Two deer hunters from the same lodge both developed fatal sporadic CJD (Creutzfeldt-Jakob Disease) after exposure to CWD-infected deer. Sporadic CJD normally affects only 1-2 people per million annually. This is the first documented case linking humans to potential CWD transmission.

[Two deer hunters ate CWD-Infected venison. Both later died of a “One-in-a-Million” brain disease.](https://www.neurology.org/doi/10.1212/WNL.0000000000204407)

In 2024, doctors reported two hunters who hunted together at the same lodge both got a super-rare brain disease called sporadic Creutzfeldt-Jakob Disease (CJD), the human version of mad cow disease, after eating deer from areas riddled with Chronic Wasting Disease (CWD).

**What are prion diseases?** Think of prions as zombie proteins. Normal proteins in your brain fold one way to work properly. Prions are the same protein but folded wrong, they act like a virus that “infects” good proteins, turning them bad too. The bad proteins clump up, destroy brain cells, and turn your brain into Swiss cheese. No symptoms for years/decades, then sudden dementia, shaking, and death in months.

**What happened to these hunters?** Both developed sporadic Creutzfeldt-Jakob Disease (CJD), a prion disease that normally affects only 1-2 people per million each year. Two hunters from the same small group getting it is statistically practically impossible, unless linked. They had both consumed venison from areas where Chronic Wasting Disease (CWD), the deer version of mad cow disease, is endemic.

What makes this particularly concerning is that Chronic Wasting Disease now affects deer populations across North America, and the prions causing it remain infectious in soil for decades. For years, scientists believed a strong species barrier protected humans from CWD, but a 2022 study using humanized mice demonstrated that CWD can cross to human tissue, contradicting earlier assumptions. Even more alarming, research suggests CWD could present differently in humans than typical prion diseases, meaning cases might be misdiagnosed with current screening methods.

**All prion diseases are 100% fatal and incurable.** The incubation period could be decades, so if this cluster represents actual transmission, we won’t know the true scope for years

as someone that loves venison this is scary af


Great!

The victims of the prion meat post?

Just to add to the fun – some prions aren’t destroyed by cooking or other conventional food preservation processes. Even if your “at risk” meat is “well done”, dried, smoked or cured doesn’t mean it is safe.

How do you know if a deer is infected? Does it act weird, does the meat look different?

This brings back bad British memories.

So transmission not limited only to brain and spinal tissue consumption?

I believe that’s what happened to a cousin of mine. He was a lifelong hunter and fisherman for his hobbies. He especially enjoyed deer hunting.

He was the much-loved son of an uncle and his family didn’t want to say too much about it. But it was a neural degenerative disease specifically tied to deer and infected meat. His doctor has described it as the negative lottery.

From the little I heard, he exhibited severe short term memory loss. Sounded like a rough way to go but he was resigned to it.

I think there was a possible case in Idaho not long back but they could not confirm or something.  Where is this lodge?

So cwd is cjd for deers? Prions?

The X Files has an episode where a community of cannibals get CJD from eating one another. It absolutely terrified me, and made me scared of prion diseases.

I am curious if this effects the Amish community at all. We have a ton of deer where I am and they pretty much live off the meat over anything else.

Does hydrogen peroxide or iodine kill prions??