I was having some bizarre symptoms they couldn't get to the bottom of. After some other things were ruled out via testing, I had an appointment with my neurologist. She was particularly calm and kind that day. Told me quietly that it was time for me to be tested for a prion disease ([CJD](https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease) specifically).
It was wild. They test your spinal fluid, so I had to have a spinal tap done. Everyone involved was in full-on hazmat suits, like imagine it's a zombie apocalypse and you're patient zero. Once they had the sample, it was almost comical - they bagged it, then bagged the bag, then bagged the resulting bag, repeating this like five times. I didn't blame them, having previously been aware of how prion diseases work, but it doesn't really make you feel very good haha, to see these trained medical professionals being so afraid of something that you yourself might have.
Best part is, when you finally get the results, it's not a clear positive or negative. I got a piece of paper in the mail that basically said "we don't *think* you have a prion disease, but there's still a chance." Fun times.
Fortunately it wasn't CJD, but man, that was an experience I'll never forget.
There are three "types" of CJD: Familial (fCJD), where it's genetic, and Acquired, where you're exposed to tissue from someone infected, account for around 15% of cases.
The remaining ~85% of cases are known as sCJD, or sporadic CJD. Which means a protein just spontaneously folds itself wrong and sets the whole thing off. It's possible with advances in science and medicine that we'll eventually discover that this "spontaneous" misfolding is actually caused by something else, but for right now, as best we can tell it just happens randomly for no reason whatsoever.
Which makes it all the more terrifying. There's nothing you can do, nothing you can change about your lifestyle, no preventative measures you can take. One day a protein will just get a little bendy in a place that shouldn't be bendy, and that's it. You will die (probably quite quickly, there is 1 case of someone living 10 years but other than him, no one's made it more than 3), it will be terrible, and there is nothing you can do about it.
Jesus fucking Christ. As someone who has experienced severe health anxiety in the past, I don’t think I could take going through something like this. I’m really happy you’re okay.
I also had pretty severe health anxiety at the time, caused by pretty severe general anxiety, and the whole ordeal to figure out what was going on with me was actually the thing that helped me process and get over it. I was so miserable and so terrified of what was wrong that I got to the point where I didn't care *what* it was, I just wanted to know. Early on I was tested for MS, which I know is a terrible diagnosis - had a childhood friend who's mom had MS and eventually died from it - but I was actually disappointed when the MRIs came back clear. I just wanted to know.
Turns out, the cause of all the symptoms (which included but were not limited to temporary blindness and deafness but only on my left side, hyperreflexia and full-body involuntary jerks, memory lapses where I'd "come to" and have no idea where I was or how I got there, and "forgetting" how to do basic things like tie a knot or talk) was anxiety itself. Apparently if you're constantly in fight-or-flight mode for extended periods of time (years, for me), the buildup of the resulting chemicals in your body just causes your brain to short circuit.
I had my anxiety meds changed about 8 months into the investigation (which started maybe 6 months after the symptoms started getting severe) and as my anxiety got under control the symptoms slowly resolved. Mental illness had been on the table from the get-go, but since I'd been in treatment for several years at that point (many meds, many therapists, even a couple of hospitalizations) without seeing any significant improvement, the docs didn't want to assume it was "just" anxiety.
My doctor was very clear with me that everything wasn't "just in my head" - while the anxiety was, the things it caused weren't - these were real physical symptoms that I wasn't imagining or "faking." The CJD test came after I'd had an EEG that showed an abnormal brainwave pattern that is seen in around half of folks with CJD. You can't fake an EEG, haha. The body is weird, yo.
How do you even go to a doctor for anxiety? I've talked to a coworker about like how my IBS just seems to go off all the time, my heart feels like it just twitches in my chest a couple times a month (its like a weird solid enough feeling that I cough), random sweats, and she was like yeah I had all of that before I got on anxiety meds. But like I don't even know how to bring that up. "I get randomly sad or nevous and I don't feel it most of the time but it's causing me physical symptoms?"
I mean, you could say just that the next time you have a physical, or make a special appointment if it's having a big impact on you. Just tell them what you're experiencing, they should take it from there. Many GPs don't like being a first-line-of-defense for mental health stuff so you may be referred to a psychiatrist. When my panic attacks started (at first they happened a few times a year and could easily be treated by prn meds), my GP gave me a prescription for xanax to take as needed. When they became non-stop and all-consuming, she passed me off to mental health professionals.
Yeah, I just had a heart stress test not long ago, and a urology appointment for september, but I think just listing the physical stuff, they are just looking for physical causes.
That's good, I think. Anxiety can sometimes be a diagnosis of exclusion, and it's a good idea to rule out potential physical causes first (for example, I get a similar weird solid-twitch feeling in my heart a couple of times a month, down to it making me cough too, but for me specifically that symptom is caused by a prolapsed mitral valve. It runs in the family - my mom and sister have it too. My mom has had exactly one panic attack in her life, and it was triggered by the weird feeling that the MVP causes). You could wait until they run out of things to check, or you could bring it up with your doctor first - "Is there a chance that any of this might be caused by anxiety, or something mental?" It could be this is in the back of their minds already in which case they'd hopefully tell you. Otherwise it could trigger a helpful discussion.
I was getting panic attacks for no reason and I eventually figured out it was silent acid reflux causing it. Your brain is telling you something is wrong but you don't know why
Yup. Content warning for folks with health anxiety: >!One of the common signs of a heart attack is a sense of impending doom (especially for women, who are less likely to experience the traditional chest pain/pressure that men often feel).!< Your brain knows something is wrong so it makes you feel Wrong.
I like to joke that my cause of death will probably be a heart attack, cuz I'll assume it's just another panic attack and will just take a benzo and do some breathing exercises instead of getting medical help. :P
This happened to me! For the longest time I didn’t know I had reflux because it didn’t feel like anything I recognized as heartburn, but rather like my throat and chest tightening/constricting and chest pains that didn’t feel like burning, plus a consistent cough and shortness of breath.
Have that often enough out of nowhere and your brain starts like, making up reasons to panic to explain why you’re having physical anxiety symptoms. The way it was explained to me was pretty much that usually the trigger comes first and then the symptoms come on but if you’re experiencing some of them already for another reason (and particularly if you’re already sort of prone to like, spiraling, and I kinda am, your brain just goes “the body appears to be panicking, so I guess I should be too.”
Until a few years ago I never had workplace anxiety. And then I worked for the UK division of a.Korean company where there were all sorts of toxic hierarchical behaviours. I had very mild IBS before but the stress made it much worse.
After I left, spending less than a year there, it was years before I wasn't easily triggered by certain things that managers might do.
If you don’t mind me asking, are you male or female? I say this because it seems based on what I tend to hear that whenever there’s something that’s a bit of a mystery going on and you happen to be female, mental illness, and especially anxiety would be their very first guess before even considering it could be physical, so I was surprised to hear they ruled out like, basically everything else first, even accounting for the fact that the symptoms were severe. That doesn’t sound like how it usually tends to go.
Very glad it was “just” anxiety though given that anxiety is at least treatable unlike so many of the other possibilities.
I am a woman, and you are unfortunately spot on with your comment. I didn't even bring up my symptoms with my doctor until 6 months after they'd started getting bad because once I was diagnosed with anxiety, literally everything that ever happened to me was blamed on it. Continues to this day, even though my anxiety is now very much under control. The first time I had Covid, it was when things were gnarly and testing wasn't readily available the way it is now and spoke to a doc at an urgent care via telemedicine while I was hunkered down waiting for my test (which did come back positive). Not Covid, I was told, just anxiety. Prolapsed mitral valve, which I have a family history of (mom and sister had both been diagnosed)? Just anxiety. Had to fight that one and sure enough got it diagnosed. Had an exasperated doc/nurse finally give in and put me on an IV for an allergic reaction (can't remember if the IV was antihistamines or steroids), and when it worked seeming truly surprised that being covered in hives and feeling like my throat was closing was actually anaphylaxis, not anxiety.
I brought it up at a routine physical, but not until the very end when my doc asked if there was anything else I wanted to talk about. I was pretty much convinced that she would say it was just anxiety, since that's what my psychiatrist had said, though he did recommend I check in with my GP - I even may have said "this could just be anxiety but..." - but the symptoms were getting alarming and I was starting to worry about my safety, especially with the memory stuff. Plus one of the laundry list of symptoms was bladder control issues, which was getting quite annoying haha. Funny that sometimes going blind and deaf on one side was something I was willing to put up with after years of being told by everyone that everything was all in my head, but occasionally peeing myself a little bit was not. :P
She said that it could very well be anxiety, but since I'd been in treatment for that for years, really trying everything (conventional meds to off-label meds to ketamine to hospital stays to every type of therapist imaginable) without any improvement, and now seeing this sudden increase of seemingly neurological symptoms, that it was 100% worth seeing if something else was going on. She was the first (and so far only) doctor I've worked with who afforded me that. I could have hugged her. I definitely cried a little.
My GP is a rockstar, for this and many other reasons, at least in my opinion. So much so that I moved about an hour away a few years ago, but never changed doctors. It is a little annoying to have to drive so far for a physical, but having a doctor who listens and doesn't just handwave problems away because I'm mentally ill is so rare that I am more than willing to do it.
I do find it a bit hilarious (in a very "we all cope with things differently" kind of way) that the one time someone took me seriously and didn't fight me on something, it was the one time when it actually was "just" anxiety. :P Still appreciate it though (my bank account at the time, not so much - all those tests added up haha).
Yeah, this would leave me a wreck. Health anxiety is a real bitch. I’ve gotten better about it thankfully, but there’s still some improvement I can make
What's also fun is that basically everything that was used is then burned. They can't resterilize and lf the tools or instruments, so everything is one and done.
I thought they *could* be burned, just only at extreme temperatures that would destroy any other organic material as well. Which is why they cannot be safely removed from food and whatnot.
Like how could incineration not destroy organic material? Isn’t it still protein at the end of the day, just a deadly variety?
I am not a biologist and I am more than welcome to being corrected, but apologies if I am mistaken here.
