4 ms·

If it's a prion disease, they sometimes take years or possibly decades to develop. The big question is how they were all infected. Is it in the food supply (me

If it's a prion disease, they sometimes take years or possibly decades to develop. The big question is how they were all infected. Is it in the food supply (meat, milk)? Was it tainted blood donations? 42 people is a lot of people... The vCJD outbreak in the 90s (mad cow) ended up with less than 250 cases total.

me_me_me 6y ago
Jeez don't even suggest that. If that shit jumped to humans we are fucked. I'd rather not imagine how that would look like in comparison to covid
- dwohnitmok 6y ago
  It already jumped to humans. Mad cow disease infects humans as well. The difference in infection levels is that mad cow disease is transmitted through ingestion, so the usual avenue for human-to-human transmission is cannibalism (see e.g. the outbreak of Kuru that resulted from ritual funerary cannibalism), which is rare. Animal-to-animal cannibalism in animal feed is more common.
  - subsubzero 6y ago
    I wonder if fully cooked meat can also transmit this disease? I know its big deal eating tainted meat as all hunters are encouraged to have the meat tested before consumption. there have been alot of deer that have been shown to have this disease.
    - AnimalMuppet 6y ago
      Fully cooked meat can also transmit prion-based diseases, because cooking isn't hot enough to destroy the prions. IIRC, you need 1500 degrees (Fahrenheit, I think). Your stove or grill isn't going to get you there, no matter how thoroughly you cook the meat.
      - seventytwo 6y ago
        Wouldn’t the deactivation temp depend on the prion?
        ruined 6y ago
        Sure, but most prion diseases are confirmed to be resultant from various misfolding of a single protein https://en.wikipedia.org/wiki/PRNP https://en.wikipedia.org/wiki/PRNP
        hinkley 6y ago
        Prions are why we use more disposable tools for surgeries instead autoclaving. Autoclaves aren’t hot enough to destroy the proteins.
  - meowface 6y ago
    Theoretically, could someone weaponize prions? Perhaps there'd be no point if the same tactics would be more effective with a typical deadly pathogen (ebola or something), but in theory could a malicious actor take human/animal brains confirmed to have a prion disease and then mash up and aerosolize the brain matter? Or would it not be possible to get infected through inhalation?
- Grazester 6y ago
  I have not eaten a piece of Beef since the 90's when there was that madcow scare. I was also very particular about trying to eat meat that was cooked on the same surface as beef. Now I am not as neurotic. I was also the kid that stayed awake at night worrying about Ebola when there was an outbreak in Zaire in 1995 I think it was.
  - yumraj 6y ago
    Genuinely curious, based on how you described yourself, if you’ll be getting the COVID-19 vaccine?
    - Grazester 6y ago
      I am not as paranoid an adult but CJD and Ebola scare the shit of me still. The survival rate of Covid-19 is higher than both Ebola and anything prion based so Covid-19 doesn't scare me as much. I will get the shot. Most of my family has already(medical profession). If my parents get Covid-19 they are as good as dead so I will be getting it for their sake also.
      - deleted 6y ago
        [deleted]
      - yspeak 6y ago
        BTW not good as dead. My 92 year old relative with fiabetes and heart disease (heart attack and bypass survivor) survived Covid and covid related pneumonia. It does happen. And now there's the vaccine so get them that.
  - hinkley 6y ago
    I stopped eating beef for a long time after that, then stayed away from ground beef for a lot longer. These days I eat gourmet burgers from time to time. The carbon footprint of beef is a disaster anyway. There are plenty of other reasons to stay away.
- betwixthewires 6y ago
  So, prions are like, a level of "life" below even viruses. They're not alive (and neither are viruses technically). They're essentially just misfolded proteins that when a cell attempts to break the protein apart and use it, it causes a problem in the enzymes that break the protein and forces them to replicate that protein. Mad cow disease was (is?) a prion disease. Since they're just proteins and not living creatures, even heating them doesn't kill them. They can't infect you like a virus or bacteria can, they basically all do the same thing, cause brain deterioration. They can't mutate to become more virulent, and they are spread through food consumption and usually caused by cannibalism. Whether a prion can damage cells in an animals brain depends on its DNA sequence and the enzymes their cells create to break apart certain proteins. We currently don't know if chronic wasting disease can hurt humans, and there are tons of prions all over the place all the time and you ingest them and most of them don't do anything bad to you. So there's no telling with CWD yet.
droopyEyelids 6y ago
Also, the prions themselves are not easily deactivated, and can linger in the environment, in an infectious state, for decades.
- mod 6y ago
  In my area we have a prion disease called CWD in the whitetail deer population. There's basically no way to destroy them in the wild. You have to bleach or burn the wilderness. They've had some suspicions that the prion has infected humans, although I haven't read any updates to that in a couple of years. Many locals still don't get their kills checked, even though it's free. They just eat them. A lot of the logic is that it's been here a long time and they've probably already eaten it. Anyway, prions are the scariest thing I've heard of in a long time, because they don't even have an agenda.
  - blaser-waffle 6y ago
    Wow didn't realize how big that was in the Rockies, like Alberta Canada or Colorado. Both places I've lived and hunted in...
michael1999 6y ago
We weren't cleanly segregating brain/spine tissue during meat processing in Canada until fairly recently. Given that CJD is only diagnosed by autopsy, I assume some fraction of dementia/Alzheimer's cases are actually prion diseases. And since we have no treatment, assigning liability is impossible, and it would destroy the beef industry, there's not much enthusiasm for learning more. The premier of Alberta was very clear: shoot, shovel, and shut up.
- robbiep 6y ago
  Whilst the amyloid plaques in Alzheimer’s could be considered a type of protein disorder with similarities to prion diseases in that a protein is aggregating in clusters that the body is unable to clear and break down (difference being, it isn’t infectious in origin and a amyloid beta protein isn’t being changed by a misfolded protein of its own type) I don’t think there is any evidence to support your assumption that some fraction of dementia or Alzheimer’s presumptive disgnostic patients (Alzheimer’s also being largely a diagnosis enabled by autopsy, although there are scans that can show the amyloid plaques) because the diseases have quite different symptoms and courses. Whilst that can’t fully rule out unknown prion diseases out there, generally we would expect such a condition to manifest in a much broader section of the population than just ‘the elderly’ and when young people develop significant neurological symptoms there’s a huge amount of diagnostic effort that is put in to determining what is going on, because, you know, it might be a prion disease and generally it shouldn’t happen unless there is a genetic determinant or another cause, such as smoking adulterated drugs which was found to cause Parkinson’s in the 80’s [0], or in fact vCJD [0] https://www.cdc.gov/mmwr/preview/mmwrhtml/00000360.htm https://www.cdc.gov/mmwr/preview/mmwrhtml/00000360.htm
  - michael1999 6y ago
    You're right that young people are investigated, and we don't see broad infection. I was thinking of causes for geriatric CJD, rather than vCJD. I'm skeptical of claims that geriatric declines are fully investigated. But the UK spike from contaminated food was very clear, and we don't see anything similar. Unless there's some low-level exposure the body can resist and a weakening blood-brain barrier/stroke/etc allows infection. But that's just speculation. Cheers.

