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Intermittent disorder affecting the behavior during adolescence characterized by hypersomnolence (up to 20 hours/day), excessive food intake, and abnormally uninhibited sexual drive.

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Critchley Syndrome; Familial Hibernation Syndrome; Hypersomnia-Bulimia Syndrome; Kleine-Levin-Critchley Syndrome; Periodic Somnolence and Morbid Hunger Syndrome.

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Approximately 200 cases have been reported. About 2/3 of patients are males. Autosomal dominant inheritance. Human leukocyte antigen (HLA)-DQB1*0201 allele frequency was significantly increased in patients with Kleine-Levin syndrome.

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Unknown. Hypotheses include a hypothalamic dysfunction and abnormalities in the central serotonin and dopamine metabolism. Several clinical symptoms also suggest an underlying autoimmune process.

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Episodic attacks of aberrant behavior with normal behavior between episodes. Attacks may last for several weeks and are characterized by hypersomnia, hyperphagia with subsequent vomiting, hallucinations with disorientation, increased sexual drive, and mood depression. Attacks may be precipitated by infections, head trauma, or alcohol consumption.

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Repeated episodes of bulimia may lead to electrolyte disorders. Mallory-Weiss tears can result from repetitive vomiting, which may lead to anemia from chronic upper gastrointestinal blood loss.

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These patients may be treated with amphetamines or lithium. Their interference with anesthetic drugs should be kept in mind.

Arnulf I, Zeitzer JM, File J, et al: Kleine-Levin syndrome: A systematic review of 186 cases in the literature. Brain 128:2763, 2005.  [PubMed: 16230322]
Dauvilliers Y, Mayer G, Lecendreux M, et al: Kleine-Levin syndrome: An autoimmune hypothesis based on clinical and genetic analyses. Neurology 59:1739, 2002.  [PubMed: 12473762]
Gadoth N, Kesler A, Vainstein G, et al: Clinical and polysomnographic characteristics of 34 patients with Kleine-Levin syndrome. J Sleep Res 10:337, 2001.  [PubMed: 11903864]

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