{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T01:52:11Z","timestamp":1789869131120,"version":"4.0.1"},"reference-count":16,"publisher":"BMJ","issue":"9","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2019,8,28]]},"published-print":{"date-parts":[[2019,9]]},"abstract":"<jats:p>Antemortem assessment of sporadic Creutzfeldt-Jakob disease (sCJD) can be significantly hampered due to its rarity, low index of clinical suspicion and its non-specific clinical features. We present an atypical case of definitive sCJD. The patient died within 5\u2009weeks of the disease onset. This unusually short duration of disease presented a significant diagnostic dilemma. The patient presented with 2-week history of sudden-onset cognitive decline, memory loss, aphasia and ataxia. MRI Diffusion-weighted sequences revealed cortical ribboning sign without cerebral atrophy. Protein 14-3-3 from cerebrospinal fluid (CSF) was detected, and postmortem brain autopsy confirmed the diagnosis of sCJD. This case underscores the importance of considering CJD as a potential diagnosis for rapidly progressive dementia. Serology tests, EEG, MRI and CSF study are invaluable diagnostic tools when assessing for sCJD. Appropriate use of those diagnostic tests, along with a detailed clinical examination, can successfully and promptly exclude other differential diagnoses and confirm sCJD.<\/jats:p>","DOI":"10.1136\/bcr-2019-230535","type":"journal-article","created":{"date-parts":[[2019,9,24]],"date-time":"2019-09-24T11:45:13Z","timestamp":1569325513000},"page":"e230535","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":19,"title":["Diagnostic challenge of rapidly progressing sporadic Creutzfeldt-Jakob disease"],"prefix":"10.1136","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0878-0120","authenticated-orcid":false,"given":"Gi Tae","family":"Kwon","sequence":"first","affiliation":[{"name":"The Canberra Hospital, ACT Health, Canberra, Australian Capital Territory, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min Sung","family":"Kwon","sequence":"additional","affiliation":[{"name":"Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2019,9,24]]},"reference":[{"key":"2025082702582641000_12.9.e230535.1","first-page":"136","article-title":"Creutzfeldt-Jakob disease: a case report and differential diagnoses","volume":"72","author":"Kojima","year":"2013","journal-title":"Hawaii J Med Public Health"},{"key":"2025082702582641000_12.9.e230535.2","doi-asserted-by":"publisher","DOI":"10.3171\/2015.9.FOCUS15497"},{"key":"2025082702582641000_12.9.e230535.3","first-page":"361","article-title":"Human transmissible spongiform encephalopathies","volume":"73","year":"1998","journal-title":"Wkly Epidemiol Rec"},{"key":"2025082702582641000_12.9.e230535.4","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199812313392707"},{"key":"2025082702582641000_12.9.e230535.5","doi-asserted-by":"publisher","DOI":"10.1126\/science.3014653"},{"key":"2025082702582641000_12.9.e230535.6","first-page":"1551","article-title":"Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis","volume":"26","author":"Young","year":"2005","journal-title":"AJNR Am J Neuroradiol"},{"key":"2025082702582641000_12.9.e230535.7","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.23.13363"},{"key":"2025082702582641000_12.9.e230535.8","doi-asserted-by":"publisher","DOI":"10.3201\/eid1806.120116"},{"key":"2025082702582641000_12.9.e230535.9","unstructured":"Organization WH. Global surveillance, diagnosis and therapy of human transmissible spongiform encephalopathies: report of a WHO consultation, 1998."},{"key":"2025082702582641000_12.9.e230535.10","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1111\/neup.12016","article-title":"Human prion diseases: molecular, cellular and population biology","volume":"33","author":"Head","year":"2013","journal-title":"Neuropathology"},{"key":"2025082702582641000_12.9.e230535.11","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp191"},{"key":"2025082702582641000_12.9.e230535.12","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000134555.59460.5D"},{"key":"2025082702582641000_12.9.e230535.13","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2004.05.032"},{"key":"2025082702582641000_12.9.e230535.14","doi-asserted-by":"crossref","first-page":"735","DOI":"10.3171\/JNS\/2008\/109\/10\/0735","article-title":"Brain biopsy in patients with suspected Creutzfeldt-Jakob disease","volume":"109","author":"Heinemann","year":"2008","journal-title":"J Neurosurg"},{"key":"2025082702582641000_12.9.e230535.15","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23589"},{"key":"2025082702582641000_12.9.e230535.16","doi-asserted-by":"publisher","DOI":"10.1227\/NEU.0000000000000756"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2019-230535","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T09:58:36Z","timestamp":1756288716000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2019-230535"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,9]]},"references-count":16,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2019,9,24]]},"published-print":{"date-parts":[[2019,9]]}},"alternative-id":["10.1136\/bcr-2019-230535"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2019-230535","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,9]]}}}