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Moyamoya Disease - Case 2

A 27 year-old woman presented with progressive headaches and a focal seizure affecting the left side.

Note the Prominent Collaterals                         Compare to an Age Matched Normal Patient

Moyamoya Disease: T1-weighted with gadolinium axial MRIs. Note the numerous and prominent collateral vessels both in the deep and superficial areas. This picture is also characteristic of Moyamoya disease.

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 11/23/06
Copyrighted 2006. David C Preston