{"_buckets": {"deposit": "2830f0f5-d892-4729-8950-2d21463acd2d"}, "_deposit": {"id": "18679", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "18679"}, "status": "published"}, "_oai": {"id": "oai:niigata-u.repo.nii.ac.jp:00018679"}, "item_7_alternative_title_1": {"attribute_name": "\u305d\u306e\u4ed6\u306e\u30bf\u30a4\u30c8\u30eb", "attribute_value_mlt": [{"subitem_alternative_title": "Clinical Features of Machado-Joseph Disease : a Special Reference for Differential Diagnosis of Autosomal Dominant Ataxia, SCA-1, SCA-2 and DRPLA (Pathophysiology of Hereditary Neurologic Diseases)"}]}, "item_7_biblio_info_6": {"attribute_name": "\u66f8\u8a8c\u60c5\u5831", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "1995-04", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "4", "bibliographicPageEnd": "161", "bibliographicPageStart": "155", "bibliographicVolumeNumber": "109", "bibliographic_titles": [{"bibliographic_title": "\u65b0\u6f5f\u533b\u5b66\u4f1a\u96d1\u8a8c"}, {"bibliographic_title": "\u65b0\u6f5f\u533b\u5b66\u4f1a\u96d1\u8a8c", "bibliographic_titleLang": "en"}]}]}, "item_7_description_4": {"attribute_name": "\u6284\u9332", "attribute_value_mlt": [{"subitem_description": "Recent advances in molecular genetics has clarified the disease locus in the chromosomal mapping, including chromosome 6p for SCA-1, chromosome 12q for Menzel (SCA-2), chromosomal 12p for DRPLA, and chromosome 14q for MJD. In this article I will discuss about the clinical viewpoints, which favorate for making the differential diagnosis of Machado-Joseph disease (MJD) from other form of autosomal dominant spinocerebellar atrophy, including Yakura (SCA-1), Menzel (SCA-2), and DRPLA. The symptoms and signs of cerebellar ataxia, including gait ataxia are the most common features among these dominantly inherited ataxic diseases, and there are only a few but important markers left for differential diagnosis. The overt external ophtalmoplegia is a cardinal feature for MJD as well as for SCA-1, but not for DRPLA and SCA-2. Slow saccade eye movement is a popular sign for both SCA-1 and SCA-2. Mental deterioration and dementia are scarecely seen in patients with MJD, but are frequently seen in DRPLA, SCA-1 and also in SCA-2. Epileptic seizure is hardly seen in MJD, and SCA-1 and SCA-2, but is one of important features of DRPLA. Investigation of family members of these diseases is needed for making clinical and genetical diagnosis precisely.", "subitem_description_type": "Abstract"}]}, "item_7_full_name_3": {"attribute_name": "\u8457\u8005\u5225\u540d", "attribute_value_mlt": [{"nameIdentifiers": [{"nameIdentifier": "116739", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Yuasa, Tatsuhiko"}]}]}, "item_7_publisher_7": {"attribute_name": "\u51fa\u7248\u8005", "attribute_value_mlt": [{"subitem_publisher": "\u65b0\u6f5f\u533b\u5b66\u4f1a"}]}, "item_7_select_19": {"attribute_name": "\u8457\u8005\u7248\u30d5\u30e9\u30b0", "attribute_value_mlt": [{"subitem_select_item": "publisher"}]}, "item_7_source_id_11": {"attribute_name": "\u66f8\u8a8c\u30ec\u30b3\u30fc\u30c9ID", "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": "\u8457\u8005", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "\u6e6f\u6d45, \u9f8d\u5f66"}], "nameIdentifiers": [{"nameIdentifier": "116738", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "\u30d5\u30a1\u30a4\u30eb\u60c5\u5831", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2019-08-08"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "109(4)_155-161.pdf", "filesize": [{"value": "1.3 