{"created":"2021-03-01T06:27:19.733272+00:00","id":22195,"links":{},"metadata":{"_buckets":{"deposit":"4ff8c8db-bebc-4d30-a609-282605424d06"},"_deposit":{"id":"22195","owners":[],"pid":{"revision_id":0,"type":"depid","value":"22195"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00022195","sets":["453:456","471:537:538:1197"]},"item_7_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"A Comparative Study on the Clinical Findings of Machado-Joseph Disease and Hereditary Dentatorubropallidoluysan Atrophy"}]},"item_7_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"1990-06","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"6","bibliographicPageEnd":"507","bibliographicPageStart":"497","bibliographicVolumeNumber":"104","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":"It is difficult to differentiate Machado-Joseph disease (MJD) from hereditary dentatorubropallidoluysian atrophy (HDRPLA) that predominantly presents cerebellar ataxia in the adulthood without overt dementia and epileptic seizure. In the present paper we performed a comparative study on the clinical findings of two cases of MJD and two cases of HDRPLA who had developed cerebellar ataxia after the third decade. On clinical signs, it was thought to be important to note the manifestations such as mental change, ophthalmoplegia, muscle atrophy, spasticity and involuntary movements. Especially, in the early stage, affective disorders in HDRPLA cases, and upward gaze limitation, facial atrophy and spasticity in MJD cases were remarkable. Electrophysiologically, EEG were abnormal in HDRPLA cases with frontal slow waves, but without epileptic discharges. SEP on tibial nerve stimulation demonstrated no elicitation of P39 in MJD cases and the slight prolongation of P39 latency in HDRPLA cases. It was conceivable that the lesion of posterior column might be responsible for the severe abnormality of P39 in MJD cases. Cranial CT scan and MRI showed brainstem and cerebellar atrophy in all cases. Furthermore, there were cerebral atrophy and cerebral white matter lesions in HDRPLA cases, which may be associated with mental changes or mental deterioration in HDRPLA. The level of HVA in CSF was decreased in MJD cases, while that of 5-HIAA was decresed in one of HDRPLA cases. Thus, it was conceivable that the abovementioned studies may reveal the extension of the lesions peculiar to each disease and be available for making distinction between the two diseases","subitem_description_type":"Abstract"}]},"item_7_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"134929","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Inoue, Yukichi"}]},{"nameIdentifiers":[{"nameIdentifier":"134930","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Yuasa, Tatsuhiko"}]},{"nameIdentifiers":[{"nameIdentifier":"134931","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Harayama, Hiromi"}]},{"nameIdentifiers":[{"nameIdentifier":"134932","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Kanda, Takemasa"}]}]},"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":"井上, 雄吉"}],"nameIdentifiers":[{"nameIdentifier":"134925","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"湯浅, 龍彦"}],"nameIdentifiers":[{"nameIdentifier":"134926","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"原山, 尋実"}],"nameIdentifiers":[{"nameIdentifier":"134927","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"神田, 武政"}],"nameIdentifiers":[{"nameIdentifier":"134928","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":"2019-08-16"}],"displaytype":"detail","filename":"104(6)_497-507.pdf","filesize":[{"value":"4.4 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"104(6)_497-507.pdf","url":"https://niigata-u.repo.nii.ac.jp/record/22195/files/104(6)_497-507.pdf"},"version_id":"bd36cdd9-9089-403c-aa67-4c0a0fc586dd"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Machado-Joseph disease","subitem_subject_scheme":"Other"},{"subitem_subject":"hereditary dentatorubropallidoluysian atrophy (HDRPLA)","subitem_subject_scheme":"Other"},{"subitem_subject":"somatosensory evoked potential (SEP)","subitem_subject_scheme":"Other"},{"subitem_subject":"magnetic resonance imaging (MRI)","subitem_subject_scheme":"Other"},{"subitem_subject":"Machado-Joseph病","subitem_subject_scheme":"Other"},{"subitem_subject":"遺伝性歯状核・赤核・淡蒼球・ルイ体萎縮症","subitem_subject_scheme":"Other"},{"subitem_subject":"体性感覚誘発電位","subitem_subject_scheme":"Other"},{"subitem_subject":"磁気共鳴画像","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病と遺伝性歯状核・赤核・淡蒼球・ルイ体萎縮症（内藤・小柳）の臨床的比較 : 自験2家系4例についての検討","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Machado-Joseph病と遺伝性歯状核・赤核・淡蒼球・ルイ体萎縮症（内藤・小柳）の臨床的比較 : 自験2家系4例についての検討"},{"subitem_title":"Machado-Joseph病と遺伝性歯状核・赤核・淡蒼球・ルイ体萎縮症（内藤・小柳）の臨床的比較 : 自験2家系4例についての検討","subitem_title_language":"en"}]},"item_type_id":"7","owner":"1","path":["456","1197"],"pubdate":{"attribute_name":"公開日","attribute_value":"2015-11-11"},"publish_date":"2015-11-11","publish_status":"0","recid":"22195","relation_version_is_last":true,"title":["Machado-Joseph病と遺伝性歯状核・赤核・淡蒼球・ルイ体萎縮症（内藤・小柳）の臨床的比較 : 自験2家系4例についての検討"],"weko_creator_id":"1","weko_shared_id":null},"updated":"2022-12-15T03:52:36.143897+00:00"}