid bone;
7sect. 16.3)
5 pathology of the cavernous sinus (7sect. 16.3)
5 cranial base process
5 leptomeningeal metastases
5 collagen disease
5 nasopharyngeal carcinoma
5 pressure caused by dental prosthesis
5 subjective in cases of muscle weakness

5psychogenic

16.2.5 Trigeminal nerve (V)

A disorder of the trigeminal nerve (7sects. 6.2.4 and 6.3.4) can
result in loss of function (neuropathy) or irritation (neuralgia;
7sect. 21.3.1), or sometimes both. In neuropathy without other

Abducens nerve palsy will often cause double vision when
looking into the distance; the image misalignment is purely
horizontal, without vertical displacement or crossing
(7sect. 6.2.3).
Although less common than infarction of the oculomotor
nerve, infarction of the abducens nerve does occur. The length
of its pathway from the pons to the orbit makes the abducens
nerve very vulnerable to intracranial hypertension; double
vision in the distance can be the first sign of such a disorder,
along with headaches. Hypotension (CSF hypotension syndrome, e.g. following lumbar puncture) can also lead to nerve
VI palsy (7sect. 10.8.4). Furthermore, the use of vincristine,
chloroquine or streptomycin can bring about (often rever­
sible) abducens nerve palsy. Palsy can occur in MS as well
(.fig. 16.1). In 30 to 50 % of patients who experience acute
palsy, the cause remains unexplained and spontaneous recovery
follows.
16.2.7 Facial nerve (VII)

In addition to its primary motor function (7sect. 6.2.6), the
facial nerve has sensory (7sect. 6.2.5) and autonomic functions
(7sect. 7.1.2). If a patient presents with facial weakness, the priority is to establish whether the disorder is central (corticobulbar tract) or peripheral (facial nerve or nucleus) (7sect. 6.2.6).

201
16.2 · Clinical presentation

Other
5hypertension
5amyloidosis
5porphyria
5diabetes
5 toxic causes
5 nuclear aplasia (Moebius syndrome)

Acute facial palsy

. Figure 16.1  Pontine lesion associated with MS in a 35-year-old woman
exhibiting right abducens nerve palsy

Causes of facial nerve palsy (nerve VII)
Infections
5 herpes simplex
5 herpes zoster oticus (7sect. 23.4.5)
5mastoiditis
5 acute otitis media
5cholesteatoma
5 purulent meningitis
5 neuroborreliosis (7sect. 23.9)
5 sarcoidosis (7sects. 23.9 and 24.8.1)
5 HIV (7sect. 23.5)
5 acute anterior poliomyelitis (7sect. 23.4.4)
5 polyradiculoneuritis (7sect. 13.3.2)

Trauma
5 basilar skull fracture
5 forceps delivery injury
Peripheral tumours
5 acoustic neurinoma
5neurofibromatosis
5 parotid tumour
5 leptomeningeal metastasis
Brainstem disorder
5 pontine glioma
5MS
5vascular

Acute facial palsy without immediately apparent cause was formerly referred to as Bell’s palsy, but is now generally known as
idiopathic peripheral facial palsy (IPFP). The incidence of the
disorder is 20 to 30 cases per 100,000 of the population per
year; it is the most common form of acute peripheral facial
palsy. The disorder can occur at any age, but is most common
between the ages of fifteen and forty-five.
A diagnosis of IPFP cannot be made until other possible
causes, principally otitis, have been excluded. Systemic conditions such as sarcoidosis, polyradiculoneuritis and infection
(neuroborreliosis) often lead to bilateral palsy. In a child, neuroborreliosis must also be considered in the event of unilateral
palsy.
In 70 % of cases, the patient exhibits total paralysis; in
the remaining cases, the disorder manifests itself as weakness
About 10 % of patients suffer relapse. The symptoms begin
suddenly or develop over a period of hours or days, often preceded and initially accompanied by pain behind the ear. One
patient in three exhibits a gustatory disorder affecting the front
two-thirds of the tongue on the same side (7sect. 6.3.5). In the
affected eye, lacrimal secretion is sometimes impaired. However, it may seem as if over-secretion is occurring, but that is

actually due to the tear duct opening no longer being the lowest
drainage point and tears consequently running over the drooping lower eyelid.
The pathophysiology of IPFP is unclear. The activation of
a latent herpes simplex virus may be involved. In some cases,
the trigeminal nerve is also affected, in which case the varicella
zoster virus is responsible and the condition is referred to as
herpes zoster oticus or Ramsay-Hunt syndrome.
Following recovery without significant residual impairment, there may be mild residual symptoms in the form of
slight palsy or contracture, and blinking may be delayed on
the affected side. Where recovery is incomplete, the patient
may continue to exhibit significant palsy and subsequent contracture, ‘crocodile tears’ (unilateral hyperlacrimation while
eating) and involuntary coordinated movement (synkinesia),
due to the fibres of the oral ramus innervating the muscles of
the optic ramus and vice versa. IPFP usually (in 85 % of cases)
heals completely or almost completely within six to eight
weeks. However, functional recovery takes at least six months.
The prognosis is less favourable following complete paralysis (61 %, compared with 94 % in cases of paresis), if signs of
recovery are not apparent within three weeks, in patients over
the age of sixty, and in cases of comorbidity (hypertension, diabetes mellitus). The prognosis for the recovery of facial nerve