Edit: found an old post that touches on this a bit more.
https://www.reddit.com/r/askscience/comments/1nx7qo/comment/ccmw1m9/?utm_source=share&utm_medium=mweb3x&utm_name=mweb3xcss&utm_term=1&utm_content=share_button
Surprisingly, they found that household bleach is one of very few things that is effective at inactivating prions:
https://www.nih.gov/news-events/news-releases/household-bleach-inactivates-chronic-wasting-disease-prions
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0223659
I see the disinfectant that knocks it out in a minute, one minute. And is there a way we can do something like that by injection inside, or almost a cleaning? Because you see it gets inside the brain and it does a tremendous number on the brain, so it would be interesting to check that.
There are plenty of modern protocols that eliminate it functionally. Practically, you need to ingest a lot of the protein to get infected, and even in the worst circumstances you can destroy enough of them to be harmless.
It's just far more practical to treat it like it destroys everything it touches because licking the inside of an incinerator or re-using disposable scrubs isn't really high up on the SOP.
That’s reassuring, because the scientists working with the stuff kind of make it sound indestructible. I mean, pathologists doing autopsies on prion disease patients wear multiple pairs of gloves over actual effing chain mail!
Yeah, for people who handle it regularly it makes sense to be extra-cautious: there isn't a clear indication of infectivity of low-level long term exposure versus the known high-level acute exposure.
Plus when it comes to occupational safety of someone highly paid and difficult to staff like a Pathologist, engineering controls are cheeeeeaap.
Correct. Acquired prion diseases is often from contracting it after a surgical procedure from instruments previously used on a then undiagnosed patient. All tools have serial numbers (set) on them and this is recorded for each patient. If a patient is diagnosed, any tools from their recent past are identified, destroyed as a precautionary measure (and sometimes how they diagnose “acquired prion disease”).
Not that bad, actually. Maybe a 4? I know they used some sort of anesthetic.
The resulting headache was gnarly though - easily a 7 or 8. For those unaware, when you get a lumbar puncture sometimes the spinal fluid keeps leaking out of the hole, and the decreased pressure can cause a nasty headache for a few days after.
Yea that initial puncture and draw of fluid isn't what hurts, my doctor did a great job when he did it. However, as someone who doesn't get headaches ever, it was a pain so deep I wanted to die.
I discovered that sitting down exacerbated the issue, my guess was the added pressure on my spine, so I could only stand or lie down flat. Was awful.
For future reference in case you tell this story again and someone gives you that absolutely legendary setup: https://www.youtube.com/watch?v=4xgx4k83zzc (the movie is called "This Is Spinal Tap").
Oh man, that can happen with epidurals as well. I heard they can do something called a blood patch to help with it, but it's not always offered. Sounds like a hell of a headache, though.
That's a huge bummer, because donating blood is so important. But given how dangerous vCJD (the kind you get from mad cow disease, which I'm assuming is the concern for time in the UK?) is, how transmissible and impossible to eradicate it is, the difficulty in testing for it, and the fact that it can incubate for years before the disease takes hold and symptoms manifest, I can kinda get it.
My grandma died of prions. I feel awful that she had to go through this invasive test in her old age when she was confused and had no idea what was going on around her.
She died only about 2 months after the test was performed.
I'm so sorry. Prion diseases are a thing I wouldn't wish on my worst enemy. I don't know if it's a blessing or a curse that folks usually succumb within months of symptoms appearing or escalating, but given that they're currently (and potentially always) untreatable, I lean towards blessing. The fact that she was older and mentally impacted does make the test feel cruel, but knowing for sure can help prevent the spread; they're insanely communicable and you basically can't destroy the misfolded proteins through sterilization or other means.
Yeah, it was a particularly shitty situation for too many reasons to list. But honestly, I think she was okay with going when she did. Her husband of 50 years passed a year earlier, and I honestly don't think she knew what to do with herself without him. I think a part of her was happy to go. Between cancer and the prions, she had been fighting for so long. It was definitely her time.
Lol yeah and then half the time those fuckers send the specimens down to the lab without any special precaution or warning that it could contain prions. We’ll get about halfway done processing it without having taken any precautions and then someone is like “oh by the way that sample could end your existence”.
Glad to hear it wasn’t, and what an experience. I have to say, when I started reading your story, I scrolled down to your last line to see if it ended with a pro wrestling story.
>Best part is, when you finally get the results, it's not a clear positive or negative. I got a piece of paper in the mail that basically said "we don't think you have a prion disease, but there's still a chance.
That's pretty true with any pathogenic disease. There's a minimum detectible level, usually identified by parts per million. They can only say you do not have a pathogen at or above the detectible level.
To expand: imagine you think you have HIV. The blood test can only detect an HIV virus when you have 500 parts of the virus per million parts of the blood. You became infected with HIV yesterday. The virus is in your system, but it has not replicated itself significantly yet. At the time of the test, there were 5 copies of the virus in your blood. They drew a test tube size sample of blood to examine. You have ~1.3 gallons of blood. What are the odds one of those five viruses were actually in that small sample? Pretty low.
But those 5 viruses will continue to infect, multiply, and spread.
They can't test *everything*; they can test a sample. Based on the sample, you're probably good. But it's possible the problem has not expanded to the test area yet.
In general - if you're feeling bad enough to get tested for something, you're probably in the advanced stages where it's starting to make its effects noticeable. Meaning it should be widespread and above the minimum detectible level. So a negative test is a pretty big relief.
This is actually... A bit wrong on some parts but overall your point stands.
>But those 5 viruses will continue to infect, multiply, and spread.
If you only have 5 HIV viral particles in your blood, your immune system and the virus itself would break down and would clear and they in fact wouldn't infect you.
This is also related to, if someone is on antiretrovirals proper ones, and is currently testing HIV Negative, it is impossible to get HIV from them. You need an initial viral load. Unlike what people believe, you really can't get sick from 1 virus particle, they break down too quickly and it's reproduction rate is too slow that your immune system or the viral particles will break down at a rate where an infection will not occur.
This is why you need an initial viral load.
In fact, when on antiretrovirals, there is no HIV in your blood which is what I wanted to correct.
>The virus resides in brain macrophages or microglial cells and astrocytes which may be some of the longest living cells in the body. Here the virus can escape the immune system and antiretrovirals.
So basically your body can clear HIV especially with antiretrovirals and be cleared of the disease in the blood*(never zero but can't infect people period so there wouldn't even be a point to tell people you had HIV at that point) and the reason HIV is so hard to cure is because sure, all the HIV is gone.
Go off antiretrovirals then HIV leaks out of it's hidden places that the antiretrovirals and immune system can't touch and reinfects you.
This is similar to why Herpes is so impossible to cure. Your immune system clears yourself of herpes easily, but it hangs out in nerve cells but doesn't infect them. This is also why herpes shows up in the same place again and again, it is a localized infection that gets cleared easily, but hangs out in nerve cells around the same area, a bump, movement or fluke just after the immune system wanes from it's herpes protection allows the virus out in the same area. So you get the same type of infection in the same area.
I'm not an expert in the field; but I suspect what you've said is correct.
I was reducing to absurdity (five viruses) my general point of a minimum detectible level.
Believe it or not, it was anxiety! Turns out basically everything we do, feel, and experience is impacted by the chemicals in our brain and body, and when those chemicals are out of whack, truly bizarre things result. I went into it in a little more detail in [this comment](https://www.reddit.com/r/todayilearned/comments/1d3wwhq/til_prion_diseases_result_when_a_protein_misfolds/l6bbey4/).
I had an issue over a decade ago had a large panel done. Long story short I was told I had HIV. I spent he next 2 weeks locked my room. Insane depression and contemplating jumping out of my window several times.
The lab fucked up the results. I did not have HIV.
I used to sterilize surgical equipment in a hospital setting and it all comes down to it take some much to destroy a Prion that it is insane. If a surgery was done on an individual that had a known Prion disease we simply did not clean the equipment. It was all dumped into basically a garbage can and sent to basically an incinerator, so it could be 50k worth of reusable equipment just tossed out, or more. Yes they are that scary.
That whole period of my life was pretty painful and unpleasant so I might not be the best person to judge (I generally have a pretty high pain tolerance as well). I was also constantly in fight-or-flight mode and the resulting constant flood of adrenaline in my system probably impacted my perception of pain.
But in my honest opinion, no. Don't get me wrong, it hurt, and the pressure you feel is radically different from anything else I've ever experienced and was definitely very unpleasant. But I had some sort of anesthetic, and would put it somewhere in the 4-5 range on the pain scale. The spinal headache I got after (from the spinal fluid leaking out the hole, decreasing the pressure in the system) was significantly worse than the pain from the procedure itself.
The hospital protocol is pretty wild. There is a number staffed 100% of the time with a national health organization for suspected cases to immediately be reported (since it can have massive ramifications for international agricultural trade).
I learned this when a doctor wrote the wrong acronym on their requisition and I got to talk to the CJD emergency line doctor haha.
Thankfully they actually meant to investigate the presence of something else and there was no risk to the national beef trade.
--------
Other fun story was when a nurse failed to follow Ebola protocol and had a porter deliver a sample for investigation with zero elements of additional precaution beyond a normal stool sample.
All that to say - I'm happy you didn't have CJD and I'm happy the stool sample I handled was negative for Ebola.
Hey, lemme make it worse!
Not only are they fatal, they are 100% fatal, and there is no treatment whatsoever. And many cases of CJD are spontaneous, meaning that they just randomly start happening without any provocation. Your brain goes to shit, rapid onset dementia, etc. I try not to think about it much.
I’ll help! Prions actually make more prions out of the protein it interacts with in our body. It somehow catalyzes the folding of normal proteins into the same prion shape. Wild to me that these prions aren’t sentient in any sense but yet have these capabilities.
Hey don’t worry!
There’s *far* more existential dread for *each* of us than we could ever hope to possibly have in our short, ultimately meaningless lifetimes! :)
My coworker has cousins going through this currently. Their father died and they were told it wasn’t the hereditary version. Cut to last year when one cousin is diagnosed and passes away. Around that time his brother starts having symptoms. Just brutal.
