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nopompic or postdormital) was introduced by Kinnier Wilson in 1928 Actually, Weir Mitchell had described this latter disorder in 1876, under the title of night palsy Sometimes sleep paralysis is accompanied or just preceded by vivid and terrifying hallucinations (hypnagogic hallucinations), which may be visual, auditory, vestibular (a sense of motion), or somatic (a feeling that a limb or nger or other part of the body is enlarged or otherwise transformed) The association of hypnagogic hallucinations with narcolepsy was rst noted by Lhermitte and Tournay in 1927 These four conditions narcolepsy, cataplexy, hypnagogic paralysis, and hallucinations constitute a clinical tetrad Wilson has reviewed the historical aspects and early accounts of this subject The most important observations regarding the pathophysiology of this process have been the special relationship to a disordered pattern of REM sleep and the more recent nding of abnormalities in hypothalamic substances that induce sleep, as discussed below Clinical Features This syndrome is not infrequent, as shown by the fact that Daly and Yoss recorded the occurrence of about 100 new cases a year at the Mayo Clinic Dement and colleagues have estimated the prevalence at 50 to 70 per 100,000 in the San Francisco and Los Angeles areas Men and women are affected about equally As a rule, narcolepsy has a gradual onset between the ages of 15 and 35 years; in fully 90 percent of narcoleptics, the condition is established by the 25th year Narcolepsy is usually the rst symptom, less often cataplexy, and rarely sleep paralysis The essential disorder is one of frequent attacks of sleepiness, which are irresistible Several times a day, usually after meals or while sitting in class or in other boring or sedentary situations, the affected person is assailed by an uncontrollable desire to sleep The eyes close, the muscles relax, breathing deepens slightly, and by all appearances the individual is dozing A noise, a touch, or even the cessation of the lecturer s voice is enough to awaken the patient The periods of sleep rarely last longer than 15 min unless the patient is reclining, when he may continue to sleep for an hour or longer At the conclusion of a nap, the patient feels somewhat refreshed It should be emphasized that there are many narcoleptics who tend to be pervasively drowsy throughout the day What distinguishes the typical narcoleptic sleep attacks from commonplace postprandial drowsiness is the frequent occurrence of the former (two to six times every day, as a rule), their irresistibility, and their occurrence in unusual situations, as while standing, eating, or carrying on a conversation Blurring of vision, diplopia, and ptosis may attend the drowsiness and may bring the patient rst to an ophthalmologist It is not generally appreciated that, in addition to episodes of outright sleep, narcoleptics, like other very drowsy persons, may experience episodes of automatic behavior and amnesia These episodes may last for a few seconds or as long as an hour or more and occur more often in the afternoon and evening than in the morning, usually when the patient is alone and performing some monotonous task, such as driving Initially the patient feels drowsy and may recall attempts to ght off the drowsiness, but gradually he loses track of what is going on The patient may continue to perform routine tasks automatically but does not respond appropriately to a new demand or answer complex questions Often there is a sudden burst of words, without meaning or relevance to what was just said Such an outburst may terminate the attack, for which there is complete or nearly complete amnesia In many respects the attacks resemble episodes of nocturnal sleepwalking Such attacks of automatic behavior and amnesia are common, occurring in more.

Nerve biopsies are processed for study by conventional histologic methods and by thin-section phase and electron microscopy These methods, sometimes supplemented by study of teased ber preparations and immunologic studies, can provide valuable histopathologic data With ordinary microscopy and staining, one may nd evidence of focal in ammation, vasculitis, amyloidosis, leprosy, or sarcoid These procedures are most valuable in the diagnosis of

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