Familial patterns of narcolepsy.
Familial patterns of narcolepsy were investigated in a clinic population of 334 unrelated narcoleptic patients. 40% of probands had at least 1 family member with an isolated daytime sleepiness complaint and 6% had a positive family history of narcolepsy. Multicase families were rare; only two families were found with 3 or more affected relatives. Family members often shared the same HLA-DR2 haplotype as the proband but did not have narcolepsy. However, the risk of disease for first-degree relatives was six to eighteen times greater than that for unrelated individuals. Although most patients were HLA-DR2+, 2 new HLA-DR2- individuals were found. The data predict that as many as 9% of unrelated North-American white patients with narcolepsy will be DR2-. Analysis of these and other data indicates that although strongly associated with disease, the HLA-DR2 haplotype is neither sufficient nor necessary for the development of narcolepsy.
Guilleminault
C
C
Stanford University School of Medicine, California.
Mignot
E
E
Grumet
F C
FC
D000328:Adult; D000368:Aged; D004200:Diseases in Twins; D005260:Female; D015802:HLA-DR2 Antigen / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009290:Narcolepsy / Q000235:genetics*; D010375:Pedigree
D015802:HLA-DR2 Antigen