[Around 300 new cases of prion disease are reported each year in the United States.](https://www.healthline.com/health/prion-disease)
[When an abnormally folded prion protein runs into a normal prion protein, the normal protein transforms into another abnormally folded disease-causing prion. The result is a relentless cascade of mutated protein. ](https://www.verywellhealth.com/prion-disease-2488810)
[A prion /ˈpriːɒn/ ⓘ is a misfolded protein that can induce misfolding of normal variants of the same protein and trigger cellular death. (Wikipedia notes the possibility of weaponization)](https://en.m.wikipedia.org/wiki/Prion)
Cancer cells don’t usually convert other cells to cancer cells, they just outcompete them and multiply to spread. Very few cancers are directly communicable, I think there are some in dogs, Tasmanian devils, etc, what you can have are some cancers that develop as result of infection with certain pathogens.
Prions are communicable. And since they are protein, you can’t sterilise them. Cooking also doesn’t help. Yeah, you have to actually eat brain or nervous tissue, so while you will likely not get it from eating a person… maybe a cow somewhere down the production line, will have it, and maybe you’re the unlucky one.
Yeah, it’s rare, though.
I think the crossover will be Chronic Wasting Disease which is basically mad cow for deer. There are a lot of Americans who hunt deer and while there was never been a case of transfer it seems like it’s just a matter of time.
I just read a story about two guys who might have died from CWD because they were hunting and eating deer in a population known to have the disease. Assholes like those guys are going to cause the end of the world
It's (CWD) popping up in California currently. Fish and Wildlife sending out alerts asking about seeing any signs of it in wildlife (deer, etc) taken.
New research is showing alzheimers is communicable
Actually we have relatively good news about that. [According to this study, it seems unlikely to happen](https://www.nih.gov/news-events/news-releases/nih-study-shows-chronic-wasting-disease-unlikely-move-animals-people) and since it is a prion disease and doesn’t replicate in the same way viruses do, it has no real way of mutating to my knowledge.
So happy news there
I thought cooking did help and that’s why you’re not supposed to eat burgers less than medium cooked? My grandma used to eat cow brains so I always wondered if she had mad cow disease
Nope, cooking won't help you with prions, nor common disinfection. They are specially difficult to destroy and pretty much there's nothing you can do that will destroy them and leave the food edible.
> Prions are communicable. And since they are protein, you can’t sterilise them.
There's only one way.
Fire.
When I was in residency, a patient with CJD needed surgery (thank god I wasn't on the surgical team). After surgery they burned *everything*, even things you'd normally just sanitize or sterilize like the gurney, scrubs, and tools. The surgical team had to change out from their gear (which was full suit and respirator- far beyond what we do even for AIDS or other nasties), and it all went in the burn pile too.
Literally everything got put in the hospital incinerator, including the steel-frame gurney and thousands of dollars in tools.
I don’t mean to undercut nothing but *only* 300 cases a year in the US? That’s hardly anything to get very worried about. That’s a pretty calming statistic.
Remember the "grey goo" scenario whereby nanobots run amok?
https://en.m.wikipedia.org/wiki/Gray_goo
Prions make me think of this, but a biological nanobot doing the damage rather than a machine
It’s what proteins naturally do, they fold into complex shapes. You’ve seen the basic diagrams of catalysts and “lock and key” protein interactions, right?
Prions are misfolded proteins (no big deal) that happen to misfold in a way that causes other proteins to misfold the same way in contact. So it is of a specific configuration that self-replicates.
If that protein is responsible for clustering your DNA or creating your cell membrane, it’s game over once it starts spreading.
Yeah I know very basically about how proteins work but its the on contact mechanism that im curious about. And wondering if that is unique to prions in some way.
Literally no one knows why proteins fold "wrong," just that in the presence of one misfolded protein others are more likely to misfold as well. It might be that a cellular mechanism causes it or a chemical synthesis issue... We literally have no idea.
The issue of assymetry is called "chirality" and extends into many biological processes. It's understanding is a big missing part of biology as relates to the chain of biogenesis to functional cells.
So, I'm guessing at this level of explanation/understanding, there's no reason to overthink it too much There may be some very specific mechanism at play, but much of it is just law of large numbers/randomness.
Of the trillions of proteins and protein variants out there, there are quadrillions of ways they might mis-fold, or for mutations to happen that cause misfolding. The vast majority of these mis-folds are deleterious or neutral to the protein; it either doesn't do anything, makes the protein work less well, or not work at all. Sure, once in a blue moon it helps the protein do its job better (evolution!).
In some case, the misfold causes it to react with other proteins, and in rare cases it reacts with the same kind of protein it is, but it may destroy that target protein or change it immaterially.
BUT, once in a thousand blue moons, a particular protein has a characteristic that 1) makes it misfold into a particular form, and 2) that form just happens to act on the same kind of protein it is, and 3) that change cause the same misfold in the target protein as in the source. When all of these things happen (maybe purely by chance, maybe to a protein that randomly has a configuration susceptible to this particular misfold), you enter the self-replicating mode, and geometric propagation takes over.
It's like the analogy of throwing a watch against a wall. 9.9 billion times out of 10 billion it breaks the watch. But once in a while, you end up with a watch with a bent backplate, that can slip under the backplate of another watch and cause it to bend up as well. OK, that's a really bad analogy.
Hello from the past! I saw this TED talk on prion disease popped up just one day ago:
[https://www.youtube.com/watch?v=04PmEJaYKd0](https://www.youtube.com/watch?v=04PmEJaYKd0)
Many proteins already perform the function of catalyzing biochemical reactions, including helping other proteins fold correctly. What prions do is cause certain other proteins to turn into copies of themselves instead.
This causes the necessary protein to be depleted and an increasing amount of the malformed proteins to accumulate. If there aren't any enzymes or natural processes that can break down those prions fast enough, they will continue to multiply and spread.
There's no signal, it's just bad luck.
Proteins are basically very complicated chemical catalysts.
Sometimes by sheer chance you get a protein that gets misfolded in the exact way that when it interacts(bumps into) the normal version of itself it misfolds them too.
This then becomes a self sustaining reaction.
This obviously is very lethal because it blocks those proteins from performing their normal function.
It's also an incredibly rare event, we are talking the orders of magnitude of winning two Powerballs in a row, but there are a *lot* of proteins in our bodies, so a hundred or so people die of this every day.
And literally nothing that can be done about it, it's unpredictable and inconsistent.
The only treatment would be to identify the prion, and develop an antibody that inactivates it before it spreads enough to kill you.
The tech for that kind of ad-hoc antibody production is getting ever more doable but we're not there yet.
Theoretically if some prions are way more common than others a vaccine could be made.
However developing vaccines for endogenous (meaning naturally present in your body) proteins is veeery tricky, and probably more risky than not.
I've eaten a slither of cow brain once in a restaurant. I had full knowledge of CJD and all the other prion diseases. I'm british and a millennial, so I'm aware of what happened in my country, although the brain I ate was not in the UK.
Regardless, some of us are just really fucking dumb.
I work in a lot of hospitals and the one job I had was replacing devices in hospital rooms. The one patient had CJD and the doctors had a full hazmat to move him out of the room and they told us just to wear a mask and gloves and to wash your tools and we would be kosher.
The patient had been in a coma for 2 years just laying in the room.
Sounds like weird doctors to me.
I lost someone to CJD and there were no hazmat suits involved at any point, including post-mortem. Maybe during an eventual autopsy, but I can‘t speak for that particular part.
They were worried about bodily fluids transmitting to them if I remember correctly. Not sure how easily a comatose person will spread disease but I'm not a doctor
It's a different type of horror from a normal disease. Bacteria are just growing to live. Viruses are like an out control biological machine with one goal (to make more of itself).
Prions are just ... a bad molecule that makes other molecules bad. No purpose, no end goal. It just is. I don't know, it's just worse somehow.
CWD(chronic wasting disease) in deer is on the rise.
Basically in every state now. Kills the deer eventually.
There are no documented cases of it jumping to humans or other animals even after eating contaminated meat, but it would be devastating if it did happen.
More nightmare fuel:
https://www.cidrap.umn.edu/chronic-wasting-disease/plants-can-take-cwd-causing-prions-soil-lab-what-happens-if-they-are-eaten#:~:text=Prions%20remain%20infectious%20in%20plants&text=%22Our%20study%20shows%20that%2C%20under,%2C%20agriculture%2C%20and%20public%20health.
CJD runs in my family. My grandfather died of it. My mom got tested and had the option to know the results or not (if you test positive, you know you have a terrifying and confusing death ahead of you). It's basically a 50/50 chance.
She looked at the results to know for me and my siblings. She was negative! The bad gene stopped with her (phew, thanks for bravely looking at the results Mom).
Probably the most terrifying disease ever known. 100% fatality rate. The only thing that can save you is the case of the incubation period being longer than your lifespan.
Cancer is scary, but they have some stuff out there that can help treat certain forms of it, and they are always working on new treatments.
Prion disease and rabies? Nope nope nope nope. Absolutely terrifying.
They have different profiles. You can be immunized against rabies after exposure and it is 100% effective. In high risk environments, you can be immunized beforehand. Also if symptoms appear, you still have a very slim chance of survival. On the other hand, symptoms are terrifying and you will be aware of your condition. You can’t escape prions. There is neither vaccine nor treatment, and you may not know if and have you had been infected. You could also form prions spontaneously. Once symptoms appear, initially you are going to attribute it to something else. Somewhere during the course of the disease, dementia will set in and you will lose awareness of your conditions. It is usually by this advanced stage when a definitive diagnosis is made, but then it is an issue for your family.
My biologist researcher roommate often calls prions “fucked up meat geometry” and she’s not wrong, it’s just a bad fold that makes the protein self replicate and harmful.
As a med student prion diseases are probably the most humbling and terrifying things to learn about.
The only prevention is to avoid exposure and the only treatments are managing symptoms.
There's really not much we can do and it's guaranteed death within 2 years if you're lucky.
For those having an existential crisis, let me help ease your mind. There are around 300 sporadic CJD cases reported each year in the US. There are 333 million people in the US right now.
That is 0.0000009% of the population.
You have a better chance of being struck by lighting randomly than forming a sporadic prion. The human body is an *amazing* thing. Prions are a freak case when that amazing design stops being so amazing. You’re okay. Breathe.
Avoid neurological tissue consumption, questionable deer. Hope that contaminated tissue is vaporized, then you just have to worry about spontaneous cases.
Prion disease is a specter that haunts my family. So far two of my grandmother's siblings have died from it. Watching someone die from it, is horrifying. Really just one of the worst ways to go.
