{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,8,2]],"date-time":"2024-08-02T07:12:41Z","timestamp":1722582761485},"reference-count":13,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2014,7,2]],"date-time":"2014-07-02T00:00:00Z","timestamp":1404259200000},"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":[[2014,12]]},"abstract":"We report a clinical case report of the MV2K<\/jats:styled-content>+C<\/jats:styled-content> subtype of sporadic C<\/jats:styled-content>reutzfeldt\u2010J<\/jats:styled-content>akob disease (sCJD<\/jats:styled-content>). The patient was a 72\u2010year\u2010old woman who exhibited progressive dementia over the course of 22 months. Diffusion\u2010weighted MRI<\/jats:styled-content> during this period showed abnormal hyperintensity in the cerebral cortex in the early stage. The clinical course was similar to that of previously reported patients with the MV2K<\/jats:styled-content> or MV2K<\/jats:styled-content>+C<\/jats:styled-content> subtype of sCJD<\/jats:styled-content>. However, histopathological examination revealed unique features: severe extensive spongiform changes with perivacuolar deposits in the cerebrum and basal ganglia, plaque\u2010like PrP<\/jats:styled-content> deposits in the cerebrum, and only mild changes in the cerebellum with small amyloid plaques (\u223c20\u2009\u03bcm in diameter), smaller than those in the MV2K<\/jats:styled-content> subtype or variant CJD<\/jats:styled-content> (40\u201350\u2009\u03bcm in diameter). Molecular analysis showed a methionine\/valine heterozygosity at codon 129 and no pathogenic mutation in the PrP<\/jats:styled-content> gene (PRNP<\/jats:styled-content><\/jats:italic>). Western blot analysis of the protease\u2010resistant PrP<\/jats:styled-content> (PrPSc<\/jats:sup><\/jats:styled-content>) in the right temporal pole revealed the type 2 pattern, which is characterized by a single unglycosylated band, in contrast to the doublet described for the typical MV2<\/jats:styled-content> subtype of sCJD<\/jats:styled-content>. The other intermediate band might exist in the cerebellum with kuru plaques. Therefore, small amyloid plaques in the cerebellum can be crucial for MV2K<\/jats:styled-content>+C<\/jats:styled-content> subtype.<\/jats:p>","DOI":"10.1111\/neup.12133","type":"journal-article","created":{"date-parts":[[2014,7,2]],"date-time":"2014-07-02T13:19:46Z","timestamp":1404307186000},"page":"541-546","source":"Crossref","is-referenced-by-count":2,"title":["Extensive cortical spongiform changes with cerebellar small amyloid plaques: The clinicopathological case of MV2K<\/scp>+C<\/scp> subtype in C<\/scp>reutzfeldt\u2010J<\/scp>akob disease"],"prefix":"10.1111","volume":"34","author":[{"given":"Kunihiko","family":"Araki","sequence":"first","affiliation":[{"name":"Department of Neurology National Epilepsy Center Shizuoka Institute of Epilepsy and Neurological Disorders Shizuoka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuta","family":"Nakano","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Tokyo Metropolitan Geriatric 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