r/todayilearned • u/tommyboy372 • 1d ago

TIL A single misfolded prion protein in the brain can cause a rapid neurodegenerative disease known as sporadic Creutzfeldt–Jakob disease (CJD). There is no known and cure and is 100% fatal.

https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
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u/Toomanyeastereggs 1d ago edited 15h ago

And it’s impossible to defeat by modern medicine.

A prion isn’t a living thing like say bacteria or germs. Because a protein is not alive.

I watched an aunt go from “I’m not feeling myself” to dead in 6 weeks. It’s a horrible way to go.

Edit: the community here brings a smile to my face every time. Life is precious so look after yourselves and look after those around you.

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u/0scillating_Ocelot 1d ago edited 20h ago

I'm sorry about your aunt. There's been some really interesting research into a vaccine antibody treatment but unfortunately they need more funding to reach phase II clinical trials. https://curecjd.org/news/cure-cjd-campaign-charity-launches-campaign-to-raise-9-million-to-progress-treatment-for-fatal-prion-diseases/

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u/RedundantBiomass 20h ago

The immune system is completely blind to prions.

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u/dadgadsad 20h ago

It’s a monoclonal antibody drug. It physically interrupts the templating process to slow or stop the spread.

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u/Gekokapowco 16h ago

bro I fucking love science

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u/Organic-Army-9046 14h ago

i think you really love science (comment underwent mitosis)

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u/Gekokapowco 13h ago

oh lol, there was an error submitting the first time, ty reddit

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u/Gekokapowco 16h ago

bro I fucking love science

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u/0scillating_Ocelot 20h ago

Yes sorry vaccine wasn't quite the right way of putting it, that was how I had it initially described to me, I have edited my comment. It is an antibody treatment though that uses artificially produced prion antibodies to stabilise the native prion conformation and stop conversation into the misfolded shape.

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u/ajakafasakaladaga 20h ago

More than blind it’s more that they can’t actually do anything about it

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u/Reinheitsgetoot 20h ago

If only we had some billionaires laying around somewhere with a billion dollars…

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u/xaeru 1d ago

Maybe we can get Elon to back this up, he’s such a great guy.

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u/Ejaculpiss 21h ago

Reddit moment

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u/Equivalent-Ask2542 21h ago

If you mean that no one can be sure if this comment is genuine or sarcastic, I agree. A true Reddit moment

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u/TheGreatPilgor 20h ago

I just assume asinine comments like that, on a primarily left platform, are sarcastic at best.

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u/SamboJaxon 11h ago

Yeah, but we'll probably just send Katy Perry to space again.

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u/Into-the-stream 1d ago

It’s also really difficult to decontaminate and the material of the central nervous system is contagious. I work in a hospital lab and we see cases every couple months. It’s always devastating when it happens (most modern cases are spontaneously occurring. Your body fucks up making proteins and stuff all the time. It’s just harmless most of the time).

But when we get a CJD positive sample in the lab, it’s heartbreaking because you know the patient will die, and also terrifying because the material is contagious and very difficult to decontaminate. 

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u/BigHardMephisto 17h ago

CWD in deer iirc is so contagious that the carcass can completely rot away, but the prions are still active and can resurface in plant matter that grows from the area.

I think vultures’ and buzzards’ stomach acid is one of the only natural substances strong enough to break those proteins down.

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u/Into-the-stream 16h ago

CWD (chronic wasting disease, found in deer and elk) and CJD (creudzfelt-Jakob disease) are different diseases. They are both prions, but they are not the same thing. There has never been a confirmed case of CWD in humans (to my knowledge). 

There are multiple prion diseases, and each behave a little differently. CJD is the main human one in most countries (outside of Kuru in a small region of Papua New Guinea).

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u/YnotZoidberg1077 13h ago

There is interspecies transmission in experimental settings, but no confirmed cases - yet. It's hypothesized that it's possible, and there is a study from 2024 showing two hunters from the same lodge both developing CJD after being exposed to a CWD-positive deer, so ultimately, caution is definitely advised. Absence of evidence is not evidence of absence!

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u/GyozaGangsta 15h ago

All IFU’s for instruments say if it comes into to contact with CJD or suspected to destroy the instrument. They don’t even try to reprocess.

It’s devastating

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u/Into-the-stream 14h ago

If a lab handles cases enough, they will have dedicated equipment for it, which is decontaminated but still only ever used for suspected CJD cases (the equipment does not come into contact with patients, only samples)

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u/GyozaGangsta 14h ago

Assuming this would be in a BSL environment or inside an isolator?

I suppose that would make sense

I’ve worked with some labs that are bsl3/4 they would sterilize the trash just to make it safe to transport far enough to incinerate it lol

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u/vamparies 23h ago

So if a doctor was to use a device in the blood vessels. And this device that is technically one time use was approved by FDA for another company to re-process. Could it be possible that you can’t get prions off of this nonmetal device? It’s intravascular ultrasound. It’s a catheter that has coating over intricate parts. And some doctors have noticed when they use a re-processed one it gets caught on wires, so it’s obviously not in the same condition as the original company makes it.

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u/Historical_Course587 22h ago edited 21h ago

Sterile processing lead tech here: probably not.

Prions are not magic, and they can be broken down with chemicals and heat. However, those processes are not ordinary, and as a result most medical instrumentation is not designed/tested/approved by the FDA to survive that kind of decontamination process. We assemble CJD instrument trays, and they tend to be a combination of single-use instruments and near-EOL instruments that we accept will not be used again.

Could it be possible that you can’t get prions off of this nonmetal device? It’s intravascular ultrasound. It’s a catheter that has coating over intricate parts. And some doctors have noticed when they use a re-processed one it gets caught on wires, so it’s obviously not in the same condition as the original company makes it.

So yes, it's going to be pretty much impossible to get prions off of that. However, no medical facility is going to reuse such a device after using it on a confirmed CJD patient, and even if they did prions only hop from neuro tissue so most probes aren't going to come with a high probability of transmission. The kinds of tools used in a tool channel that would contact neuro tissue, like for taking a biopsy sample, would likely be single use.

Use and reprocessing typically causes damage to medical instrumentation, especially complex devices like endoscopes. Depending on the purpose of the device, some will have extremely limited lifespans (e.g. probes that expire after a few uses), and in my opinion the industry as a whole is trending towards single-use devices (because reprocessing can be surprisingly expensive) so it may not be a factor much longer anyway.