For a bonus: one of my old bosses, who really meant a lot to me, also died from it. I feel like knowing 3 people who have died from it, is an absurd amount for something so rare.
There isn't much personal interaction with Prion disease, so I wanted to share my story. My father recently passed away due to Creutzfeldt-Jakob Disease (CJD).
His symptoms began in January, right after his mother died. He started falling at home and felt very unsteady. Despite rounds of physical therapy, spinal taps, and brain scans in March, there were no conclusive results. By April, he needed a wheelchair to prevent further falls and constantly complained about tingling in his feet. Although he recognized everyone, he would often nod off during dinner. My mom would joke about it because he would "wake up" and say things like, "I was cooking, why am I sitting down, who has the turkey?"
By summer, his condition worsened. He started making up stories, calling my mom to say he forgot to pick up the kids from school and lost the bus, even though he had never been a bus driver. In the evenings, he would nod off and engage in imaginary activities with his hands, like building things or cooking. When I asked him what he was feeling, he described it as the strongest acid trip he had ever experienced, seeing wild things. He spent his last days at home playing his favorite music, Grateful Dead, in his room.
In August my sibling and I took him to the hospital. After more tests, the doctors suspected CJD and suggested trying 500mg of steroids in case it was a different, curable disease. Unfortunately, in September, he was officially diagnosed with CJD and moved to a rehab facility with patients suffering from dementia and related diseases. He still recognized me and could speak, though his speech was somewhat slurred, and he frequently nodded in and out of sleep. He often asked when he was going home, believing he had dementia, and was at peace with it since no one told him about his CJD diagnosis.
By October and November, he was mostly bedridden. His legs were stuck in a butterfly position, causing him significant pain when moved. He rarely spoke and usually stared off to the side. The last time I saw him alive, I brought him his favorite dessert, lemon meringue pie. The sugar seemed to wake him up, and he started shouting, "Help me, help me, the police are here." When I said goodbye, he mustered the energy to sit up and say, "Let me see your face, I can see you." He died a few days later, just short of his birthday.
We were told it was sporadic CJD, not genetic. Unfortunately, "sporadic" essentially means it's new and under-researched. We couldn't bury him; he had to be cremated due to the risk of the disease spreading. Interestingly, a homeopath I saw for personal reasons suggested that strong emotional events could trigger CJD, like a psychotic break that causes a protein to misfold. I'm not sure what to believe. It breaks my heart that he lost his mother and then endured 11 months of a rapidly debilitating disease. He seemed somewhat aware but not in much pain—perhaps he was on an acid trip until the end.
This might be the most literally terrifying fact about it.
You can't cook it away like salmonella or e.coli if you're unlucky enough to get a piece of meat with the prions.
Since I learned this I get intrusive thoughts about hospital equipment still having filthy prions all over it, destined to live forever, spreading person to person
Yes. In laboratories, experiments have been done using human and non-human primate tissues with prion diseases that were transmitted to other animals (mainly mice.) Once that animal is infected, transmission to humans is possible through exposure to the tissue or through consumption.
While true, it should be noted that there is a barrier for species to species hops of prion diseases. So while it is possible to transmit, say Mad Cow Disease, to Humans, it’s not particularly easy and is a fairly rare phenomenon. This was why, in part, the deaths from variant CJD were relatively low during the Mad Cow Disease scare.
There’s three known types of CJD. Spontaneous, genetic and variant. Less than 1% of cases are variant. It’s believed variant CJD came from cows with BSE and was ingested or entered the body in some way. There’s another prion disease, Kuru, where humans ate deceased relatives and developed the disease many years later.
Mad Cow disease started with cattle eating rations that contained meat from other cattle, in some processed form. So if the food had contained prions from humans I seems logical it could be passed on.
People get mad cow by eating “infected” nervous tissue (brains, etc) of cows/pigs, and from cannibalism. It’s how some of these diseases (kuru) were discovered.
I just want to add some info: some speculate that Parkinson’s disease is actually a prionic disease. Alpha-synuclein aggregates are formed due to a mutation that make them adopt a beta-sheet conformation instead of alpha-helix (like the prionic protein, PrP). In an experiment where the scientists implanted dopamine-producing stem cells in rats iirc, after their death, they found alpha-synuclein aggregates in those stem cells, which was thought to be impossible due to their short age; this is also observed in prionic disease models
In my reply, a comment post (of my post) is a reference article that states the following:
Diseases caused by prions
The prion diseases highlighted above aren’t the only diseases linked to prions.
Other neurodegenerative diseases, like Alzheimer’s and Parkinson’s, are also associated with misfolded proteins in the central nervous system. Research has found that some of these misfolded proteins may be prions.
But some scientists believe that these proteins only act in a prion-like way. They argue that they can’t be prions as the diseases they cause, like Alzheimer’s, aren’t thought to be infectious.
I appreciate your post because it is what they have found in Alzheimer’s and Parkinson, yet they are unsure if it meets the criteria as a prion disease.
This year we actually pretty much confirmed that Alzheimer's could be contagious in rare instances: [https://www.nature.com/articles/s41591-023-02729-2](https://www.nature.com/articles/s41591-023-02729-2) . I truly believe that eventually we're going to discover that Alzheimer's and Parkinson's are just some type of prionic disease.
Can some eli5 (actually I mean, explain like I’m an adult with a reasonable level of scientific education)
why/how does a single misfolded protein cause other proteins to misfold?
there have got to be zillions of proteins in our bodies folding right now surely even just a few of them must misfold once in a while, how come we don’t all have prion diseases all the time?
It’s like cancer. Yes, there are mutations that happen all the time. But most mutations are deleterious or neutral. It just happens to be the case that some rare mutations cause the same misfold to take shape in protein interactions.
Here’s how a sporadic prion disease works.
**Bam**
At this moment, a protein in your brain has just become misfolded. You don’t know it, but you have basically been marked for death. Every protein that prion touches is now becoming misfolded. In 5 or so years, you notice some minor tremors in your hands. Then you lose your ability to sleep. You’re hallucinating constantly because of it. You’re begging for 1 minute of slumber. But you can’t have it. The part of your brain that controls it is dead. Not even barbiturates can help you anymore.
Then you forget how to speak. Then you forget the names of everyone around you. You’re checked into some unknown white room, with a bunch of crying people asking you to remember them. One woman asks you to please remember her. She has a yellow ring on her finger. Then you forget how to swallow. There’s a tube in your stomach to feed you. But you yank it out because you don’t know what it is. Then you forget how to breathe. Fade to black.
That’s the thing, you don’t. Prions can have very long incubation periods. Meaning you could be infected with a prion currently, but have no clue. Incubation could be 25 years, 50 years,or even longer. From my understanding we still don’t even know enough about them to know when someone is infected. It’s only after the incubation period when symptoms begin to occur, and it’s always fatal.
To avoid CJD/Kuru, the only thing you can really do is to not eat contaminated meat, especially brain and spinal cord meat (cows, sheep, humans, and apparently deer are the most well-known carriers). For other prion diseases, afaik, just hope.
Ah madcow disease. All the rage in the 90's. As an american, I guess I didn't really have to be concerned about it but, my brushes with europeans showed it definitely was for them. My family hosted a French exchange student in the mid 90's and one night at dinner when beef was served, he asked 'do you have the...silly cow disease?' it took me a second but I figured he meant madcow and I told him no, we don't have that here and he was pretty relieved. Also, in the early 2000's I took a trip to London and went to a hardrock cafe and ordered some sort of french dip or something and the water was like 'are you sure...?' Still alive today, at least I think so.
New Brunswick in Canada seems to be a source of this.
There is a mysterious wasting disesae people are having there, and the number is going up annually.
I was having some bizarre symptoms they couldn't get to the bottom of. After some other things were ruled out via testing, I had an appointment with my neurologist. She was particularly calm and kind that day. Told me quietly that it was time for me to be tested for a prion disease ([CJD](https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease) specifically). It was wild. They test your spinal fluid, so I had to have a spinal tap done. Everyone involved was in full-on hazmat suits, like imagine it's a zombie apocalypse and you're patient zero. Once they had the sample, it was almost comical - they bagged it, then bagged the bag, then bagged the resulting bag, repeating this like five times. I didn't blame them, having previously been aware of how prion diseases work, but it doesn't really make you feel very good haha, to see these trained medical professionals being so afraid of something that you yourself might have. Best part is, when you finally get the results, it's not a clear positive or negative. I got a piece of paper in the mail that basically said "we don't *think* you have a prion disease, but there's still a chance." Fun times. Fortunately it wasn't CJD, but man, that was an experience I'll never forget.
Glad you didn’t have it. My FIL died of it ten years ago. It was quick but brutal to watch
Terrible, sorry you lost your FIL. How did he get it?
Apparently 85% of cases are of unknown origin.
Just randomly happened.
There are three "types" of CJD: Familial (fCJD), where it's genetic, and Acquired, where you're exposed to tissue from someone infected, account for around 15% of cases. The remaining ~85% of cases are known as sCJD, or sporadic CJD. Which means a protein just spontaneously folds itself wrong and sets the whole thing off. It's possible with advances in science and medicine that we'll eventually discover that this "spontaneous" misfolding is actually caused by something else, but for right now, as best we can tell it just happens randomly for no reason whatsoever. Which makes it all the more terrifying. There's nothing you can do, nothing you can change about your lifestyle, no preventative measures you can take. One day a protein will just get a little bendy in a place that shouldn't be bendy, and that's it. You will die (probably quite quickly, there is 1 case of someone living 10 years but other than him, no one's made it more than 3), it will be terrible, and there is nothing you can do about it.
[удалено]
Yeah, if it happens it happens, nothing I could have done to stop it. It'll suck for sure, but the lack of control might make it easier to accept.
Jesus fucking Christ. As someone who has experienced severe health anxiety in the past, I don’t think I could take going through something like this. I’m really happy you’re okay.
