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Severity and Progression Rate of Cerebellar Ataxia in 16q-linked Autosomal Dominant Cerebellar Ataxia (16q-ADCA) in the Endemic Nagano Area of Japan
http://hdl.handle.net/10091/16901
http://hdl.handle.net/10091/1690188ace44c-fca3-48ff-8fe7-19f8ec22b61a
名前 / ファイル | ライセンス | アクション |
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2013-04-01 | |||||
タイトル | ||||||
言語 | en | |||||
タイトル | Severity and Progression Rate of Cerebellar Ataxia in 16q-linked Autosomal Dominant Cerebellar Ataxia (16q-ADCA) in the Endemic Nagano Area of Japan | |||||
言語 | ||||||
言語 | eng | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Cerebellar ataxia | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | 16q-ADCA | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | SCA6 | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | ICARS | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | SARA | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Puratrophin-1 | |||||
資源タイプ | ||||||
資源 | http://purl.org/coar/resource_type/c_6501 | |||||
タイプ | journal article | |||||
著者 |
Yoshida, Kunihiro
× Yoshida, Kunihiro× Shimizu, Yusaku× Morita, Hiroshi× Okano, Tomomi× Sakai, Haruya× Ohata, Takako× Matsumoto, Naomichi× Nakamura, Katsuya× Tazawa, Ko-ichi× Ohara, Shinji× Tabata, Kenichi× Inoue, Atsushi× Sato, Shunichi× Shimojima, Yasuhiro× Hattori, Takeshi× Ushiyama, Masao× Ikeda, Shu-ichi |
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信州大学研究者総覧へのリンク | ||||||
氏名 | Yoshida, Kunihiro | |||||
URL | http://soar-rd.shinshu-u.ac.jp/profile/ja.yhfNHFkh.html | |||||
信州大学研究者総覧へのリンク | ||||||
氏名 | Morita, Hiroshi | |||||
URL | http://soar-rd.shinshu-u.ac.jp/profile/ja.HUyNHhkh.html | |||||
信州大学研究者総覧へのリンク | ||||||
氏名 | Shimojima, Yasuhiro | |||||
URL | http://soar-rd.shinshu-u.ac.jp/profile/ja.ghTmHVkh.html | |||||
出版者 | ||||||
出版者 | SPRINGER | |||||
引用 | ||||||
内容記述タイプ | Other | |||||
内容記述 | CEREBELLUM. 8(1):46-51 (2009) | |||||
書誌情報 |
CEREBELLUM 巻 8, 号 1, p. 46-51, 発行日 2009-03 |
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抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | 16q22.1-linked autosomal dominant cerebellar ataxia (16q-ADCA) is a recently defined subtype of ADCA identified by a disease-specific C/T substitution in the 5' untranslated region of the puratrophin-1 gene. In Nagano, the central mountainous district of the main island of Japan, 16q-ADCA and spinocerebellar ataxia type 6 (SCA6) are the most and second most prevalent subtypes of ADCA, respectively. Both subtypes are classified into Harding's ADCA III, but little attention has been given to the differences in the severity and progression rate of cerebellar ataxia between 16q-ADCA and SCA6. We investigated the clinical severity and progression rate of cerebellar ataxia of 16q-ADCA patients using international cooperative ataxia rating scale and scale for the assessment and rating of ataxia and compared them with those of SCA6 patients. The age at onset was much higher in 16q-ADCA patients (60.1 +/- 9.8 years, n = 66) than in SCA6 patients (41.1 +/- 8.7 years, n = 35). Clinical features of 16q-ADCA were basically consistent with pure cerebellar ataxia, as well as in SCA6, but gaze-evoked nystagmus was observed less frequently in 16q-ADCA patients than in SCA6 patients. When compared at almost the same disease duration after onset, the severity of cerebellar ataxia was a little higher, and the progression rate seemed more rapid in 16q-ADCA patients than in SCA6 patients, but the differences were not significant. | |||||
資源タイプ(コンテンツの種類) | ||||||
内容記述タイプ | Other | |||||
内容記述 | Article | |||||
ISSN | ||||||
収録物識別子タイプ | PISSN | |||||
収録物識別子 | 1473-4222 | |||||
書誌レコードID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AA11691452 | |||||
PubMed | ||||||
識別子タイプ | PMID | |||||
関連識別子 | https://pubmed.ncbi.nlm.nih.gov/18855094 | |||||
関連名称 | 18855094 | |||||
DOI | ||||||
識別子タイプ | DOI | |||||
関連識別子 | https://doi.org/10.1007/s12311-008-0062-8 | |||||
関連名称 | 10.1007/s12311-008-0062-8 | |||||
権利 | ||||||
権利情報 | The final publication is available at www.springerlink.com | |||||
出版タイプ | ||||||
出版タイプ | AM | |||||
出版タイプResource | http://purl.org/coar/version/c_ab4af688f83e57aa | |||||
WoS | ||||||
表示名 | Web of Science | |||||
URL | http://gateway.isiknowledge.com/gateway/Gateway.cgi?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000263830500007 |