{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T10:00:21Z","timestamp":1780480821535,"version":"3.54.1"},"reference-count":27,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2024,2,14]],"date-time":"2024-02-14T00:00:00Z","timestamp":1707868800000},"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":[[2024,8]]},"abstract":"<jats:p>Methionine\/valine (MV) 2 type of sporadic Creutzfeldt\u2013Jakob (sCJD) is divided into three subtypes based on neuropathological criteria: MV2\u2010kuru (MV2K), MV2\u2010cortical (MV2C), and MV2K\u2009+\u2009C, exhibiting the co\u2010occurrence of these two pathological features. We report an autopsy case of MV2K\u2009+\u2009C subtype of sCJD. A 46\u2010year\u2010old Japanese man began to make mistakes at work. Two months later, he gradually developed gait instability. The initial neurological examination revealed limb ataxia and myoclonus. Diffusion\u2010weighted images (DWI) showed a hyperintensity in the right frontal cortex, basal ganglia, and thalamus. Ten months after the onset of disease, he fell into akinetic mutism. He died at 47\u2009years of age, 12\u2009months after the initial presentation. Pathological investigation revealed microvacuolation and confluent vacuoles in the cerebral cortex. In the basal ganglia and thalamus, there was severe neuronal loss and gliosis with mild spongiform change. Kuru plaques were found within the cerebellum. Prion protein (PrP) immunostaining revealed synaptic, perivacuolar, perineuronal, and plaque\u2010like deposits in the cerebral cortex. There were synaptic and plaque\u2010like PrP deposits in the basal ganglia, thalamus, and granular cell layer of the cerebellum. In these areas, plaque\u2010like deposits mainly consisted of small deposits, whereas plaque\u2010like deposits in the cerebral cortex consisted both of coarse granular and small deposits. Analysis of the PrP gene showed no pathogenic mutations, and Western blot examination revealed a mixture of type 2 and intermediate\u2010type PrP. The progressive cognitive decline and ataxia in addition to the hyperintensity in the basal ganglia and\/or thalamus on DWI are the basis for clinical diagnosis of MV2. The severe gliosis in the basal ganglia and various morphologies of plaque\u2010like deposits that differ by the region may be characteristic of MV2K\u2009+\u2009C. Detailed neuropathological examination together with Western blot analysis is important to collect more cases for elucidating the pathogenesis of MV2K\u2009+\u2009C.<\/jats:p>","DOI":"10.1111\/neup.12964","type":"journal-article","created":{"date-parts":[[2024,2,14]],"date-time":"2024-02-14T09:29:46Z","timestamp":1707902986000},"page":"304-313","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["An autopsy case of <scp>MV 2K<\/scp>\u2009+\u2009C subtype of <scp>C<\/scp>reutzfeldt\u2013<scp>J<\/scp>akob disease"],"prefix":"10.1111","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7792-4832","authenticated-orcid":false,"given":"Akiko","family":"Uchino","sequence":"first","affiliation":[{"name":"Department of Preventive medical center Kitasato University Kitasato Institute Hospital  Tokyo Japan"},{"name":"Department of Neuropathology (Brain Bank for Aging Research) Tokyo Metropolitan Institute for Geriatrics and Gerontology  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Neuropathology (Brain Bank for Aging Research) Tokyo Metropolitan Institute for Geriatrics and Gerontology  Tokyo Japan"},{"name":"Department of Pathology and Laboratory Medicine National Center Hospital, National Center of Neurology and Psychiatry  Kodaira\u2010shi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sho","family":"Tokuda","sequence":"additional","affiliation":[{"name":"Department of Neurology NHO Sagamihara National Hospital  Sagamihara\u2010shi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yagishita","family":"Saburo","sequence":"additional","affiliation":[{"name":"Department of Neurology NHO Sagamihara National Hospital  Sagamihara\u2010shi 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