Pial Synangiosis Ameliorates Movement Disorders in the Absence of Prior Stroke in Moyamoya Disease

Background: Moyamoya disease is a rare cerebrovascular disease characterized by progressive stenosis of the bilateral distal internal carotid arteries and their proximal branches. Both chorea and dystonia have been reported as the initial presentation of moyamoya disease. Objective: The objective was to define the clinical presentation and describe the disease course following pial synangiosis of 3 patients with dyskinesias. Methods: A retrospective chart review of 3 cases of patients presenting with movement disorders and ultimately diagnosed with moyamoya disease was performed. Results: The authors present a case series of 1 patient with dystonia and 2 patients with chorea, all diagnosed with moyamoya disease. All patients experienced resolution of their movement disorders following pial synangiosis. Magnetic resonance imaging disclosed moyamoya disease-related basal ganglia anomalies in all patients. Conclusions: Moyamoya disease is an important and surgically treatable cause of movement disorders.