title: HLA antigens in narcolepsy and idiopathic central nervous system hypersomnolence.
abstract: Several studies have shown familial incidence of narcolepsy and idiopathic central nervous system (CNS) hypersomnia. HLA antigen studies performed in mongoloid and caucasoid narcoleptic patients on the A, B, and C loci have yielded conflicting results. The aim of this study is to document a possible association between the HLA system, including the DR locus and excessive daytime somnolence. Thirty-one narcoleptic patients and 10 idiopathic hypersomniac patients were selected and typed for 54 HLA antigens. A family with narcoleptic members in 3 generations was also studied. HLA-DR2 was found in 100% of narcoleptic patients. The frequency of HLA-A3 and B7, which are in linkage disequilibrium with DR2 was also increased in this group. Idiopathic hypersomniac patients showed an increase of HLA-Cw2, DR5, and B27, three antigens known to be in linkage disequilibrium. In the family study, narcoleptic patients were also HLA-DR2; moreover, 3 subjects, one of whom was narcoleptic, were HLA-DR2 as a result of recombination (i.e., genetic crossing-over). These results locate the hypothetic gene associated with narcolepsy more precisely, and indicate that narcolepsy and idiopathic CNS hypersomnolence are two different entities.
mesh_terms: D000328:Adult; D006970:Disorders of Excessive Somnolence / Q000235:genetics*; D006680:HLA Antigens / Q000032:analysis*; D015791:HLA-A3 Antigen; D015796:HLA-B27 Antigen; D015793:HLA-B7 Antigen; D015236:HLA-C Antigens; D006684:HLA-DR Antigens; D015802:HLA-DR2 Antigen; D015805:HLA-DR5 Antigen; D000949:Histocompatibility Antigens Class II / Q000032:analysis*; D006801:Humans; D009103:Multiple Sclerosis / Q000235:genetics; D009290:Narcolepsy / Q000235:genetics*; D010375:Pedigree; D012893:Sleep Wake Disorders / Q000235:genetics*
chemical_list: D006680:HLA Antigens; D015791:HLA-A3 Antigen; D015796:HLA-B27 Antigen; D015793:HLA-B7 Antigen; D015236:HLA-C Antigens; C048767:HLA-C*02 antigen; D006684:HLA-DR Antigens; D015802:HLA-DR2 Antigen; D015805:HLA-DR5 Antigen; D000949:Histocompatibility Antigens Class II