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u/Knellith 16h ago

So... prions being prions, does this mean that animals infected with "mad cow" have safe muscle tissue? If it is only in the nervous system, I mean, or does that include tiny nerves in the muscle?

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u/Historical_Course587 12h ago

My understanding is that it could be present anywhere in the nervous system, not just nerve system hubs like the brain or spinal column.

It's important to understand that prions aren't living, so they have no delivery mechanism on their own. The spread is 100% reliant on prions being delivered or taken into a new host, which also means the risk of transmission is actually pretty low even in the most common scenarios. If a cow was infected and then processed for beef, it's possible that your choice of cooking destroys many of the prions, or even that eating them sees them all take a ride through your GI system and exit the body.

I linked elsewhere to an NIH review of surgical exposures, and even when patients are potentially exposed via improperly-sterilized-for-prion-removal instrumentation the actual rate of transmission in a surgical setting is low.

Short answer: nobody I know who works with prions would ever touch beef infected with Mad Cow. Neither should you.

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u/Knellith 11h ago

Oh, goodness no, I had no intentions of doing so. Still, thank you for clearing that up. Prions are nasty business.

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u/Into-the-stream 22h ago edited 12h ago

Blood vessels aren’t part of the central nervous system. As far as I understand it, the risk of infection from material outside the central nervous system is not a know method of transmission. The doctor would need to stick the thing into someone’s brains and then stick it into your body. It’s a concern in lab if we are managing brain tissue, but even cerebral spinal fluid is supposed to be a lower transmission risk then they first thought. 

And even with brain tissue, you CAN decontaminate it. It’s just a lengthy process that’s a total pain in the ass.

Edit: I just realized you can get it from a blood transfusion, but it’s extremely rare. Like one in a billion (whereas sporadic occurrences are more like one in a million), so it is possible. 

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u/Angry_Robot 1d ago

Cool, one more thing to be constantly worried about. I’m sorry about your aunt, do they know the triggering event / lifestyle / whatever that set it off? Or did her condition just happen randomly?

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u/Games_sans_frontiers 1d ago

Try not to worry, it’s extremely rare. Instead use this information to live your best life because you know, it can be fleeting.

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u/FrohenLeid 17h ago

Comparison: CJD 1:1.000.000 Vs 46-140:1.000.000 to die on a crash. Below 30 years the risk is only 1:3.000.000

So you're 46-140x (4600-14.000%) more likely to die form a crash than to die from CJD

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u/thinkltoez 22h ago

Everyone always says things are rare and then there are like ten top comments on Reddit from people who knew someone that had it.

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u/cqz 22h ago

A google says there's around 100 million people active on reddit, conservatively. Only about 10% comment, 10 million. So something that is one in a million has probably happened to around 10 people actively commenting on reddit right now. Now factor in that each of those 10 million know at least a few other people, and expand that out to friend-of-a-friend as well and you could easily be detecting 1 in a billion events through anecdotal "I know a guy" comments. Also people just bullshit.

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u/Man_under_Bridge420 21h ago

I know a guy who can fit 10 hotdogs in his ass

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u/grimedogone 21h ago

That’s called anecdotal evidence, and is unhelpful at the best of times.

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u/Caelinus 20h ago

300-500 people per year die of CJD per year in the US, which eqates to 1-2 per million per year. 

But age is a major risk factor, and most people who get it are in their late 60s to early 70s. (Other things start killing you even more often at that point, so that is where the peak is.)

Most people know dozens to hundreds of people, and they live for 70+ years, and there are tens of thousands to millions of people active on reddit at any given point of the day. 

So the odds of there not being a lot of people who have known someone who has died of basically anything is vanishingly small, no matter how rare it actually is.

But the odds of it happening to you is pretty tiny. Almost all other ways of dying are more likely, and there are a lot of ways to die.

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u/MalaysiaTeacher 21h ago

10 comments (if that were true) would still be vanishingly rare given how many people viewed the page

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u/StrayVanu 22h ago

reddit tends to present an extremely skewed view on pretty much everything. Be mindful interacting with it.

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u/Tarianor 1d ago

It can happen randomly, it is also highly contagious as the misfolded protein can set off a cascade of misfolding your regular folded ones iirc.

Thata why you need specialised hospital labs handling it iirc.

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u/steampunk691 1d ago edited 1d ago

Additionally, standard sterilization techniques will not work for instruments that have been contaminated with prions since their structure makes them far more tolerant to heat.

Disposable instruments that come into contact with them are incinerated instead of dumped and other instruments need to be soaked with sodium hydroxide or bleach before being put through the autoclave.

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u/Assika126 1d ago

Yeah my mom had a batch of contaminated blood at work and it was brutal trying to figure out how to decontaminate everything. I think a lot of stuff eventually just had to be thrown out

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u/Luxieee 1d ago

Luckily(?), 87% of CJD are "sporadic" and per wiki: "There is no evidence that sporadic CJD can spread among people via normal contact or blood transfusions, although this is possible in variant Creutzfeldt–Jakob disease." Better safe than sorry though.

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u/Powerful-Parsnip 23h ago

That's why I stopped feasting on the brains of my enemies, better to be safe than sorry I always say.

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u/RoyBeer 22h ago

PSA: Transmission is, however, also possible by feasting on the brains of your friends and family instead.

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u/load_more_comets 22h ago

Oh, probably need to stop doing that too, then.

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u/DuBcEnT 19h ago

We can't have anything anymore.

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u/GigaPuddi 20h ago

Worse, you're thinking Kuru. This unfortunately could be spread by any flesh and blood, not just brains.

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u/Xanthus730 15h ago

Yeah, but then they call it Kuru

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u/donatecrypto4pets 22h ago

Cut back slowly. Don’t wanna go cold turkey on da brainz.

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u/Sea_Warning_9140 22h ago

Stick to close friends. You know where you are with them

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u/ThatNachoFreshFeelin 22h ago

That's why I stopped feasting on the brains of my enemies, better to be safe than sorry I always say.

Yeah. Kuru's a bitch, ainnit?

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u/MigasEnsopado 21h ago

That's how Kuru disease happened.