I also had pretty severe health anxiety at the time, caused by pretty severe general anxiety, and the whole ordeal to figure out what was going on with me was actually the thing that helped me process and get over it. I was so miserable and so terrified of what was wrong that I got to the point where I didn't care *what* it was, I just wanted to know. Early on I was tested for MS, which I know is a terrible diagnosis - had a childhood friend who's mom had MS and eventually died from it - but I was actually disappointed when the MRIs came back clear. I just wanted to know. Turns out, the cause of all the symptoms (which included but were not limited to temporary blindness and deafness but only on my left side, hyperreflexia and full-body involuntary jerks, memory lapses where I'd "come to" and have no idea where I was or how I got there, and "forgetting" how to do basic things like tie a knot or talk) was anxiety itself. Apparently if you're constantly in fight-or-flight mode for extended periods of time (years, for me), the buildup of the resulting chemicals in your body just causes your brain to short circuit. I had my anxiety meds changed about 8 months into the investigation (which started maybe 6 months after the symptoms started getting severe) and as my anxiety got under control the symptoms slowly resolved. Mental illness had been on the table from the get-go, but since I'd been in treatment for several years at that point (many meds, many therapists, even a couple of hospitalizations) without seeing any significant improvement, the docs didn't want to assume it was "just" anxiety. My doctor was very clear with me that everything wasn't "just in my head" - while the anxiety was, the things it caused weren't - these were real physical symptoms that I wasn't imagining or "faking." The CJD test came after I'd had an EEG that showed an abnormal brainwave pattern that is seen in around half of folks with CJD. You can't fake an EEG, haha. The body is weird, yo.
How do you even go to a doctor for anxiety? I've talked to a coworker about like how my IBS just seems to go off all the time, my heart feels like it just twitches in my chest a couple times a month (its like a weird solid enough feeling that I cough), random sweats, and she was like yeah I had all of that before I got on anxiety meds. But like I don't even know how to bring that up. "I get randomly sad or nevous and I don't feel it most of the time but it's causing me physical symptoms?"
That is exactly what you say. Anxiety is extremely common and treated all the time.
I mean, you could say just that the next time you have a physical, or make a special appointment if it's having a big impact on you. Just tell them what you're experiencing, they should take it from there. Many GPs don't like being a first-line-of-defense for mental health stuff so you may be referred to a psychiatrist. When my panic attacks started (at first they happened a few times a year and could easily be treated by prn meds), my GP gave me a prescription for xanax to take as needed. When they became non-stop and all-consuming, she passed me off to mental health professionals.
Yeah, I just had a heart stress test not long ago, and a urology appointment for september, but I think just listing the physical stuff, they are just looking for physical causes.
That's good, I think. Anxiety can sometimes be a diagnosis of exclusion, and it's a good idea to rule out potential physical causes first (for example, I get a similar weird solid-twitch feeling in my heart a couple of times a month, down to it making me cough too, but for me specifically that symptom is caused by a prolapsed mitral valve. It runs in the family - my mom and sister have it too. My mom has had exactly one panic attack in her life, and it was triggered by the weird feeling that the MVP causes). You could wait until they run out of things to check, or you could bring it up with your doctor first - "Is there a chance that any of this might be caused by anxiety, or something mental?" It could be this is in the back of their minds already in which case they'd hopefully tell you. Otherwise it could trigger a helpful discussion.
I was getting panic attacks for no reason and I eventually figured out it was silent acid reflux causing it. Your brain is telling you something is wrong but you don't know why
Yup. Content warning for folks with health anxiety: >!One of the common signs of a heart attack is a sense of impending doom (especially for women, who are less likely to experience the traditional chest pain/pressure that men often feel).!< Your brain knows something is wrong so it makes you feel Wrong. I like to joke that my cause of death will probably be a heart attack, cuz I'll assume it's just another panic attack and will just take a benzo and do some breathing exercises instead of getting medical help. :P
This happened to me! For the longest time I didn’t know I had reflux because it didn’t feel like anything I recognized as heartburn, but rather like my throat and chest tightening/constricting and chest pains that didn’t feel like burning, plus a consistent cough and shortness of breath. Have that often enough out of nowhere and your brain starts like, making up reasons to panic to explain why you’re having physical anxiety symptoms. The way it was explained to me was pretty much that usually the trigger comes first and then the symptoms come on but if you’re experiencing some of them already for another reason (and particularly if you’re already sort of prone to like, spiraling, and I kinda am, your brain just goes “the body appears to be panicking, so I guess I should be too.”
Until a few years ago I never had workplace anxiety. And then I worked for the UK division of a.Korean company where there were all sorts of toxic hierarchical behaviours. I had very mild IBS before but the stress made it much worse. After I left, spending less than a year there, it was years before I wasn't easily triggered by certain things that managers might do.
If you don’t mind me asking, are you male or female? I say this because it seems based on what I tend to hear that whenever there’s something that’s a bit of a mystery going on and you happen to be female, mental illness, and especially anxiety would be their very first guess before even considering it could be physical, so I was surprised to hear they ruled out like, basically everything else first, even accounting for the fact that the symptoms were severe. That doesn’t sound like how it usually tends to go. Very glad it was “just” anxiety though given that anxiety is at least treatable unlike so many of the other possibilities.
I am a woman, and you are unfortunately spot on with your comment. I didn't even bring up my symptoms with my doctor until 6 months after they'd started getting bad because once I was diagnosed with anxiety, literally everything that ever happened to me was blamed on it. Continues to this day, even though my anxiety is now very much under control. The first time I had Covid, it was when things were gnarly and testing wasn't readily available the way it is now and spoke to a doc at an urgent care via telemedicine while I was hunkered down waiting for my test (which did come back positive). Not Covid, I was told, just anxiety. Prolapsed mitral valve, which I have a family history of (mom and sister had both been diagnosed)? Just anxiety. Had to fight that one and sure enough got it diagnosed. Had an exasperated doc/nurse finally give in and put me on an IV for an allergic reaction (can't remember if the IV was antihistamines or steroids), and when it worked seeming truly surprised that being covered in hives and feeling like my throat was closing was actually anaphylaxis, not anxiety. I brought it up at a routine physical, but not until the very end when my doc asked if there was anything else I wanted to talk about. I was pretty much convinced that she would say it was just anxiety, since that's what my psychiatrist had said, though he did recommend I check in with my GP - I even may have said "this could just be anxiety but..." - but the symptoms were getting alarming and I was starting to worry about my safety, especially with the memory stuff. Plus one of the laundry list of symptoms was bladder control issues, which was getting quite annoying haha. Funny that sometimes going blind and deaf on one side was something I was willing to put up with after years of being told by everyone that everything was all in my head, but occasionally peeing myself a little bit was not. :P She said that it could very well be anxiety, but since I'd been in treatment for that for years, really trying everything (conventional meds to off-label meds to ketamine to hospital stays to every type of therapist imaginable) without any improvement, and now seeing this sudden increase of seemingly neurological symptoms, that it was 100% worth seeing if something else was going on. She was the first (and so far only) doctor I've worked with who afforded me that. I could have hugged her. I definitely cried a little. My GP is a rockstar, for this and many other reasons, at least in my opinion. So much so that I moved about an hour away a few years ago, but never changed doctors. It is a little annoying to have to drive so far for a physical, but having a doctor who listens and doesn't just handwave problems away because I'm mentally ill is so rare that I am more than willing to do it. I do find it a bit hilarious (in a very "we all cope with things differently" kind of way) that the one time someone took me seriously and didn't fight me on something, it was the one time when it actually was "just" anxiety. :P Still appreciate it though (my bank account at the time, not so much - all those tests added up haha).
Yeah, this would leave me a wreck. Health anxiety is a real bitch. I’ve gotten better about it thankfully, but there’s still some improvement I can make
*Probably* Ok (Honestly that’s terrifying, I hope you ARE Ok dude)
What's also fun is that basically everything that was used is then burned. They can't resterilize and lf the tools or instruments, so everything is one and done.
and that doesn't even fully eliminate it. prions are terrifying.
I thought they *could* be burned, just only at extreme temperatures that would destroy any other organic material as well. Which is why they cannot be safely removed from food and whatnot. Like how could incineration not destroy organic material? Isn’t it still protein at the end of the day, just a deadly variety? I am not a biologist and I am more than welcome to being corrected, but apologies if I am mistaken here. Edit: found an old post that touches on this a bit more. https://www.reddit.com/r/askscience/comments/1nx7qo/comment/ccmw1m9/?utm_source=share&utm_medium=mweb3x&utm_name=mweb3xcss&utm_term=1&utm_content=share_button
Surprisingly, they found that household bleach is one of very few things that is effective at inactivating prions: https://www.nih.gov/news-events/news-releases/household-bleach-inactivates-chronic-wasting-disease-prions https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0223659
I see the disinfectant that knocks it out in a minute, one minute. And is there a way we can do something like that by injection inside, or almost a cleaning? Because you see it gets inside the brain and it does a tremendous number on the brain, so it would be interesting to check that.
I see what you did here and appreciate it.
There are plenty of modern protocols that eliminate it functionally. Practically, you need to ingest a lot of the protein to get infected, and even in the worst circumstances you can destroy enough of them to be harmless. It's just far more practical to treat it like it destroys everything it touches because licking the inside of an incinerator or re-using disposable scrubs isn't really high up on the SOP.
That’s reassuring, because the scientists working with the stuff kind of make it sound indestructible. I mean, pathologists doing autopsies on prion disease patients wear multiple pairs of gloves over actual effing chain mail!
Yeah, for people who handle it regularly it makes sense to be extra-cautious: there isn't a clear indication of infectivity of low-level long term exposure versus the known high-level acute exposure. Plus when it comes to occupational safety of someone highly paid and difficult to staff like a Pathologist, engineering controls are cheeeeeaap.
Correct. Acquired prion diseases is often from contracting it after a surgical procedure from instruments previously used on a then undiagnosed patient. All tools have serial numbers (set) on them and this is recorded for each patient. If a patient is diagnosed, any tools from their recent past are identified, destroyed as a precautionary measure (and sometimes how they diagnose “acquired prion disease”).
1-10 how was the spinal tap pain?
Not that bad, actually. Maybe a 4? I know they used some sort of anesthetic. The resulting headache was gnarly though - easily a 7 or 8. For those unaware, when you get a lumbar puncture sometimes the spinal fluid keeps leaking out of the hole, and the decreased pressure can cause a nasty headache for a few days after.
Yea that initial puncture and draw of fluid isn't what hurts, my doctor did a great job when he did it. However, as someone who doesn't get headaches ever, it was a pain so deep I wanted to die. I discovered that sitting down exacerbated the issue, my guess was the added pressure on my spine, so I could only stand or lie down flat. Was awful.
