Moyamoya Disease in Europeans

Kraemer, Markus; Heienbrok, Wilhelm; Berlit, Peter

doi:10.1161/strokeaha.107.513408

Cited by 200 publications

(166 citation statements)

References 17 publications

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“…A report from Europe evaluated 21 patients with moyamoya disease. 6) The mean age at onset was 31 years, the female-to-male ratio was 4.25, family illness history was unremarkable, and no peak incidence in childhood was observed. All initial symptoms were ischemic.…”

Section: Epidemiological Features Of Moyamoya Disease In Japanmentioning

confidence: 95%

Epidemiological Features of Moyamoya Disease in Japan

Hoshino

Izawa

Suzuki

2012

Neurol. Med. Chir.(Tokyo)

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To clarify the clinical features of moyamoya disease in Japan, 941 patients with definite moyamoya disease were analyzed from the databases constructed by the Research Committee on Moyamoya Disease, established by the Ministry of Health, Labour and Welfare. Moyamoya disease occurs much more frequently among women than men, with a female-to-male ratio of 1.98. A family history of the disease was observed in 14.9%. The age at onset was characterized by two peaks: one at 5-9 years and another lower peak at around 40 years. Initial clinical features were transient ischemic attack in 46%, infarction in 20%, hemorrhage in 21%, headache in 6%, and epilepsy in 4%. The distribution of the age at onset showed one peak at around 40 years in patients with hemorrhage but two peaks in patients with ischemia.

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Section: Epidemiological Features Of Moyamoya Disease In Japanmentioning

confidence: 95%

Epidemiological Features of Moyamoya Disease in Japan

Hoshino

Izawa

Suzuki

2012

Neurol. Med. Chir.(Tokyo)

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“…[1][2][3] For example, the typical bimodal incidence with one peak in childhood, typical for most Asian patients (Korean, Japanese, and Chinese), 1,2 could not be found in larger cohorts in Europe and the United States. [4][5][6] Furthermore, MMD in adult patients of Asian descent (except Taiwan and Nanjing) have much higher rates of intracerebral hemorrhage as initial symptomatic event than adult moyamoya patients in Europe and the United States. 2,5 Recent studies demonstrated that the incidence of cerebral microbleeds (cMBs) is higher in Asian patients with MMD in comparison to healthy controls.…”

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Incidence, Locations, and Longitudinal Course of Cerebral Microbleeds in European Moyamoya

Wenz

Maros

et al. 2017

Stroke

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Background and Purpose-Cerebral microbleeds (cMBs) have previously been linked with especially high incidence in Asian patients with moyamoya together with high tendency to bleed. This, presumably, is characteristic of patients with moyamoya. Herein, we, therefore, investigate retrospectively the frequency, location, and longitudinal course of cMBs in a large German cohort. Methods-We included all patients with moyamoya who underwent standard magnetic resonance imaging, including T2*-weighted images, in our department between 1998 and 2015. Two independent readers evaluated magnetic resonance imaging scans to determine the occurrence of cMBs according to the Brain Observer Microbleed Scale. Demographics, initial symptoms leading to hospitalization, and associated diseases were obtained by chart review. Results-Overall, there was a total of 242 T2* studies of 101 included moyamoya patients available with a strong female predominance (69.3%). Eight patients (7.9%) were ≤18 years of age. We detected 25 cMBs within 13 patients (12.9%).One patient <18 of age was presented with a cMB; 2 of 3 patients with an intracranial hemorrhage as initial event demonstrated cMB(s). In 72 of 101 cases, there were 1719 person months of follow-up, with 3 adult patients showing 3 de novo cMBs in the course. The majority of cMBs (64.0%) were located at the cortex/gray-white junction. Conclusions-Although the frequency of cMBs herein is much higher than the expected age-specific incidence, it is still much lower compared with previous reports on cMBs in moyamoya patients of Asian descent. These results might reflect another ethnic-specific difference in patients diagnosed with moyamoya.

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“…35,39 A number of studies have provided evidence that moyamoya disease outside of Asia can represent a different phenomenon. 4,7,21,36,38 Patient ethnicities are in proportion to the ethnicities of the people in the region of diagnosis, and adult patients present with ischemic symptoms rather than intracranial hemorrhage.…”

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confidence: 99%

“…Although no completed randomized clinical trials have addressed the benefit of surgical revascularization, the dismal prognosis of moyamoya disease [2][3][4]7,8,21,23,31,33,[41][42][43] and a large number of case series demonstrating an improved outcome in surgically treated patients have provided evidence in support of revascularization procedures in a select group of patients. 4,[7][8][9][10][11]16,17,25,29,30,32,34,40,43 Currently, the Ministry of Health and Welfare of Japan reports that bypass surgery is indicated when there are 1) repeated clinical symptoms due to apparent cerebral ischemia and (2) decreased regional cerebral blood flow, vascular response, and perfusion reserve.…”

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Optimal surgical treatment for moyamoya disease in adults: direct versus indirect bypass

Starke¹,

Komotar²,

Connolly³

2009

FOC

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Moyamoya disease is a chronic cerebrovascular occlusive disorder that results in severe morbidity and death. There is much controversy surrounding the optimal treatment for adult patients with the disorder. There have been no randomized trials to assess the efficacy of any single surgical treatment, and existing case series suffer from inadequate power, selection bias, and inherent differences in patient characteristics. In this article the authors review the literature concerning the optimal surgical treatment of adult patients with moyamoya disease.

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Moyamoya Disease in Europeans

Abstract: 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.

Cited by 200 publications

References 17 publications

Epidemiological Features of Moyamoya Disease in Japan

Epidemiological Features of Moyamoya Disease in Japan

Incidence, Locations, and Longitudinal Course of Cerebral Microbleeds in European Moyamoya

Optimal surgical treatment for moyamoya disease in adults: direct versus indirect bypass

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