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Moyamoya Disease Reviewed 12-14-2001 Get Web page suited for printing Email this to a friend or colleague Studies with patients Table of Contents (click to jump to sections) What is Moyamoya Disease?Is there any treatment? What is the prognosis? What research is being done? Organizations Related NINDS Publications and Information What is Moyamoya Disease? Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by the narrowing or occlusion of major blood vessels leading into the brain, and the formation of abnormal blood vessels called moyamoya vessels. First described in Japan in the 1960’s, the disease has since been found in the United States, Europe, Australia, and Africa. It primarily affects children, adolescents, and young adults, although it has also been seen in people beyond these ages. Females are more frequently affected than males. The cause of the disease is unknown. Researchers suspect a genetic link because of the 9 percent incidence of the disease found in certain Japanese families. A gene for familial moyamoya disease has been located on chromosome 17q25 and further study of the gene may reveal the cause of the disorder. The name “moyamoya” is Japanese for “cloud of smoke” and was chosen to describe the classic appearance of the abnormal vessels seen in diagnostic tests. Children often present with stroke or recurrent transient ischemic attacks (TIAs), frequently accompanied by hemiparesis (muscular weakness or paralysis affecting one side of the body) or seizures. Adults most often experience stroke (often a hemorrhage). Both children and adults may have disturbed consciousness, speech deficits (aphasia), sensory and cognitive impairments, involuntary movements, and vision problems. Generally, cerebral angiography—a procedure that involves injecting dye into the cerebral arteries before taking an x-ray to make the arteries easier to see—is used to diagnose moyamoya diseas Is there any treatment? What is the prognosis? What research is being done? National Rehabilitation
Information Center (NARIC)
National Organization for Rare Disorders (NORD)
National Stroke Association Related NINDS Publications and Information
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