Present status of Moyamoya disease in Japan.

To gain an overview of the current status of Moyamoya disease in Japan, we reviewed the 2002-2004 report of the Research Committee on Moyamoya Disease and the clinical data of Moyamoya patients treated at Gifu University Hospital during the past 2 years. According to the report, a nationwide epidemiological survey performed in 2004 revealed that approximately 7500 Japanese were treated for Moyamoya disease; their number doubled during the last 10 years. Moyamoya associated with headache was newly added as a subtype; as many as 5% of Moyamoya patients experience headache. Three-dimensional (3D) stereotactic statistical cerebral blood flow (CBF) analysis was reported as useful for the stratification of the cerebral hemodynamics in Moyamoya disease. To develop treatment guidelines for hemorrhagic Moyamoya, a prospective randomized control trial begun in 2001 is ongoing. During the past 2 years, 23 patients with Moyamoya disease were treated at our hospital. Of these, 17 presented with transient ischemic attacks/infarction, 4 with intracranial hemorrhage (ICH), and 2 with headache. One patient who presented with ICH died during the acute stage, the remaining 22 patients were successfully treated by direct bypass surgery.