Chapter 376

Symptoms and signs of cranial nerve pathology are common in internal medicine. They often develop in the context of a widespread neurologic disturbance, and in such situations cranial nerve involvement may represent the initial manifestation of the illness. In other disorders, involvement is largely restricted to one or several cranial nerves; these distinctive disorders are reviewed in this chapter. Disorders of ocular movement are discussed in Chap. 28, disorders of hearing in Chap. 30, and vertigo and disorders of vestibular function in Chap. 21.

### Anatomic Considerations

The trigeminal (fifth cranial) nerve supplies sensation to the skin of the face and anterior half of the head (Fig. 376-1). Its motor part innervates the masseter and pterygoid masticatory muscles.

###### Figure 376-1

The three major sensory divisions of the trigeminal nerve consist of the ophthalmic, maxillary, and mandibular nerves.

### Trigeminal Neuralgia (Tic Douloureux)

#### Clinical Manifestations

Trigeminal neuralgia is characterized by excruciating paroxysms of pain in the lips, gums, cheek, or chin and, very rarely, in the distribution of the ophthalmic division of the fifth nerve. The pain seldom lasts more than a few seconds or a minute or two but may be so intense that the patient winces, hence the term tic. The paroxysms, experienced as single jabs or clusters, tend to recur frequently, both day and night, for several weeks at a time. They may occur spontaneously or with movements of affected areas evoked by speaking, chewing, or smiling. Another characteristic feature is the presence of trigger zones, typically on the face, lips, or tongue, that provoke attacks; patients may report that tactile stimuli—e.g., washing the face, brushing the teeth, or exposure to a draft of air—generate excruciating pain. An essential feature of trigeminal neuralgia is that objective signs of sensory loss cannot be demonstrated on examination.

Trigeminal neuralgia is relatively common, with an estimated annual incidence of 4.5 per 100,000 individuals. Middle-aged and elderly persons are affected primarily, and ˜60% of cases occur in women. Onset is typically sudden, and bouts tend to persist for weeks or months before remitting spontaneously. Remissions may be long-lasting, but in most patients the disorder ultimately recurs.

#### Pathophysiology

Symptoms result from ectopic generation of action potentials in pain-sensitive afferent fibers of the fifth cranial nerve root just before it enters the lateral surface of the pons. Compression or other pathology in the nerve leads to demyelination of large myelinated fibers that do not themselves carry pain sensation but become hyperexcitable and electrically coupled with smaller unmyelinated or poorly myelinated pain fibers in close proximity; this may explain why tactile stimuli, conveyed via the large myelinated fibers, can stimulate paroxysms of pain. Compression of the trigeminal nerve root by a blood ...

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