Moyamoya disease associated with thrombotic thrombocytopenic purpura (TTP)

: A case of moyamoya disease associated with thrombotic 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 examination 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 cerebral 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 indicate 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.