A 29 year old woman with acute mental status changes

Moyamoya disease is a rare cerebrovascular disease that causes occlusion of the supraclinoid internal carotid arteries and its branches within the Circle of Willis. While rare in the United States and Europe, moyamoya disease is much more common in eastern Asian populations. We present a 29 year-old Caucasian woman who initially presented with severe mental status changes and ataxia. MRI and MRA imaging, as well as pathologic examination at autopsy, revealed occlusion of the middle cerebral and anterior cerebral arteries bilaterally with associated infarctions. This case underscores the importance considering this diagnosis as an etiologic possibility during the workup of multiple infarcts, even in non-Asian populations.