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Chapter 16 · Disorders of the cranial nerves

Sometimes, however, an intrapontine process is present
(tumour, MS). Involuntary contractions of the facial muscles
are usually attributable to tics (7sects. 5.1.2 and 26.3.7).

16.2.8 Vestibulocochlear nerve (VIII)

Neurogenic deafness

. Figure 16.2  MRI scan of the cerebellopontine angle. A loop of the right
anterior inferior cerebellar artery (AICA; top left of the image) is compres­
sing the facial nerve, leading to hemifacial spasm

16

function in Ramsay-Hunt syndrome is significantly worse. The
House-Brackmann Scale (I-VI) for the grading of facial palsy
can be used for progress monitoring.
Therapy initially consists of protecting the cornea against
drying by means of eye ointment or eye drops, and covering the eye with a dressing overnight. If the patient presents
within seventy-two hours following a major failure (inability
to close the eye), administration of oral prednisone for seven
to ten days is indicated. There is evidence to suggest that, in a
case of serious nerve damage (House-Brackmann score of V or
VI), the addition of an antiviral agent (valaciclovir/famciclovir)
improves the prognosis for full recovery. An antiviral agent is
also indicated in Ramsay-Hunt syndrome.
In the convalescent phase following permanent serious
nerve damage, it is desirable to involve a speech therapist with
a view to establishing whether mime therapy may be beneficial.
Speech exercise therapy can also help with swallowing problems, which sometimes develop following facial nerve palsy.

Irritation
Irritation of the facial nerve can induce spasms: series of massive contractions of one half of the face lasting between a
few seconds and a minute or so (hemifacial spasm). In about

10 % of cases, the spasms are a residual symptom of injury,
as described above; in the other cases, they may be due to
compression of the nerve by the anterior inferior cerebellar
artery or posterior inferior cerebellar artery (AICA or PICA;
.figs. 16.2 and 11.1) or by a branch of one of those arteries. In
rare cases, a local tumour is the cause. Surgical decompression
of the nerve is often effective (Jannetta procedure). Another
option is botulin toxin therapy. Various other medications (carbamazepine and other anti-epileptics, baclofen, clonazepam)
have little effect.
Hemifacial spasm is nearly always a unilateral disorder,
unlike blepharospasm, a form of dystonia (7sect. 26.3.3).
Facial myokymia are subtle muscle contractions affecting part of the face, sometimes unnoticed by the patient.
They are benign and may be triggered by emotions or fatigue.

If perception deafness is observed, an ENT specialist can generally establish by audiometric testing whether the deafness is
attributable to a cochlear or retrocochlear disorder; the latter is
usually due to a problem with the acoustic nerve.
The most common cause of acute unilateral perception
deafness (idiopathic sudden sensory-neural hearing loss) is
unknown. The incidence is estimated to be 8 in 100,000 per
year. There is often a temporary vestibular disorder and tinnitus
is almost always present. In 30 % of cases, the patient has suffered a respiratory infection in the previous month. About half
of patients experience spontaneous recovery, with the completeness of recovery correlating to the mildness of the initial failure.
The problem is likely to be located in the auditory organ itself.
Drugs that can induce perception deafness and disorders
of the vestibular nerve are: aminoglycosides (streptomycin and
related substances), quinine derivatives, acetylsalicylic acid,
cytostatics and some diuretics. Tumours of the cerebellopontine angle (neurinoma, usually originating in the vestibular
nerve) are dealt with in 7 chap. 22 (7sect. 22.3.5, .fig. 22.5).
Causes of neurogenic deafness

5 acoustic trauma
5 tumour of the cerebellopontine angle (7sect. 22.3.5)
5 congenital dysplasia
5drugs
5 herpes zoster oticus, measles, mumps
5 intoxication, e.g. by a solvent
5 mechanical trauma
5 meningitis (7sect. 23.2)
5 mitochondrial disorder
5 basal skull fracture (7sect. 20.5)
5 Ménière’s disease

Vestibular vertigo
Disorders of the labyrinth (7sect. 6.2.4) are usually acute. The
sudden failure of a vestibular organ (due to trauma, haemorrhage, infection, labyrinth extirpation) is accompanied by
severe vertigo with nausea and vomiting, which diminishes
over a period of weeks due to adaptation by central mechanisms. Examination should reveal vestibular nystagmus
(7sect. 6.2.8). If both vestibular organs are affected (e.g. by
intoxication), the patient will experience disorientation and
oscillopsia. Oscillopsia involves the environment seeming to
move whenever the head is moved. Normally, the sensation is
prevented by the vestibulo-ocular reflexes.


