Human Leukocyte Antigen in Patients With Moyamoya Disease

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Background and PurposeProgressive stenosis or occlusion of bilateral internal carotid arteries by fibrocellular intimal thickening results in cerebral ischemia in moyamoya disease. In an attempt to elucidate the still-unknown etiologic factors in moyamoya disease, we assessed human leukocyte antigens in patients with this disease.MethodsWe investigated 32 unrelated Japanese patients with moyamoya disease for typing of human leukocyte antigen A, B, C, and DR/DQ and compared the results with those from 178 unrelated control subjects.ResultsWe found a significant association of human leukocyte antigen B51 with moyamoya disease (corrected P less than .05, chi2 test). Although no significant associations were observed in DR/DQ typing, the frequency of the B51-DR4 combination was significantly higher in moyamoya patients than in control subjects (P less than .002, Fisher's exact test).ConclusionsThese findings suggest that there may be a genetic predisposition for moyamoya disease and that host factors may play a role in the development of intimal thickening in early childhood.(Stroke. 1995;26:415-417.)

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