Fatal cerebral infarction after intraventricular hemorrhage in a pregnant patient with moyamoya disease

Moyamoya disease is a progressive occlusive cerebrovascular disorder of unknown etiology. We describe a case of a fatal cerebral infarction after intraventricular hemorrhage in a 29-year-old primipara with moyamoya disease. The patient had been stable for about 10 days after the initial hemorrhage, but she showed a fulminant clinical course of cerebral infarction, and died on the 16th day after the initial hemorrhage. Abnormality in coagulation system and/or cerebral vasospasm may have resulted in such a fulminant clinical course. A possible pathogenesis of this rare condition is discussed. (C) 2002 Published by Elsevier Science Ltd.