Science Man eats squirrel brains, dies of bizarre disease - spooky month delivers again

https://www.popularmechanics.com/sc...utzfeldt-jakob-united-states-squirrel-brains/

According to a recently-uncovered medical case from 2015, one man may have died after eating one too many squirrels. His penchant for squirrel meat seems to have inadvertently exposed him to an incredibly rare fatal brain disorder, the first time this disorder was ever contracted in the United States.

Creutzfeldt–Jakob disease is a rare brain disorder caused by misfolding brain proteins called prions. Prion proteins are unique in that when one is misfolded, it causes other prion proteins to misfold too. These misfolded proteins are unable to function properly, and the result is that the victim suffers from memory loss, dementia, psychosis, loss of coordination, and eventually death.

The key here is that because these misfolded prions can mess up other prions, the disease is highly contagious. If prions from an infected person get into your system, you can contract Creutzfeldt–Jakob disease too. Fortunately, however, contracting CJD isn’t as easy as contracting the flu. Prions don’t spread through mucus or saliva, so you can’t get the disease from being sneezed on or whatever.

Instead, the most common way to get CJD from another person is to eat them.

For obvious reasons, CJD isn’t a very common disease, but it does crop up sometimes. One remote tribe, the Fore people of Papua New Guinea, experienced an epidemic of the diseasein the 1950s by practicing a form of ritualistic cannibalism. An even rarer version of CJD, called ‘variant CJD,’ plagued the United Kingdom in the 80s and 90s after people began inadvertently eating beef from cows infected with the bovine strain, otherwise known as mad cow disease.

This variant CJD is so rare that only four individuals in the U.S. have been confirmed to have contracted it, and all of them acquired it while overseas. But a group of researchers looking through old medical records may have found a fifth case of vCJD that originated right here in the United States.

In 2015, a Rochester man checked into a hospital with severe memory loss, declining mental faculties, and muscle problems: all the symptoms of vCJD. A brain scan reinforced researchers’ suspicions, as the MRI looked similar to other victims of vCJD. But one question for the doctors remained: Just how did this man contract vCJD in the first place?

The answer appears to lie in his hunting habit. The man enjoyed hunting and eating animals, including squirrels, and according to the report has eaten squirrel brains at least once. It wasn’t clear from the report if the man inadvertently consumed some squirrel brain along with the rest of the squirrel or if he deliberately sought out squirrels for their delicious brain meat.

Either way, he must have eaten the brain of at least one squirrel with a prion disease, which he then contracted. Of course, nobody ate that man’s brain, so there’s no risk of it spreading. But this does mean that hunters—and squirrel connoisseurs—might be more at risk for CJD than doctors and scientists believed. That could be bad news for organizations like the Red Cross, which filter out people at risk of CJD in their blood donation program. If people who eat hunted meat also present a risk to patients receiving donated blood, it could mean a lot of Americans are ineligible.

The good news is that you can stay safe but just not eating any brains.


This is why I just stick to chipmunk brains.
 
How did the squirrels get the disease? They eat nuts and seeds.
I'm not exactly sure how prions form initially, but I think that is from an error in the transcription of RNA into the protein chain or an error in how the protein is folded into it's final form. The squirrel might have had a bad roll of the dice and ended up with one misfolded protein with the capability to fuck up it's sibling proteins into more prions, leading to the disease and a high prion count for whatever wants to eat its brain.

Now that i'm thinking about it how the hell does a prion get from the digestive tract to the brain? Wouldn't it have to survive digestion and then make it past the blood-brain barrier?
 
The squirrel might have had a bad roll of the dice and ended up with one misfolded protein with the capability to fuck up it's sibling proteins into more prions, leading to the disease and a high prion count for whatever wants to eat its brain.
Ninja'd you, but yeah bingo.
Now that i'm thinking about it how the hell does a prion get from the digestive tract to the brain? Wouldn't it have to survive digestion and then make it past the blood-brain barrier?
Yep, it does. The misfolded form is so stable that to denature it you would have to basically reduce your food into a mush or nucleic acids. Hence cooking does nothing to prevent you from getting infected.
 
This is why in the country we were told to not eat brains. I never ate them, but I know some people did in the past. A game warden came around to warn everyone. When it got time to dispose of waste the brains were burned. It was just too much of a risk.

