{"created":"2023-07-25T08:13:38.899524+00:00","id":14527,"links":{},"metadata":{"_buckets":{"deposit":"35716063-a4b0-405e-8cba-f6ed3b129d26"},"_deposit":{"created_by":18,"id":"14527","owners":[18],"pid":{"revision_id":0,"type":"depid","value":"14527"},"status":"published"},"_oai":{"id":"oai:ir.kagoshima-u.ac.jp:00014527","sets":["57:86"]},"author_link":["132645"],"item_5_biblio_info_5":{"attribute_name":"収録雑誌名","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2018-08-15","bibliographicIssueDateType":"Issued"},"bibliographicPageEnd":"21","bibliographicPageStart":"15","bibliographicVolumeNumber":"391","bibliographic_titles":[{"bibliographic_title":"Journal of the Neurological Sciences","bibliographic_titleLang":"en"}]}]},"item_5_date_granted_54":{"attribute_name":"学位授与年月日 ","attribute_value_mlt":[{"subitem_dategranted":"2018-12-27"}]},"item_5_degree_grantor_53":{"attribute_name":"学位授与機関","attribute_value_mlt":[{"subitem_degreegrantor":[{"subitem_degreegrantor_language":"ja","subitem_degreegrantor_name":"鹿児島大学"}],"subitem_degreegrantor_identifier":[{"subitem_degreegrantor_identifier_name":"17701","subitem_degreegrantor_identifier_scheme":"kakenhi"}]}]},"item_5_degree_name_42":{"attribute_name":"学位名","attribute_value_mlt":[{"subitem_degreename":"博士(医学)","subitem_degreename_language":"ja"},{"subitem_degreename":"Doctor of Philosophy in Medical Science","subitem_degreename_language":"en"}]},"item_5_description_17":{"attribute_name":"ファイル（説明）","attribute_value_mlt":[{"subitem_description":"博士論文全文, 最終試験結果の要旨, 論文審査の要旨, 博士論文要旨","subitem_description_language":"ja","subitem_description_type":"Other"}]},"item_5_description_4":{"attribute_name":"要約(Abstract)","attribute_value_mlt":[{"subitem_description":"Background and purpose: Gerstmann-Sträussler-Scheinker syndrome is a rare hereditary neurodegenerative disorder with clinical heterogeneity. This study is aim to demonstrate the clinical spectrum and radiologic characteristics of patients caused by Pro102Leu mutation in PRNP.\nMaterials and methods: We retrospect clinical manifestations of five patients from four Japanese families, and comprehensively analyzed their brain MRI, SPECT (N-isopropyl-p-[123I] iodoamphetamine), and PET (18F-2-fluoro-2-deoxy-D-glucose) images.\nResults: All patients developed ataxia of lower limbs and trunk, gait disturbance, dysesthesia in legs, and lower limb hyporeflexia. In the early clinical stage before dementia began, no noticeable abnormalities could be observed from brain MRI, but SPECT and PET revealed mosaic-like pattern of blood flow and glucose metabolism of the brain. Predominant abnormalities were found in the occipital and frontal lobes on SPECT and PET analysis, respectively. In SPECT analysis, blood flow of the anterior cerebellar lobes was lower than that of the posterior cerebellar lobes.\nConclusions: Clinical symptoms resulting from failure of dorsal horn of spinal cord and spinocerebellar tracts were observed in all cases. Radiologic findings revealed individual differences of involved region in their brain, which could produce clinical diversity. We identified a downtrend of blood flow in the anterior cerebellar lobes, a projection field of the spinocerebellar tracts, which is an important feature of Gerstmann-Sträussler-Scheinker syndrome.","subitem_description_language":"en","subitem_description_type":"Other"}]},"item_5_dissertation_number_55":{"attribute_name":"学位授与番号","attribute_value_mlt":[{"subitem_dissertationnumber":"甲総研第487号"}]},"item_5_publisher_23":{"attribute_name":"公開者・出版者","attribute_value_mlt":[{"subitem_publisher":"鹿児島大学","subitem_publisher_language":"ja"},{"subitem_publisher":"Journal of the Neurological Sciences","subitem_publisher_language":"en"},{"subitem_publisher":"カゴシマ ダイガク","subitem_publisher_language":"ja-Kana"}]},"item_5_relation_11":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_type_id":{"subitem_relation_type_id_text":"10.1016/j.jns.2018.05.012","subitem_relation_type_select":"DOI"}}]},"item_5_relation_13":{"attribute_name":"情報源","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_text":"Journal