Moyamoya disease: review of the literature and estimation of excess morbidity and mortality.

As a result of greater screening with brain imaging tests, the incidence of Moyamoya Disease (MMD) is increasing. In the past, comparative mortality experience has been based on small patient numbers and relatively short follow-up periods. The objective of this article is to estimate the degree of excess morbidity and mortality associated with this rare impairment. In addition we will provide a concise review of the literature, including pathophysiology, clinical manifestation, diagnostic criteria and surgical treatment.