Moyamoya Disease in Asia

Moyamoya disease is a rare cerebrovascular disorder, characterised by progressive stenosis and/or occlusion of the intracranial internal carotid artery and its proximal branches with the development of a basal collateral network. Moyamoya disease has a high prevalence in Asia, particularly in Japan, Korea and China. Ischemic events and intracranial bleeding are the most common clinical manifestation of moyamoya disease. Although the benefi cial effect on hemorrhage is still not clear, revascularisation surgery remains the most effective way to prevent the progression of ischemic symptoms. Moyamoya disease has been investigated by numerous studies since it was fi rst described 50 years ago, many conundrums remain to be solved. In this article, we review the history, epidemiology, aetiology, clinical manifestation, diagnosis and treatment of moyamoya disease. Recent advances and future challenges of moyamoya disease are also discussed. Neurology Asia 2012; 17(3) : 175 – 181 Address correspondence to: Lian Duan, 8 Dong-Da Street, Fengtai District Beijing 100071, China. Tel: 86-10-66947159, Fax: 86-10-51128000, E-mail: [email protected] INTRODUCTION AND HISTORICAL