{"_buckets": {"deposit": "2b25a316-18e0-485d-82b7-5112718a45da"}, "_deposit": {"id": "3790", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "3790"}, "status": "published"}, "_oai": {"id": "oai:soar-ir.repo.nii.ac.jp:00003790", "sets": ["461:462"]}, "author_link": ["7054", "7055", "7056", "7057", "7058", "7059", "7060", "7061", "7062", "7063", "7064", "7065", "7066", "7067", "7068", "7069", "7070"], "item_1628147817048": {"attribute_name": "\u51fa\u7248\u30bf\u30a4\u30d7", "attribute_value_mlt": [{"subitem_version_resource": "http://purl.org/coar/version/c_ab4af688f83e57aa", "subitem_version_type": "AM"}]}, "item_6_biblio_info_6": {"attribute_name": "\u66f8\u8a8c\u60c5\u5831", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2009-03", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "1", "bibliographicPageEnd": "51", "bibliographicPageStart": "46", "bibliographicVolumeNumber": "8", "bibliographic_titles": [{"bibliographic_title": "CEREBELLUM"}]}]}, "item_6_description_20": {"attribute_name": "\u6284\u9332", "attribute_value_mlt": [{"subitem_description": "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\u0027 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\u0027s 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.", "subitem_description_type": "Abstract"}]}, "item_6_description_30": {"attribute_name": "\u8cc7\u6e90\u30bf\u30a4\u30d7\uff08\u30b3\u30f3\u30c6\u30f3\u30c4\u306e\u7a2e\u985e\uff09", "attribute_value_mlt": [{"subitem_description": "Article", "subitem_description_type": "Other"}]}, "item_6_description_5": {"attribute_name": "\u5f15\u7528", "attribute_value_mlt": [{"subitem_description": "CEREBELLUM. 8(1):46-51 (2009)", "subitem_description_type": "Other"}]}, "item_6_link_3": {"attribute_name": "\u4fe1\u5dde\u5927\u5b66\u7814\u7a76\u8005\u7dcf\u89a7\u3078\u306e\u30ea\u30f3\u30af", "attribute_value_mlt": [{"subitem_link_text": "Yoshida, Kunihiro", "subitem_link_url": "http://soar-rd.shinshu-u.ac.jp/profile/ja.yhfNHFkh.html"}, {"subitem_link_text": "Morita, Hiroshi", "subitem_link_url": "http://soar-rd.shinshu-u.ac.jp/profile/ja.HUyNHhkh.html"}, {"subitem_link_text": "Shimojima, Yasuhiro", "subitem_link_url": "http://soar-rd.shinshu-u.ac.jp/profile/ja.ghTmHVkh.html"}]}, "item_6_link_67": {"attribute_name": "WoS", "attribute_value_mlt": [{"subitem_link_text": "Web of Science", "subitem_link_url": "http://gateway.isiknowledge.com/gateway/Gateway.cgi?\u0026GWVersion=2\u0026SrcAuth=ShinshuUniv\u0026SrcApp=ShinshuUniv\u0026DestLinkType=FullRecord\u0026DestApp=WOS\u0026KeyUT=000263830500007"}]}, "item_6_publisher_4": {"attribute_name": "\u51fa\u7248\u8005", "attribute_value_mlt": [{"subitem_publisher": "SPRINGER"}]}, "item_6_relation_47": {"attribute_name": "PubMed", "attribute_value_mlt": [{"subitem_relation_name": [{"subitem_relation_name_text": "18855094"}], "subitem_relation_type_id": {"subitem_relation_type_id_text": "https://pubmed.ncbi.nlm.nih.gov/18855094", "subitem_relation_type_select": "PMID"}}]}, "item_6_relation_48": {"attribute_name": "DOI", "attribute_value_mlt": [{"subitem_relation_name": [{"subitem_relation_name_text": "10.1007/s12311-008-0062-8"}], "subitem_relation_type_id": {"subitem_relation_type_id_text": "https://doi.org/10.1007/s12311-008-0062-8", "subitem_relation_type_select": "DOI"}}]}, "item_6_rights_62": {"attribute_name": "\u6a29\u5229", "attribute_value_mlt": [{"subitem_rights": "The final publication is available at www.springerlink.com"}]}, "item_6_select_64": {"attribute_name": "\u8457\u8005\u7248\u30d5\u30e9\u30b0", "attribute_value_mlt": [{"subitem_select_item": "author"}]}, "item_6_source_id_35": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "1473-4222", "subitem_source_identifier_type": "PISSN"}]}, "item_6_source_id_39": {"attribute_name": "NII ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "1473-4222", "subitem_source_identifier_type": "PISSN"}]}, "item_6_source_id_40": {"attribute_name": "\u66f8\u8a8c\u30ec\u30b3\u30fc\u30c9ID", "attribute_value_mlt": [{"subitem_source_identifier": "AA11691452", "subitem_source_identifier_type": "NCID"}]}, "item_6_text_69": {"attribute_name": "wosonly authkey", "attribute_value_mlt": [{"subitem_text_value": "Cerebellar ataxia; 16q-ADCA; SCA6; ICARS; SARA; Puratrophin-1"}]}, "item_6_text_70": {"attribute_name": "wosonly keywords", "attribute_value_mlt": [{"subitem_text_value": "FAMILIAL AMYLOID POLYNEUROPATHY; CALCIUM-CHANNEL GENE; CAG REPEAT EXPANSION; ADCA TYPE-III; SPINOCEREBELLAR ATAXIA; CLINICAL-FEATURES; CHROMOSOME 16Q22.1; RATING-SCALE; POPULATION; LOCUS"}]}, "item_6_textarea_68": {"attribute_name": "wosonly abstract", "attribute_value_mlt": [{"subitem_textarea_value": "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\u0027 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\u0027s 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."