Sunday, October 9, 2022

 orbital

fissure

Mandibular division of trigeminal

nerve (foramen ovale)

Facial and

vestibulocochlear nerves

(internal acoustic meatus)

Abducens nerve (inferior petrosal sinus)

Anterior cranial fossa

Middle cranial fossa

Posterior cranial fossa

Fig. 7.5 Base of the cranial cavity. The dura mater, with the cranial nerves and their exits from the skull. On the right side, part of the tentorium

cerebelli and the roof of the trigeminal cave have been removed.

Hyposmia or anosmia (reduction or loss of the sense of

smell) may result from upper respiratory infection, sinus

disease, damage to the olfactory filaments after head injury or

infection, local compression (by olfactory groove meningioma,

for example; see Fig. 7.29C) or invasion by basal skull

tumours. Disturbance of smell may also occur very early in

Parkinson’s and Alzheimer’s diseases. Patients often note

hypogeusia/ageusia (altered taste) with anosmia too, as taste

is crucially influenced by the sense of smell.

Parosmia is the perception of pleasant odours as

unpleasant; it may occur with head trauma or sinus

infection, or be an adverse effect of drugs. Olfactory

hallucinations may occur in Alzheimer’s disease and focal

epilepsies.

Optic (II), oculomotor (III), trochlear (IV)

and abducens (VI) nerves

See Chapter 8.

Trigeminal (V) nerve

The V nerve conveys sensation from the face, mouth and part of

the dura, and provides motor supply to the muscles of mastication.

Anatomy

The cell bodies of the sensory fibres are located in the trigeminal

(Gasserian) ganglion, which lies in a cavity (Meckel’s cave) in

the petrous temporal dura (see Fig. 7.5). From the trigeminal

Cranial nerves • 129

7

Jaw jerk

• Ask the patient to let their mouth hang loosely open.

• Place your forefinger in the midline between lower lip and

chin.

• Percuss your finger gently with the tendon hammer in a

downward direction (Fig. 7.8), noting any reflex closing of

the jaw.

• An absent, or just present, reflex is normal. A brisk jaw

jerk occurs in pseudobulbar palsy (Box 7.5).

The ophthalmic branch leaves the ganglion and passes forward

to the superior orbital fissure via the wall of the cavernous sinus

(see Fig. 8.3). In addition to the skin of the upper nose, upper

eyelid, forehead and scalp, V1 supplies sensation to the eye

(cornea and conjunctiva) and the mucous membranes of the

sphenoidal and ethmoid sinuses and upper nasal cavity.

The maxillary branch (V2) passes from the ganglion via the

cavernous sinus to leave the skull by the foramen rotundum.

It contains sensory fibres from the mucous membranes of

the upper mouth, roof of pharynx, gums, teeth and palate

of the upper jaw and the maxillary, sphenoidal and ethmoid

sinuses.

The mandibular branch (V3) exits the skull via the foramen ovale

and supplies the floor of the mouth, sensation (but not taste)

to the anterior two-thirds of the tongue, the gums and teeth of

the lower jaw, mucosa of the cheek and the temporomandibular

joint, in addition to the skin of the lower lips and jaw area, but

not the angle of the jaw (see Fig. 7.6).

The motor fibres of V run in the mandibular branch (V3) and

innervate the muscles of mastication: temporalis, masseter and

medial and lateral pterygoids.

Examination sequence

Four aspects need to be assessed: sensory, motor and two

reflexes.

Sensory

• Ask the patient to close their eyes and say ‘yes’ each time

they feel a light touch (you use a cotton-wool tip for this

test). Do this in the areas of V1, V2 and V3.

• Repeat using a fresh neurological pin, such as a Neurotip,

to test superficial pain.

Compare both sides. If you identify an area of reduced

sensation, map it out. Does it conform to the distribution

of the trigeminal nerve or branches? Remember the angle

of the jaw is served by C2 and not the trigeminal nerve,

but V1 extends towards the vertex (see Fig. 7.6).

• ‘Nasal tickle’ test: use a wisp of cotton wool to ‘tickle’ the

inside of each nostril and ask the patient to compare. The

normal result is an unpleasant sensation easily appreciated

by the patient.

Motor (signs rare)

Inspect for wasting of the muscles of mastication (most

apparent in temporalis).

