Researchers have described the cases of two hunters who died from a rare prion disease. The two men had eaten deer meat with zombie deer disease. They could be the first confirmed cases of infection in humans.
Two American hunters I am dead for a rare prion disease found in brain. According to a new study, it is likely that these are the first victims linked to the infamous “zombie deer disease”, known in scientific literature as chronic wasting disease in deer (CWD). Although there is no definitive confirmation yet, experts believe the passage of prions is plausible – misfolded proteins and toxic – from cervids to humans. Since zombie deer disease began to spread significantly among deer populations in the US, with hundreds of cases reported in recent months, researchers have raised the alarm about the possible risks to humans, particularly who consume meat of infected deer and is exposed to body fluids of the animals hunted. This is exactly the case of the two deceased hunters, who had consumed deer meat from one population in which the spread of the disease was confirmed. Experts suspect that the prions passed to the two people, causing the fatal disease.
The first two possible cases of death associated with chronic wasting disease (CWD) in deer were determined by a research team led by scientists from the Department of Neurology at the University of Texas Health Science Center San Antonio, who collaborated in close contact with colleagues from the Department of Psychiatry and the Long School of Medicine. The researchers, coordinated by Professor Jonathan Trout, followed the case of a 72-year-old man who had long consumed deer meat from a population with circulating CWD. In 2022 he developed confusion And aggression “rapid onset” and died, as highlighted in the study abstract. Subsequently a friend of his also developed i symptoms and passed away shortly thereafter with post-mortem diagnosis of Creutzfeldt-Jakob disease (CJD), more specifically, Sporadic Creutzfeldt-Jakob disease with methionine homozygous at codon 129 (sCJDMM1). He too had eaten infected deer meat.
As explained by the scholars, the fact that the two hunters developed the rare prion disease and are part of the same social group suggests that the toxic proteins were transferred from the infected meat. “Due to the difficulty of distinguishing sCJDMM1 from CWD without detailed characterization of the prion proteins, it is not possible to definitively exclude CWD in these cases,” explained Professor Trout and colleagues. The infection would have occurred in a way not dissimilar to that linked to prion diseases in other animals, such asbovine spongiform encephalopathy (BSE), the infamous mad cow disease. By consuming the infected parts – especially the nervous tissue – of cattle, the variant form of Creutzfeldt-Jakob disease known as vCJD. In the case under study we may be present at the first known case of variant triggered by deer meat with CWD.
Zombie deer disease, expert explains risks for humans and why it is difficult to fight prions
As explained to Fanpage.it by Dr. Fabio Moda, a prion expert from the Complex Structure of Neurology 5 and Neuropathology at the Besta Institute in Milan, in our brain – and in that of animals – there is naturally prion protein, however, “for reasons still not well understood, the prion protein can undergo a structural rearrangement and transforms into a toxic prion”, which is “able to interact with healthy prion proteins inducing a similar structural rearrangement”, thus propagating in central nervous system and peripheral organs. The prion has a catastrophic impact on the brainin which holes appear and looks like a sponge (hence the name spongiform encephalopathy for mad cow disease). Among the symptoms are behavioral alterationsvision problems, cognitive decline, muscle spasms and others, resulting in coma and death. There is no cure: the disease is fatal in 100 percent of cases.
As indicated, since the zombie deer disease began to spread significantly, scientists have highlighted the possible risks for humans, precisely because the meat of these animals is widely consumed in hunting communities. Furthermore, prions are very resistant in the environment and experts are evaluating the possible risks of contamination by sick deer. The disease is known as “zombie deer disease” because the infected specimens they drool significantly, they limpthey turn out disoriented and they have a lost look. The authors of the new study point out that the causal link between consumption of infected deer meat and CJD in the two hunters remains “unproven”, however the cluster “underscores the need for further investigation into the potential risks of consuming CWD-infected deer and on its implications for public health”. They therefore highlight the importance of in-depth research. Details of the study “Two Hunters from the Same Lodge Afflicted with Sporadic CJD: Is Chronic Wasting Disease to Blame? (P7-13.002)” were published in the specialized scientific journal Neurology.
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