{"created":"2021-03-01T06:22:41.187765+00:00","id":17966,"links":{},"metadata":{"_buckets":{"deposit":"c3c1a631-521c-48ed-9878-c571ceed38e2"},"_deposit":{"id":"17966","owners":[],"pid":{"revision_id":0,"type":"depid","value":"17966"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00017966","sets":["453:456","471:537:538:1127"]},"item_7_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"Neurophysiological Findings in Machado-Joseph Disease in Japanese Families"}]},"item_7_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"1996-08","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"8","bibliographicPageEnd":"336","bibliographicPageStart":"326","bibliographicVolumeNumber":"110","bibliographic_titles":[{"bibliographic_title":"新潟医学会雑誌"},{"bibliographic_title":"新潟医学会雑誌","bibliographic_titleLang":"en"}]}]},"item_7_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"To clarify neurophysiological aspects of Machado-Joseph disease (NJD), we studied auditory brain stem response (ABR), somatosensory evoked potential (SEP), visual evoked potential (VEP), electromyography (EMG), nerve conduction velocity (NCV) and electroencephalography (EEG) in the 29 patients with MJD in Japanese families. ABR was abnormal in 21 of the 26 patients studied. Wave I was relatively normal, but the peakes after wave II could not be evoked clearly. Median nerve SEP was abnormal in 17 of the 27 patients and posterior tibial nerve SEP was abnormal in all of the 27 patients studied. Peripheral nerve conduction was almost normal but the central conduction was impaired. In VEP, the latency of P100 was prolonged in 2 of the 6 patients studied. In EMG, neuropathic changes were found in 16 of the 20 patients studied. In NCV, motor nerve conduction was delayed in 3 of the 22 patients and sensory nerve action potential could not be evoked in 2 of the 22 patients studied. In EEG, slow activity was found in 8 of the 19 patients studied, but no epileptic discharge was found in all patients. As has been revealed by neuropathological studies, MJD is a multisystem disorder. This study clarified the multisystem abnormality electrophysiologically, and a high incidence of abnormalities was found in EMG, ABR and SEP, especially in posterior tibial nerve SEP, compared with other electrophysiological markers. Electrophysiological examinations, especially EMG, ABR and SEP, are considered to be useful for diagnosing MJD.","subitem_description_type":"Abstract"}]},"item_7_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"112784","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Kondo, Hiroshi"}]}]},"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":"AN00182415","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":"近藤, 浩"}],"nameIdentifiers":[{"nameIdentifier":"112783","nameIdentifierScheme":"WEKO"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2019-08-08"}],"displaytype":"detail","filename":"110(8)_326-336.pdf","filesize":[{"value":"1.9 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"110(8)_326-336.pdf","url":"https://niigata-u.repo.nii.ac.jp/record/17966/files/110(8)_326-336.pdf"},"version_id":"2c808e54-064c-472c-a5f3-e894db475854"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Machado-Joseph disease (MJD)","subitem_subject_scheme":"Other"},{"subitem_subject":"auditory brain stem response (ABR)","subitem_subject_scheme":"Other"},{"subitem_subject":"somatosensory evoked potential (SEP)","subitem_subject_scheme":"Other"},{"subitem_subject":"visual evoked potential (VEP)","subitem_subject_scheme":"Other"},{"subitem_subject":"Machado-Joseph 病","subitem_subject_scheme":"Other"},{"subitem_subject":"聴性脳幹反応","subitem_subject_scheme":"Other"},{"subitem_subject":"体性感覚誘発電位","subitem_subject_scheme":"Other"},{"subitem_subject":"視覚誘発電位","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":"Machado-Joseph病の神経生理学的特徴に関する研究","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Machado-Joseph病の神経生理学的特徴に関する研究"},{"subitem_title":"Machado-Joseph病の神経生理学的特徴に関する研究","subitem_title_language":"en"}]},"item_type_id":"7","owner":"1","path":["456","1127"],"pubdate":{"attribute_name":"公開日","attribute_value":"2016-08-19"},"publish_date":"2016-08-19","publish_status":"0","recid":"17966","relation_version_is_last":true,"title":["Machado-Joseph病の神経生理学的特徴に関する研究"],"weko_creator_id":"1","weko_shared_id":null},"updated":"2022-12-15T03:49:16.755300+00:00"}