{"created":"2021-03-01T06:40:14.427425+00:00","id":33796,"links":{},"metadata":{"_buckets":{"deposit":"b52d065a-ec7f-4ce0-9e8a-429d2894b814"},"_deposit":{"id":"33796","owners":[],"pid":{"revision_id":0,"type":"depid","value":"33796"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00033796","sets":["453:456","471:537:538:1997"]},"item_7_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"Clinicopathological Analysis of Amyotrophic Lateral Sclerosis with Pallido-Nigro-Luysian Degeneration"}]},"item_7_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2019-01","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"31","bibliographicPageStart":"21","bibliographicVolumeNumber":"133","bibliographic_titles":[{"bibliographic_title":"新潟医学会雑誌"},{"bibliographic_title":"NIIGATA MEDICAL JOURNAL","bibliographic_titleLang":"en"}]}]},"item_7_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"　筋萎縮性側索硬化症(amyotrophic lateral sclerosis: ALS) は，主として上位・下位運動神経細胞が侵され、進行性の筋力低下を来たす神経変性疾患であり，病理学的にはTAR DNAbindingprotein of 43kDa (TDP-43) 陽性の神経細胞・グリア細胞細胞質内封人体を特徴とする．また，淡蒼球，黒質，視床下核は大脳皮質と大脳基底核を結ぶ神経路の主要な構成要素として運動機能の制御に関与しpallido-nigro-luysian (PNL) 系と呼ばれる．通常ALSではPNL系は障害されにくい部位であるが，運動神経系・非運動神経系を含めた多系統変性を示す症例ではPNLも変性し得る．その一方，文献的には運動神経系とPNL系のみが選択的に障害される例も少数報告されている．このようにPNL系に変性を生じるALS (ALS withPNL degeneration: ALS with PNLD) は比較的稀ながら存在するが，その網羅的な解析はなされておらず，臨床病理像，特にPNL系が選択的に障害される症例と非選択的に障害される症例の相違は不明である．\n　本研究では孤発性ALS剖検例97例の中からALS with PNLD を抽出し，臨床病理像とTDP-43 病理所見を解析した. ALS with PNLD は97例中11例(11.3%)であり，変性分布から2群（限局型5例，広範型6例）に分類された．広範型は運動神経系やPNL系を含めた多系統に変性が及び，非選択的にPNL系が変性している可能性があった．一方，限局型では運動神経系とPNL系に変性がほぼ限局していた．限局型ではPNL変性を伴わないALS (ALSwithout PNLD)と比較して発症年齢が低く，また錐体外路症状で発症した症例や上位運動神経症状が乏しい症例が含まれていた．限局型，広範型ともにPNL系にリン酸化TDP-43陽性構造物が認められ，その出現量はALS without PNLDと比較して有意に多かった限局型では運動野以外の大脳皮質，海馬歯状回の顆粒細胞にリン酸化TDP-43 陽性構造物がほとんど認められず， リン酸化TDP-43の広がり方はBrettschneiderらが提唱した通常のALSにおけるリン酸化TDP-43の伝播経路とは異なっていることが推測された. ALS with PNLDの中でも特に限局型は， PNL系と運動神経系が選択的に障害され，特徴的な臨床病理像とリン酸化TDP-43伝播様式を示すALSの一群である可能性が示唆された．","subitem_description_type":"Abstract"}]},"item_7_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"177795","nameIdentifierScheme":"WEKO"}],"names":[{"name":"ITO, Ayako"}]}]},"item_7_publisher_7":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"新潟医学会"}]},"item_7_select_19":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_select_item":"publisher"}]},"item_7_source_id_11":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AA00182415","subitem_source_identifier_type":"NCID"}]},"item_7_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"00290440","subitem_source_identifier_type":"ISSN"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"伊藤, 絢子"}],"nameIdentifiers":[{"nameIdentifier":"58065","nameIdentifierScheme":"WEKO"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_access","displaytype":"detail","filename":"133（1）_21-31.pdf","filesize":[{"value":"7.7 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"url":"https://niigata-u.repo.nii.ac.jp/record/33796/files/133（1）_21-31.pdf"},"version_id":"0e19bcd6-7e34-42fc-acc1-6bac489d38e3"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"筋萎縮性側索硬化症","subitem_subject_scheme":"Other"},{"subitem_subject":"淡蒼球黒質ルイ体変性","subitem_subject_scheme":"Other"},{"subitem_subject":"TDP-43","subitem_subject_scheme":"Other"},{"subitem_subject":"錐体外路症状","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":"departmental bulletin paper","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"淡蒼球・黒質・視床下核の変性を伴う筋萎縮性側索硬化症の臨床病理学的検討","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"淡蒼球・黒質・視床下核の変性を伴う筋萎縮性側索硬化症の臨床病理学的検討"},{"subitem_title":"Clinicopathological Analysis of Amyotrophic Lateral Sclerosis with Pallido-Nigro-Luysian Degeneration","subitem_title_language":"en"}]},"item_type_id":"7","owner":"1","path":["456","1997"],"pubdate":{"attribute_name":"公開日","attribute_value":"2020-04-17"},"publish_date":"2020-04-17","publish_status":"0","recid":"33796","relation_version_is_last":true,"title":["淡蒼球・黒質・視床下核の変性を伴う筋萎縮性側索硬化症の臨床病理学的検討"],"weko_creator_id":"1","weko_shared_id":2},"updated":"2022-12-15T04:04:03.534623+00:00"}