In 1936 Levin(21) described a syndrome of periodic somnolence and morbid hunger occurring exclusively in males, starting in the second decade, and characterized by attacks of somnolence lasting several days or weeks, associated with abnormal behaviour, including overeating and sexual disinhibition, and various mental features such as irritability, mild confusion, incoherent speech, and at times hallucinations. In 1942 Critchley and Hoffmann (22) reported two more cases and coined the eponymous term Kleine-Levin syndrome.
Kleine-Levin syndrome is a rare condition; in our case statistics there were 19 subjects with this condition compared with 336 narcoleptics. Adolescent males are most commonly affected, but female cases are not unknown.
Excessive sleep develops either abruptly or gradually. The subject retires to his or her bedroom and almost refuses to leave it. Sleep is either calm or agitated. Abnormal behaviours, overeating, and sexual disinhibition are generally viewed as compulsive. Patients eat all food within sight, even if it is of poor quality. Manifestations of hypersexuality, indiscriminate sexual advances regardless of age and sex, and/or overt masturbation are reported in one-third of males and less often in females. Mental disturbances are of varying types. Irritability is the most frequent symptom, then a feeling of unreality, and less often confusion and/or visual or auditory hallucinations. The duration of episodes varies from 1 day to 3 to 4 weeks and the interval between episodes varies from less than a month to several months. Between episodes physical examination is normal. There is no consistent personality disorder.
Positive diagnosis of the Kleine-Levin syndrome is based on clinical features and not on laboratory investigations.
According to Critchley,(23) the natural history of the Kleine-Levin syndrome follows a decreasing course and attacks eventually cease. However, cases have been reported in which the symptoms have persisted for over 20 years.
Treatment includes symptomatic and preventive measures. The former are oriented towards cessation of the hypersomniac episodes, and rely on stimulant or awakening drugs, the effectiveness of which does not generally exceed a few hours or so. The latter have to be considered when hypersomniac episodes are frequent enough to disturb personal and family life. Positive results have been recorded with carbamazepine, valpromide, and lithium carbonate.
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