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. The name “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.
The total number of patients with moyamoya disease was 8154 in 2011, with a female-to-male ratio of 1.8. The incidence from 2007 to 2011 was 1.7 to 2.3/10(5), and the prevalence in 2011 was 16.1/10(5). In total, 66.3% of patients aged 0 to 14 years underwent surgery, whereas only 21.5% in the older than 15 years age group underwent surgery. The 1- and 5-year survival rates of adult patients were 96.9% and 92.9%, respectively, and of child patients were 99.6% and 99.3%, respectively.
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.