Trigeminal Neuralgia (Tic Douloureux)

Frequent, excruciating paroxysms of pain in lips, gums, cheek, or chin (rarely in ophthalmic division of fifth nerve) lasting seconds to minutes. Typically presents in middle or old age. Pain is often stimulated at trigger points. Sensory deficit cannot be demonstrated. Must be distinguished from other forms of facial pain arising from diseases of jaw, teeth, or sinuses. Pain from migraine or cluster headache tends to be deep-seated and steady, unlike the superficial stabbing quality of trigeminal neuralgia. In temporal arteritis, superficial facial pain is not shock-like, pt frequently complains of myalgias and other systemic symptoms, and an elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) is usually present. Rare causes are herpes zoster or a tumor. An onset in young adulthood or if bilateral raises the possibility of multiple sclerosis (MS) (Chap. 192 Multiple Sclerosis).

Trigeminal Neuropathy

Usually presents as facial sensory loss or weakness of jaw muscles. Causes are varied (Table 190-1 Trigeminal Nerve Disorders), including tumors of middle cranial fossa or trigeminal nerve, metastases to base of skull, or lesions in cavernous sinus (affecting first and second divisions of fifth nerve) or superior orbital fissure (affecting first division of fifth nerve).

Bell's Palsy

Most common form of idiopathic facial paralysis; affects 1 in 60 persons over a lifetime. Association with herpes simplex virus type 1. Risk factors include pregnancy and diabetes mellitus. Weakness evolves gradually with maximal weakness by 48 h, sometimes preceded by retroaural pain. Hyperacusis may be present. Full recovery within several weeks or months in 80%; incomplete paralysis in first week is the most favorable prognostic sign.

Diagnosis can be made clinically in pts with (1) a typical presentation, (2) no risk factors or preexisting symptoms for other causes of facial paralysis, (3) no lesions of herpes zoster in the external ear canal, and (4) a normal neurologic examination with the exception of the facial nerve. In uncertain cases, an ESR or CRP, testing for diabetes mellitus, a Lyme titer, angiotensin-converting enzyme level and chest imaging study for possible sarcoidosis, a lumbar puncture for possible Guillain-Barré syndrome, or MRI scanning may be indicated.

Other Facial Nerve Disorders

Ramsay Hunt syndrome is caused by herpes zoster infection of geniculate ganglion; distinguished from Bell's palsy by a vesicular eruption in pharynx and external auditory canal, and by frequent involvement of eighth cranial nerve. Acoustic neuromas often compress the seventh nerve. Infarcts, demyelinating lesions of multiple sclerosis, and tumors are common pontine causes. Bilateral facial weakness may occur in Guillain-Barré syndrome, sarcoidosis, Lyme disease, and leprosy. Hemifacial spasm may occur with Bell's palsy, compression and/or demyelination of the nerve by tumor, infection or MS, or as an idiopathic disorder. Blepharospasm consists of involuntary recurrent spasms of both eyelids, usually occurring in the elderly and sometimes with associated facial spasm; may subside spontaneously. Hemifacial spasm or blepharospasm can be treated by injection of botulinum toxin into the orbicularis oculi.

Disorders of the Sense of Smell

Olfactory nerve (I) disorders are due to interference with access of the odorant to the olfactory neuroepithelium (transport loss), injury to receptor region (sensory loss), or damage to central olfactory pathways (neural loss). The causes of olfactory disorders are summarized in Table 190-2 Disorders and Conditions Associated with Compromised Olfactory Function, as Measured by Olfactory Testing; most common other than aging are severe upper respiratory infections, head trauma, and chronic rhinosinusitis. Although more than half of people between 65 and 80 years of age suffer from olfactory dysfunction that is idiopathic (presbyosmia), it is increasingly recognized that a number of neurodegenerative disorders are accompanied by olfactory impairment. Pts often present with a complaint of loss of the sense of taste even though their taste thresholds may be within normal limits.

Glossopharyngeal Neuralgia

This neuralgia involves the ninth (glossopharyngeal) and sometimes portions of the tenth (vagus) cranial nerves. Presents with paroxysmal, intense pain in tonsillar fossa of throat that may be precipitated by swallowing. There is no objective sensory or motor deficit. Other diseases affecting this nerve include herpes zoster, MS, or compressive neuropathy due to tumor or aneurysm in region of jugular foramen (when associated with vagus and accessory nerve palsies).

Dysphagia and Dysphonia

Lesions of the vagus nerve (X) may be responsible. Unilateral lesions produce drooping of soft palate, loss of gag reflex, and “curtain movement” of lateral wall of pharynx with hoarse, nasal voice. Etiologies include neoplastic and infectious processes of the meninges, tumors and vascular lesions in the medulla, motor neuron disease (e.g., ALS), or compression of the recurrent laryngeal nerve by intrathoracic processes. Aneurysm of the aortic arch, an enlarged left atrium, and tumors of the mediastinum and bronchi are much more frequent causes of an isolated vocal cord palsy than are intracranial disorders. A substantial number of cases of recurrent laryngeal palsy remain idiopathic.

With laryngeal palsy, first determine the site of the lesion. If intramedullary, there are usually other brainstem or cerebellar signs. If extramedullary, the glossopharyngeal (IX) and spinal accessory (XI) nerves are frequently involved (jugular foramen syndrome). If extracranial in the posterior laterocondylar or retroparotid space, there may be combinations of ninth, tenth, eleventh, and twelfth cranial nerve palsies and a Horner's syndrome. If there is no sensory loss over the palate and pharynx and no palatal weakness or dysphagia, lesion is below the origin of the pharyngeal branches, which leave the vagus nerve high in the cervical region; the usual site of disease is then the mediastinum.

Neck Weakness

Isolated involvement of the accessory (XI) nerve can occur anywhere along its route, resulting in paralysis of the sternocleidomastoid and trapezius muscles. More commonly, involvement occurs in combination with deficits of the ninth and tenth cranial nerves in the jugular foramen or after exit from the skull. An idiopathic form of accessory neuropathy, similar to Bell's palsy, has been described; most pts recover but it may recur.

Tongue Paralysis

The hypoglossal (XII) nerve supplies the ipsilateral muscles of the tongue. The nucleus of the nerve or its fibers of exit may be involved by intramedullary lesions such as tumor, poliomyelitis, or most often motor neuron disease. Lesions of the basal meninges and the occipital bones (platybasia, invagination of occipital condyles, Paget's disease) may compress the nerve in its extramedullary course or in the hypoglossal canal. Isolated lesions of unknown cause can occur. Atrophy and fasciculation of the tongue develop weeks to months after interruption of the nerve.