Background and Purpose—We describe the clinical, diagnostic, and outcome features of a cohort of white patients with idiopathic moyamoya disease treated in a German institution.

Methods—Our cohort included 21 white patients with moyamoya disease. Clinical and diagnostic features were obtained by retrospective chart review; follow-up information and outcome were obtained prospectively. We used the Kaplan–Meier methods to estimate stroke risk by treatment status.

Results—The mean age at onset of symptoms was 31 years. The female predominance was 4.25:1. In our cohort, the initial symptom was a cerebral ischemic event in all patients. There was no patient with a hemorrhage at onset; only one patient experienced subarachnoidal hemorrhage in the further course of disease. The Kaplan–Meier risk for recurrent stroke was very high after the first ischemic event and smaller after angiographic diagnosis. The 5-year-Kaplan–Meier risk of recurrent stroke was 80.95% after the first ischemic event for all patients. Most subsequent ischemic events appeared in the first 2 years after symptom onset. Eleven patients (52.3%) underwent neurosurgical revascularizing procedures. After surgery, the Kaplan–Meier risk of perioperative or subsequent stroke was 27.27% within the first month and was stable thereafter.

Conclusion—Clinical features and course of moyamoya disease of whites analyzed in this German study are comparable to American results. Moyamoya disease in whites differs clearly from Asian moyamoya disease in timing of onset of vasculopathy and lower rate of hemorrhages.