me_me_me 6y ago

Jeez don't even suggest that. If that shit jumped to humans we are fucked. I'd rather not imagine how that would look like in comparison to covid

dwohnitmok 6y ago
It already jumped to humans. Mad cow disease infects humans as well. The difference in infection levels is that mad cow disease is transmitted through ingestion, so the usual avenue for human-to-human transmission is cannibalism (see e.g. the outbreak of Kuru that resulted from ritual funerary cannibalism), which is rare. Animal-to-animal cannibalism in animal feed is more common.
- subsubzero 6y ago
  I wonder if fully cooked meat can also transmit this disease? I know its big deal eating tainted meat as all hunters are encouraged to have the meat tested before consumption. there have been alot of deer that have been shown to have this disease.
  - AnimalMuppet 6y ago
    Fully cooked meat can also transmit prion-based diseases, because cooking isn't hot enough to destroy the prions. IIRC, you need 1500 degrees (Fahrenheit, I think). Your stove or grill isn't going to get you there, no matter how thoroughly you cook the meat.
    - seventytwo 6y ago
      Wouldn’t the deactivation temp depend on the prion?
      - ruined 6y ago
        Sure, but most prion diseases are confirmed to be resultant from various misfolding of a single protein https://en.wikipedia.org/wiki/PRNP https://en.wikipedia.org/wiki/PRNP
      - hinkley 6y ago
        Prions are why we use more disposable tools for surgeries instead autoclaving. Autoclaves aren’t hot enough to destroy the proteins.
- meowface 6y ago
  Theoretically, could someone weaponize prions? Perhaps there'd be no point if the same tactics would be more effective with a typical deadly pathogen (ebola or something), but in theory could a malicious actor take human/animal brains confirmed to have a prion disease and then mash up and aerosolize the brain matter? Or would it not be possible to get infected through inhalation?
Grazester 6y ago
I have not eaten a piece of Beef since the 90's when there was that madcow scare. I was also very particular about trying to eat meat that was cooked on the same surface as beef. Now I am not as neurotic. I was also the kid that stayed awake at night worrying about Ebola when there was an outbreak in Zaire in 1995 I think it was.
- yumraj 6y ago
  Genuinely curious, based on how you described yourself, if you’ll be getting the COVID-19 vaccine?
  - Grazester 6y ago
    I am not as paranoid an adult but CJD and Ebola scare the shit of me still. The survival rate of Covid-19 is higher than both Ebola and anything prion based so Covid-19 doesn't scare me as much. I will get the shot. Most of my family has already(medical profession). If my parents get Covid-19 they are as good as dead so I will be getting it for their sake also.
    - deleted 6y ago
      [deleted]
    - yspeak 6y ago
      BTW not good as dead. My 92 year old relative with fiabetes and heart disease (heart attack and bypass survivor) survived Covid and covid related pneumonia. It does happen. And now there's the vaccine so get them that.
- hinkley 6y ago
  I stopped eating beef for a long time after that, then stayed away from ground beef for a lot longer. These days I eat gourmet burgers from time to time. The carbon footprint of beef is a disaster anyway. There are plenty of other reasons to stay away.
betwixthewires 6y ago
So, prions are like, a level of "life" below even viruses. They're not alive (and neither are viruses technically). They're essentially just misfolded proteins that when a cell attempts to break the protein apart and use it, it causes a problem in the enzymes that break the protein and forces them to replicate that protein. Mad cow disease was (is?) a prion disease. Since they're just proteins and not living creatures, even heating them doesn't kill them. They can't infect you like a virus or bacteria can, they basically all do the same thing, cause brain deterioration. They can't mutate to become more virulent, and they are spread through food consumption and usually caused by cannibalism. Whether a prion can damage cells in an animals brain depends on its DNA sequence and the enzymes their cells create to break apart certain proteins. We currently don't know if chronic wasting disease can hurt humans, and there are tons of prions all over the place all the time and you ingest them and most of them don't do anything bad to you. So there's no telling with CWD yet.

