Two hunters in the US have died of a rare prion disease, an aggressive disorder of the central nervous system that’s caused by strange, rogue proteins. Worryingly, scientists are speculating that the pair caught the disease from North American deer infected with Chronic Wasting Disease (CWD). If true, it would be the first known case of its kind – although the researchers stress that “no conclusive evidence” has yet to be found.
The story unfolded in 2022 when a 72-year-old man suddenly started to experience confusion and aggression, according to a new study by the University of Texas Health Science Center San Antonio.
It became apparent that he and a friend had consumed venison from a deer population , another prion disease affecting deer, elk, reindeer, and moose. Nicknamed “zombie deer disease,” the illness causes animals to experience drastic weight loss, stumbling, erratic behavior, and other neurologic symptoms
Both patients later died. A post-mortem examination of the brains revealed they had developed sporadic Creutzfeldt-Jakob disease (CJD), the prion disease most commonly seen in humans.
“The patient’s history, including a similar case in his social group, suggests a possible novel animal-to-human transmission of CWD. Based on non-human primate and mouse models, cross-species transmission of CJD is plausible,” the study authors write.
Prion diseases are strange and very scary: they’re transmissible, untreatable, and always fatal.
They’re caused by the misfolding of prion proteins that naturally exist in healthy cells throughout the body, most notably in the brain. However, in prion diseases, the harmless protein becomes triggered to fold abnormally, resulting in a hostile and infectious form. This abnormal protein can convert other normal proteins into the abnormal form, accumulating in the brain. This is devastating for the brain. Prion infections cause small holes to develop in the brain, making it appear sponge-like.
In turn, infected individuals can suffer a rapid onset of physical and mental symptoms, including severe depression, hallucinations, slurred speech, numbness, loss of coordination, memory loss, aggression, and profound behavior changes.
Unlike most other infectious agents, prions are not inactivated by heat, ultraviolet light, or any other standard sterilization procedures, making them extremely difficult to deal with and impossible to treat.
It’s not always clear why the proteins initially become misfolded, although it’s evident that prion diseases can be transmitted by consuming infected meat.
In the 1980s and ’90s, there was a huge scare around bovine spongiform encephalopathy (BSE), also known as ““, a prion disease found in cattle. As per the latest statistics from the , 232 people around the world have died from the variant of Creutzfeldt-Jakob disease linked to mad cow disease. Most cases had lived at some point in the United Kingdom where the outbreak was most significant.
With regards to deer and CWD, it’s still not clear whether the prions can jump from animals to humans. However, this latest cast study certainly suggests it is a possibility that the public, doctors, and public health authorities need to consider.
The study is published in the journal .