203
16.2 · Clinical presentation

As a result of adaptation, gradual unilateral loss of function
of the labyrinth or vestibular nerve (nerve VIII) creates few
problems in everyday life. The same is true of central disorders

that cause vertigo and sensations of movement.
Migraine patients often suffer vestibular attacks that are not
necessarily followed by the usual headaches. Such attacks do not
involve hearing loss or tinnitus. They are therefore described as
benign recurrent vertigo. Young children (one to four years old)
can experience vertigo attacks lasting seconds to minutes, in
which the children go pale. The children concerned are typically
from families in which migraine occurs, and some of them go on
to develop typical migraine. Finally, the disorder known as basilar migraine (7sect. 21.2.3) is sometimes accompanied by vertigo.

Ménière’s disease
Ménière’s disease is characterized by spontaneous vertigo
attacks, usually accompanied by vegetative symptoms, such as
nausea, vomiting, perspiration and, very occasionally, syncope,
in combination with auditory abnormalities (impaired hearing and tinnitus). Ménière’s disease may be diagnosed only if
the patient exhibits vertigo attacks, impaired hearing and tinnitus, and no other cause can be found. Before and during
the attack, the patient has the sensation that the affected ear is
blocked, often followed by hearing impairment and/or tinnitus (7sect. 6.2.8). During the attack, nystagmus is present, the
direction of which is a matter of debate. There is good reason to
assume that the labyrinth is hyperstimulated in the acute phase
– causing nystagmus towards the affected ear, 7sect. 6.2.8 –
and subsequently suppressed – causing nystagmus towards
the healthy ear. Attacks last for a few hours only, and are followed by symptoms that gradually diminish over a few days.
The frequency and seriousness of the attacks varies. However,
they peak in the first two years, and 80 % of patients experience spontaneous remission within five to ten years. Ultimately,
patients are liable to experience Tumarkin attacks, which
involve falling and the sense of being pushed over. Progressive
loss of the ability to hear high-frequency sound almost always
occurs, which is usually unilateral but is bilateral in about a
third of patients. The disorder mainly affects people between

the ages of thirty and fifty. The annual incidence is 40 to 50
cases per 100,000, while the prevalence is 300 per 100,000.
The pathophysiological mechanism responsible is uncertain. However, it is believed that the flow of endolymph from
the cochlea to the vestibular organ is intermittently excessive.
As a result, the utriculus becomes over-full and the semicircular canals are hyperstimulated. There is no therapy that
addresses the cause. Betahistidine can reduce the frequency of
the attacks, however. Numerous other therapies, including surgery to disable the cochlea, have proved largely ineffective. In
therapy-resistant cases, local treatment with aminoglycosides
appears to be beneficial. However, caution should be exercised,
because aminoglycosides are ototoxic.

Benign paroxysmal positional vertigo
The patient presents with an acute, extreme sensation of spinning triggered by changing position, e.g. going from standing
to lying or from lying to sitting, and sometimes by turning

over when lying (even while sleeping), and by rapidly tilting the head backwards or to the side. An attack can therefore be provoked by getting the patient to move from sitting
to lying within a few seconds, with his head turned, and then
supporting the patient’s head and lowering it through about
30 degrees (Hallpike manoeuvre). Typically, the spinning sensation develops after a latency period of five to ten seconds, and
disappears quite quickly (within ten to thirty seconds) if the
patient keeps still. After being repeated several times, the provoking manoeuvre ceases to be effective. The condition involves
horizontal rotatory nystagmus towards the affected ear, which
is therefore hypersensitive. In practice, however, the nystagmus
is not always observed, because it is suppression by fixation.
The disorder is observed following cranial trauma, viral neuronitis, during or after prolonged bed rest and in periods of stress.
However, in more than half of patients, no cause can be identified. Patients in whom no cause can be found are usually elderly
and two thirds of them are women. The disorder is very common.
The pathophysiology involves abnormal stimulation of the
sensory organ in the posterior canal of the labyrinth due to
an accumulation of debris impeding the flow of endolymph

(canalolithiasis). Another, less common cause is the attachment
of debris to the cupula.
After giving the patient reassurance and explanation, the
Epley manoeuvre (a variation on the Hallpike manoeuvre)
can be performed with a view to immediately resolving the
problem. If the patient has residual problems, daily positional
change exercises can be prescribed, whereby the patient repeatedly moves quickly from sitting to lying on his side, then sits
up again after thirty seconds. During the exercises, the head
should be turned so that the patient lies with the ipsilateral side
of the occipital region on the bed. The patient should lie first to
the left and then to the right. Improvement often occurs after
one to four weeks, after which the exercises may be gradually
phased out. Even without exercises, the attacks usually cease
after a few weeks or months. Medicinal therapy is not desirable.

