{"created":"2021-03-01T06:29:44.585395+00:00","id":24385,"links":{},"metadata":{"_buckets":{"deposit":"1cb506d0-c9dc-4b2e-8ef0-411998a52f8b"},"_deposit":{"id":"24385","owners":[],"pid":{"revision_id":0,"type":"depid","value":"24385"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00024385","sets":["453:456","471:537:538:1239"]},"item_7_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"Myoclonus epilepsy, Anraku type(Hereditary Spinocerebellar Degeneration : recent advances)"}]},"item_7_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"1986-12","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"12","bibliographicPageEnd":"745","bibliographicPageStart":"738","bibliographicVolumeNumber":"100","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":"Myoclonus epilepsy is neuropathologically classified into 3 major groups; Lafora body type, lipid storage type, and degenerative type. Anraku et al.（1966）made a brief description of 3 cases with brown pigments in some CNS regions as a special type of myoclonus epilepsy？. However, no such cases have been reported thereafter. We reported another case with such histological findings and described new findings. A 20-year-old woman without hereditary factors, suffered from progressive ataxic gait. Myoclonus, trunkal ataxia and dementia appeared and progressed gradually. Polyspike and wave complex were recorded in EEG. She died at the age of 28. Postmortem examination revealed severe loss of Purkinje cells and granule cells with gliosis. Globus pallidus, subthalamus, dentate and red nuclei, and pontine tegmentum showed neuronal loss and gliosis. Pontine nuclei were well preserved. Cerebral cortex also revealed mild but diffuse neuronal loss. In addition to these lesions, many autofluorescent pigments, positively stained with Fontana-Masson silver method, were present in the Ist to IIIrd layer of the cerebral cortex, Purkinje cell layer, globus pallidus and substantia nigra. The number of the pigments was not correlated with neuronal loss or gliosis. In the white matter, peripheral nerves and general visceral organs, the pigment was not observed. Ultrastructurally, the pigments were localized in astrocytes, and similar to the lipofuscin or stored substance observed in the ceroidlipofuscinosis. Our case and 3 cases previously reported strongly suggest that there is a particular subgroup in the myoclonus epilepsy, which is characterized by presence of brown pigment and degeneration of cerebellar cortex.","subitem_description_type":"Abstract"}]},"item_7_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"145754","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Takeda, Shigeki"}]},{"nameIdentifiers":[{"nameIdentifier":"145755","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Takahashi, Hitoshi"}]},{"nameIdentifiers":[{"nameIdentifier":"145756","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Ohama, Eisaku"}]},{"nameIdentifiers":[{"nameIdentifier":"145757","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Ikuta, Fusahiro"}]},{"nameIdentifiers":[{"nameIdentifier":"145758","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Naito, Haruhiko"}]},{"nameIdentifiers":[{"nameIdentifier":"145759","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Arita, Tadashi"}]}]},"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":"武田, 茂樹"}],"nameIdentifiers":[{"nameIdentifier":"145748","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"高橋, 均"}],"nameIdentifiers":[{"nameIdentifier":"145749","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"大浜, 栄作"}],"nameIdentifiers":[{"nameIdentifier":"145750","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"生田, 房弘"}],"nameIdentifiers":[{"nameIdentifier":"145751","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"内藤, 明彦"}],"nameIdentifiers":[{"nameIdentifier":"145752","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"有田, 忠司"}],"nameIdentifiers":[{"nameIdentifier":"145753","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":"2019-08-19"}],"displaytype":"detail","filename":"100(12)_738-745.pdf","filesize":[{"value":"1.3 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"100(12)_738-745.pdf","url":"https://niigata-u.repo.nii.ac.jp/record/24385/files/100(12)_738-745.pdf"},"version_id":"88e81a38-75dd-42b2-89a5-6f7eec80e996"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"myoclonus epilepsy Anraku type","subitem_subject_scheme":"Other"},{"subitem_subject":"brown pigment","subitem_subject_scheme":"Other"},{"subitem_subject":"degeneration of cerebellar cortex","subitem_subject_scheme":"Other"},{"subitem_subject":"褐色顆粒","subitem_subject_scheme":"Other"},{"subitem_subject":"小脳皮質変性","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":"6) ミオクローヌスてんかん安楽型(シンポジウム 遺伝性脊髄小脳変性症 : 最近の知見から, 第417回新潟医学会)","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"6) ミオクローヌスてんかん安楽型(シンポジウム 遺伝性脊髄小脳変性症 : 最近の知見から, 第417回新潟医学会)"},{"subitem_title":"6) ミオクローヌスてんかん安楽型(シンポジウム 遺伝性脊髄小脳変性症 : 最近の知見から, 第417回新潟医学会)","subitem_title_language":"en"}]},"item_type_id":"7","owner":"1","path":["456","1239"],"pubdate":{"attribute_name":"公開日","attribute_value":"2015-12-16"},"publish_date":"2015-12-16","publish_status":"0","recid":"24385","relation_version_is_last":true,"title":["6) ミオクローヌスてんかん安楽型(シンポジウム 遺伝性脊髄小脳変性症 : 最近の知見から, 第417回新潟医学会)"],"weko_creator_id":"1","weko_shared_id":null},"updated":"2022-12-15T03:54:31.491571+00:00"}