{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:21:11Z","timestamp":1790814071447,"version":"4.1.0"},"reference-count":25,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2018,8,6]],"date-time":"2018-08-06T00:00:00Z","timestamp":1533513600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["15K08369"],"award-info":[{"award-number":["15K08369"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:p>We present an autopsied case of non\u2010plaque\u2010type dura mater graft\u2010associated Creutzfeldt\u2010Jakob disease (dCJD) with extensive amyloid\u2010\u03b2 (A\u03b2) deposition in the brain. A 39\u2010year\u2010old Japanese woman presented with memory disturbance and abnormal behavior. The patient had a history of craniotomy with dura matter\u2010graft transplant for a head injury which occurred when she was 19\u2009years old. Magnetic resonance imaging (MRI) showed hyperintensities in the cerebral cortex and striatum on diffusion\u2010weighted images, particularly on the dura mater\u2010grafted right side. Her clinical symptoms, including rapidly progressing cognitive impairment, myoclonus, and periodic sharp wave complexes on electroencephalogram, could not be distinguished from typical sporadic CJD cases. The patient died 11\u2009months after symptom onset, and pathological investigations showed extensive spongiform degeneration with prion protein (PrP) deposition without Kuru plaques; these observations were essentially the same as those of typical sporadic CJD cases. Furthermore, A\u03b2 immunohistochemistry showed extensive diffuse staining in the cerebral neocortex, plaque\u2010type deposition, positive staining in the pia mater, and cerebral amyloid angiopathy. Although the MRI findings suggested that the pathological involvement originated from the dura mater\u2010grafted right side, the PrP and A\u03b2 depositions showed no apparent regionalization and laterality. Tau\u2010pathology including neurofibrillary tangles was hardly identified. The proteins phosphorylated \u03b1\u2010synuclein and phosphorylated transactivation response DNA\u2010binding protein 43 kDa were not detected on immunostaining. Although this report describes only one case, various speculations were made based on detailed clinical and pathological observations in conjunction with previous reports of dCJD. In particular, this report provides significant insight into the characteristics and progression of dCJD pathology and its relationship with A\u03b2 pathology.<\/jats:p>","DOI":"10.1111\/neup.12503","type":"journal-article","created":{"date-parts":[[2018,8,7]],"date-time":"2018-08-07T11:31:44Z","timestamp":1533641504000},"page":"549-556","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Autopsied case of non\u2010plaque\u2010type dura mater graft\u2010associated Creutzfeldt\u2010Jakob disease presenting with extensive amyloid\u2010\u03b2 deposition"],"prefix":"10.1111","volume":"38","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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