10:42 If what you said is true, that a pathogen is an organism which causes disease, then a prion cannot be a pathogen by virtue of not being an organism. That's a category error.
My microbiology professor in college taught us to never, ever, eat any type of brain or nervous system tissue, including eyeballs, of any animal, because of the risk for prion disease. This stuck with me. As a hospice nurse, I've taken care of a few CJD patients, and its definitely not a pleasant way to exit this world, and it is heartbreaking for families to see their loved one deteriorate and then taken so swiftly from this disease.
I have heard of certain areas where calf brain or monkey brain is a delicacy. I used to think I wouldn't mind trying it as long as it was prepared by someone known for making the dish well, but after learning about prion diseases I would have to decline the opportunity. Fortunately people aren't especially into eating brains in the US so I should be safe, for now.
As someone who was diagnosed with CJD at 14 thank you for making this video. I have gotten super tired of explaining what it is and what causes it. I get ekg, mri, and ct scans every 3 months to keep track of the holes and deterioration of my brain tissues. I suffer from gran mal seizures due to it. Im 28 now and my neurologist says by 50-55 I will enter the end stage of my life Im on many many meds to help slow the process, and to extend my life to live as fully as possible. So just know that the life expectancy isn't necessarily a rapid death. Hopefully by the then we will have a cure but if not, at least hopefully there will be better meds
Woah man I’m so sorry to hear that. At this point I’d assume you have accepted that fact. May I ask what happened at 14 to get the diagnoses? Symptoms?
Oh man what a horrible thing to have, I also have some mental issues that will follow me and burden me throughout my life but that's a whole other level, I feel so sorry for that man. On another note it is predicted that the deer wasting disease could jump to humans and if that happens a few hundred people will die and that's all horrible of course but the positive thing that could come out of it is that we finally figure out how to treat these kinds of diseases. Also, with the rapid progression of science the last 20-30 years, the next 20-30 years will probably be as groundbreaking as the past ones if not even more if technology keeps progressing in an accelerated pace like it has so far. To put it simply, if you were to have cjd no matter what, nowadays would be the best time to have it, if you get what I'm saying.
@@ildisiri The speed of our scientific advancements is in part thankfully to the computers that help us analyze it. Right now, until we find out a way to make a quantum computer (which could spell apocalypse, depending on who invents it first) we are bound by the laws of physics. The smallest possible transistor (made of 14 atoms if I recall well from my IT prof lectures) has already been made, but is not in wide use, so technology will go ahead for a couple of years, but then the development of computers will... well, kinda stop. That being said cloud computing etc will still let us make great advancements, just... don't expect the avalanche-like speed of that development to continue. Also, as a person with mental issues I get ya. I'm trying to build my life now, after 25 years of trauma and bad psychiatrists. I found a good psychiatrist, though, so it should be better. I'm sorry for the OP too, it's horrible, but... We must try to make the best of it while we can.
@@AK-fr5zv: "a quantum computer (which could spell apocalypse)" I'd like to think it's our ticket to free our conscience from the physical world, to upload the consciousness of humanity into the cloud as weaponized prions spell armageddon to everything left running on neurologic wetware.
@@mfaizsyahmi I mean, yeah, with enough storage, we absolutely could do that, conceptual question is: is it still you, or is it just a copy of your consciousness. Or do you think we could somehow link up to machines to instantly transfer any and all data held within our brains, thus, perhaps, preserving the consciousness of the living being into the cloud? I mean, I thought about that a lot, too. And, again, it all depends on who discovers quantum computering first, and how they use it.
There's also an entire system of "chaperone" proteins that are designed to catch mis-folded proteins and either re-fold them correctly or destroy them, which is probably why spontaneous CJD is fairly rare.
Saunas apparently increase the number of chaperone proteins. They also reduce "all-cause mortality". I wonder if there's a link. Anyway, I'm heading straight to the fucking Sauna shop.
@@sendmorerum8241 it sounds more like an excellent start for a cure. I’m guessing there are already labs all over the world studying this exact process so they can formulate a cure.
But despite the presence of chaperone proteins, study is still ongoing how to break down the beta sheet-laden structure of prions (which makes them virtually unkillable by proteolysis or radiation) and eventually, destroying prions without subjecting to 900°F sustained heat for 6-8 hours
I had a lecturer at university who worked with the National CJD Research and Surveillance Unit. I remember her telling my class why CJD surveillance is so vital, they suspect that the mad cow outbreak has resulted in a lot more human deaths than have been reported. Since prion diseases tend to require an incubation period of decades before an individual becomes symptomatic, they suspect that mad cow disease resulted in a number CJD cases that are currently emerging in the elderly population and that these cases could easily be misdiagnosed as some form of dementia since both these conditions have very similar symptoms.
My father died of Prion disease a little more than a decade ago. He travelled extensively, including to England, many times, during the mad-cow disease time frame. I asked whether that could have been connected, and no one has ever given me a definitive answer, as it 'could be' but could also be so many other answers, and there's really no way to tell.
@@JesterMotley i was a nursing student in Cleveland Ohio 25 years ago when I briefly cared for a cjd patient. He was a vp of a steel firm with business in England. It was suspected he had the beef. His demise was fairly rapid. He went at first to a psychologist then psychiatrist but within 2 months was hospitalized unable to speak or react to nearly anything. He died a month later. With the exception of 1 nurse who had a patient earlier in her long career with cjd, he baffled the entire medical staff. It is quite plausible for your family and many others to be misdiagnosed simply due to the lack of exposure most of medical community has to proins
@@walterwarren2402 While possible to misdiagnose while alive, a post-mortem comprehensive survey and testing of his brain was definitive and confirmed 100% the diagnosis, so no, its not plausible for myself and my family. But others might indeed be misdiagnosed. Sadly, the protocols and treatment for rapid dementia are what they did for my father, so even if there was a misdaignosis of cjd... nothing would have changed for him, or us.
@@nikkiespinosa8854 I'm not a doc, but given my family history we've... reviewed this extensibly. So take that for what its worth. Bollocks. That said? We are, all of us, routinely, 'infected' by prions, and we handle it. But we deal with it, because the human body is very resistive, in the end.. not perfect, but very resistive.
My uncle died from Creutzfeldt-Jakob Disease about a year and a half ago. One day he noticed he was having vision trouble. A week later, at which point he was already having trouble following conversations, he got the CJD diagnosis and given one month to one year to live. Alas, he didn't make it that long; two weeks after that, he passed. It was terrifying and surreal. If nothing else, the fact that it took him so quickly was a blessing, as he undoubtedly would have only suffered more had he lingered.
I've noted in other comments, my father passed away from a Prion disease. It took him 7+ years to pass (the family argues when he first started having/showing symptoms, but it was diagnosed 7 years before his death). Watching my father deteriorate year after year is... I can't put to words what it was like, but I can say, for absolute certainty, that your Uncle, and all of your family, really were blessed it took him so fast... Its a horrible thing to say, a horrible thing to think about, but I lived through the alternative, and I wouldn't wish that on my worst enemy. It's so insidious and cruel to everyone... he knew he was losing his mind, his life, we had to watch the slow crawl of him losing them. Just... yeah, your uncle was blessed to go so fast.
@@JesterMotley thanks for sharing that. I'm sorry that your father had to endure so much pain, and I hope both he and you have gained some measure of peace now that it's over. Prion diseases are well and truly terrifying. Hopefully we find a cure or even a treatment for them soon.
@@Thexaios Thank you for your kind words. I've had some time to find peace with it. I was, and still am, hoping my words might help you and yours find peace in what happened to your Uncle, and that you are right in your belief it was a blessing to go so fast. I truly understand what you and your family went through, and I hope what I've said can help even in the slightest. That was, and remains, my only reason for speaking up.
I won't lie, dementia and other diseases that cause demetia symptoms terrify the F out of me; I'd take myself out, it's not worth it. Sorry for your guys' losses.
@@John-Perry Hey mate... I understand. Watching my mother, and brothers, deal with a father who everyday was... less... was devastating. I can... support... if you will, the idea that its better to pass sooner, even if by ones own hand, than later in a state you can no longer rationally make that decision. But I hope you, or anyone else, never has to go there or anywhere near it. Peace and love.
You touched on cannibalism. Here is an interesting fact; "...some of the people who survived the kuru epidemic carry a genetic mutation called V127, whereas those who developed kuru did not have this mutation. This led the researchers to suspect that V127 conferred protection against the disease."
That is a common thing with infections, be it bacteria, viruses, parasites, or even prions. After a while the people either develop some resistance to the disease, or the infecting agent mutates into a less aggressive form. For example the 1918 "spanish" flu epidemic was Influenza A subtype H1N1, a type that is still around, but we don't see dozens of millions dying every year. And after every bubonic plague wave the population was much more resistant and it took multiple generations for the resistance to go down and the next wave to hit. Or all the diseases Europeans brought into the Americas that totally ravaged among the native population, but were rather tame across colonists. And SIV in chimpanzees doesn't cause the same, massive symptoms as HIV does in humans, they have already adopted and found a balance that keeps both, the apes and the virus around. No doubt humans will develop in the same way over the next couple centuries. Or how the Omicron variant of SARS-CoV2 has a much higher transmissbility and reinfection rate, but much less hospitalization and mortality than earlier strains. We're already moving away from the super dangerous pathogen and towards something that will stay around, but isn't a big deal.
I use to work in an hospital pathology lab, a patient w/CJD died and the pathologist said absolutely no autopsy, even though one was requested. This was circa 1999.
@MementoMori Multiple people have died from accidents working with prion infected tissue. A simple needle stick is fatal. It requires special equipment and training to minimize the risks and it is still extremely dangerous. Such an autopsy should be done at a facility where everyone is used to that level of risk and thoroughly prepared for it. Trying to do a one-off procedure that dangerous is asking for trouble.
@MementoMori If penguinista's response didn't make sense, you can be infected by prions from another source and as Simon mentioned there isn't a treatment and it is fatal. If there was not a strong reason to do so, there no reason to take the risk of performing an autopsy; the person's death is most likely from prions or complications from prion disease.
@@penguinista you don’t understand how pathological autopsies work. Even the most infectious corpse will have an autopsy done in a normal morgue. They just put in stronger regulations. Your point doesn’t make sense because some deadly pathogens aren’t even discovered until they do an internal inspection of a corpse. Tox results can take weeks even months to come back to the pathologist
I like how, although ritual cannibalism is something absolutely forbidden in Western society (for many very good reasons, not to mention kuru itself), you take the time to explain the Fore people's thought process behind it rather than sensationalizing it as "savagery."
As a deer hunter who's seen the rapid spread of CWD.... my cousin summed it up the best: When asked is CDW meat safe to eat, "Well, technically yes... but I don't want my daughter to be the first jump over case." This was about 6 years ago, before COVID. Prions are terrifying.
My compliments! About a year ago, I was at a barbecue where a man brought venison to share with everyone. I said, no thanks. I went vegetarian that day, the only one. Didn’t I like venison? Yes, I love it, but wasn’t going to eat it because of CWD. The man and his wife got upset with me, cut me off, and told me there was absolutely NO evidence of human infection, was I conspiracy theorist? If I’m going to ruin the barbecue, I could just leave. Realizing nobody would listen even if I did warn everyone, I stayed for while and excused myself early. Just as in the UK, nobody WANTED to know or even consider it. Keep your eyes open in the US among consumers of deer, elk, moose, etc.
