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Moyamoya disease: Etiology, clinical features, and diagnosis

Nijasri Charnnarong Suwanwela, MD
Section Editors
Jose Biller, MD, FACP, FAAN, FAHA
Douglas R Nordli, Jr, MD
Deputy Editor
John F Dashe, MD, PhD


Moyamoya disease (MMD) is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. "Moyamoya" is a Japanese word meaning puffy, obscure, or hazy like a puff of smoke in the air. Thus, the term was used to describe the smoky angiographic appearance of the vascular collateral network [1,2].

Moyamoya disease was first described in Japan in 1957. Many similar cases have subsequently been reported, mainly in Japan and other Asian countries. The disease is found less frequently in North America and Europe.

This topic will review the etiology and clinical aspects of moyamoya disease. Prognosis and treatment are discussed elsewhere. (See "Moyamoya disease: Treatment and prognosis".)


The etiology of MMD is unknown. The high incidence among the Japanese and Asian population, together with a familial occurrence of approximately 10 to 15 percent of cases, strongly suggests a genetic etiology.

Accumulating evidence suggests that the RNF213 gene on chromosome 17q25.3 is an important susceptibility factor for MMD in East Asian populations [3-8].


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