Moyamoya Disease

Moyamoya Disease: progressive occulsive disease of the cerbral vasculature with particular involvement of the Circle of Willis and the arteries that feed it. Moyamoya is Japansese for a puff of smoke. Moyamoya disease is characterized by intimal thickening in the walls of the terminal portions of the internal carotid vessels bilaterally. The orgin of Moyamoya is unknown. Moyamoya can be hereditary and multifactorial. This disease has the highest rate in Japan and occurs in 10% of adults and 4.3% in children. Moyamoya ranges from the ages of 6 months to 67 years.

Causes: thrombosis, cerebral ischemia, transient ischemic attacks, and athersclerosis.

Symptoms: stroke, recurring transient ischemic attacks, hemiparasis, seizures, disturbed consciousness, speech deficits, sensory impairments, cognitive impairments, involuntary movements and vision problems.

Tests: Cerebral angiography is done to diagnose Moyamoya disease.

Treatment: anticoagulats (Heparin and Coumadin), antiplatelet agents, aspirin, and omental transplantation.



Figure 2a. Moyamoya disease in a 9-year-old boy. (a) Right internal carotid arteriogram in lateral projection shows the right ICA (curved arrow) is occluded, and thus the anterior cerebral and middle cerebral arteries are occluded.