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Moyamoya Disease - Case 1

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")

Moyamoya Disease: Cerebral Angiogram. (Left) Right Internal Carotid Artery (ICA) injection (AP view); (Middle) Left Internal Carotid Artery injection (AP view); (Right) Left Internal Carotid Artery injection (Lateral view). Note that the right ICA injection (left image) is completely normal. However, the left internal carotid artery is severely narrowed distally (middle image). In addition, prominent collateral vessels are seen (middle and right images). MCA = middle cerebra artery, ACA = anterior cerebral artery.

This patient has Moyamoya disease, a rare idiopathic disorder characterized by progressive narrowing of the distal internal carotid arteries and their branches. It is typically seen in children, although rarely reported in adults. As the carotid artery is compromised, there is progressive enlargement of the collateral circulation, especially among the lenticulostriate vessels. This results in a characteristic angiographic picture of a blush or "puff of smoke" in the area of the lenticulostriate vessels. Moyamoya disease typically presents with recurrent, progressive cerebral infarctions; in some cases, the fragile collateral vessels can rupture resulting in intracerebral hemorrhage.


Revised 11/30/06
Copyrighted 2006. David C Preston