PMID- 9456468 OWN - NLM STAT- MEDLINE DCOM- 19980423 LR - 20190512 IS - 0161-8105 (Print) IS - 0161-8105 (Linking) VI - 20 IP - 11 DP - 1997 Nov TI - Familial aspects of narcolepsy-cataplexy in the Czech Republic. PG - 1021-6 AB - A group of 153 probands with narcolepsy included 38 subjects (24.8%) with a familial incidence of excessive daytime sleepiness (EDS). In 15 cases (9.8%), at least one additional family member suffered from narcolepsy-cataplexy; only EDS was present in the remaining 23 cases (15.0%). One thousand eighty-two relatives were evaluated. The percentage of first degree relatives affected with narcolepsy-cataplexy was 2.28% (1.20% if only clinically confirmed cases were accounted); the adequate value for second degree relatives was 1.49%. The occurrence of EDS exceeded these values several times (4.28% in first degree relatives, 6.57% in second degree relatives). The vertical mode of transmission was found in most families. Human leukocyte antigen (HLA) typing was performed in six families with multiple-case incidence of narcolepsy. Forty-one blood samples were analyzed (12 patients with narcolepsy, 7 with only EDS, 2 with sleep apnea syndrome, and 20 healthy relatives). HLA DR2+ and DQB1*0602+ were found in only 8 out of 12 narcoleptic patients with cataplexy and in six out of seven patients with isolated attacks of sleepiness. These findings support the hypothesis that there is a common genetic basis for narcolepsy associated with cataplexy and "monosymptomatic" forms of narcolepsy and suggest the existence of non-major histocompatibility complex (MHC) susceptibility factors for narcolepsy. FAU - Nevsimalova, S AU - Nevsimalova S AD - Department of Neurology 1st Medical Faculty, Charles University, Prague, Czech Republic. FAU - Mignot, E AU - Mignot E FAU - Sonka, K AU - Sonka K FAU - Arrigoni, J L AU - Arrigoni JL LA - eng GR - NS 23724/NS/NINDS NIH HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, P.H.S. PL - United States TA - Sleep JT - Sleep JID - 7809084 RN - 0 (HLA-DQ Antigens) RN - 0 (HLA-DR Antigens) SB - IM MH - Adolescent MH - Adult MH - Cataplexy/etiology/*genetics/immunology MH - Czech Republic MH - Female MH - Genotype MH - HLA-DQ Antigens/immunology MH - HLA-DR Antigens/immunology MH - Humans MH - Infectious Disease Transmission, Vertical MH - Male MH - Middle Aged MH - Narcolepsy/complications/*genetics/immunology MH - Pedigree EDAT- 1998/02/11 00:00 MHDA- 1998/02/11 00:01 CRDT- 1998/02/11 00:00 PHST- 1998/02/11 00:00 [pubmed] PHST- 1998/02/11 00:01 [medline] PHST- 1998/02/11 00:00 [entrez] AID - 10.1093/sleep/20.11.1021 [doi] PST - ppublish SO - Sleep. 1997 Nov;20(11):1021-6. doi: 10.1093/sleep/20.11.1021.