{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T06:07:55Z","timestamp":1760854075880,"version":"3.40.5"},"reference-count":21,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2019,9,12]],"date-time":"2019-09-12T00:00:00Z","timestamp":1568246400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:p>Comprehensive analysis is required for the accurate diagnosis of MV2\u2010type sporadic Creutzfeldt\u2013Jakob disease (sCJD) because it shows a wide clinicopathological spectrum. Here, we describe the clinical findings and neuropathologic observations of an autopsy\u2010confirmed MV2K\u2010type sCJD case with extensive spongiform changes of the cerebral cortex. In the early disease stages, the patient exhibited gait disturbance with ataxia and gradually showed cognitive dysfunction. Diffusion\u2010weighted magnetic resonance images revealed hyperintense regions in the cerebral cortex, basal ganglia, and particularly in the thalamus. Prion protein (PrP) gene analysis revealed no mutations, and polymorphic codon 129 exhibited methionine and valine heterozygosity. During the course of the disease, a startle reaction was observed, whereas myoclonus was not observed. Electroencephalography showed no periodic sharp wave complexes. The patient died at age 61\u2009years with 13\u2009months total disease duration and did not reach the akinetic mutism state. Pathologic investigation revealed extensive fine vacuole\u2010type spongiform change in the cerebral cortex, and the appearance of vacuolation tended to be more pronounced in the deeper layers. Numerous kuru plaques were observed in the cerebellum. PrP immunostaining revealed extensive diffuse synaptic\u2010type PrP deposition in the cerebral cortex, and the finding was prominent in the deeper layer with perineuronal\u2010type PrP deposition. In the limbic system, basal ganglia, and thalamus, mixed small plaque\u2010type PrP with synaptic\u2010type PrP deposition was observed. In the cerebellar cortex, diffuse synaptic\u2010type PrP depositions were observed with numerous strongly immunopositive plaques. Western blot analysis of examined brain samples revealed mixed type 2 PrP<jats:sup>Sc<\/jats:sup> (scrapie type) and intermediate\u2010type PrP<jats:sup>Sc<\/jats:sup>.<\/jats:p>","DOI":"10.1111\/neup.12595","type":"journal-article","created":{"date-parts":[[2019,9,13]],"date-time":"2019-09-13T05:00:00Z","timestamp":1568350800000},"page":"452-460","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Autopsy case of MV2K\u2010type sporadic Creutzfeldt\u2010Jakob disease with spongiform changes of the cerebral cortex"],"prefix":"10.1111","volume":"39","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 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Institute for Medical Science of Aging Aichi Medical University  Nagakute Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3452-0963","authenticated-orcid":false,"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"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0723-0047","authenticated-orcid":false,"given":"Hiroaki","family":"Miyahara","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute for Medical Science of Aging Aichi Medical University  Nagakute Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Atsushi","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Laboratory of Comparative Pathology, Faculty of Veterinary Medicine Hokkaido University  Sapporo 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