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Intracranial Disease - Case 3 - Moyamoya Disease

A 3 year-old boy developed headaches and spells of difficulty speaking associated with right sided weakness.

Note the Normal Right ICA   Show the Top of the Carotid Stenosis    Show the Proximal MCA Stenosis   Show the Prominent Deep Collaterals ("Puff of Smoke")

Cerebral Angiogram. (Left) Right Internal Carotid injection (AP); (Middle) Left Internal Carotid injection (AP); (Right) Left Internal Carotid injection (lateral view). Note that the right ICA injection (left image) is completely normal. However, on the left internal carotid artery is severely narrowed distally. In addition, prominent collateral vessels are seen. This is Moyamoya disease, a rare idiopathic disorder characterized by progressive narrowing of the distal internal carotid arteries. These cases are typically seen in children although rarely reported in adults.

Revised 05/20/06.
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