Current and future overview of moyamoya disease

Abstract

About 40 years has passed since moyamoya disease, a unique cerebrovascular disorder occurring widely in far-east Asia, was established as a disease entity. In the present article, the authors review the historical background, disease entity, diagnosis, radiological findings, and medical and surgical management of moyamoya disease. MR imaging and angiography represent the most powerful tools for the diagnosis and follow-up of patients with moyamoya disease. Evaluations of cerebral blood flow and metabolism, using single photonemission tomography (SPECT) and positron emission tomography (PET). tend to be more important for managing cases effectively and safely. Surgical cevascularization has been accepted as valuable for preventing further ischemia attacks in both pediatric and adult patients who present with TIA or cerebral infarction. However, the efficacy of bypass surgery for patients who develop intracranial bleeding due to moyamoya disease needs to be clarified by the on going multi-center randomized clinical trial in Japan (Japan Adult Moyamoya Trial : JAM Trial). Additionally, in this article, the authors present an overview of future aspects of moyamoya disease, including research to find the responsible genes of moyamoya disease, and the manage-ment of asymptomatic or pregnant patients with moyamoya disease.