@@mariekatherine5238 Yeah, not until we know more about it. There’s no knowledge that it can yet, but…well, assuming that BSE couldn’t naturally cross the barrier because scrapie couldn’t was what led to problems. (BSE does seem to be the odd man out here so far since it pretty readily hopped to humans, cats, oryx, bison, gemsbok and other exotic hoofed mammals…whereas the only time a non-sheep caught scrapie was in a lab.) Still…never go into it not knowing.
My father-in-law passed from CJD. I've never been so scared in my life as when watching him progress through it. He died a mere 46 days after diagnosis, but hindsight revealed we'd missed obvious symptoms months earlier. He was such a strong willed, focused man. Seeing the slow-yet-fast, unstoppable descent into dementia was traumatic. The only positives I've taken from the experience were that he died relatively painlessly, and that I now know how much he meant to me.
There are also proteins that are labeled “prion like” such as beta amyloid, tau, and TDP-43 that could be responsible for aspects of the pathogenesis of neurodegenerative diseases.
Yet nobody dares to admit this, because this is going to cause a lot of problems in the health system. Imagine screening everyone suspect for a neurological disease to avoid transmitting it to others.
@koekoek It has absolutely nothing to do with the SARS-CoV-2 vaccines. We don’t have a endogenous (originating from humans) spike protein so the spike protein from the vaccine can’t act as a prion. Second, the spike protein from the vaccine is almost identical to the one from the virus, with just minor amino acid changes to improve its stability. If the virus doesn’t cause prion disease then why would the vaccine. There is no biological plausibility to think the vaccine can cause prion disease. Anyone who has told you otherwise made it up, like the countless other stupid claims about the vaccine that were false like have luciferase (which isn’t even bad and would be useless in the vaccine since it mostly used as a research tool not medicine) or claims that changes your DNA (it doesn’t).
There's a small but very important misread Simon made in this video. He said "about one to two million people are diagnosed every year". It's actually one to two *per* million people in the notes onscreen. So if it makes you feel any better, prion diseases are much, much rarer than the video (accidentally) suggests. One reason why so much is unknown about prion diseases at present is that they're extremely difficult to study due to the lack of available subjects.
He didn’t even get into the fact that deer get chronic wasting disease yet don’t regularly eat brain meaning that chronic wasting disease is incredibly contagious within the deer population, so if it ever spreads to people it will probably be around as contagious. And people thought Covid was bad. Deer have been known to eat the heads of baby birds for calcium ect that might be how they get it or got it.
Simon, ive been a fan for a while now. You always bring quality content. Not to mention your sheer willpower in keeping up with so many channels. Keep up the good work!!
@@hiigara1 more likely with the fact that simon most likely "just" films the video itself and sometimes write/co-writes the script and there are people for everything else
“The Family that Couldn’t Sleep” is an excellent and very easy-to-understand read for anyone looking to know more about prions and the history of its study. I listened to it a few months before watching this episode, and I loved it
While an anthropology student in the mid 80's we did a deep dive in the Fore (Fo' rei/ For ā) and Kuru. My professor worked with tribe in 60's and had firsthand knowledge. One detail not mentioned was the chance observation of Allen while at a seminar on Scrapies. Scrapies is prevalent in sheep and he noticed the similarities in brain tissues which put him on the track of transmission since environmental factors had been studied with no results so it had to be cultural ie some practice that is the cause. Bear with me as my Medical Anthropology was almost 40 years ago. Damn I'm old.
@@wendychavez5348 not to mention terrifying. Every now and then here in the states, someone goes swimming in warm water in the southeast, or once, in Colorado, and they wind up with that one that winds up up your nose and bam, they're dead in days to weeks. Reminds me not to swim in ponds where I live when the temperature gets high.
@@MegaKat Ah yeah, good ole en.wikipedia.org/wiki/Naegleria_fowleri - Gotta say, regardless of medical accuracy of the show, I'd never heard of the little bastards until an episode of "House M.D." - makes me glad I don't swim.
@@WindKing0 yes, that's the name of it, thank you, I was having a brain fart on my reply and google was being a pain. That shit ain't nothing to fuck with; do NOT swim in warm water that isn't a treated pool!
Chronic wasting disease happens in deer elk and moose. If you see them walking in circles they are affected and need to be put down. Contact your local fish and wildlife if you see them.
Not just walking around in circles, they also slam their head against rocks (my buddy saw that first hand) and casually come up to humans, granted the latter could be multiple different reasons. But in the end, like you said, they need to be put down immediately. God forbid it spreads to other deer or worse, to a human who may eat it
It's a good thing I carry my dope .40 cal hi-point with the 100 dollar bill finish. Drop that moose with my glawk whenever I see one bussin fr fr. It's gon be lit dog
Thankfully it hasn't been known to spread to humans, but we definitely don't want to take the chance! On top of this the way it spreads so well is because it can survive for a very long time outside of deer.
As for why prions do what they do, there probably isn't an answer beyond accident. Sometimes, when things randomly go wrong, the consequences can seem bigger than they should, but that doesn't imply any intent, it's just how randomness works. The fact that proteins that are folded in a particular way will disrupt other proteins and thus start a chain reaction isn't particularly surprising. There are many instances of natural processes that, through random movement, are self-sustaining. A recent example that has been in the news is xenobots.
Based on my (admittedly only ‘okay’) understanding of biochemistry that’s probably the case. Protein structure relies on chemical bonding and the more stable the bonds the more difficult it is to break that up. Prions may just be this…extremely stable structure so they’re basically chemically inert. The amino acids link together, then the chain ‘folds’ usually via hydrogen bonds, then side chains link up to make a 3D structure and then multiple folded chains link up… So somehow the chemical reaction went an odd direction and it was stable. VERY stable.
Love your content Simon. My father passed from sporadic CJD on May 6, 1996. He started becoming symptomatic in the summer of 1995. He was from the rural United States. One of the members of my family started a website for family members . A page dedicated to people who needed support for dealing with the grief of losing their family members to this disease. There were hundreds of cases in the US in 97 over 50 in Texas alone. The CDC sent out a lot of misinformation and down played it's impact to the food in the United States. The numbers the CDC sent out were a fabrication, I personally called them in 1996 . The CDC said it was a non reportable illness and there was no CJD problem in the US , and he had to have went to the UK. I am 52 years old , my father died at 57. I am slightly concerned. Thank you for your work. BTW Deadly Feasts by Richard Rhodes is a little dated , but a great and terrifying read.
Very high doses of “protein cleavers” are showing promising in treating these malignant variants. Such as bromelain. It’s interesting how some of these enzymes have been overlooked for years. Bromelain has been used to break down the spike proteins of covid19. Nattokinase is a very strong fibrin cleaver that is used in Japan for thrombosis.
does that mean that pineapple, kiwi fruit, and pawpaw could all be used to treat it? or are we talking higher levels of bromelain and other related chemicals than found naturally?
@@bronhallerPresumably higher levels than would be found naturally. It’s my understanding that prions are quite stable, they won’t be inclined to react with just anything, so you might need some very concentrated enzymes to do the trick…
Thank you for this video, and for the manner and method you delivered it. This hit home to me. My father passed away due to a Prion disease a bit more than a decade ago. It took me several days to work up the gumption to watch this, much less comment, as I hope are self-evident reasons. And I was relieved, though unsurprised, that you handled it to the usual respectful, intelligent, and brilliant standards I've come to expect of this channel. Anyway, just wanted to say a thank you for doing this, and to your writers and staff for treating it well. My father, as a scientist, would have applauded your efforts, too.
Great video. Sadly, GSS is in my family and my middle sister is rapidly nearing the end of her life. My paternal grandmother, dad, his brother and both my brothers have all died from it. Also a few other nieces, nephews and cousins. Indiana University School of Neurology has done a genealogy back to Europe. I was tested when my kids were in high school and thank, goodness I was negative so my kids cannot get it. It is a terrible disease. Thank you for posting this video. Genetic diseases suck.
@@sendmorerum8241 Well considering judith does not look like a spring chicken from her profile picture, I would image the test did not exist, or at least not widely available or cheap enough to consider.
@@joelhernstrom6060 How it is antinatalist? What she did created unnecessary terror to experience for at least a decade! That is a bit too much, seeing your relatives then look at your children and imagine them in the same condition, knowing you are responsible. Knowing that one day, you have to tell them about this sickness. And no, it doesn't matter if you didn't want to pass on this condition. Thinking it is something outside of her control is nothing more than dodging responsibility, the good ol' "it's God's/nature's will". You're not obliged to have children, not matter how much gratification you get from having them.
I watched a co-worker wither and die within 14 months of his retirement from prion infection, squirrel brains was the suspected source for him. Awful sight to see a healthy man turn into a husk almost overnight! Scary indeed
@@drabnail777 I’ve seen you comment under all these. I’m now going to spread your IP. Good luck buddy. Learn to shut up. Can save your families life one day ;)
Okay, here's my question. How the hell thought it was a good idea to feed cattle other cattle? Cattle are herbivores. No good comes of trying to change such a fundamental element of an animal's life. Would we try to make lions herbivores? No, so why do it the other way to cattle? That was never going to end well.
Herbivores will often eat meat if the opportunity arises, especially in biomes with scarce plant growth. I'm not at all justifying feeding cattle to themselves - the consequences are manifest in this video. But herbivores' diets are not quite as fundamental as people think.
like the other reply said herbivores do eat meat occasionally in nature, and this feeding cattle to other cattle WAS effective at helping them grow, it became problematic when cattle who had scrapie were fed to other cattle with the assumption that it wouldn't spread, which it did. the whole process does sound messed up and "unnatural" but I don't think it inherently is, and I think we shouldn't dismiss this stuff just because it seems that way at first glance. idk I think utilizing the waste products of butchered animals (bones and lower grade meat) is a good thing, we've been doing this with chickens for a while now, using their remains for stuff like chicken nuggets or fertilizer from their bones etc, utilizing the parts of cattle that would otherwise be thrown away by feeding them to cattle who benefit from it isn't such a bad idea on its own imo
Of obviously, this was a mistake, but feeding animals (or humans) diets with slight alterations from what they might encounter in nature can bring a lot of good, if it means avoiding nutritional deficiencies. Hell, just giving animals stable diets in the first place is a change from nature.
My son's godmother died of Creutzfeldt-Jakob disease. One day she was fine. Then she started having coordination issues and slurred speech. Then she became unable to talk or walk, and finally descended into a vegetative state before she died. All this happened within a month.
Literally prions and Tau proteins are done of the freakiest things but I think once we fully learn about them we will greatly improve neurodegenerative illnesses. My grandma just passed from a rare unknown brain dementia that hit out of no where for her last years of life after being sharp all her life. We still have no clue what happened to her and it bothers me and really makes me want to study brain degenerative Illnesses.
Small correction here, prions actually can be pretty reliably destroyed by heat, however the temperatures required are hotter than even autoclaves get. Sustained heat at around 900 degrees Fahrenheit (about 483 degrees Celsius) will reliably destroy a prion to the point of no longer being infectious. For obvious reasons though, meat isn't cooked to anywhere even close to that temperature, as it destroys all the proteins in the meat, to the point of being left with essentially a brick of charcoal. But, the point remains that heat can destroy prions, just not at a temperature anywhere close that most people can even produce in their house, let alone heat contaminated meat to
I saw a rather unscientific experiment on tv a few years ago where they heated scrappie from sheep to temperatures that nearly reduced it to ash, buried it in a rose garden for 2 years, and samples of the dirt could still infect sheep who ate it with scrappie.... Scary stuff...