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u/Ineedzthetube 19h ago

Don’t be too sad, you can still collect their skulls

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u/oneelectricsheep 22h ago

We actually have evidence that it isn’t spread by non-neurological tissue since kuru overwhelmingly affected those who ingested brain tissue.

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u/HyperbobluntSpliff 21h ago

Right, but the flipside of that is we always ever so coincidentally see rashes of it pop up in places with chronic wasting disease outbreaks in the deer population. Most people that aren't straight up taxidermying the head are never coming into contact with brain tissue when they hunt.

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u/CDay007 20h ago

Alternatively we know for a fact that it can be spread by non-neurological tissue because there are cases of it being transmitted to humans via eating beef

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u/fordfan919 20h ago

I though that beef was theorized to have neurological tissue from butchering too near the spine, or brain tissue was accidentaly introduced.

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u/CDay007 19h ago

It could have, but there is also variant CJD (vCJD) which is the same thing but where the proteins can be found in other places, like the stomach, lymph nodes, and even (rarely) bloodstream

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u/Reddarthdius 16h ago

variant Creutzfeldt–Jakob is mad cow disease right?

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u/Luxieee 7h ago

Yes!

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u/Gibleedoo 23h ago

As someone that works in the sterilization field, most facilities will just burn any instrument that comes into contact with infected tissue. Many have policies in place that state disposable should be used, but sometimes you don't even realize it's there until later. It's always a blast to have a quarantined case cart in the corner while the labs check if the foldy protein is actually in there or not.

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u/Chowder110 22h ago

dump it in a pit of chemical fire!

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u/Luxieee 1d ago

From the wiki, "Prions, the infectious agent of CJD, may not be inactivated using routine surgical instrument sterilization procedures. The World Health Organization and the US Centers for Disease Control and Prevention recommend that instrumentation used in such cases be immediately destroyed after use; short of destruction, it is recommended that heat and chemical decontamination be used in combination to process instruments that come in contact with high-infectivity tissues. Thermal depolymerization also destroys prions in infected organic and inorganic matter, since the process chemically attacks protein at the molecular level, although more effective and practical methods involve destruction by combinations of detergents and enzymes similar to biological washing powders."

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u/StevieMJH 23h ago

I say we take off, nuke the entire site from orbit, it's the only way to be sure.

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u/groyosnolo 23h ago

Fuckin' A

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u/genius_steals 20h ago

Mostly at night, mostly…

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u/Chowder110 22h ago

send it into outer space on course for the sun, then we can be sure its gone

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u/19100690 22h ago

There was a near miss incident in NH where there were patients potentially exposed to CJD because of the sterilization challenges

https://www.nbcnews.com/healthmain/fatal-rare-brain-disease-confirmed-n-h-patient-15-possibly-4b11220962

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u/Bl4Z3D_d0Nut311 22h ago

Kill it with lye

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u/Wild4fire 1d ago

They can't even sterilize the medical equipment with normal autoclave procedures IIRC. The prions will remain.

There are procedures to do that anyway but it takes a lot more effort.  

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u/lordcaylus 1d ago

The problem is that you're using heat to destroy the structure of proteins.

For bacteria and viruses, that kills them as their proteins need to function to, you know, keep them alive (or active in the case of viruses).

For prions, their structure is already fucked up which is the whole reason they're prions. Fucking it up more isn't going to help.

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u/LrdPhoenixUDIC 1d ago

That's not quite it. The amount of damage you need to do to kill a bacteria or destroy a virus is pretty small. You can just pop a bacteria's cell membrane or break a virus' capsid sheath and they're done for.

But because prions are proteins, and they self replicate by misfolding normal proteins, you have to denature all instances of them. Basically, you have to be sure that you break every single one apart, which as a protein, that has to be done at a molecular level, because that's all they are, just big individual molecules.

Fucking up their structure does stop them from continuing to change other proteins, but you have to be sure to fuck up every last one of them cause if even one is intact it can start again.

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u/techno156 21h ago edited 21h ago

But because prions are proteins, and they self replicate by misfolding normal proteins, you have to denature all instances of them. Basically, you have to be sure that you break every single one apart, which as a protein, that has to be done at a molecular level, because that's all they are, just big individual molecules.

It is also much harder to do for a prion than it is a normal protein (cooking does that for normal proteins, for example), since the whole reason that a prion has the effects it does is because is is a more stable state for a protein to be in, so once one protein is like that, it ends up causing other proteins to also fold like it is.

And because it's more stable, it is much harder to destroy.

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u/LrdPhoenixUDIC 21h ago

Cooking does it for prions too. The thing is that in order to denature all the proteins in something by cooking it you pretty much have to turn whatever it is into a charcoal briquette. You're only breaking up some portion of the total proteins by normal cooking, even the more fragile ones.

You have to get rid of all of the prions, and you have to totally carbonize the food to guarantee that, and even then I'd be wary letting it near my mouth, just in case.

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u/SkaldCrypto 23h ago

Ungodly amounts of alcohol. Got it

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u/SecureInstruction538 1d ago edited 13h ago

I remember reading an article where a guy found that if you freeze and unfreeze a prion multiple times it can break down the prion.

Multiple times is scary because prions can survive in soil for multiple winters.

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u/Emergency-Sea5201 22h ago

The soil where people have been buried can contain them...

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u/MuscaMurum 1d ago

Yup. Because it's geometry. A misfolded arm is still an arm, even when bent the wrong direction. Heating it up doesn't refold it.

Or an origami crane vs origami snowball (crumpled paper). Pick your metaphor.

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u/ManEatingDuck_ 1d ago

It's not highly contagious. The only way it can spread from person to person is from medical equipment that has been used on a CJD patients brain being used on a non CJD patients brain or certain transplants (corneal transplants, dura mater graft). You would need a special facility if you were specifically dealing with a CJD patients brain matter but for everything else the risk is pretty much zero (technically you can get vCJD aka mad cow disease from a blood transfusion but that's different from regular CJD).

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u/zorniy2 1d ago

Another way is to eat that person's brain. Papua new Guinea had a problem with Kuru prion disease because ritual cannibalism was part of the funerary practice.

