Distinct clinical and radiographic characteristics of moyamoya disease amongst <scp>E</scp>uropean Caucasians

Acker, Güliz; Goerdes, Susanne; Schneider, Udo; Schmiedek, Peter; Czabanka, Marcus; Vajkoczy, Peter

doi:10.1111/ene.12702

Cited by 78 publications

(75 citation statements)

References 25 publications

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“…In the Japanese national survey, the rate of steno-occlusion of ICA reported to be more than half of the patients; however, the frequency of steno-occlusion of ICA with ACA/MCA together is not stated [3] . Angiographic analysis revealed steno-occlusive involvement of the posterior circulation in 31% with a higher involvement in pediatric patients (40%) compared to adults (27%) similar to the reported European Caucasian MMD cohort [7] . Unfortunately, we could not find any data about posterior circulation concerning quasi-MMD in Asian.…”

Section: Discussionsupporting

confidence: 78%

“…Whereas mean age at presentation was comparable to the Asian population [3] , the age distribution revealed a reverse biphasic distribution with a minor pediatric peak and a major adult peak similar to Caucasian MMD series [7] . This represents an important difference in our population; although arteriosclerosis patients were not involved in our study, the frequency of quasi-MMD in late adulthood is prominent.…”

Section: Discussionmentioning

confidence: 73%

“…Cerebral hemorrhage occurred interestingly only in adults. We previously reported a higher hemorrhage rate in juveniles in MMD that was not observed in quasi-MMD cohort [7] . The hemorrhage rate as an onset symptom has also been reported to be low in Asian and North American series [3,14] .…”

Section: Discussionmentioning

confidence: 74%

“…Sixty-one patients with an accompanying disease were categorized as quasi-MMD. We categorized patients with additional involvement of extracranial carotid artery steno-occlusion as atypical quasi-MMD as described before [7] . 'Atypical MMD' is not a uniform official term [8,9] ; thus, we defined it for our study individually for patients with additional involvement of extracranial internal carotid artery stenosis without signs of atherosclerotic changes [7] .…”

Section: Methodsmentioning

confidence: 99%

“…3 ) [7] . Occlusion of both intracranial internal carotid arteries were seen in 30%, steno-occlusive changes of both ICA or unilaterally in the case of unilateral quasi-MMD was 8% ( fig.…”

Section: Angiographic Characteristicsmentioning

confidence: 99%

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Characterization of Clinical and Radiological Features of Quasi-Moyamoya Disease among European Caucasians Including Surgical Treatment and Outcome

Acker

Goerdes²,

Schmiedek³

et al. 2016

Cerebrovasc Dis

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Background: Moyamoya disease (MMD) associated with a potentially underlying disease, such as genetic disorders or other coexisting hematological pathologies, is called quasi-MMD. This very rare disease has been characterized mainly in Asian countries, so far. As MMD reveals several significant ethnic differences, the question is raised whether characteristics of quasi-MMD would also vary among different ethnic backgrounds. Here, we report a series of 61 patients with quasi-MMD and highlight the specific clinical features of this rare disease among European Caucasians. Methods: We retrospectively identified 61 European Caucasians with quasi-MMD who were treated in our institution between 1997 and 2014. We analyzed demographic data, clinical symptoms, associated diseases, angiographic characteristics and functional hemodynamic studies. Results: Thirty-three percent of our patients were juvenile. We observed an overall female predominance of 2.8:1. Seventy-nine percent presented with a typical quasi-MMD with more pronounced unilateral and atypical quasi-MMD in pediatric population (unilateral/atypical: pediatric patients 20/15%, adults 7/7%). We identified a wide range of associated diseases. Overall, 84 and 8% of our cohort presented initially with ischemic and hemorrhagic manifestation, respectively. The hemorrhagic manifestation of quasi-MMD occurred however only in adults. Angiographic analysis revealed steno-occlusive involvement of the posterior circulation (in addition to the anterior circulation) in 31% with a higher involvement in pediatric patients (40%) compared to adults (27%). Conclusions: The characterization of our European Caucasian cohort reveals several differences when compared to reported Asian quasi-MMD cohorts and also compared to European Caucasian MMD cohort. We conclude that quasi-MMD represents a distinct disease with different ethnic clinical features.

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Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 74%

Section: Methodsmentioning

confidence: 99%

“…3 ) [7] . Occlusion of both intracranial internal carotid arteries were seen in 30%, steno-occlusive changes of both ICA or unilaterally in the case of unilateral quasi-MMD was 8% ( fig.…”

Section: Angiographic Characteristicsmentioning

confidence: 99%

See 3 more Smart Citations

Characterization of Clinical and Radiological Features of Quasi-Moyamoya Disease among European Caucasians Including Surgical Treatment and Outcome

Acker

Goerdes²,

Schmiedek³

et al. 2016

Cerebrovasc Dis

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Incidence of Moyamoya Disease in Denmark: A Population-Based Register Study

Birkeland

Lauritsen

2018

Acta Neurochirurgica Supplement

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In this first population-based study of moyamoya disease (MMD) in Europe, the authors identified 56 patients with MMD in Denmark during the period 1994-2015 using nationwide registers. The overall incidence was 0.047 per 100,000 person-years, which is about one-tenth that reported in Japan. Otherwise the epidemiological features were comparable: there was a bimodal age distribution with peaks in the age groups 0-9 years and 30-39 years, with twice as many females as males.

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Natural History and Long-Term Clinical Outcome After Conservative and Surgical Management

Cho

Kim

2017

Current Topics in Environmental Health and Preventive Medicine

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Distinct clinical and radiographic characteristics of moyamoya disease amongst European Caucasians

Cited by 78 publications

References 25 publications

Characterization of Clinical and Radiological Features of Quasi-Moyamoya Disease among European Caucasians Including Surgical Treatment and Outcome

Characterization of Clinical and Radiological Features of Quasi-Moyamoya Disease among European Caucasians Including Surgical Treatment and Outcome

Incidence of Moyamoya Disease in Denmark: A Population-Based Register Study

Natural History and Long-Term Clinical Outcome After Conservative and Surgical Management

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