Vestibular neuritis
In vestibular neuritis or neuronitis, the patient experiences
fairly sudden, continuous vertigo accompanied by vegetative symptoms. Horizontal rotatory nystagmus away from the
affected ear (slow phase towards the affected ear) is observable,
and the patient is liable to fall and point off-centre, towards the
affected side. There is no hearing loss or tinnitus. The vertigo
remains severe only for a few days (three to five), during which
time it is best for the patient to remain in bed. It then gradually
diminishes over a period of four to six weeks, although oscillopsia (see above) following rapid head movements may persist. About half of patients have residual symptoms, which are
sometimes manifest only under particular circumstances (e.g.
on fairground rides and boats).
The affected labyrinth is usually insensitive to stimuli during the acute phase and subsequently exhibits reduced sensitivity for a prolonged period. The nystagmus is second or
third degree in the acute phase and subsequently present only
when the patient looks to the healthy side. The disorder occurs
mainly in middle age, particularly in the autumn and winter. It

16


204

Chapter 16 · Disorders of the cranial nerves

is assumed to be a viral disorder, but the responsible pathogen
has not been identified and not all patients have experienced a
prior infection. Nor is it clear whether it is the vestibular nerve
or the labyrinth that becomes infected. The same vertigo syndrome can develop in the event of infection by herpes zoster
oticus (7sects. 16.2.7 and 23.4.5). Therapy consists of rest and
possibly anti-emetics in the first days, followed by mobilization
as soon as possible. Antivertiginous medication may help to
mitigate the symptoms in the early stages. Antiviral agents do
not appear to help, but corticosteroids can expedite recovery.

Non-vestibular vertigo
The presence of vertigo does not necessarily imply a problem
with the vestibular organ. Many other problems are referred
to as vertigo well. Thorough history taking is the main way of
differentiating between other forms of ‘vertigo’ and vestibular
vertigo. Vestibular vertigo is accompanied by a sensation of
movement, whereas other forms involve light-headedness or
blackness in front of the eyes, and sometimes ringing in the
ears. A lack of sensory information (tactile, visual or auditory)
can induce a sense of disorientation; the problem accumulates
if multiple sensory problems are present (multi sensory deficit).
Non-vestibular vertigo

5 blood pressure regulation disorders
5 hyperventilation/panic attacks
5 disorders of central control regulation
5 gnostic sensory disorders of the legs
5 impaired vision
5diplopia
5 impaired hearing

Semi-solid non-fibrous food usually gives rise to fewest problems, while watery fluids are particularly difficult for
patients to swallow. Dairy products are undesirable, due to
their influence on mucous production. Patients can often sense
(and therefore swallow) cold foodstuffs much better than hot
foodstuffs (particularly in ALS and myasthenia). In cases of
unilateral function loss, it helps to turn the head, usually to the
affected side. In acute dysphagia, e.g. after a stroke, it is often
necessary to temporarily stop oral intake and to instigate saliva
suction and temporary nasogastric or parenteral feeding. In
chronic cases (ALS), percutaneous gastrostomy followed by
gastric tube feeding is often the best solution.
Causes of neurological swallowing disorders
– diminished level of consciousness
– pseudobulbar
disorders (7sect. 6.6)

– bilateral cerebral infarction in
the acute phase of unilateral
cerebral infarction
– MS (7sect. 24.2)
– ALS (7sect. 13.2.3)

– acute phase following
stroke

– (7sects. 6.6 and 17.5.1)

– extrapyramidal
disorders

– Parkinson’s disease
(7sect. 26.1.2)
– dystonia (7sect. 26.3.3)

– disorders of the
medulla oblongata

– Wallenberg’s syndrome
(7sects. 6.7.3 and 17.4.6)
– ALS (7sect. 13.2.3)
– poliomyelitis (7sect. 23.4.4)

– cranial nerves VII, IX, X
and XII

– skull base tumour
– polyradiculoneuritis
(7sect. 13.3.2)
– diphtheria
– leptomeningeal metastases
(7sect. 22.5)

– neuromuscular
transmission

– myasthenia gravis
(7sect. 12.6.2)
– botulism (7sect. 12.6.4)
– botulin toxin therapy

– muscular disorders

– oculopharyngeal dystrophy
– polymyositis (7sect. 12.5.1)
– myotonic dystrophy
(7sect. 12.4.3)

– autonomic plexopathy
of the oesophagus

– achalasia

– unexplained

– ‘psychogenic’ – globus
sensation

16.2.9 Glossopharyngeal nerve (IX) and vagus

nerve (X)

