Moyamoya disease associated with Behcet’s disease

A 32-year-old woman with Behcet’s disease suffered repeated transient ischemic attacks (TIA) consisting of left hemiparesis. Cerebral angiography revealed the typical findings of moyamoya disease, with occlusion of the supraclinoid portion of both internal carotid arteries, coupled with abnormal collateral vessels. She underwent right superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis and encephalomyosynangiosis, due to decreased reserve capacity demonstrated on acetazolamide single positron emission computed tomography (SPECT). Postoperatively, the TIA symptoms subsided. This is the first report of moyamoya disease associated with Behcet’s disease, and moyamoya disease should be considered in the differential diagnosis of cerebrovascular events in patients with Behcet’s disease. Revascularization surgery is recommended for the prevention of ischemic insults resulting in permanent deficits.