title: [HLA and narcolepsy. Apropos of 28 cases including 2 negative HLA-DR2].
abstract: Association between narcolepsy and HLA-DR2 antigen is the strongest so far described between an HLA antigen and a disease. Among 28 narcoleptic patients, we found two HLA-DR2 negative cases: a caucasoid woman also suffering from dystrophia myotonica and a negroid. All of our patients were HLA-DQW1 positive. An hypothetical narcolepsy susceptibility gene could be located in the HLA region, closer to the DQ than to the DR gene. It could be a pathologic allele of a sleep controlling gene in linkage disequilibrium with DQW1. Presence of DQW1 is a quasi-requisite for the expression of narcolepsy. It is not sufficient as it is observed in 70 p. 100 of controls.
authors.affiliation: Service de Neurologie, Hôpital Neurologique de Lyon.
authors.forename: C
authors.initials: C
authors.lastname: Confavreux
authors.forename: L
authors.initials: L
authors.lastname: Gebuhrer
authors.forename: H
authors.initials: H
authors.lastname: Betuel
authors.forename: C
authors.initials: C
authors.lastname: Freidel
authors.forename: H
authors.initials: H
authors.lastname: Bastuji
authors.forename: M
authors.initials: M
authors.lastname: Jouvet
authors.forename: G
authors.initials: G
authors.lastname: Aimard
authors.forename: M
authors.initials: M
authors.lastname: Devic
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004247:DNA / Q000235:genetics; D005260:Female; D006681:HLA-D Antigens / Q000032:analysis*; D006683:HLA-DQ Antigens / Q000032:analysis; D006684:HLA-DR Antigens / Q000032:analysis* / Q000235:genetics; D015802:HLA-DR2 Antigen; D006801:Humans; D008297:Male; D008875:Middle Aged; D009290:Narcolepsy / Q000235:genetics / Q000276:immunology* / Q000503:physiopathology; D011110:Polymorphism, Genetic
chemical_list: D006681:HLA-D Antigens; D006683:HLA-DQ Antigens; C051568:HLA-DQ1 antigen; D006684:HLA-DR Antigens; D015802:HLA-DR2 Antigen; D004247:DNA
vernacular_title: HLA et narcolepsie. A propos de 28 cas dont deux HLA-DR2 négatifs.