@@Galen-864 prions can't be "killed" because they aren't alive. they are proteins. proteins are carbon hydrates. carbon hydrates can be burned if you bring enough heat and oxygen. if you ever did any research on the subject you'd know that the standard procedure for mad cow disease is to incinerate the carcass, they wouldn't bother doing that if the heat wouldn't get rid of it.
Aww that's sad. I'm fascinated with legionella and did a presentation on it for my pathophys class this summer, but I had no idea about that part of Dr. Thacker's history. What a shame
In high school (before Dr. Thacker died), I shadowed one of his colleagues who discovered the link between Legionnaires and hospital water systems and it was beyond cool to hear stories about their discoveries. Both of them, along with learning about epidemiology at a young age, inspired me to get my degree in public health. I work more on the education side now, but I’m still an epidemiology nerd and a trained contact tracer.
@@nlc43 If you feel like it, would you be down to elaborate about your work and the niche you're in now? I'm a public health nerd myself and particularly I'm enamored with the relationship between patient behaviors/adherence/outcomes and the variation in medical epistemology across different social strata (I'm nuts about all things upstreamist really, but especially the epistemology part). So I'd love to know more about the education work you do and what you could share about the state of the public health scene at large with regard to your work and the work you see being done and foresee coming in the future. I'm well-networked and experienced in nichey weird ways (specifically I'm chronically homeless and do a lot informally on the ground educating and referring out people I encounter in the unhoused community, since for some reason people are quick to ask me for advice despite the cursory and eclectic nature of my actual medical experience) ... but even with recently finally going back to school, overall I just don't have many feelers out in the sphere of Normal Employed People Who Are Educated Formally And Doing Work That's Much Better Defined Than Mine lol, so I love to gather insight where I can! If you don't have the time or otherwise don't want to expound that's also totally cool of course 💚 Thanks for the work you do!
CJD is no joke. Last year a priest in my diocese had to retire early and move out of state after he was diagnosed with it. Barely a month later, he was dead.
Yep, my microbiology professor in college warned us against consuming any type of nervous system tissue, including eyeballs. When we dissected cow eyeballs for anatomy class, we had to wear full PPE, including eye protection also for this reason. Scared the hell out of me back then. I've now seen people die from this as a hospice nurse, just confirms that it still rightly scares the hell out of me
You can get it from consuming pituitary gland. World found that out the hard way when using pituitary glands from cadavers for fertility treatments in the 90s
I've always likened prion diseases to the neural version of false vacuum decay of the universe. It takes just one particle to seed the reaction, and the reaction is unstoppable and total.
I always thought these diseases were a thing of the past or extremely rare, but now I know just how dangerous it can truly be, especially with companies and governments that don't care about the people it rules over.
Prions were what put me off of biology when I was at school. You would think disections would be the cause but no, prions, a disease with a 100% mortality rate and it is just fortunate its very rare.
My great-grandpa died of CJD. I never met him, but my mom said it was a very rapid decline. My grandpa on the other side really liked to eat cow-brains. He never got sick from them, but it's kinda freaky and gross thinking about it.
Always love the videos Simon, and there's so many channels to choose from! I think an interesting topic for a future video could be forever chemicals, like PFOA and PFOS, which are everywhere, don't naturally break down, are extremely difficult to remove during drinking water treatment and are known to cause cancer and multi other diseases. They have been around since World War 2 and became big in the 60s. They were largely unregulated and unknown about (by authorities, the companies using them knew what they did but covered it up) until the 2000s and are now found in the blood of almost every human on the planet. I think they're in equal parts terrifying and fascinating, and the fact they're found in so much of our everyday lives makes them a particular challenge
CWD is transmissable through saliva too, this is why white tail deer who are social deer are more affected by CWD than other deer types that are less social like Elk. This is a big reason not to bait deer.
Prions, like a fair amount of genetic illnesses, are one of those things that doctors look at in theory, but most people will never see... Thankfully because the outbreak in England effectively destroyed their beef economy for a decade or more. There is a lot of things like that that keep doctors and anyone else with an interest in public health awake at night. I don't think I've ever seen it, but have you done a episode on the sin nombre virus? Proper name Hantavirus, identified in 1993 and having killed just shy of a thousand people in nearly 30 years, for those of us in the western US it is always a concern.
Chronic Wasting Disease, which is caused by a prion, is endemic in deer populations in the eastern US. We will be seeing more and more of it as time goes on, unfortunately..
I recently read Cold Plague by Daniel Kalla. It's fiction and involves a lot of espionage and intrigue in the French countryside, but involves a lot of interesting science too, and prions are qt the centre of it all!
Fatal familial insomnia would be an absolutely brutal way to go. Weaponizing that would be a horrific act of inhumanity. Imagine a country full of people who are incapable of sleeping for months at a time, and have succumbed to total and utter madness as a result. This is the zombie apocalypse.
The symptom onset changes according to how those proteins are introduced to the body. Maybe if they’re introduced from the outside insomnia won’t be the first symptom.
At least with most diseases you can sleep through the symptoms or fall into a coma... Not with that. Sounds like the worst thing to die from next to rabies.
I haven't heard the term "Mink Transmissible Encephalopathy" in years. Once knew of a farmer who lost some of their mink to MTE. I remember my dad teaching a new-hire on the mink farm to watch for the symptoms of MTE.
The Kuru outbreak is where most movies got their idea that eating human would cause you to get ticks and act crazy, but didn't really know that it was actually just from a prion.
I lived in the UK in the 80's. As a result I can not donate blood in my home country of Canada. Neither of my parents are suffering from "mad cow" disease fortunately. I can still donate my organs upon death.
Years ago we OR staff were discussing Prions and how we would have to deal with the possibility of performing a craniotomy or biopsy on such an individual. I have dealt with VRE, MRSA, necrotizing fasciitis (great video as well) but prions scared the living hell out of me. Since sterilization could not denature it, any instrumentation would need to be treated as an extreme biohazard and destroyed as it could not be reused. Scary stuff.
honestly I was surprised you didn't mention this, and it might not be online anymore, but there's people who have covered it: there was a guy who stopped being able to sleep, and started getting really sick, but doctors couldn't tell him what was wrong I think? so he just started making videos to document his decline. Its horrifying, terrifying. He goes from this intelligent, well-spoken man who's frustrated with his medical treatment, to a religious lunatic raving about the apocalypse, to finally being completely incoherent, where you couldn't even really understand what words he was trying to say. It's so sad, so scary, but also painfully real. He didn't want to be forgotten just because he couldn't afford treatment. It's a really heartbreaking story :(
if you search "man documents fatal insomnia mental breakdown" its the first video that pops up, by ScareTheatre. The second video that pops up is the original guys' channel, actually. I just searched this myself. I'm kind of surprised its still up. His name was Richard Siagian. RIP.
I haven’t watched the vid yet, but seeing the topic being prions reminds me of the level I’m on in Plague Inc. Crossing my fingers that no new plagues are gonna start irl, but this is Into the Shadows, after all.
This video was excellent…. I’ve been reading recently in New Scientist about some ideas around the possibility that certain forms of dementia are the cause of prions, not the result… that’s all very much up in the air at the moment…. However, to cover so many aspects of this topic in a 17 minute video is very impressive! I enjoyed this video immensely 👏👏👏👏
My Uncle was diagnosed with CJD just after New Years... Today he has to be moved into hospice. This is the worst disease in the world & i pray that one day nobody else has to go thru this.😢
Just the word "Prion" is enough to give me chills and fill me with dread. Allow me to point to the album 'vCJD' for one of the most terrifying listens. It's an Everywhere At The End of Time inspired album but with CJD.
i think the infectious misfolding occurs due to prions having multiple low energy states (think false vaccum from quantum physics) and basicly the infection state could be the more desired energy state.
Ooh, yes, I remember that episode! It was actually pretty neat; I remember the Mad Cow outbreak in the UK back when. We'd visited family there for around a month or so back in 1981, so, yeah. I know the concern was it passing to humans, of course; but I don't remember the proper name for it (CJD) being used back then, and now I've been fascinated by it ever since that episode. Plus it taught me how to pronounce it, lol! I also remember 2003 when a cow was found with it in Alberta and the US closed its border to Canadian meat for like a year-- same year as the drought out here in western Canada; that was a rough year for farmers. Suddenly not only could they not grow any hay for their animals, but they also were stuck with all these *extra* animals, and no income! Farmers who previously could feed their animals over the winter for the price of the gas to run the tractors to bring in the hay were suddenly stuck looking at tens of thousands of dollars in feed; I remember one farmer saying she was looking at going from her own free hay to at least $30,000 to feed them over the winter. That was the year of the wildfires too-- well, the first one, anyways. :/ I was involved locally up in the Interior of BC in cattle ranching country volunteering for Noah's Wish and helping to evacuate and house animals-- from house-pets to livestock-- and a farmer I visited who was helping to house evacuated cattle showed me what was supposed to be his hay field. Looked like an abandoned lot with some scraggly weeds in it. Said it would cost him more in the gas to bring in what little was there than to just buy it elsewhere. He was just going to leave it; figured there was only around twelve hay bales there, tops. So he couldn't afford to feed his cattle, not even the reduced herd he'd usually have after selling his cows, because without being able to sell them, he had no income for the year, of course. And in BC (and presumably elsewhere in Canada) any cow you aren't just eating yourself *must* be butchered down at the government slaughterhouse (which is how they can test every single cow that enters our food supply, which is how they found the one in Alberta in the first place). But that costs $250 per animal, and with the US border shut, the price per cow dropped from $800-1000 (depending on whether or not the animal had been branded, which reduces the value of the leather-- but helps to prevent cattle rustling! Which at a profit of close to a grand per cow stolen is absolutely a thing still, it ends up) down to $200-- which meant a net *loss* of fifty bucks per cow butchered. :( He said the only way he could have managed was if he had been allowed to butcher them himself and sell it on the roadside (something you see a fair bit in our fruit-growing areas out here-- with fruit, of course), but as it was, all he could do was to dig a big hole, shoot them, and shovel them in. But the thing is, that after that whole big scare we'd all had with Mad Cow in the UK a decade and a half or so before, Canada changed its own regulations too, and is now *obsessive* about testing for it-- and for excellent reason, apparently!! Every single cow that enters the public food supply (i.e. anything other than what a farmer might slaughter and dress for his own direct use; I don't believe they're even allowed to give the meat away) *must* go through the government slaughterhouses, as I said, and every single animal is tested for Mad Cow. If one is found, it, its herd, and any other cow that it was in contact with in its journey there are all pulled from the supply chain and incinerated, and the farmers are paid full market value for each animal they lose, so there is zero financial incentive for them to hide any instances of it. Any meat processed by the slaughterhouse or butcher since that animal came in is also pulled and destroyed. Every cow in Canada has its own ear tag, so they can be tracked all the way through, for precisely this reason. --- Which meant that we were all pretty pissed off with the US for closing the border for that year, because our food supply is *safe,* and actually much safer than theirs! People up here thought (and I agree) that their government at the time -- Dubya, iirc-- was just taking the excuse to basically outlaw imports and give their own farmers a boost, rather than looking at it scientifically. Which, understandable, I guess? But it screwed us big time, and under NAFTA that kind of protectionism is outlawed. :/ Anyways, TL;dr: Canada took the UK's unfortunate lesson to heart and ever since has been extremely proactive about keeping it out of our own meat supply. Plus I remember that episode too! :D
Been wondering how this channel was going to incorporate sponsor messages. Good choice leading with it. Better choice would have been at the end, "and if you can't wait for a prion to destroy your brain, you can melt it faster with RAID: SHADOW LEGENDS!"