}]}, "item_creator": {"attribute_name": "\u8457\u8005", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Yoshida,  Kunihiro", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7054", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Shimizu,  Yusaku", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7055", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Morita,  Hiroshi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7056", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Okano,  Tomomi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7057", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Sakai,  Haruya", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7058", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Ohata,  Takako", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7059", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Matsumoto,  Naomichi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7060", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Nakamura,  Katsuya", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7061", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Tazawa,  Ko-ichi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7062", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Ohara,  Shinji", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7063", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Tabata,  Kenichi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7064", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Inoue,  Atsushi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7065", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Sato,  Shunichi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7066", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Shimojima,  Yasuhiro", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7067", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Hattori,  Takeshi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7068", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Ushiyama,  Masao", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7069", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Ikeda,  Shu-ichi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7070", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "\u30d5\u30a1\u30a4\u30eb\u60c5\u5831", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2015-09-24"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "Severity_progression_rate_cerebellar_ataxia_16q-linked_autosomal_dominant_cerebellar_ataxia.pdf", "filesize": [{"value": "679.2 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_note", "mimetype": "application/pdf", "size": 679200.0, "url": {"label": "Severity_progression_rate_cerebellar_ataxia_16q-linked_autosomal_dominant_cerebellar_ataxia.pdf", "url": "https://soar-ir.repo.nii.ac.jp/record/3790/files/Severity_progression_rate_cerebellar_ataxia_16q-linked_autosomal_dominant_cerebellar_ataxia.pdf"}, "version_id": "a79f0e83-781a-49ef-bda4-924fe15dd71c"}]}, "item_keyword": {"attribute_name": "\u30ad\u30fc\u30ef\u30fc\u30c9", "attribute_value_mlt": [{"subitem_subject": "Cerebellar ataxia", "subitem_subject_scheme": "Other"}, {"subitem_subject": "16q-ADCA", "subitem_subject_scheme": "Other"}, {"subitem_subject": "SCA6", "subitem_subject_scheme": "Other"}, {"subitem_subject": "ICARS", "subitem_subject_scheme": "Other"}, {"subitem_subject": "SARA", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Puratrophin-1", "subitem_subject_scheme": "Other"}]}, "item_language": {"attribute_name": "\u8a00\u8a9e", "attribute_value_mlt": [{"subitem_language": "eng"}]}, "item_resource_type": {"attribute_name": "\u8cc7\u6e90\u30bf\u30a4\u30d7", "attribute_value_mlt": [{"resourcetype": "journal article", "resourceuri": "http://purl.org/coar/resource_type/c_6501"}]}, "item_title": "Severity and Progression Rate of Cerebellar Ataxia in 16q-linked Autosomal Dominant Cerebellar Ataxia (16q-ADCA) in the Endemic Nagano Area of Japan", "item_titles": {"attribute_name": "\u30bf\u30a4\u30c8\u30eb", "attribute_value_mlt": [{"subitem_title": "Severity and Progression Rate of Cerebellar Ataxia in 16q-linked Autosomal Dominant Cerebellar Ataxia (16q-ADCA) in the Endemic Nagano Area of Japan", "subitem_title_language": "en"}]}, "item_type_id": "6", "owner": "1", "path": ["461/462"], "permalink_uri": "http://hdl.handle.net/10091/16901", "pubdate": {"attribute_name": "PubDate", "attribute_value": "2013-04-01"}, "publish_date": "2013-04-01", "publish_status": "0", "recid": "3790", "relation": {}, "relation_version_is_last": true, "title": ["Severity and Progression Rate of Cerebellar Ataxia in 16q-linked Autosomal Dominant Cerebellar Ataxia (16q-ADCA) in the Endemic Nagano Area of Japan"], "weko_shared_id": -1}