A recent medical case report has raised significant concerns about the possibility of a deadly prion disease, known as chronic wasting disease, making its way from deer into humans. The report suggests that two hunters have tragically lost their lives after consuming venison from a deer population infected with chronic wasting disease. This fatal prion disease, sometimes referred to as “zombie deer” disease, shares similarities with bovine spongiform encephalopathy, also known as mad cow disease.
The doctors at the University of Texas detailed the case of a 72-year-old man who passed away after experiencing sudden confusion and aggression. Shockingly, his friend, a member of the same hunting lodge, also later succumbed to similar symptoms. A post-mortem examination revealed that the second patient had died from Creutzfeldt-Jakob disease (CJD), a form of prion disease that is incurable and fatal. Given that prion diseases are rare in humans, these cases raise concerns that chronic wasting disease may have crossed over into humans.
Prion diseases such as Creutzfeldt-Jakob disease are incredibly frightening due to the way misfolded proteins spread dysfunction throughout the brain. These abnormal proteins trigger surrounding healthy proteins to fold incorrectly, leading to a cascade effect that cannot be stopped or reversed. The resulting deterioration of brain tissue mimics symptoms of accelerated dementia, eventually leading to the patient’s demise. What makes prion diseases even more challenging is the absence of an immune response, making it nearly impossible to detect in living patients.
Chronic wasting disease poses a significant risk as it easily infects animals like deer, elk, and moose. The disease is believed to spread through bodily fluids such as blood or saliva, either through direct contact between animals or contamination in the environment. Even more concerning is the fact that chronic wasting disease has been shown to infect mice carrying human genetic material in laboratory studies. Despite these risks, a concerning number of animals with the disease are still consumed each year, with a projected increase in consumption annually.
While it is unclear whether the two individuals in the case report succumbed to chronic wasting disease, the possibility of zoonotic transmission remains a significant concern. Prion diseases can potentially arise spontaneously, though this occurrence is exceedingly rare. With chronic wasting disease present in wild populations across North America and even among farmed deer, the risk of cross-species transmission to humans cannot be ignored. The doctors emphasize the importance of further investigation into the potential risks associated with consuming CWD-infected deer and its implications for public health.
The emergence of prion diseases such as chronic wasting disease in humans underscores the need for vigilance and caution when it comes to interactions with infected animal populations. While the exact origin of the disease in these cases remains uncertain, the possibility of zoonotic transmission highlights the importance of continued research and monitoring to protect public health from the devastating consequences of prion diseases.
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