{"_buckets": {"deposit": "06051303-8981-4678-bd91-3416338acfbf"}, "_deposit": {"id": "16625", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "16625"}, "status": "published"}, "_oai": {"id": "oai:niigata-u.repo.nii.ac.jp:00016625"}, "item_7_alternative_title_1": {"attribute_name": "\u305d\u306e\u4ed6\u306e\u30bf\u30a4\u30c8\u30eb", "attribute_value_mlt": [{"subitem_alternative_title": "Neurophysiological Findings in Triplet Repeat Disease : Pathophysiology and Correlation to the CAG Repeat"}]}, "item_7_biblio_info_6": {"attribute_name": "\u66f8\u8a8c\u60c5\u5831", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "1998-07", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "7", "bibliographicPageEnd": "428", "bibliographicPageStart": "414", "bibliographicVolumeNumber": "112", "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": "To clarify the neurophysiological abnormality of triplet repeat diseases, and to elucidate how the expanded CAG repeats affect physiological properties of the nervous system, I investigated the correlation between CAG repeat and various parameters of evoked potentials. Nerve conduction studies (NCS), somatosensory evoked potentials (SEP) and auditory brainstem responses (ABR) were performed in patients with bulbospinal muscular atrophy (SBMA), Machado-Joseph disease (MJD) and SCA 6. In SBMA patients, main characteristic findings were reduction of the amplitudes in NCS and SEP. Only the N24 amplitudes in tibial nerve SEP inversely correlated the CAG repeat number. The central conduction time (CCT) of tibial nerve SEP in MJD patients was significantly prolonged and correlated with the expanded CAG repeat size. The wave III latency of ABR in MJD patients and the wave I and V latencies of ABR in SCA 6 patients were significantly prolonged without correlation to the expanded CAG repeat sizes. The amplitude of N24 in SBMA patients and tibial nerve CCT in MJD patients correlated the expanded CAG repeat size, which may provide an evidence that expanded CAG repeats affect the sensory system. Electrophysiological analyses are useful elucidation how the expanded CAG repeat affect the function of nervous system in patients with triplet repeat diseases.", "subitem_description_type": "Abstract"}]}, "item_7_full_name_3": {"attribute_name": "\u8457\u8005\u5225\u540d", "attribute_value_mlt": [{"nameIdentifiers": [{"nameIdentifier": "105640", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Homma, Atsushi"}]}]}, "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": 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