{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T21:24:34Z","timestamp":1773869074172,"version":"3.50.1"},"reference-count":15,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2016,7,20]],"date-time":"2016-07-20T00:00:00Z","timestamp":1468972800000},"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":[[2017,2]]},"abstract":"<jats:p>A 78\u2010year\u2010old Japanese man presented with rapidly progressive dementia and gait disturbances. Eight months before the onset of clinical symptoms, diffusion\u2010weighted magnetic resonance imaging (DWI) demonstrated hyperintensities in the right temporal, right parietal and left medial occipital cortices. Two weeks after symptom onset, DWI showed extensive hyperintensity in the bilateral cerebral cortex, with regions of higher brightness that existed prior to symptom onset still present. Four weeks after clinical onset, periodic sharp wave complexes were identified on an electroencephalogram. Myoclonus was observed 8\u00a0weeks after clinical onset. The patient reached an akinetic mutism state and died 5\u00a0months after onset. Neuropathological examination showed widespread cerebral neocortical involvement of fine vacuole\u2010type spongiform changes with large confluent vacuole\u2010type spongiform changes. Spongiform degeneration with neuron loss and hypertrophic astrocytosis was also observed in the striatum and medial thalamus. The inferior olivary nucleus showed severe neuron loss with hypertrophic astrocytosis. Prion protein (PrP) immunostaining showed widespread synaptic\u2010type PrP deposition with perivacuolar\u2010type PrP deposition in the cerebral neocortex. Mild to moderate PrP deposition was also observed extensively in the basal ganglia, thalamus, cerebellum and brainstem, but it was not apparent in the inferior olivary nucleus. PrP gene analysis showed no mutations, and polymorphic codon 129 showed methionine homozygosity. Western blot analysis of protease\u2010resistant PrP showed both type 1 scrapie type PrP (PrP<jats:sup>Sc<\/jats:sup>) and type 2 PrP<jats:sup>Sc<\/jats:sup>. Based on the relationship between the neuroimaging and pathological findings, we speculated that cerebral cortical lesions with large confluent vacuoles and type 2 PrP<jats:sup>Sc<\/jats:sup> would show higher brightness and continuous hyperintensity on DWI than those with fine vacuoles and type 1 PrP<jats:sup>Sc<\/jats:sup>. We believe the present patient had a combined form of MM1\u00a0+\u00a0MM2\u2010cortical with thalamic\u2010type sporadic Creutzfeldt\u2010Jakob disease (sCJD), which suggests a broader spectrum of sCJD clinicopathological findings.<\/jats:p>","DOI":"10.1111\/neup.12327","type":"journal-article","created":{"date-parts":[[2016,7,20]],"date-time":"2016-07-20T07:54:46Z","timestamp":1469001286000},"page":"78-85","source":"Crossref","is-referenced-by-count":24,"title":["An autopsied case of MM1\u00a0+\u00a0MM2\u2010cortical with thalamic\u2010type sporadic Creutzfeldt\u2010Jakob disease presenting with hyperintensities on diffusion\u2010weighted MRI before clinical onset"],"prefix":"10.1111","volume":"37","author":[{"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":"Keiko","family":"Mori","sequence":"additional","affiliation":[{"name":"Department of Neurology Oyamada Memorial Spa Hospital  Yokkaichi Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masumi","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of Neurology Oyamada Memorial Spa Hospital  Yokkaichi 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":"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":[[2016,7,20]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/bmb\/66.1.213"},{"key":"e_1_2_8_3_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_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2010.01181.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2007.00854.x"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-006-0131-3"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-006-0111-7"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2008.10.026"},{"key":"e_1_2_8_9_1","first-page":"1285","article-title":"Abnormal isoform of prion proteins accumulates in the synaptic structures of the central nervous system in patients with Creutzfeldt\u2010Jakob disease","volume":"140","author":"Kitamoto T","year":"1992","journal-title":"Am J Pathol"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1128\/JVI.02387-09"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000151847.57956.FA"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-012-1002-8"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2010.11.069"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2014.04.011"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2008.155242"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2012.04.004"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fneup.12327","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fneup.12327","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/neup.12327","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T22:04:43Z","timestamp":1695506683000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/neup.12327"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,7,20]]},"references-count":15,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2017,2]]}},"alternative-id":["10.1111\/neup.12327"],"URL":"https:\/\/doi.org\/10.1111\/neup.12327","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"value":"0919-6544","type":"print"},{"value":"1440-1789","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,7,20]]}}}