title: Familial Kleine-Levin syndrome: two siblings with unusually long hypersomnic spells.
abstract: Kleine-Levin syndrome is a rare, sporadic disorder, with discrete spells of hypersomnolence occurring during adolescence, variously accompanied by megaphagia, behavioral changes, psychosis, and mild autonomic symptoms. Familial cases have not previously been reported. We describe 2 siblings who shared uncharacteristically prolonged episodes of hypersomnolence, and the HLA-DR2 haplotype. In one patient, levels of cerebrospinal fluid orexin (hypocretin) during an attack were normal. The presence of an increased sleep drive, despite the occurrence of large amounts of ostensibly restorative sleep, suggests the possible existence of a disorder of sleep satiety.
authors.affiliation: Neurology Service, St Elizabeth's Medical Center, Tufts University School of Medicine, Boston, MA 02135, USA. neuron@massmed.org
authors.forename: Joshua D
authors.initials: JD
authors.lastname: Katz
authors.forename: Allan H
authors.initials: AH
authors.lastname: Ropper
mesh_terms: D000293:Adolescent; D006970:Disorders of Excessive Somnolence / Q000134:cerebrospinal fluid / Q000235:genetics / Q000503:physiopathology; D005260:Female; D015802:HLA-DR2 Antigen / Q000235:genetics; D006801:Humans; D017593:Kleine-Levin Syndrome / Q000134:cerebrospinal fluid / Q000235:genetics* / Q000503:physiopathology*; D008297:Male; D035781:Siblings
chemical_list: D015802:HLA-DR2 Antigen