• Ask the patient to clench their teeth; feel the masseters,

estimating their bulk.

• Ask the patient to open their jaw and note any deviation;

the jaw may deviate to the paralysed side due to

contraction of the intact contralateral pterygoid muscle.

Corneal reflex

Routine testing of the corneal reflex is unnecessary, but may

be relevant when the history suggests a lesion localising to

the brainstem or cranial nerves V, VII or VIII. The afferent limb

is via the trigeminal nerve, the efferent limb via the facial

nerve.

• Explain to the patient what you are going to do and ask

them to remove their contact lenses, if relevant.

• Gently depress the lower eyelid while the patient looks up.

• Lightly touch the lateral edge of the cornea with a wisp of

damp cotton wool (Fig. 7.7).

• Look for both direct and consensual blinking.

Fig. 7.7 Testing the corneal reflex. The cotton-wool wisp should touch

the cornea overlying the iris, not the conjunctiva, and avoid visual stimulus.

Fig. 7.8 Eliciting the jaw jerk.

7.5 Comparison of bulbar and pseudobulbar palsy

Bulbar palsy Pseudobulbar palsy

Level of motor lesion Lower motor

neurone

Upper motor neurone

Speech Dysarthria Dysarthria and dysphonia

Swallowing Dysphagia Dysphagia

Tongue Weak, wasted

and fasciculating

Spastic, slow-moving

Jaw jerk Absent Present/brisk

Emotional lability Absent May be present

Causes Motor neurone

disease

Cerebrovascular disease,

motor neurone disease,

multiple sclerosis

130 • The nervous system

from the lateral pontomedullary junction in close association with

the VIII nerve (Fig. 7.11); together they enter the internal acoustic

meatus (see Fig. 7.5). At the lateral end of the meatus the VII

nerve continues in the facial canal within the temporal bone,

exiting the skull via the stylomastoid foramen. Passing through

the parotid gland, it gives off its terminal branches. In its course

in the facial canal it gives off branches to the stapedius muscle

and its parasympathetic fibres, as well as being joined by the

taste fibres of the chorda tympani (see Fig. 7.10).

Examination sequence

Examination is usually confined to motor function; taste is

rarely tested.

Motor function

Inspect the face for asymmetry or differences in blinking or

eye closure on one side. Note that minor facial asymmetry

is common and rarely pathological.

• Watch for spontaneous or involuntary movement.

• For the following actions it is often easiest to demonstrate

the actions yourself and ask the patient to copy you,

observing for any asymmetry.

• Ask the patient to raise their eyebrows and observe for

symmetrical wrinkling of the forehead (frontalis muscle).

• Ask the patient to screw their eyes tightly shut and resist

you opening them (orbicularis oculi).

• Ask the patient to bare their teeth (orbicularis oris).

• Ask the patient to blow out their cheeks with their mouth

closed (buccinators and orbicularis oris).

Sensory symptoms include facial numbness and pain. Unilateral

loss of sensation in one or more branches of the V nerve may

result from direct injury in association with facial fractures

(particularly V2), local invasion by cancer or Sjögren’s syndrome.

Lesions in the cavernous sinus often cause loss of the corneal

reflex and V1 or V2 cutaneous sensory loss. Cranial nerves III, IV

and VI may also be involved (see Fig. 8.3). Trigeminal neuralgia

causes severe, lancinating pain, typically in the distribution of V2

or V3. Reactivation of herpes varicella zoster virus (chickenpox)

can affect any sensory nerve, but typically either V1 or a thoracic

dermatome (Fig. 7.9). In herpes zoster ophthalmicus (affecting V1)

there is a risk of sight-threatening complications. Hutchinson’s

sign, vesicles on the side or tip of the nose, may be present.

Clinically significant weakness of the muscles of mastication

is unusual but may occur in myasthenia gravis, with fatigable

chewing.

Facial (VII) nerve

The facial nerve supplies the muscles of facial expression (frontalis,

orbicularis oculi, buccinators, orbicularis oris and platysma) and

carries parasympathetic fibres to the lacrimal, submandibular and

sublingual salivary glands (via nervus intermedius). It receives

taste sensation from the anterior two-thirds of the tongue (via

the chorda tympani; Fig. 7.10).

