Moyamoya, primary

8-year-old female with history of multiple cerebral infarcts and gradual decline in mental status. There are numerous regions of cystic encephalomalacia in the periatrial white matter will multiple tiny vessels in the Sylvian fissure. There is patchy gliosis in the periventricular and deep white matter of the left greater than right parietal lobes. There is also abnormal T2/FLAIR hyperintense signal in the visualized cerebral cortical sulci and Sylvian fissures. Numerous, tiny, serpiginous flow voids are identified in the basal cisterns and Sylvian fissures. The flow voids of the supraclinoid termini as well as the anterior, middle, and posterior cerebral arteries are not identified. The findings are compatible with Moyamoya, and are confirmed on the 3-D time of flight maximum intensity projection. Moyaomoya represents progressive narrowing of internal carotid artery termini and proximal circle of Willis vessels with numerous tiny collateral vessels. There are primary and secondary forms. This is a case of primary moyamoya.