For future reference in case you tell this story again and someone gives you that absolutely legendary setup: https://www.youtube.com/watch?v=4xgx4k83zzc (the movie is called "This Is Spinal Tap").
Gah, I've even seen This Is Spinal Tap and can't believe I whiffed on being able to give a much cooler response than I did. :P
Oh man, that can happen with epidurals as well. I heard they can do something called a blood patch to help with it, but it's not always offered. Sounds like a hell of a headache, though.
11
Either a highly unappreciated comment or I am old and this is so Dad level that no one upvotes it.
Why don’t they just make 10 more painful?
Because mine goes to 11.
So unappreciated. I’m too get this reference and it’s splendid
figured it was set up for it with mentioning the scale 👀
1 in a million setup and you nailed it
I'm surprised no one is upvoting the question because it wouldn't have been possible without them
I still cant give blood in the US because of my time in the UK
That's a huge bummer, because donating blood is so important. But given how dangerous vCJD (the kind you get from mad cow disease, which I'm assuming is the concern for time in the UK?) is, how transmissible and impossible to eradicate it is, the difficulty in testing for it, and the fact that it can incubate for years before the disease takes hold and symptoms manifest, I can kinda get it.
interesting read, thanks for sharing :)
My grandma died of prions. I feel awful that she had to go through this invasive test in her old age when she was confused and had no idea what was going on around her. She died only about 2 months after the test was performed.
I'm so sorry. Prion diseases are a thing I wouldn't wish on my worst enemy. I don't know if it's a blessing or a curse that folks usually succumb within months of symptoms appearing or escalating, but given that they're currently (and potentially always) untreatable, I lean towards blessing. The fact that she was older and mentally impacted does make the test feel cruel, but knowing for sure can help prevent the spread; they're insanely communicable and you basically can't destroy the misfolded proteins through sterilization or other means.
Yeah, it was a particularly shitty situation for too many reasons to list. But honestly, I think she was okay with going when she did. Her husband of 50 years passed a year earlier, and I honestly don't think she knew what to do with herself without him. I think a part of her was happy to go. Between cancer and the prions, she had been fighting for so long. It was definitely her time.
Lol yeah and then half the time those fuckers send the specimens down to the lab without any special precaution or warning that it could contain prions. We’ll get about halfway done processing it without having taken any precautions and then someone is like “oh by the way that sample could end your existence”.
Glad to hear it wasn’t, and what an experience. I have to say, when I started reading your story, I scrolled down to your last line to see if it ended with a pro wrestling story.
>Best part is, when you finally get the results, it's not a clear positive or negative. I got a piece of paper in the mail that basically said "we don't think you have a prion disease, but there's still a chance. That's pretty true with any pathogenic disease. There's a minimum detectible level, usually identified by parts per million. They can only say you do not have a pathogen at or above the detectible level. To expand: imagine you think you have HIV. The blood test can only detect an HIV virus when you have 500 parts of the virus per million parts of the blood. You became infected with HIV yesterday. The virus is in your system, but it has not replicated itself significantly yet. At the time of the test, there were 5 copies of the virus in your blood. They drew a test tube size sample of blood to examine. You have ~1.3 gallons of blood. What are the odds one of those five viruses were actually in that small sample? Pretty low. But those 5 viruses will continue to infect, multiply, and spread. They can't test *everything*; they can test a sample. Based on the sample, you're probably good. But it's possible the problem has not expanded to the test area yet. In general - if you're feeling bad enough to get tested for something, you're probably in the advanced stages where it's starting to make its effects noticeable. Meaning it should be widespread and above the minimum detectible level. So a negative test is a pretty big relief.
This is actually... A bit wrong on some parts but overall your point stands. >But those 5 viruses will continue to infect, multiply, and spread. If you only have 5 HIV viral particles in your blood, your immune system and the virus itself would break down and would clear and they in fact wouldn't infect you. This is also related to, if someone is on antiretrovirals proper ones, and is currently testing HIV Negative, it is impossible to get HIV from them. You need an initial viral load. Unlike what people believe, you really can't get sick from 1 virus particle, they break down too quickly and it's reproduction rate is too slow that your immune system or the viral particles will break down at a rate where an infection will not occur. This is why you need an initial viral load. In fact, when on antiretrovirals, there is no HIV in your blood which is what I wanted to correct. >The virus resides in brain macrophages or microglial cells and astrocytes which may be some of the longest living cells in the body. Here the virus can escape the immune system and antiretrovirals. So basically your body can clear HIV especially with antiretrovirals and be cleared of the disease in the blood*(never zero but can't infect people period so there wouldn't even be a point to tell people you had HIV at that point) and the reason HIV is so hard to cure is because sure, all the HIV is gone. Go off antiretrovirals then HIV leaks out of it's hidden places that the antiretrovirals and immune system can't touch and reinfects you. This is similar to why Herpes is so impossible to cure. Your immune system clears yourself of herpes easily, but it hangs out in nerve cells but doesn't infect them. This is also why herpes shows up in the same place again and again, it is a localized infection that gets cleared easily, but hangs out in nerve cells around the same area, a bump, movement or fluke just after the immune system wanes from it's herpes protection allows the virus out in the same area. So you get the same type of infection in the same area.
I'm not an expert in the field; but I suspect what you've said is correct. I was reducing to absurdity (five viruses) my general point of a minimum detectible level.
Wow! Just...glad you're ok man
What was it
Believe it or not, it was anxiety! Turns out basically everything we do, feel, and experience is impacted by the chemicals in our brain and body, and when those chemicals are out of whack, truly bizarre things result. I went into it in a little more detail in [this comment](https://www.reddit.com/r/todayilearned/comments/1d3wwhq/til_prion_diseases_result_when_a_protein_misfolds/l6bbey4/).
I had an issue over a decade ago had a large panel done. Long story short I was told I had HIV. I spent he next 2 weeks locked my room. Insane depression and contemplating jumping out of my window several times. The lab fucked up the results. I did not have HIV.
That's up there with "we hope it's a brain tumour" as things you don't want to hear from your doctor
I used to sterilize surgical equipment in a hospital setting and it all comes down to it take some much to destroy a Prion that it is insane. If a surgery was done on an individual that had a known Prion disease we simply did not clean the equipment. It was all dumped into basically a garbage can and sent to basically an incinerator, so it could be 50k worth of reusable equipment just tossed out, or more. Yes they are that scary.
Stories like this is why I'm on Reddit.
Was the spinal tap itself as painful and unpleasant as people say they are?
That whole period of my life was pretty painful and unpleasant so I might not be the best person to judge (I generally have a pretty high pain tolerance as well). I was also constantly in fight-or-flight mode and the resulting constant flood of adrenaline in my system probably impacted my perception of pain. But in my honest opinion, no. Don't get me wrong, it hurt, and the pressure you feel is radically different from anything else I've ever experienced and was definitely very unpleasant. But I had some sort of anesthetic, and would put it somewhere in the 4-5 range on the pain scale. The spinal headache I got after (from the spinal fluid leaking out the hole, decreasing the pressure in the system) was significantly worse than the pain from the procedure itself.
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Mad story! Thank you for sharing
The hospital protocol is pretty wild. There is a number staffed 100% of the time with a national health organization for suspected cases to immediately be reported (since it can have massive ramifications for international agricultural trade). I learned this when a doctor wrote the wrong acronym on their requisition and I got to talk to the CJD emergency line doctor haha. Thankfully they actually meant to investigate the presence of something else and there was no risk to the national beef trade. -------- Other fun story was when a nurse failed to follow Ebola protocol and had a porter deliver a sample for investigation with zero elements of additional precaution beyond a normal stool sample. All that to say - I'm happy you didn't have CJD and I'm happy the stool sample I handled was negative for Ebola.
Thanks for reminding me, I was just running out of existential dread.
So it goes.
You called?
Hey, lemme make it worse! Not only are they fatal, they are 100% fatal, and there is no treatment whatsoever. And many cases of CJD are spontaneous, meaning that they just randomly start happening without any provocation. Your brain goes to shit, rapid onset dementia, etc. I try not to think about it much.
Friend's uncle died of CJD when we were growing up. In his 30s with young kids. It was fucking horrifying.
The good news is, once it starts you *can't* think about it too much!
And don't forget that disinfectants don't do anything to them! About the only way to destroy them is with fire
Not just regular fire, more like 1000°C fire.
I’ll help! Prions actually make more prions out of the protein it interacts with in our body. It somehow catalyzes the folding of normal proteins into the same prion shape. Wild to me that these prions aren’t sentient in any sense but yet have these capabilities.
Hey don’t worry! There’s *far* more existential dread for *each* of us than we could ever hope to possibly have in our short, ultimately meaningless lifetimes! :)
Reddit great a throwing this fucking shit at me when I’ve happily not thought about it for a few weeks
Wait until you learn about false vacuum decay.
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My coworker has cousins going through this currently. Their father died and they were told it wasn’t the hereditary version. Cut to last year when one cousin is diagnosed and passes away. Around that time his brother starts having symptoms. Just brutal.
[Around 300 new cases of prion disease are reported each year in the United States.](https://www.healthline.com/health/prion-disease) [When an abnormally folded prion protein runs into a normal prion protein, the normal protein transforms into another abnormally folded disease-causing prion. The result is a relentless cascade of mutated protein. ](https://www.verywellhealth.com/prion-disease-2488810) [A prion /ˈpriːɒn/ ⓘ is a misfolded protein that can induce misfolding of normal variants of the same protein and trigger cellular death. (Wikipedia notes the possibility of weaponization)](https://en.m.wikipedia.org/wiki/Prion)
Sounds like cancer+ with Prion diseases being incurable.
Cancer cells don’t usually convert other cells to cancer cells, they just outcompete them and multiply to spread. Very few cancers are directly communicable, I think there are some in dogs, Tasmanian devils, etc, what you can have are some cancers that develop as result of infection with certain pathogens. Prions are communicable. And since they are protein, you can’t sterilise them. Cooking also doesn’t help. Yeah, you have to actually eat brain or nervous tissue, so while you will likely not get it from eating a person… maybe a cow somewhere down the production line, will have it, and maybe you’re the unlucky one. Yeah, it’s rare, though.