Anatomy

From its motor nucleus in the lower pons, fibres of the VII nerve

pass back to loop around the VI nerve nucleus before emerging

A B C

D

Fig. 7.9 Herpes zoster. A The ophthalmic division of the left trigeminal (V) nerve is involved. B The maxillary division of the left V nerve. C Cervical

spinal root left C5. D Thoracic spinal root right T8.

Cranial nerves • 131

7

angle tumours (including acoustic neuroma), trauma and

parotid tumours. Synkinesis (involuntary muscle contraction

accompanying a voluntary movement: most commonly, twitching

of the corner of the mouth with ipsilateral blinking) is a sign of

aberrant reinnervation and may be seen in recovering lower

motor neurone VII lesions.

In unilateral VII nerve upper motor neurone lesions, weakness

is marked in the lower facial muscles with relative sparing of the

upper face. This is because there is bilateral cortical innervation

of the upper facial muscles. The nasolabial fold may be flattened

and the corner of the mouth drooped, but eye closure is

usually preserved (Fig. 7.12B). Hemifacial spasm presents with

synchronised twitching of the ipsilateral eye and mouth.

Bilateral facial palsies are less common, but occasionally occur,

as in Guillain–Barré syndrome, sarcoidosis, or infection such as

Lyme disease, HIV or leprosy. Facial weakness, especially with

respect to eye closure, can also be found in some congenital

myopathies (facioscapulohumeral or myotonic dystrophies).

Distinct from VII nerve palsies, Parkinson’s disease can cause loss

of spontaneous facial movements, including a slowed blink rate,

and involuntary facial movements (levodopa-induced dyskinesias)

may complicate advanced disease.

Involuntary emotional movements, such as spontaneous

smiling, have different pathways and may be preserved in the

presence of paresis.

Vestibulocochlear (VIII) nerve

See page 173.

Glossopharyngeal (IX) and vagus (X) nerves

The IX and X nerves have an intimate anatomical relationship.

Both contain sensory, motor and autonomic components. The

glossopharyngeal (IX) nerve mainly carries sensation from the

pharynx and tonsils, and sensation and taste from the posterior

one-third of the tongue. The IX nerve also supplies the carotid

chemoreceptors. The vagus (X) nerve carries important sensory

In a unilateral lower motor neurone VII nerve lesion, there is

weakness of both upper and lower facial muscles. Bell’s palsy

is the term used to describe an idiopathic acute lower motor

neurone VII nerve paralysis, often preceded by mastoid pain. It

may be associated with impairment of taste and hyperacusis

(high-pitched sounds appearing unpleasantly louder than normal).

Bell’s phenomenon occurs when a patient closes their eyes:

as eye closure is incomplete the globe can be seen to roll

upwards, to avoid corneal exposure (Fig. 7.12A). Ramsay Hunt

syndrome occurs in herpes zoster infection of the geniculate (facial)

ganglion. This produces a severe lower motor neurone facial

palsy, ipsilateral loss of taste and buccal ulceration, and a painful

vesicular eruption in the external auditory meatus. Other causes

of a lower motor neurone VII lesion include cerebellopontine

Submandibular ganglion

Stylomastoid foramen

Nucleus solitarius

Abducens nucleus

Superior salivatory

nucleus

Pterygopalatine

ganglion

Geniculate ganglion

Chorda

tympani

To facial muscles

To stapedius

muscle

Palate

Lacrimal gland

Anterior two-thirds

of the tongue

Sublingual

gland

Submandibular

gland

Mucous mambranes

of nasal and oral

cavities

Fourth ventricle

Facial nucleus

= Motor fibres

= Sensory

= Parasympathetic

Fig. 7.10 Component fibres of the facial nerve and their peripheral distribution.

Fig. 7.11 Lesions of the pons. Lesions at (1) may result in ipsilateral VI

and VII nerve palsies and contralateral hemiplegia. At (2) ipsilateral

cerebellar signs and impaired sensation on the ipsilateral side of the face

and on the contralateral side of the body may occur.

