Intermittent disorder affecting the behavior during
adolescence characterized by hypersomnolence (up to 20 hours/day),
excessive food intake, and abnormally uninhibited sexual drive.
Critchley Syndrome; Familial Hibernation Syndrome;
Hypersomnia-Bulimia Syndrome; Kleine-Levin-Critchley Syndrome; Periodic
Somnolence and Morbid Hunger Syndrome.
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.
Unknown. Hypotheses include a hypothalamic
dysfunction and abnormalities in the central serotonin and dopamine
metabolism. Several clinical symptoms also suggest an underlying autoimmune
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.
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.
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
Dauvilliers Y, Mayer G, Lecendreux M, et al: Kleine-Levin syndrome: An
autoimmune hypothesis based on clinical and genetic analyses. Neurology
Gadoth N, Kesler A, Vainstein G, et al: Clinical and polysomnographic
characteristics of 34 patients with Kleine-Levin syndrome. J Sleep Res