{"created":"2024-12-12T01:19:01.950065+00:00","id":2009150,"links":{},"metadata":{"_buckets":{"deposit":"cc47d02d-e750-4640-98fa-369fe51d30be"},"_deposit":{"created_by":7,"id":"2009150","owners":[7],"pid":{"revision_id":0,"type":"depid","value":"2009150"},"status":"published"},"_oai":{"id":"oai:tokushima-u.repo.nii.ac.jp:02009150","sets":["1713853213384:1713853295607"]},"author_link":["573","1623","1589","530","576","724","1622","396"],"control_number":"2009150","item_10001_alternative_title_1":{"attribute_name":"タイトル別表記","attribute_value_mlt":[{"subitem_alternative_title":"MRS of HDLS and CSF1R Mutation Carrier","subitem_alternative_title_language":"en"}]},"item_10001_biblio_info_7":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2016-12-26","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"4","bibliographicPageEnd":"303","bibliographicPageStart":"297","bibliographicVolumeNumber":"16","bibliographic_titles":[{"bibliographic_title":"Magnetic Resonance in Medical Sciences","bibliographic_titleLang":"en"}]}]},"item_10001_description_5":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Purpose: Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is a rare neurodegenerative disorder with various clinical presentations. Mutation of the colony-stimulating factor 1 receptor (CSF1R) gene is considered to be a cause of this autosomal dominant disorder. The purpose of this study was to report magnetic resonance spectroscopy (MRS) findings in patients with HDLS and asymptomatic carriers and to clarify the use of MRS in this disease.\nMaterials and Methods: In this retrospective, institutional review board-approved study, we included four consecutive patients, genetically diagnosed with HDLS, and two asymptomatic carriers after acquiring informed consent. We performed single-voxel MRS of the left centrum semiovale on a 3T clinical scanner. We also included a sex-matched normal dataset. We quantified N-acetylaspartate (NAA), creatine, choline-containing compounds (Cho), glutamine, glutamate (Glu), myo-inositol (Ins), glutathione, lactate (Lac), and gamma-amino butyric acid using LCModel. We performed statistical analysis, and P value <0.05 was considered significant.\nResults: In HDLS cases, MRS revealed decreased NAA and Glu concentrations, which probably reflected neuronal damage and/or loss, and a subsequent reduction of neurotransmitters. A patient with HDLS also had increased Cho and Ins concentrations, indicating gliosis, and increased Cho concentration was also observed in an asymptomatic carrier. This suggests that metabolic changes had already occurred in an asymptomatic state.\nConclusion: We demonstrated changes in metabolite concentrations not only in patients with HDLS but also in asymptomatic CSF1R mutation carriers. Our study indicates that MRS is a potentially useful tool for the analysis of metabolic and pathophysiological findings of HDLS, even during the early stages of disease.","subitem_description_language":"en","subitem_description_type":"Abstract"}]},"item_10001_publisher_8":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"Japanese Society for Magnetic Resonance in Medicine","subitem_publisher_language":"en"}]},"item_10001_rights_15":{"attribute_name":"権利情報","attribute_value_mlt":[{"subitem_rights":"This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives International License.","subitem_rights_language":"en"}]},"item_10001_source_id_9":{"attribute_name":"収録物ID","attribute_value_mlt":[{"subitem_source_identifier":"18802206","subitem_source_identifier_type":"ISSN"},{"subitem_source_identifier":"13473182","subitem_source_identifier_type":"ISSN"},{"subitem_source_identifier":"AA11648770","subitem_source_identifier_type":"NCID"}]},"item_10001_version_type_20":{"attribute_name":"出版タイプ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_1715043197608":{"attribute_name":"アクセス権","attribute_value_mlt":[{"subitem_access_right":"open access"}]},"item_1722929371688":{"attribute_name":"出版社版DOI","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_language":"ja","subitem_relation_name_text":"10.2463/mrms.mp.2016-0016"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://doi.org/10.2463/mrms.mp.2016-0016","subitem_relation_type_select":"DOI"}}]},"item_1723180141928":{"attribute_name":"EID","attribute_value_mlt":[{"subitem_identifier_type":"URI","subitem_identifier_uri":"328253"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"阿部, 