Clinical course and outcome in French adults with Moyamoya disease

Background and purpose. - Moyamoya disease is rare among non-Asian populations and its clinical features are ill-defined. We report 12 new cases of Moyamoya disease in French, non-Asian adults. Methods. - We identified adults with Moyamoya disease managed at a French University Hospital from 1998 through 2006. We reviewed baseline clinical and radiological data and collected follow-up data between March and June 2008. The risk of recurrent stroke was determined using the Kaplan-Meier method. Results. - Twelve patients, 10 women and two men, were included. The mean age at baseline was 31.1 years. The initial clinical manifestation was ischemic stroke in 10 patients. The disease was clinically asymptomatic in two patients. The mean follow-up in the 10 symptomatic patients was 52.4 months. Only one patient was surgically treated. The one and five-year risk of recurrent stroke were both 50%. At the end of the follow-up, one patient was dead, four patients had no functional impairment (grade 0-1 on the Rankin scale), and seven patients had moderate functional impairment (grade 2-3 on the Rankin scale). A cognitive dysfunction as identified by failure on the Trail Making Test part B was found in six of ten patients evaluated. Conclusion. - Moyamoya disease in this cohort of French, non-Asian adults was associated with high rates of both recur-rent stroke and cognitive impairment. The findings suggest that Moyamoya disease in non-Asian adults is a potentially severe disease. (c) 2008 Elsevier Masson SAS. All rights reserved.