I knew a woman who died of CJD. She was in her late 50's, still working and a few years away from retirement. One day, she just didn't show up for work and she never came back. She was dead a few months later. It was heartbreaking for everyone who knew her. I hope the researchers will discover a cure for this terrible disease.
An interesting thought is that with how long it takes between the introduction of prions and the development of prion disease, up to decades in some cases, there are very likely people who will live their entire lives with prions multiplying in them, without even realizing it and end up dying of something entirely unrelated. Which means the number of people infected during outbreaks could very well be much higher than reported. Since there are bound to be people who die of other causes before they can develop prion disease. And I for one say they're lucky as hell. As ironic as it is to call a dead person lucky. But that's nature for you. Never fits into our tidy little idea of this planet being the perfect safe haven for life and humanity. Especially when you consider everything else in this world that's either trying to kill us, or make us suffer, or both.
They may be lucky by themselves, but some people who have been influenced by them may be not. What about if they were blood donors, sperm donors, or eventually organ donors? what if they have been surgically operated upon without the knowledge of the medical staff about their infection?
And to think, those of us from britain who grew up during the BSE scandal could be just waiting for those slowly accumulating prions to take hold and kill us, kind of an awful thought really... :(
@@StromSports For one brief moment, I thought you were Amy Macdonald's hub, same name, wrong person though... :P But yeah, not a fun thought knowing that I was born in '85, and possibly could have consumed those prions in tainted beef, and I'm just on a waiting game, hoping it isn't the case... :(
@@leechowning2712 It'd be illegal to do so, due to data protection the scanners are operated by people who have no knowledge of who is going through, they only have access to an alarm to notify the security people to let them know of suspicious items on the images...
@@leechowning2712 Not sure those x-ray machines would pick it up, anyways. Besides, that would probably manage to hold up clearing security even longer; and, knowing the US, if it worked at all, people would use it for free testing, as an alternate to the ridiculously-priced system in place there now, which means that (a) they'd probably start charging a similar fee to go through it, making air travel damned near as expensive as getting healthcare; and (b) you just *know* that it would only be a matter of time before someone sued the airport for not detecting their cancer or something. :/ TL;dr: this is why we can't have nice things. Probably really would be simpler (and cheaper!) to just switch over to a proper universal healthcare system so you can just go get screened when you need to and be done with it. But it's not a bad idea!
My favourite protein and disease class, PrPc is somewhat related to Alzheimer’s pathogenesis too (more links may have been found or disproved since I studied neuroscience almost 10 years ago though). The cause of nvCJD is not solely the addition of cattle products into animal feed though; we used to pasturise those animal products at temperatures high enough to denature (destroy) those prions. However, somebody realised they could make a lot more money by reducing the pasteurisation temperature, which obviously requires less fuel and therefore costs far less. The new temperature was lower than the denaturisation temperature of the prions, hence it was at that point that the prions entered the food chain and cascaded down to everything else. Human greed is the ultimate cause of the Scrapie, BSE and nvCJD epidemics that have/will follow.
Correction: Prions CAN be destroyed with heat. Incineration destroys them. Of course you can't eat incinerated carbon (well you can actually but it isn't really food anymore) so, yeah, for practical purposes you can't use heat to destroy them.
Fuggin' thanks Simon. No sleep for 3 days at least and nightmares to follow. :( Seriously though, good video as usual. Surface level, but good enough to get the point and information across to anyone watching it. Anyone wanting to learn more has a couple of good books to read.
This stuff is genuine nightmare fuel. In the mid-90s, I worked with a USAF wife. They had been stationed in the UK in the 80s. The entire family were forbidden from donating blood. Ever. I've often wondered if any of them later discovered they'd been affected.
Unfortunately one of my cousins daughter has a Prion disease. It's been really difficult. I believe symptoms began showing up when she was around 3 years old.
Weaponizing? Well, that's GOOD. Just couldn't finish the day without that. I thought I had everything I needed till I heard about this. Thank GOD someone is going to make it easily available.
Bro where was this when I was doing my report about prions. This would’ve been an absolute life saver. Great video btw absolutely loved it, when u said the names of the diseases I spoke to myself saying which one was which absolutely brilliant👍
yea i think theyre more comparable to cancers than to parasites, viruses, or bacteria. its more like something in the body fucking up in a very destructive way, rather than some separate entity trying to utilize your body for its survival like a parasite/virus/bacteria
I have been studying prions on an amateur level since I learned about it. Cannibals are one of my favorite subjects. I also hunt and now cwd is spreading at a rapid rate.
@gg gg they think cwd is actually an unintentional consequence of American hunting laws. To keep people from poaching or over harvesting it is illegal for me to sell game meat(any products I obtained from public means, hunting trapping or gathering), I'm split on this law as I get it but it mine now so... but that's a different conversion. Anyway as it is illegal for me to sell game meat and game meat is desirable people in the Midwest started farming deer and elk(techniquely no longer game, it's farmed meat now) deer farming being the questionable practice. Deer are fairly independent they live in small groups. They think a deer in a farm was exposed and it exploded from there then wild deer visited the farms and it got into the wild so now it's in most of the US.
@gg gg I live in Oregon and 20 years ago it was a Midwest thing no concern of ours. Last year we passed a law now out of state game parts allowed. Trying to keep it out now. The 90s is when it started spreading lime wildfire.
@gg gg @john veach Deer farming was the culprit… where deer were fed byproducts that included brain and spinal cord material (yes… again.) Now the genie’s out of the bottle and it’s spreading through the wild population. So far, no humans have caught cwd, so it might not affect us… but only time will tell.
My Mom Passed away from CJD. From normal to dead in 12 months. I will never forget how fast that decline was. I suffered so much watching and taking care of her all that time. Need a cure fast.
Correction: Prions _can_ be destroyed with chemicals, radiation or heat. The problem is, enough treatment to kill the prions is far more than the host can take before it kills _them._
I remember learning about Fatal Familial Insomnia when I was very very little, I don't know where I learned it from, but I've always been 'aware' of its existence. I had no idea it was classified as a prion disease, another topic I like to learn about often. I hope we can learn more about it all some day, its very interesting, prions.
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You might want to change your title from "uncurbable" to "uncurable" .... and then to "incurable".
Nordvpn, the software version of a prion disease.
I love sponsor block so much.
How the fuck are prions immune to the denaturing process?
10:42 If what you said is true, that a pathogen is an organism which causes disease, then a prion cannot be a pathogen by virtue of not being an organism. That's a category error.
My microbiology professor in college taught us to never, ever, eat any type of brain or nervous system tissue, including eyeballs, of any animal, because of the risk for prion disease. This stuck with me. As a hospice nurse, I've taken care of a few CJD patients, and its definitely not a pleasant way to exit this world, and it is heartbreaking for families to see their loved one deteriorate and then taken so swiftly from this disease.
Wish I knew that before I ate lamb brain
Guess I'll know what it is when I start suddenly dying
@@imhere1303 Why tf would you eat that
@@anthrazite Well, it's food
@@anthrazite same could be said about most foods lol
I have heard of certain areas where calf brain or monkey brain is a delicacy. I used to think I wouldn't mind trying it as long as it was prepared by someone known for making the dish well, but after learning about prion diseases I would have to decline the opportunity. Fortunately people aren't especially into eating brains in the US so I should be safe, for now.
As someone who was diagnosed with CJD at 14 thank you for making this video. I have gotten super tired of explaining what it is and what causes it. I get ekg, mri, and ct scans every 3 months to keep track of the holes and deterioration of my brain tissues. I suffer from gran mal seizures due to it. Im 28 now and my neurologist says by 50-55 I will enter the end stage of my life Im on many many meds to help slow the process, and to extend my life to live as fully as possible. So just know that the life expectancy isn't necessarily a rapid death. Hopefully by the then we will have a cure but if not, at least hopefully there will be better meds
Woah man I’m so sorry to hear that. At this point I’d assume you have accepted that fact. May I ask what happened at 14 to get the diagnoses? Symptoms?
Oh man what a horrible thing to have, I also have some mental issues that will follow me and burden me throughout my life but that's a whole other level, I feel so sorry for that man.
On another note it is predicted that the deer wasting disease could jump to humans and if that happens a few hundred people will die and that's all horrible of course but the positive thing that could come out of it is that we finally figure out how to treat these kinds of diseases.
Also, with the rapid progression of science the last 20-30 years, the next 20-30 years will probably be as groundbreaking as the past ones if not even more if technology keeps progressing in an accelerated pace like it has so far. To put it simply, if you were to have cjd no matter what, nowadays would be the best time to have it, if you get what I'm saying.
@@ildisiri The speed of our scientific advancements is in part thankfully to the computers that help us analyze it. Right now, until we find out a way to make a quantum computer (which could spell apocalypse, depending on who invents it first) we are bound by the laws of physics. The smallest possible transistor (made of 14 atoms if I recall well from my IT prof lectures) has already been made, but is not in wide use, so technology will go ahead for a couple of years, but then the development of computers will... well, kinda stop. That being said cloud computing etc will still let us make great advancements, just... don't expect the avalanche-like speed of that development to continue.
Also, as a person with mental issues I get ya. I'm trying to build my life now, after 25 years of trauma and bad psychiatrists. I found a good psychiatrist, though, so it should be better. I'm sorry for the OP too, it's horrible, but... We must try to make the best of it while we can.
@@AK-fr5zv: "a quantum computer (which could spell apocalypse)"
I'd like to think it's our ticket to free our conscience from the physical world, to upload the consciousness of humanity into the cloud as weaponized prions spell armageddon to everything left running on neurologic wetware.
@@mfaizsyahmi I mean, yeah, with enough storage, we absolutely could do that, conceptual question is: is it still you, or is it just a copy of your consciousness. Or do you think we could somehow link up to machines to instantly transfer any and all data held within our brains, thus, perhaps, preserving the consciousness of the living being into the cloud? I mean, I thought about that a lot, too.
And, again, it all depends on who discovers quantum computering first, and how they use it.
There's also an entire system of "chaperone" proteins that are designed to catch mis-folded proteins and either re-fold them correctly or destroy them, which is probably why spontaneous CJD is fairly rare.
Saunas apparently increase the number of chaperone proteins. They also reduce "all-cause mortality". I wonder if there's a link.
Anyway, I'm heading straight to the fucking Sauna shop.
That makes me feel much better, thanks 😅😮💨
Wait, is that the cure? It must be used as a cure!
@@sendmorerum8241 it sounds more like an excellent start for a cure. I’m guessing there are already labs all over the world studying this exact process so they can formulate a cure.
