{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T17:06:48Z","timestamp":1787504808000,"version":"build-2736575974"},"reference-count":21,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,12,11]],"date-time":"2014-12-11T00:00:00Z","timestamp":1418256000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2015,6]]},"abstract":"<jats:p>\n                    Spinocerebellar ataxia type 31 (\n                    <jats:styled-content style=\"fixed-case\">SCA31<\/jats:styled-content>\n                    ) is an autosomal dominant cerebellar ataxia commonly observed in\n                    <jats:styled-content style=\"fixed-case\">J<\/jats:styled-content>\n                    apan. However, few neuropathological examinations have been conducted. Here we report the case of a 76\u2010year\u2010old\n                    <jats:styled-content style=\"fixed-case\">J<\/jats:styled-content>\n                    apanese male\n                    <jats:styled-content style=\"fixed-case\">SCA31<\/jats:styled-content>\n                    patient. He noticed dysarthria and difficulty walking at 65 years old. His symptoms subsequently deteriorated, although he could still walk with assistance at 70 years. At 73 years, when he could no longer walk, he was admitted to our hospital. He showed severe limb and truncal ataxia. His father and older brother had shown the same symptoms. Brain magnetic resonance imaging showed cerebellar atrophy of the anterior lobe and white matter hyperintensities. He was diagnosed with\n                    <jats:styled-content style=\"fixed-case\">SCA31<\/jats:styled-content>\n                    by genetic analysis. Gradually, his cognitive functions and ability to communicate declined. He died of respiratory failure at the age of 76. Neuropathological examination revealed severe\n                    <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>\n                    urkinje cell loss that was accentuated in the anterior lobe of the cerebellum. Furthermore, the remaining Purkinje cells showed abnormal processes (that is, halo\u2010like amorphous materials), as has been reported previously. Severe deposition of hyperphosphorylated tau\u2010positive neurites, many senile plaques and amyloid angiopathy were observed in the neocortex. Our findings suggest that in\n                    <jats:styled-content style=\"fixed-case\">SCA31<\/jats:styled-content>\n                    , accelerated tau and amyloid pathology in the neocortex might induce dementia at the terminal stage.\n                  <\/jats:p>","DOI":"10.1111\/neup.12184","type":"journal-article","created":{"date-parts":[[2014,12,12]],"date-time":"2014-12-12T00:18:37Z","timestamp":1418343517000},"page":"273-279","source":"Crossref","is-referenced-by-count":14,"title":["Autopsy case of spinocerebellar ataxia type 31 with severe dementia at the terminal stage"],"prefix":"10.1111","volume":"35","author":[{"given":"Tadashi","family":"Adachi","sequence":"first","affiliation":[{"name":"Division of Neurology Department of Brain and Neurosciences Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michio","family":"Kitayama","sequence":"additional","affiliation":[{"name":"Division of Neurology Department of Brain and Neurosciences Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiya","family":"Nakano","sequence":"additional","affiliation":[{"name":"Division of Neurology Department of Brain and Neurosciences Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshiki","family":"Adachi","sequence":"additional","affiliation":[{"name":"Department of Neurology National Hospital Organization Matsue Medical Center  Matsue Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinsuke","family":"Kato","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenji","family":"Nakashima","sequence":"additional","affiliation":[{"name":"Division of Neurology Department of Brain and Neurosciences Faculty of Medicine Tottori University  Yonago Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,12,11]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2009.09.019"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3182377e3a"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21443"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1086\/432518"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2008.00947.x"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/neup.12032"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.2007.00815.x"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10038-007-0182-x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(96)00231-6"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2010.01142.x"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00308809"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.58.12.1791"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.2008.00147.x"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.41.4.479"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-011-0910-3"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/63.9.911"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-14"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000173065.75680.e2"},{"key":"e_1_2_7_20_1","first-page":"87","article-title":"Fine structural changes in the cerebellar cortex in kinky hair disease","volume":"101","author":"Hirano A","year":"1976","journal-title":"Trans Am Neurol Assoc"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnmol.2013.00057"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1176\/jnp.16.3.367"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fneup.12184","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/neup.12184","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,1]],"date-time":"2023-10-01T15:43:55Z","timestamp":1696175035000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/neup.12184"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,12,11]]},"references-count":21,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2015,6]]}},"alternative-id":["10.1111\/neup.12184"],"URL":"https:\/\/doi.org\/10.1111\/neup.12184","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"value":"0919-6544","type":"print"},{"value":"1440-1789","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,12,11]]}}}