gms | German Medical Science

Objective: Occlusive cerebrovascular disease “Moyamoya” (MMD) has been increasingly reported worldwide with emphasize on different patient characteristics. However there is no sufficient epidemiological characterization of Caucasian population in Europe. The term “moyamoya angiopathy” has been recently accepted to simplify communication including MMD, moyamoya syndrome and unilateral moyamoya disease. The aim of our study is to increase awareness and provide new clinical insights to moyamoya angiopathy (MMA) in Europe.

Method: We retrospectively identified Caucasians with MMA presented to Neurosurgical Department of Mannheim University from 1997 to 2007 and to Charité – Universitymedicine Berlin from 2007 to 11/2013. MMA was diagnosed according to the guidelines by Research Committee on Moyamoya Disease of Ministry of Health and Welfare, Japan and to Suzuki classification. We analyzed demographic data, clinical symptoms and angiographic characteristics.

Results: 216 (53 male,163 female) patients were included. Subgroup frequencies were 58% MMD, 21% MMS, 17% unilateral MMD/MMS and 3% atypical MMD with involvement of extracranial vessels. 21% of our patients were younger than 18 years old. Initial symptom was overall ischemic event in 82%, hemorrhage in 8,7%, whereas in children with MMD hemorrhage rate was 16%. Involvement of posterior cerebral artery was seen overall in 29% (MMD: 31%, MMS: 34%). 3.7% of the patients had cerebral aneurysms.

Conclusions: The epidemiological analysis has identified distinct characteristics compared to Asian or North American populations suggesting that MMA in Europe is a unique cerebrovascular disease entity.