Abstract

MOYAMOYA DISEASE (MMD) is a progressive cerebrovascular occlusive disease of the internal carotid arteries and anterior and middle cerebral arteries that affects children and young adults. The distinctive appearance of these collateral vessels on angiograms gives it the name “moyamoya,” which in Japanese means puff of smoke. 1 Suzuki J. Takaku A. Cerebrovascular “moyamoya” disease Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969; 20: 288-299 Crossref PubMed Scopus (1756) Google Scholar MMD is a relatively common cause of ischemic 2 Bruno A. Adams Jr, H.P. Biller J. et al. Cerebral infarction due to moyamoya disease in young adults. Stroke. 1988; 19: 826-833 Crossref PubMed Scopus (71) Google Scholar and hemorrhagic strokes in pediatric patients in Asia, especially Korea and Japan. Genetic linkage studies 3 Ikeda H. Sasaki T. Yoshimoto T. et al. Mapping of a familial moyamoya disease gene to chromosome 3p24.2-p26. Am J Hum Genet. 1999; 64: 533-537 Abstract Full Text Full Text PDF PubMed Scopus (238) Google Scholar and the study of the factors involved in the pathogenesis have shed new light on this disease. There is some suggestion that the pathogenesis may vary between races. 4 Gosalakkal J.A. Moyamoya disease: A review. Neurol India. 2002; 50: 6-10 PubMed Google Scholar

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