血栓性血小板減少性紫斑病（TTP）を合併し広汎な脳梗塞を来たしたモヤモヤ病の1例

Moyamoya Disease Associated with Thrombotic Thrombocytopenic Purpura (TTP)

I．はじめに

　今回われわれは血栓性血小板減少性紫斑病（TTP：thrombotic thrombocytopenic purpura）を合併し，大脳半球に広汎な脳梗塞を来たしたモヤモヤ病の1例を経験した．その特異な臨床経過を報告するとともに，本症例におけるTTPとモヤモヤ病の成因を探り両疾患で類似する病態について文献的考察を加えた．

A case of moyamoya disease associated with throm-botic thrombocytopenic purpura (TTP) was reported.

A 26-year-old male patient was admitted on April 11, 1992, with sudden onset of right cerebral hemorrhage. Cerebral angiography revealed moyamoya disease and bilateral encephalo-duro-arterio-synangiosis (EDAS)was performed. In March, 1993, however, he suffered from left cerebral hemorrhage. Neurological examina-tion on the second admission showed disturbance of consciousness, motor aphasia and right hemiplegia. Emergency operation for the hematoma removal was performed and neurological functions rapidly improved.However, on the day following the operation, he was in stupor and restlessness. Microangiopathic hemolytic anemia and severe thrombocytopenia were identified and he gradually sank into a comatose state. Systemic purpura, fever, renal dysfunction also appeared. CT scan 22 days after the onset demonstrated diffuse cere-bral infarction in the region of the bilateral anterior and middle cerebral arteries, and cerebral angiography on the next day demonstrated the development of bilateral internal carotid stenosis. Though laboratory findings in-dicate gradual improvement, he has remained in very weak state.

This is the first case of moyamoya disease associated with TTP. The etiology of both diseases was discussed.