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 1300000.0, "url": {"label": "109(4)_155-161.pdf", "url": "https://niigata-u.repo.nii.ac.jp/record/18679/files/109(4)_155-161.pdf"}, "version_id": "b88c0d63-043d-4c2b-b004-3aa9ddb87e73"}]}, "item_keyword": {"attribute_name": "\u30ad\u30fc\u30ef\u30fc\u30c9", "attribute_value_mlt": [{"subitem_subject": "Machado-Joseph disease", "subitem_subject_scheme": "Other"}, {"subitem_subject": "SCA-1", "subitem_subject_scheme": "Other"}, {"subitem_subject": "SCA-2", "subitem_subject_scheme": "Other"}, {"subitem_subject": "DRPLA", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u810a\u9ac4\u5c0f\u8133\u5909\u6027\u75c7", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u30b8\u30e7\u30bb\u30d5\u75c5", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u30e1\u30f3\u30c4\u30a7\u30eb\u75c5", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u907a\u4f1d\u6027 OPCA", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u6b6f\u72b6\u6838", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u8d64\u6838", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u6de1\u84bc\u7403", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u30eb\u30a4\u4f53\u840e\u7e2e\u75c7", "subitem_subject_scheme": "Other"}]}, "item_language": {"attribute_name": "\u8a00\u8a9e", "attribute_value_mlt": [{"subitem_language": "jpn"}]}, "item_resource_type": {"attribute_name": "\u8cc7\u6e90\u30bf\u30a4\u30d7", "attribute_value_mlt": [{"resourcetype": "departmental bulletin paper", "resourceuri": "http://purl.org/coar/resource_type/c_6501"}]}, "item_title": "1)Machado-Joseph\u75c5\u306e\u81e8\u5e8a\u50cf\u306b\u3064\u3044\u3066 : \u7279\u306b\u5468\u8fba\u75be\u60a3\u3068\u306e\u9451\u5225\u3092\u4e2d\u5fc3\u306b(\u30b7\u30f3\u30dd\u30b8\u30a6\u30e0 \u5206\u5b50\u907a\u4f1d\u75c5\u306e\u81e8\u5e8a\u3068\u75c5\u7406, \u7b2c494\u56de\u65b0\u6f5f\u533b\u5b66\u4f1a)", "item_titles": {"attribute_name": "\u30bf\u30a4\u30c8\u30eb", "attribute_value_mlt": [{"subitem_title": "1)Machado-Joseph\u75c5\u306e\u81e8\u5e8a\u50cf\u306b\u3064\u3044\u3066 : \u7279\u306b\u5468\u8fba\u75be\u60a3\u3068\u306e\u9451\u5225\u3092\u4e2d\u5fc3\u306b(\u30b7\u30f3\u30dd\u30b8\u30a6\u30e0 \u5206\u5b50\u907a\u4f1d\u75c5\u306e\u81e8\u5e8a\u3068\u75c5\u7406, \u7b2c494\u56de\u65b0\u6f5f\u533b\u5b66\u4f1a)"}, {"subitem_title": "1)Machado-Joseph\u75c5\u306e\u81e8\u5e8a\u50cf\u306b\u3064\u3044\u3066 : \u7279\u306b\u5468\u8fba\u75be\u60a3\u3068\u306e\u9451\u5225\u3092\u4e2d\u5fc3\u306b(\u30b7\u30f3\u30dd\u30b8\u30a6\u30e0 \u5206\u5b50\u907a\u4f1d\u75c5\u306e\u81e8\u5e8a\u3068\u75c5\u7406, \u7b2c494\u56de\u65b0\u6f5f\u533b\u5b66\u4f1a)", "subitem_title_language": "en"}]}, "item_type_id": "7", "owner": "1", "path": ["453/456", "471/537/538/1139"], "permalink_uri": "http://hdl.handle.net/10191/42312", "pubdate": {"attribute_name": "\u516c\u958b\u65e5", "attribute_value": "2016-06-27"}, "publish_date": "2016-06-27", "publish_status": "0", "recid": "18679", "relation": {}, "relation_version_is_last": true, "title": ["1)Machado-Joseph\u75c5\u306e\u81e8\u5e8a\u50cf\u306b\u3064\u3044\u3066 : \u7279\u306b\u5468\u8fba\u75be\u60a3\u3068\u306e\u9451\u5225\u3092\u4e2d\u5fc3\u306b(\u30b7\u30f3\u30dd\u30b8\u30a6\u30e0 \u5206\u5b50\u907a\u4f1d\u75c5\u306e\u81e8\u5e8a\u3068\u75c5\u7406, \u7b2c494\u56de\u65b0\u6f5f\u533b\u5b66\u4f1a)"], "weko_shared_id": null}