of the Neurological Sciences"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://www.elsevier.com/locate/jns","subitem_relation_type_select":"URI"}}]},"item_5_subject_15":{"attribute_name":"NDC","attribute_value_mlt":[{"subitem_subject":"492","subitem_subject_scheme":"NDC"}]},"item_5_text_44":{"attribute_name":"備考","attribute_value_mlt":[{"subitem_text_value":"【指導教員:髙嶋博】"}]},"item_5_text_47":{"attribute_name":"date.appl","attribute_value_mlt":[{"subitem_text_value":"【学位申請日】2018/10/3"}]},"item_5_version_type_14":{"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_access_right":{"attribute_name":"アクセス権","attribute_value_mlt":[{"subitem_access_right":"open access","subitem_access_right_uri":"http://purl.org/coar/access_right/c_abf2"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"吉村, 道由","creatorNameLang":"ja"},{"creatorName":"ヨシムラ, ミチヨシ","creatorNameLang":"ja-Kana"},{"creatorName":"Yoshimura, Michiyoshi","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":"2019-01-31"}],"displaytype":"detail","filename":"Diss_吉村_道由_ISK487_2018.pdf","filesize":[{"value":"1.8 MB"}],"format":"application/pdf","licensetype":"license_11","mimetype":"application/pdf","url":{"label":"Diss_吉村_道由_ISK487_2018","objectType":"fulltext","url":"https://ir.kagoshima-u.ac.jp/record/14527/files/Diss_吉村_道由_ISK487_2018.pdf"},"version_id":"f9d9dc90-5aca-4e66-966a-14934ecb7da2"},{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2019-01-24"}],"displaytype":"detail","filename":"Result_Yoshimura_Michiyoshi_isk_487_2018.pdf","filesize":[{"value":"973.5 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"Result_Yoshimura_Michiyoshi_isk_487_2018","objectType":"other","url":"https://ir.kagoshima-u.ac.jp/record/14527/files/Result_Yoshimura_Michiyoshi_isk_487_2018.pdf"},"version_id":"7432ce10-8029-41c0-b4ea-b57294b19f45"},{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2019-01-24"}],"displaytype":"detail","filename":"Comments_Yoshimura_Michiyoshi_isk_487_2018.pdf","filesize":[{"value":"442.9 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"Comments_Yoshimura_Michiyoshi_isk_487_2018","objectType":"other","url":"https://ir.kagoshima-u.ac.jp/record/14527/files/Comments_Yoshimura_Michiyoshi_isk_487_2018.pdf"},"version_id":"c49fc865-3371-4788-be6f-8d4bc6bb014c"},{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2019-01-31"}],"displaytype":"detail","filename":"Abstract_吉村_道由_4202830239_2018.pdf","filesize":[{"value":"24.7 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"Abstract_吉村_道由_4202830239_2018","objectType":"abstract","url":"https://ir.kagoshima-u.ac.jp/record/14527/files/Abstract_吉村_道由_4202830239_2018.pdf"},"version_id":"a4d47bb7-aa26-48bf-ab59-e88d27563d29"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"eng"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"doctoral thesis","resourceuri":"http://purl.org/coar/resource_type/c_db06"}]},"item_title":"Correlation between clinical and radiologic features of patients with Gerstmann-Sträussler-Scheinker syndrome (Pro102Leu)","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Correlation between clinical and radiologic features of patients with Gerstmann-Sträussler-Scheinker syndrome (Pro102Leu)","subitem_title_language":"en"},{"subitem_title":"Gerstmann-Sträussler-Scheinker症候群(Pro102Leu)の患者の臨床的特徴と放射線学的特徴との相関","subitem_title_language":"ja"}]},"item_type_id":"5","owner":"18","path":["86"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2019-01-24"},"publish_date":"2019-01-24","publish_status":"0","recid":"14527","relation_version_is_last":true,"title":["Correlation between clinical and radiologic features of patients with Gerstmann-Sträussler-Scheinker syndrome (Pro102Leu)"],"weko_creator_id":"18","weko_shared_id":-1},"updated":"2024-01-09T01:48:46.583037+00:00"}