But despite the presence of chaperone proteins, study is still ongoing how to break down the beta sheet-laden structure of prions (which makes them virtually unkillable by proteolysis or radiation) and eventually, destroying prions without subjecting to 900°F sustained heat for 6-8 hours
I had a lecturer at university who worked with the National CJD Research and Surveillance Unit. I remember her telling my class why CJD surveillance is so vital, they suspect that the mad cow outbreak has resulted in a lot more human deaths than have been reported. Since prion diseases tend to require an incubation period of decades before an individual becomes symptomatic, they suspect that mad cow disease resulted in a number CJD cases that are currently emerging in the elderly population and that these cases could easily be misdiagnosed as some form of dementia since both these conditions have very similar symptoms.
My father died of Prion disease a little more than a decade ago. He travelled extensively, including to England, many times, during the mad-cow disease time frame. I asked whether that could have been connected, and no one has ever given me a definitive answer, as it 'could be' but could also be so many other answers, and there's really no way to tell.
@@JesterMotley i was a nursing student in Cleveland Ohio 25 years ago when I briefly cared for a cjd patient. He was a vp of a steel firm with business in England. It was suspected he had the beef. His demise was fairly rapid. He went at first to a psychologist then psychiatrist but within 2 months was hospitalized unable to speak or react to nearly anything. He died a month later. With the exception of 1 nurse who had a patient earlier in her long career with cjd, he baffled the entire medical staff. It is quite plausible for your family and many others to be misdiagnosed simply due to the lack of exposure most of medical community has to proins
@@walterwarren2402 While possible to misdiagnose while alive, a post-mortem comprehensive survey and testing of his brain was definitive and confirmed 100% the diagnosis, so no, its not plausible for myself and my family. But others might indeed be misdiagnosed. Sadly, the protocols and treatment for rapid dementia are what they did for my father, so even if there was a misdaignosis of cjd... nothing would have changed for him, or us.
is it possible we could all already be infected with prions?
@@nikkiespinosa8854 I'm not a doc, but given my family history we've... reviewed this extensibly. So take that for what its worth. Bollocks. That said? We are, all of us, routinely, 'infected' by prions, and we handle it. But we deal with it, because the human body is very resistive, in the end.. not perfect, but very resistive.
My uncle died from Creutzfeldt-Jakob Disease about a year and a half ago. One day he noticed he was having vision trouble. A week later, at which point he was already having trouble following conversations, he got the CJD diagnosis and given one month to one year to live. Alas, he didn't make it that long; two weeks after that, he passed. It was terrifying and surreal. If nothing else, the fact that it took him so quickly was a blessing, as he undoubtedly would have only suffered more had he lingered.
I've noted in other comments, my father passed away from a Prion disease. It took him 7+ years to pass (the family argues when he first started having/showing symptoms, but it was diagnosed 7 years before his death). Watching my father deteriorate year after year is... I can't put to words what it was like, but I can say, for absolute certainty, that your Uncle, and all of your family, really were blessed it took him so fast... Its a horrible thing to say, a horrible thing to think about, but I lived through the alternative, and I wouldn't wish that on my worst enemy. It's so insidious and cruel to everyone... he knew he was losing his mind, his life, we had to watch the slow crawl of him losing them. Just... yeah, your uncle was blessed to go so fast.
@@JesterMotley thanks for sharing that. I'm sorry that your father had to endure so much pain, and I hope both he and you have gained some measure of peace now that it's over. Prion diseases are well and truly terrifying. Hopefully we find a cure or even a treatment for them soon.
@@Thexaios Thank you for your kind words. I've had some time to find peace with it. I was, and still am, hoping my words might help you and yours find peace in what happened to your Uncle, and that you are right in your belief it was a blessing to go so fast. I truly understand what you and your family went through, and I hope what I've said can help even in the slightest. That was, and remains, my only reason for speaking up.
I won't lie, dementia and other diseases that cause demetia symptoms terrify the F out of me; I'd take myself out, it's not worth it. Sorry for your guys' losses.
@@John-Perry Hey mate... I understand. Watching my mother, and brothers, deal with a father who everyday was... less... was devastating. I can... support... if you will, the idea that its better to pass sooner, even if by ones own hand, than later in a state you can no longer rationally make that decision. But I hope you, or anyone else, never has to go there or anywhere near it. Peace and love.
You touched on cannibalism. Here is an interesting fact;
"...some of the people who survived the kuru epidemic carry a genetic mutation called V127, whereas those who developed kuru did not have this mutation. This led the researchers to suspect that V127 conferred protection against the disease."
Or conversely, the lack of V127 caused the epidemic in the first place.
That is a common thing with infections, be it bacteria, viruses, parasites, or even prions. After a while the people either develop some resistance to the disease, or the infecting agent mutates into a less aggressive form.
For example the 1918 "spanish" flu epidemic was Influenza A subtype H1N1, a type that is still around, but we don't see dozens of millions dying every year.
And after every bubonic plague wave the population was much more resistant and it took multiple generations for the resistance to go down and the next wave to hit.
Or all the diseases Europeans brought into the Americas that totally ravaged among the native population, but were rather tame across colonists.
And SIV in chimpanzees doesn't cause the same, massive symptoms as HIV does in humans, they have already adopted and found a balance that keeps both, the apes and the virus around. No doubt humans will develop in the same way over the next couple centuries.
Or how the Omicron variant of SARS-CoV2 has a much higher transmissbility and reinfection rate, but much less hospitalization and mortality than earlier strains. We're already moving away from the super dangerous pathogen and towards something that will stay around, but isn't a big deal.
I use to work in an hospital pathology lab, a patient w/CJD died and the pathologist said absolutely no autopsy, even though one was requested. This was circa 1999.
good call from that pathologist.
@MementoMori Multiple people have died from accidents working with prion infected tissue. A simple needle stick is fatal.
It requires special equipment and training to minimize the risks and it is still extremely dangerous. Such an autopsy should be done at a facility where everyone is used to that level of risk and thoroughly prepared for it. Trying to do a one-off procedure that dangerous is asking for trouble.
@MementoMori If penguinista's response didn't make sense, you can be infected by prions from another source and as Simon mentioned there isn't a treatment and it is fatal. If there was not a strong reason to do so, there no reason to take the risk of performing an autopsy; the person's death is most likely from prions or complications from prion disease.
@@ROMAQHICKS Thank you for clarifying.
@@penguinista you don’t understand how pathological autopsies work. Even the most infectious corpse will have an autopsy done in a normal morgue. They just put in stronger regulations.
Your point doesn’t make sense because some deadly pathogens aren’t even discovered until they do an internal inspection of a corpse. Tox results can take weeks even months to come back to the pathologist
I like how, although ritual cannibalism is something absolutely forbidden in Western society (for many very good reasons, not to mention kuru itself), you take the time to explain the Fore people's thought process behind it rather than sensationalizing it as "savagery."
Agreed. He didn't say whether they'd stopped doing it, though, which I always thought to be the case.
@@ewestner I thought they did stop, but I’m not 100% sure.
It is savagery
@@ismailah4576 bro it's possible to get a loved one taxidermied in the usa in places. That's fucked up on the same level. We also eat sheep's brain
@@Aztesticals Still Savagery. Besides, Livestock are safer to consume anyways.
As a deer hunter who's seen the rapid spread of CWD.... my cousin summed it up the best:
When asked is CDW meat safe to eat, "Well, technically yes... but I don't want my daughter to be the first jump over case." This was about 6 years ago, before COVID. Prions are terrifying.
I always wondered about the cases in deer… my uncle died from prion disease and he used to be a deer hunter. It’s a terrifying thing :/
My compliments! About a year ago, I was at a barbecue where a man brought venison to share with everyone. I said, no thanks. I went vegetarian that day, the only one. Didn’t I like venison? Yes, I love it, but wasn’t going to eat it because of CWD. The man and his wife got upset with me, cut me off, and told me there was absolutely NO evidence of human infection, was I conspiracy theorist? If I’m going to ruin the barbecue, I could just leave. Realizing nobody would listen even if I did warn everyone, I stayed for while and excused myself early. Just as in the UK, nobody WANTED to know or even consider it. Keep your eyes open in the US among consumers of deer, elk, moose, etc.
@@mariekatherine5238I guess they never heard of mad cow. Why tempt fate if it's not necessary, you know. F them for being so weird about it lol
@@mariekatherine5238 Yeah, not until we know more about it. There’s no knowledge that it can yet, but…well, assuming that BSE couldn’t naturally cross the barrier because scrapie couldn’t was what led to problems.
(BSE does seem to be the odd man out here so far since it pretty readily hopped to humans, cats, oryx, bison, gemsbok and other exotic hoofed mammals…whereas the only time a non-sheep caught scrapie was in a lab.)
Still…never go into it not knowing.
2:15 - Chapter 1 - What are prions ?
2:55 - Chapter 2 - How are prions made ?
4:40 - Chapter 3 - Symptoms of prion disease
5:35 - Chapter 4 - Mad cow disease
8:35 - Chapter 5 - Kuru disease
12:10 - Chapter 6 - Sporadic prion disease
- Chapter 7 -
- Chapter 8 -
- Chapter 9 -
- Chapter 10 -
Ayo bro u saved me a good 1 minute of my life and I Thank you greatly for that lmao
Thanks mate!
My father-in-law passed from CJD. I've never been so scared in my life as when watching him progress through it. He died a mere 46 days after diagnosis, but hindsight revealed we'd missed obvious symptoms months earlier. He was such a strong willed, focused man. Seeing the slow-yet-fast, unstoppable descent into dementia was traumatic. The only positives I've taken from the experience were that he died relatively painlessly, and that I now know how much he meant to me.
There are also proteins that are labeled “prion like” such as beta amyloid, tau, and TDP-43 that could be responsible for aspects of the pathogenesis of neurodegenerative diseases.
Multiple System Atrophy has recently been named a prion disease. My husband died from it, it is horrible.
Yet nobody dares to admit this, because this is going to cause a lot of problems in the health system. Imagine screening everyone suspect for a neurological disease to avoid transmitting it to others.
@koekoek It has absolutely nothing to do with the SARS-CoV-2 vaccines. We don’t have a endogenous (originating from humans) spike protein so the spike protein from the vaccine can’t act as a prion.
Second, the spike protein from the vaccine is almost identical to the one from the virus, with just minor amino acid changes to improve its stability. If the virus doesn’t cause prion disease then why would the vaccine.
There is no biological plausibility to think the vaccine can cause prion disease. Anyone who has told you otherwise made it up, like the countless other stupid claims about the vaccine that were false like have luciferase (which isn’t even bad and would be useless in the vaccine since it mostly used as a research tool not medicine) or claims that changes your DNA (it doesn’t).
isn’t the tau protein the one responsible for dementia?
@@koekoek4145what
Prions have terrified me since I was little.
Thanks, Simon.
There's a small but very important misread Simon made in this video. He said "about one to two million people are diagnosed every year". It's actually one to two *per* million people in the notes onscreen. So if it makes you feel any better, prion diseases are much, much rarer than the video (accidentally) suggests. One reason why so much is unknown about prion diseases at present is that they're extremely difficult to study due to the lack of available subjects.
He didn’t even get into the fact that deer get chronic wasting disease yet don’t regularly eat brain meaning that chronic wasting disease is incredibly contagious within the deer population, so if it ever spreads to people it will probably be around as contagious. And people thought Covid was bad. Deer have been known to eat the heads of baby birds for calcium ect that might be how they get it or got it.