droopyEyelids 6y ago

Also, the prions themselves are not easily deactivated, and can linger in the environment, in an infectious state, for decades.

mod 6y ago
In my area we have a prion disease called CWD in the whitetail deer population. There's basically no way to destroy them in the wild. You have to bleach or burn the wilderness. They've had some suspicions that the prion has infected humans, although I haven't read any updates to that in a couple of years. Many locals still don't get their kills checked, even though it's free. They just eat them. A lot of the logic is that it's been here a long time and they've probably already eaten it. Anyway, prions are the scariest thing I've heard of in a long time, because they don't even have an agenda.
- blaser-waffle 6y ago
  Wow didn't realize how big that was in the Rockies, like Alberta Canada or Colorado. Both places I've lived and hunted in...

michael1999 6y ago

We weren't cleanly segregating brain/spine tissue during meat processing in Canada until fairly recently. Given that CJD is only diagnosed by autopsy, I assume some fraction of dementia/Alzheimer's cases are actually prion diseases. And since we have no treatment, assigning liability is impossible, and it would destroy the beef industry, there's not much enthusiasm for learning more. The premier of Alberta was very clear: shoot, shovel, and shut up.

robbiep 6y ago
Whilst the amyloid plaques in Alzheimer’s could be considered a type of protein disorder with similarities to prion diseases in that a protein is aggregating in clusters that the body is unable to clear and break down (difference being, it isn’t infectious in origin and a amyloid beta protein isn’t being changed by a misfolded protein of its own type) I don’t think there is any evidence to support your assumption that some fraction of dementia or Alzheimer’s presumptive disgnostic patients (Alzheimer’s also being largely a diagnosis enabled by autopsy, although there are scans that can show the amyloid plaques) because the diseases have quite different symptoms and courses. Whilst that can’t fully rule out unknown prion diseases out there, generally we would expect such a condition to manifest in a much broader section of the population than just ‘the elderly’ and when young people develop significant neurological symptoms there’s a huge amount of diagnostic effort that is put in to determining what is going on, because, you know, it might be a prion disease and generally it shouldn’t happen unless there is a genetic determinant or another cause, such as smoking adulterated drugs which was found to cause Parkinson’s in the 80’s [0], or in fact vCJD [0] https://www.cdc.gov/mmwr/preview/mmwrhtml/00000360.htm https://www.cdc.gov/mmwr/preview/mmwrhtml/00000360.htm
- michael1999 6y ago
  You're right that young people are investigated, and we don't see broad infection. I was thinking of causes for geriatric CJD, rather than vCJD. I'm skeptical of claims that geriatric declines are fully investigated. But the UK spike from contaminated food was very clear, and we don't see anything similar. Unless there's some low-level exposure the body can resist and a weakening blood-brain barrier/stroke/etc allows infection. But that's just speculation. Cheers.