INSTRUCTION

Examine this patient's cranial nerves.

SALIENT FEATURES

History

The clinical presentation consists of symptoms of impaired function of the last four cranial nerves:· Hoarseness of voice.· Nasal quality to the speech.· Nasal regurgitation and dysphagia.· Aspiration of food with choking attacks.· Weakness of the sternomastoids and trapezii.· Wasting of the tongue (often noticed by the dentist).· Pain in and around the ear (due to damage of the ninth and tenth cranial nerves which carry sensation to the external auditorymeatus and behind the ear).· Headache.· Ptosis (due to Homer's syndrome).

Examination

· Sluggish movement of the palate when the patient says 'aah' on the affected side.· Absent gag reflex on the same side.· Flattening of the shoulder on the same side.· Wasting of the sternomastoid.· Weakness when the patient moves her chin to the opposite side.· Difficulty in shrugging the shoulder on the same side.

Proceed as follows:

· Look for wasting and deviation of the tongue (twelfth cranial nerve palsy).· Tell the examiner that you would like to check for two signs: - Bovine cough. -Husky voice.

DIAGNOSIS

This patient has features of jugular foramen syndrome (lesion) which could be due to several causes including pharyngeal neoplasm(aetiology); she has difficulty in swallowing and requires a nasogastric tube (functional status).

ADVANCED-LEVEL QUESTIONS

Where is the jugular foramen located?

Between the lateral part of the occipital bone and the petrous portion of the temporal bones.

Which cranial nerves leave the skull through the jugular foramen?The ninth, tenth and eleventh cranial nerves.

Through which foramen does the twelfth cranial nerve leave the skull?

The anterior condylar foramen.

Mention a few causes of jugular foramen syndrome.

· Carcinoma of the pharynx is the commonest cause.· Fractured base of the skull.· Paget's disease.· Basal meningitis.· Neurofibroma or any tumour.· Thrombosis of jugular vein.

Do you know of any eponymous syndromes of the lower cranial nerves ?

· Vernet's syndrome: paresis of the ninth, tenth and eleventh cranial nerves due to extension of tumour into the jugular foramen.· Collet-Sicard syndrome: fracture of the floor of the posterior cranial fossa, causing palsy of the last four cranial nerves.· Villaret's syndrome: ipsilateral paralysis of the last four cranial nerves and cervical sympathetics.· Syndrome of Schmidt: vagus and accessory nerve involvement.· Syndrome of Hughlings Jackson: accessory and hypoglossal nerve involvement.

What is the cause of unilateral eleventh cranial nerve palsy?

· Trauma to the nerve in the neck.· In hemiplegia.

How would you test for eleventh cranial nerve palsy?

· Sternocleidomastoids are tested by having the patient turn her head forcibly against the examiner's hand in a direction awayfrom the muscle being tested while the muscle is observed and palpated.· Upper portion of the trapezii is tested by having the patient forcibly elevate (shrug) her shoulder while the examiner attempts todepress the shoulder.M. Vernet (b. 1887), a French neurologist, described this syndrome in 1916.M. Villaret (1877-1946), Professor of Neurology in France, described this syndrome in 1918.RJ. Collet (b. 1870), a French otolaryngologist, described his patient in 1915.J.A. Sicard (1872-1929), a French physician and radiologist, was the first to perform myelography, use alcohol in trigeminalneuralgia and inject a sclerosing substance in varicose veins. He described his patient in 1917.