Antepartum intracranial hemorrhage due to unrecognized unilateral moyamoya disease: a case report.

PURPOSE: Moyamoya (meaning a "hazy puff of smoke" in Japanese) disease is a rare cerebrovascular occlusive disease. Moyamoya disease may become symptomatic for the first time during pregnancy. We report a case of antepartum intracranial hemorrhage due to unrecognized unilateral moyamoya disease, which was subsequently diagnosed as HELLP syndrome during the postpartum period.

STUDY DESIGN: A case report of a 29-year-old Japanese primigravida who was transported to our hospital at 39 weeks of gestation because of sudden loss of consciousness and left hemiplegia. On arrival, her blood pressure was 143/94 mmHg with 1+ proteinuria by dipstick. Brain computed tomography revealed a right putaminal hemorrhage with intraventricular hemorrhage. The patient delivered a neonate by emergency cesarean section, and an intracranial hematoma was subsequently evacuated. Approximately 3 h postoperatively, she was diagnosed with HELLP syndrome and the following were initiated: IV magnesium sulfate, antihypertensive agents, and transfusion of 10 units of platelets. Angiographic findings were consistent with unilateral moyamoya disease.

CONCLUSIONS: Moyamoya disease is a rare entity that must be considered in the differential diagnosis of hemorrhagic stroke during pregnancy. It is important to perform careful monitoring and adequate management with cooperation between obstetricians and other specialists when serious complications arise.