{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T06:05:50Z","timestamp":1760853950377,"version":"3.37.3"},"reference-count":11,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2017,4,12]],"date-time":"2017-04-12T00:00:00Z","timestamp":1491955200000},"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":[[2017,10]]},"abstract":"<jats:p>A 62\u2010year\u2010old man presented with abnormal behavior and cognitive impairment. Diffusion\u2010weighted images (DWI) obtained on MRI showed extensive hyperintense regions in the cerebral cortex and striatum. Myoclonus was recognized, and the patient died 1\u00a0month after the onset; his condition did not reach the akinetic mutism state. The brain weighed 1300\u00a0g and showed no apparent atrophy. Extensive spongiform changes were observed in the cerebral neocortex, striatum, thalamus and cerebellar cortex, but gliosis was mild or absent. Neuropil rarefaction and neuron loss were not apparent. Mild proliferation of anti\u2010 GFAP\u2010positive astrocytes was observed in the cerebral cortex, but unaffected regions were noted. Regions without spongiform changes and GFAP\u2010positive astrocytes included the hippocampal formation and subiculum. PrP immunostaining showed extensive diffuse synaptic\u2010type PrP deposition in the gray matter, including the hippocampal region, but it was also mild. <jats:italic>PrP<\/jats:italic> gene analysis revealed no mutation with methionine homozygosity at polymorphic codon 129. Western blot analysis of proteinase K\u2010resistant PrP indicated type 1 PrP<jats:sup>Sc<\/jats:sup>. The clinicopathological findings of the present case confirm several hypotheses: (i) the earliest pathologic evidence observed by HE staining in CJD are spongiform changes; (ii) DWI hyperintense regions indicate these spongiform changes; and (iii) regions without spongiform changes, gliosis and proliferation of GFAP\u2010positive astrocytes, but with PrP deposition, exist in the early disease stage.<\/jats:p>","DOI":"10.1111\/neup.12379","type":"journal-article","created":{"date-parts":[[2017,4,12]],"date-time":"2017-04-12T09:13:34Z","timestamp":1491988414000},"page":"420-425","source":"Crossref","is-referenced-by-count":6,"title":["MM1\u2010type sporadic Creutzfeldt\u2010Jakob disease with 1\u2010month total disease duration and early pathologic indicators"],"prefix":"10.1111","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0059-3104","authenticated-orcid":false,"given":"Yasushi","family":"Iwasaki","sequence":"first","affiliation":[{"name":"Department of Neuropathology, Institute for Medical Science of Aging Aichi Medical University  Nagakute Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroko","family":"Kato","sequence":"additional","affiliation":[{"name":"Department of Neurology Anjo Kosei Hospital  Anjo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tetsuo","family":"Ando","sequence":"additional","affiliation":[{"name":"Department of Neurology Anjo Kosei Hospital  Anjo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maya","family":"Mimuro","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute for Medical Science of Aging Aichi Medical University  Nagakute Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tetsuyuki","family":"Kitamoto","sequence":"additional","affiliation":[{"name":"Division of CJD Science and Technology, Department of Neurological Science Tohoku University Graduate School of Medicine  Sendai Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mari","family":"Yoshida","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute for Medical Science of Aging Aichi Medical University  Nagakute Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,4,12]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.2005.00697.x"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-006-0111-7"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2014.04.011"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-005-0981-0"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/101.2.333"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.46.6.1690"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/neup.12327"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181a18846"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/neup.12013"},{"key":"e_1_2_8_12_1","first-page":"583","volume-title":"Carpenter's Human Neuroanatomy","author":"Andre P","year":"1996"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fneup.12379","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/neup.12379","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,1]],"date-time":"2023-10-01T19:38:54Z","timestamp":1696189134000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/neup.12379"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,4,12]]},"references-count":11,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2017,10]]}},"alternative-id":["10.1111\/neup.12379"],"URL":"https:\/\/doi.org\/10.1111\/neup.12379","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"type":"print","value":"0919-6544"},{"type":"electronic","value":"1440-1789"}],"subject":[],"published":{"date-parts":[[2017,4,12]]}}}