MDLinxTwo Men Die After Eating Deer Meat Infected with Prion Disease

Rare Prion Disease Cases in Hunters Spark Debate on Zoonotic Transmission Potential

A recent report of two hunters developing Creutzfeldt-Jakob disease (CJD) after consuming deer meat infected with chronic wasting disease (CWD) has ignited discussions about the potential for zoonotic transmission of prion diseases. Although the connection between these cases and CWD remains uncertain, the incident highlights the need for continued vigilance and research in the field of prion diseases, particularly those that affect both animals and humans.

Key Points:

• Two hunters who frequently ate deer meat infected with CWD developed CJD and subsequently died.
• CJD is a rare, fatal prion disease that typically affects older adults and can cause dementia-like symptoms.
• CWD is a prion disease affecting cervids (deer, elk, moose) and has not been proven to infect humans.
• The CDC states that zoonotic transmission of CJD from deer to humans is unlikely.
• Variant CJD (vCJD), a different prion disease, can be transmitted to humans from cows infected with bovine spongiform encephalopathy (BSE).
• A recent NIH study found that human organoids exposed to CWD prions did not become infected, suggesting a substantial barrier to transmission.
• Researchers acknowledge limitations in their study, including potential genetic susceptibility and the possibility of new CWD strains emerging.
• The CDC recommends that neurologists utilize the National Prion Disease Pathology Surveillance Center for accurate diagnosis of prion diseases.
• To reduce surgical transmission risks, doctors should consider using disposable instruments, stringent sterilization methods, or quarantining instruments when prion disease is suspected.
• Ruling out more common, treatable diseases is crucial before diagnosing CJD, as there is currently no cure.

“A history of hunting and/or eating venison does not mean that someone got CJD that way. Many Americans hunt and even more eat venison. Some will develop sporadic CJD by chance and others will not.”
– Ryan A. Maddox, PhD, CDC epidemiologist

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