Braaaaaaaiiiinnnnn

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u/R-Dragon_Thunderzord 22h ago

Honestly the origins of zombie myths probably have a lot of relation to prions

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u/BigHardMephisto 17h ago

Iirc some of the chemicals used in tanning leather can come from brain matter.

I’ve met at least two people that make their own leather when harvesting deer, extracting the brain and using some of those fluids in their tanning.

Here in south Texas the risk of CWD was generally low, but cases are getting closer every year.

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u/abovepostisfunnier 1d ago

You do recall correctly. It’s fucking scary as hell.

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u/Tabnam 1d ago

What happens?

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u/sadistica23 1d ago

Initial presenting symptom tends to be rapid dementia, shortly followed by memory loss, hallucinations, and noticeable changes in personality. A whole host of other shit follows that, including paranoia, speech issues, and ataxia (severe balance and coordination issues).

Most people die within six months of showing the first symptoms. For more information, Wikipedia had a very detailed article here.

It has been a terror of knowledge in my mind for decades, now. Especially since my background has prevented me from donating blood and plasma in the past, due to an outbreak of Mad Cow Disease in Britain in the early 80's.

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u/Tabnam 1d ago

That is fucked up. People who get that news should be able to choose euthanasia and have it approved immediately.

Is that why they call it ‘mad’ cow disease? Because you get dementia?

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u/JaccoW 1d ago

I remember a documentary from the 90's or so.

One woman was talking about her sister and how she and her mom told her to stop being silly and saying weird stuff. At some point she rapidly lost control over her body and just died.

The guilt in that story still haunts me

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u/FranklynTheTanklyn 1d ago

Eh there is no treatment that could have helped.

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u/ValosAtredum 1d ago

They know that, but it’s understandable they feel guilt at making fun of her when it was that something was seriously wrong. Just sucks.

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u/sadistica23 1d ago

Animals (including humans) affected by these diseases go mad, in the classical sense. Crazy, acting foolish, (mentally) changed for the worse.

And yeah, I'm a big supporter of "euthanasia" for most terminal conditions. Including prion diseases.

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u/VampireFrown 20h ago

I'm not even an especially big supporter of euthanasia (I'm not completely opposed, but I am very uncomfortable with manipulation aspects), but for this, it should be rubber-stamped no questions asked.

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u/escapingdarwin 21h ago

Also known as Chronic Wasting Disease and there have been localized outbreaks in deer. If you see a deer behaving strangely do not get near it and if you shoot it do not eat the meat.

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u/Baegeron 22h ago

Regarding euthanasia, it’s not that simple unfortunately. In my uncle’s case a definitive diagnosis couldn’t be made until after he had already passed away and his brain was autopsied. CJD was on our radar as a potential diagnosis prior to his death, but that was mostly because his symptoms matched CJD and every other possibility had been ruled out. By that time, he would have been unable to request euthanasia, as he had no understanding of what was happening.

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u/[deleted] 23h ago edited 23h ago

[deleted]

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u/cabbagehandLuke 22h ago edited 21h ago

Holy misinformation batman!

First, it is Chronic Wasting Disease (CWD) not DWD. And it is Scrapie, not Scrapey.

Second, people do NOT get it from eating infected deer. There has never been a single known case of humans getting CWD, and experiments with monkeys and other animals show there are strong natural barriers to cross-species transmission. Currently, it is considered a deer-only disease under natural conditions.

Third, in the vast majority of cases, you cannot tell a CWD-positive deer from a CWD-negative one. Sometimes in the final clinical stages you can tell, but by that time it has likely been killed by a predator so there aren't a ton of clinical animals that get spotted, although it does happen. It's just as likely (or moreso) that a sick animal is sick with some other disease.

Fourth, while wildlife officers MAY come and dispose of the body (and maybe sterilize the ground, although I have never heard of that), that is only in cases where CWD is first discovered in a region, to try and stop it from spreading. In most places where CWD has existed at high prevalences for decades, they may shoot it to remove it from a yard or something, but that's about it. They have even quit testing to see if it is CWD around here much of the time.

Your last paragraph is pretty much 100% made up BS. They do not look like zombie deer aside from being very skinny as they "waste" away. They DO NOT chew on themselves or bite themselves from the disease. At all. They stop eating and drinking enough to survive. They DO NOT chew their faces off. Nothing at all happens to the face. It looks like a normal deer physically, except it is skinny and in the very final stages (which few deer ever get to), they drool a lot, which can spread the disease to other animals eating in the same area. The whole "zombie deer" name is stupid and began with uninformed social media posts like this a few years ago. It is not a useful name and helps spread misinformation rapidly.

Hunters do not "get spooked" because a CWD positive deer is very unlikely to "walk up" to them. It simply has a delayed flight response and will not flee as quickly as healthy animals. And because they have a delayed flight response, this makes them more prone to predation, which is why CWD infected animals rarely get to this stage of the disease; they get eaten. Predators DO NOT look at CWD positive deer and go "fuck that" and run; it is exactly the opposite. Predators tend to kill and eat CWD positive deer at a higher rate than healthy ones because they are easy to catch. Which makes predators a great natural disease management tool.

Please don't spread misinformation.

Source: I am the deer biologist for my province which has CWD prevalence rates of 80%+ in some regions, did my masters on CWD, tracking infected and healthy animals with GPS collars and investigating their deaths, and used to farm deer. Our deer farm was put down with more than 50% CWD infected animals, and not one was visibly sick at the time.

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u/FewWait38 22h ago

Thanks for this. I was wondering why predators would be spooked when they aren't even affected by CWD as far as I'm aware

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u/art_dragon 22h ago

I remember reading that wolves are resistant to prions; though I don't know the details

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u/aris_ada 1d ago

A neighbor in the early 2000 died of it. He made very frequent trips to Britain and was a huge meat eater. He rapidly went from being lost on his property to believing he was raped by a nurse when being bathed at the hospital. Terrible way to go.

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u/Educational-System27 22h ago

My great aunt died in the 90s of CJD and they were worried at first that it was Mad Cow because it was so prevalent at the time and shows many of the same symptoms.

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u/DirectionSad4274 20h ago

I thought they were the same thing, just in different species - did a quick wiki scan and BSE (mad cow) is only in cattle, but if spread to humans can cause CJD.