I thought they were a baddie from Star Trek!
No need to be terrified if you're vegan
@@drabnail777 The whole world turning vegan terrifies me even more....
Simon, ive been a fan for a while now. You always bring quality content. Not to mention your sheer willpower in keeping up with so many channels. Keep up the good work!!
Except the brain blaze, it is get infected with prion, but still fun to watch.
@Yummy Spaghetti Noodles it may have something to do with a certain white pounder...
@@hiigara1 that and the other people involved in the channels
@@Iamtheliquor this is also true
@@hiigara1 more likely with the fact that simon most likely "just" films the video itself and sometimes write/co-writes the script and there are people for everything else
“The Family that Couldn’t Sleep” is an excellent and very easy-to-understand read for anyone looking to know more about prions and the history of its study. I listened to it a few months before watching this episode, and I loved it
Fatal familial insomnia. So freaky, you don't need to eat prions to die from it, sometimes you're just born with it.
I'm reading that book now. Prion is definitely the scientific conundrum of the century.
Fatal Familial Insomnia becomes even more terrifying, once you realize some cases are sporadic and not familial.
While an anthropology student in the mid 80's we did a deep dive in the Fore (Fo' rei/ For ā) and Kuru. My professor worked with tribe in 60's and had firsthand knowledge. One detail not mentioned was the chance observation of Allen while at a seminar on Scrapies. Scrapies is prevalent in sheep and he noticed the similarities in brain tissues which put him on the track of transmission since environmental factors had been studied with no results so it had to be cultural ie some practice that is the cause. Bear with me as my Medical Anthropology was almost 40 years ago. Damn I'm old.
This stuff never ceases to be fascinating! Thank you for sharing your knowledge.
@@wendychavez5348 not to mention terrifying. Every now and then here in the states, someone goes swimming in warm water in the southeast, or once, in Colorado, and they wind up with that one that winds up up your nose and bam, they're dead in days to weeks. Reminds me not to swim in ponds where I live when the temperature gets high.
@@MegaKat Ah yeah, good ole
en.wikipedia.org/wiki/Naegleria_fowleri
- Gotta say, regardless of medical accuracy of the show, I'd never heard of the little bastards until an episode of "House M.D." - makes me glad I don't swim.
@@WindKing0 yes, that's the name of it, thank you, I was having a brain fart on my reply and google was being a pain. That shit ain't nothing to fuck with; do NOT swim in warm water that isn't a treated pool!
They permanently closed one of the disnay water parks for that reason
Chronic wasting disease happens in deer elk and moose. If you see them walking in circles they are affected and need to be put down. Contact your local fish and wildlife if you see them.
Not just walking around in circles, they also slam their head against rocks (my buddy saw that first hand) and casually come up to humans, granted the latter could be multiple different reasons. But in the end, like you said, they need to be put down immediately. God forbid it spreads to other deer or worse, to a human who may eat it
It's a good thing I carry my dope .40 cal hi-point with the 100 dollar bill finish. Drop that moose with my glawk whenever I see one bussin fr fr. It's gon be lit dog
Thanks for that information.It is very important to know!
Thankfully it hasn't been known to spread to humans, but we definitely don't want to take the chance! On top of this the way it spreads so well is because it can survive for a very long time outside of deer.
Or if the deer stands on two legs/limping and stumbling. Also call, because the disease could be damaging its joints and causing pain
As for why prions do what they do, there probably isn't an answer beyond accident. Sometimes, when things randomly go wrong, the consequences can seem bigger than they should, but that doesn't imply any intent, it's just how randomness works. The fact that proteins that are folded in a particular way will disrupt other proteins and thus start a chain reaction isn't particularly surprising. There are many instances of natural processes that, through random movement, are self-sustaining. A recent example that has been in the news is xenobots.
Based on my (admittedly only ‘okay’) understanding of biochemistry that’s probably the case. Protein structure relies on chemical bonding and the more stable the bonds the more difficult it is to break that up. Prions may just be this…extremely stable structure so they’re basically chemically inert.
The amino acids link together, then the chain ‘folds’ usually via hydrogen bonds, then side chains link up to make a 3D structure and then multiple folded chains link up…
So somehow the chemical reaction went an odd direction and it was stable. VERY stable.
Love your content Simon. My father passed from sporadic CJD on May 6, 1996. He started becoming symptomatic in the summer of 1995. He was from the rural United States. One of the members of my family started a website for family members . A page dedicated to people who needed support for dealing with the grief of losing their family members to this disease. There were hundreds of cases in the US in 97 over 50 in Texas alone. The CDC sent out a lot of misinformation and down played it's impact to the food in the United States. The numbers the CDC sent out were a fabrication, I personally called them in 1996 . The CDC said it was a non reportable illness and there was no CJD problem in the US , and he had to have went to the UK. I am 52 years old , my father died at 57. I am slightly concerned. Thank you for your work.
BTW Deadly Feasts by Richard Rhodes is a little dated , but a great and terrifying read.
Very high doses of “protein cleavers” are showing promising in treating these malignant variants. Such as bromelain. It’s interesting how some of these enzymes have been overlooked for years. Bromelain has been used to break down the spike proteins of covid19. Nattokinase is a very strong fibrin cleaver that is used in Japan for thrombosis.
does that mean that pineapple, kiwi fruit, and pawpaw could all be used to treat it? or are we talking higher levels of bromelain and other related chemicals than found naturally?
@@bronhaller it would have to be isolated because eating lots of fruit can cause other problems
Doubt it
@@bronhallerPresumably higher levels than would be found naturally. It’s my understanding that prions are quite stable, they won’t be inclined to react with just anything, so you might need some very concentrated enzymes to do the trick…
Thank you for this video, and for the manner and method you delivered it. This hit home to me. My father passed away due to a Prion disease a bit more than a decade ago. It took me several days to work up the gumption to watch this, much less comment, as I hope are self-evident reasons. And I was relieved, though unsurprised, that you handled it to the usual respectful, intelligent, and brilliant standards I've come to expect of this channel. Anyway, just wanted to say a thank you for doing this, and to your writers and staff for treating it well. My father, as a scientist, would have applauded your efforts, too.
Great video. Sadly, GSS is in my family and my middle sister is rapidly nearing the end of her life. My paternal grandmother, dad, his brother and both my brothers have all died from it. Also a few other nieces, nephews and cousins. Indiana University School of Neurology has done a genealogy back to Europe. I was tested when my kids were in high school and thank, goodness I was negative so my kids cannot get it.
It is a terrible disease. Thank you for posting this video. Genetic diseases suck.
I would've gotten the test before getting kids, because it's something you wouldn't wish on your enemies, not to mention your offspring.
@@sendmorerum8241 Well considering judith does not look like a spring chicken from her profile picture, I would image the test did not exist, or at least not widely available or cheap enough to consider.
@@goldenhate6649 Then I wouldn't consider children at all. It's an illness too terrible to play Russian roulette with your kids.
@@sendmorerum8241 antinatalist cringe
@@joelhernstrom6060 How it is antinatalist? What she did created unnecessary terror to experience for at least a decade!
That is a bit too much, seeing your relatives then look at your children and imagine them in the same condition, knowing you are responsible. Knowing that one day, you have to tell them about this sickness.
And no, it doesn't matter if you didn't want to pass on this condition. Thinking it is something outside of her control is nothing more than dodging responsibility, the good ol' "it's God's/nature's will". You're not obliged to have children, not matter how much gratification you get from having them.
I watched a co-worker wither and die within 14 months of his retirement from prion infection, squirrel brains was the suspected source for him. Awful sight to see a healthy man turn into a husk almost overnight! Scary indeed
Can I ask - how/why did he consume squirrel brain? I’ve heard about squirrel meat being tasty, but not so much about their brain…
@@BuddyLee23 Maybe strains of brain tissue has remained.
Should have been vegan
Rodents don’t have any recognized prion disease in the wild.
@@drabnail777 I’ve seen you comment under all these. I’m now going to spread your IP. Good luck buddy. Learn to shut up. Can save your families life one day ;)
Okay, here's my question. How the hell thought it was a good idea to feed cattle other cattle? Cattle are herbivores. No good comes of trying to change such a fundamental element of an animal's life. Would we try to make lions herbivores? No, so why do it the other way to cattle? That was never going to end well.
Herbivores will often eat meat if the opportunity arises, especially in biomes with scarce plant growth. I'm not at all justifying feeding cattle to themselves - the consequences are manifest in this video. But herbivores' diets are not quite as fundamental as people think.
like the other reply said herbivores do eat meat occasionally in nature, and this feeding cattle to other cattle WAS effective at helping them grow, it became problematic when cattle who had scrapie were fed to other cattle with the assumption that it wouldn't spread, which it did. the whole process does sound messed up and "unnatural" but I don't think it inherently is, and I think we shouldn't dismiss this stuff just because it seems that way at first glance. idk I think utilizing the waste products of butchered animals (bones and lower grade meat) is a good thing, we've been doing this with chickens for a while now, using their remains for stuff like chicken nuggets or fertilizer from their bones etc, utilizing the parts of cattle that would otherwise be thrown away by feeding them to cattle who benefit from it isn't such a bad idea on its own imo
I want to say it was from bone meal which was given as a calcium supplement.
Cattle will eat bones or even snakes if the plants in an area lacks minerals
Of obviously, this was a mistake, but feeding animals (or humans) diets with slight alterations from what they might encounter in nature can bring a lot of good, if it means avoiding nutritional deficiencies. Hell, just giving animals stable diets in the first place is a change from nature.
My son's godmother died of Creutzfeldt-Jakob disease. One day she was fine. Then she started having coordination issues and slurred speech. Then she became unable to talk or walk, and finally descended into a vegetative state before she died. All this happened within a month.
Literally prions and Tau proteins are done of the freakiest things but I think once we fully learn about them we will greatly improve neurodegenerative illnesses. My grandma just passed from a rare unknown brain dementia that hit out of no where for her last years of life after being sharp all her life. We still have no clue what happened to her and it bothers me and really makes me want to study brain degenerative Illnesses.
" The Family That Couldn't Sleep " Great book, easy read. The history of Prions to present.
ive always been terrified of prion diseases.
Small correction here, prions actually can be pretty reliably destroyed by heat, however the temperatures required are hotter than even autoclaves get. Sustained heat at around 900 degrees Fahrenheit (about 483 degrees Celsius) will reliably destroy a prion to the point of no longer being infectious. For obvious reasons though, meat isn't cooked to anywhere even close to that temperature, as it destroys all the proteins in the meat, to the point of being left with essentially a brick of charcoal. But, the point remains that heat can destroy prions, just not at a temperature anywhere close that most people can even produce in their house, let alone heat contaminated meat to
I saw a rather unscientific experiment on tv a few years ago where they heated scrappie from sheep to temperatures that nearly reduced it to ash, buried it in a rose garden for 2 years, and samples of the dirt could still infect sheep who ate it with scrappie.... Scary stuff...
I had heard temperatures could not get high enough to kill prions.
@@Galen-864 prions can't be "killed" because they aren't alive. they are proteins. proteins are carbon hydrates. carbon hydrates can be burned if you bring enough heat and oxygen.
if you ever did any research on the subject you'd know that the standard procedure for mad cow disease is to incinerate the carcass, they wouldn't bother doing that if the heat wouldn't get rid of it.
