{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T06:46:34Z","timestamp":1782283594935,"version":"3.54.5"},"reference-count":17,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T00:00:00Z","timestamp":1782259200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Public Health"],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive prion disease with established zoonotic transmission only in its variant form. Chronic wasting disease (CWD), a prion disease affecting cervid populations in North America, continues to expand geographically, raising public health concerns regarding potential interspecies transmission.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We describe the clinical course, diagnostic evaluation, and public health investigation of a patient with confirmed sporadic CJD and long-term venison exposure from CWD-endemic regions in Louisiana. Clinical data, neuroimaging, electroencephalography, cerebrospinal fluid (CSF) biomarkers, neuropathology, and epidemiologic findings were reviewed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case report<\/jats:title>\n                    <jats:p>A 72-year-old lifelong hunter developed rapidly progressive dementia with startle myoclonus and characteristic MRI findings of cortical ribboning and basal ganglia diffusion restriction. CSF biomarkers (RT-QuIC, total tau, 14-3-3) and postmortem neuropathology confirmed sporadic CJD, MM1 subtype. During hospitalization, the patient developed posterior reversible encephalopathy syndrome (PRES), complicating radiographic interpretation. Given extensive cervid exposure and reports of similar illness among hunting peers from the same lodge, the case was reported to public health authorities. Given extensive cervid exposure and reports of similar illness among hunting peers from the same lodge, the apparent clustering of suspected prion disease prompted epidemiologic investigation and public health review.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights diagnostic and public health challenges posed by prion disease in individuals with relevant zoonotic exposure histories. Although no evidence supporting confirmed CWD-to-human transmission was identified, the apparent clustering of suspected prion disease within a shared exposure network appropriately prompted epidemiologic investigation, structured exposure assessment, and public health review.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fpubh.2026.1847770","type":"journal-article","created":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T05:47:15Z","timestamp":1782280035000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Sporadic Creutzfeldt-Jakob disease following venison exposure in a chronic wasting disease-endemic region: a zoonotic surveillance perspective"],"prefix":"10.3389","volume":"14","author":[{"given":"Eithan","family":"Kotkowski","sequence":"first","affiliation":[{"name":"Department of Neurology, UT Health San Antonio","place":["San Antonio, TX, United States"]},{"name":"Research Imaging Institute, UT Health San Antonio","place":["San Antonio, TX, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan","family":"Trout","sequence":"additional","affiliation":[{"name":"Department of Neurology, UT Health San Antonio","place":["San Antonio, TX, United States"]},{"name":"Department of Neurology, University of Utah Health","place":["Salt Lake City, UT, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Roberts","sequence":"additional","affiliation":[{"name":"Department of Neurology, UT Health San Antonio","place":["San Antonio, TX, United States"]},{"name":"University of South Carolina School of Medicine Greenville","place":["Greenville, SC, United States"]},{"name":"Division of Neurology, Prisma Health Upstate","place":["Greenville, SC, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Tabet","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, UT Health San Antonio","place":["San Antonio, TX, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlayne","family":"Jackson","sequence":"additional","affiliation":[{"name":"Department of Neurology, UT Health San Antonio","place":["San Antonio, TX, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Horn","sequence":"additional","affiliation":[{"name":"Department of Neurology, UT Health San Antonio","place":["San Antonio, TX, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,6,24]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"106103","DOI":"10.1016\/j.clineuro.2020.106103","article-title":"Creutzfeldt\u2013Jakob disease: in-hospital demographics report of national data in the United States from 2016 and review of a rapidly progressive case","volume":"197","author":"Kotkowski","year":"2020","journal-title":"Clin Neurol Neurosurg"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"767","DOI":"10.1002\/ana.410390613","article-title":"Molecular basis of phenotypic variability in sporadic Creutzfeldt\u2013Jakob disease","volume":"39","author":"Parchi","year":"1996","journal-title":"Ann Neurol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"1134225","DOI":"10.3389\/fneur.2023.1134225","article-title":"Case report: two clusters of Creutzfeldt\u2013Jakob disease cases within 1 year in West Michigan","volume":"14","author":"Rong","year":"2023","journal-title":"Front Neurol"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"89","DOI":"10.7589\/0090-3558-16.1.89","article-title":"Chronic wasting disease of captive mule deer: a spongiform encephalopathy","volume":"16","author":"Williams","year":"1980","journal-title":"J Wildl 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presenting as posterior reversible encephalopathy syndrome","volume":"12","author":"Bittar","year":"2020","journal-title":"Cureus"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"914","DOI":"10.1016\/S1474-4422(15)00111-8","article-title":"Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions","volume":"14","author":"Fugate","year":"2015","journal-title":"Lancet Neurol"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"1121","DOI":"10.1038\/nm922","article-title":"The sympathetic nervous system is involved in variant Creutzfeldt\u2013Jakob disease","volume":"9","author":"Ha\u00efk","year":"2003","journal-title":"Nat Med"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"977","DOI":"10.3201\/eid1006.031082","article-title":"Chronic wasting disease and potential transmission to humans","volume":"10","author":"Belay","year":"2004","journal-title":"Emerg Infect 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