Familial moyamoya disease in Caucasians.
There are few reports of moyamoya disease (MMD) in the Caucasian population and even fewer descriptions of the natural history of the disease. The study reports a 12-year follow-up of two white male siblings with MMD. Although both brothers had a persistently abnormal electroencephalogram and a learning disorder, the siblings recovered on aspirin and antiepileptic therapy with minimal neurologic residua. The occurrence of MMD in these siblings contributes to the evidence that MMD has a hereditary basis in Caucasians, as well as the Japanese. Even with the use of conservative measures, children may still have persistent yet minor cerebrovascular insults. In addition to the two patients discussed, this study reviewed the literature on all cases of MMD reported within families. Future follow-up studies are required to determine the natural history and the appropriate medical and surgical management of MMD.
Shetty-Alva
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Department of Internal Medicine, Roger Williams Medical Center, Boston University School of Medicine, Providence, Rhode Island 02906, USA.
Alva
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S
D002648:Child; D002675:Child, Preschool; D006801:Humans; D008297:Male; D009072:Moyamoya Disease / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D044465:White People / Q000235:genetics*