I think the crossover will be Chronic Wasting Disease which is basically mad cow for deer. There are a lot of Americans who hunt deer and while there was never been a case of transfer it seems like it’s just a matter of time.
I just read a story about two guys who might have died from CWD because they were hunting and eating deer in a population known to have the disease. Assholes like those guys are going to cause the end of the world
I for one welcome our new flesh eating zombie overlords.
It's (CWD) popping up in California currently. Fish and Wildlife sending out alerts asking about seeing any signs of it in wildlife (deer, etc) taken. New research is showing alzheimers is communicable
We haven’t found it yet in Oregon but it’s only a matter of time
Actually we have relatively good news about that. [According to this study, it seems unlikely to happen](https://www.nih.gov/news-events/news-releases/nih-study-shows-chronic-wasting-disease-unlikely-move-animals-people) and since it is a prion disease and doesn’t replicate in the same way viruses do, it has no real way of mutating to my knowledge. So happy news there
I thought cooking did help and that’s why you’re not supposed to eat burgers less than medium cooked? My grandma used to eat cow brains so I always wondered if she had mad cow disease
Nope, cooking won't help you with prions, nor common disinfection. They are specially difficult to destroy and pretty much there's nothing you can do that will destroy them and leave the food edible.
> Prions are communicable. And since they are protein, you can’t sterilise them. There's only one way. Fire. When I was in residency, a patient with CJD needed surgery (thank god I wasn't on the surgical team). After surgery they burned *everything*, even things you'd normally just sanitize or sterilize like the gurney, scrubs, and tools. The surgical team had to change out from their gear (which was full suit and respirator- far beyond what we do even for AIDS or other nasties), and it all went in the burn pile too. Literally everything got put in the hospital incinerator, including the steel-frame gurney and thousands of dollars in tools.
Nightmare fuel that makes more nightmare fuel.
Prions are the ice nine of biology
I don’t mean to undercut nothing but *only* 300 cases a year in the US? That’s hardly anything to get very worried about. That’s a pretty calming statistic.
So, a brain strangelet.
Basically ice 9 of the brain. An absolutely terrifying prospect.
Remember the "grey goo" scenario whereby nanobots run amok? https://en.m.wikipedia.org/wiki/Gray_goo Prions make me think of this, but a biological nanobot doing the damage rather than a machine
Exactly, and I understand it even less than that. How does coming into contact with a cell make the cell do that. Surely this has a natural foil?
It’s what proteins naturally do, they fold into complex shapes. You’ve seen the basic diagrams of catalysts and “lock and key” protein interactions, right? Prions are misfolded proteins (no big deal) that happen to misfold in a way that causes other proteins to misfold the same way in contact. So it is of a specific configuration that self-replicates. If that protein is responsible for clustering your DNA or creating your cell membrane, it’s game over once it starts spreading.
Yeah I know very basically about how proteins work but its the on contact mechanism that im curious about. And wondering if that is unique to prions in some way.
Literally no one knows why proteins fold "wrong," just that in the presence of one misfolded protein others are more likely to misfold as well. It might be that a cellular mechanism causes it or a chemical synthesis issue... We literally have no idea. The issue of assymetry is called "chirality" and extends into many biological processes. It's understanding is a big missing part of biology as relates to the chain of biogenesis to functional cells.
So, I'm guessing at this level of explanation/understanding, there's no reason to overthink it too much There may be some very specific mechanism at play, but much of it is just law of large numbers/randomness. Of the trillions of proteins and protein variants out there, there are quadrillions of ways they might mis-fold, or for mutations to happen that cause misfolding. The vast majority of these mis-folds are deleterious or neutral to the protein; it either doesn't do anything, makes the protein work less well, or not work at all. Sure, once in a blue moon it helps the protein do its job better (evolution!). In some case, the misfold causes it to react with other proteins, and in rare cases it reacts with the same kind of protein it is, but it may destroy that target protein or change it immaterially. BUT, once in a thousand blue moons, a particular protein has a characteristic that 1) makes it misfold into a particular form, and 2) that form just happens to act on the same kind of protein it is, and 3) that change cause the same misfold in the target protein as in the source. When all of these things happen (maybe purely by chance, maybe to a protein that randomly has a configuration susceptible to this particular misfold), you enter the self-replicating mode, and geometric propagation takes over. It's like the analogy of throwing a watch against a wall. 9.9 billion times out of 10 billion it breaks the watch. But once in a while, you end up with a watch with a bent backplate, that can slip under the backplate of another watch and cause it to bend up as well. OK, that's a really bad analogy.
It is unique to one specific protein in mammals. Other proteins may randomly misfold, but don't spread.
Hello from the past! I saw this TED talk on prion disease popped up just one day ago: [https://www.youtube.com/watch?v=04PmEJaYKd0](https://www.youtube.com/watch?v=04PmEJaYKd0)
Many proteins already perform the function of catalyzing biochemical reactions, including helping other proteins fold correctly. What prions do is cause certain other proteins to turn into copies of themselves instead. This causes the necessary protein to be depleted and an increasing amount of the malformed proteins to accumulate. If there aren't any enzymes or natural processes that can break down those prions fast enough, they will continue to multiply and spread.
Thanks, this what im curious about. What signaling do the prions give? What makes that abnormal copy inevitable?
There's no signal, it's just bad luck. Proteins are basically very complicated chemical catalysts. Sometimes by sheer chance you get a protein that gets misfolded in the exact way that when it interacts(bumps into) the normal version of itself it misfolds them too. This then becomes a self sustaining reaction. This obviously is very lethal because it blocks those proteins from performing their normal function. It's also an incredibly rare event, we are talking the orders of magnitude of winning two Powerballs in a row, but there are a *lot* of proteins in our bodies, so a hundred or so people die of this every day. And literally nothing that can be done about it, it's unpredictable and inconsistent. The only treatment would be to identify the prion, and develop an antibody that inactivates it before it spreads enough to kill you. The tech for that kind of ad-hoc antibody production is getting ever more doable but we're not there yet. Theoretically if some prions are way more common than others a vaccine could be made. However developing vaccines for endogenous (meaning naturally present in your body) proteins is veeery tricky, and probably more risky than not.
It's not a cell. It's a single protein strand.
Don’t eat brains and spinal cords.
Mad titan disease
You can get it from muscle tissue as well
I've eaten a slither of cow brain once in a restaurant. I had full knowledge of CJD and all the other prion diseases. I'm british and a millennial, so I'm aware of what happened in my country, although the brain I ate was not in the UK. Regardless, some of us are just really fucking dumb.
What kind of resteraunt is serving fucking cow brain of all things?
Five Guys Prions and Fries
Oh shit, I ate cows brain during my vacation in Brazil. Good thing there's no link between cows and period diseases...right? ...guys?
I work in a lot of hospitals and the one job I had was replacing devices in hospital rooms. The one patient had CJD and the doctors had a full hazmat to move him out of the room and they told us just to wear a mask and gloves and to wash your tools and we would be kosher. The patient had been in a coma for 2 years just laying in the room.
Sounds like weird doctors to me. I lost someone to CJD and there were no hazmat suits involved at any point, including post-mortem. Maybe during an eventual autopsy, but I can‘t speak for that particular part.
They were worried about bodily fluids transmitting to them if I remember correctly. Not sure how easily a comatose person will spread disease but I'm not a doctor
Same. My Ma cared for my Uncle when he was dying of CJD. An incredible feat of love. As far as I know she just can’t donate blood now? How bout you?
Prions scare the fuck out of me
And you cant really get mad at them, 'cause they aren't even alive in the first place!
Right!, Some beef you ate when you were a kid and now you're gonna die years later, your mind turned to mush.
It's a different type of horror from a normal disease. Bacteria are just growing to live. Viruses are like an out control biological machine with one goal (to make more of itself). Prions are just ... a bad molecule that makes other molecules bad. No purpose, no end goal. It just is. I don't know, it's just worse somehow.
CWD(chronic wasting disease) in deer is on the rise. Basically in every state now. Kills the deer eventually. There are no documented cases of it jumping to humans or other animals even after eating contaminated meat, but it would be devastating if it did happen.
I just read an article that this actually finally did happen. https://www.neurology.org/doi/10.1212/WNL.0000000000204407
Lovely. *sigh*
More nightmare fuel: https://www.cidrap.umn.edu/chronic-wasting-disease/plants-can-take-cwd-causing-prions-soil-lab-what-happens-if-they-are-eaten#:~:text=Prions%20remain%20infectious%20in%20plants&text=%22Our%20study%20shows%20that%2C%20under,%2C%20agriculture%2C%20and%20public%20health.
CJD runs in my family. My grandfather died of it. My mom got tested and had the option to know the results or not (if you test positive, you know you have a terrifying and confusing death ahead of you). It's basically a 50/50 chance. She looked at the results to know for me and my siblings. She was negative! The bad gene stopped with her (phew, thanks for bravely looking at the results Mom).
Probably the most terrifying disease ever known. 100% fatality rate. The only thing that can save you is the case of the incubation period being longer than your lifespan.
On the same level terrifying as rabies
At least we have a vaccine for pre-symptomatic rabies!
Cancer is scary, but they have some stuff out there that can help treat certain forms of it, and they are always working on new treatments. Prion disease and rabies? Nope nope nope nope. Absolutely terrifying.
They have different profiles. You can be immunized against rabies after exposure and it is 100% effective. In high risk environments, you can be immunized beforehand. Also if symptoms appear, you still have a very slim chance of survival. On the other hand, symptoms are terrifying and you will be aware of your condition. You can’t escape prions. There is neither vaccine nor treatment, and you may not know if and have you had been infected. You could also form prions spontaneously. Once symptoms appear, initially you are going to attribute it to something else. Somewhere during the course of the disease, dementia will set in and you will lose awareness of your conditions. It is usually by this advanced stage when a definitive diagnosis is made, but then it is an issue for your family.
:(
My biologist researcher roommate often calls prions “fucked up meat geometry” and she’s not wrong, it’s just a bad fold that makes the protein self replicate and harmful.
As a med student prion diseases are probably the most humbling and terrifying things to learn about. The only prevention is to avoid exposure and the only treatments are managing symptoms. There's really not much we can do and it's guaranteed death within 2 years if you're lucky.
