{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T09:55:34Z","timestamp":1780912534599,"version":"3.54.1"},"reference-count":28,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2021,6,26]],"date-time":"2021-06-26T00:00:00Z","timestamp":1624665600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2021,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Sporadic Creutzfeldt\u2013Jakob disease is a rapidly progressing and highly variable neurodegenerative disease with heterogeneous clinical presentation and a median survival time from diagnosis to death of 4\u20136\u00a0months.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We report a rare case of a 61\u2010year\u2010old woman with a history of initially rapidly progressive dementia, with subsequent development of pyramidal and extrapyramidal signs and with an unusually long survival period of 14\u00a0years. Initial magnetic resonance imaging evaluation, single\u2010photon emission computed tomography, and electroencephalogram did not show relevant alterations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The postmortem examination of the brain showed diffuse spongiform change, gliosis, and neuronal loss along with abnormal immunostaining of prion protein in the grey matter, especially in the cerebellum. Indirect <jats:italic>PRNP<\/jats:italic> genetic analysis was negative.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This case is, to our knowledge, the sporadic Creutzfeldt\u2013Jakob disease patient with the longest survival period ever documented. This surprisingly long duration highlights the importance of histopathological confirmation with brain autopsies for suspected cases, as the disease can easily be misdiagnosed in such slowly progressing cases.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.14946","type":"journal-article","created":{"date-parts":[[2021,6,1]],"date-time":"2021-06-01T09:39:09Z","timestamp":1622540349000},"page":"2901-2906","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Sporadic Creutzfeldt\u2013Jakob disease with extremely long 14\u2010year survival period"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8107-660X","authenticated-orcid":false,"given":"Izaro","family":"Kortazar\u2010Zubizarreta","sequence":"first","affiliation":[{"name":"Department of Neurology Bioaraba Health Research Institute Araba University Hospital\u2010Txagorritxu Vitoria\u2010Gasteiz 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