Puff of Smoke

Moyamoya is a rare cause of stroke caused by progressive distal carotid occlusion. Children and adults may be affected, but it is more common in Asian populations, particularly in patients presenting with intracranial hemorrhage. Angiographic findings of distal internal carotid artery stenosis with lenticulostriate or choroidal neocollaterals are important in the diagnosis of moyamoya disease. A similar pathophysiological state to moyamoya disease is associated with several clinical conditions, some of which are acquired and some of which have a genetic etiology. Treatment is with surgical revascularization, which can reduce both ischemic and hemorrhagic complications.