{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T10:07:58Z","timestamp":1780481278697,"version":"3.54.1"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,4,19]],"date-time":"2022-04-19T00:00:00Z","timestamp":1650326400000},"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":[[2022,6]]},"abstract":"<jats:p>In Japan, because MV2\u2010type sporadic Creutzfeldt\u2013Jakob disease (CJD) is rare, little is known about its clinical and neuropathological characteristics. An autopsy case of MV2K\u2010type sporadic CJD is presented, and the characteristic clinical, radiological, and neuropathological findings are discussed. The patient was a Japanese woman who died at the age of 72\u2009years. Her initial symptom was rapidly progressive dementia. She then developed truncal ataxia and delusions. Approximately nine months after onset, she exhibited akinetic mutism. The total clinical course was 11\u2009months. Magnetic resonance imaging revealed hyperintensity areas in the basal ganglia, thalamus, and hippocampus on diffusion\u2010weighted images. In the cerebral cortex, this finding was slight and inconspicuous. Electroencephalography revealed no periodic sharp wave complexes. Prion protein (PrP) gene analysis revealed no mutations, and polymorphic codon 129 exhibited methionine and valine heterozygosity. In the cerebrospinal fluid, levels of both total tau and 14\u20103\u20103 proteins were elevated. Grossly, the brain weighed 1050\u2009g before fixation and exhibited diffuse cortical atrophy. On histopathological examination, extensive fine vacuole\u2010type spongiform degeneration was noted in the cerebral cortex. Numerous kuru plaques were observed in the cerebellum. PrP immunohistochemistry revealed extensive diffuse synaptic\u2010 and perineuronal\u2010type PrP deposits in the cerebral cortex. Kuru plaques were strongly immunoreactive for PrP. Western blot analysis of brain tissue samples revealed mixed type 2 and intermediate type. Systematic and comprehensive investigations of both clinical and neuropathological aspects are required for accurate diagnosis.<\/jats:p>","DOI":"10.1111\/neup.12804","type":"journal-article","created":{"date-parts":[[2022,4,20]],"date-time":"2022-04-20T00:39:45Z","timestamp":1650415185000},"page":"245-253","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["An autopsy case of <scp>MV2K<\/scp>\u2010type sporadic <scp>Creutzfeldt\u2010Jakob<\/scp> disease presenting with characteristic clinical, radiological, and neuropathological findings"],"prefix":"10.1111","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5799-362X","authenticated-orcid":false,"given":"Koushun","family":"Matsuo","sequence":"first","affiliation":[{"name":"Division of Neurology Ohmihachiman Community Medical Center  Ohmihachiman 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