16

The functions of the glossopharyngeal nerve and vagus nerve
(7sect. 6.2.9) are closely connected in a swallowing action. Swallowing disorders are not always spontaneously reported, particularly by the elderly. Eating more slowly, eating less, weight
loss, increased salivation, coughing after drinking, food or
drink getting up the back of the nose and repeated respiratory
infections are important indicators and the patient should be
asked specifically about such symptoms. Coughing or a ‘watery
voice’ after drinking and fluid getting up the back of the nose
suggest choking due to palatal weakness. Such weakness is readily observable by asking the patient to drink a glass of water.
Problems such as a sensation of thea, 257, 259
rhombencephalon, 338
rib resection, 172
rigidity, 37, 52, 312, 313
riluzole, 152
Rinne’s test, 75
rising sensation, 72
rods, 106
Romberg’s test, 42, 56
rt-PA, 216
rubella, 341
–– ataxia, 306


370

Register

S
saccular, 129
sacral sparing, 185, 186

saddle anaesthesia, 187
sagittal, 14
salaam spasm, 345
salt loss, cerebral, 221
salt-wasting, cerebral, 87
saltatory conduction, 18
Sandhoff disease, 344
sarcoglycan, 19, 140
sarcoidosis, 351
–– neuropathy, 164
sarcolemma, 19, 20
sarcoplasmic reticulum, 20
Saturday night palsy, 155
SCA, 306
SCA6 gene, 309
SCA14 gene, 309
scalenotomy, 172
schizophrenia, 247
Schwabach’s test, 75
Schwann cells, 16
sciatica, 159, 172
scintillations, 264
SCN1A gene, 266
scotoma, central, 110
second-wind phenomenon, 141
seizure, psychogenic, 238
selective serotonin reuptake inhibitors (SSRIs), 318
sella turcica, 86, 356
semioval centre, 356
senile plaques, 328

sensation
–– epicritic, 30
–– examination, 32
–– gnostic, 30
–– protopathic, 30, 32
sense of detachment from
reality, 72
sense of motion, 42
sense of temperature, 42
sense of vibration, 34
sensibility, 31
–– epicritic, 34
–– protopathic, 34
–– vital, 34
sensitivity, 9
sensory disturbance
–– dissociated, 32
sensory evoked potentials (SEP), 46
sensory extinction, 42
sensory impairment
–– objective, 41
–– subjective, 41
sensory information, course, 65
sensory neuronopathy, 43, 281
sentence melody, 97
sequestration, 173
serial seven test, 103
serotonergic drugs, 238
serotonergic system in Parkinson’s
disease, 315

serotonin, 18, 61, 63, 170, 242
sexual function, disorders, 91
shingles, 290

short-term memory, 100
shoulder amyotrophy, neuralgic,
155
shoulder dystocia, 342
shunt, ventriculoperitoneal, 120
sIADH, 87
sight field, 107
–– disorder, 107
–– failure, 107
–– testing, 110
sigmoid sinus, 124
signe du rideau, 77
simultanagnosia, 109
single-photon emission computed
tomography (SPECT), 15
sinus
–– cavernous, 198, 200, 205
–– frontal, 287, 356
–– sphenoid, 356
–– superior sagittal, 116
sinus thrombosis, 210, 223, 244, 350
–– causes, 223
–– diagnosis, 223
–– symptoms, 223
–– treatment, 223
sinusitis, 200, 223

Sjögren’s disease, 303, 351
Sjögren’s syndrome, 163
–– neuropathy, 164
skeletal muscle, 18
skew deviation, 199
skin discolouration, 149
skull fracture, 256
–– cranial vault, 257
–– depressed, 256
–– linear, 256
sleep apnoea syndrome, 237
sleep myoclonus, 238
sleep paralysis, 238
sleep state (EEG), 23
sleepiness, excessive, 237
sleepwalking, 238, 247
slurring, 55
small-cell lung cancer, 144
small-fibre, 160
small fibre neuropathy, 42
small-fibre neuropathy, 150, 160,
161, 163
small vessel disease, 329
smell, 65, 73
smoking
–– risk of cerebral infarction, 209
–– risk of subarachnoid haemorrhage, 222
SNAP, 23
sodium valproate, 234
soma (neuron), 16

somatosensory evoked potentials
(SSEPs)
–– coma, 246
somatotopy, 34
somatotropin, 88
somnambulism, 247
somnolence, 243
space-occupying lesion, intracranial
–– general symptoms, 273
–– local symptoms, 274
spasm, hemifacial, 202

spasmodic dysphonia, 322, 323
spasmodic torticollis, 322
spastic bladder, 91
spastic paraparesis, tropical, 194,
310
spastic paraplegia (SPG), 310
spastic paresis, 29
spasticity, 13, 29, 37, 52, 343
spatial disorder, 100
specificity, 9
SPECT, 15
speech
–– impaired, 55
speech arrest, 228
speech disorder, 73
speech rhythm
–– impaired, 55
sphingolipidosis, 343

