Moyamoya: epidemiology, presentation, and diagnosis.

Moyamoya syndrome is an increasingly recognized arteriopathy associated with cerebral ischemia and has been associated with approximately 6% of childhood strokes. It is characterized by chronic progressive stenosis at the apices of the intracranial internal carotid arteries, including the proximal anterior cerebral arteries and middle cerebral arteries and ultimately results in decreased cerebral blood flow with an increased resultant risk of stroke. This article discusses the epidemiology, presentation, and diagnosis of this condition in children.