@@windhelmguard5295 uhh.. aren't carbs what you mentioned? Proteins are folded amino acids.
@@TimberWulfIsHere biologically speaking you are right, chemically speaking amino acids are also carbon hydrates, as are all organic molecules.
Dr. Stephen Thacker at CDC (famous for identifying Legionnaires disease) died from CJD in 2013. It was devastating to watch.
Aww that's sad. I'm fascinated with legionella and did a presentation on it for my pathophys class this summer, but I had no idea about that part of Dr. Thacker's history. What a shame
Yes it is. Devastating.
In high school (before Dr. Thacker died), I shadowed one of his colleagues who discovered the link between Legionnaires and hospital water systems and it was beyond cool to hear stories about their discoveries. Both of them, along with learning about epidemiology at a young age, inspired me to get my degree in public health. I work more on the education side now, but I’m still an epidemiology nerd and a trained contact tracer.
He was very active in welcoming new fellows into the CDC and offering mentorship. Working with him will remain one of my high points in my career.
@@nlc43 If you feel like it, would you be down to elaborate about your work and the niche you're in now? I'm a public health nerd myself and particularly I'm enamored with the relationship between patient behaviors/adherence/outcomes and the variation in medical epistemology across different social strata (I'm nuts about all things upstreamist really, but especially the epistemology part). So I'd love to know more about the education work you do and what you could share about the state of the public health scene at large with regard to your work and the work you see being done and foresee coming in the future. I'm well-networked and experienced in nichey weird ways (specifically I'm chronically homeless and do a lot informally on the ground educating and referring out people I encounter in the unhoused community, since for some reason people are quick to ask me for advice despite the cursory and eclectic nature of my actual medical experience) ... but even with recently finally going back to school, overall I just don't have many feelers out in the sphere of Normal Employed People Who Are Educated Formally And Doing Work That's Much Better Defined Than Mine lol, so I love to gather insight where I can!
If you don't have the time or otherwise don't want to expound that's also totally cool of course 💚 Thanks for the work you do!
CJD is no joke. Last year a priest in my diocese had to retire early and move out of state after he was diagnosed with it. Barely a month later, he was dead.
God didn’t save him.
You can also get prion diseases by eating any nervous tissue, not just brain.
Yep, my microbiology professor in college warned us against consuming any type of nervous system tissue, including eyeballs. When we dissected cow eyeballs for anatomy class, we had to wear full PPE, including eye protection also for this reason. Scared the hell out of me back then. I've now seen people die from this as a hospice nurse, just confirms that it still rightly scares the hell out of me
You can get it from consuming pituitary gland. World found that out the hard way when using pituitary glands from cadavers for fertility treatments in the 90s
I've always likened prion diseases to the neural version of false vacuum decay of the universe. It takes just one particle to seed the reaction, and the reaction is unstoppable and total.
Just like a rotten apple influencing its neighbours. we have every day examples.
I always thought these diseases were a thing of the past or extremely rare, but now I know just how dangerous it can truly be, especially with companies and governments that don't care about the people it rules over.
The entirety of the epidemic of mad cow disease was stopped cause the goverment intervened.
Prions were what put me off of biology when I was at school. You would think disections would be the cause but no, prions, a disease with a 100% mortality rate and it is just fortunate its very rare.
I did not know Fatal Familial Insomnia was a prion disease. WOW.
My great-grandpa died of CJD. I never met him, but my mom said it was a very rapid decline. My grandpa on the other side really liked to eat cow-brains. He never got sick from them, but it's kinda freaky and gross thinking about it.
Prople used to mix with scrambled eggs
Always love the videos Simon, and there's so many channels to choose from!
I think an interesting topic for a future video could be forever chemicals, like PFOA and PFOS, which are everywhere, don't naturally break down, are extremely difficult to remove during drinking water treatment and are known to cause cancer and multi other diseases. They have been around since World War 2 and became big in the 60s. They were largely unregulated and unknown about (by authorities, the companies using them knew what they did but covered it up) until the 2000s and are now found in the blood of almost every human on the planet. I think they're in equal parts terrifying and fascinating, and the fact they're found in so much of our everyday lives makes them a particular challenge
CWD is transmissable through saliva too, this is why white tail deer who are social deer are more affected by CWD than other deer types that are less social like Elk. This is a big reason not to bait deer.
The perfect channel for this. Prions are one of the most scary things in the world.
Prions, like a fair amount of genetic illnesses, are one of those things that doctors look at in theory, but most people will never see... Thankfully because the outbreak in England effectively destroyed their beef economy for a decade or more.
There is a lot of things like that that keep doctors and anyone else with an interest in public health awake at night. I don't think I've ever seen it, but have you done a episode on the sin nombre virus? Proper name Hantavirus, identified in 1993 and having killed just shy of a thousand people in nearly 30 years, for those of us in the western US it is always a concern.
I thought he already did.
Chronic Wasting Disease, which is caused by a prion, is endemic in deer populations in the eastern US. We will be seeing more and more of it as time goes on, unfortunately..
@@penguinista I live in an area in the eastern US where hunting is popular and people do eat the meat. I think most are totally unaware of this.
And I just realized how many deer heads are worked on by taxidermists. Ick!
Prions aren't a genetic disease. Protection from prions is genetic though. Mutations m129v and g127v (only found in the Fore.) are the mutations.
I recently read Cold Plague by Daniel Kalla. It's fiction and involves a lot of espionage and intrigue in the French countryside, but involves a lot of interesting science too, and prions are qt the centre of it all!
You should read Hardwired by Walter Jon Williams! 30+Year old novel about Big Pharma creating diseases n the cures for them!!!!
Fatal familial insomnia would be an absolutely brutal way to go. Weaponizing that would be a horrific act of inhumanity. Imagine a country full of people who are incapable of sleeping for months at a time, and have succumbed to total and utter madness as a result. This is the zombie apocalypse.
The symptom onset changes according to how those proteins are introduced to the body.
Maybe if they’re introduced from the outside insomnia won’t be the first symptom.
I'm afraid you might be right about this...
At least with most diseases you can sleep through the symptoms or fall into a coma... Not with that. Sounds like the worst thing to die from next to rabies.
I haven't heard the term "Mink Transmissible Encephalopathy" in years. Once knew of a farmer who lost some of their mink to MTE. I remember my dad teaching a new-hire on the mink farm to watch for the symptoms of MTE.
The Kuru outbreak is where most movies got their idea that eating human would cause you to get ticks and act crazy, but didn't really know that it was actually just from a prion.
There's a NOVA doc on a man who came down with FFI. Fascinating to watch, as it shows him in videos and filmed documentation of the progression.
I lived in the UK in the 80's. As a result I can not donate blood in my home country of Canada. Neither of my parents are suffering from "mad cow" disease fortunately. I can still donate my organs upon death.
I just visited the Britain for a holiday in 1990.
So, I can no longer donate blood.
No coincidence that "prions" is French for "let us pray"
Years ago we OR staff were discussing Prions and how we would have to deal with the possibility of performing a craniotomy or biopsy on such an individual. I have dealt with VRE, MRSA, necrotizing fasciitis (great video as well) but prions scared the living hell out of me. Since sterilization could not denature it, any instrumentation would need to be treated as an extreme biohazard and destroyed as it could not be reused. Scary stuff.
honestly I was surprised you didn't mention this, and it might not be online anymore, but there's people who have covered it: there was a guy who stopped being able to sleep, and started getting really sick, but doctors couldn't tell him what was wrong I think? so he just started making videos to document his decline. Its horrifying, terrifying. He goes from this intelligent, well-spoken man who's frustrated with his medical treatment, to a religious lunatic raving about the apocalypse, to finally being completely incoherent, where you couldn't even really understand what words he was trying to say. It's so sad, so scary, but also painfully real. He didn't want to be forgotten just because he couldn't afford treatment. It's a really heartbreaking story :(
if you search "man documents fatal insomnia mental breakdown" its the first video that pops up, by ScareTheatre. The second video that pops up is the original guys' channel, actually. I just searched this myself. I'm kind of surprised its still up. His name was Richard Siagian. RIP.
prions are one of the creepiest and weirdest infectious agents. literally just a glitch, but a deadly one.
I haven’t watched the vid yet, but seeing the topic being prions reminds me of the level I’m on in Plague Inc. Crossing my fingers that no new plagues are gonna start irl, but this is Into the Shadows, after all.
This video was excellent…. I’ve been reading recently in New Scientist about some ideas around the possibility that certain forms of dementia are the cause of prions, not the result… that’s all very much up in the air at the moment…. However, to cover so many aspects of this topic in a 17 minute video is very impressive! I enjoyed this video immensely 👏👏👏👏
My Uncle was diagnosed with CJD just after New Years... Today he has to be moved into hospice. This is the worst disease in the world & i pray that one day nobody else has to go thru this.😢
I love the flood of notifications I get when Fact Boi releases numerous videos at once
Just the word "Prion" is enough to give me chills and fill me with dread. Allow me to point to the album 'vCJD' for one of the most terrifying listens. It's an Everywhere At The End of Time inspired album but with CJD.
Prion diseases have terrified me since I began reading up on BSE over 20 years ago
My lord, man. Between all your channels, I could listen to you all day!
i think the infectious misfolding occurs due to prions having multiple low energy states (think false vaccum from quantum physics) and basicly the infection state could be the more desired energy state.
Holy shit! 🎯
Loved Prions as a subject since X Files aired 'Our Town' in 1995 about an immortal cannibal cult that caught CJD!
Ooh, yes, I remember that episode! It was actually pretty neat; I remember the Mad Cow outbreak in the UK back when. We'd visited family there for around a month or so back in 1981, so, yeah. I know the concern was it passing to humans, of course; but I don't remember the proper name for it (CJD) being used back then, and now I've been fascinated by it ever since that episode. Plus it taught me how to pronounce it, lol!
I also remember 2003 when a cow was found with it in Alberta and the US closed its border to Canadian meat for like a year-- same year as the drought out here in western Canada; that was a rough year for farmers. Suddenly not only could they not grow any hay for their animals, but they also were stuck with all these *extra* animals, and no income!
Farmers who previously could feed their animals over the winter for the price of the gas to run the tractors to bring in the hay were suddenly stuck looking at tens of thousands of dollars in feed; I remember one farmer saying she was looking at going from her own free hay to at least $30,000 to feed them over the winter.
That was the year of the wildfires too-- well, the first one, anyways. :/ I was involved locally up in the Interior of BC in cattle ranching country volunteering for Noah's Wish and helping to evacuate and house animals-- from house-pets to livestock-- and a farmer I visited who was helping to house evacuated cattle showed me what was supposed to be his hay field. Looked like an abandoned lot with some scraggly weeds in it. Said it would cost him more in the gas to bring in what little was there than to just buy it elsewhere. He was just going to leave it; figured there was only around twelve hay bales there, tops.