考志","creatorNameLang":"ja"},{"creatorName":"アベ, タカシ","creatorNameLang":"ja-Kana"},{"creatorName":"Abe, Takashi","creatorNameLang":"en"}],"familyNames":[{"familyName":"阿部","familyNameLang":"ja"},{"familyName":"アベ","familyNameLang":"ja-Kana"},{"familyName":"Abe","familyNameLang":"en"}],"givenNames":[{"givenName":"考志","givenNameLang":"ja"},{"givenName":"タカシ","givenNameLang":"ja-Kana"},{"givenName":"Takashi","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"576","nameIdentifierScheme":"WEKO"},{"nameIdentifier":"40645347","nameIdentifierScheme":"e-Rad","nameIdentifierURI":"https://nrid.nii.ac.jp/ja/search/?qm=40645347"}]},{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"瓦井, 俊孝","creatorNameLang":"ja"},{"creatorName":"カワライ, トシタカ","creatorNameLang":"ja-Kana"},{"creatorName":"Kawarai, Toshitaka","creatorNameLang":"en"}],"familyNames":[{"familyName":"瓦井","familyNameLang":"ja"},{"familyName":"カワライ","familyNameLang":"ja-Kana"},{"familyName":"Kawarai","familyNameLang":"en"}],"givenNames":[{"givenName":"俊孝","givenNameLang":"ja"},{"givenName":"トシタカ","givenNameLang":"ja-Kana"},{"givenName":"Toshitaka","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"530","nameIdentifierScheme":"WEKO"},{"nameIdentifier":"50614137","nameIdentifierScheme":"e-Rad","nameIdentifierURI":"https://nrid.nii.ac.jp/ja/search/?qm=50614137"}]},{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"藤田, 浩司","creatorNameLang":"ja"},{"creatorName":"フジタ, コウジ","creatorNameLang":"ja-Kana"},{"creatorName":"Fujita, Kouji","creatorNameLang":"en"}],"familyNames":[{"familyName":"藤田","familyNameLang":"ja"},{"familyName":"フジタ","familyNameLang":"ja-Kana"},{"familyName":"Fujita","familyNameLang":"en"}],"givenNames":[{"givenName":"浩司","givenNameLang":"ja"},{"givenName":"コウジ","givenNameLang":"ja-Kana"},{"givenName":"Kouji","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"573","nameIdentifierScheme":"WEKO"},{"nameIdentifier":"246775/profile-ja.html","nameIdentifierScheme":"徳島大学 教育研究者総覧","nameIdentifierURI":"http://pub2.db.tokushima-u.ac.jp/ERD/person/246775/profile-ja.html"},{"nameIdentifier":"80601765","nameIdentifierScheme":"e-Rad","nameIdentifierURI":"https://nrid.nii.ac.jp/ja/search/?qm=80601765"}]},{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"佐光, 亘","creatorNameLang":"ja"},{"creatorName":"サコウ, ワタル","creatorNameLang":"ja-Kana"},{"creatorName":"Sako, Wataru","creatorNameLang":"en"}],"familyNames":[{"familyName":"佐光","familyNameLang":"ja"},{"familyName":"サコウ","familyNameLang":"ja-Kana"},{"familyName":"Sako","familyNameLang":"en"}],"givenNames":[{"givenName":"亘","givenNameLang":"ja"},{"givenName":"ワタル","givenNameLang":"ja-Kana"},{"givenName":"Wataru","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"724","nameIdentifierScheme":"WEKO"},{"nameIdentifier":"60581155","nameIdentifierScheme":"e-Rad","nameIdentifierURI":"https://nrid.nii.ac.jp/ja/search/?qm=60581155"}]},{"creatorNames":[{"creatorName":"寺澤, 由佳","creatorNameLang":"ja"},{"creatorName":"テラサワ, ユカ","creatorNameLang":"ja-Kana"},{"creatorName":"Terasawa, Yuka","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"マツダ, ツヨシ","creatorNameLang":"ja"},{"creatorName":"マツダ, ツヨシ","creatorNameLang":"ja-Kana"},{"creatorName":"Matsuda, Tsuyoshi","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"サカイ, ワカ","creatorNameLang":"ja"},{"creatorName":"サカイ, ワカ","creatorNameLang":"ja-Kana"},{"creatorName":"Sakai, Waka","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"宮城, 愛","creatorNameLang":"ja"},{"creatorName":"ツカモト ミヤシロ, アイ","creatorNameLang":"ja-Kana"},{"creatorName":"Tsukamoto-Miyashiro, Ai","creatorNameLang":"en"}]},{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"松井, 尚子","creatorNameLang":"ja"},{"creatorName":"マツイ, ナオコ","creatorNameLang":"ja-Kana"},{"creatorName":"Matsui, Naoko","creatorNameLang":"en"}],"familyNames":[{"familyName":"松井","familyNameLang":"ja"},{"familyName":"マツイ","familyNameLang":"ja-Kana"},{"familyName":"Matsui","familyNameLang":"en"}],"givenNames":[{"givenName":"尚子","givenNameLang":"ja"},{"givenName":"ナオコ","givenNameLang":"ja-Kana"},{"givenName":"Naoko","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"396","nameIdentifierScheme":"WEKO"},{"nameIdentifier":"193494/profile-ja.html","nameIdentifierScheme":"徳島大学 教育研究者総覧","nameIdentifierURI":"http://pub2.db.tokushima-u.ac.jp/ERD/person/193494/profile-ja.html"},{"nameIdentifier":"10547954","nameIdentifierScheme":"e-Rad","nameIdentifierURI":"https://nrid.nii.ac.jp/ja/search/?qm=10547954"}]},{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"和泉, 