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MRIs were evaluated with a semiquantitative rating score, which separately considered abnormal signal extent and intensity in 22 brain regions. Clinical findings at onset, disease duration, cerebrospinal-fluid 14-3-3 and t-tau protein levels, and EEG data were recorded.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Among patients with definite\/probable diagnosis of CJD or genetic prion disease, 2\/107 had normal DWI-MRI: in one patient a 2-months follow-up DWI-MRI showed CJD-related changes while the other had autopsy-proven CJD despite no DWI abnormalities 282\u00a0days after clinical onset. CJD-related cortical changes were detected in all lobes and involvement of thalamus was common. In the npRPD groups, 6\/40 patients showed DWI alterations that clustered in three different patterns: (1) minimal\/doubtful signal alterations (limbic encephalitis, dementia with Lewy bodies); (2) clearly suggestive of alternative diagnoses (status epilepticus, Wernicke or metabolic encephalopathy); (3) highly suggestive of CJD (mitochondrial disease), though cortical swelling let exclude CJD.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>In the diagnostic work-up of RPD, negative\/doubtful DWI makes CJD diagnosis rather unlikely, while specific DWI patterns help differentiating CJD from alternative diagnoses. The pulvinar sign is not exclusive of the variant form.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00415-023-11962-1","type":"journal-article","created":{"date-parts":[[2023,9,12]],"date-time":"2023-09-12T08:02:27Z","timestamp":1694505747000},"page":"300-309","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["MRI abnormalities in Creutzfeldt\u2013Jakob disease and other rapidly progressive dementia"],"prefix":"10.1007","volume":"271","author":[{"given":"Renzo","family":"Manara","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Fragiacomo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Ladogana","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luana","family":"Vaianella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Camporese","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni","family":"Zorzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Vicinanza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianluigi","family":"Zanusso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Pocchiari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annachiara","family":"Cagnin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,9,12]]},"reference":[{"key":"11962_CR1","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1002\/ana.21430","volume":"64","author":"MD Geschwind","year":"2008","unstructured":"Geschwind MD, Shu H, Haman A, Sejvar JJ, Miller BL (2008) Rapidly progressive dementia. 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F Fragiacomo reports no disclosures. A Ladogana reports no disclosures. L Vaianella reports no disclosures. G Camporese received a grant by Veneto Region Finalized Research (RP-2014\u201300000400). G Zorzi reports no disclosures. S Vicinanza reports no disclosures. G Zanusso reports no disclosures. M Pocchiari reports no disclosures. A Cagnin served as scientific advisory board for Biogen and Angelini and was funded by a grant from Veneto Region (n: RP-2014\u201300000400).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}}]}}