Spielmeyer-Vogt-Sjögren-Batten
disease, 344
spina bifida, 339, 340
–– occulta, 339
spinal anaesthetic, 192
spinal canal stenosis
–– cervical, 189, 190
–– rheumatoid arthritis, 189
spinal column
–– diagnosis, 182
–– injury, 183
spinal cord, 13, 15, 34, 62
–– anterior horn, 28
–– compression, 183, 189, 191
–– course, 181
–– diagnosis, 182
–– dorsal funiculi, 56
–– injury, 183, 187
–– reflex response, 28
–– somatotopy, 32
spinal cord compression, 43
spinal cord injury
–– effects, 181, 184
–– incomplete, 182
–– prognosis, 187
–– treatment, 187
spinal cord segment, 13, 181, 182
spinal dural arteriovenous fistula,
193
spinal metastasis, 281

spinal muscle atrophy (SMA), 151
spinal muscular atrophy, 39, 151
–– adult form, 151
–– distal, 151
–– progressive, 151, 152
–– scapuloperoneal, 151
–– spinobulbar, 151
–– symptoms, 308
spinal posterior horn, 63, 85
spinal reflex arc, 29, 41
spinal shock, 64
spinal syphilis, 310
spinocerebellar ataxia, 306
–– autosomal dominant, 309
–– autosomal recessive, 309
–– classification, 306
–– symptoms, 308
spinocerebellum, 53
spinothalamic tract, 31, 34
spondylarthrosis deformans, 174

spondylitis, 176
spondylolisthesis, 174
spondylolysis, 172
spongiform encephalopathy, 332
spontaneous spinal epidural haematoma, 186
spreading depression, 265
Spurling’s compression sign, 171
St Vitus’ dance, 321
Staphylococcus aureus, 286

startle disease, 324
state of detachment, 247
statin, 218
statoliths, 72
status epilepticus, 227
–– non-convulsive (NCSE), 246
status migrainosus, 266
stenosis
–– lumbar, 176
steppage gait, 159
stereognosis, 42
–– testing, 103
stereotactic surgery, 317
steroid-responsive encephalopathy
with autoimmune thyroiditis,
350
stiff man syndrome, 281
stiff person syndrome, 281
strabismus, 73, 74
–– convergent, 74
–– divergent, 74
strength
–– examination, 32
–– loss of, 34
–– loss of, differential diagnosis, 38
Streptococcus agalactiae, 286
Streptococcus pneumoniae, 286
stretching (M2), 78
striatum, 49
stridor, 73

stroboscopy, 231
stroke
–– blood pressure, 217
–– clinical diagnosis, 210
–– completed, 210
–– complications, 216
–– depression, 218
–– epidemiology, 209
–– imaging, 210
–– prevention, 217
–– prognosis, 209
–– progressive, 210
–– rehabilitation, 218
stroke service, 216
stroke unit, 216
Sturge-Weber-syndrome, 345, 346
stuttering, 55
subarachnoid haemorrhage, 116,
119, 129, 219, 221, 244
subarachnoid space, 114, 116
subdural haematoma, 258, 259, 261
subdural hygroma, 253
subependymal nodule, 345
substance, Nissl, 16
substantia nigra, 49, 62, 63, 67, 315
subthalamic nucleus, 49
superior orbital fissure, 205
suppression, 74

371
Register

supranuclear
–– bladder disorder, 90
–– cranial nerve disorder, 65
–– disorder, 79
supratentorial structures, 114
Susac syndrome, 299
swallowing, 73
–– oesophageal phase, 73
–– oral phase, 73
–– pharyngeal phase, 73
swallowing disorder, 204
–– cause, 204
–– pseudobulbar, 204
swallowing problem
–– stroke, 216
sweat secretion, 149
Sydenham’s chorea, 321
Sylvian fissure, 356
sympathetic, 85
sympathetic fibres, 171
sympathetic tract, central, 62
symptoms, 149
–– normal, 8
synapse, 18–20
synapse, neuromuscular, 135
syncope, 350
synkinesia, 201

synucleinopathy, 315
syphilis, 192, 210, 285, 292
–– ataxia, 306
syphilitic meningomyelitis, 310
syringobulbia, 70, 190, 200, 205
syringomyelia, 43, 189, 190
syrinx
–– post-traumatic, 187
system, limbic, 96
systemic lupus erythematosus
(SLE), 163, 223, 351
–– neurological complications, 303
–– neuropathy, 164

