• Jun Kakogawa, Miyuki Sadatsuki, Norio Masuya, Hideto Gomibuchi, Hiroyasu Ohno, Tetsuo Hara, Hiroko Oda, Akio Kimura, and Shigeki Minoura.
• Department of Obstetrics and Gynecology, National Center for Global Health and Medicine, 1-21-1, Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan. jkakogaw@hosp.ncgm.go.jp
• Arch. Gynecol. Obstet. 2011 Mar 1; 283 Suppl 1: 19-22.

PurposeMoyamoya (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 DesignA 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.ConclusionsMoyamoya 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.

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