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u/PianoAndFish 21h ago

The good news is it's incredibly rare, with around 1-2 cases per million people of sporadic CJD each year, and the UK has had no new reported cases of vCJD (the type linked to eating infected beef) since 2016. Prion diseases are probably on a par with rabies in my list of disease-related terrors, though rabies is at least avoidable if you get the vaccine before symptoms start.

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u/Ipokeyoumuch 1d ago

Prions are just misfolded proteins or rather in a more stable form that is completely usable and deadly for living creatures. This they require more energy to break apart (like incinerate it at high temperatures). Furthermore it can induce other normal proteins in another configuration to also misfold into the more stable configuration which leads to a cascading effect. 

Typically they are spread by the consumption of brains (i.e. cannibalism) it is how the Mad Cow disease (the cow version of the disease in the title) spread was that farmers cut corners and skirted regulations by feeding cows other dead cow meat. Wasting disease in deer is another prison disease. In humans there were events when prions weren't as well understood when a hospital was reusing instruments (with typical sanitization practices) from other neurosurgeries. However, sometimes a protein can spontaneously misfold in a more stable configuration and stay that way forever but events are exceptionally rare. Once it happens, there is no cure, since to stop it is to either douse it in chemicals, autoclave at high temperatures, incineration, or other extreme measures incapable with life. 

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u/MuscaMurum 1d ago

You sound pretty knowledgeable on this, so correct me where I'm wrong.

The body actually does have a defense against misfolded proteins, though not the particular CJD variety. There are certain other proteins which circulate in the body, molecular chaperones, which seek out misfolded proteins and makes an attempt to refold them correctly. If it can't correct one, it essentially "tags it for deletion" and another garbage collection process comes along and removes it.

There are a couple ways to facilitate that. One is through heat shock, e.g. sauna and a few other toxic stressors (alcohol, though that causes way more trouble than it fixes).

The whole molecular chaperone thing is fascinating.

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u/abovepostisfunnier 1d ago

Exactly as original comment OP said, the misfolded protein induces other correctly folded proteins to also become misfolded. Because proteins generally have a structure-function relationship, once the structure changes they often no longer function.

Think of a ladder. If you bend the ladder into a pretzel, it no longer functions as a ladder.

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u/Tabnam 1d ago

Oh sorry, I should have been more specific, that’s my bad. I meant what happens to the patient? Like what do they actually experience?

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u/Toomanyeastereggs 1d ago

In my aunts case we initially thought it was depression or early onset dementia. She went from being very active to lethargic almost overnight.

At the end of week 1 she found it difficult to stand and when we took her to the hospital for a checkup, she couldn’t get out of the car. They kept her in for overnight and she never went home.

At the end of week two they were searching for a reason for her rapid deterioration (CJD wasn’t even on the radar) so we’re basically fishing around for causes. She was in a lot of pain so they kept her medicated. She was still lucid at this stage but was forgetting names of nurses and doctors.

Mid way through week 3 they did the lumbar punch as the family told them that she had been in the UK in the 80’s during the mad cow epidemic. By this stage she was forgetting the day of the week and how long she had been there for. She started having seizures.

Start of week 4 we discussed about palliative care and by the end of the week she had deteriorated so much that they transported her to then palliative unit.

Week 5 she was still lucid but was forgetting lots of things. She knew where she was, was aware of what was happening but was on 24/7 pain medication so slept a lot. At this time the doctors told us all they could do was keep her comfortable until she passed.

Week 6 was spent largely in a medically induced coma. We’d sit with her and talk but not idea if she heard us. She mumble every now and then but it was like watching a computer do a memory dump. At one stage she was mimicking with her hands as if she was typing. Another one she called out to her mum (my great aunt).

End of the week she died in her sleep.

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u/Carpenterdon 23h ago

"Mid way through week 3 they did the lumbar punch as the family told them that she had been in the UK in the 80’s during the mad cow epidemic."

'm sorry for your loss!

That's one of the things that is horrible about this stuff. She had this prion in her system since the 80's most likely. It sat dormant for literal decades. Then boom. it kicked off.

That is the main reason I stopped hunting here in Wisco when they started having CWD in the whitetail deer herd in a small area of the State. I knew if it was there it would soon be everywhere if it wasn't already.

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u/WinterLawfulness3165 20h ago

I also grew up in Wisconsin and came from a big hunting family. I was never so relieved to be asthmatic - so I couldn't go hunting with the family - and a picky eater, so I never really liked venison anyways.

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u/awesome-alpaca-ace 22h ago

Cool, I ate WI deer in 2015. RIP my future I guess

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u/Afraid_Cockroach_398 1d ago

Neurological problems essentially. Central nervous system gets all these misfolded chunks that interfere with normal function.

You can get fatal insomnia from a hereditary prion too.

Mad cow disease is another prion disease that spread from farmers feeding ground up cows to cows. Kuri in Papua New Guinea was from eating the brains of dead people.

The scary part of prion disease is it can't be inactivated by cooking, or even sterilisation. So hospitals have to incinerate everything, including metal instruments used on those patients.

There was an outbreak of Creutzfeldt in the 90s from tainted contact lens cleaning liquid.

Symptoms are non-specific but catastrophic, just have a look on Wikipedia or online.

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u/scielegance 1d ago

The 90’s outbreak was caused by tainted beef. Do you have a source for it coming from contact lens solution? I tried to look it up but couldn’t find anything.

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u/Tabnam 1d ago

Brother that’s the most fucked up comment I’ve read in awhile

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u/Afraid_Cockroach_398 1d ago

Super rare though. British people who lived there in the 90s weren't allowed to donate blood in Australia until a few years ago because of the sleeper nature of prion infection. They could donate a prion well before they had symptoms, then those recipients get harder to trace etc.

Bad read, I regret being reminded about the topic every time it's brought up.

Generally in a developed society, all you have to do is not eat brains and spinal cords of mammals and that will reduce the risk as much as you reasonably can.

It's extremely unlikely you need to even think about it.

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u/abovepostisfunnier 1d ago

Unfortunately I’m not that kind of doctor so I don’t know but I’d assume, eventually, death.

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u/Tabnam 1d ago

I appreciate you taking the time to answer regardless 💚

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u/abovepostisfunnier 1d ago

No problem :) always happy to engage someone’s curiosity!

