Genetic and immunologic analysis on moya-moya.
The genetic and immunologic abnormalities associated with the pathogenesis of moya-moya were assessed in 23, 13 children and 10 adults with angiographically diagnosed moya-moya. In HLA-A, -B, -C stereotyping, an association was found of AW24, BW46, and BW54 with relative risks of 3.83, 6.50, and 3.58 respectively. Natural T cell toxic autoantibody was detected by FACS analysis in sera from five out of 23 patients. Millipore filter assay for autoantibody against double-stranded DNA revealed higher than normal binding in sera from four out of 18 patients. Anti-vessel antibody which might be responsible for vascular change associated with moya-moya was not detected in any of the 23 patients studied. Significant association of the disease with certain HLA types, in addition to the presence of natural T cell toxic autoantibody and anti-double-stranded DNA antibody in patients' sera, supports the theory that genetic and immunologic disturbances may underly the pathogenesis of moya-moya.
D000293:Adolescent; D000328:Adult; D001157:Arterial Occlusive Diseases / Q000235:genetics*; D001323:Autoantibodies / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D004247:DNA / Q000276:immunology; D005260:Female; D006680:HLA Antigens / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D009072:Moyamoya Disease / Q000235:genetics* / Q000276:immunology; D009131:Muscle, Smooth, Vascular / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology
D001323:Autoantibodies; D006680:HLA Antigens; D004247:DNA