{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:04:33Z","timestamp":1791295473360,"version":"4.1.0"},"reference-count":24,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,4,6]],"date-time":"2015-04-06T00:00:00Z","timestamp":1428278400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2015,6]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Occlusive cerebrovascular moyamoya disease (<jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content>) is rare and has been characterized mainly in Asian countries, so far. In recent years, <jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content> has been increasingly reported worldwide, raising the question whether its clinical presentation would vary amongst different ethnic backgrounds. Here, a homogeneous series of 153 patients with <jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content> are reported and the specific clinical features of this rare disease amongst European Caucasians are highlighted.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 153 European Caucasians with <jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content> who were treated in our institution between 1997 and 2014 were retrospectively identified. Demographic data, clinical symptoms, angiographic characteristics and functional hemodynamic studies were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Moyamoya disease presented with a female predominance of 2,9:1.,78% presented with a typical <jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content>, 17% with a unilateral <jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content> and 5% with an atypical <jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content>. 16% of our patients belonged to the pediatric population. Overall, 81% and 8.5% of our cohort presented initially with ischaemic and hemorrhagic manifestation, respectively. The rate of hemorrhagic manifestation of <jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content> amongst the pediatric group was slightly higher (12%). Angiographic analysis revealed steno\u2010occlusive involvement of the posterior circulation in 34% with a higher involvement in pediatric patients (64%) compared to adults (28%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The characterization of our homogeneous European Caucasian cohort reveals several significant differences compared to Asian cohorts. In contrast, <jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content> presents similarly amongst European and North American cohorts, suggesting that non\u2010Asian <jats:styled-content style=\"fixed-case\">MMD<\/jats:styled-content> is characterized by distinct clinical features.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.12702","type":"journal-article","created":{"date-parts":[[2015,4,6]],"date-time":"2015-04-06T13:28:09Z","timestamp":1428326889000},"page":"1012-1017","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":83,"title":["Distinct clinical and radiographic characteristics of moyamoya disease amongst <scp>E<\/scp>uropean Caucasians"],"prefix":"10.1111","volume":"22","author":[{"given":"G.","family":"Acker","sequence":"first","affiliation":[{"name":"Department of Neurosurgery and Center for Stroke Research Berlin (CSB) Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Goerdes","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery and Center for Stroke Research Berlin (CSB) Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"U. 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