Symptomatic Moyamoya disease: clinical features and outcome after indirect bypass surgery in four French adults.

Background: There are a few reports of moyamoya disease (MMD) in the European Caucasian adult population. We present the clinical manifestations, the neuroradiological aspects, the treatment, and the outcome after surgical revascularization of four French patients with MMD.

Patients And Methods: We identified four adults (age >18 years; three women and one man) with MMD who underwent digital subtraction catheter angiography at our institution from 1997 through 2006. The median age at symptom onset was 35 years (range, 22 to 41 years). The initial clinical presentation was intracerebral hemorrhage in three patients and ischemic stroke in one patient.

Results: Three patients underwent bilateral surgical revascularization and one patient underwent unilateral surgical revascularization. All patients underwent the same surgical revascularization procedure (encephalo-duro-arterio-myo-synangiosis). The mean (+/-SD) period of follow-up after diagnosis of MMD was 6 years and 9 months (+/-3 years and 5 months). No patient experienced any recurrent hemorrhagic or ischemic stroke. No perioperative stroke occurred. No patient was severely disabled or unable to walk. Three patients out of four were employed.

Conclusion: Our data suggest safety and a potential benefit of surgical revascularization (indirect bypass surgery) in European adult patients with symptomatic MMD. Further long-term prospective multicenter studies are needed. The establishment of a registry would be useful in order to accumulate data in large numbers of European patients with this uncommon disease.