132 • The nervous system

Isolated unilateral IX nerve lesions are rare. Unilateral X nerve

damage leads to ipsilateral reduced elevation of the soft palate,

which may cause deviation of the uvula (away from the side of

the lesion) when the patient says ‘Ah’. Unilateral lesions of IX and

X are most commonly caused by strokes, skull-base fractures or

tumours. Damage to the recurrent laryngeal branch of the X nerve

due to lung cancer, thyroid surgery, mediastinal tumours and

aortic arch aneurysms causes dysphonia and a ‘bovine’ cough.

Bilateral X nerve lesions cause dysphagia and dysarthria, and may

be due to lesions at the upper motor neurone level (pseudobulbar

palsy) or lower motor neurone level (bulbar palsy; see Box 7.5).

Less severe cases can result in nasal regurgitation of fluids and

nasal air escape when the cheeks are puffed out (dysarthria and

nasal escape are often evident during history taking). Always

consider myasthenia gravis in patients with symptoms of bulbar

dysfunction, even if the examination seems normal.

information but also innervates upper pharyngeal and laryngeal

muscles. The main functions of IX and X that can be tested

clinically are swallowing, phonation/articulation and sensation

from the pharynx/larynx. In the thorax and abdomen, the vagus

(X) nerve receives sensory fibres from the lungs and carries

parasympathetic fibres to the lungs, heart and abdominal viscera.

Anatomy

Both nerves arise as several roots from the lateral medulla and

leave the skull together via the jugular foramen (see Fig. 7.5). The

IX nerve passes down and forwards to supply the stylopharyngeus

muscle, the mucosa of the pharynx, the tonsils and the posterior

one-third of the tongue, and sends parasympathetic fibres to the

parotid gland. The X nerve courses down in the carotid sheath

into the thorax, giving off several branches, including pharyngeal

and recurrent laryngeal branches, which provide motor supply

to the pharyngeal, soft palate and laryngeal muscles. The main

nuclei of these nerves in the medulla are the nucleus ambiguus

(motor), the dorsal motor vagal nucleus (parasympathetic) and

the solitary nucleus (visceral sensation; Fig. 7.13).

Examination sequence

• Assess the patient’s speech for dysarthria or dysphonia

(p. 125).

• Ask them to say ‘Ah’. Look at the movements of the

palate and uvula using a torch. Normally, both sides of the

palate elevate symmetrically and the uvula remains in the

midline.

• Ask the patient to puff out their cheeks with their lips

tightly closed. Listen for air escaping from the nose. For

the cheeks to puff out, the palate must elevate and

occlude the nasopharynx. If palatal movement is weak, air

will escape audibly through the nose.

• Ask the patient to cough; assess the strength of the

cough.

• Testing pharyngeal sensation and the gag reflex is

unpleasant and has poor predictive value for aspiration.

Instead, and in fully conscious patients only, use the

swallow test. Administer 3 teaspoons of water and

observe for absent swallow, cough or delayed cough, or

change in voice quality after each teaspoon. If there are no

problems, observe again while the patient swallows a

glass of water.

Preserved function

in upper face

Loss of

nasolabial fold

Mouth deviates

to normal side

A B

Loss of frontal

wrinkling

Loss of

nasolabial fold

Mouth deviates

to normal side

Bell’s phenomenon

Fig. 7.12 Types of facial weakness. A Right-sided lower motor neurone lesion (within facial nerve or nucleus); Bell’s phenomenon is also shown.

B Right-sided upper motor neurone lesion.

Glossopharyngeal

Sensory to pharynx

Motor to stylopharyngeus

Taste from posterior one-third

of tongue

Pharyngeal nerve

Elevators of palate and

closure of nasopharynx

Peristaltic movement of

constrictor muscles

(superior middle

and inferior)

Recurrent laryngeal

nerve

Motor to all the

intrinsic muscles

of the larynx

Spinal part of

accessory nerve

Motor to sternomastoid and

trapezius muscles

Jugular

foramen

PONS

MEDULLA IX X XI

XI

Superior laryngeal

nerve

Vagus

nerve

Internal

External

Fig. 7.13 The lower cranial nerves: glossopharyngeal (IX), vagus (X)

and accessory (XI).

Motor system • 133

7

• Ask the patient to put out their tongue. Look for deviation

or involuntary movement.

• Ask the patient to move their tongue quickly from side to

side.

• Test power by asking the patient to press their tongue

against the inside of each cheek in turn while you press

from the outside with your finger.