So he couldn't afford to feed his cattle, not even the reduced herd he'd usually have after selling his cows, because without being able to sell them, he had no income for the year, of course. And in BC (and presumably elsewhere in Canada) any cow you aren't just eating yourself *must* be butchered down at the government slaughterhouse (which is how they can test every single cow that enters our food supply, which is how they found the one in Alberta in the first place). But that costs $250 per animal, and with the US border shut, the price per cow dropped from $800-1000 (depending on whether or not the animal had been branded, which reduces the value of the leather-- but helps to prevent cattle rustling! Which at a profit of close to a grand per cow stolen is absolutely a thing still, it ends up) down to $200-- which meant a net *loss* of fifty bucks per cow butchered. :(
He said the only way he could have managed was if he had been allowed to butcher them himself and sell it on the roadside (something you see a fair bit in our fruit-growing areas out here-- with fruit, of course), but as it was, all he could do was to dig a big hole, shoot them, and shovel them in.
But the thing is, that after that whole big scare we'd all had with Mad Cow in the UK a decade and a half or so before, Canada changed its own regulations too, and is now *obsessive* about testing for it-- and for excellent reason, apparently!!
Every single cow that enters the public food supply (i.e. anything other than what a farmer might slaughter and dress for his own direct use; I don't believe they're even allowed to give the meat away) *must* go through the government slaughterhouses, as I said, and every single animal is tested for Mad Cow.
If one is found, it, its herd, and any other cow that it was in contact with in its journey there are all pulled from the supply chain and incinerated, and the farmers are paid full market value for each animal they lose, so there is zero financial incentive for them to hide any instances of it. Any meat processed by the slaughterhouse or butcher since that animal came in is also pulled and destroyed. Every cow in Canada has its own ear tag, so they can be tracked all the way through, for precisely this reason.
--- Which meant that we were all pretty pissed off with the US for closing the border for that year, because our food supply is *safe,* and actually much safer than theirs! People up here thought (and I agree) that their government at the time -- Dubya, iirc-- was just taking the excuse to basically outlaw imports and give their own farmers a boost, rather than looking at it scientifically. Which, understandable, I guess? But it screwed us big time, and under NAFTA that kind of protectionism is outlawed. :/
Anyways, TL;dr: Canada took the UK's unfortunate lesson to heart and ever since has been extremely proactive about keeping it out of our own meat supply. Plus I remember that episode too! :D
I remember that. My first exposure to the idea. Refused to eat fried chicken for awhile after that…
Consistently some of the best content and production on UA-cam. Also love that Simon and I have the same beard and haircut.
Awesome beard but I wouldn't call being bald a haircut LOL
"Warheads Filled With Brains" is the name of my Napalm Death tribute band.
Been wondering how this channel was going to incorporate sponsor messages. Good choice leading with it. Better choice would have been at the end, "and if you can't wait for a prion to destroy your brain, you can melt it faster with RAID: SHADOW LEGENDS!"
This video hits home, my uncle is suffering from a super rare prion disease called cruetzfeldt-jacob and ots absolutely shocking
I knew a woman who died of CJD. She was in her late 50's, still working and a few years away from retirement. One day, she just didn't show up for work and she never came back. She was dead a few months later. It was heartbreaking for everyone who knew her. I hope the researchers will discover a cure for this terrible disease.
Oh good, you’re just running through my greatest fears on this channel
An interesting thought is that with how long it takes between the introduction of prions and the development of prion disease, up to decades in some cases, there are very likely people who will live their entire lives with prions multiplying in them, without even realizing it and end up dying of something entirely unrelated. Which means the number of people infected during outbreaks could very well be much higher than reported. Since there are bound to be people who die of other causes before they can develop prion disease. And I for one say they're lucky as hell. As ironic as it is to call a dead person lucky. But that's nature for you. Never fits into our tidy little idea of this planet being the perfect safe haven for life and humanity. Especially when you consider everything else in this world that's either trying to kill us, or make us suffer, or both.
They may be lucky by themselves, but some people who have been influenced by them may be not.
What about if they were blood donors, sperm donors, or eventually organ donors? what if they have been surgically operated upon without the knowledge of the medical staff about their infection?
This is a sadly timely video - a friends daughter passed away yesterday from CMD.
Oh man, that's harsh. My condolences! :(
And to think, those of us from britain who grew up during the BSE scandal could be just waiting for those slowly accumulating prions to take hold and kill us, kind of an awful thought really... :(
Hadn’t thought of that.
Cheers.
@@StromSports For one brief moment, I thought you were Amy Macdonald's hub, same name, wrong person though... :P
But yeah, not a fun thought knowing that I was born in '85, and possibly could have consumed those prions in tainted beef, and I'm just on a waiting game, hoping it isn't the case... :(
And this is why, since the international airports in the US have full body scanners, I asked why not have a doctor watching out for us.
@@leechowning2712 It'd be illegal to do so, due to data protection the scanners are operated by people who have no knowledge of who is going through, they only have access to an alarm to notify the security people to let them know of suspicious items on the images...
@@leechowning2712 Not sure those x-ray machines would pick it up, anyways. Besides, that would probably manage to hold up clearing security even longer; and, knowing the US, if it worked at all, people would use it for free testing, as an alternate to the ridiculously-priced system in place there now, which means that (a) they'd probably start charging a similar fee to go through it, making air travel damned near as expensive as getting healthcare; and (b) you just *know* that it would only be a matter of time before someone sued the airport for not detecting their cancer or something. :/
TL;dr: this is why we can't have nice things. Probably really would be simpler (and cheaper!) to just switch over to a proper universal healthcare system so you can just go get screened when you need to and be done with it.
But it's not a bad idea!
Great! I was just thinking I could use another panic attack whenever I misplace my keys.
you should do episodes on rabies and naegleria fowleri (100% lethal brain parasite).
He has done one on rabies already
@@martinottesen1053 this comment is from before he uploaded it, check the dates…
My favourite protein and disease class, PrPc is somewhat related to Alzheimer’s pathogenesis too (more links may have been found or disproved since I studied neuroscience almost 10 years ago though).
The cause of nvCJD is not solely the addition of cattle products into animal feed though; we used to pasturise those animal products at temperatures high enough to denature (destroy) those prions. However, somebody realised they could make a lot more money by reducing the pasteurisation temperature, which obviously requires less fuel and therefore costs far less.
The new temperature was lower than the denaturisation temperature of the prions, hence it was at that point that the prions entered the food chain and cascaded down to everything else.
Human greed is the ultimate cause of the Scrapie, BSE and nvCJD epidemics that have/will follow.
🎯
It was common practice to feed ground up waste beef to calves? That’s cold man…
En-sef-aloh-fathy (or pathy) .. Can be tricky to pronounce. Helps top think of cephal/o/pod (head foot) sef-a-lo-pod
Simon:”… collapse into a gruesome puddle of human.”
I feel attacked.
Mad respect for your flawless pronunciation of words like "ungulate spongiform encephalopathy."
/ɛnˌsɛfəˈlɒpəθi/ wrong pronunciation of encephalopathy
He didn't pronounce encephalopathy right
Correction: Prions CAN be destroyed with heat. Incineration destroys them. Of course you can't eat incinerated carbon (well you can actually but it isn't really food anymore) so, yeah, for practical purposes you can't use heat to destroy them.
Video begins at 1:16
If anyone wants to know more about this, the book “Deadly Feasts” is great
Read it. Father died from it in 96 in the US
Some of the creepiest comments I've ever read on UA-cam were about the possible connection between prion disease and hunters.
Fuggin' thanks Simon. No sleep for 3 days at least and nightmares to follow. :( Seriously though, good video as usual. Surface level, but good enough to get the point and information across to anyone watching it. Anyone wanting to learn more has a couple of good books to read.
This stuff is genuine nightmare fuel. In the mid-90s, I worked with a USAF wife. They had been stationed in the UK in the 80s. The entire family were forbidden from donating blood. Ever. I've often wondered if any of them later discovered they'd been affected.
You never fail to deliver a healthy dose of depression and despair when one needs it. Thanks for being there for us 😁
“The Family That Couldn’t Sleep” was an excellent read! FFI and rabies are some of the creepiest disease.
Okay, Simon: It isn't 1-2 million people diagnosed per year. It's 1-2 people per million.
What's a few orders of magnitude between friends?
@@disorganizedorg All the extra death in this manner.
It’s the same thing?
@@hx5525 No it isn't.
@@ZeoViolet Ah,ur right,doing the calculations it would be around 8000 ppl per year
Unfortunately one of my cousins daughter has a Prion disease. It's been really difficult. I believe symptoms began showing up when she was around 3 years old.
My mother is trying to help a friend who is working to cure fatal familial insomnia because this friend is gonna develop it.
Disease videos are my FAVORITE
Weaponizing? Well, that's GOOD. Just couldn't finish the day without that. I thought I had everything I needed till I heard about this. Thank GOD someone is going to make it easily available.
Bro where was this when I was doing my report about prions. This would’ve been an absolute life saver. Great video btw absolutely loved it, when u said the names of the diseases I spoke to myself saying which one was which absolutely brilliant👍
It’s not so much why the proteins are doing it but why they exist in a way the body can’t handle.
yea i think theyre more comparable to cancers than to parasites, viruses, or bacteria. its more like something in the body fucking up in a very destructive way, rather than some separate entity trying to utilize your body for its survival like a parasite/virus/bacteria
Simon Whistler : give him a spot on your feed and come morning he's everywhere.
Loveable little Super-Prion.
I have been studying prions on an amateur level since I learned about it. Cannibals are one of my favorite subjects. I also hunt and now cwd is spreading at a rapid rate.
@gg gg they think cwd is actually an unintentional consequence of American hunting laws. To keep people from poaching or over harvesting it is illegal for me to sell game meat(any products I obtained from public means, hunting trapping or gathering), I'm split on this law as I get it but it mine now so... but that's a different conversion. Anyway as it is illegal for me to sell game meat and game meat is desirable people in the Midwest started farming deer and elk(techniquely no longer game, it's farmed meat now) deer farming being the questionable practice. Deer are fairly independent they live in small groups. They think a deer in a farm was exposed and it exploded from there then wild deer visited the farms and it got into the wild so now it's in most of the US.
@gg gg off the top of my head I wanna say the early 80s. I'll ask my buddy he probably remembers when cwd became an issue.
@gg gg I live in Oregon and 20 years ago it was a Midwest thing no concern of ours. Last year we passed a law now out of state game parts allowed. Trying to keep it out now. The 90s is when it started spreading lime wildfire.
@gg gg @john veach
Deer farming was the culprit… where deer were fed byproducts that included brain and spinal cord material (yes… again.) Now the genie’s out of the bottle and it’s spreading through the wild population. So far, no humans have caught cwd, so it might not affect us… but only time will tell.
My Mom Passed away from CJD. From normal to dead in 12 months. I will never forget how fast that decline was.
I suffered so much watching and taking care of her all that time. Need a cure fast.
Correction: Prions _can_ be destroyed with chemicals, radiation or heat. The problem is, enough treatment to kill the prions is far more than the host can take before it kills _them._
"Inhalation of aerosolized brain matter" is a solid contender for most fucking _insane_ sentence I've ever heard.
Great video! But encephalopathy is not pronounced with a K, but with a C ("En-Se-Fa-lopathy"). Also PH is pronounced F.
I remember learning about Fatal Familial Insomnia when I was very very little, I don't know where I learned it from, but I've always been 'aware' of its existence.
I had no idea it was classified as a prion disease, another topic I like to learn about often. I hope we can learn more about it all some day, its very interesting, prions.
That was a terrible choice of video to watch while making meatloaf. 🤣