When you are hungry enough, squirrel is actually pretty good
 
The prion protein is weird. Yes, it's THE prion protein. As in there's only one in humans. And in mammals in general, in fact. It's produced by a highly conserved gene that has no known function, though the protein is being expressed in you right now. It just has a tendency to misfold. This one protein can do this in quite a wide variety of ways. As has been brought up upthread, it's also almost unnaturally resistant to heat and ph. Very difficult to denature compared to other proteins. Why something seemingly so maladapative has stuck with mammals for many tens of millions of years is beyond anyone. It's almost like God just hates us and wants us to suffer.
 
Squirrels are parasyte and disease ridden little pests.
Why would you even eat their brain to begin with.

They taste pretty yummy. I know Sam Jackson wouldn’t eat sewer rat if it tasted like pumpkin pie, but a “wild harvested” squirrel isn’t much different from any other game. Deer, duck, boar, whatever.

It’s kind of a joke nowadays, though, but there’s still some weirdos who go through the effort to hunt squirrel in the more backwoods rural areas.
 
I think that original "mad cow" outbreak that hit the UK a few years ago was traced to contaminated cattle feed, sounds like someone isn't abiding by the "no dead cattle as filler in the cattle feed" rule, perhaps?
I believe it was also that they used sheep in the feed - there’s a sheep equivalent of the disease called scrapie.
This is why in the country we were told to not eat brains. I never ate them, but I know some people did in the past. A game warden came around to warn everyone. When it got time to dispose of waste the brains were burned. It was just too much of a risk.

When you are hungry enough, squirrel is actually pretty good
It used to be pretty standard poor people food in rural areas- I know young Elvis used to eat it before he got rich and switched to a diet consisting of everything.

Ironically, during the last BSE crisis in the UK, there was a fad for unusual meats, and squirrels were one of the animals that briefly became popular.
 
Do the squirrels also get dementia? Those little guys are wacky enough as is.
Theoretically yes they could.
Realistically, probably the prions don't accumulate to a level significant enough to cause any damage, but take that with a grain of salt. I much prefer to imagine shaking squirrels running around laughing maniacally.
 
I'm not exactly sure how prions form initially, but I think that is from an error in the transcription of RNA into the protein chain or an error in how the protein is folded into it's final form. The squirrel might have had a bad roll of the dice and ended up with one misfolded protein with the capability to fuck up it's sibling proteins into more prions, leading to the disease and a high prion count for whatever wants to eat its brain.

Now that i'm thinking about it how the hell does a prion get from the digestive tract to the brain? Wouldn't it have to survive digestion and then make it past the blood-brain barrier?

Ninja'd you, but yeah bingo.

Yep, it does. The misfolded form is so stable that to denature it you would have to basically reduce your food into a mush or nucleic acids. Hence cooking does nothing to prevent you from getting infected.

There was a quiet and quickly hushed up panic regarding this in the Medical Comunity of Neurosurgeons a couple of years ago. It was discovered that prions are so stable and hard to denature, that they could and often would survive modern cleaning and sterilization techniques of the surgical instruments used in brain surgery. So it was highly possible to cross contaminate brain surgery patients.
 
How did the squirrels get the disease? They eat nuts and seeds.

Given how much ranchers bitch about wolves, maybe they should consider the alternative.
squirrels don't just eat nuts and seeds.
From Wikipedia:
Some squirrels, however, also consume meat, especially when faced with hunger. Squirrels have been known to eat small birds, young snakes, and smaller rodents, as well as bird eggs and insects. Indeed, some tropical squirrel species have shifted almost entirely to a diet of insects.
 
I understand that was common practice up to that point. It wasn't until the Mad Cow plague that they realized that maybe they shouldn't recycle diseased corpses.
They still do it in the USA, you're only allowed to do it if you feed your animals a different species of offal. Pigs and chickens are still eating diseased cow. Cows are still eating diseased chicken and pigs. It's a stupid system.
 
I read a book about prion diseases back then called "The Family that Couldn't Sleep" by TD Max and he goes into the whole history of the discovery and research of prions and prion diseases. It's really good and I'd recommend it.

The book featured a very horrifying genetic form of a prion disease called Fatal Familial Insomnia in which the sufferer eventually loses the ability to sleep and eventually dies. This form of prion disease is so torturous and insidious in that it only affects the thalamus leaving the higher cortex and thus consciousness, intact whereas the other prion diseases affect the whole brain. So basically you are sleep deprived for a few months, then you lose the ability to walk, talk or even eat but the thing is, you are entirely cognizant the entire time until your expiration.
 
Atrás
Top Abajo