The love of a man vis-à-vis squirrel meat might have given him a terrible deadly brain disease

A resident of Rochester, New York, might have caught one of the scariest brain diseases in one of the strangest ways possible: eating squirrel contaminated with meat. proteins that look like zombies.

The bizarre story of this man was featured in a preliminary report released earlier this month at IDWeek, the annual conference of the Infectious Diseases Society of America. His case was one of many cases detailed by doctors at Rochester Regional Health, an important network of city hospitals.

According to the report, the 61-year-old man had been admitted to a hospital in the Rochester Regional Area for cognitive impairment, schizophrenia, and psychosis in 2015; he was also unable to walk under his own power. But there was nothing to do. Five months after the onset of his symptoms, he died. Judging by his brain scans and other tests, the man was probably suffering from variant Creutzfeldt-Jakob Disease (vCJD), a rare and universally fatal form of neurodegenerative disease.

CJD is one of many diseases caused by a kind of protein called a prion. Prions naturally exist in the brain and seem harmless to us. But when these proteins begin to fold as they should not, they also force other neighboring prions to retreat, creating a growing army that can effectively annihilate the brain over the years or decades.

Prion diseases can occur spontaneously or be transmitted by families. But they can also be infectious and some, including CJD, are even able to spread between different animal species. These diseases are also known as transmissible spongiform encephalopathies, a reference to the hollowed-out appearance of the brain, looking like a sponge, in a person who has died.

VCJD was first identified in 1996, when relatively young British residents began to die unexpectedly from CJD. These victims had unfortunately eaten beef infected with prions from cows with their own form of prion disease, bovine spongiform encephalopathy (or what most people probably know, mad cow disease). And the cows had probably caught him eating animal feed infected with brain material from other cows, as well as another animal vulnerable to prions, the sheep.

There have been only about 200 documented cases of vCJD in the world, the majority occurring in the UK. Mad cow disease and its human variant have never entered the United States, largely because of food security practices that have always prevented farmers from feeding their cows with food made from most other animals (the United Kingdom did the same in 1989 while slaughtering thousands of cows as a precaution). Not all consumers of infected meat seem to contract vCJD, which probably means that our genetics also play a role.

But by 1997, American scientists feared that regional dishes such as squirrels and other hunted animals would spread prion disease. And in the case of the man, said Live Science, the family told his doctors that he was a passionate hunter and connoisseur of squirrel meat.

The report's authors, led by Tara Chen, a resident physician, discovered the case, having not treated the man himself. They began searching the hospital records after seeing four suspected cases of CJD develop within six months last winter – an unusually high number for a rare disease that affects about 350 people in the whole country every year. Of the five cases detailed in their report, however, two were finally confirmed as not ultimately being a CJD.

Although current tests distinguish vCJD from the classic form of the disease, Chen told Live Science that it was not known if the man was definitively diagnosed with CJD. With many deadly brain diseases, such as Alzheimer's disease, doctors can only be certain of the diagnosis by examining the brain after death. If it is confirmed that the man was suffering from v-CJD, he could very well be the only person to have ever contracted it in the United States. Four other officially recognized cases of v-CJD in the United States have been reported. before entering the country.

According to Live Science, Chen and his team are currently seeking human autopsy records. By publishing their preliminary case report, they also hope to sensitize physicians to CJD, pointing out that it took an average of two weeks to diagnose or rule out the disease in their cases. And this delay could increase the risk of cross-infection in hospitals, thanks to contaminated medical equipment that is not properly sterilized.

Adding to their zombie nature, prions can survive things that easily kill other pathogens, such as radiation and chemicals such as formaldehyde.