{"created":"2023-05-15T11:33:03.638532+00:00","id":3474,"links":{},"metadata":{"_buckets":{"deposit":"0f2fcdae-bb57-4b6d-a80e-3e20b7cecb6e"},"_deposit":{"created_by":1,"id":"3474","owners":[1],"pid":{"revision_id":0,"type":"depid","value":"3474"},"status":"published"},"_oai":{"id":"oai:nsg.repo.nii.ac.jp:00003474","sets":["6:10:11:95"]},"author_link":["12899","12901","12900","12898","12897","12902"],"item_19_biblio_info_8":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2003-12","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"2","bibliographicPageEnd":"110","bibliographicPageStart":"104","bibliographicVolumeNumber":"3","bibliographic_titles":[{"bibliographic_title":"新潟医療福祉学会誌"}]}]},"item_19_description_16":{"attribute_name":"フォーマット","attribute_value_mlt":[{"subitem_description":"application/pdf","subitem_description_type":"Other"}]},"item_19_description_45":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"subitem_description":"論文(Article)","subitem_description_type":"Other"}]},"item_19_description_6":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"筋強直性ジストロフィー(以下Mypとする)は第19番染色体長腕にあるMyD原因遺伝子のCTGリピート数の異常増大により発症する常染色体優性遺伝の疾患である。本症では高頻度に中枢神経障害を伴うことが知られており、記憶力や注意力、遂行機能能力、構成能力などの高次脳機能障害がみられる。今回、本症例7例について海馬領域が陳述記憶の中枢として重要であることから頭部MRIで海馬領域の計測を行い、その計測結果と日本語版ウェクスラー記憶検査との比較及び正常コントロール群の計測結果との比較を行った。同時に大脳萎縮についても計測、比較した。さらに知能検査、記憶検査、事象関連電位などとCTGリピート数との関連についも検討した。結論としてMyD群の海馬領域計測では記憶の様式特異性障害が示され、さらに海馬領域を含めた大脳全体の萎縮が示された。また事象関連電位P300とCTGリピートの増大との相関が新たに示された。\n","subitem_description_type":"Abstract"},{"subitem_description":"Myotonic dystrophy (Myp) is an autosomaI dominantly inherited disease. This abnormal gepe for Myp is localized to chromosome 19ql3.3 as an expansion of CTG repeat. There is a correIation between the number of CTG repeats and the severity of clinical symptoms. MyD has been noticed accompanying CNS disorders such as disturbance of memory, attention, executive function and construction. We measured cerebral and hippocampal areas of 7 patients with MyD by MRI Hippocampus is one of important areas of declarative memory, therefore it was examined if there was a correlation between the hippocampaI areas of the patients and the scores of Wechsler Memory Scale-Revised (WMS-R). WechsIer Adult Intelligent Scale-Revised (WAIS-R) and event-related potentia1 P300 were also performed for the patients. Then we anaIyzed a correlation to the number of CTG repeats and each datum. In conclusion, an expansion of CTG repeat was correIated to onset, WMS-R andP300 in the patients. And diffuse brain atrophy was observed and correlated to WAIS-R. Also, the degree of hippocampaI atrophy was significantly correIated to the score of WMS-R, especially verbaI memory tests. It was first found that there was a significant correlation between the latency of P300 and the number of CTG repeats in MyD. These results suggest that CTG expansion and brain atrophy relate to disorder of higher brain function in MyD.","subitem_description_type":"Abstract"}]},"item_19_publisher_35":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"新潟医療福祉学会"}]},"item_19_rights_14":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"本文データは国立情報学研究所により電子化されたものである"}]},"item_19_source_id_11":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AA11614531","subitem_source_identifier_type":"NCID"}]},"item_19_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"1346-8774","subitem_source_identifier_type":"ISSN"}]},"item_19_text_1":{"attribute_name":"Title in Japanese","attribute_value_mlt":[{"subitem_text_value":"筋強直性ジストロフィーの高次脳機能障害の検討"}]},"item_19_text_2":{"attribute_name":"タイトル（英）","attribute_value_mlt":[{"subitem_text_language":"en","subitem_text_value":"Disorder of Higher Brain Function in Myotonlc Dystrophy"}]},"item_19_text_47":{"attribute_name":"資源タイプ・NII","attribute_value_mlt":[{"subitem_text_value":"Journal Article"}]},"item_19_text_48":{"attribute_name":"資源タイプ・DCMI","attribute_value_mlt":[{"subitem_text_value":"text"}]},"item_19_version_type_18":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"工藤, 由理"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"今村, 徹"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"近藤, 浩"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Kudo, Yuri","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Imamura, Toru","creatorNameLang":"en"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Kondo, Hiroshi","creatorNameLang":"en"}],"nameIdentifiers":[{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2019-10-30"}],"displaytype":"detail","filename":"KJ00004093891.pdf","filesize":[{"value":"666.6 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"KJ00004093891.pdf","url":"https://nsg.repo.nii.ac.jp/record/3474/files/KJ00004093891.pdf"},"version_id":"02596dce-89f5-41cd-8faa-63cdbc7bfdac"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"筋緊張性ジストロフィー","subitem_subject_scheme":"Other"},{"subitem_subject":"CTGリピート","subitem_subject_scheme":"Other"},{"subitem_subject":"高次脳機能","subitem_subject_scheme":"Other"},{"subitem_subject":"事象関連電位P300","subitem_subject_scheme":"Other"},{"subitem_subject":"様式特異性記憶障害","subitem_subject_scheme":"Other"},{"subitem_subject":"myotonic dystrophy","subitem_subject_scheme":"Other"},{"subitem_subject":"CTG repeat","subitem_subject_scheme":"Other"},{"subitem_subject":"higher brain function","subitem_subject_scheme":"Other"},{"subitem_subject":"event related potential P300","subitem_subject_scheme":"Other"},{"subitem_subject":"modality sp ecific memory disturbance","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"jpn"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"journal article","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"筋強直性ジストロフィーの高次脳機能障害の検討","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"筋強直性ジストロフィーの高次脳機能障害の検討"},{"subitem_title":"Disorder of Higher Brain Function in Myotonlc Dystrophy","subitem_title_language":"en"}]},"item_type_id":"19","owner":"1","path":["95"],"pubdate":{"attribute_name":"公開日","attribute_value":"2010-03-05"},"publish_date":"2010-03-05","publish_status":"0","recid":"3474","relation_version_is_last":true,"title":["筋強直性ジストロフィーの高次脳機能障害の検討"],"weko_creator_id":"1","weko_shared_id":1},"updated":"2023-05-15T12:08:56.214006+00:00"}