{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,14]],"date-time":"2026-01-14T16:34:10Z","timestamp":1768408450634,"version":"3.49.0"},"reference-count":28,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2024,10,28]],"date-time":"2024-10-28T00:00:00Z","timestamp":1730073600000},"content-version":"vor","delay-in-days":31,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,9,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Globally, Creutzfeldt-Jakob disease (CJD) affects one in one million people annually, but there is a paucity of recent Canadian data. This study summarizes epidemiology trends and diagnostic timelines of laboratory-confirmed CJD cases in three tertiary Ontario hospitals.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Method<\/jats:title>\n                  <jats:p>Using laboratory information systems, we identified 30 patients with a laboratory-confirmed CJD diagnosis between 2012 and 2022 at three major tertiary hospitals in Ontario. Retrospective chart reviews were then completed.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Patients had a mean of 2.2 hospital visits (SD, 1.2) prior to being admitted for testing. The most common symptom presentations included loss of coordination (63.3%), behavioral changes (60%), progressive mobility loss (53.4%), memory loss (50.0%), and involuntary movements (50.0%). Magnetic resonance imaging findings showed potential CJD in 76.7% of cases, and 56.7% exhibited periodic sharp wave complexes characteristic of CJD on electroencephalogram. The mean duration from symptom onset to microbiologic testing was 91 days (SD, 90.7). End-point quaking-induced conversion (EP-QuIC) testing of cerebrospinal fluid was positive in 90.0% of patients, while 83.3% tested positive for 14-3-3 on enzyme-linked immunosorbent assay. Elevated cerebrospinal fluid 14-3-3 levels significantly correlated with shorter duration from symptom onset to death (R2 = 0.71, F = 19.55, P = .0022). Post-diagnosis, 46.7% of patients were discharged home, 16.6% were transferred to external palliative care or hospice facilities, and 36.7% died during admission. The mean time from symptom onset to death was 121 days (SD, 120.7), and from diagnosis to death 35 days (SD, 83.9).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>This study highlights the importance of early CJD consideration and laboratory testing when appropriate neurologic symptoms are present.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ofid\/ofae551","type":"journal-article","created":{"date-parts":[[2024,10,28]],"date-time":"2024-10-28T22:51:33Z","timestamp":1730155893000},"source":"Crossref","is-referenced-by-count":1,"title":["Characterization of Laboratory-Confirmed Creutzfeldt-Jakob Disease From 3 Ontario Tertiary Care Centers Between 2012 and 2022: A Retrospective Cohort Study"],"prefix":"10.1093","volume":"11","author":[{"given":"Kayla","family":"Gaete","sequence":"first","affiliation":[{"name":"Biological Sciences Platform, Sunnybrook Health Sciences Centre , Toronto, Ontario ,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Soma","family":"Dalai","sequence":"additional","affiliation":[{"name":"Kingston Health Sciences Centre , Kingston, Ontario ,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana","family":"Cabrera","sequence":"additional","affiliation":[{"name":"London Health Sciences Centre (Victoria Hospital) , London, Ontario ,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xena","family":"Li","sequence":"additional","affiliation":[{"name":"Shared Hospital Laboratory , Toronto, Ontario ,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Prameet M","family":"Sheth","sequence":"additional","affiliation":[{"name":"Shared Hospital Laboratory , Toronto, Ontario ,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert A","family":"Kozak","sequence":"additional","affiliation":[{"name":"Biological Sciences Platform, Sunnybrook Health Sciences Centre , Toronto, Ontario ,","place":["Canada"]},{"name":"Shared Hospital Laboratory , Toronto, Ontario ,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mia J","family":"Biondi","sequence":"additional","affiliation":[{"name":"School of Nursing, York University , Toronto, Ontario ,","place":["Canada"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2024,10,28]]},"reference":[{"key":"2024102921293027200_ofae551-B1","author":"Public Health Agency of Canada"},{"key":"2024102921293027200_ofae551-B2","author":"National Institutes of Health"},{"key":"2024102921293027200_ofae551-B3","author":"Public Health Agency of Canada"},{"key":"2024102921293027200_ofae551-B4","doi-asserted-by":"crossref","first-page":"e2","DOI":"10.1016\/S1473-3099(19)30615-2","article-title":"Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation","volume":"20","author":"Uttley","year":"2020","journal-title":"Lancet Infect Dis"},{"key":"2024102921293027200_ofae551-B5","doi-asserted-by":"crossref","first-page":"1345","DOI":"10.1056\/NEJMra2119323","article-title":"Laboratory diagnosis of Creutzfeldt-Jakob disease","volume":"386","author":"Zerr","year":"2022","journal-title":"N Engl J Med"},{"key":"2024102921293027200_ofae551-B6","volume-title":"Creutzfeldt Jakob disease","author":"Sitammagari","year":"2024"},{"key":"2024102921293027200_ofae551-B7","author":"CJD Centers for Disease Control and Prevention"},{"key":"2024102921293027200_ofae551-B8","doi-asserted-by":"crossref","first-page":"2053","DOI":"10.12688\/f1000research.12681.1","article-title":"Creutzfeldt-Jakob disease: recent developments","volume":"6","author":"Mackenzie","year":"2017","journal-title":"F1000Res"},{"key":"2024102921293027200_ofae551-B9","author":"Public Health Agency of Canada"},{"key":"2024102921293027200_ofae551-B10","author":"Public Health Agency of Canada"},{"key":"2024102921293027200_ofae551-B11","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1001\/jamaneurol.2023.4678","article-title":"Change in epidemiology of Creutzfeldt-Jakob disease in the US, 2007\u20132020","volume":"81","author":"Crane","year":"2024","journal-title":"JAMA Neurol"},{"key":"2024102921293027200_ofae551-B12","doi-asserted-by":"crossref","first-page":"15509","DOI":"10.1038\/s41598-020-72519-0","article-title":"A nationwide trend analysis in the incidence and mortality of Creutzfeldt-Jakob disease in Japan between 2005 and 2014","volume":"10","author":"Nishimura","year":"2020","journal-title":"Sci Rep"},{"key":"2024102921293027200_ofae551-B13","author":"Public Health Agency of Canada"},{"key":"2024102921293027200_ofae551-B14","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1007\/s10654-023-01004-5","article-title":"The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis","volume":"38","author":"Denouel","year":"2023","journal-title":"Eur J Epidemiol"},{"key":"2024102921293027200_ofae551-B15","first-page":"e45757","article-title":"Sporadic Creutzfeldt-Jakob disease with COVID-19 infection: a case report","volume":"15","author":"Singh","year":"2023","journal-title":"Cureus"},{"key":"2024102921293027200_ofae551-B16","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1016\/j.bbi.2020.07.007","article-title":"Creutzfeldt-Jakob disease in a man with COVID-19: SARS-CoV-2\u2013accelerated neurodegeneration?","volume":"89","author":"Young","year":"2020","journal-title":"Brain Behav Immun"},{"key":"2024102921293027200_ofae551-B17","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1080\/19336896.2022.2095185","article-title":"Creutzfeldt-Jakob disease after COVID-19: infection-induced prion protein misfolding? 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