Future perspectives in moyamoya disease

Abstract

It has been half a century since K. Takeuchi and K. Shimizu first reported a new entity in vascular disease in 1957, moyamoya disease (MMD) [1]. This disease is a relatively rare vascular disease and has a high prevalence in children and Asian ethnics; thus, it has not been of great interest to physicians and researchers in non-Asian countries. The Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare in Japan launched a project in 1977 to investigate the disease, and continues to study its pathogenesis, epidemiology, and treatment. Most of the reported literature about MMD is from Japan. The annual report of the Committee has accumulated many clinical as well as research data, and studies are ongoing. The incidence of reports on MMD is increasing in Western countries. It is important to understand the current status of MMD management with its unsolved problems. No standardized surgical method of choice has yet been established. One reason for this is the absence of standardized evaluation measures and protocols. Here, current evaluation measures for pre-and post-operative outcomes are reviewed, and novel therapeutic research trials are presented as potential future perspectives on this disease. © 2010 Springer-Verlag Tokyo.