Present status of Moyamoya disease in Japan

Abstract

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. Threedimensional (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. © 2008 Springer-Verlag.