Adult-Onset Moyamoya Disease with Angiographically Verified Progression

Abstract

We describe an adult case of Moyamoya disease (MMD) with progression, and review the clinical and radiographic records obtained in previous published reports of adult-onset (over 20 years) MMD with progression. A 43-year-old Japanese woman presented with MMD with angiographically verified progression. The first angiogram depicted severe stenoses at the terminations of both internal carotid arteries. Four years later, she was readmitted to our hospital for surgical treatment because of recurrent TIA once or more per month. An angiogram demonstrated marked progressive appearance. We confirmed only 19 cases of MMD with progression including our present one. We could roughly divide these cases into two groups by angiographic appearance (unilateral or bilateral lesions at diagnosis). Ischemic stroke was the most common initial presentation, and there were only two cases with hemorrhagic stroke. The mean period until follow-up angiography was 35.3 months, and most cases (84.2%) progressed to advanced stage within five years. About half (42.9%) of the patients were asymptomatic when angiographic progression was confirmed. Adult-onset MMD with progression includes several different pathological patterns. We suggest that careful observation is needed for at least five years after initial presentation to assess progression of MMD even in adult-onset cases.