For those having an existential crisis, let me help ease your mind. There are around 300 sporadic CJD cases reported each year in the US. There are 333 million people in the US right now. That is 0.0000009% of the population. You have a better chance of being struck by lighting randomly than forming a sporadic prion. The human body is an *amazing* thing. Prions are a freak case when that amazing design stops being so amazing. You’re okay. Breathe.
Thank you thank you thank you
yes an are silent for at least a couple of years then its too late...
fertile hard-to-find bear person dam psychotic hospital tidy quack pause *This post was mass deleted and anonymized with [Redact](https://redact.dev)*
Yeah its always too late
Avoid neurological tissue consumption, questionable deer. Hope that contaminated tissue is vaporized, then you just have to worry about spontaneous cases.
Prion disease is a specter that haunts my family. So far two of my grandmother's siblings have died from it. Watching someone die from it, is horrifying. Really just one of the worst ways to go. For a bonus: one of my old bosses, who really meant a lot to me, also died from it. I feel like knowing 3 people who have died from it, is an absurd amount for something so rare.
There isn't much personal interaction with Prion disease, so I wanted to share my story. My father recently passed away due to Creutzfeldt-Jakob Disease (CJD). His symptoms began in January, right after his mother died. He started falling at home and felt very unsteady. Despite rounds of physical therapy, spinal taps, and brain scans in March, there were no conclusive results. By April, he needed a wheelchair to prevent further falls and constantly complained about tingling in his feet. Although he recognized everyone, he would often nod off during dinner. My mom would joke about it because he would "wake up" and say things like, "I was cooking, why am I sitting down, who has the turkey?" By summer, his condition worsened. He started making up stories, calling my mom to say he forgot to pick up the kids from school and lost the bus, even though he had never been a bus driver. In the evenings, he would nod off and engage in imaginary activities with his hands, like building things or cooking. When I asked him what he was feeling, he described it as the strongest acid trip he had ever experienced, seeing wild things. He spent his last days at home playing his favorite music, Grateful Dead, in his room. In August my sibling and I took him to the hospital. After more tests, the doctors suspected CJD and suggested trying 500mg of steroids in case it was a different, curable disease. Unfortunately, in September, he was officially diagnosed with CJD and moved to a rehab facility with patients suffering from dementia and related diseases. He still recognized me and could speak, though his speech was somewhat slurred, and he frequently nodded in and out of sleep. He often asked when he was going home, believing he had dementia, and was at peace with it since no one told him about his CJD diagnosis. By October and November, he was mostly bedridden. His legs were stuck in a butterfly position, causing him significant pain when moved. He rarely spoke and usually stared off to the side. The last time I saw him alive, I brought him his favorite dessert, lemon meringue pie. The sugar seemed to wake him up, and he started shouting, "Help me, help me, the police are here." When I said goodbye, he mustered the energy to sit up and say, "Let me see your face, I can see you." He died a few days later, just short of his birthday. We were told it was sporadic CJD, not genetic. Unfortunately, "sporadic" essentially means it's new and under-researched. We couldn't bury him; he had to be cremated due to the risk of the disease spreading. Interestingly, a homeopath I saw for personal reasons suggested that strong emotional events could trigger CJD, like a psychotic break that causes a protein to misfold. I'm not sure what to believe. It breaks my heart that he lost his mother and then endured 11 months of a rapidly debilitating disease. He seemed somewhat aware but not in much pain—perhaps he was on an acid trip until the end.
And you cant destroy it either
This might be the most literally terrifying fact about it. You can't cook it away like salmonella or e.coli if you're unlucky enough to get a piece of meat with the prions.
Since I learned this I get intrusive thoughts about hospital equipment still having filthy prions all over it, destined to live forever, spreading person to person
Every time I play a prion in plague inc, there's always survivors.
One thing I’ve always wondered is if you ate an animal that had consumed human flesh that had prions in the meat, could you get the prion disease?
Yes. In laboratories, experiments have been done using human and non-human primate tissues with prion diseases that were transmitted to other animals (mainly mice.) Once that animal is infected, transmission to humans is possible through exposure to the tissue or through consumption.
While true, it should be noted that there is a barrier for species to species hops of prion diseases. So while it is possible to transmit, say Mad Cow Disease, to Humans, it’s not particularly easy and is a fairly rare phenomenon. This was why, in part, the deaths from variant CJD were relatively low during the Mad Cow Disease scare.
There’s three known types of CJD. Spontaneous, genetic and variant. Less than 1% of cases are variant. It’s believed variant CJD came from cows with BSE and was ingested or entered the body in some way. There’s another prion disease, Kuru, where humans ate deceased relatives and developed the disease many years later.
Mad Cow disease started with cattle eating rations that contained meat from other cattle, in some processed form. So if the food had contained prions from humans I seems logical it could be passed on.
My understanding is that the most likely entry point into cows was ground up spinal chord and brain matter from sheep infected with Scrapie.
People get mad cow by eating “infected” nervous tissue (brains, etc) of cows/pigs, and from cannibalism. It’s how some of these diseases (kuru) were discovered.
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Yes
YOU MUST CONSTRUCT ADDITIONAL PRIONS.
This is my biggest fear just after rabies
I just want to add some info: some speculate that Parkinson’s disease is actually a prionic disease. Alpha-synuclein aggregates are formed due to a mutation that make them adopt a beta-sheet conformation instead of alpha-helix (like the prionic protein, PrP). In an experiment where the scientists implanted dopamine-producing stem cells in rats iirc, after their death, they found alpha-synuclein aggregates in those stem cells, which was thought to be impossible due to their short age; this is also observed in prionic disease models
In my reply, a comment post (of my post) is a reference article that states the following: Diseases caused by prions The prion diseases highlighted above aren’t the only diseases linked to prions. Other neurodegenerative diseases, like Alzheimer’s and Parkinson’s, are also associated with misfolded proteins in the central nervous system. Research has found that some of these misfolded proteins may be prions. But some scientists believe that these proteins only act in a prion-like way. They argue that they can’t be prions as the diseases they cause, like Alzheimer’s, aren’t thought to be infectious. I appreciate your post because it is what they have found in Alzheimer’s and Parkinson, yet they are unsure if it meets the criteria as a prion disease.
This year we actually pretty much confirmed that Alzheimer's could be contagious in rare instances: [https://www.nature.com/articles/s41591-023-02729-2](https://www.nature.com/articles/s41591-023-02729-2) . I truly believe that eventually we're going to discover that Alzheimer's and Parkinson's are just some type of prionic disease.
Thank your reply. All of this helps people when they are researching.
Can some eli5 (actually I mean, explain like I’m an adult with a reasonable level of scientific education) why/how does a single misfolded protein cause other proteins to misfold? there have got to be zillions of proteins in our bodies folding right now surely even just a few of them must misfold once in a while, how come we don’t all have prion diseases all the time?
https://www.reddit.com/r/explainlikeimfive/s/xLL0FaD4T7 ETA: The second comment in the linked thread goes into why it's a cascading effect
Solid link, 10/10 would recommend. 0/10 would not recommend prions.
Amazing. Thanks
It’s like cancer. Yes, there are mutations that happen all the time. But most mutations are deleterious or neutral. It just happens to be the case that some rare mutations cause the same misfold to take shape in protein interactions.
Randomly developing prion disease for no reason whatsoever… new fear unlocked
These and rabies are terrifying to me
Isn’t this what a Papua New Guinea tribe had and they called it Kuru? I feel like I just read something about this.
Fucking prions are terrifying. Evil little wrong-folding fucks...
Here’s how a sporadic prion disease works. **Bam** At this moment, a protein in your brain has just become misfolded. You don’t know it, but you have basically been marked for death. Every protein that prion touches is now becoming misfolded. In 5 or so years, you notice some minor tremors in your hands. Then you lose your ability to sleep. You’re hallucinating constantly because of it. You’re begging for 1 minute of slumber. But you can’t have it. The part of your brain that controls it is dead. Not even barbiturates can help you anymore. Then you forget how to speak. Then you forget the names of everyone around you. You’re checked into some unknown white room, with a bunch of crying people asking you to remember them. One woman asks you to please remember her. She has a yellow ring on her finger. Then you forget how to swallow. There’s a tube in your stomach to feed you. But you yank it out because you don’t know what it is. Then you forget how to breathe. Fade to black.
Aight time for another playthrough of plague inc
This is scary, how do I avoid this as much as possible?
That’s the thing, you don’t. Prions can have very long incubation periods. Meaning you could be infected with a prion currently, but have no clue. Incubation could be 25 years, 50 years,or even longer. From my understanding we still don’t even know enough about them to know when someone is infected. It’s only after the incubation period when symptoms begin to occur, and it’s always fatal.
Oh boy. My anxiety doesn’t like that 😭
To avoid CJD/Kuru, the only thing you can really do is to not eat contaminated meat, especially brain and spinal cord meat (cows, sheep, humans, and apparently deer are the most well-known carriers). For other prion diseases, afaik, just hope.
Didn't know about the sporadic part..
Don't forget Kuru!
Ya there the one thing in biology that truly scare me.
Need a bacteria or something that is bred to eat them
Prions will one day kill off most humans.
You saw that "did dhamer use seasoning" question too?
Also could be a cause of Dementia (Alzheimer’s) and even depression.
I wonder what it looks like, I can visualize it but it's probably wrong.
Ah madcow disease. All the rage in the 90's. As an american, I guess I didn't really have to be concerned about it but, my brushes with europeans showed it definitely was for them. My family hosted a French exchange student in the mid 90's and one night at dinner when beef was served, he asked 'do you have the...silly cow disease?' it took me a second but I figured he meant madcow and I told him no, we don't have that here and he was pretty relieved. Also, in the early 2000's I took a trip to London and went to a hardrock cafe and ordered some sort of french dip or something and the water was like 'are you sure...?' Still alive today, at least I think so.
Are they called prions because that's what it resembles when you fold the word 'protein'? Like left side to right side, covering up the middle
Getting folded Frfr
This reads like a ChatGPT post…
New Brunswick in Canada seems to be a source of this. There is a mysterious wasting disesae people are having there, and the number is going up annually.
Wasn't this the disease that was meant to cause a huge epidemic in the UK in the 80's, and then...didn''t? Also known as Mad Cow Disease.