T
T-tubule system, 18, 20
tabes dorsalis, 292
Takayasu’s disease, 224
tangles, 328
tarsal tunnel syndrome, 159
taste, 66, 70
–– examination, 75
tau pathology, 331
tau protein, 328
tauopathy, 319, 320
Tay-Sachs disease, 344
TDP-43 pathology, 331
tectum, 63, 66, 68
telangiectasia, 129, 347
telencephalon, 13

temperature sensation, 34
–– impaired, 190
temporal arteritis, 142, 224, 268
temporal lobe, 356
temporomandibular dysfunction,
267
tendency to fall backwards, 102
tension headache, 268
–– diagnostic criteria, 269

tentorium cerebelli, 114
teratogenicity, 341
Terson’s syndrome, 220
test
–– Hallpike’s, 76
testicular atrophy
–– myotonic dystrophy, 140
testing sensation, 41
tetanus, 145, 291
tetany, 239, 351, 352
tethered cord, 339
tetraparesis, 38, 245
tetraplegia, 30
thalamic pain syndrome, 43
thalamus, 13, 31, 34, 49, 216, 356
–– infarct, 43
theory of mind
–– loss of, 331
thin-fibre neuropathy, 43
thinking

–– testing abstract, 104
third ventricle, 356
Thomsen’s disease, 140
thoracic outlet syndrome, 171
thrombectomy, 223
thrombocytosis, risk of cerebral
infarction, 210
thrombolysis, 223
–– intravenous, 216
thunderclap headache, 220, 222,
269
thymectomy, 143
thymoma, 143
thyroid disorder, double vision, 199
thyroid peroxidase antibodies, 351
TIA. Zie transient ischaemic attack
tic, 52, 54, 324
–– complex, 324
–– douloureux, 266
–– motor, 324
–– phonetic, 324
–– sensory, 324
–– simple, 324
–– tonic, 324
time-of-flight (TOF), 130
Tinel’s sign, 158, 170
tingling, 18, 43
tinnitus, 353
titubation, 54, 320
tobacco amblyopia, 351

Todd’s paralysis, 228
Tolosa-Hunt syndrome, 205
tone, 37
tongue atrophy, 205
topographical disorientation, 100
torsion dystonia, 322
torsion spasm, 52
torticollis, 54, 200, 322
touch, 42
–– neutral, 34
Tourette’s syndrome, 324, 348
toxic gain-of-function hypothesis,
309
Toxoplasma, 290
Toxoplasma gondii, 290
toxoplasmosis, 341
tract, 13
–– anterior corticospinal, 31

––
––
––
––
––
––
––
––
––
––
––

––
––
––
––
––
––
––
––

ascending, 13
corticobulbar, 62, 64, 66, 73
corticopontine, 62, 69
corticospinal, 29, 31, 62, 66
corticospinal, construction, 340
corticospinal, decussation, 61
corticospinal, HSP, 307
corticospinal impairment, 29
corticospinal (pyramidal), 31
corticospinal, spinal cord, 181
descending, 13, 64
descending decussated, 28
dorsolateral, 61
lateral corticospina, 31
lateral pyramidal, 51
medial corticospinal, 64
optical, 67, 107
reticulospinal, 51, 64
reticulospinal, function in respiration, 79
–– rubrospinal, 51
–– solitary, 71

–– spinocerebellar, disorder in SCA,
306
–– spinocerebellar, 62
–– spinothalamic, 61–63
–– tectospinal, 51, 63
–– vestibulospinal, 51, 64
transient epileptic amnesia, 101
transient global amnesia, 101, 240
transient ischaemic attack (TIA), 127
–– differential diagnosis, 212
–– limb shaking, 239
–– risk of infarction, 212
transmitter, 17
transmitter mediation, 18
transplantation, 256
transport
–– anterograde, 16
–– retrograde, 16
transverse lesion
–– bladder disorders, 91
transverse sinus, 124
traumatic brain injury, 41
tremor, 52
–– action, 54
–– cerebellar, 319, 320
–– drug-induced, 319
–– dystonic, 320
–– essential, 319, 320
–– exaggerated physiological, 8,
320

–– frequency, 319
–– functional, 321
–– Holmes, 320
–– hyperthyroidism, 350
–– intention, 53, 54
–– kinetic, 320
–– orthostatic, 319
–– palatal, 320
–– Parkinson’s, 312
–– Parkinson’s disease, 319
–– physiological, 319
–– polyneuropathy, 319
–– postural, 319, 320
–– resting, 319
–– rubral, 319, 320
–– task-specific, 320