• Assess speech by asking the patient to say ‘yellow lorry’.

• Assess swallowing with a water swallow test (p. 132).

Unilateral lower motor XII nerve lesions lead to tongue wasting

on the affected side and deviation to that side on protrusion (Fig.

7.14). Bilateral lower motor neurone damage results in global

wasting, the tongue appears thin and shrunken and fasciculation

may be evident. Normal rippling or undulating movements may be

mistaken for fasciculation, especially if the tongue is protruded;

these usually settle when the tongue is at rest in the mouth.

When associated with lesions of the IX, X and XI nerves, typically

in motor neurone disease, these features are termed bulbar

palsy (see Box 7.5).

Unilateral upper motor XII nerve lesions are uncommon; bilateral

lesions lead to a tongue with increased tone (spastic) and the

patient has difficulty flicking the tongue from side to side. Bilateral

upper motor lesions of the IX–XII nerves are called pseudobulbar

palsy (see Box 7.5). Tremor of the resting or protruded tongue

may occur in Parkinson’s disease, although jaw tremor is more

common. Other orolingual dyskinesias (involuntary movements

of the mouth and tongue) are often drug-induced and include

tardive dyskinesias due to neuroleptics.

Motor system

Anatomy

The principal motor pathway has CNS (corticospinal or pyramidal

tract – upper motor neurone) and PNS (anterior horn cell –

lower motor neurone) components (Fig. 7.15). Other parts of

Accessory (XI) nerve

The accessory nerve has two components:

a cranial part closely related to the vagus (X) nerve

a spinal part that provides fibres to the upper trapezius

muscles, responsible for elevating (shrugging) the

shoulders and elevation of the arm above the horizontal,

and the sternomastoid muscles that control head turning

and flexing the neck.

The spinal component is discussed here.

Anatomy

The spinal nuclei arise from the anterior horn cells of C1–5.

Fibres emerge from the spinal cord, ascend through the foramen

magnum and exit via the jugular foramen (see Fig. 7.5), passing

posteriorly.

Examination sequence

• Face the patient and inspect the sternomastoid muscles

for wasting or hypertrophy; palpate them to assess their

bulk.

• Stand behind the patient to inspect the trapezius muscle

for wasting or asymmetry.

• Ask the patient to shrug their shoulders, then apply

downward pressure with your hands to assess the

power.

• Test power in the left sternomastoid by asking the patient

to turn their head to the right while you provide resistance

with your hand placed on the right side of the patient’s

chin. Reverse the procedure to check the right

sternomastoid.

• Test both sternocleidomastoid muscles simultaneously by

asking the patient to flex their neck. Apply your palm to

the forehead as resistance.

Isolated XI nerve lesions are uncommon but the nerve may

be damaged during surgery in the posterior triangle of the neck,

penetrating injuries or tumour invasion. Wasting of the upper fibres

of trapezius may be associated with displacement (‘winging’) of the

upper vertebral border of the scapula away from the spine, while

the lower border is displaced towards it. Wasting and weakness

of the sternomastoids are characteristic of myotonic dystrophy.

Weakness of neck flexion or extension, the latter causing head

drop, may occur in myasthenia gravis, motor neurone disease

and some myopathies. Dystonic head postures causing antecollis

(neck flexed), retrocollis (neck extended) or torticollis (neck twisted

to one side) are not associated with weakness.

Hypoglossal (XII) nerve

The XII nerve innervates the tongue muscles; the nucleus lies

in the dorsal medulla beneath the floor of the fourth ventricle.

Anatomy

The nerve emerges anteriorly and exits the skull in the hypoglossal

canal, passing to the root of the tongue (see Fig. 7.5).

Examination sequence

• Ask the patient to open their mouth. Look at the tongue at

rest for wasting, fasciculation or involuntary movement.

Fig. 7.14 Left hypoglossal nerve lesion. From Epstein O, Perkin GD, de

Bono DP, et al. Clinical Examination. 2nd edn. London: Mosby; 1997.

134 • The nervous system

The group of muscle fibres innervated by a single anterior

horn cell forms a ‘motor unit’. A lower motor neurone lesion

causes weakness and wasting in these muscle fibres, reduced

tone (flaccidity), fasciculation and reduced or absent reflexes.

Basal ganglia lesions

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