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Inherited or acquired disorders and conditions may present in conjunction with MMD. This condition is known as quasi-moyamoya disease. To identify the clinical features of quasi-MMD in Japan, a nationwide survey was conducted. Patients and methods: A questionnaire was mailed directly to 241 departments that had acknowledged treating quasi-MMD patients in the primary survey. We ascertained the sex, age, family history, clinical manifestation, radiological findings, treatments, course of the disease, and daily activity of the patients. Results: A total of 114 departments replied to the questionnaire. The data of 108 patients (66 female and 42 male; female to male ratio 1.57) were registered and analyzed. Mean age was 30.6 years old, with a peak in children. Seven patients (7 %) exhibited familial MMD. The initial clinical manifestation was motor weakness, followed by transient ischemic attack and headache. 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Nationwide survey on quasi-moyamoya disease in Japan
http://hdl.handle.net/10069/34711
http://hdl.handle.net/10069/347118ecabe98-a417-4d08-918b-269ffa2b2352
名前 / ファイル | ライセンス | アクション |
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ActNeu156_935.pdf (261.1 kB)
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2015-06-01 | |||||
タイトル | ||||||
タイトル | Nationwide survey on quasi-moyamoya disease in Japan | |||||
言語 | ||||||
言語 | eng | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Epidemiology | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Moyamoya disease | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Nationwide survey | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Quasi-moyamoya disease | |||||
資源タイプ | ||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
資源タイプ | journal article | |||||
著者 |
Hayashi, Kentaro
× Hayashi, Kentaro× Horie, Nobutaka× Izumo, Tsuyoshi× Nagata, Izumi |
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抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | Objectives: Moyamoya disease (MMD) is a unique occlusive disease of the bilateral internal carotid arteries with moyamoya vessels. Inherited or acquired disorders and conditions may present in conjunction with MMD. This condition is known as quasi-moyamoya disease. To identify the clinical features of quasi-MMD in Japan, a nationwide survey was conducted. Patients and methods: A questionnaire was mailed directly to 241 departments that had acknowledged treating quasi-MMD patients in the primary survey. We ascertained the sex, age, family history, clinical manifestation, radiological findings, treatments, course of the disease, and daily activity of the patients. Results: A total of 114 departments replied to the questionnaire. The data of 108 patients (66 female and 42 male; female to male ratio 1.57) were registered and analyzed. Mean age was 30.6 years old, with a peak in children. Seven patients (7 %) exhibited familial MMD. The initial clinical manifestation was motor weakness, followed by transient ischemic attack and headache. Their imaging study type included ischemic type in 64 patients (63.4 %), bleeding type in seven (6.9 %), and normal in 27 (26.7 %). Stenoocclusive lesion was seen in the internal carotid artery in more than half of the patients. Development of moyamoya vessels was mild in approximately 40 % of the patients. Almost all cases were accompanied by cerebral hypoperfusion. About half of them were unilateral lesion. Vascular reconstruction was employed for the approximately 60 % patients. The prognosis did not change significantly. Conclusion: Clinical features of quasi-MMD were revealed in the nationwide study. In quasi-MMD, unilateral lesion is dominant, and the development of moyamoya vessels and intracranial hemorrhage are less dominant. | |||||
書誌情報 |
Acta Neurochirurgica 巻 156, 号 5, p. 935-940, 発行日 2014-05 |
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出版者 | ||||||
出版者 | Springer Wien | |||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 00016268 | |||||
EISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 09420940 | |||||
DOI | ||||||
関連タイプ | isVersionOf | |||||
識別子タイプ | DOI | |||||
関連識別子 | 10.1007/s00701-014-2013-0 | |||||
権利 | ||||||
権利情報 | c 2014 Springer-Verlag. | |||||
権利 | ||||||
権利情報 | The final publication is available at link.springer.com | |||||
著者版フラグ | ||||||
出版タイプ | AM | |||||
出版タイプResource | http://purl.org/coar/version/c_ab4af688f83e57aa | |||||
引用 | ||||||
内容記述タイプ | Other | |||||
内容記述 | Acta Neurochirurgica, 156(5), pp.935-940; 2014 |