–– toxic, 320
tremor/ataxie syndrome, 306
Trendelenburg’s sign, 173
Treponema pallidum, 292
trigeminal neuralgia, 266
–– stereotactic radiotherapy, 266
–– treatment, 266
trigeminal neurinoma, 200
trigeminal neuropathy, 267
trigger point for trigeminal neuralgia, 266
trinucleotide repeat, 139
triptans, 266
trophic disorder, 149

tropical spastic paraparesis, 310
troponin, 19
troponin-tropomyosin complex, 19
trunk ataxia, 54
tuber, 345
tuberculoma, 291
tuberculosis, 177, 192, 291
tuberous sclerosis, 345
tumefactive multiple sclerosis, 298
tumour
–– cerebral, 274
–– intraspinal, extramedullary, 189
–– intraspinal, intramedullary, 189
tuning fork, 41
tunnel vision, 108, 214
twilight state
–– causes, 247
tympanic chords, 70

U
ubiquitin pathology, 331
ulceration, 128
ultrasonography, 15
umami, 71
uncinate fit, 229
unresponsive wakefulness syndrome, 256
Unterberger’s test, 76
uraemia, 145, 245
urea, 163
urea cycle, 343

urge to speak, 97
urinary incontinence
–– causes, 90
–– overflow, 91
–– stress, 91
–– urge, 90
utilization behaviour, 102

V
vacuolar myelopathy, 194
vagal stimulation, 236
valproate, 246
Van Wiechen chart, 338
varicella zoster virus
–– ataxia, 306
vascular abnormality, congenital,
350
vascular claudication, 176
vascular cognitive impairment
(VCI), 329

S–V


372

Register

vascular dementia (VaD), 329
vascular mild cognitive impairment

(VaMCI), 329
vasculitis, 163, 192, 210, 232
–– cerebral, 224
–– cerebral infarction, 210, 350
–– granulomatous, 351
–– necrotizing, 351
–– neuropathy, 350
vasogenic cerebral oedema, 128
vasopressin, 86, 88
vasospasm, 222
vegetative state, 243, 247, 256
vein
–– internal jugular, 205
venous sinus system, 114
venous sinus thrombosis, 128
ventral root, 13
ventricle
–– fourth, 15, 116, 356
–– third, 116, 239
ventriculostomy, 119
vermis syndrome
–– caudal, 54
–– rostral, 54
vertebral arteries, 124
vertebral metastase, 177
vertebral metastasis, 280
–– diagnosis, 280
–– origin, 280
–– treatment, 281
vertigo, 72, 202

–– benign paroxysmal positional,
203
–– central, 76
–– inner ear, 72
–– multisensory defect, 204
–– peripheral, 76
very long chain fatty acid, 344
vestibular disorder
–– central, 73
–– peripheral, 73
vestibular schwannoma, 277
vestibulocerebellum, 53
vibratory sense, 41, 42
vigilance, 242
visceral autonomic disorder, 149
vision, 106
–– impairment, 111, 197
–– testing, 110
visual acuity
–– impairment, 197
visual centre
–– cortical, 69
–– cortical frontal, 68
–– pontine, 66, 68, 69
–– vertical, 68
visual disorder, 108
visual evoked potentials (VEP), 46,
297
visual extinction, 110
visual illusion, 109

vital functions, 78
vitamin A
–– deficiency, 351
vitamin B1
–– deficiency, 101, 161–163, 198,
200, 246, 351

–– neonatal convulsion, 231
vitamin B6
–– deficiency, 162, 163, 351
–– neonatal convulsion, 231
vitamin B deficiency, coma, 245
vitamin B6 intoxication, 43
vitamin B12 deficiency, 43, 161, 163,
192, 193, 198, 341, 351
vitamin D deficiency, 352
vitamin E deficiency, 352
vitamin E malabsorption, 306
vocal cords
–– function, 73
voltage-sensitive (Na+)-channel ion
channels, 170
vomiting centre, 86
Von Hippel-Lindau disease, 346
–– tumour, 346
von Recklinghausen’s disease, 198,
345

W
wakeableness, 78

Waldenström’s macroglobulinaemia, 163
–– risk of cerebral infarction, 210
Wallenberg syndrome, 43, 80, 81,
204, 205
watershed infarction, 109, 124
watery voice, 204
wearing-off, 317
Weber’s test, 75
Wegener’s disease, 223, 351
–– neuropathy, 164
Wegener’s granulomatosis, 198, 224
Werdnig-Hoffmann disease, 151
Wernicke-Korsakoff syndrome, 101
Wernicke’s aphasia, 98
Wernicke’s area, 97–99
Wernicke’s encephalopathy, 101
Wernicke’s syndrome, 101, 246
West, syndrome van, 345
whiplash-associated disorder, 188
whiplash injury, 188
whole brain radiotherapy, 278
Willis, circle of, 114
Wilson’s disease, 321
withdrawal in response to pain
stimuli, 245
withdrawal response (M4), 77
word choice, 97
word salad, 98
working memory, 100
writer’s cramp, 323

writing
–– testing, 103, 104

Z
Zellweger syndrome, 344