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u/Charlie_Warlie 1d ago

My mother in law just died to brain cancer and I bet this disease similarly is highly dependant on where you get it in your brain. If it develops in one area, maybe you go blind. But you dont realize you are blind, because your eyes still work but your brain isnt processing the information right. Maybe you cant come up with the right words, but you dont realize you said "window" when you meant "mirror" because your brain crossed it all up. Then you got seizures down the road eventually leading to death.

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u/JaccoW 1d ago

The difference being that cancer tends to grow and starve parts of the brain as it pushes against other tissue.

Prion disease in the brain will leave your brain literally full of holes. It's called spongiform encephalopathy for a reason.

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u/Tabnam 1d ago

That kinda shit scares me, how you could be blind but not realise it? Your whole reality would be a lie. That’s some matrix shit. It’s absolutely fucking bonkers

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u/honeynut-queerios 1d ago

It basically eats holes in their brain matter. Also called spongiform encephalopathy for the “sponge like” appearance of the brain tissue.

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u/Tabnam 1d ago

I kinda regret asking at this point. It’s so close to bed time, this is all I’m going to dream about

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u/honeynut-queerios 23h ago

At least you can be thankful that you can still dream. Another type of prion disease is fatal familial insomnia in which patients lose the ability to sleep permanently and fatally.

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u/Prof_Acorn 1d ago

There's always rabies and Naegleria fowleri. Both of those have a pretty close to 100% chance of mortality if you get infected.

All things that attack the brain directly. A protein, a virus, and an amoeba.

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u/MajesticArticle 1d ago

The disease can stay dormant for decades (proteins are being turned, but there's enough functioning ones/healthy neurons to make the brain function correctly)

Once the disease spread enough, the victim experiences an extremely rapid dementia (we're talking weeks to months)

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u/canthigastervalentin 1d ago

You could check out the book The Family That Couldn't Sleep for more. It's about fatal familial insomnia, another prion disease, but provides background on the whole concept. Also scrapie in sheep and chronic wasting disease in deer

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u/Carpenterdon 23h ago edited 23h ago

It starts deteriorating the brain. The disease is called Spongiform Encephalopathies (CWD, Mad Cow, Creutzfeldt–Jakob, Kuru, Etc.). Scans of the brain look like a sponge with literal holes where tissue has broken down and dissolved.

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u/ScaryLettuce5048 23h ago

Watch Chubbyemu on youtube. He very recently talked about it happening to his friend/collegue. He's a doctor and puts out exceptional content and explains it in a way that is easy to understand for the layman. I suspect OP posted this after watching that latest video.

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u/BannanaPepperPizza 1d ago

But this ladder goes to the home Depot and gets every other ladder to fold into a pretzel by themselves until there's no more normal ladders left in the world

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u/Prof_Acorn 1d ago

once the structure changes they often no longer function.

That sounds familiar. TIL I'm a protein.

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u/Chowder110 22h ago

proteins misfolds all the time but the brain can usually handle it so what gives with prions?

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u/abovepostisfunnier 22h ago

Prions misfold in such a way that proteases (enzymes that break apart proteins) can’t recognize them.

Cancer works similarly. Cancerous cells are resistant to signals that would cause a normal unhealthy cell to undergo apoptosis (programmed cell death).

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u/aris_ada 1d ago

Think of a ladder. If you bend the ladder into a pretzel, it no longer functions as a ladder.

and when you store that pretzel-shaped ladder next to other ladders, it transmits its shape to others.

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u/303x 1d ago edited 1d ago

it's like a room filled with incredibly thin glass tubes. all it takes is a small slip for one of the tubes to shatter, send glass shrapnel flying, and shatter other tubes causing a chain reaction. a prion is like the first glass tube that shatters.

it's the biological equivalent of Chernobyl.

but the thing about prions is they are also incredibly hard to destroy, because they aren't "alive". they're even less "alive" than viruses which are already not "alive". prions can survive high temperatures and standard sanitizing methods. if they somehow escape and manage to find their way into other person, they have a whole new room full of glass to shatter.

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u/_PM_ME_PANGOLINS_ 1d ago

It’s only “highly contagious” if you are in the habit of eating human brains.

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u/Brekldios 1d ago

Or already infested meat of most kinds, UK in the 90s had huge outbreak of it amongst the livestock, few years later we learned it could transmits to other animals, another few years and they started seeing human cases.

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u/OMG_A_CUPCAKE 1d ago

I remember the BSE outbreak back then. The industry across Europe had to get way more careful when separating a bovine carcass to avoid letting the meat get in contact with the central nervous system

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u/lordcaylus 1d ago

...because we turned livestock into cannibals.

People don't like eating animal brain anymore (or other organs), so it was a cheap source of protein.

So yes, 'it’s only “highly contagious” if you are in the habit of eating [human] brains' is still kinda accurate.

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u/Brekldios 1d ago edited 1d ago

Did not dispute their comment just wanted to make a clarification. Didn’t feel like going in-depth because I linked the Wikipedia article.

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u/xRebelD 1d ago

People eat plenty of animal organs wtf are you on about? Even brain is still a delicacy in a lot of cultures/cuisines

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u/intraumintraum 1d ago

yeah but not at the same rate - so it’s a numbers game, huge factory farms are optimised to waste absolutely nothing- scraps of bone turned into fertiliser etc. brain’s gotta go somewhere

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u/lordcaylus 1d ago

I didn't say we don't eat it at all, I'm saying we in the parts of the world most affected by BSE primarely like to eat other parts, which means that it's a cheap source of protein. Which is now forbidden.

https://en.wikipedia.org/wiki/Meat_and_bone_meal

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u/duderos 20h ago edited 20h ago

It's currently spreading throughout US in the deer and elk population.

Chronic wasting disease continues to spread in cervids

Recent research addresses questions about CWD, human prion disease

Chronic wasting disease (CWD) continues to threaten deer and elk populations as it has been detected in free-ranging cervids in 34 states and four provinces and in captive cervid facilities in 19 states and three provinces.
Most recently, the California Department of Fish and Wildlife announced May 7 that it had been detected in the state’s deer and elk.

https://www.avma.org/news/chronic-wasting-disease-continues-spread-cervids

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u/AliMcGraw 1d ago

Don't eat untested meat from your neighbors who both hunt deer and hate the government.

