Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. “Moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage. It primarily affects children, but it can also occur in adults. In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks.
From 2002 to 2009, an estimated 518 patients (mean ± SD age, 16.2 ± 1.68 years) with coexisting moyamoya disease and Down syndrome were admitted. The estimated prevalence was 3.8% (3760 per 100,000) among patients admitted with moyamoya disease and 9.5% (9540 per 100,000) among moyamoya patients < 15 years of age.
There are several types of revascularization surgery that can restore blood flow to the brain by opening narrowed blood vessels or by bypassing blocked arteries. Children usually respond better to revascularization surgery than adults, but the majority of individuals have no further strokes or related problems after surgery.
Recent investigations have established that both moyamoya disease and arteriovenous fistulas (AVFs) of the lining of the brain, the dura, are associated with dural angiogenesis. These factors may represent a mechanism for ischemia contributing to the formation of dural AVFs. At least one case of simultaneous unilateral moyamoya disease and ipsilateral dural arteriovenous fistula has been reported at the Barrow Neurological Institute.