Moyamoya Disease

Definition

Moyamoya disease is a rare disorder of blood vessels in the brain known as internal carotid arteries (ICA). The condition is characterized by stenosis (narrowing) or occlusion (blockage) of one or both ICA with subsequent formation of an abnormal network of blood vessels adjacent to the ICA.

Description

Moyamoya disease was first described in Japan in 1955. The term moyamoya, a Japanese word that means "puff of smoke," describes the appearance of the abnormal vessels that form adjacent to the internal carotid arteries. Alternate names for the disorder include spontaneous occlusion of the circle of Willis, and basal occlusive disease with telangiectasia.

Moyamoya disease can occur in children (juvenile type) or in adults (adult type). Children tend to be less than age 10 and adults are usually between ages 30 and 49. Affected individuals typically present with signs of

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