Jugular foramen syndrome

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83 Jugular foramen syndrome

Instruction

Examine this patient’s cranial nerves.

Salient features

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 (Fig. 83.1)

• Weakness when the patient moves her chin to the opposite side

• Difficulty in shrugging the shoulder on the same side (Fig. 83.2).

Fig. 83.1 Wasting of the sternomastoid. (A) Normal left muscle with forceful thrust of chin to the right. (B) Movement to the left shows loss of muscle bulk.

(With permission from Bradley et al. 2008.)

Fig. 83.2 Left spinal accessory palsy with scapular winging upon attempted left arm abduction.

(With permission from Bradley et al. 2008.)

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) that could have 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

• Glomus jugulare paraganglioma (accounts for 80% of jugular foramen neoplasms)

• 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 syndrome: paresis of the ninth, tenth and eleventh cranial nerves caused by 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 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 the 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 the head forcibly against the examiner’s hand in a direction away from 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) the shoulder while the examiner attempts to depress the shoulder.

FJ Collet (b. 1870), a French otolaryngologist, described his patient in 1915.

M Vernet (1887–1974), a French neurologist, described this syndrome in 1916.

JA Sicard (1872–1929), a French physician and radiologist, was the first to perform myelography, used alcohol in trigeminal neuralgia and injected a sclerosing substance in varicose veins. He described his patient in 1917.

M Villaret (1877–1946), Professor of Neurology in France, described this syndrome in 1918.

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