唯信","creatorNameLang":"ja"},{"creatorName":"イズミ, ユイシン","creatorNameLang":"ja-Kana"},{"creatorName":"Izumi, Yuishin","creatorNameLang":"en"}],"familyNames":[{"familyName":"和泉","familyNameLang":"ja"},{"familyName":"イズミ","familyNameLang":"ja-Kana"},{"familyName":"Izumi","familyNameLang":"en"}],"givenNames":[{"givenName":"唯信","givenNameLang":"ja"},{"givenName":"ユイシン","givenNameLang":"ja-Kana"},{"givenName":"Yuishin","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"1623","nameIdentifierScheme":"WEKO"},{"nameIdentifier":"60227/profile-ja.html","nameIdentifierScheme":"徳島大学 教育研究者総覧","nameIdentifierURI":"http://pub2.db.tokushima-u.ac.jp/ERD/person/60227/profile-ja.html"},{"nameIdentifier":"10335812","nameIdentifierScheme":"e-Rad","nameIdentifierURI":"https://nrid.nii.ac.jp/ja/search/?qm=10335812"}]},{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"梶, 龍兒","creatorNameLang":"ja"},{"creatorName":"カジ, リュウジ","creatorNameLang":"ja-Kana"},{"creatorName":"Kaji, Ryuji","creatorNameLang":"en"}],"familyNames":[{"familyName":"梶","familyNameLang":"ja"},{"familyName":"カジ","familyNameLang":"ja-Kana"},{"familyName":"Kaji","familyNameLang":"en"}],"givenNames":[{"givenName":"龍兒","givenNameLang":"ja"},{"givenName":"リュウジ","givenNameLang":"ja-Kana"},{"givenName":"Ryuji","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"1622","nameIdentifierScheme":"WEKO"},{"nameIdentifier":"60224/profile-ja.html","nameIdentifierScheme":"徳島大学 教育研究者総覧","nameIdentifierURI":"http://pub2.db.tokushima-u.ac.jp/ERD/person/60224/profile-ja.html"},{"nameIdentifier":"00214304","nameIdentifierScheme":"e-Rad","nameIdentifierURI":"https://nrid.nii.ac.jp/ja/search/?qm=00214304"}]},{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"原田, 雅史","creatorNameLang":"ja"},{"creatorName":"ハラダ, マサフミ","creatorNameLang":"ja-Kana"},{"creatorName":"Harada, Masafumi","creatorNameLang":"en"}],"familyNames":[{"familyName":"原田","familyNameLang":"ja"},{"familyName":"ハラダ","familyNameLang":"ja-Kana"},{"familyName":"Harada","familyNameLang":"en"}],"givenNames":[{"givenName":"雅史","givenNameLang":"ja"},{"givenName":"マサフミ","givenNameLang":"ja-Kana"},{"givenName":"Masafumi","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"1589","nameIdentifierScheme":"WEKO"},{"nameIdentifier":"60159/profile-ja.html","nameIdentifierScheme":"徳島大学 教育研究者総覧","nameIdentifierURI":"http://pub2.db.tokushima-u.ac.jp/ERD/person/60159/profile-ja.html"},{"nameIdentifier":"20228654","nameIdentifierScheme":"e-Rad","nameIdentifierURI":"https://nrid.nii.ac.jp/ja/search/?qm=20228654"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_access","date":[{"dateType":"Available","dateValue":"2021-09-03"}],"displaytype":"detail","filename":"mrms_16_4_297.pdf","filesize":[{"value":"3.27 MB"}],"format":"application/pdf","licensetype":"license_5","mimetype":"application/pdf","url":{"objectType":"fulltext","url":"https://tokushima-u.repo.nii.ac.jp/record/2009150/files/mrms_16_4_297.pdf"},"version_id":"4d3a6961-cfea-4715-8771-46b383f599af"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"magnetic resonance imaging","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"hereditary diffuse leukoencephalopathy with spheroids","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"colony stimulating factor-1 receptor","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"Mescher–Garwood point-resolved spectroscopy","subitem_subject_language":"en","subitem_subject_scheme":"Other"}]},"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":"journal article","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"MR Spectroscopy in Patients with Hereditary Diffuse Leukoencephalopathy with Spheroids and Asymptomatic Carriers of Colony-stimulating Factor 1 Receptor Mutation","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"MR Spectroscopy in Patients with Hereditary Diffuse Leukoencephalopathy with Spheroids and Asymptomatic Carriers of Colony-stimulating Factor 1 Receptor Mutation","subitem_title_language":"en"}]},"item_type_id":"40001","owner":"7","path":["1713853295607"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2021-09-03"},"publish_date":"2021-09-03","publish_status":"0","recid":"2009150","relation_version_is_last":true,"title":["MR Spectroscopy in Patients with Hereditary Diffuse Leukoencephalopathy with Spheroids and Asymptomatic Carriers of Colony-stimulating Factor 1 Receptor Mutation"],"weko_creator_id":"7","weko_shared_id":-1},"updated":"2025-04-28T06:32:52.236583+00:00"}