An abstract presented in the April issue of Neurology details an occurrence in which two elderly men from the same lodge contracted Creutzfeldt-Jakob disease (CJD, also known as Mad Cow disease). Both had a history of consuming meat from a CWD-infected deer population.
CJD is a rapidly progressive central nervous system disorder caused by misfolded prion proteins.
CWD, a prion disease that is prevalent and fatal to North American deer, has raised concerns due to its possible link to CJD. Currently, however, there is no conclusive evidence suggesting that cross-species prion transmission exists.
“Details are scarce, so we can’t conclude that these human cases were caused by the consumption of CWD-infected meat,” Ontario Federation of Anglers and Hunters Manager of Policy Mark Ryckman said. “But CJD is rare, so finding two cases of CJD in a single social group is a serious concern and demands closer inspection.”
CWD species crossing possible
Thousands of pounds of CWD-infected meat are consumed every year by hunters in North America, and yet routine surveillance for the human version (CJD) has not detected any increase in CJD cases, he said.
“This suggests that CWD has not yet crossed the species barrier into humans, but there is no scientific reason that it couldn’t,” Ryckman said.
The incident highlights the importance of surveillance and monitoring for CJD in people and getting your deer tested if you’re hunting in an area where CWD is endemic, he added.
CJD can occur spontaneously and develop without reasons, or may be inherited, or contracted by contact with infected tissue or during a surgical procedure, blood transfusion, or consuming contaminated beef, Ryckman noted.
The authors of the study concluded, “Clusters of sporadic CJD cases may occur in regions with CWD-confirmed deer populations, hinting at potential cross-species prion transmission. Surveillance and further research are essential to better understand this possible association.”
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