PMID- 20404354 OWN - NLM STAT- MEDLINE DCOM- 20110225 LR - 20101115 IS - 1708-8283 (Electronic) IS - 0883-0738 (Linking) VI - 25 IP - 11 DP - 2010 Nov TI - Familial recurrent hypersomnia: two siblings with Kleine-Levin syndrome and menstrual-related hypersomnia. PG - 1408-10 LID - 10.1177/0883073810366599 [doi] AB - Kleine-Levin syndrome and menstrual-related hypersomnia are rare idiopathic sleep disorders occurring primarily in adolescence. They are characterized by intermittent periods of excessive sleepiness, cognitive disturbances, and behavioral abnormalities. In both, the etiology remains unknown but autoinmune, hormonal, infectious, and inflammatory mechanisms have been proposed. The authors describe, for the first time, the association of Kleine-Levin syndrome and menstrual-related hypersomnia in 2 adolescent siblings who shared the human leukocyte antigen (HLA) loci DQB1*0501. The same haplotype has been associated with sleepwalking and with rapid eye movement (REM) sleep behavior disorder. This gender differences in the manifestation of a probably genetic influenced sleep disorder suggests that hormonal mechanisms could be implicated in the phenotypical expression of this sleep disorder. The male sibling with Kleine-Levin syndrome was easily controlled with carbamazepine in low doses, but his sister could be only efficaciously treated with oral contraceptives. FAU - Rocamora, Rodrigo AU - Rocamora R AD - Department of Neurology, Hospital del Mar, Barcelona, Spain. rrocamora@imas.imim.es FAU - Gil-Nagel, Antonio AU - Gil-Nagel A FAU - Franch, Oriol AU - Franch O FAU - Vela-Bueno, Antonio AU - Vela-Bueno A LA - eng PT - Case Reports PT - Journal Article DEP - 20100419 PL - United States TA - J Child Neurol JT - Journal of child neurology JID - 8606714 SB - IM MH - Adolescent MH - Disorders of Excessive Somnolence/*genetics MH - Female MH - Humans MH - Male MH - *Menstrual Cycle MH - Siblings EDAT- 2010/04/21 06:00 MHDA- 2011/02/26 06:00 CRDT- 2010/04/21 06:00 PHST- 2010/04/21 06:00 [entrez] PHST- 2010/04/21 06:00 [pubmed] PHST- 2011/02/26 06:00 [medline] AID - 0883073810366599 [pii] AID - 10.1177/0883073810366599 [doi] PST - ppublish SO - J Child Neurol. 2010 Nov;25(11):1408-10. doi: 10.1177/0883073810366599. Epub 2010 Apr 19.