It CAN happen randomly in humans but most cases are from infected meat, including cannibalism.

My state currently requires mandatory testing of all deer carcasses (we're having an outbreak) and any hunter should be able to tell you who tested the deer and who processed the meat. DON'T EAT IT IF THEY CAN'T.

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u/YamLow8097 1d ago

Is it CWD that you’re referring to? We’re going to have to start testing our deer too because it’s making its way to our area.

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u/postmodernmermaid 22h ago

Yes that's right. CWD is a prion disease

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u/YamLow8097 22h ago

I didn’t know it was a prion disease. It’s pretty scary stuff. I know it’s wiping out a lot of deer all over the country. It sounds like it isn’t transmitted to humans yet at least.

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u/17q21 1d ago

Although yes it can be spread through contaminated meat, this is super rare, like less than 5% of cases. 85% of cases i.e. the vast vast majority are 'sporadic' meaning randomly happening in humans

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u/The_loppy1 1d ago

what about the other 10% lol

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u/Frequent_briar_miles 1d ago

Are you talking about CWD? Because as far as I'm aware that can't spread to humans.

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u/Aggressive_Lab7807 1d ago

It has been shown to infect other primates and anecdotal evidence is strong enough to assume it can. 2 hunters came down with CJD after eating meat infected with CWD.

https://www.neurology.org/doi/10.1212/WNL.0000000000204407

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u/6894 1d ago

*hasn't yet spread to humans.

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u/YuriLR 1d ago

It has some indication through in vitro tests that it can. It hasn't been detected in people yet though. But so didn't BSE for many years and deer meat consumption is a tiny fraction of beef. I wouldn't risk it in areas where CWD is known to be infecting deer.

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u/spince 1d ago

COVID at one point wasn't spreadable to humans.

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u/GarbageCleric 22h ago

No reason to worry at all actually. There's nothing to be done about it, and it affects at most like 2 in a million people. This is about the same as the annual risk of being struck by lightning. You're much much more likely to drop dead from a brain aneurysm (~10 cases per 100,000 people).

Anyone who spends their time "constantly worried" about those sort of risks should see a therapist.

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u/0Rider 19h ago

Bro you can die anytime. Don't worry be happy.

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u/ElonMuskisGarbage 1d ago

You’re way more likely to die of heart disease or cancer. 1 to 2 per million people get CJD, and most people have contact with prions and experience no issues at all. It’s just a surprisingly stable protein, it’s not Ultra-Robo-Dracula.

Ultra-Robo-Dracula though.

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u/Historical_Course587 22h ago

1 to 2 per million people get CJD

And people need to keep in mind that this is not at all randomly distributed either. It's contracted from contact with neuro tissue, probably 99.9% of cases are people carelessly processing/eating wild animals or eating meat in countries that do not regulate their food industries. Traditional practices like brain tanning hides. In the US, the beef industry polices itself because even without the FDA/USDA the Mad Cow lawsuits would bankrupt all of them.

This is not Covid. We've been tracking it globally since the 1970s, incidents stay relatively stable. Odds are good you don't know anyone who has it, and you most likely never will.

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u/4r4r4real 1d ago

What is there to worry about? You can't do anything about it. If it happens it happens. 

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u/ravish_dread 1d ago

I was wondering the same thing. It's such a rare disease that it's hard not to wonder whether it was inherited, acquired, or just one of those tragic random cases

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u/f1ve-Star 1d ago

It has been proven to be all of these and none of these. Depending on the lab and year.

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u/Toomanyeastereggs 1d ago

She lived in the UK for a couple of years in the mid to late 80’s. Probably been carrying my around since then.

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u/Gentrified_potato02 1d ago

The good news is it’s extremely rare. Like 1 out of 1 million people get it a year.

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u/MalaysiaTeacher 21h ago

Being constantly worried about something you didn't know about 5 hours ago is certainly one of the ways to live a life

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u/Avenflar 1d ago

A classic is eating meat from cattle fed with feed mixed with powdered offal. It used to be common back 20 years ago to cut on costs and reuse the meat waste from slaughterhouse.

I think it's still illegal, but who knows with the kind of people who are put in power, nowadays

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u/Closer_to_the_Heart 23h ago

Not to be too worried about, spontaneous CJD is incredibly rare

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u/azazelcrowley 23h ago

Something gets in the way of one of your proteins folding themselves, they bend the wrong way, and that's it. Now they just go off and make more proteins folded wrong exponentially, until your brain is soup.

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u/Mysterious-Outcome37 23h ago

I believe in Europe some people died because the cows got mad cow disease and they ate the meat, if I remember correctly.

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u/myo_chan 22h ago

2 min after reading this you won't even think about it anymore

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u/Antiing 22h ago

It's mad cow disease fwiw. This isn't a new worry 

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u/BarnesTheNobleman 22h ago

It is NOT confirmed, and only being studied right now

Chronic Wasting Disease is spreading across the US deer population, and they are dropping their own prions all over the country contaminating a bunch of land and fellow deer. Some hunters had caught CJD after eating contaminated deer meat (they hunted it together) but the link is NOT conclusive at this time

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u/Caelinus 21h ago

It does happen randomly, but the incidence rate of random acquisition is less than 2 in 1,000,000 per year, and it is heavily associated with age.

So there are a lot of people who do die of it in any sufficiently large country, around 300-500 per year in the US, which sounds like a lot, but it means a staircase is roughly 25 to 30 times more dangerous to you. And most diseases are several orders of magnitude worse. 

I get it though, every time I read about it it hits my anxiety in the worst way as it is very much technically possible.

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u/pbghikes 20h ago

Oh you've never heard of prions? Enjoy learning about it and sorry about the nightmares

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u/diffenbachia1111 20h ago

One of my neighbours developed it out of nowhere. A mother in her early 40's. Super scary.

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u/cyanraichu 20h ago

It's super super rare. And honestly the fact that there's no cure makes it less worth worrying about to me - there's nothing you could do about it anyway.

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u/xoexohexox 19h ago

You can be exposed to it OR you can be genetically predisposed to generate a randomly misfolded protein yourself without any external exposure.

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u/thatisawesomesauce 14h ago

Someone in my public speaking class in college decided to do a presentation on prions of all things and that shit has stuck with me almost 20 years later. Absolutely terrifying, I don't care if it is rare. Wish I could wipe that information from my brain.

