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Monozygotic twins concordant for Kleine-Levin syndrome. 2012

Taro Ueno, and Akira Fukuhara, and Azusa Ikegami, and Fumihiro Ohishi, and Kazuhiko Kume
Institute of Molecular Embryology and Genetics, Kumamoto University, Honjo, Kumamoto, 860-0811, Japan.

BACKGROUND Kleine-Levin syndrome is a rare sleep disorder of unknown etiology. It is characterized by intermittent periods of excessive sleepiness, cognitive disturbances and behavioral abnormalities. Nine cases of familial Kleine-Levin syndrome have been identified, but there are no reported cases describing twins that are affected by the syndrome. METHODS We report the cases of 16-year-old monozygotic twin boys who both suffered from Kleine-Levin syndrome. In both cases, the onset of the first episode was preceded by an influenza infection. During symptomatic periods they slept for the entire day except for meals and bathroom visits. Actimetry recordings revealed that during symptomatic periods, daily activity was lower than that of asymptomatic periods, on the other hand, activity during the night was significantly higher in symptomatic periods than asymptomatic periods. Polysomnography (PSG) data during symptomatic periods revealed a decrease in sleep efficiency. Human leukocyte antigen (HLA) typing revealed no DQB1*02 loci. They were administered lithium carbonate but the beneficial effect was limited. CONCLUSIONS Our observations suggest that Kleine-Levin syndrome may be due to genetic and autoimmune processes, although etiologic relationship to specific HLA type remains controversial.

UI MeSH Term Description Entries
D008297 Male Males
D004200 Diseases in Twins Disorders affecting TWINS, one or both, at any age. Diseases in Twin,Twin, Diseases in,Twins, Diseases in,in Twin, Diseases,in Twins, Diseases
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D014430 Twins, Monozygotic Two off-spring from the same PREGNANCY. They are from a single fertilized OVUM that split into two EMBRYOS. Such twins are usually genetically identical and of the same sex. Twins, Identical,Identical Twin,Identical Twins,Monozygotic Twin,Monozygotic Twins,Twin, Identical,Twin, Monozygotic
D017593 Kleine-Levin Syndrome A rare condition characterized by recurrent hypersomnias associated with hyperphagia, occurring primarily in males in the second to third decade of life. Clinical features include mental confusion, excessive sleep requirements (approximately 18 hours per day), restlessness, and in some cases hallucinations. Episodes have a duration of days to weeks, and may recur several times per year. This condition may resolve spontaneously over several years. (From Adams, et al., Principles of Neurology, 6th ed, p569) Hypersomnia, Periodic,Familial Hibernation (Kleine-Levin) Syndrome,Familial Kleine-Levin Syndrome,Kleine-Levin Hibernation Syndrome,Kleine-Levin-Critchley Syndrome,Familial Kleine Levin Syndrome,Hibernation Syndrome, Kleine-Levin,Kleine Levin Critchley Syndrome,Kleine Levin Hibernation Syndrome,Kleine Levin Syndrome,Kleine-Levin Syndrome, Familial,Periodic Hypersomnia,Periodic Hypersomnias,Syndrome, Familial Kleine-Levin,Syndrome, Kleine-Levin,Syndrome, Kleine-Levin Hibernation,Syndrome, Kleine-Levin-Critchley

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