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This disorder is often encountered in Japan, but seems to be extremely rare outside Japan. DRPLA can display a wide variety of clinical symptoms including cerebellar ataxia, dementia and hypenkinetic involuntary movements such as chorea and choreoathetosis. The age of onset ranges from 6 to 69 years of age, the mean age being 31 years. There is a particular correlation between the age of onset and the clinical symptoms\uff1a Progressive myoclonus epilepsy syndrome is usual features of DRPLA patients with juvenile onset under 20 years of age (juvenile form), whereas those with onset in late adult life over 40 years of age (late adult form) have cerebellar ataxia and choreoathetosis often without myoclonus or epilepsy or either. There are various transitional forms between the juvenile form and the late adult form. This disease, although having the characteristic features of the dominant heredity, dementia and choreoathetosis, can be differentially diagnosed from Huntington chorea by the marked cerebellar ataxia, by CT findings suggesting atrophy of the brain stem and the cerebellum, and by the neuropathologic findings, i.e. the dentatorubral-pallidoluysian atrophy and lack of prominent atrophy of the striatum.", "subitem_description_type": "Abstract"}]}, "item_7_full_name_3": {"attribute_name": "\u8457\u8005\u5225\u540d", "attribute_value_mlt": [{"nameIdentifiers": [{"nameIdentifier": "116751", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Naito, Haruhiko"}]}]}, "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": "\u5185\u85e4, \u660e\u5f66"}], "nameIdentifiers": [{"nameIdentifier": "116750", "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)_174-179.pdf", "filesize": [{"value": "1.1 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 1100000.0, "url": {"label": "109(4)_174-179.pdf", "url": "https://niigata-u.repo.nii.ac.jp/record/18682/files/109(4)_174-179.pdf"}, "version_id": "88c23957-afed-4f49-b860-772d2af477e2"}]}, "item_keyword": {"attribute_name": "\u30ad\u30fc\u30ef\u30fc\u30c9", "attribute_value_mlt": [{"subitem_subject": "DRPLA", "subitem_subject_scheme": "Other"}, {"subitem_subject": "clinical features", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u6b6f\u72b6\u6838\u8d64\u6838\u6de1\u84bc\u7403\u30eb\u30a4\u4f53\u840e\u7e2e\u75c7", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u81e8\u5e8a\u50cf", "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": "4)\u6b6f\u72b6\u6838\u8d64\u6838\u6de1\u84bc\u7403\u30eb\u30a4\u4f53\u840e\u7e2e\u75c7(DRPLA)\u306e\u81e8\u5e8a\u50cf(\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": "4)\u6b6f\u72b6\u6838\u8d64\u6838\u6de1\u84bc\u7403\u30eb\u30a4\u4f53\u840e\u7e2e\u75c7(DRPLA)\u306e\u81e8\u5e8a\u50cf(\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": "4)\u6b6f\u72b6\u6838\u8d64\u6838\u6de1\u84bc\u7403\u30eb\u30a4\u4f53\u840e\u7e2e\u75c7(DRPLA)\u306e\u81e8\u5e8a\u50cf(\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/10191/42315", "pubdate": {"attribute_name": "\u516c\u958b\u65e5", "attribute_value": "2016-06-27"}, "publish_date": "2016-06-27", "publish_status": "0", "recid": "18682", "relation": {}, "relation_version_is_last": true, "title": ["4)\u6b6f\u72b6\u6838\u8d64\u6838\u6de1\u84bc\u7403\u30eb\u30a4\u4f53\u840e\u7e2e\u75c7(DRPLA)\u306e\u81e8\u5e8a\u50cf(\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}