Sporadic Creutzfeldt-Jakob disease following venison exposure in a chronic wasting disease-endemic region: a zoonotic surveillance perspective.

Background: 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. Methods: 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. Case report: 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. Conclusion: 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.