가족에서 발생한 모야모야병

A Case of Familial Moyamoya Disease: A Case in Brother and Sister.

저자들은 남매에서 각각 경련과 사지에 저린 증상으로 내원하여 뇌 자기공명영상과 뇌 자기공명혈관조영술로 진단된 가족성 모야모야병 1를 경험하였기에 이를 보고하는 바이다.

Moyamoya disease is a progressive and occlusive disorder of the cerebral vasculature with particular involvements of the circle of Willis and the arteries that feed it. It occurs commonly in Japan and Korea, but less frequently in the Western countries. The etiology of moyamoya disease is still unclear, but frequent familial occurrence suggests that some genetic factors may be important in its etiology. Approximately 7-10% of moyamoya disease are familial. We experienced 2 siblings with moyamoya disease, and report the case with a review of previously published cases of moyamoya disease within a family.