Possible Link Between Chronic Wasting Disease and Human Prion Disease: Urgent Research Needed

Possible Link Between Chronic Wasting Disease and Human Prion Disease: Urgent Research Needed

2024-05-13 16:21:37

/image alliance, Duluth News Tribune, Steve Kuchera

San Antonio Two people in the United States may have been infected with Chronic Wasting Disease (CWD), a condition that normally occurs in deer. This is suggested by a recently published study of the cases that occurred in 2022 (Neurology 2024; DO I: 10.1212/WNL.0000000000204407).

CWD is a prion disease that occurs in deer, reindeer and elk. Like bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob disease, CWD is one of the transmissible spongiform encephalopathies. Early symptoms include apathy, confusion, and motor problems. In the late stages, the sick animals lose weight. So far, there is no known transmission to humans.

Researchers at the University of Texas Health San Antonio are now reporting two possible cases of transmission. In 2022, a 72-year-old man ate meat from a CWD-infected deer population and subsequently developed confusion and aggression.

His friend, who had also eaten wild meat from the same deer population, had recently died of Creutzfeldt-Jakob disease, raising concerns regarding a possible link between CWD and human prion disease, the research team wrote.

Although the diagnosis of Creutzfeldt-Jakob disease was confirmed postmortem based on homozygous methionine in codon 129 (sCJDMM1), CWD cannot be ruled out due to the similarity between the prion proteins, the researchers explain. They recommend further research into the potential risk of transmission when consuming infected meat.

As far as we know, there is no scientific evidence that the two men actually contracted CWD and died from it, according to the Friedrich Lffler Institute (FLY) on request. Although transmission cannot be ruled out with complete certainty, the barrier to transmission from animals to humans appears to be very high.

This is also the result of a review published in 2023 (MDPI 2023; DO I: 10.3390/foods12040824). Most experimental data suggest that the zoonotic risk of CWD is very low, it said. However, knowledge regarding CWD remains incomplete (eg, regarding its origin, transmission characteristics, and ecology).

The Norwegian authors Michael A. Tranulis and Morten Tryland therefore recommend precautions to minimize human exposure. Veterinarian Tranulis considers the current case to be very interesting and also explains that there is a need for further investigations. Human and animal exposure to this pathogen should be reduced to an absolute minimum, Tranulis said German Medical Journal.

The first CWD cases in Europe occurred in Norway in 2016, first in reindeer and later also in deer and elk. Tranulis has previously conducted research on prion diseases and has contributed to scientific opinions and assessments for Norwegian food safety authorities since CWD first appeared. There are currently no known cases in Germany. In the United States, CWD appeared in the United States in the 1960s and has now spread there. mim/aerzteblatt.de

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