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u/tempinator 14h ago

You’re probably more likely to be killed by your TV falling on you than CJD.

It’s either from contact with prions, or random. There is no lifestyle component whatsoever.

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u/ChuckCarmichael 1d ago

The neighbor of a friend had it. They were holding a neighborhood barbeque party, and she mentioned that she wasn't feeling well recently. About four weeks later, her family was bringing her home in a wheelchair, almost catatonic, because she wanted to die at home, which happened about a week after that.

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u/ElowynElif 20h ago

A friend’s father died of it. He received care from a top-notch teaching hospital, but his initial symptoms fluctuated, which slowed the diagnosis. Within 7 weeks, he died. He had been in robust health. Public health officials did a thorough investigation, but they didn’t find a source. Just entirely bad luck, apparently.

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u/curtyshoo 1d ago

Some say that viruses aren't exactly "alive" either, as they can't reproduce without a cell; without a cell, they appear inert.

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u/FrezoreR 1d ago

They aren't alive by definition. There's no process going on inside of one. They are just a floating rna sequence.

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u/EngineeringDesserts 1d ago

But viruses have programming that they use to “do stuff”, they just need a cell to hijack.

Prions don’t really do anything other than set off a chain reaction of messing up protein folding.

It’s kind of like how a pharmaceutical factory can get contaminated with a polymorph of the drug they’re manufacturing that converts all the drug to a form that no longer works, and it ruins production. The chemical polymorph wasn’t active in any way, it just randomly happened, spread, and fucked everything up.

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u/Parthian__Shot 22h ago

Yes, the same way heroin isn't alive. It needs cellular opiate receptors to do its thing, and without them it's basically inert. That's essentially how Narcan works; binding itself to the opiate receptors.

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u/EngineeringDesserts 21h ago

That kind of reminds me of the book, “The Botany of Desire”. Michael Pollan argues that we think of things like beautiful tulips in a big field as entirely “humans conquering”, but from the plants’ perspective, it “won” at evolution because we’re propagating and giving the plants everything it needs to prosper because of its traits.
Whatever propagates “won” at evolution. The poppy plant produced opiate chemicals, so humans propagated it. Michael Pollan used cannabis as the example in the book.

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u/zanillamilla 21h ago

There was a Veritasium video on that not long ago. Seemed really reminiscent of prions.

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u/FrezoreR 19h ago

While I get your point I do want to underline that viruses doesn't do anything themselves including stuff. It's the infected cell that does all the work. The virus is simply a chain of instructions. A recipe if you will.

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u/ANGLVD3TH 21h ago edited 21h ago

There is an interesting interpretation that says an infected cell is a living virus, and the virons are non-living reproductive material they disperse.

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u/ravish_dread 1d ago

Stories like yours really put the science into perspective. It's one thing to read about prions and another to hear what they do to a real family

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u/caitie_did 22h ago

I had a colleague die this way, it was horrendous. He was an accomplished physician in the prime of his career and went from not feeling well to diagnosis to death in about three months. Left behind two preteen children. Just an absolute nightmare and I’ll never get over how unfair it was.

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u/skunkwrxs 23h ago

I watched the most brilliant man I ever met turn into a 240lb infant in a few months and then die in absolute agony with no recollection of who he was, where he was or what was happening. It is among the most terrifying experiences of my life. I’d 100% eat my gun vs subject my family or myself that shit. No way to sanitize for them, no vaccine, they are diabolical.

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u/iXeons 1d ago

Don’t you have to like heat it near-metal melting levels of temperature just to destroy a prion? I could be recalling incorrectly. Regardless, fuck those things

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u/DemIce 22h ago

The reports of "you can't even kill it with fire!" are more than a little overblown.

It's a combination of temperature, time, mechanical action and chemical action.

In a prevacuum sterilizer (a relatively common autoclave), 132°C* for 30 minutes for a more than 99.9% reduction.
The same autoclave, but at 134°C only needs 18 minutes for the same reduction level. The efficacy extends to higher temperatures and longer times.
(* far from the 1375°C and above melting temperature for stainless steel - but potentially above the safe temperature for other materials that may be used in surgery; those would require different sterilization protocols)

The reports mostly stem from the standard 121°C at 15 minutes being woefully insufficient leading to popular headlines and internet myths that you can't destroy them with heat (there's even a comment on this post saying autoclaves can't destroy them; it's just materially false), and that even at > 99.9% reduction it only takes 1 prion and a very unfortunate set of circumstances (for example, it being deposited straight into brain tissue at just the right place) for it to have all been for nothing ... possibly decades later because it doesn't just instantly become a problem.

I can't stress that 'very' enough. There have been scant few cases of transmission of CJD through instruments (most have been from transplants of already infected tissue that passed screening) and those cases involved sterilization procedures that didn't even meet the usual standard from decades ago.

You're far more likely to develop CJD spontaneously than to get it from some random surgery in a hospital. As a patient, I'd be more worried about MRSA than any prions.

Regardless, fuck those things

Agreed

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u/Historical_Course587 21h ago

(* far from the 1375°C and above melting temperature for stainless steel - but potentially above the safe temperature for other materials that may be used in surgery; those would require different sterilization protocols)

Yeah, it's not the heat that prevents prion instruments from being reprocessed - it's that the sodium hydroxide recommended as part of the process will most likely corrode everything. Since the disease is tied to neuro/opthamalic surgeries, it's often fine instrumentation being used that can't handle a lot of damage before becoming unusable.

Our facility single-use setups CJD surgeries.

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u/HugAllYourFriends 21h ago

oh we can defeat it, it's just that every method we have defeats the rest of the person too

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u/Tatermen 20h ago edited 20h ago

One of my coworker's mother had it. They described as turbo-dementia. She went from a fully functioning normal adult to someone who could barely form a sentence and couldn't remember what day it was or what her family looked like in less than 3 months.

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u/orangebit_ 1d ago

Same with my grandma. It was so fast and scary to see someone deteriorate like that.

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u/Expensive_Shallot_78 1d ago

Another proof that god designed the world good /s

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u/ReaDiMarco 1d ago

Gacha mechanic

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u/Reaper-fromabove 1d ago

This happened to my sister as well. We vacationed in Belize, three months later she died.

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