{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,20]],"date-time":"2026-03-20T09:20:41Z","timestamp":1773998441675,"version":"3.50.1"},"reference-count":22,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2004,10,7]],"date-time":"2004-10-07T00:00:00Z","timestamp":1097107200000},"content-version":"vor","delay-in-days":7341,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1984,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Five to 10% of patients with Creutzfeldt\u2010Jakob disease have a clinical course that extends for 2 years or more. In the present series 33 (9%) of 357 histopathologically verified cases, including 15 (7%) of 225 transmitted cases, fell into this long\u2010duration category, the longest transmitted case having had a 13\u2010year duration. As a group these cases were characterized by a higher familial representation (30%), a younger age at onset (average, 48 years), and lower frequencies of myoclonus (79%) and periodic electroencephalographic activity (45%) than occur in series of unselected cases. The clinical course most often showed a long, very slowly progressive terminal stage with both mental and physical deterioration. Although transmission of disease by primate inoculation periods and durations of illness in the long\u2010duration cases (64%) than in cases of shorter duration (88%), incubation periods and duration of illness in the inoculated animals bore no relation to the duration of illness in the patients. Clinical differentiation from other chronic dementing processes, particularly Alzheimer's disease, can prove impossible; however, the correct pathologica diagnosis was readily evident by light microscopic examination, and spongiosis was at least as prominent as gliosis in all but 4(15%) of the 27 patients studied postmortem, the same frequency found in case series not selected for length of illness.<\/jats:p>","DOI":"10.1002\/ana.410160305","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T17:14:59Z","timestamp":1104599699000},"page":"295-304","source":"Crossref","is-referenced-by-count":123,"title":["Creutzfeldt\u2010Jakob disease of long duration: Clinicopathological characteristics, transmissibility, and differential diagnosis"],"prefix":"10.1002","volume":"16","author":[{"given":"Paul","family":"Brown","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pamela","family":"Rodgers\u2010Johnson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fran\u00e7oise","family":"Cathala","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr","given":"Clarence J.","family":"Gibbs","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D. 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